Documente Academic
Documente Profesional
Documente Cultură
The Surgical
Examination of Children
Second Edition
Authors
John M. Hutson, AO, BS, M.D. Spencer W. Beasley, MB. ChB, M.S.
(Monash), M.D., DSc (Melb) (Melb), FRACS
FRACS, FAAP (Hon) Paediatric Surgery Department
Department of Urology Otago University
Royal Children’s Hospital Christchurch Hospital
Melbourne New Zealand
Australia
This book aims to teach medical students and other medical and clinical
personnel how to perform a clinical examination in the infant or child who
has a potential surgical condition. Most textbooks of paediatric surgery focus
on the pathological classification and overall management of disease, rather
than on the practical details of how a diagnosis is made. However, no matter
how much knowledge trainees and surgeons have about diseases and their
treatment, this is useless if the diagnosis is wrong: the foundations must be
solid if we wish to construct a sophisticated plan of management. In clinical
practice, experienced physicians and surgeons usually use a problem-oriented
approach to clinical diagnosis, yet this is rarely taught to students. This book
attempts to redress this imbalance by providing a clinical approach to the
patient which the student can learn with a minimum of factual information.
Therefore, it should remain useful to the practitioner throughout his or her
medical career. This book includes a detailed coverage of the common pre-
sentations of common diseases and does not attempt to cover all aspects of
the presentations of uncommon diseases unless their recognition is important
for the well-being or survival of the child. With the increasing sophistication
and specialization of children’s hospitals, the exposure of medical students to
rare diseases has increased in recent years. This is a counterproductive trend
since it means they obtain little experience in the range of problems that they
will meet in practice subsequently. For this reason, the commonest problems
are discussed in the early chapters of the book, and the last few chapters deal
with uncommon or postgraduate topics.
Clinical examination is a very subjective process: one should not shy away
from this in a quest for objectivity, but rather one should use deliberately the
subjective nature of the mind to advantage.
vii
Acknowledgements for the First Edition
ix
Acknowledgements for the Second Edition
Sincere thanks to Shirley D’Cruz for scanning the original text and with great
patience turning it into a useable electronic document, thereby enabling this
book to be updated and edited for a new edition. We also thank Lloyd Ellis
for transforming the negatives of the original diagrams into digital format.
xi
Contents
xiii
xiv Contents
Clinical diagnosis in any branch of medicine in sequence is the chosen one, beginning with 1
requires more finesse than knowing merely how and continuing for up to 999 questions until the
to elicit physical signs. It is often a great mystery correct number is identified! An alternative
to medical students how an experienced doctor approach is based on the realization that a ‘yes/
can reach a correct diagnosis with the minimum no’ question identifies specifically a number in a
of history and examination while they may have two-element set. Therefore, all that is needed is to
spent an hour or more with the same patient to no divide the 1,000 choices into two and ask: ‘Is the
avail. They may despair of ever attaining the same number greater than 500?’ The answer will
level of expertise. Shrewd students, however, sus- immediately eliminate half the possibilities. With
pect that their senior colleague uses a direct this simple device, the unknown number can
approach to reach the diagnosis, avoiding the always be found in ten questions or fewer. The
lengthy process of aimless and exhaustive his- first technique is analogous to the student who
tory-taking and examination, much of which may asks every conceivable question and then exam-
be irrelevant to the patient’s needs. It is the aim of ines every part of the body from head to toe,
this book to teach students of paediatric surgery before thinking about the possible diagnosis. The
how to improve their clinical acumen and to latter approach echoes that of the experienced
enable them to reach the correct diagnosis by the physician who reaches the diagnosis after limited
simplest route. This direct approach to clinical questioning and physical examination.
diagnosis is problem-oriented, where the diagno-
sis can be anticipated by the early elimination of
alternatives. It is the same method that is used in What Knowledge Is Needed
‘decision trees’, algorithms and flow charts. to Make a Diagnosis or
Although students may be aware already of this Solve a Clinical Problem?
type of decision-making process, its formal
description in the following pages underlines the First, an understanding of the scientific founda-
strength of the technique and demonstrates how it tions is required; in paediatric surgery, this
can be applied in clinical diagnosis. Consider the involves knowledge of the embryology, anatomy
following difference in approach to the simple and physiology of the neonate and infant and of
mathematical problem of identifying an unknown normal growth and development. Secondly, it is
number between 1 and 1,000 by asking questions essential to be familiar with the common patho-
with a ‘yes’ or ‘no’ answer. How many such ques- logical processes (which vary in different times,
tions are needed? There are two ways of solving cultures and localities) to ensure a sensible dif-
this problem. One is to ask whether every number ferential diagnosis.
Why Does a Problem-Oriented evident that the secret of rapid and accurate diag-
Approach Work? nosis lies in having a prepared mind at the com-
mencement of the interview and examination.
The answer to this question is not obvious immedi- Only then can important clues actively be sought
ately, yet it is a fundamental principle of problem- from the history and examination.
solving by observation. In a clinical context, the
clues to the diagnosis are often subtle and will be
found only by someone who knows exactly where Does the Problem-Orientated
to look and then knows how to interpret the findings. Approach Work for Fields of Medicine
A physical sign may be detectable if the examiner Other Than Paediatric Surgery?
is working with a hypothesis and specifically looks
to see whether the sign is present, but will be missed The approach is applicable to all branches of
on ‘routine’ examination. It is rare that important medicine, but the lack of degenerative diseases in
evidence from the history or physical examination paediatrics means that multiple disease processes
is found by accident – the observer must be con- are less likely to account for the presenting com-
sciously prepared for it (Fig. 1.1). plaint; this is an example of the principle known
While it may seem improbable at first, it is a as Ockham’s razor. William of Ockham was an
well-recognized characteristic of the brain and English monk who provided scientific method
senses to ignore apparently irrelevant observa- with its fundamental principle 700 years ago
tions. Pasteur expressed the sentiment when he when he suggested we should always favour sim-
said: ‘Fortune favours the prepared mind’. Once ple explanations when trying to account for the
this limitation of the senses is appreciated, it is world’s mysteries. In paediatric surgery, this
Presenting
complaint
Basic
principles of
embryology and
‘Routine’
physiology
history and
Basic
examination
principles
of
pathology
Important
Hunch about
clues
likely diagnosis
missed
means that a range of different physical signs are gery. While no attempt has been made to provide
most likely to be caused by a single anomaly. a comprehensive description of embryology,
those aspects which are useful for clinical pur-
poses have been highlighted.
If Anticipation of the Diagnosis Comes
with Experience, How Can the Student
Hope to Learn the Technique While Specific Principles of Clinical
Still Inexperienced? Examination
This is the ‘Catch-22’ of medical education. It is The flow chart presented here (Fig. 1.2) provides
our belief that the technique can be learnt after a plan of how a goal-oriented interview and phys-
limited clinical experience, subject to certain ical examination can be conducted. Important
conditions. points in each step are described below.
First, the significance of each piece of evi-
dence must be considered from the beginning of
the interview, so that a simple list of differential Introduction
diagnoses can be formulated early. Failure to rec-
ognize the significance of information as it is pro- A medical interview, like any other social inter-
vided will prejudice recognition of those clues action, needs to conform to social customs. First
which assist in the identification of possible diag- impressions are important, and these can be used
noses. Therefore, when first learning the method, to advantage. The style adopted should be a per-
students must ask themselves continuously what sonal one accepting that there are several mini-
the evidence suggests and which relevant ques- mum requirements which are necessary,
tions should be asked. Later, with experience and regardless of your personality. It is important to
practice, this process occurs automatically. introduce yourself to both the parents and the
Secondly, it must be understood that many child by telling them your name and position.
problems can be solved by using basic principles Many parents (and even children) will expect you
and that this can compensate in part for lack of to shake hands, and this may be an important step
experience. The basic principles are derived from in the social interaction. It represents mutual rec-
knowledge of the scientific foundations of sur- ognition that you will be friends and not antago-
gery and paediatrics, many of which already will nists during the interview, and helps both parties
be known to the student. The following chapters to relax. Many students and, unfortunately, many
are arranged in such a way that the most impor- doctors in senior positions neglect to tell the par-
tant principles are highlighted at the end of each ents their name and position. No one wants to be
section for easy reference as ‘key points’. For confronted by a nameless ‘white coat’ (Fig. 1.3):
many students, several years will have elapsed personal accountability is important even when
between the time they learned the basic sciences still a student. It is not acceptable to remain anon-
and their first encounter with paediatric surgery ymous through lack of social confidence; such
– yet their knowledge of the basic sciences is action may insult the child and parents and make
essential if their examination of children is to be rapport difficult to achieve.
effective. In the following chapters, it will become
apparent that examination in paediatric surgery
demands the fusion of the preclinical sciences Gain Rapport
such as embryology, anatomy and physiology,
with the clinical science of pathology. In no other This is an extension of the introduction and is
branch of medicine is a knowledge of embryol- directed primarily at gaining the friendship and
ogy more useful, but unfortunately many students confidence of the child. If the child can be spo-
will have forgotten their embryology by the time ken to in a relaxed fashion, the parents will be
they commence their training in paediatric sur- reassured that you are competent and comfortable
4 1 General Principles
Introduction
A Gain rapport
History/visual clues
Goal-oriented
direct questions B
Tentative diagnosis
New tentative
diagnosis
Predicted
historical clues Predicted physical
signs
Diagnosis unconfirmed
Goal-oriented or
examination unexpected findings
Laboratory
investigation
Confirmed diagnosis
Correct
treatment
family history, but it is important not to become nary diagnosis is either substantiated or refuted.
too preoccupied with these areas unless they are There is a greater chance of elucidating subtle
directly relevant to the problem. It is easy to signs and of detecting other more obvious signs
become distracted collecting unnecessary which are rarely elicited, if the examiner is look-
information. ing for particular features. A good example of a
Sometimes, cross-examination will reveal rare but important sign is perineal anaesthesia; it
clues which suggest an alternative diagnosis. is obvious when sought but would never be noticed
Should this occur, reappraisal of the evidence in a ‘routine’ examination, yet it is an essential
already obtained may allow formulation of a clue to the diagnosis of a neurogenic bladder.
revised diagnosis. Further direct questions may The goal-oriented examination will usually
then be required to document the predicted fea- confirm the preliminary diagnosis if the hypoth-
tures of the new diagnosis. This process is repre- esis is correct. With the diagnosis correctly ascer-
sented by circle A in the flow chart (Fig. 1.2). It tained, management can begin immediately.
is reasonable to proceed around the circular path Occasionally, confirmation of the diagnosis
as often as is necessary to reach a firm prelimi- depends on further corroboration by laboratory
nary diagnosis. Obviously, with greater experi- investigations, such as imaging or blood tests.
ence, fewer circuits will be needed before a
confident diagnosis is made.
Diagnosis in Doubt
Further Reading
Golden Rules
1. A ‘routine examination’ finds nothing Fulginiti V.A. (1981). Pediatric Clinical Problem Solving.
because you only see what you are look- Baltimore: Williams and Wilkins.
Green M. (1986). Pediatric Diagnosis. Interpretation of
ing for. Symptoms and Signs in Different Age Periods, 4th
Even obvious visible or palpable signs edn. Philadelphia: Saunders.
will be missed if you do not have a pos- Hutson JM, O’Brien M, Woodward AA, Beasley SW
sible diagnosis in your head, from which (2008) Jones’ Clinical Paediatric Surgery. Diagnosis
and Management, 6th edn. Blackwell Publishing,
the visible and palpable clues can be Oxford.
predicted. Beasley SW, Hutson JM & Myers NA, (1993) Pediatric
2. Use the subjective nature of the mind Diagnosis: An Illustrated Guide to Disorders of
deliberately by trying to think of a pos- Surgical Significance. London: Chapman Hall
Medical.
sible diagnosis (historical and visual
clues), and then actively search for the
evidence like Sherlock Holmes.
3. Because children are basically healthy
(no degenerative diseases that accumu-
late with age), Ockham’s razor applies:
no matter how many signs and symp-
toms there are, there is usually only one
disease or underlying cause.
Is the Baby Normal?
2
may be present.
1% malformed
40 at birth
Embryology
Embryo Errors of
(3–10 weeks) organogenesis
e.g.: oesophageal
atresia
Abnormalities
Fetus
of fetal movement,
(10–40 weeks)
compression or
disruption
e.g.: amniotic band
talipes
the basic shape and organs of the embryo form influence. If one major anomaly is present,
and this is when most surgical malformations look for others.
arise. Anomalies occurring during embryogene- 2. Multiple anomalies are related to each other in
sis may be caused by innate genetic defects or by time or space. Different parts of the body may
extrinsic teratogens (e.g. rubella or other congen- be sensitive to a particular extrinsic teratogen
ital infections, x-rays, drugs, chemicals or mater- or mutation because they are all undergoing
nal dietary abnormalities). rapid cell division and morphogenesis at the
Knowledge of the causation of many surgical same time. Also, several organs in the same
malformations is poor because the embryonic part of the body may be affected by a ‘field’
stage is the most difficult phase of pregnancy to anomaly of morphogenesis. These defects
study. Despite this limitation, there are several occur with mutations in genes controlling
important principles of surgical anomalies which embryonic segmentation (e.g. Hox genes).
are essential if the clinical situation at birth is to A good example of time-related defects is con-
be understood: genital rubella: the group of organs involved
1. Anomalies are often multiple because several depends on the age of the embryo at infection.
organs may be sensitive to the same teratogenic The heart, eyes and teeth will be affected at
Embryology 11
‘Redundant’
Common Uncommon morphogenesis
e.g.: ear tag
‘Incomplete’ polydactyly
morphogenesis Rare
e.g.: cleft lip ‘Aberrant’
malrotation morphogenesis
e.g.: sacrococcygeal
tumour
6 weeks, while at 9 weeks, the ear will be involved, (Fig. 2.3). This is a normal process in which
leading to deafness, but the eye is spared. only the timing is disrupted. Less common
The “VACTERL” association is a further is redundant morphogenesis, where the nor-
example of time-related defects, where the fol- mal process has been partially or completely
lowing abnormalities are seen in combination: duplicated. Truly aberrant morphogenesis is
V – vertebral anomaly, for example, sacral rare because the abnormality will usually
agenesis, hemivertebrae lead to death well before birth.
A – anorectal anomaly
C – cardiac anomaly
T – tracheo-oesophageal fistula Does It Matter If the Baby
E – (o)esophageal atresia Is Dysmorphic?
R – renal anomaly
L – limb anomalies, for example, deficiency Yes! Where one major anomaly is observed, the
or atresia of the radius presence of others must be sought. Dysmorphism
Imperforate anus often provides an example may indicate that the child has a number of malfor-
of space-related anomalies since it is com- mations which may be well enough known to be
monly associated with other pelvic abnormali- called a ‘syndrome’. A syndrome is a collection of
ties, such as sacral agenesis, deficiency of features or defects which is recognized as belong-
sacral nerves and pelvic floor muscles and ing to the one entity. Individual syndromes are rare
urogenital anomalies. and may carry unwieldy eponymous names which
3. The genetic control of morphogenesis is a make them an anathema to the average student or
multi-tiered hierarchy within which only clinician, although obsessive/compulsive learners
certain abnormalities are possible and only seem drawn to them. Their real importance is that
a few are common. In other words, there definitive diagnosis of the syndrome will allow
is not an infinite array of possible defects, other features of the syndrome not yet identified to
but only a small number of common lesions be actively sought, usually after consultation with a
sufficiently compatible with survival to geneticist or database. Moreover, the prognosis
reach birth. One never finds a baby with will be predicted more readily than in complex
antlers(!), but pre-auricular skin tags do cases without recognized syndromes, since out-
occur. The commonest form of abnormal come usually has been well defined by previously
morphogenesis is incomplete development reported cases. This information is essential in the
12 2 Is the Baby Normal?
}
Hand
Eye hands will usually yield significant information, even
71 %
Face
Mouth
when performed by inexperienced examiners.
Ears........10 %
Chest........8 %
Skin..........7 %
Feet..........4 %
Surgical Assessment of the Neonate
should be wrapped in plastic food wrap to pre- sounds are normal, next listen to the heart. The
vent excessive evaporative heat loss. If there are rapid heart rate of the neonate makes auscultation
no external abnormalities which require immedi- difficult for the inexperienced clinician, but an
ate attention, commence examination by observ- attempt should be made to detect murmurs and
ing the overall posture and activity of the child. abnormalities in rhythm. Confirm the location of
Does the appearance of the infant match the pre- the heart to exclude situs inversus.
sumed gestation? Next, observe the infant breath- Move to the abdomen. Here, three questions
ing. Is there tachypnoea, respiratory distress or should be answered: (1) Is the abdomen distended
cyanosis? Look for sternal retraction. Does the (or scaphoid)? (2) Are there any abnormal masses
infant seem to be ‘working hard’ with breath- palpable? (3) Is there evidence of perforation or
ing? Once respiratory distress is recognized, its peritonitis? Observation of the contour of the
cause should be established promptly. Details of ventral abdominal wall of many normal newborn
how this is achieved are provided in Chap. 20. infants provides the experience necessary to
It should not be forgotten that distension of the judge minor degrees of abdominal distension.
abdomen exacerbates respiratory distress by ele- Gross abdominal distension is obvious even to
vating and splinting the diaphragm, and interfer- the untrained eye and usually indicates bowel
ing with the mechanics of ventilation. obstruction (Chap. 21). Several organs are nor-
The colour of the skin, sclera and mucous mem- mally palpable in the abdomen: the liver, kidneys,
branes should be noted, looking for jaundice, pallor bladder and less commonly the spleen can be
or cyanosis. Turn now to inspection of the head. identified in most babies (Chap. 15). The promi-
Feel the anterior and posterior fontanelles and the nence of the vertebral column enables the pulsa-
suture lines. Assess head size (Chap. 10), measure tion of the overlying aorta to be felt easily. Clues
head circumference, inspect the eyes, test the pat- to the presence of peritonitis include redness and
ency of each nostril and look at the hard palate for oedema of the skin of the ventral abdominal wall,
evidence of a cleft (Chap. 11). Is the tongue normal tenderness to palpation and increasing distension.
in size or excessively large? Is there excessive sali- A localized perforation may become apparent as
vation? Study the shape of the face: are the ears in a fixed, tender mass.
the correct position, and is the distance between the The distal part of the umbilical cord rapidly
eyes normal? Are the pinnae fully developed, and desiccates and shrivels up to become the black
is there an external auditory meatus? Is the lower rigid umbilical stump, but remains attached for
jaw normal or underdeveloped? about 1 week before separating from the umbili-
The neck of the neonate is relatively short but cus itself. Swelling at the umbilicus is common
should be inspected for abnormal skin lesions or in the first weeks after separation, but usually dis-
deeper swellings (Chap. 9), such as a vascular appears as the umbilical cicatrix contracts. In
anomaly (e.g. a lymphatic malformation or ‘cys- most babies, there are three vessels in the umbili-
tic hygroma’). cal cord: (two arteries and a vein) the presence of
Is the chest wall normal, and is chest expan- only two vessels may be related to other congeni-
sion symmetrical? Is air entry equal on both tal anomalies. Failure of the umbilicus to contract
sides? Listen to the lung fields for breath sounds. after involution of the fetal vessels will produce
If these are abnormal, percuss the chest. The an umbilical hernia (Chap. 6).
liver, which is relatively large in the neonate, is Next examine the inguinoscrotal and perineal
percussible in the right lower chest. Dullness of regions (Chaps. 4 and 5). What sex is the child
the chest (other than the liver) suggests fluid. (Chap. 22)? Look for groin swellings indicat-
Hyper-resonance may indicate a pneumothorax, ing inguinal herniae. In boys, identify the posi-
in which case breath sounds will appear distant tion of the testes to determine whether they are
on auscultation. If chest movement and breath fully descended. Remember, inguinoscrotal
14 2 Is the Baby Normal?
descent occurs at about 7 months’ gestation such Table 2.1 Assessment of gestation
that the testes will not be in the scrotum if the Ears Cartilage
infant is extremely premature. Inspect the fore- Form (incurving)
skin and location of the urethral meatus. A dorsal Breast Breast tissue
hood of foreskin and chordee is suggestive of Nipple and areola
hypospadias. Sole creases
In both sexes, look for the anus. Two ques- Skin Thickness
tions must be answered: (1) Is it in the correct Fat deposition
Desquamation
position? (2) Is it patent? If the anus is absent in
Genitalia Testes
the male, carefully inspect the median raphe of
Scrotum
the perineum, scrotum and ventral surface of the
Labia majora and clitoris
penis for a fistulous opening which may be
Vernix
stained black with meconium. In the female, part Hair
the labia to inspect the vestibule for an opening. Skull
Where the anus is anteriorly displaced, its pat- Posture Resting posture
ency must be established (Chap. 21). Recoil of extremities
Examine the limbs for congenital dysplasia of Muscle tone (horizontal
the hip, club foot and other orthopaedic anomalies suspension)
(Chap. 13). Count the fingers and toes. Inspect the
skin for lesions: certain hamartomata are not pres-
ent at birth but become apparent in the first weeks
of life as slightly elevated red or purple lesions 34 weeks
the edge of the superior part of the pinna com- At about 34 weeks’ gestation, the areola
mences at about 34 weeks and proceeds down- becomes raised and the nipple well defined. Two
wards towards the lobe. If no incurving is evident, weeks later, the breast tissue begins to increase in
the gestation is likely to be less than 34 weeks. size to form a nodule 1–2 mm in diameter, and by
Where incurving is well defined and has reached term, this breast nodule has reached 6–10 mm in
the lobe, the baby is near term (Fig. 2.6). diameter (Fig. 2.7).
Genitalia
In the male, the testes are not palpable outside the
inguinal canal until after 28 weeks of gestation,
when they commence their descent into the
32 34 36
Areola flat Areola raised Breast
34 36 40 Nipple formed nodule
Fig. 2.6 Development of the folds of the pinna relative to Fig. 2.7 Breast bud development compared with gesta-
gestational age tional age
28
External
ring
36 40
weeks
37 Neurological Development
A full neurological examination of a neonate in
the first hour of life is not usually appropriate or in
Scrotum the best interests of the infant, but three character-
Fig. 2.9 The timing of testicular descent through the
istics can be assessed readily: (1) resting posture,
external ring of the inguinal canal and down into the (2) recoil of the limbs and (3) muscle tone during
scrotum horizontal suspension. The assessment of nervous
development may be affected significantly by
perinatal mishaps, such as asphyxia:
1. Muscle tone first appears in the lower extremities.
Until 28 weeks, the infant will lie in an extended
position (Fig. 2.12). Hip flexion develops first,
and by 34 weeks, the infant assumes a ‘frog’ posi-
tion. The arms are flexed by 36 weeks.
2. Recoil to the resting posture after extending
28 36 40 the limb can be seen to follow the acquisition
Weeks of the flexed posture of that limb. Recoil of the
Fig. 2.10 Scrotal development related to gestational age
legs begins at 34 weeks and that of the arms by
about 36–38 weeks.
3. Horizontal suspension can be performed dur-
scrotum. They are usually in the scrotum by ing weighing of the infant. The manoeuvre
37 weeks (Fig. 2.9). tests tone in the trunk and extremities. The
There are no rugae in the scrotum until 28 infant is allowed to hang in the prone position
weeks, and only a few appear in subsequent (face down) over the hand of the examiner
weeks until 36 weeks, at which stage they become with the arms and legs dangling (Fig. 2.13).
prominent, particularly in the anterior half of the Until 34 weeks, the infant appears hypotonic
scrotum. At term, the scrotum is pendulous and the limbs hang straight. After this time,
(Fig. 2.10). The clitoris is prominent and the labia there is some flexion of the limbs and straight-
majora small, poorly developed and widely sepa- ening of the body. The head cannot be raised
rated until 36 weeks. In the last month before effectively until term at which stage the limbs
term, the labia majora enlarge to nearly cover the are tightly flexed.
Surgical Assessment of the Neonate 17
28 34 36
weeks
a b
28 34
Abdominal pain is extremely common in children treated before peritonitis develops. Therefore,
and may reflect a variety of conditions (Table 3.1). examination of the abdomen is directed at local-
Often, the cause cannot be determined with cer- izing the likely origin of the pain and detecting
tainty, or is caused by pathology unrelated to the the presence of peritonitis. Peritonitis is a sur-
abdomen. Pain which lasts for more than 4–6 h, gical emergency and should almost always be
which is becoming worse or is associated with treated by operation within hours of diagnosis.
persistent vomiting or prolonged diarrhoea, When a child presents with persistent or worsen-
should be taken seriously and a surgical cause ing abdominal pain, every effort must be made to
excluded. If appendicitis is the cause, it is best obtain a clinical diagnosis.
Frequently, children with significant abdomi-
Table 3.1 Differential diagnosis for acute abdominal nal pain are anxious and scared. Their fear that
pain palpation of the abdomen will cause further suf-
Very common Acute appendicitis fering may make them appear uncooperative.
Non-specific viral infection Patience and skill are needed to assess the abdo-
(mesenteric adenitis) men accurately to avoid the disaster of not recog-
Gastroenteritis nizing significant intra-abdominal pathology.
Constipation Where children are old enough to understand,
Urinary tract infection each stage of the examination must be explained
Less common Intussusception before it is commenced so that they know what to
Lower lobe pneumonia expect. The clinician must appear understanding,
Intestinal obstruction
sympathetic and gentle, and it is helpful to
Congenital (1 %)
develop a rapport with the child before commenc-
Adhesions (2 %)
ing palpation of the abdomen. Interesting toys, a
Urinary tract obstruction
torch or stethoscope may act as a distractor dur-
Strangulated inguinal hernia
Rare Henoch-Schönlein purpura
ing examination in the smaller child. Most chil-
Primary peritonitis dren are cooperative if they are comfortable with
Pancreatitis the situation and if they see that they are not
Diabetic ketoacidosis going to be hurt unnecessarily.
Lead poisoning In the vast majority of children with abdomi-
Acute porphyria nal pain of unknown cause, the symptoms resolve
Herpes zoster spontaneously over several hours. At the other
Sickle-cell anaemia end of the spectrum, abdominal pain may reflect
Haemophilia (retroperitoneal significant pathology and necessitate precise
haematoma) clinical assessment before the appropriate
Appendicitis
Intussusception
Incidece
Mesenteric
adenitis
Volvulus
Cholecystitis
0 5 10 15
Age Years
therapeutic measures can be undertaken. In young lasting several days, with general malaise, recent
children, infection outside the abdomen (e.g. diarrhoea and the passage of mucus rectally, sug-
lung, hip) may be interpreted as abdominal pain. gests a diagnosis of untreated appendicitis caus-
This pain must be distinguished from pain arising ing a pelvic abscess, and so the clinician should
within the abdomen. As a general rule, conditions perform a digital rectal examination to feel a ten-
which cause peritonitis need surgery, and there- der swelling bulging from the anterior rectal wall.
fore one of the more important aspects of the A 7-month-old child with episodes of screaming
examination of the abdomen is the ability to and pallor accompanied by vomiting should be
detect the presence of peritonitis. subjected to a careful examination of the abdo-
The age of the patient is helpful in diagnosing men in search of an intussusception mass. Failure
acute abdominal disease because some disorders are to make the appropriate provisional diagnosis
confined largely to specific age ranges (Fig. 3.1). after taking a history will usually result in failure
Intussusception occurs rarely in children over to detect the key physical sign which will tend to
3 years of age and, when it does, may signify the confirm the diagnosis. The importance of a care-
existence of a pathological lesion as the lead point. ful history cannot be over emphasized.
Appendicitis is uncommon below the age of
three, but in the older age groups becomes the
predominant condition requiring surgery. General Considerations
Knowledge of the age of the patient, supple-
mented by a detailed history of the onset, dura- Physical Characteristics
tion, location and characteristics of the pain and of the Abdomen in Children
related symptoms, provides substantial diagnos-
tic evidence even before physical examination is The shape of the abdomen in an infant differs
commenced. This allows a physical examination from that of an older child in three respects:
directed at confirming or refuting the provisional 1. It is more protuberant (Fig. 3.2).
diagnosis and obviates the need for many unnec- 2. It is wider (Fig. 3.2). In the infant, the abdo-
essary laboratory and radiological investigations. men tends to bulge between the costal margin
For example, a history of lower abdominal pain and the iliac crest. This is one reason most
General Considerations 21
Width>Height
Height >
Width
Pouch of
Douglas
Patent processus
vaginalis
Scrotum
Midgut
Hindgut
As with most autonomic pathways, localization of cutaneous to the shoulder region. Biliary colic
pain is not precise and the distribution described is rare in children but may produce pain in the
serves as a rough guide only. Localized distension back, below the inferior angle of the right
with peristalsis against obstruction causes colic, scapula. Uterine and rectal pain may be referred
whereas inflammatory lesions produce constant to the lumbosacral region of the back, while
pain, usually in association with anorexia and loin pain emanating from the kidney may radi-
nausea. It is the visceral pain that so commonly ate to the ipsilateral testis. The close proximity
causes the vomiting seen early in the develop- of the ovary to the obturator nerve as it courses
ment of appendicitis and other inflammatory on the lateral pelvic wall accounts for the pain
lesions of the abdomen. radiating to the inner aspect of the thigh in
In contrast, irritation of the parietal perito- ovarian disease.
neum supplied by segmental somatic nerves is
localized, sharp, continuous and made worse by
movement. Not all parts of the parietal perito- Masses Palpable in the Normal
neum are subserved by the segmental nerves, Abdomen
which also supply the muscles of the ventral
abdominal wall. Irritation of the pelvic perito- Faeces may be palpable in the colon and can be
neum produces no abdominal wall guarding. distinguished from pathological masses in that
Rectal examination may be the only way to diag- they are indentable (see Chap. 15, Fig. 15.2).
nose pelvic peritonitis. Their distribution follows the line of the colon,
Referred pain is not recognized commonly and it must be remembered that the sigmoid colon
in children, but when appreciated may provide may lie in the right iliac fossa. The lower pole of
valuable clues as to the underlying pathology. the right kidney is often palpable with deep inspi-
Irritation of the underside of the diaphragm by ration as the diaphragm pushes the kidney down-
blood or pus may produce pain referred to the wards. The liver causes the right kidney to assume
shoulder tip. The explanation for this is that a slightly lower position than the left. The liver in
the phrenic nerve which supplies the diaphragm infancy is often palpable 2–3 finger breadths
is largely made up of fibres from the fourth below the right costal margin. Further details are
cervical nerve root, of which other fibres are found in Chap. 15.
24 3 Abdominal Pain: Is It Appendicitis?
Table 3.2 Characteristics of the pain of the development of parietal peritoneal signs
1. Time of onset Duration (see the section on appendicitis). The exten-
2. Location At onset sion of pain from a lateral position to involve
At examination the whole abdomen suggests that the pathol-
‘Referred’ pain ogy has extended to other parts of the perito-
3. Severity Mild to severe neal cavity. For example, after a blow to the
Progression left upper abdomen that has caused splenic
Alleviating factors injury, pain may be first located in the left
Exacerbating factors hypochondrium but with continued bleeding
4. Type Colicky progresses to generalized abdominal tender-
Constant
ness. Pain commencing in the right iliac fossa
5. Associated symptoms
which extends across the midline may indicate
extension of peritonitis following rupture of an
inflamed appendix.
The History
Duration of Pain
Septic arthritis
Shifting of Pain of hip
Torsion of
testis
Movement of pain away from a vague central
location to one or other side is often indicative Fig. 3.7 Extra-abdominal illnesses causing abdominal pain
The History 25
The child with colic is restless and may writhe Vomiting commonly accompanies abdominal
around unable to obtain a position of comfort. pain in children because autonomic reflexes stim-
On the other hand, children with peritonitis ulate vomiting in response to any inflammation
will remain still and dislike being disturbed or or severe pain. The significance of the symptom
handled. Anything that causes movement is determined by:
between adjacent surfaces of inflamed perito- 1. The relationship of its onset to the develop-
neum will exacerbate the pain. If they walk, ment of pain
they will do so slowly, bent forwards with both 2. Its frequency
hands holding their abdomen in an attempt to 3. The nature of the vomitus
prevent movement of the peritoneal surfaces In appendicitis, vomiting generally commences
(Fig. 3.8). several hours after the onset of pain. It is unusual
It will hurt to cough, sneeze, vomit or to take a for it to appear before the pain except in the very
deep breath. Where the inflammation involves small child where the time of onset of the pain may
the pelvis (as in pelvic appendicitis), micturition be difficult to ascertain. In acute colic of the ureter,
and defecation will exacerbate the pain. the onset of vomiting coincides with that of pain,
In toddlers, pneumonia may masquerade as both being sudden and dramatic. In obstruction of
abdominal pain which is made worse by deep the intestinal tract, the onset of vomiting is depen-
inspiration and relieved by breathing quietly. It is dent on the level of obstruction. Where the obstruc-
caused by abdominal muscle contraction to splint tion is high (e.g. duodenum or upper small bowel),
the diaphragm. vomiting commences early, but where the obstruc-
tion is low (e.g. distal small bowel or colon), it may
be a day or longer before vomiting appears.
The frequency of vomiting correlates with the
severity of the pathology. For example, in acute
appendicitis, severe vomiting is often associated
with rapid progression of abdominal signs and
may indicate an obstructed appendix which is
likely to perforate early. On the other hand, in
Pained facies distal intestinal lesions (e.g. intussusception), the
Bent forward frequency of vomiting may not be impressive and
Slow shuffle
Hands supporting may belie the seriousness of the underlying
abdomen pathology. Proximal obstruction of the bowel is
Prefers to lie still associated with frequent and forceful vomiting.
It is essential to determine whether the vomit-
ing contains bile (Table 3.3). The first clue to the
life-threatening condition of malrotation with
volvulus may be bile-stained vomitus. In this
condition, there is obstruction at the second part
of the duodenum from twisting of the midgut.
Where volvulus is suspected, early confirmation
of the diagnosis by a barium meal or ultrasonog-
raphy and treatment by operation is required.
Severe gastroenteritis with profuse vomiting also
can produce bile-stained vomiting. In distal small
Fig. 3.8 The ‘appendix shuffle’. Movement of adjacent
inflamed peritoneal surfaces makes the pain worse. The
bowel obstruction, the vomitus is initially of
pain can be minimized by adopting this posture whilst gastric contents, followed by bile-stained mate-
walking rial and, ultimately, yellow/brown feculent fluid.
26 3 Abdominal Pain: Is It Appendicitis?
Table 3.3 Relationship between type of vomitus and its underlying cause
Vomitus Cause Reason
Bile-stained Malrotation with volvulus Duodenum obstructed just beyond
the ampulla
Gastric contents, then bile Gastroenteritis Inflamed stomach or secondary to
pain/ileus
Gastric contents, then bile, then Intestinal obstruction Fluid collection above distal block
faecal fluid
Not bile-stained Pyloric stenosis Pylorus blocked (above ampulla)
Blood (including ‘coffee grounds’) Oesophageal varices Portal hypertension
Gastritis Infection/corrosion/aspirin ingestion
Gastro-oesophageal reflux Oesophagitis
Table 3.4 Relationship between abnormal stools and their underlying cause
Stools Cause Reason
High volume and watery Gastroenteritis Primary effect on absorption
Small volume and mucousy Appendix abscess Secondary irritation of the rectum
Initial evacuation then Intussusception Mass induces reflex emptying and
blood ± mucus vascular compression leading to
venous congestion
In pyloric stenosis, pyloric outlet obstruction (Table 3.4). The significance of blood in the bowel
means the vomitus contains no bile. In patients with actions is discussed in more detail in Chap. 16.
a long history, development of gastritis may cause
the vomitus to be blood-tinged, a feature also seen
with other causes of gastritis. Copious quantities of Preparation for Examination
fresh blood in the vomitus are suggestive of oesoph-
ageal varices or acute gastritis such as that caused Children should be positioned on the examination
by aspirin ingestion (Table 3.3). couch so that they are comfortable and in a way
The pattern and character of bowel actions may which facilitates full examination of the abdomen.
indicate the diagnosis (Table 3.4). In acute gastro- An explanation of each manoeuvre gains the
enteritis, there is dramatic onset on loose stools child’s confidence and provides reassurance which
which, in young children, may result in loss of helps them relax. The examination must be per-
continence. The volume of stool passed is usually formed in a warm room, particularly with infants
considerable and may be associated with vomiting or small children where heat loss can occur
and colicky abdominal pain (‘cramps’). It is quickly. If the examination is to take place in the
unusual for the symptoms to persist for more than ward, the curtains should be pulled to avoid dis-
24–48 h. The passage of a small volume of loose tractions and provide privacy. Most pubertal and
motions, shortly following the onset of pain, is a many younger children are hesitant to remove
feature of appendicitis in many children, especially their clothing unless privacy is assured. The cloth-
when the appendix is retro-ileal. Persistence of ing should be removed to expose the abdomen,
‘diarrhoea’ with or without the passage of mucus chest and external genitalia. Testicular torsion and
in the presence of low abdominal pain is sugges- strangulated herniae will be overlooked unless
tive of a pelvic abscess, of which the most com- this is done, since in these conditions the pain
mon cause is an unrecognized pelvic appendicitis. may be referred to the ipsilateral iliac fossa and
In intussusception, small amounts of loose flank. The child should lie comfortably in a supine
motions, which may subsequently be tinged with position with a pillow behind the head. The torso
blood and mucus (‘red currant stools’), are passed must be symmetrical with the legs together and
Is There Peritonitis? 27
Correct Incorrect
arms at the side (Fig. 3.9). Failure to ensure that a feeds. During the drive to hospital, the child may
child is positioned correctly may result in an erro- complain that every time the car goes over a
neous assessment of the site and severity of ten- bump the pain is made worse.
derness, muscle tone and guarding. Contraction of the diaphragm causes the
abdominal wall to move with respiration. Such
movement is painful in peritonitis, and the child
Is There Peritonitis? will use the chest wall muscles in breathing to a
greater degree in an effort to keep the abdomen
In the child with acute abdominal pain, the most still. This is assisted by tonic contraction of the
important question is whether they have peritoni- abdominal wall muscles which, in a thin child, is
tis. The infant is less able than the adult to con- seen as prominence of the rectus abdominis mus-
tain the spread of intraperitoneal sepsis because cles at rest in an abdomen which moves little with
the greater omentum is small and poorly devel- respiration. Children should be asked to puff out
oped. Peritonitis in this group progresses rapidly their abdomen as far as they can. In the absence of
and, if untreated, may result in septicaemia and peritonitis, this is a painless manoeuvre, but the
death. The presence of peritonitis is an indication child with peritonitis is either unable or unwilling
for surgery in virtually every situation. It is to protrude the abdomen: attempts to do so cause
imperative, therefore, that the clinician is familiar severe pain. Likewise, in peritonitis, attempts to
with the signs of peritonitis, which in the infant draw in the abdomen and ‘be as thin as possible’
or young child may be difficult to elucidate. will result in severe pain (Fig. 3.11). The volun-
Clinical assessment of peritonitis or inflam- tary excursion of the abdomen is reduced.
mation of the peritoneum involves the demon- Coughing and sitting up cause similar discomfort,
stration of pain produced by movement between which adds support to the findings above.
adjacent inflamed peritoneal surfaces. Certain The tests so far described are dependent on the
clues may be obtained from the history. They cooperation of the patient to move the peritoneal
may be reluctant to move about because move- surfaces. The following tests detect peritoneal
ment makes the pain worse, and may refuse to irritation more directly, and of these, the detec-
run. They may walk stooped forwards clasping tion of guarding is the most specific and
their abdomen with both hands (Fig. 3.8). Older sensitive.
children may go to bed and lie motionless Children with a sore abdomen fear palpation
(Fig. 3.10) preferring to be left undisturbed. An as it will increase the pain. For this reason, it is
infant may protest at being handled and refuse imperative to gain as much information as
28 3 Abdominal Pain: Is It Appendicitis?
Normal Peritonitis
possible before palpating the abdomen. A useful abdominal wall, and the middle finger of the
test is to hold the child firmly on each side of the other hand taps the back of the middle finger on
chest wall with the fingers and palms of the hands the abdomen. This sends an impulse through the
and to shake the chest from side to side. The pres- abdominal wall and jars the peritoneum, causing
sure on the chest wall causes no discomfort, but if discomfort where there is inflammation of the
there is peritonitis, the shaking will cause pain in peritoneum. The examination is commenced
the abdomen. A similar manoeuvre can be con- away from where maximal tenderness is
ducted by holding the pelvis below the iliac crest suspected.
or over the greater trochanters firmly between the The existence of involuntary contraction
hands (Fig. 3.12) and assessing the degree of dis- (‘guarding’) of the muscles of the abdominal wall
comfort caused by shaking the pelvis quickly in a can be confirmed also by gentle palpation start-
lateral direction. ing with the region of the abdomen in which the
Initially, the pelvis is rocked gently to avoid least tenderness is anticipated. For example,
excessive discomfort in the child who is sus- where a child complains of maximal pain in the
pected of having peritonitis. right iliac fossa, examination should start below
The next step in the examination is to elicit the left costal margin. It is essential that initial
percussion tenderness. This is done in exactly palpation is extremely gentle in order to (1) main-
the same way as in percussing the chest wall: the tain the child’s confidence and to enable them to
fingers of one hand are placed gently on the remain relaxed until the degree of tenderness has
Is There Peritonitis? 29
been established and (2) detect subtle differences tips of the fingers should gently test the tension in
in muscle tone. Guarding is sometimes described the muscles as they progress around the abdo-
as being either ‘voluntary’ or ‘involuntary.’ men. If abdominal wall spasm and tenderness are
Voluntary guarding is a conscious tightening of present, the patient has peritonitis and no further
the abdominal wall musculature as a protective palpation is needed. The most sensitive areas for
device in anticipation of tenderness. demonstrating guarding are lateral to the rectus
It suggests apprehension, but may or may not abdominis muscle (Fig. 3.14), and it is often use-
signify underlying peritoneal irritation. In-voluntary ful to assess muscle tone on both sides simultane-
guarding is a reflex spasm of the muscles, even in ously (Fig. 3.14). Each side is palpated alternately
the absence of palpation, to immobilize the adja- to allow detection of minor differences in tone. If
cent parietal peritoneal surface (Fig. 3.13). The the first journey around the abdomen elicits little
tenderness, the process should be repeated with
firmer palpation but is unlikely to reveal much
more information.
The exception to this is seen when deep palpa-
tion in one area causes pain in an adjacent area,
ow
! the most well-known example of which occurs in
appendicitis where deep palpation of the left iliac
fossa may cause sharp pain in the right iliac fossa
(Rovsing’s sign) (Fig. 3.15). This occurs because
deep indentation of the peritoneal cavity in the
left iliac fossa causes displacement of loops of
bowel in the region of inflammation in the right
ow iliac fossa (Fig. 3.16).
!
Where the child refuses to relax, or it is sus-
pected that guarding is voluntary, percussion of
the abdomen best identifies those with genuine
guarding. In children, percussion produces a
vibration of the underlying tissues, which is not
dampened by the obesity often present in adults.
Children under 3 years of age may be uncoop-
Fig. 3.12 Is peritonitis present? Shaking the chest or pel-
vis from side to side will cause the child to grimace with erative and extremely difficult to examine. Severe
abdominal pain abdominal pain from peritonitis may be the reason
Muscle contraction
Spinal reflex
Fig. 3.13 Guarding: the
involuntary reflex contraction
or spasm of anterior
Inflammatory
abdominal wall muscles when Inflamed mass
the anterior parietal perito- peritoneum
neum is inflamed. Spasm
persists even when the child is
asleep or distracted
30 3 Abdominal Pain: Is It Appendicitis?
Pain
Press
Inflamed loops and peritoneum
The demonstration of rebound tenderness as vomiting at some stage of the history, although it
performed in an adult is not justified in the child may have stopped by the time of presentation.
because it provides unreliable information and, if Pyrexia, fetor and furred tongue may be present
peritonitis is present, is cruel. Rebound tender- but are not specific to peritonitis. A tachycardia is
ness elicited by gentle percussion is a more valu- usually present, but a normal pulse rate for age
able sign (Fig. 3.19). does not rule out peritonitis.
In long-standing peritonitis, the signs of
septicaemic shock develop. Loops of intestine
Non-specific Signs of Peritonitis become distended and paretic, causing abdomi-
nal distension. The abdomen is silent on aus-
Facial expression will show the child in pain. The cultation. Vomiting of small bowel contents
complexion may be pale or flushed. Sometimes, develops, and the respiratory rate becomes
the cheeks are red in association with perioral rapid. It is unfortunate if signs have progressed
pallor. In early peritonitis, and in the young child, to this stage before a diagnosis of peritonitis is
the face may provide few clues. There is usually made.
Appendicitis
Cholecystitis
Pancreatitis Appendicitis commences with a dull continuous
central abdominal pain which moves after 6–24 h
to the right iliac fossa where it is perceived as
Appendicits
Mesenteric being more severe and sharper in nature. The pain
adenitis is accompanied by anorexia and nausea, and in
most children vomiting occurs. There may be
some alteration in bowel habit.
There is an anatomical and physiological basis
Fig. 3.17 Sites of maximal tenderness of abdominal for these symptoms. The appendix is an appendage
diseases
Skin
Muscle
Peritoneum
Skin
2
Muscle
Inflamed
peritoneum
rectum by the adjacent inflammatory mass and doubt about the diagnosis, where other diagno-
(3) marked tenderness anteriorly on rectal ses are entertained or where pelvic appendicitis
examination. is likely, a rectal examination is indicated. The
examination is unpleasant to the child, and if
useful information is to be obtained, it must be
When Should a Rectal Examination Be performed gently and with care. In children
Performed in Suspected Appendicitis? under 3 years of age, the lubricated little finger
is used, whereas in older children, the index
Where the diagnosis of appendicitis is obvious finger is preferred. The finger must be intro-
from the history and clinical findings, and the duced slowly and carefully, with the child in
decision to perform an appendicectomy has the left lateral position with the knees bent up
been made, there is no advantage in performing towards the chest (see Fig. 3.22). An alterna-
a rectal examination. In all cases where there is tive method in the infant is to have the child
34 3 Abdominal Pain: Is It Appendicitis?
Fig. 3.23 The pointing test in locating the site of abdominal pain in children
Retro-ileal and retrocaecal appendicitis may also A Mass in the Right Iliac Fossa
produce loose bowel actions. If the appendix per-
forates, the infected material released collects in Appendicitis may produce a mass in the right
the most dependent parts of the peritoneal cavity – iliac fossa (Fig. 3.24). If there is overlying guard-
the retrovesical pouch in the male and the retro- ing, it may be extremely difficult to palpate until
uterine recess in the female – and a pelvic abscess the patient is anaesthetized; where there is no
develops. As the abscess increases in size, it guarding, it can be felt as a tender, immobile
causes irritation of the rectum and can be palpated mass deep to the muscles of the ventral abdomi-
as a hot, tender bulge of the anterior rectal wall. nal wall. It is an inflammatory phlegmon of an
The abscess causes small and frequent loose infected appendix, stuck by inflammatory exu-
stools containing mucus, which is a direct result date to adjacent oedematous loops of small bowel
of irritation of the rectal mucosa. The pelvic and greater omentum. Depending on the patient’s
abscess takes several days or weeks to develop, ability to localize infection, and on whether the
and there may be no clear history of antecedent appendix perforates, the mass will either resolve,
abdominal pain. Persistent small volume diar- develop into an appendix abscess or cause gener-
rhoea and the passage of mucus in a vaguely alized peritonitis.
unwell child should signal the possibility of a In girls, a large ovarian cyst may be palpable as
pelvic collection. a smooth surfaced mass in the right iliac fossa. The
mass is non-tender and mobile, unless there is tor-
sion of its pedicle. It may disappear over the pelvic
Does Appendicitis Produce a Fever? inlet into the pelvis and be ballotable by bimanual
examination between the rectum and the anterior
In most children with appendicitis, the tem- abdominal wall. If torsion has occurred, the mass
perature is slightly elevated (37.5–38 °C). A is tender and there are signs of peritoneal irritation
normal or slightly subnormal temperature does in a girl who has severe pain and no fever.
not preclude the diagnosis of appendicitis – nor In the child under 2 years of age, the mass
does a grossly elevated temperature (39–40 °C), may be an intussusception. The sudden onset of a
although this is unlikely unless peritonitis is pres- colicky pain with drawing up of the legs, vomit-
ent. Unfortunately, most of the other conditions ing, pallor, lethargy and short duration of symp-
from which appendicitis must be distinguished toms with or without rectal bleeding would be
are inflammatory or infective in nature and pro- suggestive of intussusception (see Chap. 8 for
duce elevation of the temperature. more details); a longer history of 4 or 5 days,
36 3 Abdominal Pain: Is It Appendicitis?
Tender Mobile
Immobile Ballottable
Lymphoma
Splenomegaly
Hepato-megaly
Fixed
Non tender
with generalized anorexia, malaise and marked of the terminal ileum and right colon. Infection
abdominal tenderness would make one more sus- by a number of enteric organisms, for example,
picious of appendicitis. Yersinia or Campylobacter, causes enlargement
In the older child, a relatively painless, fixed and even suppuration of these nodes. It is likely
mass in either iliac fossa would raise the possibility that many viral organisms, yet to be isolated or
of lymphoma, in which case there may be evidence identified, also cause mesenteric adenitis.
of weight loss, fever, lymphadenopathy or hepato- Infection with these organisms causes diffuse
splenomegaly. Rare causes of a right iliac fossa tenderness of the abdomen and signs which may
mass include Meckel’s diverticulitis, Yersinia be compatible with early acute appendicitis
lymphadenitis, tuberculosis, actinomycosis, dupli- (Fig. 3.25). Tenderness is often maximal in the
cation cyst and lymphangioma. Children with cys- right iliac fossa and is simply a reflection of the
tic fibrosis may develop a mass from ileal location of the enlarged nodes. The inflamed
obstruction with viscous faecal contents. ileum becomes distended with fluid and gas such
that deep palpation may produce a succussion
splash, which is a physical sign that is absent in
Other Pathologies in Suspected appendicitis. In mesenteric adenitis, there may be
Appendicitis other signs of viral infection with slight puffiness
around the eyes and a slight conjunctivitis, head-
Viral Enteritis (‘Mesenteric Adenitis’) ache, generalized aches and pains and rhinor-
rhoea. There may be some moderate enlargement
In addition to the localized collection of lym- of cervical, axillary and inguinal lymph nodes,
phoid tissue seen in the terminal ileum (Peyer’s but it should be acknowledged that in most nor-
patches), there are numerous lymph nodes at the mal children, these nodes are palpable. The tem-
mesenteric edge of the bowel and within the mes- perature may reach 40 °C, but usually returns to
entery of both small and large intestine. These normal within 48 h. Despite abdominal pain and
nodes are particularly prominent in the mesentery fever, the child is often hungry – unlike the
Other Pathologies in Suspected Appendicitis 37
a b
Flushed and ‘viraemic’
<38 °C
>38 °C
Red throat
Anorexia
Peritonitis Shifting
tenderness
Succussion
splash
Fig. 3.25 The differences between appendicitis (a), and mesenteric adenitis (b). (Adapted from Hull and Johnston
(1981)).
situation in appendicitis. The disorder is self-lim- or diarrhoea should make one suspicious of alter-
iting, and all symptoms resolve within 3 or 4 native diagnoses, of which appendicitis is one.
days. With serial examination of the abdomen,
the area of maximal tenderness may vary, and in
most cases, there is no evidence of peritonitis. If Constipation
the clinical signs are suggestive of peritonitis,
operation is indicated. When in doubt, the child Poor diet, a constitutional predisposition and poor
can be admitted to hospital and the examination bowel training may all contribute to constipation.
repeated in a few hours. Often, there is a history suggestive of an anal
fissure: acute anal pain on defecation and small
amounts of fresh red blood of the surface of the
Gastroenteritis stool or on the toilet paper. In other children,
there is a long history of infrequent passage of
This common condition is characterized by vomit- hard bowel actions over a long period. The pain
ing and diarrhoea. The diarrhoea usually com- is often colicky and may be relieved by a bowel
mences as the first symptom or shortly after the action. Examination of the abdomen reveals fae-
onset of vomiting. There may be associated abdom- cal material in both the left and right sides of the
inal pain which tends to be cramping and diffuse. colon. There is no peritonitis. Digital examina-
In the majority of children, symptoms are improv- tion of the rectum will reveal a capacious rectum
ing within 24–72 h. Persistence of either vomiting full of faecal material.
38 3 Abdominal Pain: Is It Appendicitis?
9. The infant’s abdomen is more protuber- 13. Late signs of peritonitis include a rigid
ant, wider and has a shallower pelvis and distended abdomen and signs of
than that of the older child or adult. septicaemia.
10. Narcotic analgesia should be given to a 14. Beware diarrhoea persisting for more
child with abdominal pain only once a than 48 h; it may reflect a pelvic abscess.
definite clinical diagnosis or decision
to operate has been made.
11. Abdominal pain may be caused by
pathology originating from outside the Further Reading
abdomen (eg. testicular torsion).
12. The abdominal signs of peritonitis are Hutson JM, O’Brien M, Woodward AA (2008) Jones’
Clinical Paediatric surgery. Diagnosis and Management
pain on movement of the abdomen,
(6th edn). Blackwell Publishing, Oxford.
reluctance to protrude or indraw the Puri P, Höllwarth M (eds) (2009). Pediatric Surgery.
abdomen, involuntary guarding and Diagnosis and Management, Springer, Dordrecht.
rigidity of the abdominal wall muscula- Hull D, Johnston DI (1981). Essential Paediatrics.
Edinburgh: Churchill Livingstone.
ture and rebound percussion tenderness.
Inguinoscrotal Lesions
4
c d e f
the more common situation, only the proximal responsible for the second phase of descent
processus remains patent and the hernia presents through the inguinal canal into the scrotum.
as a lump at the external ring. Occasionally, the Testosterone acts at least in part via the genitofem-
entire processus vaginalis remains widely patent, oral nerve. Therefore, in children with unde-
and the hernia presents clinically as a swelling scended testes, one needs to consider androgen
extending from the external ring into the scrotum deficiency, even though it is much more common
(‘inguinoscrotal’ or ‘complete’ hernia) (Fig. 4.2f). for maldescent to be caused by mechanical abnor-
Involution of the processus begins as soon as the malities or defects in the genitofemoral nerve.
testis has descended; delay in this process of oblit-
eration may result in a hernia because, after birth,
increases in abdominal pressure (with crying, etc.) Anatomical Landmarks
push the small bowel into the still patent processus for the Undescended Testis
and keep it open. A premature infant, therefore, is
more likely to have incompletely descended testes The key to the clinical assessment of undescended
and patent hernial sacs. testes is determination of their location.
Undescended testes are arrested in the line of
descent, or have deviated to an ‘ectopic’ or abnor-
The Undescended Testis mal position. Rarely, the testis may remain inside
the inguinal canal or abdomen, in which case it
Testicular descent is a complex process initiated will be impalpable. A testis which has descended
by hormones and executed by mechanical events; beyond the external inguinal ring but has not
abnormalities of either of these may cause unde- reached the scrotum will be deviated from the
scended testes. The hormonal signals are not normal line of descent by the bony prominence of
known completely, but testosterone appears to be the pubic tubercle to lie lateral to the external ring
The Undescended Testis 43
Femoral testis
Pubopenile testis
Perineal testis
Scrotal testis
External Inguinal
inguinal ligament
ring
Anterior superior
iliac spine
Pubic tubercle
(the commonest site for an ectopic testis). Here, it They are (1) lateral to the scrotum in the thigh
overlies the external oblique aponeurosis midway (femoral testis), (2) medial to the external ring
between the pubic tubercle and anterior superior (pubopenile testis) and (3) behind the scrotum
iliac spine (Fig. 4.3). In this location, the testis (perineal testis).
remains within its tunica vaginalis under Scarpa’s It is vital to identify the position of the exter-
fascia, and the ‘pouch’ this layer forms is some- nal ring and inguinal ligament to establish the
times referred to as the superficial inguinal pouch. anatomical location of the undescended testis
If the testis cannot be palpated lateral to the exter- (Fig. 4.4). The pubic tubercle marks the inferior
nal ring, there are three rare sites in which an margin of the external ring, and the inguinal liga-
ectopic testis may be located. ment extends laterally from there to the anterior
44 4 Inguinoscrotal Lesions
a b
Squatting
-Small child
flat, suggesting the testes are not normally resi- the pubic skin can be retracted which often reveals
dent within it. the testes (Fig. 4.7b). It should be remembered that
An alternative method is to get the small child to a testis is easy to see and feel only because of the
squat on the examining couch (Fig. 4.6a). This posi- extreme thinness of the skin of the scrotum. When
tion causes automatic relaxation of the cremaster the testis is in any other position, it is not only
muscle and allows the testes to be seen in the scro- difficult to feel but also difficult to see (Fig. 4.8). If
tum. Older boys can be asked to sit on the couch, with the scrotum is pulled up over the pubis to reveal
the knees pulled up against the chest (Fig. 4.6b). the testis, it can then be palpated by the other hand
Often, when a normal child lies supine, the tes- and gently pulled caudally (Fig. 4.7c). Parental
tes can be seen within the scrotum. In obese boys, anxiety can be relieved by the clear demonstration
the scrotum may be difficult to see completely of both testes in the scrotum (Fig. 4.7d).
and, if there is cremasteric retraction, the testes Where the testis is truly undescended, the first
may be lying near the upper part of the scrotum or step is to locate its position. The flat of the hand
higher where they are concealed by the skin is placed gently over the inguinal region in the
because of the increased subcutaneous fat area of the superficial inguinal pouch (Fig. 4.9a).
(Fig. 4.7a). Without touching the scrotum itself, By gently rolling the fingertips round and round,
The Undescended Testis 47
Thick layer of
Insulates and
fat
conceals testis
Common area
to locate testis
Fig. 4.9 The technique of locating and delivering to the hand then pushes the testis towards the scrotum (b), where
top of the scrotum an undescended or retractile testis. The the other hand attempts to snare it by holding the cord, to
mobile testis is located in the inguinal region (a). One see how far down it will reach (c)
48 4 Inguinoscrotal Lesions
the testis usually can be felt as a mobile, spherical 1. Finger rolls over cord as it
structure between the subcutaneous fat and the emerges from external ring
compensatory hypertrophy, which may be appar- Torsion of the testis is the most important cause
ent on clinical examination. of the acute scrotum, but is less common than its
It is important to remember that the size of the minor counterpart, torsion of a testicular append-
normal testis changes little between 6 months age. Testicular torsion occurs most frequently at
and 10 years until the onset of puberty, when it two ages: first, in the perinatal period, when the
grows rapidly. The hypertrophied testis is con- whole tunica and contents twist (‘extravaginal tor-
spicuous once this fact is appreciated. The nor- sion’), and secondly at puberty, when an exces-
mal testis is roughly the same size as the glans sively mobile testis twists inside the tunica vaginalis
penis (i.e. 1–2 ml vol). (‘intravaginal torsion’) (Fig. 4.12). Extravaginal
torsion occurs at, or shortly after, testicular descent
into the scrotum, when the processus vaginalis is
The Acutely Painful Scrotum not yet fixed tightly to the scrotal fascia. After the
first few weeks, torsion becomes relatively less
The term ‘acute painful scrotum’ or acutely pain- common again, until the approach of puberty, when
ful scrotum describes the scrotum with acute pain rapid enlargement of the testis predisposes to twist-
and swelling, or other signs of inflammation. ing of those testes which are hanging inside the
There are four main causes, which include tor- tunica vaginalis by an abnormally long mesentery,
sion of the testis or one of its appendages, or ‘mesorchium’. The peak incidence at 13 years
epididymitis (or ‘epididymo-orchitis’) and idio- corresponds to the high circulating testosterone
pathic scrotal oedema (Table 4.2). levels and rapid growth of the testis at that time.
Testicular torsion needs to be recognized quickly,
as immediate operation is required to avert infarc-
Table 4.2 Causes of the acutely painful scrotum in
children tion of the organ. Infarction is the presumed cause
of the absent testis after perinatal torsion.
Condition Frequency (%)
Torsion of a testicular appendage is the com-
Torsion of a testicular 60
appendage monest cause of an acutely painful scrotum in
Torsion of the testis 30 childhood. The hydatid of Morgagni, or ‘appen-
Idiopathic scrotal oedema <10 dix testis’, situated near the upper pole of the tes-
Epididymitis <10 tis, is a normal remnant of the cranial end of the
Twist inside
tunica vaginalis
Twist above
tunica vaginalis
Intravaginal
Extravaginal torsion
torsion
Incidence 13 years
‘Paradidymis’ Epididymitis
2° to reflux up
2° to urinary
a wide vas
tract anomalies
with secretions
Hydatid of Morgagni
Epididymis ‘Appendix testis’ Incidence
Testis -Commonly twists Vas narrow,
-Müllerian duct no semen
remnant
0 5 10 15
‘Vas aberrans’
Age (years)
Fig. 4.13 The testicular appendages
Fig. 4.15 The relationship of incidence of epididymitis to age
Swollen testis,
epididymis
± ischaemia
Horizontal
Scrotal oedema
‘Bell-clapper’
testis
b Tender
blue
‘pea’
Swollen Effusion
epididymis
2. Finger gently
palpates upper pole
Exquisitely
tender lump Blue ‘pea’
often visible
through scrotum
1. Hand gently
holds lower pole
of testis
Urinary
malformation Swollen cord
Swollen epididymis
Testis Effusion
Scrotal oedema
Non-tender Shadow of
fluid collection testis and
cord
Torch
Brilliantly
transilluminates
Fig. 4.23 In the large hydrocele, transillumination of the
Fig. 4.22 Signs of a hydrocele of the scrotum scrotum will cause the shadow of the normal testis and
cord to be cast against the wall of the scrotum
varicose pampiniform plexus fills with blood on the groin of a baby, often during crying; in an older
standing, or on cough impulse, but may be com- child, it may appear with coughing or walking
pletely impalpable when lying down. In small (related to peaks of high intra-abdominal pressure);
boys, the appearance of a varicocele should alert (2) the variability in the size of the swelling (bowel
the examiner to the possibility of a renal tumour is able to enter or leave the sac); (3) the periodic
with secondary compression and occlusion of the disappearance of the lump (the contents may return
testicular veins (see Fig. 15.12 in Chap. 15). completely to the abdomen); and (4) the apparent
Rarely, a testicular tumour presents as a painless lack of pain, except when the bowel becomes stuck
scrotal swelling. The testis is enlarged, irregular and in the sac (incarceration) causing venous congestion
non-tender and may be surrounded by a secondary and/or ischaemia (strangulation). Many mothers
hydrocele. Where a tumour is suspected, the central can point to the exact location of the swelling.
abdomen needs to be palpated to identify secondary
spread to the para-aortic lymph nodes.
Physical Examination
A haematocele is a collection of blood in the
processus vaginalis and may result from direct
Lump Present
trauma to the scrotum. It is uncommon in child-
When the lump is present at the time of consultation,
hood, but can be recognized by bruising of the
the key point in the examination is to determine the
scrotal tissues – in contrast to testicular torsion
exact position of the swelling in relation to the exter-
where the overlying skin is inflamed.
nal ring of the inguinal canal (Fig. 4.25). The bony
landmarks (anterior superior iliac spine and pubic
tubercle) define the position of the inguinal ligament
The Groin Lump
and the external ring, both of which are cranial to the
most obvious skin crease in the groin (Fig. 4.4).
An indirect hernia is the commonest cause of a
Inguinal or femoral lymph nodes are more lateral in
groin lump (Table 4.5). In most children with ingui-
position and may be associated with a primary septic
nal herniae, the distal part of the processus vagina-
focus in the lower leg or perineum.
lis has closed off, so that the so-called incomplete
The very rare femoral hernia is below and lateral to
sac does not extend far beyond the external ring.
the pubic tubercle, closer to the femoral skin crease.
The diagnosis can be made from the history and the
The common hydrocele is within the scrotum, but the
exact anatomical location of the lump.
less common encysted hydrocele in the processus of
the cord may be found at the external ring.
The History A retractile or undescended testis located at the
external ring can be confused with a hernia, an error
There are four points which indicate an inguinal that is avoided by palpation of a normally sited testis
hernia: (1) the sudden appearance of a swelling in within the scrotum. Remember that an undescended
Inguinal hernia
(most common)
Encysted
Inguinal or hydrocele
femoral (uncommon)
Iymph nodes
Femoral hernia
(very rare) Hydrocele
(common)
Fig. 4.26 The clinical ‘Cannot get above it’ – wide cord extends
signs of a complete inguinal up to external ring
hernia
External ring
± Ischaemia ± Ischaemia
of testis of bowel
Transilluminates
poorly
testis may have an adjacent patent processus and ‘cannot get above it’, which distinguishes it
can present because of its associated hernia. from a hydrocele that does not extend up to and
Palpation of the neck of the scrotum between through the external ring. The diagnostic fea-
the finger and thumb reveals whether the lump ture of an inguinal hernia is the ability to
reaches the scrotum (Fig. 4.26). When the reduce it by manual compression, a manoeuvre
hernia extends into the scrotum, the fingers known as ‘taxis’.
The Groin Lump 57
Short canal
Narrow external ring
Hernia obstructs
Long canal at internal ring
Wide external ring
Upper hand
disengages Direction of
hernia from upper hand pressure
external ring
Most herniae reduce readily, and often complete return of the hernial contents to the
spontaneously, without the need to resort to abdomen.
taxis. Sometimes, however, particularly in The external inguinal ‘ring’ is in reality a nar-
infants, an inguinal hernia becomes irreducible row V-shaped slit in the fibres of the external
(‘obstructed’), a term employed to indicate a oblique aponeurosis and may provide a barrier to
hernia which fails to reduce spontaneously once the manual reduction of a hernia by taxis
the infant ceases straining or crying. In children, (Fig. 4.28), although in practice, most so-called
a hernia obstructs at the external ring, unlike irreducible herniae are reducible, given the right
the situation in adults where it obstructs at the circumstances and technique. The child needs to
internal ring (Fig. 4.27). Because the internal be relaxed to allow the slit of the external ring to
ring does not prevent reduction, compression be pushed open; crying or straining causes
of the hernia through the external ring leads to contraction of the external oblique aponeurosis
58 4 Inguinoscrotal Lesions
Distal
‘Cyst’ processus
obliterated
Bowel
If the child is old enough, he should be exam- Small children or infants are not able to
ined while standing and the external ring palpated cough on demand. An alternative way to
during a cough. A cough impulse may be both increase the abdominal pressure is by pressing
felt and seen, provided the examiner’s eyes are on the abdomen (Fig. 4.33) using the flat of the
level with the groin (Fig. 4.32). hand or a closed fist. The hernia will commonly
‘pop out’ when the abdominal pressure rises,
after which its characteristics can be deter-
mined by palpation.
Lastly, when the hernia cannot be demon-
Child stands and strated, the spermatic cord should be palpated
is asked to cough
carefully for the presence of an empty sac
(Fig. 4.34). By rolling the cord against the pubis
with the finger tips, the empty sac can be felt like
multiple folds of silk sliding together (‘the silk
sign’). The thickened, rustling cord is compared
Examiner’s with the contralateral side.
Palpation
eyes level
for cough A clear history from the mother in the absence
with groin
impulse of a demonstrable hernia may be accepted as
sufficient evidence of a hernia being present,
justifying immediate referral to a paediatric
Fig. 4.32 Examining for a cough impulse with the child
surgeon.
standing
Abnormalities of the penis are an important part separate as a result of erections and masturbation
of paediatric surgery because of their frequency. by the boy himself.
In addition, the emotional response of parents to
perceived anomalies of their boys’ genitalia can
be extreme; the pressure this puts on the clinician Smegma Deposits
may lead to hasty, inappropriate or even incorrect
diagnosis and treatment. The range of anomalies If the foreskin separates from the glans around
is shown in Table 5.1. the coronal groove before the more distal fore-
skin is free, secretions and desquamation of ker-
atin can accumulate as lumpy deposits of
smegma (Fig. 5.1). The characteristics of
The Normal Uncircumcised Penis smegma are: (1) a bright yellow collection
because of its high cholesterol content (similar
The loose foreskin protrudes well beyond the to xanthelasma), (2) a lump covered by foreskin
glans of the penis (Fig. 5.1). Postnatally, the fore- which is thin enough to allow the yellow colour
skin is adherent to the glans for a variable period: to show through and (3) absence of inflammation
In many primary school-age boys, the foreskin or infection when the smegma is entirely con-
still is partially adherent. Nevertheless, forced tained within the congenital adhesions of the
retraction of the foreskin by parents or doctor is foreskin to the glans.
not necessary since separation will occur spon- The diagnosis is important because this trivial
taneously as childhood progresses. Any residual condition requires no treatment but is misdiag-
adhesions as the child approaches puberty nosed often as serious pathology, such as a ‘cyst’
Double fold
of prepuce Scar of
(foreskin) suture line
Coronal groove
c
Glans
Exposed urethral
meatus
Adherent inner
prepuce
Inner folds
open to expose
glans
Scar
(phimosis)
Scarring
fissures readily. The glans cannot be visualized at and the inner prepuce (Fig. 5.6). Therefore, bal-
all, and there is a history of marked ballooning of anitis cannot occur in correctly circumcised boys.
the foreskin which persists after micturition has The foreskin has very loose stroma, ensuring that
ceased (Fig. 5.4). This is the cardinal sign of phi- even minor superficial infection, such as balani-
mosis because it indicates urinary obstruction tis, causes marked swelling. In other words, the
requiring immediate treatment. The bladder should infection looks much worse than it really is.
be palpated since distension may indicate significant Moreover, not only is the degree of swelling dra-
subacute, or even chronic, urinary retention. matic, but also it can develop very quickly (i.e.
within a few hours).
Prompt treatment results in rapid resolution of
The Red, Swollen Penis the superficial infection and avoids the progres-
sive scarring which leads to phimosis. Where
The penis becomes swollen and red in response to topical antibiotics cannot be applied (because of
either inflammation (balanitis) or venous conges- apprehension or pain), systemic antibiotic treat-
tion (paraphimosis). The dramatic onset and magni- ment may be required.
tude of the swelling reflect the rich blood supply
and loose skin of the shaft and foreskin. Commonly,
balanitis and paraphimosis are confused with each Paraphimosis
other because their effect on the penis is similar
(Fig. 5.5). An accurate diagnosis to distinguish Paraphimosis is purely mechanical in origin
these two conditions is important because their and not an inflammatory condition. The fore-
treatment is different. A red, swollen penis may be skin has been retracted proximal to the coronal
produced also by local allergy (e.g. as part of idio- groove where it has become stuck and has not
pathic scrotal oedema). returned to its former position. If the opening in
the distal foreskin is small (i.e. mildly phimo-
tic) the retracted foreskin will compress the
Balanitis shaft of the penis at the coronal groove, and the
arrowhead shape of the glans prevents it from
This is a superficial infection of the foreskin that returning to its normal position over the glans.
occurs in response to infected, pooled urine or Venous obstruction of the glans rapidly leads to
retained secretions (smegma) between the glans engorgement and oedema of the glans and
The Red, Swollen Penis 67
Oedematous
foreskin
b
Painful
swelling of
glans
Exposed
meatus
Grossly oedematous
foreskin
b
Gross
oedema
a
Pooled drops
of urine Partial
adherence
foreskin and, at first glance, may mimic the bal- the foreskin is drawn forwards over the glans to
looning seen in balanitis, except that in balani- reassume its original position.
tis the glans is not visible. It must be remembered that (1) paraphimosis only
When the oedema is severe, it causes distortion occurs in uncircumcised or incompletely circumcised
and deviation of the glans: The foreskin appears boys and (2) the swelling of paraphimosis is caused
as a pale and eccentric ‘tyre’ immediately proxi- by venous obstruction, whereas in balanitis it is the
mal to the glans. Sometimes, the oedematous inflammatory response and, in phimosis, from urine
swelling of the foreskin dwarfs the glans which is collecting in the space beneath the foreskin.
unable to swell to the same degree.
The clinical features which distinguish para-
phimosis from balanitis are the painful, gross Meatal Ulcer
swelling of the glans with a visible urethral
meatus and the retracted swollen foreskin behind This is caused by chemical and/or physical irrita-
the corona of the glans (Fig. 5.5). tion at the tip of the glans around the urethral
Venous congestion from compression of the meatus in the circumcised child. Ammonia pro-
penis at the coronal groove occurs when the size duced by micro-organisms in sodden cloth nappies
of the distal preputial opening is narrower than causes a chemical dermatitis of the nappy area,
that of the penile shaft, while the greater width of including the glans where the mucosa at the ure-
the glans prevents easy replacement of the fore- thral opening is particularly vulnerable. The sensi-
skin to its correct position (Fig. 5.7). tive tip of the recently circumcised penis also may
Diagnosis is important since delay allows pro- be abraded by friction on hard, rough nappies. In
gression of oedema and makes subsequent reduc- uncircumcised babies, ammoniacal dermatitis
tion more difficult. The oedema of the glans and may lead to a mild phimosis by inflammation of
foreskin is reduced by manual compression, and the foreskin but does not cause a meatal ulcer.
Venous
compression
at corona
c
Gross congestion
Fig. 5.7 The pathogenesis of and oedema
paraphimosis. A tight
foreskin is retracted behind
the coronal groove (a)
leading to venous occlusion
in the shaft (b). Gross
congestion and oedema
quickly ensue (c)
Hypospadias 69
Diverging raphé
hypospadias invariably is present and commonly part of the glans, which itself is splayed out and
is severe. All babies with ‘hypospadias’ as well flattened rather than being conical. There is a
as a bifid scrotum and/or undescended testes variable groove where the urethral orifice should
should be investigated urgently for disorders of be situated, and one or more tiny blind pits in the
sex development (DSD) (see Chap 22). floor of the groove are often visible: These are
There are three major abnormalities in a penis always distal to the urethral opening.
with hypospadias: (1) the dorsal hood, (2) a prox- The orifice of the urethra is commonly near the
imal urethral orifice and (3) chordee, a ventral coronal edge of the glans, but can be anywhere
bend in the shaft of the penis (Table 5.2). along the ventral shaft of the penis, or even over the
The end of the foreskin has a characteristic scrotum in the severest cases when there is an asso-
square end like a spade, unlike the cylindrical ciated DSD. The orifice is marked by a horizontal
foreskin of the normal child (Fig. 5.10). When slit, which can be difficult to see. The position of
the examining fingers hold up the square corners the urethral orifice can be determined by pulling
of the dorsal hood of foreskin, the abnormalities out the ventral skin of the shaft, while holding the
on the ventral (anterior) surface of the penis are penis by the foreskin in the other hand. This opens
exposed. The foreskin is missing over the ventral up the orifice, confirming its position (Fig. 5.11).
Hypospadias 71
Meatal skin
pulled outwards to
demonstrate meatus
a b
Around the orifice, the line of fusion of the (chordee). The bend may not be visible without
urethral tube is deficient, and the skin immediately manipulation or observation of the erect penis.
proximal to it is thin. Deficient ventral tissue, The chordee is demonstrated by pulling the
either proximal or distal to the urethral orifice, dorsal hood upwards or by retracting the skin
leads to a flexion deformity of the penile shaft of the shaft downwards (Fig. 5.12). The degree
72 5 Abnormalities of the Penis
= ? virilized female
of chordee is shown by the angle between the The clinical examination is discussed in
glans and the shaft of the penis. This is a defor- greater detail in Chap. 22.
mity which is crucial to recognize since, unless
corrected, chordee will make intercourse pain-
ful or impossible after sexual maturity. The
degree of chordee often becomes worse with
growth of the penis, which means that it should Golden Rules
not be assumed that neonates with little chordee 1. Do not confuse the normal adherence of
will not need corrective surgery later. the foreskin with phimosis; both prevent
If the urethral meatus is on the scrotum or at retraction of the foreskin.
the penoscrotal junction, there is a high risk of 2. Phimosis may cause severe urinary tract
disorders of sex development (DSD) or urinary obstruction.
tract development. In severe hypospadias, there- 3. Marked and persistent ballooning of the
fore, the clinician must suspect the presence of foreskin on micturition is pathognomonic
DSD and urinary anomalies. The absence of tes- of phimosis with urinary obstruction.
tes in the scrotum, with or without a bifid scro- 4. Where the urethral meatus can be seen,
tum, is a cardinal sign of a DSD such as congenital severe phimosis cannot be present.
adrenal hyperplasia with severe virilization – the 5. The red, swollen penis is caused by infec-
gonads may be ovaries and will not be palpable tion (balanitis), venous congestion (para-
(Fig. 5.13). Females with complete virilization phimosis) or, rarely, allergic inflammation
caused by adrenal hyperplasia can have a ‘penis’, (idiopathic scrotal oedema).
which appears normal, although usually the phal- 6. In balanitis, the maximum pain, redness
lus is obviously less well developed than that of a and swelling are over the glans rather
normal male. The degree of masculinity, there- than the distal foreskin, which is oedem-
fore, does not of itself exclude DSD. In babies atous but not sore.
with ambiguous genitalia, there may be a history 7. Paraphimosis can be recognized by the
of maternal ingestion of androgens or even a fam- retracted oedematous foreskin behind
ily history of adrenal hyperplasia. the glans, which is identified by its
The two key physical signs to look for in neo- meatus.
nates are the presence of palpable testes and a uterus 8. Paraphimosis does not occur after ade-
on rectal examination. In neonates, the little finger quate circumcision.
introduced into the rectum can feel an infantile uterus 9. Meatal ulcers may be covered by a scab,
and confirm the existence of a DSD when ‘hypospa- simulating meatal stenosis.
dias’ and ‘undescended testes’ occur together.
Further Reading 73
Further Reading
10. Complete ulceration of the surface of
the glans makes the meatus invisible. Beasley SW, Hutson JM, Auldist AW (1996). Essential
Paediatric Surgery, Arnold, London.
11. A baby with ‘hypospadias’ with impal-
pable gonads and/or a bifid scrotum
should be managed as a case of DSD.
12. Hypospadias, even when mild, needs
early recognition to avoid unwitting cir-
cumcision (the skin of the dorsal hood
is required for surgical correction).
13. Acquired diseases of the penis produce
parental anxiety, often out of propor-
tion to the severity of pathology. The
clinician should be wary of being pres-
sured or panicked into a hasty or incor-
rect diagnosis, since most conditions
are remedied easily once recognized.
The Umbilicus
6
In the embryo, the primitive umbilical ring is a underside of the umbilicus. Continued growth of
relatively large defect in the ventral abdominal the duct at the intestinal wall produces a Meckel’s
wall transmitting a number of structures which diverticulum (Fig. 6.4). Failure of the mid-gut to
connect the fetus to the cord and placenta (Fig. 6.1). re-enter the abdomen from the umbilical cord, or
In the 5-week embryo, the mid-gut communicates reherniation through the umbilical ring at a later
with the yolk sac via the vitelline duct or yolk stage, results in exomphalos. In this condition,
stalk. The yolk sac provides an early nutrient the intestines and liver protrude through the
source for the embryo. In the next few weeks, the abnormal umbilical ring into a thin avascular sac
mid-gut develops more rapidly than the abdominal between the body wall and cord.
cavity and herniates through the umbilical ring Another structure passing through the umbili-
into the extra-embryonic coelom (Fig. 6.2). By cal ring is the allantois, which contributes to the
10 weeks, the mid-gut has returned to the abdomi- formation of the bladder after initial outgrowth
nal cavity and the vitelline duct subsequently from the hindgut into the body stalk.
degenerates (Fig. 6.3). If the vitelline duct does not
involute, a vitello-intestinal fistula may result.
Partial degeneration may result in a sinus, cyst
or fibrous band connecting the ileum to the
Stomach
Yolk sac
Vitelline Midgut
duct
Umbilical
vein
Vitelline
Umbilical artery
Allantois
arteries
Umbilical ring
Vitelline duct
Cloaca
6 Week embryo
Clinical Diagnosis
Vitelline duct
becoming atretic Is the Umbilicus Abnormal?
10-week embryo
At birth, the umbilical cord is clamped and
divided several centimetres from the umbilical
Fig. 6.3 Return of the mid-gut to the abdominal cavity ring. Over the next week, it desiccates and sepa-
with rotation in the 10-week embryo rates from the umbilicus. The umbilicus dries
rapidly and appears as a corrugated dimple about
The part of the allantois extending from the 1 cm in diameter. Apart from an umbilical hernia,
umbilicus to the bladder is called the urachus, but the commonest abnormality of the umbilicus is a
this structure is vestigial in man and its lumen persistent discharge which commences after
closes well before 10 weeks of development. The detachment of the umbilical cord stump. More
fibrous cord so formed persists into adult life as major umbilical abnormalities present as passage
the median umbilical ligament, running between of urine or faeces from the umbilicus through a
the peritoneum and transversalis fascia from the sinus opening or as the obvious defects of
dome of the bladder to the umbilicus. exomphalos and gastroschisis.
Failure of the urachus to obliterate results in
urinary discharge from the umbilicus and can
be associated with bladder outflow obstruction. The Weeping Umbilicus
Patency of one part of the urachus produces a
urachal diverticulum, sinus or cyst (Fig. 6.5). The umbilicus dries within days of separation of
As the fetus grows, the umbilical ring remains the umbilical cord stump. If it remains moist and
constant, making it appear relatively smaller. At there is a weeping discharge from granulation tis-
birth, the umbilical vessels are the only major sue at its base, an umbilical granuloma should be
structures in the cord, and when they involute, the suspected. This condition is diagnosed on clini-
umbilical ring becomes empty. If the ring does cal grounds and requires no further investigation.
not constrict quickly, some of the contents of the However, several points must be considered
abdomen may herniate through the skin-covered before a diagnosis of umbilical granuloma is
defect, a week or so after birth, as an umbilical made. First, it should not have urine or faecal dis-
hernia. These herniae tend to become smaller charge; if these are present, the umbilicus should
with time, such that 90 % have disappeared by be examined carefully for evidence of a sinus or
1 year of age. fistula opening. This may lie at the base and may
After separation of the umbilical cord, the be concealed in part by the rest of the umbilicus.
exposed surface of the stump may become Secondly, age is of some significance in that an
infected with the formation of sessile or pedun- umbilical granuloma develops in the first month
Clinical Diagnosis 77
Prolapsed
Vitello-intestinal vitello-intestinal
duct Fibrous duct
band
a Patent b
urachus Urachal
sinus
Posterior urethral
valves
c
Urachal
cyst
Bladder
Fig. 6.5 Urachal malforma- diverticulum
tions. These include simple d e Urachal
patency (a), a urachal sinus abscess
at the umbilicus (b), or a
urachal cyst (c). Sometimes
there is a diverticulum at the
apex of the bladder (d).
Urachal anomalies may
become infected to form an
abscess (e)
or two of life, whereas sinuses and cysts of ura- since it represents subacute infection of the
chal and vitelline origin usually appear at a later necrotic cord stump.
date. Thirdly, an umbilical granuloma is not An umbilical granuloma should be treated
associated with other congenital abnormalities, with local application of silver nitrate at weekly
78 6 The Umbilicus
Umbilical cord
Liver
Small bowel
Umbilical artery
Small empty
abdomen
intervals for 3 weeks. If complete cure is not tract. Careful examination of the umbilicus will
achieved within this time, it suggests that the reveal a small opening which can be probed with
diagnosis may be wrong and a paediatric surgical a No.5 feeding tube. If the feeding tube is intro-
opinion should be sought. duced as far as the ileum, larger quantities of fae-
cal fluid can be aspirated. Treatment involves a
small laparotomy with complete excision of the
Mucus Discharge from the Umbilicus vitello-intestinal tract.
Rarely, intussusception of the patent vitello-
A sequestrated island of intestinal mucosa may intestinal tract may at first glance appear similar
occur at the umbilicus. This ectopic tissue pro- to that of gastroschisis. However, in most cases,
duces mucus and appears as a small, red, shiny the appearance is that of a double-horned protru-
area at the base of the umbilical cicatrix. Exposure sion, and it is the mucosal surface of the bowel
to the air may form crusting. This condition is which is protruding rather than the serosal sur-
often difficult to distinguish clinically from umbil- face. No mesentery is evident.
ical granuloma, but is of little importance as the Treatment of a prolapsed vitello-intestinal
management of both conditions is identical. tract involves reduction of the prolapse with exci-
Ectopic mucosa may be distinguished from gran- sion of the vitello-intestinal remnants.
ulation tissue because it is smooth, while a granu-
loma is nodular with visible capillary loops.
Discharge of Urine from the Umbilicus
The Umbilicus Discharging Faeces A patent urachus causes discharge of urine from
and Air the umbilicus. The urine may be infected, mak-
ing it difficult to distinguish from faecal fluid.
Faeces and air discharging from the umbilicus is Again, with careful inspection of the umbilicus, a
strongly suggestive of a patent vitello-intestinal sinus opening will be evident. A patent urachus
Clinical Diagnosis 79
Neck of exomphalos
The Large Umbilical Defect Covered
by a Thin Membrane (Exomphalos) Fig. 6.8 A lateral view of exomphalos, showing skin
encroachment
In exomphalos, there is a large abdominal wall
defect at the umbilicus with protrusion of abdom- sia, visceromegaly and abnormal facies with
inal viscera into a thin membranous sac formed grooving of the ear lobes. The organomegaly is
by the amniotic membrane and peritoneum believed to be caused by excessive insulin-like
(Fig. 6.6). In most cases, the sac is intact, and for growth factor 1 (IGF-1) production by the fetus.
several hours after birth, the contents can be The exomphalos occurs because viscera are too
clearly seen through the translucent membrane, large to fit inside the fetal abdomen. The impor-
which becomes increasingly opaque with drying. tance of this lesion relates to profound neonatal
The size and shape of the defect vary consider- hypoglycaemia secondary to hyperinsulinaemia
ably – those with a relatively small neck appear and necessitates immediate clinical recognition,
pedunculated and poorly supported, whereas in blood glucose estimation and the commencement
others the greatest diameter of the defect is at the of a glucose infusion.
neck (Fig. 6.7). The skin of the ventral abdominal As soon as the diagnosis of exomphalos is
wall extends up the side of the sac to a variable made, immediate measures must be undertaken
degree (Fig. 6.8). The umbilical cord is expanded to avoid heat and water loss. The large moist sur-
over the dome of the defect, and the umbilical face of the exomphalos sac allows rapid evapora-
vessels can be seen coursing across its surface. tion, leading to hypothermia. Therefore, the
The contents of the sac include small bowel, liver, infant should be placed in an incubator with the
stomach and colon. whole abdomen wrapped in transparent plastic
Exomphalos is often associated with other kitchen sheeting; this also reduces the likelihood
major congenital abnormalities, particularly car- of trauma or infection to the sac. The infant then
diac and renal anomalies. A less common but should be transferred to a major paediatric hospi-
important association is Beckwith-Wiedemann tal, where the subsequent management is depen-
syndrome, where there is hemihypertrophy dent on the size of the defect and the presence of
(asymmetrical overgrowth of limbs), macroglos- other abnormalities.
80 6 The Umbilicus
Scaphoid
abdomen
Defect
Prolapsed
bowel
- Oedematous
- Inflamed
- Thickened
Bowel Protruding from an Umbilical parenteral nutrition until bowel function returns
Defect (Gastroschisis) to normal.
The mechanical causes of non-bile-stained vom- The vomitus contains no bile because the
iting in children correlate closely with age obstruction is proximal to the bile duct opening
(Fig. 7.1). Hypertrophic pyloric stenosis occurs (Fig. 7.2).
between 3 and 12 weeks of age (most commonly The cardia of the normal child acts as a sphinc-
between 4 and 7 weeks), while intussusception is ter to prevent retrograde flow of stomach contents
most frequent in older infants between 3 months up the oesophagus. In children with gastro-
and 2 years. Gastro-oesophageal reflux, which is oesophageal reflux, there is no barrier to prevent
extremely common in the first few weeks of life, this flow, with consequent free regurgitation of
becomes progressively less so with time. feeds.
In pyloric stenosis, there is postnatal thicken-
ing of the circular muscle of the pylorus which
results in gastric outlet obstruction. As the nar- Clinical Features
rowing progresses, the stomach becomes hyper-
trophied, producing visible gastric peristalsis. Non-bile-stained vomiting in an infant is caused
most commonly by a feeding problem (Table 7.1).
Where the mother is inexperienced or the baby is
greedy, excessive feeding leads to gastric disten-
sion and vomiting. This problem is diagnosed by
a careful history and observation of the feeding
technique. The major challenge to the physician,
however, is separating the trivial causes of vomit-
ing (like excessive feeding) from more sinister
Frequency
a b
Milk
HCI
Bile
Fig. 7.2 The pathology of pyloric stenosis (a) compared with gastro-oesophageal reflux (b). The hypertrophied pylo-
rus causes outlet obstruction of the stomach and prevents bile from reaching the stomach
Table 7.1 Causes of Feeding problem Greedy, ‘healthy’ baby and/or inexperienced mother
non-bile-stained vomiting in
‘Hidden’ infection Systemic illness – vague systemic symptoms and signs
infancy
Meningitis in an ‘unwell’ baby
Urinary tract infection
Gastroenteritis
Gastro-oesophageal reflux Mechanical problem – ‘healthy’ baby with or without
failure to thrive
Pyloric stenosis Mechanical problem – ‘healthy’ baby with or without
failure to thrive
Inguinal hernia Intermittent pain and/or mechanical obstruction
strongly age-dependent (Fig. 7.1). The history Overfeeding is seen in the most robust (or frankly
should be aimed at answering the following obese) and greedy infant, who in all other
questions: respects appears healthy and well nourished.
1. Is the infant well and active, and keen to take There is no weight loss, and careful history will
feeds? Reason: to separate mechanical or reveal that the infant is receiving a more-than-
feeding problems from septic causes of adequate volume of feed. Many of these infants
vomiting. have coexisting gastro-oesophageal reflux, the
2. What is the duration, nature and timing of the symptoms of which settle with reduction of milk
vomiting? Reason: to separate gastro-oesoph- volume.
ageal reflux from pyloric stenosis. When sepsis is the cause of vomiting, the
3. Is the vomiting forceful? Reason: strongly pro- infant is usually unwell, lethargic and feeds
jectile vomiting is suggestive of pyloric steno- poorly. In contrast, in pyloric stenosis, the infant
sis (with secondary gastric hypertrophy). is hungry, restless, crying and will feed with
Clinical Features 85
enthusiasm, but is simply unable to empty his the vomiting are nondescript and variable. The
stomach except by vomiting. The vomiting is vomiting usually commences at birth, but
characteristically forceful or projectile and occurs becomes less pronounced with age. Minor inter-
after every feed. As soon as vomiting has current infection often exacerbates the
occurred, the infant is ready to resume feeding. symptoms.
Most infants with pyloric stenosis present with
symptoms of less than 1 week’s duration. Where The Examination
the duration is longer, blood (coffee-ground Although specific symptoms and signs may
material) may be mixed with the vomitus, indicate a site of infection, unwell babies
reflecting gastritis. The infant may also become should have a urine test and a lumbar puncture
severely dehydrated with a metabolic alkalosis if there is any doubt about underlying urinary
from loss of water and hydrochloric acid in the or meningeal infection. Commonly, there are
vomitus. There is weight loss, the nappies are no localizing signs of these infections in young
rarely wet or dirty and there is no diarrhoea, as infants.
might be found in gastroenteritis. There are no Physical signs in gastro-oesophageal reflux
clinical signs pathognomonic of gastro-oesopha- are those of the complications rather than the
geal reflux. Effortless vomiting of small or large reflux itself. Severe oesophagitis may cause irri-
volumes of non-bile-stained material before, dur- tability from discomfort or pain, haematemesis
ing or after feeds in an otherwise healthy child is (usually coffee-ground vomitus) or pallor from
suggestive, but frequently the characteristics of chronic anaemia. Development of an
86 7 Non-Bile-Stained Vomiting in Infancy
Sunken eyes
Dry tongue
Active child
(except in late cases)
Epigastric fullness
(distended stomach)
Scrawny infant
Dry nappies
(low urine output)
oesophageal stricture may result in food stick- The diagnosis of pyloric stenosis is made on
ing in the oesophagus, with retrosternal discom- clinical grounds and it is rarely that other investi-
fort, choking and excessive salivation. Aspiration gations are required.
into the respiratory tract may result in recurrent The clinical features of pyloric stenosis
chest infections with clinical signs of pneumo- include:
nia. Weight loss and failure to thrive may be 1. Epigastric fullness
present. In children under 3 months of age, the 2. Visible gastric peristalsis
diagnosis should be made following the exclu- 3. Palpable pyloric ‘tumour’
sion of sepsis and pyloric stenosis. In gastro- All babies have epigastric fullness after feeds as a
oesophageal reflux without complications, result of a full stomach, but in pyloric stenosis,
empirical improvement in symptoms with atten- the stomach remains full between feeds and
tion to posturing and food thickening assists in becomes hypertrophied. Waves of peristalsis,
the diagnosis. commencing under the left costal margin and
Dehydration commonly is evident in pyloric directed to the right, can be seen when the abdo-
stenosis: The anterior fontanelle is depressed, the men is illuminated from the side (Fig. 7.5).
eyes sunken, the mouth dry and skin turgor The waves take 5–10 s to move across the
reduced. Loss of subcutaneous fat produces a abdomen and occur every 30 s. They are easier to
scrawny-looking and thin infant and is the result see after a test feed, but caution must be taken to
of malnutrition (Fig. 7.4). avoid overfeeding the infant until the pyloric
Clinical Features 87
Obligue
light
source
‘tumour’ has been palpated. The palpation of an may be positioned in the mother’s left arm
enlarged pylorus or pyloric ‘tumour’ provides (Fig. 7.6) or supine in bed. The examining doctor
absolute confirmation of the diagnosis of pyloric should be seated on the infant’s left side, and pal-
stenosis, but visible peristalsis is enough to trig- pation is best achieved with the index, middle
ger immediate surgical referral. and ring fingers of either hand. The examining
The pylorus is a relatively mobile structure fingers must be warm. Examination begins gently
connected on the right to the less mobile first part to avoid frightening the infant and to avoid reflex
of the duodenum. In pyloric stenosis, the hyper- contraction of the abdominal wall musculature.
trophied pylorus can be felt as a hard but mobile In the relaxed infant, where it is easy to feel
mass, a little to the right of the midline in the through the rectus abdominis, the pylorus may be
upper abdomen. Traditionally, it has been palpable near the midline. In most infants, the
described as ‘feeling like an olive’. In the calm, rectus abdominis presents a barrier to palpation
cooperative infant, the pylorus may be easily pal- such that the best area to palpate the ‘tumour’ is
pable, but in many it is discernible only with either in the midline itself or in the angle formed
careful and patient examination. Both the child between the liver edge and the lateral margin of
and examiner must be comfortable. The child the right rectus abdominis (Fig. 7.7). The pylorus
88 7 Non-Bile-Stained Vomiting in Infancy
Colicky abdominal pain, with or without vomit- Other causes of congenital or acquired bowel
ing, is seen often in infants and in most instances obstruction are rare and may be indistinguishable
does not reflect significant pathology (Table 8.1). clinically from intussusception, but will become
The most common colic is the so-called colic of apparent at laparotomy or laparoscopy. Bowel
infancy or wind colic, a condition of little obstruction caused by adhesions should be con-
significance but one whose cause is unknown. It sidered where there has been previous abdominal
may result from air swallowing, constipation or surgery. Systemic diseases, such as Henoch-
immaturity of gut motility, but these theories are Schönlein purpura, may simulate intussuscep-
unproven. It is seen in the first 3 months of life tion, probably because the allergic vasculitis
and is characterized by episodes of uncontrolla- produces submucosal haemorrhages which lead
ble crying or screaming which may occur at any to intermittent intestinal obstruction and occa-
time during the day but usually are 1–2 h after sionally even intussusception.
feeds. It is not progressive, is not associated with In most infants with colic and vomiting, the
vomiting and the infant remains well. differential diagnosis lies between gastroenteritis
Constipation is rare in breast-fed babies, but and intussusception. Appendicitis is exceedingly
may develop in children receiving other feeds. rare in children under 3 years of age.
Sometimes, the left colon contains palpable fae-
ces, or there are hard lumps of faeces distending
the rectum and anal canal. Intussusception
A more serious cause of colic is intussuscep-
tion which occurs most commonly in the child Intussusception is an acquired form of bowel
aged 3–12 months, but can occur at any age. obstruction in which one part of the bowel is
drawn inside the lumen of adjacent distal bowel
(Fig. 8.1). Compression of the vessels in the mes-
Table 8.1 Colic in infants and children entery leads to lymphatic and venous obstruction
‘Wind colic’ with secondary oedema. The lumen of the intus-
Gastroenteritis suscepted bowel is occluded by the intussuscep-
Constipation tum and leads to dilatation of proximal bowel
Intussusception and colic. The bowel at the commencement of
Appendicitis (faecolith) the intussusceptum is called the lead-point. The
Bowel obstruction common lead-points in intussusception are shown
Congenital in Fig. 8.2. Pathological causes of intussuscep-
Acquired (adhesions) tion account for only 10 % but include an inverted
Henoch-Schönlein purpura Meckel’s diverticulum, intestinal polyps or,
Intussuscipiens
± Proximal
Apex obstruction
Intussusceptum
Peyer’s
patches
occasionally, duplication cysts of the small age (Fig. 8.3). One possible explanation why
bowel, lymphosarcoma or submucosal hae- intussusception occurs most commonly between
morrhage with Henoch-Schönlein purpura. 4 and 7 months is shown in Table 8.2. The hyper-
‘Idiopathic intussusception’ is most likely to plastic Peyer’s patch is propelled by peristalsis
occur between 3 months and 2 years of age, through the ileocaecal valve and drags the rest of
whereas pathological lead-points present at any the ileum with it.
The Clinical Presentation 93
Vomiting
Pallor
Screaming
Pulling up the knees
Sweating
5. Pain may not be present in some infants with
intussusception
3 12 24
Age (months) Table 8.4 Symptoms of intussusception
Presence of “Diarrhoea”
Time Hours
Onset Time Hours
Onset
Fig. 8.4 The presence of vomiting in relation to the onset
Fig. 8.6 The relationship of ‘diarrhoea’ to the onset of
of intussusception. Early reflex vomiting from pain occurs
intussusception. Reflex peristalsis from the mass, or blood
until the stomach is empty. After a variable interval, late
in the proximal colon, causes early and rapid evacuation
vomiting develops with progressive bowel obstruction
of the distal colon
Recent
U.R.T.I.
Vomiting Blood & mucus
Clenched in stools
fists
Table 8.5 Signs of intussusception and is nearly always more medial in the abdomen
Abdominal mass 70 % than the inexperienced examiner expects, within
Rectal blood 53 % the line of the normal surface markings of the
Tenderness 40 % colon (Fig. 8.10). As the intussusception pro-
Dehydration (greater than 5 %) 15 % gresses, it draws its mesentery with it, which has
Rectal mass 10 % the effect of pulling the colon towards the root of
Peritonitis 5% the bowel mesentery, behind the rectus abdomi-
Shock 5% nis muscles (Fig. 8.11). The mass can be palpated
beneath the lateral edge of the right rectus muscle
but is usually best felt in the midline above the
umbilicus (Fig. 8.12).
await the cessation of the episode of pain: the At this point, the colon is maintained close to
colic of intussusception lasts only a few minutes the anterior abdominal wall by the spine and great
so the abdomen is examined once the colic has vessels. The hand of the examiner should be
ceased. Evidence of constipation should be placed lightly on the epigastrium until the child is
sought by palpation for hard faeces in the left relaxed. The fingertips can then be rolled for-
colon and sigmoid. The abdomen should be wards and backwards over the surface of the hori-
examined for tenderness and guarding indicating zontally placed sausage-shaped mass. The
peritonitis. Appendicitis, although exceedingly palpation of an intussusception mass is most
rare in the normal age group for intussusception, difficult when it is behind the rectus abdominis
must be considered, particularly if the pain is not muscles or when the abdomen is distended or
overtly colicky. tender. The longer the duration of symptoms, the
Palpation of an intussusception mass clinches less likely is a mass to be palpable.
the diagnosis. The mass is sausage-shaped and Examination of the rectum will usually reveal
usually behind the right rectus abdominus muscle it to be empty. The appearance of blood on the
on the right side of the abdomen but may cross glove or a palpable lead-point would be further
the midline just above the umbilicus (Fig. 8.9). evidence of intussusception. Rectal examination
The right iliac fossa often feels ‘empty’. The is also useful to exclude constipation where diag-
mass may be difficult to feel, particularly in the nostic difficulty exists. In less than 10 % of
presence of abdominal distension or tenderness, patients, the apex of the intussusception is
96 8 Colic
Fig. 8.8 An algorithm for the Algorithm for the clinical examination
clinical examination of a child with
suspected intussusception General condition Dehydration
U.R.T.I.
Other conditions
Intussusception
Henoch-Schonlein purpura
Prevertebral
mass ~ 70 %
R.I.F.
“empty”
palpable in the rectum and occasionally may pro- examination of the sulcus between the protruding
trude from the anus, in which situation it needs to mucosa and the anal margin. If there is a pro-
be distinguished from prolapse of the rectum lapsed intussusception, there is no fornix on digi-
(Fig. 8.13). This is readily achieved by digital tal examination, whereas if the protruding mucosa
is caused by prolapse of the rectum, there is a
short, blind-ending fornix.
Once the diagnosis of intussusception is
confirmed in a child outside the usual age range
of intussusception, clinical examination is
directed at looking for a possible cause. In prac-
tice, the only conditions causing intussuscep-
tion which have external manifestations are
Henoch-Schönlein purpura and some forms of
polyposis. Therefore, the legs and buttocks of
older children should be examined for the hae-
morrhagic rash of Henoch-Schönlein purpura.
The urine should be examined for proteinuria or
haematuria. Inherited forms of polyposis rarely
cause intussusception in small children because
the polyps take years to grow to a size that
causes intussusception, but do cause intussus-
ception in the child over 5 years of age. In
Peutz-Jegher syndrome, there is pigmentation
Fig. 8.10 The common position of the intussusception around the mucocutaneous junctions of the
mass compared with the normal position of the colon mouth and the anus.
D - J flexure
Fig. 8.12 Palpation of the sausage-shaped mass where it crosses the midline above the umbilicus. The fingers roll over
the horizontally oriented sausage
The surgical lesions affecting the neck are numer- shortness of one sternomastoid muscle or not
ous and common, with enlarged lymph nodes (Table 9.1). It is important to realize that alterna-
being responsible for most examinations. To tive diagnoses must be sought if the sternomas-
enable discussion of the various presentations of toid muscle is not short and tight (Fig. 9.1).
neck abnormalities, the chapter has been divided
into postural deformities (i.e. wry-neck) and
lesions laterally or in the midline of the neck. Differential Diagnosis
2. ? Squint
examine eye 3. ? Posterior
movements fossa tumour
Examine
cranial nerves
4. ? Vertebral
anomaly
Inspect
1. ?Short muscle
and palpate
palpate
spine
the eyes, such that the squint may not be obvious at with that of other causes. Congenital anomalies
first. By straightening the head passively, the squint such as hemivertebrae will produce torticollis from
will become apparent and can be confirmed by birth without progression. Vertebral lesions can be
checking eye movements. identified clinically by inspection and palpation of
the dorsal cervical spines and confirmed on x-ray.
Is There a Brain Tumour?
Posterior fossa tumours, which are not uncom-
mon in children, may compress the brain stem at Sternomastoid Torticollis
the foramen magnum to produce acute stiffness of
the neck and cause the head to be held to one side. A sound clinical understanding of this abnormal-
The neck is ‘frozen’ in this position and is difficult ity depends on knowledge of how the sternomas-
to move actively or passively. The presence of a toid muscle works (Fig. 9.2). It has a complicated
central nervous system tumour may be known action which combines lateral flexion with rota-
already, but occasionally, acute torticollis is the tion towards the opposite shoulder. The mastoid
first presentation. A careful neurological exami- is not only above the sternum but also significantly
nation should be performed with emphasis on the posterior to it. The rotation towards the contralat-
lower cranial nerves and cerebellar function. eral side on contraction of the sternomastoid is
important to appreciate because it is the primary
Is There a Vertebral Anomaly? effect of shortening in infancy (Fig. 9.3).
Structural lesions in the cervical vertebrae will Ipsilateral head flexion occurs as well, but is less
produce a tilt of the head which may be confused noticeable in a tiny infant bundled up in a rug!
Torticollis (Wry-Neck) 103
Sternum
Fulcrum
(atlanto-axial
joint)
Lateral flexior
The sternomastoid tumour may affect all or and pliable in the small baby and readily deform,
part of the muscle, including its two inferior but once the torticollis resolves, the plagioceph-
heads (Fig. 9.3). The resulting fibrosis likewise aly tends to resolve as well.
can affect a localized part of the muscle.
Remember that not all ‘tumours’ lead to The Effect of Growth
significant fibrosis and shortening later in life: Progressive deformity with growth is seen in
The majority resolve with healing after a few older children when one tight sternomastoid
months with no sequelae. Furthermore, there are muscle immobilizes the face for a long time. The
some older children who present with fibrosis side of the face that is limited by the fibrotic mus-
without a history of a ‘tumour’ in infancy. cle grows more slowly than the normal side and
causes progressive asymmetry (Fig. 9.5). This
inhibition of the growth of the mandible and
The Mechanisms of Secondary maxilla embodies an important principle of pae-
Deformity diatrics: normal growth of the bones depends on
normal muscular movement.
The Effect of Gravity The degree of hemihypoplasia of the face can
In small infants with torticollis and fixed rotation be determined by the angle between the plane of
of the head, gravity deforms the head as it lies on the eyes and the plane of the mouth. Normally,
the bed because it remains in the same position these lines are parallel but form an angle to each
for a prolonged period. The baby’s head is turned other when the face is asymmetrical. The larger
towards the contralateral side with the contralat- the angle between these lines, the more severe is
eral occiput pressing on the bed. Flattening of the the asymmetry. Hemihypoplasia is a crucial sign
occiput leads to secondary flattening of the ipsi- to observe because it indicates that surgery is
lateral forehead (Fig. 9.4). This asymmetrical necessary to release the tight muscle.
skull deformity is called plagiocephaly and is
best observed from above the head. The Effect of Postural Compensation
Plagiocephaly can become marked if the child When children are old enough to walk, the eyes
is not nursed prone with the head turned towards are kept horizontal to facilitate balance and cop-
the affected side. The skull bones are very soft ing with gravity. If a short, fibrous sternomastoid
104 9 The Neck
Fusiform
swelling
b
(i) (ii) (iii)
muscle causes tilting and rotation of the head, the How to Assess Limitation of Movement
child will compensate for this by elevating the
ipsilateral shoulder to keep the plane of vision The rotational component of the action of the
horizontal (Fig. 9.6). Examination of the child sternomastoid is easy to measure. Rotation is
will show the eyes to be level and (possibly) assessed by standing behind the child’s head
hemihypoplasia of the face. The ipsilateral shoul- and passively rotating the head while it is
der is lifted up to relieve tension on the sterno- held between both hands. The aim is to rotate
mastoid. There is cervical and thoracic scoliosis the head until the nose reaches each shoulder
to compensate further for this. Other adjacent (180° total range) (Fig. 9.7). Note that the ster-
muscles, such as the trapezius, may be wasted nomastoid is stretched to its maximum length
because of inactivity. by lateral rotation to the side of the affected
Torticollis (Wry-Neck) 105
Gravity
Angle of tilt
Hypoplastic
right face
Fig. 9.5 Estimating the degree
of hemihypoplasia of the face by
comparing the plane of the eyes
with the plane of the mouth,
which should be parallel. When
the shoulders are level, the head Tight
is tilted muscle
106 9 The Neck
Wasted
trapezius
Compensatory
scoliosis
High shoulder
a b
Normal
range
Normal
stretched
Limited
muscle
range
1. Stand behind
child
muscle. Where the muscle is fibrotic, it can- Table 9.2 Lateral neck swellings
not be stretched to its full length and rotation 1. Acute lymphadenitis
to the ipsilateral side is restricted. The angle Short history (days)
reached at the limit of movement is judged eas- Signs of inflammation (unless exposed to antibiotics)
ily from above and can be recorded for future 2. Atypical tuberculous (MAIS) lymphadenitis
reference. 1–2-month history in a 1–2-year-old
Very-low-grade inflammatory signs (= cold abscess)
Purple discoloration of the skin (when near the surface)
The Side of the Neck Collar-stud abscess
3. Malignant nodes
Child <5 years – leukaemia, neuroblastoma
Lumps in the side of the neck are caused most
Child >5 years – lymphoma, leukaemia
commonly by inflammation in lymph nodes. The
Non-tender, rubbery/stony
mouth permits entry of pyogenic bacteria, viruses
+ History/signs of disease elsewhere
or rarer organisms (such as atypical mycobacte- 4. Branchial cyst (2nd or 3rd clefts)
ria), which colonize the surrounding lymphatic Single cyst beneath the middle third of the
tissue. In addition, the sheer volume of lymphatic sternomastoid
tissue in the neck means that often malignancies Transilluminable (clear mucus)
affecting lymph nodes will be found here first. Branchial sinus opening at the anterior border of the
Leukaemia, lymphoma and secondary neuroblas- lower part of the sternomastoid
toma may present in this way (Table 9.2). The 5. Vascular malformation
embryological development of the neck is com- Sign of emptying (cavernous spaces)
+ Overlying skin vessels/colour
plicated by the large number of structures within
+ Bruit
it, including the branchial clefts and arches – the
(+ Excess flow – cardiac failure – enlarged liver)
distant homologues of the gill slits. Various bran-
(+ Platelet trapping – Haemorrhage)
chial cysts and sinuses may occur, although most
6. Lymphatic malformation (= ‘cystic hygroma’)
are rare. Also, hamartomatous overgrowth of Brilliantly transilluminable
some tissues, for example, jugular lymphatic Enlarges rapidly with infection or haemorrhage
spaces or blood vessels, may produce various Single cyst/diffuse multilocular
vascular anomalies, such as a ‘cystic hygroma’ or Soft, flabby, non-tender (except if secondary infection)
haemangioma. 7. Sternomastoid ‘tumour’
Fusiform swelling within the sternomastoid
Neonate/infant
Where Is It? No obvious signs of inflammation
Torticollis
The sternomastoid muscle is the essential land- 8. Salivary gland swellings
mark for all lesions in the lateral part of the neck, (Parotid) pain and swelling worse with eating
and often the key to diagnosis is localization of (Sialectasis) infected saliva massaged from Stensen’s duct
the lump in relation to this muscle (Fig. 9.8). (Submandibular calculus) palpable/visible in the floor
of the mouth
Lesions (inflammatory or neoplastic) in the
lymph nodes will be found along the jugular
chain or other known sites (Fig. 9.9). Infected
nodes are more likely to be in the submandibular (Fig. 9.9). Nodes at the base of the neck (supra-
and tonsillar regions (anterior border of the upper clavicular nodes) may become involved from
third of the sternomastoid) than elsewhere, since infections arising on the arm, axilla or chest wall
the commonest portals of entry drain through or upper abdomen (Fig. 9.9).
these nodes. Infections gaining entry through Remnants of the branchial system of clefts and
abrasions (particularly on the scalp) may involve arches are found most commonly near the ante-
less common sites, such as the occipital nodes rior border of the lower third of the sternomastoid
108 9 The Neck
(second cleft) (Fig. 9.10) or, rarely, near the upper gland or near the region of the thoracic duct at the
end of the sternomastoid (first cleft) (Fig. 9.11). base of the neck (Fig. 9.12).
Deep-seated lesions behind the lower third of the If the swelling is in the sternomastoid muscle
sternomastoid may be in the thyroid: Neoplasms itself, then a sternomastoid ‘tumour’ is the likely
are rare but well documented in adolescents, diagnosis (see under Torticollis). The relationship
especially in girls. Uniform enlargement of the of a mass to the sternomastoid is easier to determine
thyroid is not difficult to separate from other when the muscle is made to contract (Fig. 9.13).
lesions because of its characteristic shape.
The hamartomatous conditions, such as lymp-
hangioma (‘cystic hygroma’), occur along the What Are Its Characteristics?
jugular lymph channels, in or around the parotid
Pyogenic infection in the tonsillar nodes, which is
common in the first year of life, will demonstrate
all the features of acute inflammation unless
modified by antibiotic treatment. The duration of
symptoms is short (a few days), but the symptoms
progress rapidly. The systemic effect of pyrogens
will cause fever, malaise and loss of appetite.
All the signs of acute inflammation will be
present, namely, (1) heat, (2) redness, (3) swell-
ing, (4) tenderness and (5) loss of function/lack
of movement. The heat produced by the
inflammation can be felt by the back of the exam-
iner’s fingers, and this should be sought first
before the skin temperature is altered by the pal-
Fig. 9.8 The sternomastoid muscle: the key to the local-
pation process itself. When the infection is near
ization and identification of cervical masses. It divides the
neck into anterior and posterior triangles the surface, the erythema of the overlying skin is
Scalp Face
Tongue
mouth
Occipital
node
Submandibular
node
Sternomastoid Pharynx
overlying jugular
lymphatic chain
Thyroid
Fig. 9.9 The lymphatic
drainage of the head
and neck channelling into Axilla,
the jugular lymphatic chain thorax
The Side of the Neck 109
Tonsillar fossa
External opening:
anterior low-middle
thirds sternomastoid
Cyst:
Cyst
External
opening
obvious. However, infection in the deep jugular Tenderness of a mass of inflamed nodes is
chain may not exhibit this sign because of the determined on palpation, although it may not be
thick intervening sternomastoid muscle and evident if the inflammatory process has been
investing deep cervical fascia. modified by previous antibiotics. Reflex spasm of
110 9 The Neck
adjacent muscles may produce an acute stiff and mine the ease with which these signs are elicited:
wry-neck. Both active and passive movements a thick overlying muscle provides effective
will be limited and or painful. ‘camouflage’ for even a large abscess.
The relationship of inflammatory lesions to Fluctuation may indicate an abscess with a
the sternomastoid muscle (Fig. 9.8) will deter- liquid centre. The tight, deep cervical fascia,
however, may mask the softness of the lesion if it
is deep seated. The mass should be grasped
between the fingers of one hand, and the other
Parotid hand should compress the centre (Fig. 9.14). The
region proof of fluctuance can be obtained on some
occasions by needle aspiration of pus. In small
infants, where abscesses are common, aspiration
Jugular of the fluid will confirm the diagnosis and, on
channels occasions, may alleviate the need for further
intervention since the antibiotics will cause reso-
lution once the avascular nidus has been
Thoracic
evacuated.
duct
region
1. Examiner
2. Child turns
stands behind
head away
child
3. Hand pushes
against turning
head
4. Tight muscle
palpated to
determine its
relationship
to the mass
Fig. 9.13 How to determine the relationship between a lateral cervical mass and the sternomastoid muscle
The Side of the Neck 111
by antibiotics and (2) atypical mycobacterial motion’ – the duration of the lump is more likely
infection. Suppressed pyogenic infections are to be 6 weeks to 2 months rather than 2 days.
found in all the common drainage areas from the Pain, tenderness and heat are usually not severe
mouth and pharynx. The duration of symptoms is enough to be noticed and a typical ‘cold abscess’
prolonged and inflammatory signs are less obvi- is produced. The natural history of MAIS infec-
ous, particularly if the focus is deep to the sterno- tion is important because if untreated, it will
mastoid. One way to reveal the signs is to stop form a chronic discharging sinus (Fig. 9.15). The
antibiotic treatment and repeat the examination aim is to diagnose the condition before erup-
several days later, at which stage the inflammatory tion through the skin occurs, so that the lesion
signs may reappear. can be excised before an ugly scar develops. The
Mycobacteria from the ‘MAIS’ complex characteristic sign of MAIS infection is purplish
(Mycobacterium avium intracellulare scrofu- discoloration of the skin which precedes the skin
laceum) are common organisms in the soil in breakdown. The colour is caused by low-grade
temperate climates. They will produce disease inflammation in the skin as the infection spreads
in populations not previously immunized or towards it through the subcutaneous tissue and
exposed to the human strain. The symptoms signifies progression of MAIS infection to form a
and signs of inflammation are less than with ‘collar-stud’ abscess (Fig. 9.15). Recognition of
pyogenic infections: MAIS infection produces the ‘collar-stud’ abscess stage is useful for distin-
a diminished inflammatory response in ‘slow guishing it from other lesions (Fig. 9.16).
112 9 The Neck
Skin
Fascia
Fig. 9.15 The four stages of mycobacterium avium intracellulare scrofulaceum (MAIS) infection in a lymph node
b c
Non-inflammatory Masses
This is the cardinal sign of a lymphatic malfor- Does the Mass Move?
mation, also known as a lymphangioma (or cystic
hygroma) (Fig. 9.18), provided that there has not Unlike masses in the midline of the neck, which
been secondary infection or haemorrhage into the move if attached to the tongue, hyoid or larynx,
114 9 The Neck
lateral cervical masses are usually immobile. One Secondary Effects of the Lesion
exception is a mass in the lateral lobe of the thy-
roid, which moves up and down with swallowing When a swelling appears anywhere in the neck,
(Fig. 9.19). Inflammatory swellings tend to have the effects on adjacent normal structures need to
reduced local mobility because cellulitis sur- be considered. Large lesions beneath the deep fas-
rounding an abscess obliterates the normal tissue cia and sternomastoid will compress not only the
planes along which movement would normally carotid sheath but also the central viscera (trachea
occur (Fig. 9.20). and oesophagus). Rare lesions, such as retroster-
nal goitre, may compress the venous drainage of
the head and upper limbs. Oropharyngeal dis-
placement by a large mass (e.g. branchial cyst) is
not a serious concern, but the small calibre of the
trachea makes it vulnerable to lateral compres-
sion. An abscess in the lower jugular nodes or a
thyroid nodule can compress and displace the tra-
chea. Therefore, the position of the trachea should
be determined accurately (Fig. 9.21); displace-
ment suggests that x-rays are needed to confirm
whether there is compression.
Vascular malformations of lymph and blood
vessels are not confined by tissue planes and may
undergo rapid enlargement (secondary to infec-
Moves on Thyroid attached
swallowing to trachea tion, haemorrhage or thrombosis) and compress
the trachea if they are close to the midline.
Vascular anomalies may cause difficulties at birth
by virtue of their enormous size resulting in tra-
Fig. 9.19 Movement of a lateral thyroid swelling on swal- cheal compression and displacement of the head
lowing occurs because the thyroid is attached to the trachea to the contralateral side.
a
Skin
Non - inflamed
mass-mobile
Fascia
Inflamed mass-
Immobile
because tissue
planes obliterated
Fig. 9.20 The different
mobility of a non-inflamed
mass (a), compared with
that of an abscess with
surrounding cellulitis (b)
The Side of the Neck 115
Examination of the neck is not complete until branchial cleft defect may present as a small
contiguous areas have been examined since a punctum at the junction of the middle and lower
pathological process (or its effects) has no regard thirds of the anterior border of the sternomastoid.
for the arbitrary boundaries between the neck and Recurrent infection is common but non-specific;
the head, chest and axillae. however, dribbling of saliva/mucus when infec-
Examination of the upper lobes of the lungs tion is absent is pathognomonic.
by percussion and auscultation will reveal Large vascular anomalies may produce two
whether a low cervical mass extends into the tho- systemic effects of importance:
rax. Particular lesions may have secondary effects 1. Cardiac failure secondary to high blood flow – this
which are the key to their diagnosis. A second manifests itself in infants as tachypnoea and an
enlarged liver (easily diagnosed on percussion).
2. Haemorrhagic disease secondary to platelet
trapping in the lesion, which produces
thrombocytopenia.
Retropharynx
Tonsil
Tongue
Tooth
socket
Cervical nodes
Axillary nodes
Liver
Spleen
Neuroblastoma
Inguinal nodes
tissues, including axillae, groins, liver and spleen mandible, it is likely to be a submental node or
(Fig. 9.23). Particular attention should be paid to dermoid cyst. If it is situated in relation to the
examination of the abdomen to look for evidence hyoid bone, a thyroglossal cyst should be consid-
of neuroblastoma, which arises from the adrenals ered (Fig. 9.25). Submental lymph nodes often
or sympathetic ganglia and commonly spreads to are small and may be multiple and slightly to one
lymph nodes, particularly Virchow’s node. side of the midline. Likewise, thyroglossal cysts
close to the thyroid cartilage may be pushed to
one side, whereas dermoid cysts almost always
Midline Swellings of the Neck remain in the midline.
Table 9.3 Midline neck swellings present in this way. The erythema surrounding an
Likely lesions infected thyroglossal cyst extends laterally fur-
1. Submental lymph node ther than it does in the midline (i.e. it progresses
Immediately behind mandible transversely more than vertically). This is so
Usually small, may be multiple characteristic of a thyroglossal cyst, it is pathog-
Evidence of regional infection (usually inside the nomonic. The swelling should be inspected for
mouth) evidence of a fistula opening, through which a
2. Dermoid cyst
discharge may be intermittent or have occurred
Slow growing and spherical
previously – this would be consistent with a thy-
Does not transilluminate (contains sebaceous material)
roglossal fistula (Fig. 9.26).
Painless
Not attached to the hyoid – usually just behind the
mandible or just above sternal notch
3. Thyroglossal cyst Does It Transilluminate?
Unilocular hemisphere
May be large or enlarge rapidly This requires a strong torch and dark room. The
Attached to the hyoid dermoid cyst contains thick secretions and will not
Often transilluminable (contains clear mucus) be translucent, whereas a thyroglossal cyst may
Commonly develops secondary infection transilluminate (Fig. 9.27). A thyroglossal cyst
4. Ectopic thyroid will not be translucent when it is infected, contains
Similar in site to thyroglossal cyst solid thyroid tissue or is lying more deeply.
Solid
Not translucent
Normal thyroid is absent
Does It Move?
5. Goitre
Neonate or pubertal girl
The dermoid cyst moves with the skin in all direc-
Diffuse
Lower neck
tions but moves little on swallowing or tongue
Submental nodes
Submental dermoid
Hyoid
Common area
Thyroglossal
for thyroglossal
cyst/ectopic
cysts
thyroid
Suprasternal
Fig. 9.24 The common dermoid
positions of midline cervical
masses
118 9 The Neck
Lingual
thyroid
Thyroglossal cyst
or ectopic thyroid
Laterally-
spreading
cellulitis
Fistula
Pus/saliva/mucus
Fig. 9.31 Thyroid palpation. 1. Stand behind the child. 2. Insinuate the tips of the fingers under the edges of the ster-
nomastoid to feel each lobe. 3. Feel the gland while the child swallows to detect movement
Midline Swellings of the Neck 121
Bulging fontanelle
Wide sutures
Head
circum-
ference
Head within
Fig. 10.3 The clinical signs of hydrocephalus (lateral
normal range view)
but enlarging
quickly
veins are visible under an attenuated and tight
scalp. Sutures not commonly open, such as the
Age
metopic and lambdoid sutures, may be palpated.
Fig. 10.2 The head circumference chart, showing the The eyes appear to deviate downwards to give the
two common patterns seen in a child with a large head. so-called setting-sun appearance. The explana-
Increase in size at a rate faster than normal is suggestive of
tion for this is not completely understood but may
hydrocephalus
be compression of brain stem oculomotor con-
nections in the quadrigeminal plate of the fourth
be seen and palpated, may be larger than normal ventricle. Occasionally, the shape of the occiput
and bulging with increased pressure from within. is helpful since the size of the posterior fossa is
The sutures are widely placed, with a gap palpa- determined by the type and site of the obstruction
ble between adjacent bones of the vault. The to CSF (Fig. 10.5). Percussion of the normal skull
forehead tends to overhang the relatively smaller (Fig. 10.6) produces a high-pitched, drum-like
face, with the anterior horns of each dilated lat- resonance compared with the hydrocephalic skull
eral ventricle pushing out the sides of the fore- which has a sound which has been likened to that
head to produce ‘bossing’. Superficial, congested of a cracked earthenware pot in the older child
The Head 125
Persistent
metopic suture
Frontal Bossing
Enlarged
bulging
anterior
fontanelle
Setting-sun
eyes
Scalp
a b
where the sutures are interdigitating, and a more gradually replaced by CSF) will produce focal
hollow percussion note in the infant. areas of transillumination.
The child should be taken to a dark room for All children with a proven or suspiciously
transillumination of the skull (Fig. 10.7). A bright large head should be referred to a paediatric cen-
torch is needed, but it can provide useful infor- tre for detailed investigation. Skull x-rays, cranial
mation even before CT/ultrasound or MRI is ultrasonography (if the fontanelles are open),
undertaken. Extreme dilatation of all the ventri- computed tomography scan or magnetic reso-
cles with little brain present will make the entire nance imaging may be performed.
vault transilluminate, whereas a localized cyst or The classic signs and symptoms of hydrocepha-
subdural hygroma (haematoma which has been lus are seen only in the infant, before closure of the
126 10 The Head and Face
The 2 presentations
of
hydrocephalus
vomiting
headache
papilloedema
Cause Cause
sutures (Fig. 10.8). After closure of the sutures, the ous shunt surgery is vital. It is also important to
cranium cannot expand readily, and an increase in know whether there has been a delay or deteriora-
its contents leads to an increase in intracranial pres- tion in milestones and mental development, or a
sure. The sutures close at about 4 years of age or change in recent behaviour, which suggests a
earlier if previous hydrocephalus has been treated slowly progressive lesion (e.g. tumour). Depression
with a ventricular shunt. This may have drained the of the conscious state may be present, but often
CSF too well and allowed the bones to stick this is a late feature and a normal conscious state
together before the brain tissue expanded to fill the does not necessarily indicate that all is well. The
space. The cardinal symptoms and signs of raised falling pulse and rising blood pressure are classic
intracranial pressure are vomiting (often early in signs of raised intracranial pressure. However, in
the morning), headache and papilloedema. childhood, these parameters may remain normal
Assessment of possible raised intracranial pres- until the intracranial pressure is so high that the
sure must take into account whether there is a CSF patient is unconscious and close to death.
shunt already present (Fig. 10.9). If there is a shunt Examination of the retina will confirm the
in situ, it is highly likely that malfunction of the presence of papilloedema, the cardinal sign of
shunt is the cause. If there is no shunt, the cause is raised intracranial pressure (see below). The sides
likely to be a neoplasm, unless the child has had of the head behind and above the ears should be
meningitis. Therefore, in taking a history of a child palpated to find the ventriculoperitoneal shunt
with headaches and vomiting, knowledge of previ- and test its function (see below). If there is no
Headache
Drowsiness
Papilloedema
?Shunt
malfunction
?Neoplasm
Fig. 10.9 The presentation of
hydrocephalus after fusion of
the sutures: there may be a
Vomiting
history of previous shunt
surgery and/or mental
impairment. Shunt blockage is
the likely cause if hydrocepha-
lus was treated previously. If
there is no CSF shunt,
localizing neurological signs
should be sought to exclude a
brain tumour CSF drain in heart /abdomen
128 10 The Head and Face
1. Focus on vessels
2. Wait for disc Optic disc
ICP
Swollen disc
2) Change
focus to see
top of disc
1) Focus on
lateral vessels
shunt present, a cerebral neoplasm is possible and in the same focal plane, the ophthalmoscope is
localizing neurological signs should be sought. focused on the peripheral vessels first (Fig. 10.10).
The examiner then waits for the disc to move into
the field of view as the child’s eye moves.
Looking for Papilloedema When papilloedema is present, the disc is
blurred at the normal focal length at which the
Small children are not able to keep their eyes peripheral vessels are clear (Fig. 10.11). The
still, which makes examination of the optic disc blurred image is easily mistaken for inadequate
difficult. Since the disc and peripheral vessels are technique or an uncooperative child. If the periph-
The Head 129
c
Papilloedema
b - Disc in focus
- Vessels blurred
Papilloedema
- Vessels in focus
- Disc blurred
Haemorrhages
Brain
CSF in fibrous
sheath
b
Distal CSF
leak
Pump empties
and refills
Abnormal or premature fusion of the sutures Lumps in the midline between the nose anteriorly
(craniosynostosis) will distort the shape of the and the foramen magnum posteriorly may be
vault (Fig. 10.17), but the commonest anomaly is manifestations of incomplete fusion of the neural
plagiocephaly, associated with torticollis (see tube or its overlying ectoderm. Dermoid cysts,
Chap. 9). similar to the common external angular dermoid,
132 10 The Head and Face
Mandible
Rare: Common:
internal external
angular dermoid angular dermoid
1. Soft
2. Transilluminable
Cyst over glabella:
3. Bony defect
4. ± Pulsation
Normal
Antihelical
fold
Hypertelorism
‘Bat’
ear
Concave
concha
The Ears
Helix
Pinna deficient
or deformed
Antihelix
Preauricular
sinus cartilage
Tragus
Preauricular skin
tag/ cartilage
Inner ear
Inner ear absent
present
Mucosa inflamed
around orifice
Ear lobe
displaced
laterally
Surgical conditions arising within the mouth form pale band which fetters the tip and, in severe
a heterogeneous collection (Table 11.1), and the cases, prevents protrusion of the tongue beyond
approach to diagnosis, therefore, depends initially the lower incisor teeth. The tongue may be
on inspection of the site of origin of the lesion. anchored so closely to the floor of the mouth
Specific points in the examination of each lesion behind the lower gum that the frenulum is difficult
are described separately according to their site. to see.
It can cause interference with breastfeeding
and speech but never affects swallowing as this is
The Tongue a function of the posterior part of the tongue.
The aim of examination is to determine how
Tongue-Tie far the tongue can protrude. Articulation is
impaired when the tongue cannot protrude past
Tongue-tie is caused by a very short frenulum the teeth (Fig. 11.1), when attempts at poking it
extending from the tip of the tongue to the floor out simply result in it rolling up (Fig. 11.2). It is,
of the mouth. The frenulum appears as a tight therefore, its relationship to the incisor teeth
which determines the necessity for surgical divi-
Table 11.1 Pathological conditions in the mouth sion of the frenulum. Many children with tongue-
tie also have a tight maxillary frenulum.
Site of Common Uncommon
origin conditions conditions
Tongue Tongue-tie Macroglossia
Lymphangiomatosis Macroglossia
Lingual thyroid
Floor of Ranula Dermoid cyst Macroglossia describes real or apparent enlarge-
mouth Lymphatic Submandibular ment of the tongue. It is a well-known feature of
malformation calculus Down syndrome. Any space-occupying lesion
Gums Alveolar abscess Dentigerous cyst inside the tongue will produce macroglossia.
Lips Mucus retention Papilloma Alternatively, a normal tongue within a small oral
cyst
cavity will appear relatively large as in Pierre-
Cleft lip Vascular
malformation Robin syndrome (Table 11.2). Macroglossia may
Palate Cleft palate Submucous cleft occur as part of a generalized syndrome, for
palate example, hypothyroidism, Beckwith-Wiedemann
Oropharynx Tonsillitis Quinsy syndrome or as a manifestation of local pathol-
Retropharyngeal ogy, for example, a lymphatic anomaly with
abscess lymphangiomatosis.
Frenulum
Short frenulum
Fig. 11.2 Protrusion of the tongue as the diagnostic test for tongue-tie requiring treatment. Normally, the tongue protrudes
well beyond the teeth (a), but in severe tongue-tie, the tongue cannot be protruded past the teeth and tends to roll up (b)
Inspection and palpation of the tongue should neurofibroma or rare tumour, e.g. rhabdomyosar-
determine whether there is a space-occupying coma). When the tongue is enlarged uniformly, a
lesion. Where a localized lesion is found, further general examination to identify the cause involves
examination determines whether it is composed looking for organomegaly (Beckwith-Wiedemann
of lymphatic fluid (which may transilluminate or syndrome) or features of Down syndrome
have multiple ‘blebs’ on the surface), blood (vas- (Fig. 11.3). Micrognathia and a rectangular defect
cular spaces which empty on compression) or in the palate confirm the diagnosis of Pierre-
solid tissue (feels hard and may be due to a Robin syndrome (Fig. 11.4).
Lesions in the Floor of the Mouth 141
Simian
crease
? Duodenal
atresia
Exomphalos
Big baby
Sloping
eyes
? Cardiac
anomaly
Grooved General
ear lobes organomegaly
Fig. 11.3 Macroglossia: diagnosis of the underlying cause may be evident on general examination, such as in Down
syndrome or Beckwith-Wiedemann syndrome
142 11 The Mouth
± Micrognathia
Respiratory
distress
Rectangular
palatal defect
a predominance on one side, but it can extend There may be some disturbance in speech or
across the midline beneath the frenulum of the swallowing. Most ranulas are 2–4 cm in diame-
tongue (Fig. 11.6). It has a translucent blue or ter and lie immediately beneath the thin mucosal
grey appearance, and the submandibular duct lining of the floor of the mouth. Ranulas may
may be seen draped across its superficial sur- rupture with exudation of clear mucus and then
face. Larger cysts lift the tongue up towards the reaccumulate over several days or weeks.
roof of the mouth or extend downwards produc- On examination, ranulas should be distin-
ing a lump under the chin (the ‘plunging’ ranula). guished from dermoid cysts and lymphatic
Lesions in the Floor of the Mouth 143
Lip mucus
retention cyst
Dentigerous cyst
Calculus
stuck in
Saliva and duct opening
patent orifice
anomalies of the tongue. The rare dermoid cyst is Salivary Duct Calculus
firm, deeper within the tissue of the tongue and
usually in the midline. There may be a tiny punc- Stones of the salivary glands are limited almost
tum on the surface of the tongue. A lymphatic entirely to the submandibular duct. The calculus
anomaly (‘cystic hygroma’) is less well defined gets stuck at the narrowest part of the duct which is
and lacks the extremely superficial and discrete immediately proximal to the orifice in the floor of
appearance of the ranula. The ranula is best the mouth, lateral to the frenulum. The calculus is
thought of as being on top of the floor of the visible through the thin layer overlying it as a white
mouth, while the lymphatic anomaly (‘cystic or yellow hard lump about 2–5 mm in diameter
hygroma’) is in the floor of the mouth. The cystic (Fig. 11.7).
hygroma of the floor of the mouth usually has a Where the calculus blocks the flow of saliva,
substantial cervical component. the ipsilateral submandibular gland is enlarged
144 11 The Mouth
Dentigerous Cyst
The alveolar abscess results from a deep-seated infec- A short upper lip frenulum may extend between
tion around a permanent tooth, particularly a lower the two halves of the maxillary arch and may
molar. The infection causes severe pain, local ten- cause separation of the upper incisor teeth
derness and fever. Uncontrolled inflammation may (Fig. 11.10). It tethers the upper lip and may
Lesions of the Palate 145
Fronto-nasal
process
Globular
process
(premaxilla)
Maxillary
process
acquire a horizontal plane and fuse (Fig. 11.13). the soft palate where the two edges of the palate
Failure of this fusion results in cleft palate. Clefts are joined only by mucous membrane. In the
of the palate can occur as isolated lesions and older child with a previously unrecognized sub-
extend anteriorly only as far as the incisive fora- mucous cleft, the palatal speech impediment is
men or may be associated with anterior defects in obvious.
the alveolus (gum) and lip. The more severe and common lesion is the
As with cleft lip, the severity of deformity is palatal cleft which extends anteriorly as far as the
variable (Fig. 11.14). The observation of a bifid incisive foramen immediately behind the gums.
uvula is very suggestive of a submucous cleft. This is obvious when the infant cries.
This condition is missed easily because the pal- Isolated cleft palate occurs also as part of the
ate appears intact at first glance (» 95 %) but, Pierre-Robin syndrome. It is due to failure of the
on closer examination, a notch can be felt in the tongue to sink into the floor of the mouth which
midline at the posterior edge of the secondary prevents fusion of the two halves of the palate
or hard palate, and there is a midline groove in (Fig. 11.13). The other features of the syndrome
Lesions of the Oropharynx 147
a Vomer
Palatal
shelves
Tongue
Macroglossia
Fig. 11.13 Development of the hard palate. (a) Primary of the vomer is visible. (b) Secondary failure of palatal
failure of fusion of the palatal shelves and vomer produces fusion caused by overgrowth (macroglossia) or upward
the common slit-like cleft, through which the lower edge displacement (micrognathia) of the tongue
include micrognathia and a posteriorly positioned losing its relationship to the hard palate which is
tongue with respiratory distress at birth. derived from the maxillary processes.
The cleft extends up either side of the nasal
septum to produce an open communication
Clefts of Both Lip and Palate between the oral cavity and both nasal cavities.
Complete Complete
cleft (unilateral) cleft (bilateral)
Golden Rules
1. Tongue-tie requires no treatment unless
the tongue cannot be protruded beyond
the teeth.
2. Lingual thyroid: should not be removed
because there is no normal thyroid.
Fig. 11.15 The technique of palpating a retropharyngeal
abscess. Note the hyperextended neck, which is the posi-
3. A ranula is on top of the floor of the
tion adopted by the child to keep the airway open mouth, while a lymphatic anomaly
(‘cystic hygroma’) is within or below
the floor of the mouth.
Acute Retropharyngeal Abscess 4. Lip pigmentation in association with
intussusception indicates Peutz-Jehger
The retropharyngeal space contains lymph nodes syndrome with intestinal polyposis.
which drain the oropharynx and nasopharynx. 5. Cleft lip or palate are common features
They may become infected and suppurate, lead- of recognizable syndromes.
ing to the development of a retropharyngeal 6. The dribbling child with neck extension
abscess. This usually occurs in the child under may have peritonsillar or retropharyn-
2 years of age. The abscess extends to the glottis geal abscess.
and may cause respiratory obstruction and, if not
recognized, death. The infant or small child holds
the mouth open and the head in full extension;
this posture is an early clue to the diagnosis. The Further Reading
child is febrile and toxic. Occasionally, depres-
sion of the tongue enables the abscess to be seen Puri P, Höllwarth M (eds) (2009). Pediatric Surgery.
Diagnosis and Management. Springer, Dordrecht.
as a unilateral swelling in the pharynx: the tight
Trauma
12
Children are particularly susceptible to injury. or is inconsistent with the stage of develop-
Their perception of danger and their ability to ment of that child.
recognize a potentially hazardous situation have 3. Repeated clinical examination is required to
not developed adequately, and their motor diagnose injuries not apparent initially.
response to that danger may be neither appropri-
ate nor effective. Furthermore, their dependence
on adults and lack of physical prowess makes Initial Assessment of the Severely
them vulnerable to abuse and maltreatment, Injured Child
which in turn may produce significant physical
(as well as mental) injuries. The types of trauma The initial approach of the clinician to the severely
children suffer differ from those of the adult in a injured child may be life-saving (Table 12.1). In all
number of important respects which are discussed situations, the highest priority is the establishment
in this chapter. and maintenance of an airway, for without this,
To perform a goal-oriented examination of an anoxia will develop and all other manoeuvres
injured child, the clinician needs to know the mech- become futile. Once a patent airway is established,
anism of injury. Since the severity and type of ventilation must be adequate to ensure oxygen-
pathology produced varies with the type of injury, ation. Attention is then turned to the assessment
an exact account of the accident allows certain inju- and stabilization of the circulatory state. External
ries to be predicted. Conversely, the type of injury bleeding must be arrested, either by pressure or
provides information about the nature of the trauma, elevation. Subsequently, the whole body is exam-
which may be a key step in the diagnosis of child ined for evidence of other injuries, but it must be
abuse. Pedestrian accidents are an example where emphasized that this examination must not delay
certain injuries can be predicted even in the uncon- treatment of the life-threatening injuries.
scious child (Fig. 12.1). Likewise, motor accidents
where the child is wearing a lap seat belt cause pre-
dictable injuries (Fig. 12.2). A fall off a bicycle Ensure a Patent Airway
may produce a handlebar injury (Fig. 12.3) with an
isolated injury to the spleen, pancreas or liver. The first priority is to rule out upper airway
Three general principles should be applied to all obstruction. Severe obstruction causes cyanosis,
cases of trauma: while lesser degrees are evident as a rapid respi-
1. Look for injuries which match the severity and ratory rate, restlessness, anxiety and an increase
type of accident. in respiratory effort manifested by indrawing of
2. Suspect child abuse if the history of the ‘acci- the ribs, sternal retraction and use of the accessory
dent’ does not match the severity of the injuries muscles of respiration. The tongue may fall
Head hits
bonnet
Variable
injuries
Fig. 12.1 The mechanism of pedestrian injury in a collision with a fast-moving car
Head hits
front seat/door
Lap-belt
Lumbar
ligaments
torn
Fig. 12.2 The mechanism of lap-belt injury. Acute hyperflexion of the spine may tear posterior vertebral ligaments.
The bowel may burst from compression against the sacral promontory
Abdominal Trauma 153
Small circular
bruise from
end of handle-bar
Table 12.1 Priorities in the assessment of the severely cyanosis. Inadequate breathing may require addi-
injured child tional oxygen or assisted ventilation (e.g. ‘bag-
(a) Airway patency ging’ by mask or endotracheal intubation).
(b) Breathing established
(c) Circulation state
(d) General and neurological examination Stabilize the Circulation
backwards and obstruct the oropharynx in the External bleeding can be controlled by direct pres-
supine child. This can be overcome by lifting the sure over the wound, digital pressure of the regional
chin upwards or by pulling the mandible forwards artery against a bony prominence (e.g. control of the
with the fingers behind the angle of the jaw femoral artery by pressure in the groin, and the bra-
(Fig. 12.4). Secretions, blood and vomitus also chial artery by pressure against the medial aspect of
may obstruct the upper airway: the mouth and the mid-humerus) or by elevation of the injured limb.
pharynx should be inspected and suctioned clear Internal bleeding is suspected from the nature of the
if necessary. Insertion of an oropharyngeal tube injury, superficial bruising and evidence of blood
may be necessary to maintain airway patency or, loss (tachycardia, pallor, poor peripheral perfusion,
in the unconscious child who is unable to breathe, cool periphery and hypotension). Common sites of
endotrachial intubation should be performed. significant internal bleeding include the chest, abdo-
men, pelvis, thigh and, in the infant, the head.
Angle of jaw
history and preliminary examination as to which bruising and lacerations. Palpation of the abdo-
of these groups the patient belongs. Abdominal men will demonstrate tenderness.
trauma most often affects boys and is blunt and
non-penetrating in 99 % (except in societies with
high gun use, where bullet wounds are common). Which Organ Is Involved?
In 85 %, the injury occurs during a motor vehicle
accident, during sport or play, or following a fall. The age of the child, the mode of injury and exter-
If the cause and circumstance of the trauma are nal signs will suggest which organ is likely to be
not clear, or if the injuries do not match the history injured. For example, a kick to the loin with over-
provided, then child abuse should be considered. lying bruising might suggest a renal injury, whereas
Examination of a child with abdominal trauma a fall onto a handlebar bruising the left hypochon-
includes examination of the genitals, perineum drium would raise the possibility of splenic or pan-
and back. creatic trauma (Fig. 12.3). Injury to specific organs
results in specific effects: the spleen, liver, kidney
and great vessels bleed; the bowel leaks its con-
How Do I Know That There Is Trauma tents; the urinary tract leaks urine; and the pan-
to the Abdomen? creas releases exocrine enzymes. Determination of
which of these processes has occurred provides
Where the injury is isolated, there will be a clear further clues as to the affected organ. More specific
story of direct trauma to the abdomen, for exam- details are obtained through imaging investiga-
ple, a kick in the belly or fall onto a bicycle han- tions or laparotomy/laparoscopy.
dlebar (Fig. 12.3), and the patient will complain
of abdominal pain. Often, the story will be of
progressive pain starting at the time of, or shortly How Do I Know That Bleeding Has
after, the accident. The discomfort may be local- Occurred?
ized to one part of the abdomen.
The abdomen should be inspected closely for A child can compensate for moderate blood loss
external evidence of trauma such as abrasions, without showing clinical evidence of shock, a
Abdominal Trauma 155
fact which leads the inexperienced clinician to men observed. Blood irritating the undersurface
underestimate the amount of blood loss. There is of the diaphragm causes pain which is referred to
a rise in the pulse rate which the unwary may the shoulder tip, reflecting the cervical innerva-
attribute entirely to pain. The blood pressure is tion of the muscle.
normal until a relatively late stage, but then it Retroperitoneal haematomata occur as a result
may drop rapidly. The limbs remain surprisingly of injury to retroperitoneal organs, the common-
warm and well perfused despite moderate blood est of which is the kidney. The degree of anterior
loss. Remember, however, that limb temperature abdominal tenderness on examination is less than
in a child depends partly on the ambient air tem- with intraperitoneal bleeds, but abdominal disten-
perature and the amount of clothing worn and sion still occurs because of the retroperitoneal
should be assessed accordingly. haematoma itself and as a result of the secondary
Major blood loss causes pallor, but again other ileus that it produces. Further confirmation of
factors contribute to the child’s colour – if vomit- intra-abdominal bleeding may be seen on plain
ing has occurred, many children look pale with- x-rays of the abdomen where fluid displaces adja-
out any blood loss. Sweating and poor peripheral cent loops of bowel and on observing a fall in the
perfusion are seen with greater degrees of blood haemoglobin level during the first 12–48 h.
loss. In short, it is difficult to estimate the amount
of blood loss in children from their general signs
until a late stage. Yet, it is important to estimate How Do I Know That Bowel Contents
the amount of blood lost so that adequate fluid Are Leaking?
resuscitation can be implemented before cardio-
vascular collapse occurs. Careful examination of When perforation or laceration of the bowel
the abdomen, therefore, is required to assess the occurs, intestinal contents leak, usually into the
presence and extent of bleeding there. peritoneal cavity. These may be gastric juice,
The signs of intraperitoneal bleeding are (1) bile, small bowel fluid or faeces according to the
distension and (2) tenderness. The distension site of injury, and each produces signs of rapidly
increases with continued bleeding. Assessment progressing peritonitis. The child looks unwell
of abdominal distension involves first removing and toxic. Within hours, the abdomen becomes
any clothes or blankets which are obscuring the distended and, while tenderness may be localized
abdomen and then observing the degree of protu- initially, it rapidly becomes generalized, severe
berance whilst the patient is lying relaxed in a and accompanied by involuntary guarding. It
supine position. The girth can be measured by should be apparent that the signs of perforated
placing a tape measure around the trunk and bowel and haemoperitoneum are not dissimilar
under the lumbar lordosis. The exact position of except in:
the tape during the first recording should be 1. Degree – the tenderness with perforation is
marked on the skin of the flanks and abdomen. more marked.
Subsequent measurements at regular intervals 2. Progression – leaking of bowel fluid into the
will identify progressive abdominal distension. peritoneal cavity persists and causes rapid
The child’s smaller abdominal cavity makes progression of signs, whereas with rupture of
serial girth measurements more sensitive than in a solid viscus the bleeding usually ceases
the adult but is subject to certain limitations: free spontaneously and the physical signs change
blood within the peritoneal cavity causes a tem- slowly or are static.
porary paralysis and dilatation of the bowel 3. Other signs of blood loss – the signs of shock
(ileus), which increases distension. Likewise, air are less evident with peritonitis.
swallowing increases abdominal distension and 4. Mode of injury – occasionally, knowledge of
makes girth measurements taken in isolation less the mode of injury is helpful.
valuable in the detection of continued intraperito- Nevertheless, it is often very difficult to distin-
neal bleeding. The location of maximal tender- guish the two situations, in which case careful
ness to palpation should be recorded and its reappraisal of the clinical signs, serial examina-
severity and extension to other parts of the abdo- tion at short intervals and recourse to other inves-
156 12 Trauma
tigations (e.g. CT scan) may be necessary. An time of a plain x-ray or initial imaging of the
abdominal x-ray showing free gas would confirm abdomen. Extravasation of urine is evident as
gut perforation. contrast outside the normal urinary tract. In other
Leakage into the retroperitoneum produces situations, ultrasonography or nuclear medicine
less dramatic signs, and delay in diagnosis is more scan may be more appropriate.
likely. The child may develop a profound ileus,
remain obtunded and be febrile. Retroperitoneal
duodenal injury may occur after a lap-belt acci- When Should Specific Investigations
dent and presents as a high bowel obstruction Be Performed?
(duodenal haematoma obstructing the lumen) or
with progressive but non-specific signs of sepsis The necessity for further investigations depends on:
(duodenal laceration and retroperitoneal abscess 1. The suspected lesion as determined on clinical
formation). Pancreatic injuries often present late grounds
with a tender epigastric mass, representing a pan- 2. The general condition of the patient
creatic pseudocyst. 3. The facilities available
The organs most commonly injured are the
spleen and liver. Where clinical examination sug-
How Do I Know That There Is Urinary gests that splenic injury is likely to be an isolated
Tract Damage? lesion and the child is stable, surgery is not
required immediately (if at all), and the liver and
Direct trauma to the back and loins is more likely spleen can be outlined with ultrasound or CT
to damage the urinary tract than the gastrointesti- scan with contrast as a non-urgent procedure.
nal tract. A fracture of the pelvis, particularly if it
involves the pubic rami, may injure the bladder or
urethra. Unless there has been complete interrup- Thoracic Injuries
tion of the urethra, there will be haematuria.
Therefore, examination of the urine is mandatory Major thoracic injuries are uncommon in child-
where the possibility of urinary tract trauma hood and tend to occur in association with mul-
exists. Injury to the kidney may result in major tiple injuries of other organs, for example, the
retroperitoneal haemorrhage causing abdominal head and abdomen.
tenderness and distension, and signs of blood The elastic and pliable rib cage of the child
loss. Disruption of the pelvicalyceal system or results in compression injuries of the intratho-
transection of the ureter allows urine to leak into racic structures (Fig. 12.5). Consequently, multi-
the retroperitoneal space, contributing to the ret- ple rib fractures are not seen often, whereas the
roperitoneal collection. The urinary tract can be upper abdominal organs and lungs are damaged
outlined by performing a CT with contrast at the frequently (Fig. 12.6).
Rib cage
Rib intact
Organ
Thoracic injury should be suspected in any of intrathoracic injury which may be life-threat-
child with multiple system trauma, respiratory ening and, although not common, must be recog-
difficulties or evidence of blood loss (Fig. 12.7). nized and treated promptly (Table 12.2).
Again, the first priority is to rule out upper airway
obstruction by inspection of the mouth and phar-
ynx for secretions, blood and vomitus and by
confirming that the tongue is not obstructing the Table 12.2 Consequences of intra-thoracic trauma
oropharynx. There are a number of complications Early life-threatening 1. Tension pneumothorax
complications 2. Major haemothorax
(uncommon)
3. Cardiac tamponade
(uncommon)
4. Flail chest (very rare)
Left 5. Open pneumothorax
hemidiaphragm (very rare)
Underlying injuries 1. Pulmonary contusion
2. Pneumothorax
Liver Spleen 3. Haemothorax
(posterolateral)
4. Fractured ribs
5. Traumatic asphyxia
Kidney 6. Tracheobronchial tear
(posterior) 7. Oesophageal tear
8. Diaphragmatic tear
Fig. 12.6 Surface markings of organs likely to be dam-
9. Aortic tear
aged by rib compression: the organs at greatest risk are
actually below the diaphragm. 10. Myocardial contusion
Head injury
Tongue obstructing
airway
Vomitus in airway
Lung contusion
Pneumothorax
# ribs
Ruptured
Haemothorax diaphragm
Pneumoperitoneum
Acute gastric
dilatation
Fig. 12.7 The causes of Haemoperitoneum
respiratory distress in trauma
158 12 Trauma
Air
volume
Heart with
Heart each breath
I.V.C
IVC flow
obstructed
Diaphragm
depressed
Open pneumothorax virtually never occurs in chil- Head injury is one of the commonest types of
dren but is readily recognized as a wound which trauma in childhood and is the main cause of
transmits air (bubbles and sucks) during ventila- morbidity and mortality. Head injuries account
tion. The defect should be covered to prevent fur- for 80 % of deaths from trauma, and most chil-
ther air movement and a chest tube inserted. dren with multiple system injuries have a head
injury. Injury to the brain occurs in two phases:
Traumatic Asphyxia first, there is primary brain damage sustained at
the time of the accident, and second, there is
Severe compression of the chest may completely additional injury which occurs as a consequence
block venous return in the large veins of the tho- of both the local and distant injuries (Table 12.3).
160 12 Trauma
The four stages of assessment Table 12.4 Basic neurological assessment in head
of head injury injuries
1. Establish airway and 1. History
ventilation Mechanism of injury
State when first seen
2. Baseline neurologic -Conscious level
examination pupillary signs Focal signs
limb movement Deterioration
vital signs 2. Level of consciousness
compound injury 3. Pupils
3. More detailed -Detailed neurological Size
examination examination Reactivity to light and accommodation
associated injuries
4. Fundi
4. Look for change -Conscious level Retinal haemorrhages
in signs pupils
neurological deficit (Papilloedema)
vital signs 5. Movement of limbs
Posture
Fig. 12.11 The four stages of assessment in a child with Movement of each limb
a head injury Flaccidity/spasticity
6. Plantar response
This assessment can be divided into four 7. Pulse rate
stages as outlined in Fig. 12.11. 8. Blood pressure
9. Respiratory rate
Apnoea
The Initial Neurological Assessment Cheyne-Stokes respirations
10. Leak of cerebrospinal fluid
Otorrhoea
This is a simple and quick assessment of the neu-
Rhinorrhoea
rological status of the child, with a more detailed
examination being deferred until initial resuscita-
tion and stabilization (ABC) are complete. This full (e.g. ‘alert and orientated’ at one extreme or
initial examination determines the urgency of ‘unresponsive to painful stimuli’ at the other
neurosurgical care and provides baseline infor- extreme). Assessment of consciousness involves
mation for the assessment of subsequent progress observation of the child’s motor response to sen-
(Table 12.4). sory stimuli and evidence of higher cerebral func-
tion (verbalization, orientation).
History One useful method of documenting conscious-
The history provides important information about ness level is to employ the Glasgow coma scale
the time and mode of injury, the state of the child (Table 12.5). This method has gained widespread
immediately after the accident and whether deterio- acceptance throughout the world; it enables the cli-
ration or improvement in the child’s condition has nician to assess consciousness level objectively and
occurred during transport to hospital. Some of this to monitor its progress with time. It tests neurologi-
information can be gained from eyewitness accounts, cal function in eye opening, verbalization and
but the most useful source of detail is often the motor response. The score of each is recorded along
ambulance officer who was first on the scene. It is with the time and date at which the observation was
essential to speak to the ambulance officers before made. A cumulative score of 10 or less signifies a
they leave the emergency department. serious head injury. In the absence of shock, a
decrease in the score of three or more strongly sug-
Level of Consciousness gests development of a major complication.
It is not adequate to describe a child as being Although the Glasgow coma scale is widely appli-
‘conscious’, ‘semi-conscious’ or ‘unconscious’. cable, determination of the best verbal response of
The level of consciousness must be described in children under 3 years of age in whom verbal skills
162 12 Trauma
are not fully developed may be difficult. In this situ- Table 12.6 Causes of fixed, dilated pupils
ation, it is reasonable to give a verbal score of five Traumatic iridoplegia Damage to the orbit and eye
if the child cries in response to stimulation. Associated with direct trauma
to the face and head
Pupils Child is otherwise alert and
Pupillary size and reactivity afford information on orientated
the severity, prognosis and progression of head Direct nerve damage Injury to the third nerve
Optic nerve injury
injury. The size of each pupil is measured separately
Cerebral ischaemia Profound shock
and its diameter in millimetres recorded (Fig. 12.12).
Cerebral anoxia
The pupillary reflex is tested by shining a bright
‘Brain death’
light into each eye in turn and recording the rapidity
Increased intracranial Pressure on the third nerve
and degree of pupillary response. If the light is pressure Intracranial bleeding
shone into both eyes at once, an intact consensual Gross cerebral oedema
reflex permits a normal bilateral pupillary response
even though one optic nerve may be divided.
There are several causes of fixed, dilated pupils pressure signify severe injury to the brain and have
(Table 12.6). The fixed, dilated pupils of traumatic a very poor prognosis. An expanding intracranial
iridoplegia and third nerve palsy from direct injury space-occupying lesion (e.g. extradural haema-
to the eye may be associated with a good neurologi- toma) may produce initial dilatation of one pupil
cal prognosis, whereas fixed, dilated pupils caused and may be followed shortly by dilatation of the
by cerebral ischaemia or increased intracranial contralateral pupil. These findings frequently will
Head Injuries 163
a Bilateral
Feet plantar
flexed
b Unilateral
Legs
Arms extended
extended
Wrists
flexed
‘Downgoing’
- Normal in child
who has walked
be controlled by direct pressure and application Table 12.7 Normal respiratory rates in children
of pressure dressings. In some fractures, the dural
Age (years) Breaths (min)
sinuses may be torn; in this situation, an external
0–1 25–30
pressure dressing must be applied before elevat-
1–5 20
ing the head, to prevent air embolism. 6–12 15
The development of bradycardia in the pres- 13 → 12–15
ence of increasing blood pressure is a late sign of
increasing intracranial pressure and often indi-
cates the need for neurosurgical intervention. neurological insult or a high cervical spinal injury
Other signs of deterioration may have appeared (phrenic nerve palsy). The coexistence of gener-
already. alized flaccidity of the limbs and hypoventilation
demands careful examination of the cervical
Blood Pressure spine. Although apnoea is a poor prognostic sign
Hypotension with a head injury is caused usually in head injuries, resuscitation should proceed as
by internal haemorrhage elsewhere rather than some of these children recover.
from intracranial bleeding. The exceptions to this Assessment of the respiratory rate should
rule are (1) where there is obvious external haem- take into account the age of the patient
orrhage from the scalp, (2) where a scalp haema- (Table 12.7). Infants and toddlers have a much
toma is present in an infant or (3) where intracranial faster rate than older children. At rest, and in the
haemorrhage occurs in the neonate and infant. absence of other stimuli, respiratory rate remains
Increasing blood pressure in conjunction with relatively constant. A pattern of increasing res-
a decline in the pulse rate (Cushing’s response) is piration followed by decreasing amplitude with
a late sign of increasing intracranial pressure. a period of apnoea (Cheyne-Stokes respirations)
suggests upper midbrain or diencephalic
Respiratory Rate derangement and, if it appears following previ-
Complete absence of respiratory effort after a ously normal respiration, is indicative of neuro-
head injury suggests a major and probably fatal logical deterioration.
Head Injuries 165
Boggy
temporal
swelling
Dilating
ipsilateral
Drowsiness
pupil
Tone in
contralateral
limb
Subdural haematomata result from injury to decrease the intracranial pressure and to increase
the brain surface, including the bridging veins oxygenation to the brain. A localized, depressed
which run between the cerebral cortex and the fracture to the skull may cause local brain dam-
dura. They are seen in younger children after age from the bone ends (Fig. 12.17). This is an
high-speed injuries or the violent shaking of child important condition not only because of possible
abuse. There is usually a concomitant severe pri- underlying cortical injury but also because the
mary brain injury so that deterioration is less diagnosis may be overlooked if the depression
obvious. The outcome of a child with a subdural is assumed to be a scalp haematoma with a soft
haematoma is not as good as one with an extra- centre (Fig. 12.18).
dural haematoma.
An intracerebral haematoma (a bleed within Foreign Bodies
the parenchyma of the brain itself) presents as A foreign body penetrating the skull and lodged
slow neurological deterioration 1–2 days after in the brain should be left in situ as it is likely to
a severe head injury. It is diagnosed on CT be acting as a tamponade controlling cerebral
scan but usually is not amenable to surgical bleeding. The object is removed by the neurosur-
intervention. geon in theatre.
Progressive cerebral oedema and hyperae-
mia over the first two days following a severe
head injury also may produce signs of neuro- Lacerations
logical deterioration, particularly if there has
been inadequate attention to the prevention of Lacerations Are Extremely Common
hypoxia, hypercarbia, and fluid and electrolyte in Children
imbalance. A CT scan shows profuse oedema They are sustained during falls or play, when
with compression of the ventricles and subarach- handling dangerous objects (e.g. scissors, broken
noid space, with no space-occupying lesions. glass) or are received during major trauma. The
Treatment involves institution of measures to circumstances, site and extent of a laceration may
Head Injuries 167
Cortex
Fracture springs
back into
normal position
Torn cortex
a
Liquified
centre
Scalp
haematoma
Firm rim of
haematoma
Perineal Injuries
Male
Straddle Injury
A boy who slips while walking along the top of a
fence or climbing out of a bath (Fig. 12.20) is
likely to sustain a straddle injury. The urethra is
at risk as it passes through the urogenital fascia to
lie superficially in the perineum. A straddle injury Fig. 12.20 A common cause of perineal injury – slipping
causes bruising in the perineum behind the scro- while climbing out of bath
tum and oedema or bruising of the scrotum. The
testes tend to ‘ride with the injury’ and are infre-
quently damaged because of their mobility. The management of a urethral injury is dependent on
important question is: “Has the urethra been its site, exact nature and associated bony or uri-
injured?” It may be contused or ruptured. A drop nary tract injuries. Where perineal contusion has
of blood at the urethral meatus strongly suggests occurred without urethral injury, adequate anal-
major urethral injury, and if it occurs in associa- gesia normally permits continued voiding, and no
tion with acute urinary retention and an enlarged further treatment is required.
bladder, complete urethral transection should be
considered (Fig. 12.21). Blood-stained urine Penetrating Spike Injury
indicates urethral injury without complete dis- Boys, in particular, may jump from a tree onto
ruption. When the anterior urethra is torn, urine an exposed branch, or land on a picket fence
leaks into the space deep to Colles’ fascia (the while attempting to hurdle it, and sustain a
membranous layer of the subcutaneous tissue) to penetrating injury of the perineum. Although
cause swelling of the scrotum and penis with any perineal and pelvic structure can be
extension to the ventral abdominal wall. involved, the main danger is penetration of the
The urinary extravasation produces a rectum which may cause severe pelvic perito-
progressive, diffuse boggy swelling which nitis, septicaemia and death if not recognized.
becomes inflamed (from tissue reaction to For this reason, all penetrating perineal inju-
urine), bruised (from blood in addition to urine) ries should be explored carefully, usually under
or frankly infected. Where urethral trauma is general anaesthesia. One finger or a probe is
suspected, referral to hospital enables a retrograde inserted into the wound at the same time as
urethrogram to be obtained so that the continuity another finger is inserted into the rectum. This
of the urethra can be established. The surgical allows the examiner to assess the relationship
170 12 Trauma
Swollen
b penis (except glans)
Lower abdomen
swollen
Attachement of fascia
Swellon
scrotum
sion across the posterior fourchette may Table 12.10 Presenting complaint versus likelihood of
indicate a penetrating vaginal injury. sexual abuse
Symptom/sign Possibility of sexual abuse
The Discharging Vagina Direct report from child
Mucopurulent or offensive blood-stained dis- Pregnancy Very high
charge from the vagina is the sign of an unrecog- Venereal disease
nized foreign body. The exploratory and Genital/rectal trauma
inquisitive nature of the girl may be responsible Precocious sexual interest
Inappropriate sexual High
for the introduction of the foreign material but, as
activity
with all female perineal injuries, molestation Indiscrete masturbation
should be excluded. Treatment involves removal Genital inflammation/
under general anaesthesia. Infections sometimes discharge
cause vaginal discharge, but these are rare, and Behavioural disturbance Moderate
the diagnosis is made from culture swabs after Runaway from home
the existence of a foreign object has been
excluded.
Table 12.11 Differential diagnosis of perineal injury
and vaginal discharge
the examiner. All details, including date, time, diagnosis difficult. Soft tissue injuries to the
place of examination and name of witness, need limbs with nerve or tendon damage may be par-
to be recorded accurately. The impending proce- ticularly difficult to diagnose because the fright-
dures should be explained to the child as the ened, distressed infant is not able to cooperate in
examination progresses. If forensic specimens, the physical examination. Even if no deformity is
such as vaginal swabs and smears, are likely to be present, it is important to remember that a frac-
required, the examiner should wear gloves. ture is more common than a sprain since the liga-
The general appearance and emotional state of ments are much stronger than the bones during
the child is noted, and obvious injuries should be childhood. The appearance of the epiphysis and
photographed. Clothing should be removed only the adjacent cartilaginous growth plate also may
in the presence of the clinician and a witness, with lead to confusion during diagnosis because the
the child standing on a sheet of paper, to avoid loss cartilaginous parts are not visible on x-ray. An
of forensic evidence. The clothing is documented, injury near to or involving the growth plate needs
examined and stored. The face, mouth, throat, referral to someone with significant orthopaedic
head, limbs, thorax, abdomen and buttocks are experience since inadequate treatment of the
examined for bruises, abrasions or bite marks. fracture may lead to later deformity or limb
The ear drums require careful inspection to shortening.
exclude traumatic rupture, which is a common The most important point to remember is that
sequel to slapping the side of the face. If the injury pain along with loss of function in a limb is nearly
suggests recent sexual intercourse, the clothing always caused by a fracture. Minor fractures,
and skin should be examined under UV light such as buckling of the cortex or a greenstick
(Wood’s lamp) since dried semen will fluoresce. fracture, where the cortex is broken only on one
The external genitalia and perineum should be side, are common in childhood and produce little
examined carefully for semen, blood and mucus deformity. This type of fracture follows minor
as well as for obvious signs of trauma such as accidents around the home or school. More seri-
bruising or lacerations. The age of bruises should ous fractures through the long bones may occur
be estimated by their colour. The anus should be with a major fall from the roof of a house or a tree
examined for signs of rectal trauma. The laxity of or are sustained in a motor vehicle accident.
anal sphincters is not a reliable sign of anal inter- Fractures through the epiphysis are important
course in children since the sphincters are often because they may be ‘invisible’ on x-ray, and the
relaxed normally in sleep. prognosis for growth of the limb following treat-
Where a labial laceration is found in a small ment is uncertain. Pathological fractures do occur
girl, examination under general anaesthesia is the but are rare in childhood. Benign bony cysts or
best way to determine the extent and severity of malignant tumours (osteosarcoma), infection
the injury. In adolescents, a speculum examina- (osteomyelitis) or metabolic diseases (osteogen-
tion of the vagina may be quite satisfactory to esis imperfecta) may cause pathological frac-
identify trauma and obtain swabs. tures. Children on steroid therapy for malignant
In cases where sexual abuse appears likely, disease or chronic inflammatory disorders may
expert medical and police help should be obtained develop fractures secondary to cortisone-induced
immediately, so that medical management and osteoporosis.
forensic and legal proceedings can begin. Fracture of the clavicle is the commonest frac-
ture seen in childhood but does not need to be
described at length since its clinical features are
Fractures identical to those seen in adults. Diagnosis can be
made simply by palpation. Reduction usually is
The diagnosis of fractures in older children is not needed in children, and the only treatment
straightforward. However, in small children, lack required is to rest the arm in a sling for 2–3 weeks.
of cooperation and communication may make They virtually always heal without problems.
Fractures 173
Intact
posterior
periosteum
Artery impaled by
bone end
Distal humerus
displaced and
titled backwards
? Radial pulse
present
Ischaemia of
Pain muscle (eg. forearm)
Pallor
Puffiness
Paralysis
Muscle infarction
Annular
ligament
Normal arm
supporting
injured arm Subluxation
radial head
Local
Pronated immobile tenderness over
forearm radial head
Type of Burn
Swollen, painful
thigh
Transverse fracture
of shaft
10 32 10 10 30 10
13 13 19 19
How long was it before the burnt area was Area of Burn
dowsed in cold water?
3. The type of liquid involved. Fats are hotter The infant has a proportionally larger head and
and retain their heat longer than water. smaller lower limbs than the older child or adult
The worst flame burns involve flammable (Fig. 12.31). When estimating the area of a burn,
clothing and are proportional to the temperature therefore, a figure chart should be used and the
of the flame and duration of exposure. Synthetic age of the child taken into account. There is a ten-
materials often melt, producing molten blobs dency to overestimate the area burned, which is
which become adherent to the skin and cause fur- another reason for mapping out the area on a
ther damage. In children, chemical burns are figure chart. This chart should become part of the
received through contact with household or gar- medical record.
dening agents, particularly strong alkalis and
acids. The severity of burn is dependent on their
concentration and the time elapsed before being Depth of Burn
washed off.
Electrical burns damage all the tissues between It is important to know the depth of the burn
the entry and exit points of the current. At low (Fig. 12.32) because:
voltage, the skin – being a poor conductor – is 1. The metabolic upset is greater with deeper burns.
less affected than muscle, nerves and blood ves- 2. Healing occurs more slowly with increasing
sels, belying the gravity of the injury to the deeper depth of burn.
structures. 3. Deep burns (unless small) require skin grafting.
178 12 Trauma
Epidermis Red
Superficial
blistered
Deep Red/Grey
Dermis
White/Black
Full
coagulated fat
thickness
visible painless
Fat
Hair follicle
4. Circumferential deep burns, involving limbs, necrosis and thrombosis of underlying vessels is
digits or the chest wall, may cause vascular seen as dark red puncta, and there may be islets
impairment or restrict ventilation and necessi- of surviving skin. Deep partial thickness burns
tate urgent decompression with escharotomy. vary in colour and may be difficult to distinguish
When assessing the depth of burns in children, it both from more superficial and from deeper
is useful to appreciate that: burns. Blisters are common, but the skin is often
1. Small children have thinner skin, and therefore grey or a darker red than the erythema of epider-
a certain insult will cause greater damage. mal burns. Where doubt persists, re-examination
2. Skin thickness varies from area to area (e.g. after about 1 week clarifies matters.
the skin of the back is thicker than that of the
forearm, and plantar or palmar skin is thicker
than the skin of the eyelids). General Signs in Burns
3. Parts of the body with good blood supply (e.g.
the face) conduct the heat of the burn away more Restlessness
quickly and heal more rapidly after a burn. Superficial burns are extremely painful and may
4. Some areas of the skin may be more deeply cause restlessness, which must be distinguished from
burned than others. hypoxia or restlessness caused by psychological
Burns involving the epidermis alone (first factors following the accident. In major burns,
degree/superficial) are painful and appear as a hypoxia must be suspected and blood gases obtained.
bright erythematous area which has a tendency to
blister and is sensitive to pinprick. Full thickness Shock
burns (third degree/subdermal) imply death of all Thirst, nausea and lethargy are symptoms of
layers of the skin and exposure of the subcutane- hypovolaemic shock which can be assessed
ous layers. The sensitive nerve endings in the clinically by observing general pallor and by
dermis have been destroyed, producing a rela- measurement of the pulse, blood pressure, qual-
tively painless lesion which is insensitive to pin- ity of peripheral perfusion (warmth of the extrem-
prick. When the injury is caused by a scald, the ities and rapidity of capillary return), degree of
surface appears pale or white with a transitional dehydration (moistness of mucous membranes,
zone of more superficially burned skin between it tissue turgor, sunken eyes, collapsed fontanelle)
and unaffected skin. In flame burns, the surface is and urine output. An extensive area of burn
black or brown. As the skin lifts or is burnt off, (greater than 10–15 % surface area), deep burns
subcutaneous fat becomes visible. Coagulative and young age are factors predisposing to hypo-
Burns 179
Associated Injuries
The possibility of other injuries sustained with
the burn must be entertained from knowledge of
the circumstances of the accident and ruled out
by clinical examination.
An inhalational injury is caused by hot gas or
poisonous smoke damaging the airways and lung
parenchyma. Inhalation trauma can be antici-
pated from the history of the injury, by examina-
tion of the nose and mouth for evidence of burns
and by observation for the development of stridor Tongue
and respiratory distress. Early onset of respira- depressed
tory symptoms suggests severe injury to the
respiratory passages.
A history of smoke inhalation, of being trapped
in an enclosed space or of the combustion of syn-
thetic materials or toxic industrial chemicals, Fig. 12.33 Examination of the mouth and oropharynx in
makes inhalation injury likely. Flame burns to the a small child
face and mouth suggest that the inspired gases
were of high temperature. Look at the nostrils for Table 12.12 Causes of respiratory insufficiency in burns
burns to the nasal hairs and at the mucous mem- Site of pathology Cause
brane of the mouth and pharynx for oedema, ery- Upper airway obstruction Coma or shock
thema or soot (Fig. 12.33). A hoarse voice indicates Posture
an inhalational injury with vocal cord oedema. Associated injuries
Hot gases and toxic agents damage the respi- Laryngeal burns
ratory passages, causing oedema and bronchial Lower airway obstruction Bronchospasm
irritation (Table 12.12). The irritated and swollen Soot/smoke
mucosa of the bronchi produces excessive mucus Mucus plugging
and bronchospasm. Obstruction of the airways Parenchymal damage Hot gas
may occur from bronchospasm, soot and mucus Toxic chemical
plugging and be exacerbated by poor posture, Impairment of ventilation Chest wall burns
Pneumonia Secondary infection
coma or aspiration of vomit.
The chest should be examined for evidence of
hyperexpansion and increased respiratory rate or manifest clinically. The signs of hypoxia are late
effort. The use of accessory muscles of respiration and imply severe lack of oxygen. The child
and the amount of indrawing that occurs during appears restless, confused and disoriented or
inspiration should be noted. There may be an audi- shows a change in behaviour. However, the cir-
ble wheeze and auscultation may reveal few breath cumstances of major injury in a child experienc-
sounds, crepitations and rhonchi. A localized area ing pain in an unfamiliar and frightening
of poor air entry may be caused by mucus plugging environment limit the specificity of these param-
and, if unrelieved, becomes an area of atelectasis eters. Increasing respiratory effort also may
with crepitations and dullness on percussion. reflect hypoxia or anxiety, pain or another respi-
In major burns, the presence of hypoxia should ratory complication, but cyanosis indicates that
be detected biochemically before it becomes severe hypoxia has occurred already.
180 12 Trauma
a b
Cricopharyngeus
coin stuck
in trachea
aortic arch
gastro-oesophageal junction
Coins stuck
in oesophagus
Sagittal
Coronal plane
plane in in trachea
oesophagus
Fig. 12.34 Coins stuck in the
oesophagus (a) or trachea (b)
Foreign Bodies 181
Trachea
Retrieve
because
of high
risk of
complications
Right upper
lobe bronchus
Left main
20 c. bronchus
Pass safely
Right main
bronchus
Fig. 12.35 The approach to foreign bodies in the stomach
Fig. 12.36 Inhaled foreign bodies in the trachea. A pea-
nut is the common foreign material, and the right main
the head and neck, thorax and abdomen, as the bronchus is obstructed because it runs almost vertically
foreign body could be located anywhere from the downwards
nasopharynx to the anus. Coins, which are amongst
the commonest radio-opaque items swallowed, Has a Foreign Body Been Inhaled?
usually become arrested in the upper oesophagus Most children who have inhaled a foreign body
(see Fig. 12.34) and can be shown to be in the present early either because of sudden onset of
oesophagus rather than the trachea by observing coughing, spluttering and an audible wheeze or
the orientation of the coin or by obtaining a lateral because the object was observed to have been
x-ray (Fig. 12.34). Coins in the oesophagus are inhaled. In less than one third, the episode of
common and lie in a coronal plane, whereas coins inhalation is not recognized at the time, and the
in the trachea are extremely rare and have a sagit- child presents weeks or months later with a
tal orientation. If the coin is below the diaphragm, wheeze, bronchitis or chronic chest infection.
no further action is required. Foreign bodies The usual foreign body inhaled is a peanut,
lodged in the oesophagus require oesophagoscopy and most children are aged 3 years or under.
and removal under general anaesthesia. Those Cyanosis is rare and reflects almost complete
which reach the stomach, including open safety obstruction of the trachea or the presence of mul-
pins, can be allowed to pass spontaneously unless tiple inhaled particles. The usual symptoms are
they are very long (Fig. 12.35) or likely to cause coughing and a wheeze, although the child may
secondary complications (e.g. button batteries). In appear to gag and vomit. If there is diagnostic
these situations, consideration must be given to delay, the child may develop pyrexia and chest
their endoscopic removal. infections (Fig. 12.36).
In the rare event that a foreign body causes An inhaled foreign body may produce a ‘ball-
perforation of the bowel, there is often no history valve’ effect, whereby dilatation of the bronchus
of foreign body ingestion and the diagnosis is during inspiration allows air in, but no air escapes
made at operation for peritonitis. Retrosternal during expiration. This will cause the lung distal
discomfort and prolonged or progressive dys- to the obstruction to become hyperinflated while
phagia may be caused by impaction of a foreign remaining poorly ventilated. Clinically, this can
body in the oesophagus without complete obstruc- be detected by observing the movement of the
tion. Perforation of the oesophagus produces chest wall with respiration and looking for asym-
signs of mediastinitis, which include malaise, metry. There may be an inspiratory stridor and
fever, pain, and swelling and subcutaneous increased use of the accessory muscles of respi-
emphysema in the neck. Subcutaneous emphy- ration. The child will tend to sit leaning forward,
sema, caused by air spreading along tissue planes, breathing through the mouth, with retraction of
is diagnosed by feeling crepitus and ‘bubbling’ in the supraclavicular and intercostal areas during
the soft tissues. inspiration. The increased respiratory effort
182 12 Trauma
Look for flaccidity of all limbs and 6. Persisting pain after reduction of a
hypotension. fracture suggests muscle ischaemia.
5. Assessment of head injuries involves 7. Loss of capillary circulation suggests
repeated examinations at intervals and muscle ischaemia.
accurate recording of information: only 8. All potential fractures should be
in this way will deterioration be detected x-rayed, including the joints above and
early. below the injury, and in two planes.
6. Unilateral fixed, dilated pupil may rep- 9. Sprains are rare in children: it is usu-
resent local damage to the orbit and the ally a fracture without displacement
nerves of the pupillary reflex arc or may be since bones are weaker than ligaments
the first indicator of an expanding intrac- during childhood.
ranial lesion and increased pressure. 10. Dislocations occur only at the elbow in
7. Scalp laceration may represent a com- childhood.
pound fracture of the skull. 11. All lacerations should be explored for
8. The child who becomes drowsy and vom- evidence of tendon, nerve or vascular
its after an apparently minor head injury injury.
may have intracranial haemorrhage. Burns
Perineal Injuries 1. Severity of burn depends on the tem-
1. Female perineal injury needs sexual perature and duration of exposure to the
abuse excluded. causative agent, the area involved and
2. The straddle injury in a boy may rupture the depth of the burn.
the urethra. 2. Knowledge of the circumstances of a burn
3. A boggy swelling of the perineum, scro- provides useful information for determin-
tum and penis after perineal trauma sug- ing the severity of injury and predicting
gests urine extravasation. likely complications and sequelae.
4. Penetrating perineal injuries should be 3. In assessing burns to the head in the
explored to rule out rectal or lower uri- infant, remember the head accounts for
nary tract injury. one fifth of the surface area.
5. Lacerations extending across the poste- Foreign Bodies
rior fourchette suggest deeper vaginal or 1. Virtually all ingested foreign bodies
intraperitoneal injury. pass spontaneously.
Limb Injuries 2. Radiography for a swallowed object
1. Femoral fractures in infants suggest should include the head and neck, tho-
child abuse. rax and abdomen.
2. Acute loss of function and pain in a 3. Long rigid foreign bodies may get stuck
limb indicates a fracture until proven at the duodenojejunal flexure.
otherwise. 4. Mercury oxide micro-batteries disinte-
3. Pallor/anaemia in femoral fractures is grate in the stomach and release corrosive
more likely from internal haemorrhage substances which damage the bowel wall.
than fracture bleeding. 5. Oesophagoscopy is indicated for all for-
4. Supracondylar fractures carry high risk eign bodies impacted in the oesophagus.
of distal ischaemia and neurological
loss.
5. Loss of finger movement in elbow Further Reading
injuries suggests median, ulnar or
radial nerve damage. Puri P, Höllwarth M (eds) (2009). Pediatric Surgery.
Diagnosis and Management. Springer, Dordrecht.
The Limbs
13
Abnormalities in the limbs affect the legs more still between the thumb and fingers of one hand
frequently than the arms, except for fractures (see while the other hand holds the thigh with the
Chap. 12). There are many problems which are thumb in the groin (anteriorly) and the middle
minor aberrations of shape during growth, both finger on the greater trochanter (posteriorly). The
before birth (e.g. compression deformities) and hip is flexed to 90° and then abducted to 45°. In
during childhood (e.g. ‘bow legs’ and ‘knock this position, forward pressure with the middle
knees’). This chapter discusses the common finger will tend to push the femoral head into the
anomalies (1) as they present at birth and (2) in joint, while backward pressure of the thumb will
early childhood, as well as the important patho- tend to dislocate the head posteriorly. When the
logical conditions (e.g. osteomyelitis) which may femoral head relocates, there is an audible and
occur at any time throughout life. palpable ‘clunk’. The baby should be referred for
ultrasonography to confirm the abnormality and
then for an orthopaedic opinion and application
of a harness (e.g. Pavlik) to hold the femoral head
The Limbs at Birth within the joint until dislocation can no longer
occur.
Abnormalities of the hip joints or the shape of the
feet at birth are common. The attention of the
physician often will be directed to misshapen Foot Deformity
feet, but the more important abnormality to detect
is congenital dislocation of the hip. This is one Most abnormalities are caused by extrinsic com-
area in paediatric surgery where a ‘screening’ pression of the feet within the uterus and resolve
examination is very helpful because the hip spontaneously after birth (Fig. 13.2). These pos-
treated at birth (by splinting) will grow normally, tural deformities can be recognized on examina-
whereas diagnosis of a dislocation after the child tion by a normal range of passive movement. The
has learnt to walk (with a limp) may be too late to deformity may be caused by abnormalities of fetal
preserve function. position or of the uterus (e.g. septum or fibroids)
and will be predisposed to by oligohydramnios,
a small amniotic cavity (especially with chromo-
The Hip Joint somal abnormalities), or paralysis/stiffness of the
legs (e.g. spina bifida, arthrogryposis). Weak legs
The Ortolani test is used to diagnose congenital cannot kick hard enough to allow the feet to main-
dysplasia with dislocation of the hips (Fig. 13.1). tain their normal posture in utero. Consequently,
The baby should be relaxed with the pelvis held all babies with deformed feet must be examined
b c
carefully for (1) major chromosomal defects, (2) contralateral leg. If the foot has full passive move-
multiple joint abnormalities (arthrogryposis) and ment, casts or splints should not be needed.
(3) myelomeningocele. Talipes equinovarus, or ‘club foot’ (Fig. 13.3),
Particular postural deformities, such as calca- needs to be assessed carefully since any limita-
neovalgus, are of themselves trivial but may sig- tion of passive movement indicates that immedi-
nal associated dislocation of the hip since the ate treatment is required. This is because the lax
position of the dislocated leg in utero predisposes joints in the first week of life allow rapid correc-
to this deformity. tion with serial plaster casts or splints. If the foot
Metatarsus adductus (or metatarsus varus) is a cannot be pushed into the calcaneovalgus posi-
relatively minor angulation deformity of the foot tion easily, an orthopaedic surgeon should be
caused by compression of the foot around the consulted.
The Legs in Early Childhood 187
1. 2.
+ −
0−10º
Fig. 13.7 Gowers’ test. The child sits on the floor and is and on the legs (c) to reach the standing position (d).
asked to stand up (a) A positive test is seen when the child A positive Gowers’ sign indicates weak leg muscles (as in
has to lever the trunk up using the hands on the floor (b) muscular dystrophy)
Tiptoe test
The initial infection in the metaphysis causes Table 13.2 The signs and symptoms of osteomyelitis
local oedema with elevation of the adjacent perios-
Symptoms Signs
teum. There is generalized fever and malaise, and
Early:
the child holds the limb still. The elevated perios- Pain Local tenderness
teum produces exquisite local tenderness. As the Fever No spontaneous movement
disease progresses, pus begins to collect under the Limb held still Normal passive movement
periosteum to form a subperiosteal abscess. The Refusal to weight-bear Bone scan positive
inflammatory swelling within the bone com- Late:
presses the blood supply to the bone and may Severe toxicity Swelling
cause local ischaemia. The physical signs of a Fever Tenderness
subperiosteal abscess include swelling, local heat, Immobile limb Fluctuance
redness, fluctuance and very severe pain. If the Overlying erythema
disease remains untreated or misdiagnosed, par- Nearby joint effusion
ticularly in older children, a chronic form of X-rays positive
osteomyelitis may develop. The subperiosteal
abscess eventually ruptures through the perios-
teum and drains through a sinus on the skin. The
area of ischaemic bone becomes necrotic, forming Time
a sequestrum. New bone (the ‘involucrum’) begins before
to form around the cavity and under the elevated diagnosis
periosteum, resulting in obvious bony deformity.
Clinical Picture 1 10
Age (years)
The clinical presentation of osteomyelitis depends Local signs Misdiagnosed ‘sprain’
on the duration of infection (Table 13.2). In the Communication Systemic signs
early phases, the child has pain, a high fever, an
Fig. 13.11 The relationship between delay in the diag-
immobile limb and refuses to weight-bear. On nosis of osteomyelitis or septic arthritis and the age of the
examination, there is reduced spontaneous move- child. Both infants and adolescents may present difficulties
ment with exquisite tenderness and swelling usu- which hamper early diagnosis
ally over a metaphysis. Examination of the
adjacent joints shows a normal range of passive positive at an early stage.) In the later phases of
movement. If x-rays are taken at this stage, they infection, the degree of toxicity and fever may be
usually show no abnormality since the pathologi- increased and the limb is completely immobi-
cal changes that are seen on x-ray take about 10 lized. The physical findings usually demonstrate
days to develop. (By contrast, the bone scan is the presence of a subperiosteal abscess, with
Osteomyelitis 193
marked swelling and tenderness over the affected Examination of a child with possible osteomyelitis
bone and a fluctuant, hot, red mass. The adjacent 1. Child points to tender spot (Femur/tibia in 60%)
joint has an effusion. Once a subperiosteal abscess
has formed, x-ray changes usually are evident. 2. Inspect for swelling, redness, abrasions
Although osteomyelitis and septic arthritis
may occur at any age during childhood, the diag- 3. Feel for local heat − use back of fingers
nosis is often delayed in infants (Fig. 13.11). In − compare with limb
or rest of same limb
addition, the pain in adolescents is often misdiag-
nosed as a sprain or other minor injury. Systemic
4. Percussion of bone localize tenderness
signs may be less obvious than in younger chil-
dren because the infection is localized more effec-
5. Compression of bone - useful in − early infection
tively. In neonates and infants, the natural history − deep-seated bones
of the disease is somewhat different (Table 13.3).
Local signs are less obvious because the perios- 6. Exmaine neighbouring joints − effusion
teum is less tightly fixed to the bone and is more − limits of movement
easily elevated by oedema or pus. When pus is
not under high pressure, it causes less pain. The 7. Exmaine local lymph nodes and spleen
inflammatory response spreads rapidly through
Fig. 13.12 Examination of a child with possible osteo-
the leg to produce diffuse redness and oedema. myelitis. A preliminary step is to observe the gait for a
These inflammatory signs are easily recognized limp, but commonly the child refuses to walk and has to
but commonly are misdiagnosed as cellulitis. be carried by a parent
Temperature control in small infants is less stable,
such that systemic infections may reduce rather site for organisms, such as superficial abrasions.
than elevate the temperature. Radiological signs The presence of increased local heat caused by the
appear at an earlier stage because bony changes inflammatory response is assessed by feeling the
occur more rapidly than in older children. limb with the back of the fingers and comparing the
two limbs to detect even minor elevations in local
temperature. In addition, the fingers should be run
Clinical Examination up and down the limb past the affected area to dem-
onstrate that the whole limb is not equally affected.
When osteomyelitis is suspected, the clinical rou- Palpation of the full length of both limbs will
tine which should be followed is shown in enable any local swelling to be recognized. Local
Fig. 13.12. If the child can walk, the gait should be tenderness often is diagnosed best by percussion,
observed for a limp. The common situation, how- particularly in areas where the bone is superficial.
ever, is refusal to weight-bear or walk, and the par- If bony percussion fails to elicit local tenderness,
ents will carry the child. An older child should be the bone can be squeezed directly by manual com-
asked to point to the tender area. In more than 50 % pression. This may be useful if the infection is early
of cases, this will be localized to the femur or tibia. or if the bone is deep within the limb.
Next, the area should be examined for evidence of Once the area of local tenderness has been
inflammation (swelling, redness, heat) or an entry identified precisely, the neighbouring joints
194 13 The Limbs
should be examined for the presence of an effu- and Streptococcus, produce a clinical picture of
sion and for limitation of movement. This is generalized illness with fever, malaise, rigors and
important because the joint may be affected by collapse, with local signs of synovitis. This causes
direct spread from the metaphysis, particularly pain and local tenderness over the distended joint
the hip and shoulder joints. Alternatively, the capsule, restriction of all joint movements and
diagnosis of osteomyelitis may be incorrect and signs of inflammation, including redness and heat
the origin of the infection is the joint itself. in the overlying skin.
Finally, the lymph nodes draining the area The cardinal signs of septic arthritis are refusal
involved should be examined for local enlargement to move the joint and extreme tenderness along
and tenderness as a guide to the rate and extent of the line of the joint surfaces. In most joints in the
spread of the infection. Splenomegaly should be body, this is determined easily, although the hip
sought since this may accompany a severe sys- may provide some difficulties (Fig. 13.13). Septic
temic infection such as osteomyelitis. Splenomegaly arthritis of the hip is common in neonates follow-
also may be present with systemic disorders, such ing an episode of bacteraemia or septicaemia.
as rheumatoid arthritis or leukaemia. The inflammation spreads rapidly beyond the
synovium and joint capsule in a way analogous to
that seen in neonatal osteomyelitis, which almost
Septic Arthritis always is present as well, and can cause the hip to
sublux or dislocate. Marked inflammation in the
Suppurative infection of the joint may occur from adjacent soft tissues produces a swollen thigh,
blood-borne spread, a skin wound or from adja- and the distension of the joint capsule pushes
cent osteomyelitis (Table 13.4). The three com- the femur into a position of flexion, abduction
mon organisms, Staphylococcus, Haemophilus and external rotation. This is the so-called frog
Localising tenderness
-Below inguinal ligament
-Lateral to femoral artery
-Close to skin crease
Limp 195
Osteomyelitis/Septic arthritis/trauma
0 5 10 15
Age (years)
position and is quite characteristic. The diagnosis will produce a limp, but the aetiology should be
of septic arthritis can be confirmed by pain with determined readily by neurological examination.
movement (and decreased mobility because of The two causes of a chronic intermittent limp aris-
pain) and the demonstration of tenderness over ing later in childhood are Perthes’ disease and
the anterior surface of the femoral head. This is slipped epiphysis of the femoral head.
identified below the inguinal ligament, lateral to There are five different forms of limp (spastic,
the femoral arterial pulse and adjacent to the fem- antalgic, Trendelenburg, short leg and ataxic),
oral skin crease which, in the infant, corresponds which can be distinguished by observation of the
to the level of attachment of Scarpa’s fascia. As gait (Table 13.6). The gait of a child with spastic
in other forms of arthritis, the diagnosis is proven hemiparesis or cerebral palsy is characterized by
by aspiration and culture of the joint fluid.
Table 13.5 Causes of a chronic limp
Congenital dislocated hip
Limp Spastic hemiparesis
Congenital short leg
Osteomyelitis, septic arthritis and trauma occur Perthes’ disease
throughout childhood, but there are some dis- Slipped epiphysis of the femoral head
eases which have a predilection for specific age Transient synovitis (irritable hip)
groups (Fig. 13.14). The two common conditions
affecting the head of the femur, i.e. Perthes’ dis-
Table 13.6 Types of limp
ease and slipped upper femoral epiphysis, affect
boys more commonly than girls (4 to 1), with 1. Spastic hemiparesis (cerebral palsy)
Perthes’ disease being the common abnormality Arm held flexed at the elbow
Walks on toes
in boys aged less than 10 years, while slipped
2. Painful weight-bearing (antalgic)
epiphysis affects boys older than 10 years.
Weight transferred more quickly to the normal leg,
A chronic limp may be caused by a congenital producing hopping
abnormality of the leg or by diseases with an 3. Unstable hip
insidious onset (Table 13.5). Important causes of a Pelvis tilts down when weight is on the abnormal leg
limp from the commencement of walking include (positive Trendelenburg sign)
congenital dislocation of the hip or a short leg. If 4. Short leg
undiagnosed before the onset of walking, a dislo- Pelvis tilted down when standing still
cated hip produces an unstable hip with a positive Opposite knee kept bent during walking
Trendelenburg sign (see below). Neuromuscular 5. Ataxia
diseases such as cerebral palsy, muscular dystro- Poor balance and coordination
phy or various forms of spastic hemiparesis also Worse during turning
196 13 The Limbs
* Limp
Age 5−10 years * Limitation
of all movements
Dense +
compressed
femoral head
Paresis
of arm
Walking on
toes
Irritable Hip
Slipped
epiphysis
A–P x-ray
Lateral x-ray
collapses and acquires a mushroom shape, with genitalia. The epiphysis usually slips off the fem-
the epiphysis appearing smaller and irregularly oral neck downwards and posteriorly and causes
dense on x-ray. Most children affected are boys the leg to be pushed into external rotation. The
between 3 and 10 years of age. mechanical disturbance of the hip joint produces
The presenting features of Perthes’ disease a secondary synovitis. In most cases, the onset is
include an intermittent limp, which may be pain- insidious with gradual progression of pain and
less or sometimes painful, without evidence of limp involving one or both hip joints, although
systemic toxicity. If the disease is longstanding, there is a history of injury in many. In some, the
the gluteal muscles on the affected side are wasted epiphysis slips suddenly and produces more dra-
slightly and produce a flat buttock. In addition, matic symptomatology and clinical signs which
there may be a positive Trendelenburg test caused suggest a sprain or fractured neck of the femur.
by the effective shortening of the neck of the The cardinal physical sign is the demonstration
femur from collapse of the femoral head. The of some degree of fixed external rotation with
greater trochanter may be more prominent than definite, painful limitation of internal rotation
on the normal side. There is limitation of the hip (Fig. 13.18). Radiological signs may be subtle,
joint movements, particularly abduction of the with the earliest abnormality being irregularity
flexed leg and medial rotation. and widening of the epiphyseal plate. Biplanar
In younger children, where only part of the x-rays are essential since the abnormalities are
femoral head is involved, the prognosis is good often shown clearly only in the lateral view.
and the disease is self-limiting after 1–2 years.
However, in older children and those in whom
the whole femoral head is involved, permanent Osgood-Schlatter Disease
deformity of the hip joint may ensue.
This is another form of osteochondritis juvenilis
and affects the tibial tuberosity of boys and girls
Slipped Upper Femoral Epiphysis aged 8–14 years (Fig. 13.19). The child presents
with a tender lump on the tuberosity, usually fol-
This is a fairly common cause of pain and limp in lowing vigorous activity. It hurts most on kneel-
the hip joint during adolescence (Fig. 13.17). ing and on active extension against resistance.
Most children affected are boys of above average The tibial tuberosity appears fragmented on
height and weight, with relatively small external x-rays, but no other physical signs are present.
198 13 The Limbs
Limited medial
rotation
Limited abduction
Age 10−15 years Table 13.7 Physical examination of a child with a limp
boy
Standing
Local pain +
1. Observe walking to determine the type of limp
tenderness
2. Observe hopping on one leg to exclude trivial lesions
3. Examine the level of the pelvis to exclude a short leg
4. Carry out the Trendelenburg test to identify an
unstable hip
Pain with extension 5. Examine the buttocks and thighs for wasting (hip
against resistance diseases)
Lying on the couch
Partial avulsion
6. Examine the area of abnormality/pain
of tibial tubercle
7. Measure leg lengths (short leg, dislocated hip)
Fig. 13.19 The features of Osgood-Schlatter disease 8. Carry out Thomas’ test (hip disease)
9. Check joints for:
Range of movement
Clinical Examination of Limp Tenderness
Masses
The procedure to be followed in the physical 10. Check bones for tenderness/masses
examination of a child with a limp is shown in 11. Examine soles of the feet for foreign bodies, plantar warts
Table 13.7. The examination may be difficult 12. Examine shoes for:
because of the young child’s inability to localize Type/degree of wear
pain causing the limp. It should commence with Protruding nails
the child walking to observe the type of limp, as
often this will allow the diagnosis to be made will not interfere with hopping. This is a simple
immediately. Next, the child should be asked to test of leg strength and coordination which can be
hop on one leg because trivial causes of a limp performed in children older than 3 or 4 years.
Limp 199
Correct tillt
with block
under foot
Thirdly, the level of the pelvis should be examined abnormal leg, the pelvis tilts downwards under
to determine the position of the iliac crests, either the body weight because the hip abductor mus-
from the anterior or from the posterior aspect, to cles are ineffective. The Trendelenburg test is
exclude a short leg (Fig. 13.20). Pelvic tilt can be positive in abnormalities which cause paralysis
corrected by insertion of blocks of different thick- or weakness of the gluteus medius or minimus
ness under the foot of the short leg. This provides but is observed most frequently in patients with a
an accurate measure of the degree of shortening. dislocated hip or Perthes’ disease. After perform-
An unstable hip can be identified by perform- ing the Trendelenburg test, the buttocks are
ing the Trendelenburg test (Fig. 13.21). The examined in the standing position for signs of
examiner stands behind the child to determine muscle wasting, which may indicate a subacute
the tilt of the pelvis. When the child stands on the or chronic hip disorder.
200 13 The Limbs
a b
Anterior superior
Apparent short iliac spine
thigh length
Avoid patella
Asymmetrical
thigh folds
Leg flexed by
examiner
-Transilluminates
-NOT emptied by
compression
Ant. Post.
lat.
Med
Lax cyst
Fig. 13.25 The features of an easily felt
Tense, medial cyst when knee
enlarged semimembranosus
visible posteriorly flexed
bursa
There is marked variation in the presentation and is one serious cause of sepsis in the infant, and
clinical features of infection of the urinary tract. thus it shares with other infections the general
Neonates and infants usually present with gen- features of fever, lethargy or irritability; refusal to
eral symptoms not specific to the urinary tract, feed; malaise; and vomiting. These are general
whereas in the older child, although general fea- signs of sepsis, and, as in any search for infec-
tures may be present, there are clinical features tion, the urinary tract should be excluded by
suggestive of a urinary tract problem (Table 14.1). looking for white cells or bacteria in the urine.
This means that urinary tract infection can pres- This can be achieved by obtaining a bag speci-
ent in a number of ways that are described men or suprapubic aspirate of urine.
below.
Posterior
iliac
crest
Bulging
of renal
angle
Lower
ribs
Fig. 14.2 Assessment of fullness of the renal angle: view from above looking down the patient’s back
Fullness of
renal angle
Scoliosis
Bladder
this reason, is used infrequently. A bag collection produces a pure growth of >105 organisms/ml,
of urine is the least reliable technique because of infection is considered to be present. Where a
contamination from the perineum and is only mixed growth of organisms is obtained, the
significant if the specimen produces no growth. significance becomes less certain, and collection
Where culture of a midstream urine specimen of a second specimen is indicated.
Collecting a Specimen of Urine 207
Suprapubic Needle Aspiration of puncture to make the skin taut. The needle
is introduced with a quick controlled movement
Suprapubic needle aspiration is the most reliable enabling the resistance as it pierces the skin and
method of obtaining urine in an infant. It relies then the bladder to be felt. Once the bladder
on the intra-abdominal position of the infant has been entered, 1–2 ml of urine is aspirated.
bladder attached to the umbilicus and on the The needle is then withdrawn quickly from the
small size and capacity of the pelvis (Fig. 14.5). skin, and the syringe sent directly to the labo-
The technique is simple but attention to detail is ratory or the urine transferred to a sterile urine
important (Fig. 14.6). When full, the bladder can container for immediate transfer to the labora-
be percussed in the lower abdomen or readily pal- tory. Delay between collection of the specimen
pated in the relaxed infant and may extend as far and inspection by the microbiologist should be
as the umbilicus. Local anaesthetic cream (e.g. avoided to prevent overgrowth of contaminating
‘EMLA’) is applied to the suprapubic area and organisms.
covered with a plastic dressing (e.g. ‘Tegaderm’)
for half an hour. The child is given extra fluids
during this period to fill the bladder. The cream Bag Specimen of Urine
is then removed and the lower abdomen and
external genitalia are thoroughly swabbed with The sterile collection bag is attached to the
antiseptic solution. A 23-gauge needle attached perineum by an adherent watertight rim after the
to a 2-ml syringe is introduced perpendicular to skin has been cleaned with a mild antiseptic. As
the skin in the midline just above the pubis. It soon as the infant or child has passed urine, the
is often helpful to place the index and middle bag is removed and the contents immediately
fingers of the left hand on either side of the point placed in the refrigerator or sent to the laboratory
208 14 Urinary Tract Symptoms
Urethral
opening
Vagina
for culture. Contamination of the urine by perineal voiding effectively, and it may be difficult for
organisms may make interpretation difficult them to pass an adequate volume on demand.
unless culture shows no bacterial growth or a
mixed growth of <103 organisms/ml. This indi-
cates no urinary tract infection. A colony count Catheter Specimen
between 103 and 105 organisms/ml may indicate
urinary infection or merely represent contamina- A catheter specimen of urine is more reliable
tion. Hence, a bag specimen of urine is best used than a midstream or bag specimen because it
to exclude an infection and should not be used eliminates preputial and perineal contamina-
where infection is suspected strongly. tion. It suffers from being an invasive tech-
nique, however, with the potential to damage
the urethra or introduce organisms into the
Midstream Urine bladder. In the normal child, it is an uncomfort-
able and distressing procedure. Its place is
This technique can be used in children of largely limited to spina bifida children and to
4 years or greater and is appropriate for the children who have a catheter inserted for
routine collection of urine. A midstream urine another reason.
specimen is obtained because the few bacteria
which colonize the urethra are washed out at
the commencement of micturition. This method What Is the Significance of Proven
of collection is best in the circumcised male. Infection?
To minimize contamination in the uncircum-
cised male, the prepuce should be retracted to In boys, urinary tract infection should be taken as
expose the glans, and in the female, the labia an indicator of a possible underlying congenital
should be parted (Fig. 14.7). Midstream urine abnormality, for example, pelviureteric junction
collection is not applicable to younger children obstruction, vesicoureteric reflux, posterior urethral
because they are unable to initiate and control valves, ureterocele or diverticulum. Girls have a
Wetting 209
100
Systolic
Blood
pressure
(mmHg)
50 Diastolic
Premature term 2 4 8 16
Age (years)
4.5 3 7 10 13
Table 14.2 Causes of hypertension minutes. The dribbling starts at birth and occurs
1. Renal disease (commonest) every day without exception. Urine in the bladder
(i) Transient: at any time can be expressed by increasing the
Acute post-streptococcal glomerulonephritis intra-abdominal pressure. Thus straining, cough-
Haemolytic uraemic syndrome ing or exertion by the baby, or the deliberate
Henoch-Schönlein purpura application of pressure over the suprapubic area,
(ii) Persistent: will result in expression of urine from the blad-
Chronic glomerulonephritis der. On the other hand, when the infant is relaxed
Chronic pyelonephritis or asleep, the rate of dribbling may decrease.
Vesicoureteric reflux
Urinary tract obstruction
Ectopic Ureter
Collagen disease
The ureter normally enters the bladder proximal
(iii) Wilms’ tumour
to the bladder neck. In some girls, an ectopic
(iv) Renal trauma
(v) Renal artery stenosis
ureter may open into the urethra (or rarely, the
(vi) Renal artery aneurysm, arteriovenous fistula
vagina) distal to the sphincter. Urine emanating
(vii) Renal transplant from the ectopic ureter, which is often part of a
2. Coarctation of the aorta duplex system, is not subject to normal sphinc-
3. Endocrine disease teric control and produces dribbling, whereas the
(i) Phaeochromocytoma normal ureters drain into the bladder and are
(ii) Neuroblastoma subject to normal sphincteric control with a nor-
(iii) Cushing’s syndrome mal pattern of micturition. The function of the
4. Central nervous system disease subservient renal moiety is often poor, and only
(i) Brain tumour small volumes of urine arise from the ectopic
(ii) Abscess ureter. The characteristic pattern in these girls is
(iii) Haemorrhage of normal micturition occurring independently
(iv) Encephalitis of constant dribbling or dripping. The longer
male urethra precludes this presentation in boys.
Table 14.3 Causes of wetting
Phimosis
Cause Main points In a boy who has phimosis, there may be a history
Psychological Acute onset, previously of ballooning of the foreskin during micturition,
continent, emotional upset,
continent during sleep after which there is slight dribbling of urine for
Infection Acute onset +/− general 1–2 min. The stenotic foreskin obstructs urine flow
signs of infection leading to accumulation of urine between the fore-
Neurological Continuous wetting since skin and glans, which slowly leaks through the
birth narrowed opening after micturition is completed.
Ectopic ureter Continuous dribbling since The diagnosis is obvious on examination.
birth in a girl with a normal
micturition pattern
Urethral obstruction Poor flow of urine Urethral Obstruction
(including phimosis) Lesions which obstruct the urethra (e.g. posterior
Bladder exstrophy Continuous wetting since urethral valve, bladder diverticula and ureteroce-
(including epispadias) birth les) cause an inadequate stream with poor flow,
Sphincter damage History of perineal trauma after which continued leakage of urine may occur.
Urine retained in the dilated prostatic urethra
continues to drain after micturition. Once the
from the ureters passes into the urethra in an entire urethra is empty, no further dribbling
uncontrolled manner. This may be apparent clini- occurs until the next void. In many of these
cally as dribbling which occurs every few patients, infection is superimposed.
212 14 Urinary Tract Symptoms
Table 14.4 Causes of haematuria Fever, malaise, anorexia and vomiting indicate
1. Penile infection of the urinary tract. The older child may
Meatal ulcer describe urinary frequency with dysuria and
Trauma abdominal pain. The pain may be located in the
Trauma to the foreskin suprapubic region, loin or back depending on the
2. Infection site and extent of inflammation.
Urinary tract infection A history of recent weight loss and lethargy
Cystitis with vague abdominal discomfort points to a
Pyelonephritis (often secondary to obstruction or renal malignancy.
stasis)
A history of a sore throat or impetigo preced-
3. Tumour
ing the onset of haematuria by 2 or 3 weeks,
Wilms’ tumour
4. Obstruction and stasis
puffiness of the face, generalized oedema,
Pelviureteric obstruction anorexia and malaise in a child aged 5–12 years
Urinary calculi strongly suggests acute post-streptococcal glom-
Vesicoureteric obstruction erulonephritis. There is often oliguria and either
Vesicoureteric reflux obvious haematuria or dark brown urine.
Ureterocele Haemolytic uraemic syndrome should be con-
Polyp, diverticulum sidered in the sick child under 3 years of age who
5. Trauma has diarrhoea (sometimes bloody) or bruising of
6. ‘Medical’ disease the limbs, who develops low urine output with
IgA nephropathy blood.
Glomerulonephritis Henoch-Schönlein purpura involving the kid-
Bleeding diathesis neys results in haematuria. Interrogation will
Henoch-Schönlein purpura reveal migratory joint pain commonly involving
Hereditary haematuria
the wrists, elbows, ankles and knees, abdominal
7. Misleading ‘haematuria’
pain and a rash over the buttocks and legs.
Vaginal bleeding
When trauma is responsible for haematuria,
Ingested dye and pigments
the history is usually obvious. In child abuse,
however, there may be no story of physical
Clues from the History injury.
A history of excessive bruising or prolonged
Evenly blood-stained urine throughout micturi- bleeding after minor trauma provides a clue to
tion suggests the problem resides at, or proximal the existence of a bleeding disorder. In the major-
to, the bladder. Spots of blood passed at the end of ity of patients, the nature of the bleeding diathesis
micturition indicate an abnormality of the lower will have been determined already.
urinary tract, and a meatal ulcer or preputial
pathology is likely. Recent circumcision points to
a meatal ulcer and may be associated with spray- Clues from the Examination
ing of the stream or a thin stream and pain at the
tip of the penis on micturition (see Chap. 5). In circumcised boys with a history of painful
The pain is sharp and occurs at the commence- voiding, inspect the meatus for a meatal ulcer.
ment of micturition, leading to the passage of urine The external urethral meatus appears small with a
in short painful spurts. Characteristically, the child tiny scab at its entrance. After micturition, a small
screams when he attempting to pass urine. In an drop of blood appears at the site of the ulcer.
uncircumcised boy, attempts at forced retraction of A number of systemic, non-infective causes of
the foreskin in the presence of phimosis or con- haematuria can be identified on clinical grounds. In
genital adhesions of the undersurface of the pre- acute post-streptococcal glomerulonephritis, there
puce to the glans may cause spots of bleeding. is generalized oedema most usually recognized as
214 14 Urinary Tract Symptoms
Golden Rules
1. Suspected urinary infection in all unwell
children where there is no obvious cause.
Abdominal Masses
15
There are numerous organs in the peritoneal mass is a key feature in the diagnosis, since
cavity and retroperitoneal space that can produce mobile masses must be within the peritoneal cav-
an abnormal mass. Neoplastic or hamartomatous ity itself, rather than in the retroperitoneal tissue.
enlargements of the organs may be solid or cys- In addition, mobile lumps usually are benign.
tic, while mechanical obstruction to the hollow The last question to be answered is: ‘What sec-
tubes in the abdominal cavity produces cystic ondary effects does the mass cause?’ The blood
dilatation. pressure may need to be measured, particularly if
a Wilms’ tumour, neuroblastoma or renal disease
is suspected. A varicocele may be present if the
left renal vein is obstructed. Metastases may be
Questions to Be Answered present in distant lymph nodes or bones.
Secondary effects, such as bowel obstruction,
A mass may be found within the abdomen by vena caval-obstruction or ascites, may be present
accident or by design. In either event, the physi- within the abdomen itself.
cian should adopt the same approach to diagnosis
as would be followed elsewhere in the body 1. Age of child? Neoplasms common in 0−5 years
(Fig. 15.1). First, the age of the child should be group
considered, since nearly all diseases in children 2. Organ of origin? Knowledge of organ allows
occur at specific ages. In particular, malignant elimination of numerous
tumours of childhood which present commonly other possibilities
as incidental abdominal masses are much more 3. What are its features?
common in preschool children. -Mobile? intraperitoneal+/or benign
-Fixed? retroperitoneal+/or malignant
The second question to be answered is: ‘From -Cystic/solid?
which organ does the mass arise?’ Knowledge of -Smooth/nodular?
the organ of origin will eliminate numerous pos- -Signs of inflammation?
sibilities from any list of differential diagnoses.
The third question is: ‘What are the features of 4. Are 2˚effects present?
the mass?’ - B.P. ? renal/adrenal tumour
-Varicocele? left testicular vein blocked
This is answered by careful examination to -Metastases? look at nodes and bones
elicit whether it is mobile or fixed, cystic or solid -Bowel obstruction?
and smooth or nodular or whether there are signs -Vena caval obstruction? look at leg veins
-Ascites?
of inflammation. These features assist in the
determination of which pathological process has Fig. 15.1 The systematic approach to adopt when an
produced the mass. The mobility of an abdominal abdominal mass is found
is more medial than the usual position of the right should be ballottable (Fig. 15.8). Bimanual pal-
colon because of telescoping of the mesentery (see pation with the lower hand pushing the kidney
Chap. 8 for details). forwards from behind should allow the hand at
Urinary obstruction produces different types the front of the abdomen to feel the mass. A mass
of cystic dilatation of the kidney according to the anterior to the plane of the kidney cannot be
time of obstruction in relation to renal develop- pushed forward by a hand in the loin. Finally, a
ment (Fig. 15.7). In ureteric atresia, early fetal large cystic kidney in an infant with little body fat
obstruction prevents normal nephron develop- can be transilluminated provided that the room is
ment and results in dysplastic cysts. This leads to dark and the torch is bright (Fig. 15.9).
a multicystic kidney in the neonate. The cysts are
of different sizes, and the kidney feels like an
irregular bunch of grapes. In late fetal or postna- Tumour Presenting as an Abdominal
tal obstruction, the nephrons have had time to Mass
develop normally before obstruction occurs, and
the more usual hydronephrotic kidney is pro- The embryonal tumours – neuroblastoma, Wilms’
duced. If the obstruction is in the distal urinary tumour and hepatoblastoma (Fig. 15.10) – are
tract, a dilated ureter also may be palpable. relatively common in the first five years of life.
Hydronephrosis produces a smooth cystic Leukaemias and lymphomas are the common
enlargement of the kidney which retains its shape. neoplasms which present as an abdominal mass
Gerota’s fascia confines the kidney so that it does later in childhood. Hepatosplenomegaly, with
not cross the midline, and it may be felt to move enlargement of lymph nodes in other parts of the
downwards on inspiration. A loin or flank mass body, in association with fever, weight loss or
218 15 Abdominal Masses
Urachal abscess
Very rare
b
Mass
within
abdominal
wall
Fig. 15.4 The bladder as an ‘abdominal mass’. (a) Full abscess is very rare: It is tender on palpation but remains
bladder is very common: It is tender on palpation and dis- after voiding. Careful examination reveals its location
appears on voiding or aspiration of urine. (b) A urachal within, rather than behind, the abdominal wall
night sweats, should arouse suspicion of an without a significant capsule, its surface is irregu-
underlying haematological neoplasm. lar and nodular to palpation. When it arises from
Neuroblastoma is an embryonal neoplasm of the adrenal or adjacent sympathetic chain, it dis-
the neural crest cells which arises most com- places the kidney downwards and spreads rapidly
monly from the adrenal medulla but is also found to the para-aortic nodes, the liver, the cervical
anywhere along the sympathetic chain. The pro- nodes and, occasionally, to more distant sites
tuberant upper abdomen of the infant may con- such as the bones and skin. Neuroblastoma com-
ceal the tumour until it is quite large (Fig. 15.11). mencing in utero may spread to the skin before
There is significant malaise, perhaps related to birth, producing multiple bluish skin nodules
metabolic effects of the tumour, with fever, which have been described as ‘blueberry muffins’.
weight loss and anorexia. The tumour is non- Clinical characteristics caused by the invasive-
cystic and feels stony hard, and, since it grows ness of the tumour are its extension across the
Tumour Presenting as an Abdominal Mass 219
Mass with
inflammatory
signs
Fixed to
surrounding
structures
± Bowel
obstruction
Hidden
by rectus
muscles
Tender
intussusception
mass
midline and its immobility. Examination is not tumour may produce noradrenaline or adrenaline
complete without a search for distant metastases and may directly compress the renal arteries.
and measurement of the blood pressure, since the The physical signs and symptoms of a Wilms’
tumour often allow it to be distinguished from a
a b neuroblastoma. Commonly, the loin mass is an
Early ureteric atresia incidental finding because the tumour has grown
Late ureteric block
dysplastic
to a large size before producing symptoms. The
hydronephrosis
nephrons mass is non-cystic and firm and has a smooth sur-
face because it expands without infiltration, cre-
multicystic
± dilated
ating a pseudocapsule (Fig. 15.12). Except in
kidney
tortuous advanced disease, the tumour remains confined
ureter by Gerota’s fascia, moves downwards on inspira-
tion and does not cross the midline. An attempt
Lumpy
Smooth
should be made to ballot the tumour since this is
good confirmatory evidence that it arises from
the kidney. Unlike the child with neuroblastoma,
Fig. 15.7 The clinical features of a renal cyst caused by
obstruction depend on the stage of renal development. (a) a child with Wilms’ tumour appears reasonably
Early obstruction (ureteric atresia) causes a multicystic kidney. well. The urine is examined for evidence of hae-
(b) Late obstruction produces hydronephrosis. Both lesions maturia, even if this has not been noted by the
are cystic and soft and may transilluminate. In addition, both
parents. The scrotum is examined with the boy
are ballottable and move downwards on inspiration
Anterior
mass cannot
be pushed
from behind
Renal mass
can be pushed
from behind
Sensing
hand
Pushing hand
Fig. 15.8 Ballotting a renal mass: the posterior hand pushes the kidney forward to enable it to be felt by the anterior hand
Uncommon Abnormal Masses 221
standing, to look for a varicocele. The testicular of the lower poles of the kidneys prevents ascent
vein, particularly on the left side, may be during embryogenesis because the isthmus of the
obstructed by the enlarging renal tumour: Wilms’ horseshoe kidney is arrested by the inferior mes-
tumour may spread by direct growth into the enteric artery. The kidney remains anterior to the
renal vein and obstruct the left testicular vein. lumbosacral promontory and is palpable behind
The veins of the pampiniform plexus remain full, the rectus abdominis muscles at, or below, the
even with the child lying down, a feature which umbilicus. The pelvic or dysplastic pancake-like
distinguishes it from the usual type of varicocele. kidney has failed to ascend to the normal renal
Hypertension is a common sequel of this tumour position and usually is found overlying the iliac
because of renal artery compression and produc- vessels. It may be confused with an abdominal
tion of renin. tumour or an appendix mass when it is present on
the right side.
Both masses are quite superficial in the abdo-
Uncommon Abnormal Masses men because of the proximity of the sacral prom-
ontory to the anterior abdominal wall, causing
Two relatively uncommon renal masses which them to be detected on incidental palpation.
may confound the examiner are the horseshoe Diagnosis is confirmed by ultrasonography or
kidney and the pelvic kidney (Fig. 15.13). Fusion other imaging studies.
222 15 Abdominal Masses
Wilms’ tumour
Frequency
Hepatoblastoma
5 10 15
Age (years)
Cervical
Hepatomegaly
(? 2
Rare causes of pathological masses near The transitional epithelium which lines the ura-
the bladder include cysts or abscesses of the chus is an effective barrier to the spread of infec-
urachus – the embryological connection between tion, such that urachal abscess presents as a
the bladder and the allantois. In some verte- localized chronic disease. There may be minor
brates, including man, the allantois is a vestigial systemic symptoms of infection in association
structure in the umbilical cord, and the urachus with a mass and tenderness in the lower abdo-
is of little practical significance. Where some men. Characteristically, the mass is within the
part of the urachus remains patent, it produces abdominal wall rather than within the abdominal
an isolated cyst, sinus or abscess (see Fig. 15.4b). cavity. The tenderness is superficial and may be
Uncommon Abnormal Masses 223
±
BP
- Smooth
- Firm
- Ballottable
- Moves down on inspiration
- One side only
±Haematuria ±Varicocele
Inferior
mesenteric
artery
a
Horseshoe
kidney
b
Discoid
pelvic
kidney
Mobile
in centre
exquisite, without the child appearing unwell. (Fig. 15.15). The most important criterion is
Radiological examination may reveal ectopic mobility of the cyst, which will distinguish intra-
calcification in the suprapubic region, caused by peritoneal from retroperitoneal masses. Cystic
chronic inflammatory change and calcium depo- lesions in the retroperitoneal plane arise from
sition within the cavity. obstructed or dysplastic kidneys. Less common
Ultrasonography demonstrates the cyst wall. masses, which are usually solid, include a swol-
len kidney from renal vein thrombosis or an adre-
nal haemorrhage. Current high standards of
Neonatal Abdominal Masses obstetric care result in these conditions now being
seen rarely. Benign or malignant renal tumours,
There are a large number of masses which may along with neuroblastoma, can occur in the neo-
be palpable at, or shortly after, birth – but fortu- nate, but their clinical presentation is similar to
nately most of these are rare (Tables 15.1 and that described already in this chapter.
15.2). The three common areas in which masses Intraperitoneal masses can be diagnosed by the
are found are shown in Fig. 15.14. In the middle associated physical signs, including partial or
of the abdomen, there may be mesenteric, dupli- complete bowel obstruction, jaundice and, occa-
cation or ovarian cysts, all of which have consid- sionally, by the characteristics of the cyst itself,
erable mobility. In the flank, there may be cystic that is, whether it is unilocular or multilocular.
enlargement of the kidneys or ureters which are Intestinal duplication causes cystic dilatation
fixed by the retroperitoneum. In the right upper within the peritoneal cavity. This is a rare abnor-
quadrant, there may be choledochal or hepatic mality of gut development in which two intesti-
cysts immobilized by the attachments of the liver nal tubes have been formed. Many share a
and the porta hepatis. In addition, abdominal common wall where they are adjacent. The more
masses may be caused by neonatal tumours or common sites for duplication are the duodenum
bowel obstruction (Chap. 21). The neonatal pel- and ileum. The duplication may be tubular or a
vis is so narrow and shallow that any large pelvic simple cyst (Fig. 15.16). Tubular duplications
mass tends to be displaced into the abdominal may open into the normal bowel and hence do not
cavity. cause obstruction or even a palpable mass. On
An algorithm can be used to approach the occasions, they present with bleeding secondary
assessment of an abdominal cyst in the neonate to ulceration from acid produced by ectopic
Table 15.1 Clinical characteristics of abdominal masses
Mass Symptoms Location Mobility Transillumination Characteristics
Hydronephrosis ± Abdominal pain Flank Little Yes Smooth
Neonatal Abdominal Masses
Mobile?
Yes No
Intraperitoneal Retro/extraperitoneal
Single/tubular
± communication with gut
Mesenteric cyst
± bleeding
(Iymphangioma of
mesentery)
Fig. 15.15 Assessment of
an abdominal cyst in the Duplication
neonate cyst
Pelvic Lesions 227
Proximal
bowel
obstruction
Cystic duplication
gastric mucosa within them. Cystic duplications immobile on account of its location in the porta
do not communicate with the bowel but enlarge hepatis. The diagnosis can be confirmed on
in size with accumulation of secretions. The cyst abdominal ultrasound scan, endoscopic retro-
is in the mesentery and is very mobile. As the grade cholangiopancreatography (ERCP) or other
cyst enlarges, the normal bowel is stretched imaging studies.
across its surface and obstructed.
‘Mesenteric’ cysts are usually lymphatic mal-
formations arising in the mesenteric lymphatics Pelvic Lesions
(Fig. 15.17). As with the cystic hygroma in the
neck, they are lax, fluid-filled cysts which may be Palpable masses in the pelvis are common,
so ill-defined that the fluid collection mimics although many may be felt only on rectal exami-
ascites. Occasionally, the ‘lymphangioma’ is nation. Benign lesions are particularly common,
pedunculated, containing cysts of varying diam- as shown in Fig. 15.19. Excluding a full bladder
eters, but still arises from the mesentery. It may and palpable faeces, the most frequent mass
undergo torsion and produce acute symptoms. found in the pelvis is an appendix abscess, which
The aetiology of choledochal cysts, or dilata- has obvious signs of inflammation, including
tion of the bile ducts, is unknown. One explana- pain, heat and tenderness. A clinical diagnosis
tion is that the choledochal cyst forms as a following rectal examination is straightforward
‘blow-out’ of the bile duct secondary to (see Chap. 3). Cystic lesions in the pelvis may be
inflammation caused by reflux of pancreatic more difficult to diagnose. Rare but significant
enzymes in a child with an anomalous biliary- cystic lesions include the anterior sacral menin-
pancreatic duct system. Partial destruction of the gocele and the presacral teratoma. These lesions
duct wall produces cystic dilatation. Choledochal are characterized by their palpation on the poste-
cysts may be caused by the same pathological rior wall of the rectum, in front of the bony
process which leads to biliary atresia. They pres- sacrum or coccyx.
ent as an incidental right upper quadrant mass or This is in contrast to most other pelvic masses
are associated with intermittent pain and jaundice which, on rectal examination, are palpable anteri-
(Fig. 15.18). The firm and spherical mass is orly or laterally.
228 15 Abdominal Masses
Pedunculated
lymphangioma
Stalk
‘Blow-out’
pseudocyst
of biliary tree
Spherical -Fixed in R.U.Q.
firm cyst
Choledochal cyst
intermittent
Sclerosis
Fig. 15.18 The features of Pain of distal
a choledochal cyst, which Jaundice bile duct
can present at any time in R.U.Q.mass
childhood
Pelvic Lesions 229
An algorithm for distinguishing benign from masses, are (1) the age of the child, (2) the organ
malignant pelvic masses is shown in Fig. 15.20. of origin of the mass, (3) the mobility and other
The four important features, as for abdominal characteristics of the mass and (4) the secondary
effects of the lesion elsewhere in the patient.
The commonest benign lesion in a preadolescent
Common girl is an ovarian cyst, which is very mobile. The
secondary effects include pain and vomiting from
torsion of the cyst, and these may mimic appendici-
Full bladder
faeces
tis. Diagnosis is confirmed by rectal examination.
appendix mass Malignant pelvic masses usually occur in much
younger children and in infants. Often, the mass is
fixed to the pelvic wall by direct extension of the
Ovarian cyst/teratoma tumour and feels hard or stony. There may be direct
mesenteric cyst extension of tumour to produce a nodular mass in
the introitus. This is known as sarcoma botryoides
Anterior sacral meningocele and is caused by a rhabdomyosarcoma of the blad-
presacral teratoma der or vagina. The secondary effects of this tumour
include urinary and bowel obstruction or vaginal
bleeding. The important malignant tumours which
Rare
may arise in the pelvis are ovarian carcinoma, rhab-
Fig. 15.19 The relative frequency of benign pelvic
domyosarcoma, malignant sacrococcygeal teratoma
masses and neuroblastoma.
Is it benign or malignant
pelvic mass??
Benign Malignant
?Age pre-adolescent infant
Fig. 15.20 The criteria for distinguishing benign from malignant pelvic masses
230 15 Abdominal Masses
± Colic/shock
2° to torsion
Mobile
cyst in
suprapubic
region
Cyst
palpable
on rectal
examination
Bone/teeth
on x−ray
Further Reading
Golden Rules
1. The key to diagnosis of an abdominal Jones P.G., Campbell P.E., eds. (1976). Tumours of
mass is its organ of origin and the patho- Infancy and Childhood. Oxford: Blackwell Scientific
logical characteristics. Publications.
Filston H.C., Izant R. (1978). The Surgical Neonate. New
2. Mobile lesions are usually benign, York: Appleton-Century-Crofts.
unless there is acute inflammation which Hutson J.M., O’Brien M., Woodward A.A., Beasley S.W.,
causes secondary fixation. eds. (2008). Jones’ Clinical Paediatric Surgery, 6th
3. Fluctuant or cystic masses are usually edn. Blackwell Publishing.
benign (e.g. cysts or hydronephrosis).
Gastrointestinal Bleeding
16
Gastrointestinal bleeding is a relatively common colitis and proctitis, which may be caused by acute
symptom in childhood but only rarely does it sig- infection. Bleeding with chronic diarrhoea is more
nify serious disease. Most children with gastro- suggestive of inflammatory bowel disease.
intestinal bleeding have an anal fissure or
gastroenteritis, rather than the more dramatic
bleeding of the Meckel’s diverticulum or oesoph- The Appearance of the Vomitus
ageal varices.
When a child presents with bleeding from the Rapid bleeding from the oesophagus, stomach or
gut, the history is paramount, since the cause duodenum may cause a child to vomit bright red
often may be determined by careful questioning. blood (haematemesis), which will be observed in
association with pallor and signs of shock. With
slower bleeding from the upper gastrointestinal
The Appearance of the Stool tract, the blood becomes altered by the gastric
acid to appear as ‘coffee-ground’ vomitus.
Blood entering the alimentary tract, unless vom- However, bleeding may occur from these organs
ited, will appear ultimately on or within the without vomiting.
stools. The longer it stays in the bowel, the more
it is broken down and the darker it becomes.
Therefore, it can be seen that the appearance of Minor Bleeding in the Healthy
the blood in the rectum is a function of its point Neonate
of entry (i.e. how far it has to travel) and of its
transit time (i.e. how long it takes to traverse the In the neonate, the most common cause of bleed-
alimentary tract). As a general rule, bleeding ing is an anal fissure, which may result from the
from the oesophagus, stomach and duodenum passage of a stool or may be iatrogenic and sus-
appears as black and tarry stools (melaena), from tained during introduction of a rectal thermome-
the small bowel as dark red-brown stools and ter. Small, bright red streaks of blood can be seen
from the colon as red ‘altered blood’ mixed with on the stool or as spots on the nappy. The child is
the stools. Bleeding from the anorectal region otherwise well, feeds normally and has a soft,
itself appears as bright red streaking on the sur- non-tender abdomen.
face of the stool or on the toilet paper, because The anus should be examined carefully for
the stool has been formed already at the time of evidence of a fissure by parting the perianal skin
contact with the blood. in a lateral direction (Fig. 16.1) with the baby
Blood mixed with diarrhoea is seen in lying supine and hips flexed. It may be helpful to
inflammatory conditions of the bowel, particularly use an assistant to support the legs. A fissure
Little
finger
Thumb
the posterior abdominal wall. Failure of this pro- rare but nevertheless should be suspected in all
cess makes it vulnerable to subsequent twisting cases of neonatal gastrointestinal bleeding.
and ischaemia (see Chap. 21).
The sudden onset of blood-stained stools in
association with bilious vomiting in an infant is Small Amounts of Rectal Bleeding
strongly suggestive of malrotation with volvulus. in a Well Child
This may occur in the absence of abdominal dis-
tension. Malrotation with volvulus is a surgical Small amounts of bleeding in a well child suggest
emergency if the entire midgut is to be saved. benign pathology of the anorectum. Often, it fol-
Examination of the abdomen may be unremark- lows an acute episode of diarrhoea or constipa-
able or may demonstrate some tenderness. tion. The passage of a hard stool may cause a
Abdominal distension in malrotation with volvu- small tear in the mucosa of the anus, producing a
lus is a late sign and suggests that the blood sup- painful anal fissure. Further defecation is avoided,
ply to the midgut has become severely and the faeces become hard and more difficult to
compromised with gangrene impending. As the pass. When they are passed eventually, further
bowel becomes increasingly ischaemic, it dilates trauma to the anal canal causes a small amount of
and fluid transudate leaks into the peritoneal cav- bleeding which can be seen as red streaks on the
ity. Diagnosis can be confirmed with contrast stool or spots of blood on the nappy or toilet
studies or at immediate laparotomy. paper. In severe cases, there is build-up of faeces
Now that vitamin K is administered routinely in the rectum (faecal impaction), and fluid escapes
at birth, haemorrhagic disease of the newborn is around the mass (spurious diarrhoea). Continuous
234 16 Gastrointestinal Bleeding
Fissure
Painful
defecation
Constipation
Faecal impaction
Fluid overflow
Excoriation
of perianal skin
Fig. 16.4 The mechanism by which an anal fissure leads to progressive constipation and eventual impaction with
overflow, which causes excoriation of perianal skin
leakage of fluid from the anus is highly irritating pressure) and chronic diarrhoea, but most often
to the perianal skin, which becomes severely occurs without an obvious underlying cause. It
excoriated and bleeds (Fig. 16.4). Rectal exami- occurs rarely after the age of 3 years and is usu-
nation is exquisitely painful and not mandatory ally seen in boys during the second year of life.
where an anal fissure is seen but if it is performed Prolapse occurs after straining at stool and
will provide information on the amount and hard- may follow either constipation or an episode of
ness of faeces within the rectum. It is now recog- acute diarrhoea. The rectal mucosa is normal in
nized that there is a close association between appearance and has a pink-red, glistening sur-
anal fissures and food allergy, commonly in face. With chronic prolapse, it may become con-
response to dairy protein. gested and ulcerated but almost never becomes
In rectal prolapse, part or all of the rectum gangrenous. The mucosa bleeds readily on con-
protrudes through the anal canal. The part pro- tact with nappies or dressings. Frequently, the
lapsed may involve the mucosa alone or the entire prolapse reduces spontaneously after straining
wall of the rectum. Rectal prolapse is seen some- is ceased, but sometimes the rectum remains
times in association with other conditions, for prolapsed and requires gentle manual reduction.
example, myelomeningocele (paresis of the pel- It can be distinguished from a prolapsing intus-
vic floor), exstrophy of the bladder (disturbance susception in that the child is otherwise well and
of the structural support of the rectum), cystic that there is no sulcus between the prolapse and
fibrosis (chronic cough with increased abdominal the anal margin (Fig. 16.5). At first glance, a
Bleeding with an Acute Abdomen 235
Rectal
prolapse
No sulcus
Rectal
Intussusception Polyp prolapse
rectal polyp may be similar in appearance, but caused by a reflex autonomic response to pain.
this will be distinguished by demonstration of a Palpation of the abdomen may reveal a tender
sulcus and its pedunculated stalk. A rectal pro- mass (see Chap. 8). Where intussusception is
lapse can be reduced by gentle pressure on the suspected, a rectal examination will identify
exposed rectum. whether there is blood in the rectum or whether
the lead-point has advanced as far as the rectum.
The small amount of blood is dark red and has the
Bleeding with an Acute Abdomen appearance of red currant jelly because it is mixed
with excessive mucus and poorly formed stool.
Outside the neonatal period, the most common Rectal bleeding in intussusception is a late sign,
cause of rectal bleeding in association with an and one should not wait for its appearance before
acute abdomen is intussusception. Intussusception making the diagnosis.
can occur at any age, but 75 % of cases involve A less common, but extremely important,
children between 3 and 12 months of age. Usually, cause of small volume rectal bleeding is ischae-
episodic abdominal pain and vomiting are pres- mia of the bowel with impending gangrene. In
ent, but there may be only lethargy and pallor. small children, this is caused by volvulus, which
The pain is colicky and lasts a minute or two, may be secondary to malrotation and failure of
with a longer period without pain between epi- fixation, a Meckel’s diverticulum and band, or by
sodes. As the intussusception progresses, the pain a closed-loop obstruction from postoperative
becomes continuous and less colicky. A small adhesions. The child is anorexic, unwell and
child is unable to communicate pain except by vomits, and with progression of symptoms
crying or by drawing up their legs. Between becomes toxic and dehydrated. The abdomen is
attacks, the child is lethargic and feeds are distended, and one or more dilated loops of bowel
refused. Pallor is often pronounced and probably may be palpable. There is abdominal tenderness
236 16 Gastrointestinal Bleeding
with guarding if peritonitis is present. These chil- diarrhoea in older children is suggestive of
dren require vigorous resuscitation and early inflammatory bowel disease. Explosive bowel
laparotomy. movements of loose, mucousy stools preceded by
A rare cause of rectal bleeding with an acute cramping abdominal pain may be the first indica-
abdomen is Henoch-Schönlein purpura. Its charac- tion of ulcerative colitis. There may be associated
teristic rash and arthralgia may be absent initially. growth failure and weight loss, anaemia and
There may be either localized or generalized arthritis. Insidious mild diarrhoea with occasional
abdominal tenderness and the passage of blood mucus or blood and intermittent pain in a preado-
within the stools. The abdominal pain and rectal lescent is more common in regional enteritis
bleeding are the result of (1) vasculitis with bleed- (Crohn’s disease).
ing into the peritoneal cavity and lumen of the bowel Examination of the abdomen may reveal an
or (2) intussusception (usually of the small bowel). inflammatory mass in the right iliac fossa, or
there may be a chronic anal fissure or perianal
sepsis which antedated the onset of diarrhoea.
Major Gastrointestinal Haemorrhage Alternatively, there may be no specific signs with
either form of inflammatory bowel disease, and
The commonest causes are a bleeding Meckel’s diagnosis is confirmed on contrast studies,
diverticulum and haemorrhage from juvenile colonoscopy and histology.
polyps. Upper gastrointestinal haemorrhage,
although less common, is often major and results
from peptic ulcer disease or bleeding oesopha- Golden Rules
geal varices. Oesophageal varices are a manifes- 1. Major bleeding can occur from the
tation of portal hypertension and are seen in oesophagus, stomach and duodenum
association with features of liver disease (see without vomiting (haematemesis).
Chap. 17). 2. The appearance of blood in the rectum is
A Meckel’s diverticulum causes haemorrhage related to the distance travelled and the
because it contains ectopic gastric mucosa. time taken by the red cells to get from
Hydrochloric acid from the gastric mucosa causes the point of bleeding to the rectum.
an ulcer in the adjacent ileal mucosa. The blood 3. Beware haemorrhagic disease of the
loss may be profound and rapid and, while usu- newborn: Check that the infant received
ally painless, is associated sometimes with vitamin K.
cramping or colicky abdominal pain localized to 4. The commonest cause of rectal bleeding
the periumbilical region. The blood is brick red in in the neonate is an anal fissure.
colour, and there may be a history of several pre- 5. A fissure-in-ano is usually in the mid-
vious episodes of bleeding. With major blood line anteriorly or posteriorly.
loss, the child is pale and has a tachycardia. The 6. Malrotation with volvulus is an emer-
haemoglobin falls after some hours. The diagno- gency which requires immediate
sis is confirmed by performing a 99mTc sodium surgery.
pertechnetate scan. 7. Do not wait for the appearance of rectal
bleeding before making a diagnosis of
intussusception: It is a late sign which
Chronic Diarrhoea and Bleeding indicates that the blood supply to the
intussusceptum has been compromised.
Bleeding associated with persistent diarrhoea 8. A chronic, painless and lateral anal
may be caused by certain organisms, such as fissure may indicate chronic inflammatory
Campylobacter or Salmonella. Where there is no bowel disease.
history of prior gastroenteritis, chronic bloody
Jaundice
17
Jaundice is a common sign in the first 2 weeks of of life (Fig. 17.1). Severe hepatic damage associ-
life. It is usually ‘physiological’, in that it is due ated with cholestasis may show a similar clinical
to the functional immaturity of the normal liver, picture. Neonatal hepatitis of unknown cause or
which matures rapidly after birth. Deep jaundice hepatic damage secondary to septicaemia, viral
or the development of jaundice within 24 h of infections (e.g. cytomegalovirus) or inborn errors
birth implies that additional factors such as hae- of metabolism (e.g. galactosaemia) also needs to
molysis, infection or inborn metabolic errors may be considered. Jaundice without obstructive fea-
be present, and demands urgent investigation and tures (i.e. normal stool and urine colour) may
treatment. An unconjugated bilirubin level above follow less severe hepatic injury without
300 mmol/l (or 250 mmol/l in premature infants) cholestasis or be due to impaired bilirubin uptake
may damage the brain (kernicterus). and conjugation (e.g. hypothyroidism, haemoly-
Jaundice that appears later than, or persists sis, breast milk jaundice or rare congenital
into, the third week of life is not likely to be enzyme defects).
benign ‘physiological’ jaundice and should be
treated as requiring urgent investigation, as rapid
diagnosis and prompt treatment may prevent Assessment of a Patient with
death or permanent damage to the infant Persistent Neonatal Jaundice
(Table 17.1). Jaundice at this stage, accompanied
by pale grey (acholic) stools, dark urine and an When jaundice persists beyond the second week
elevated serum bilirubin which is largely conju- after birth, biliary atresia must be considered,
gated bilirubin, may have a ‘surgical’ cause. Biliary although the diagnosis is usually one of exclu-
atresia (or less commonly a choledochal cyst) sion. Rhesus incompatibility, other forms of hae-
causes an obstructive jaundice in the first months molytic disease of the newborn and congenital
infections of the fetus, such as rubella, herpes,
cytomegalovirus, syphilis and toxoplasmosis,
Table 17.1 Potential causes of persistent jaundice after present with jaundice in the first day or two of life
birth (Table 17.2). In the haemolytic diseases, the
Surgical Non-surgical Coombs’ test is positive.
Biliary atresia Breast milk jaundice The common causes of jaundice between the
Choledochal cyst Primary hepatitis first day and the first week are produced by imma-
Secondary hepatitis ture enzymes, so-called physiological jaundice.
Galactosaemia The bilirubin is unconjugated and there are no
Cretinism anti-red cell antibodies (Coombs’ test negative).
Congenital enzyme defects The children are often premature but otherwise
Persisting
jaundice 1. Percuss
(sclera) area of
despite dullness
thriving
baby
Dark urine
(urobilinogen)
Clay-coloured stools
2. Palpate
edge with notch 3. Feel movement
starting in R.I.F. downwards and
medially on
inspiration
Collateral drainage to
systemic veins
a Supine b On side
a b c
Refilling from
Fig. 17.10 Examination of centre
a spider naevus Compression
Cholelithiasis 243
Golden Rules
1. Jaundice which fails to resolve within
2 weeks of birth must be referred imme-
diately to exclude biliary atresia.
2. Diagnosis of biliary atresia is urgent
because delay in operative correction
beyond 10 weeks significantly worsens
the prognosis.
Spina Bifida
18
Defects caused by failure of neural tube fusion Hence, neural tube defects represent a very early
may present as posterior midline swellings any- and fundamental abnormality of embryogenesis.
where from the bridge of the nose to the tip of the In the severe abnormality of myelomeningocele,
coccyx. Many are associated with severe neuro- the neural tube remains completely unfused
logical deficit or related malformations of the and incompletely covered by skin, exposing
central nervous system such as hydrocephalus. the meninges to the outside. In the less severe
One of the most common neural tube abnormali- meningocele, there is protrusion of the menin-
ties is anencephaly, in which the cephalic part of ges through a defect in the spinous processes,
the neural tube has failed to close. The vault of but the swelling is completely covered by skin
the skull is absent, exposing the deficient cere- (Fig. 18.1).
brum with only the brain stem and cerebellum In the least severe form of spina bifida, spina
remaining. The abnormality is not compatible bifida occulta, there is a defect of the dorsal
with life, and fortunately, most fetuses with this arches of the vertebrae but no protrusion of the
abnormality are stillborn. Meningoceles and meninges.
encephaloceles are discussed further in the chap- The cystic form of spina bifida, including
ter on cranial abnormalities (Chap. 10). myelomeningocele, is readily apparent at birth,
In this chapter, the various presentations of while occult spina bifida may not present until
spina bifida are described so that an adequate ini- adolescence. In the latter abnormality, a posterior
tial assessment can be made at birth or whenever defect in the spinal canal is associated sometimes
the abnormality is diagnosed. These anomalies with either a dermal sinus connecting with the
require highly specialized neurosurgical, ortho- subarachnoid space, a vascular malformation,
paedic and urological management, but the pri- lipoma, hairy patch or hairy naevus. These abnor-
mary physician needs some knowledge of the malities represent dysplasia of the ectoderm
clinical problems so that initial management and overlying the neural tube defect.
referral can be performed correctly.
Neural
crest
10 % 90 %
Meningocele Myelomeningocele
Frequency
Cervical
Thoracic
Lumbar
Sacral
Paralysis
Anaesthesia
Arachnoid + skin
forming sac
Normal anus
Skin-covered
cyst
± Lipoma
± CSF connection
Straight leg
hip flexion
L3
L4
Total paraplegia
High lesion
Low lesion
S1
S3
Legs normal
but bowel and
bladder incontinence
persists
Fig. 18.8 The four common Hip, knee movement
presentations of weakness in foot dorsiflexed
the legs, depending on the
upper level of the spinal
defect
250 18 Spina Bifida
Lumbosacral
myelomeningocele
Hydrocephalus
Setting-sun
Posture of
eyes
L4 paralysis
Club feet
(often 2° to deformation
in utero)
Fig. 18.9 The usual physical signs of lumbosacral myelomeningocele in the neonate
segment (L3), the legs are totally paralysed. If the Assessment of the Sensory Deficit
third lumbar segment is functioning, as occurs
with lesions affecting the fourth and fifth lumbar Determination of the level of sensory deficit will
segments (L4,5), the baby has a characteristic complement the assessment of motor loss, but
posture with flexion of the hip and extension of sensory deficiency is usually found to be slightly
the knee but essentially no other limb movement. less than the motor loss.
When L4 and L5 spinal segments are preserved, The pattern of dermatomes in the neonate is
there is movement of the hip and the knee, and similar to that seen in adults (Fig. 18.10), with tho-
the foot can dorsiflex but cannot plantarflex. The racic dermatomes (TlO-12) supplying the lower
imbalance in the muscles around the ankle may abdomen and the lumbar segments (Ll-5) inner-
manifest itself by a talipes calcaneovalgus vating the skin from the groin down to the lateral
deformity or may only be obvious on close side of the calf. The first and second sacral seg-
inspection of conscious movement. When the ments (S1, 2) innervate the lateral side of the foot
first and second sacral segments (S1,2) are pre- and the dorsum of the leg from the heel to perineum.
served, leg movements are essentially normal but The third and fourth sacral segments (S3,4) supply
bowel and bladder incontinence is likely to per- the perianal region (Fig. 18.11). By holding the
sist. This is usually the case in low sacral lesions. baby’s legs in the air, the level of sensory loss
The common appearance of infants with lum- should be tested by beginning with a painful stim-
bosacral myelomeningoceles is shown in ulus in the perianal region. A blunt-ended safety
Fig. 18.9, where the straight legs are flexed at the pin should be sufficiently sharp to elicit a painful
hips and the feet are commonly deformed by stimulus without harming the child. Starting
malposition in utero. around the anus, test the fourth and third sacral
Neonatal Assessment of Spina Bifida 251
T10
L3 L3
T11
L1
T12 S2 L2 L2
S4 L2
L1 S2
L2 L2
L3 L2 L3
S3 S3
S5
L4 S2 in midline L4
posteriorly
L5 from heel to Fig. 18.11 Perineal dermatomes
buttocks L5
segments, and then proceed down the back of the Bowel and Bladder Function
leg following the second sacral segment to the
heels (Fig. 18.12). From the heel, the first sacral The perineum is a critical area for examination
segment can be tested on the lateral side of the since the likely function of the bowel and bladder
sole, proceeding to the medial side of the sole to can be predicted from the appearance and sensa-
include L5 and L4 segments. By continuing up the tion elicited in the perineum. The appearance of a
anteromedial side of the leg from the medial mal- rectal prolapse or absence of a natal cleft indicates
leolus to the groin, the fourth, third, second and paralysis of the pelvic floor muscles and adjacent
first lumbar segments can be tested in succession. bowel and bladder sphincters (Fig. 18.13).
T10
T11
T12
L2
L1
Fig. 18.12 Testing the L3
level of sensory loss. Start
in the perineum, proceed
down the back of the leg to S4 S3 S2 L4
the heel, test the sole from
lateral to medial and then L5
proceed up the anteromedial
side of the leg from the S2 S1
malleolus to the groin
252 18 Spina Bifida
Paralysis of pelvic
floor muscles
This can be confirmed by testing for anaes- Paralysis and the absence of the dorsal arches of
thesia in the perianal region (Fig. 18.14). The the spinal canal over a large number of segments
legs of the baby should be held up in one hand, will destabilize the posture of the spine.
while the other hand scratches the perianal skin The secondary kyphoscoliosis caused by these
with a blunt-ended pin. Reflex contraction of abnormalities, and by associated hemivertebrae,
the external anal sphincter and gluteal muscles may be present at birth. Such spinal deformity is
will occur if the low sacral segments are intact. a poor prognostic sign because of the high level
The demonstration of perineal anaesthesia indi- of the abnormality. The lumbosacral myelom-
cates that bladder function will be abnormal, eningocele commonly produces imbalance of the
and this can be confirmed by manual compres- hip muscles and secondary congenital dislocation
sion of the lower abdomen to produce inconti- of the hip. The position of the hips, therefore,
nence of urine. should be determined by Ortolani’s test, as
described in Chap. 13. Deformity of the feet may
be caused by imbalance of the muscles when the
Presence of Hydrocephalus lesion affects the fifth lumbar and first sacral seg-
or Meningitis ments or may be caused by deformity in utero of
a completely paralysed foot. This latter variety is
Once the neurological deficit has been deter- common in high lumbar lesions.
mined, the child should be examined carefully
for signs of hydrocephalus and for meningitis,
which are described in Chap. 10. Coexisting Anomalies
Perianal pain
contraction of
Flat perineum external anal sphincter
+ gluteal muscles
Fig. 18.14 Testing the perianal reflex. Hold up the legs traction of the external anal sphincter and gluteal muscles.
and examine whether the perineum is flat. Prick the peria- A normal response requires intact S4 and S5 nerves and
nal skin with a pin to see whether this induces reflex con- pelvic floor muscles
complete sacral agenesis. The most important Table 18.1 The presentations of occult spina bifida
abnormalities in terms of long-term management 1. Asymptomatic
are those affecting the urinary tract, since the risk 2. Lesion on the back
of infection will be exacerbated by any abnor- Vascular anomaly
malities of urinary anatomy in the presence of Hairy patch
incontinence or urinary retention. Naevus
Lipoma
Sinus
Spina Bifida Occulta 4. Short leg/limp
5. Dribbling urine (neurogenic bladder)
Occult spina bifida is an extremely common con- 6. Recurrent meningitis
dition; on x-ray, it is present in one fifth of the
population. Usually, there is a gap in the
ossification of dorsal arches of the fourth and structural anomaly, involving all the germ cell
fifth lumbar vertebrae and the first sacral verte- layers. All children found to have one of these
bra, without any other abnormality. However, in a lesions should be referred for a neurosurgical
small number of patients, there may be important opinion, since associated tethering of the cord
symptoms or signs (Table 18.1). A lesion on the may cause neurological signs to develop later in
skin overlying the spine (vascular anomaly, hairy childhood.
patch, naevus or lipoma), or a narrow sinus con- The level of the lowest point of the spinal cord
necting the skin with the subarachnoid space, sig- changes with age, ascending from the lower bor-
nals that there could be a more widespread der of the third lumbar vertebra to the second
254 18 Spina Bifida
Neonate Adult
lumbar vertebra (Fig. 18.15). This change in the These children commonly present with the
relative position of the spinal cord to the verte- mother complaining that she is unable to buy
brae may impair the mobility of the cord and shoes for the child. Occasionally, the growth of
stretch it if the filum terminale or nerve roots are the foot is not significantly abnormal, but the
abnormally tethered in the region of the bony whole leg may be slightly shorter or thinner than
defect. This produces secondary neurological the contralateral normal leg. These children pres-
signs which appear during the period of rapid ent with a minor limp or stumbling, which may
growth in late childhood, even in the absence of be noticeable only during sports or other physical
the cutaneous manifestations of spina bifida activity. Such children should be referred for
occulta. careful neurological and orthopaedic opinion.
Uncommon lesions which may be difficult to Two rare but important presentations of occult
diagnose by examination of the back include the spina bifida include the gradual onset of neuro-
split spinal cord syndrome, or diastematomyelia, genic bladder and recurrent meningitis.
where the spinal cord is divided into two parts by Frequent dribbling of small volumes of urine
a central bony spike in which the overlying exacerbated by raising the abdominal pressure
spinous process is broader than those above or and frequent infections are both suggestive of a
below. In some patients, the spinal lesion is first neurogenic bladder. In lesions affecting only the
manifested by weakness of the lower limb, lead- fourth and fifth sacral nerves, leg movements may
ing to progressive deformity of the foot believed be normal, yet examination of the perineum will
to be secondary to nerve traction during growth. reveal perianal anaesthesia. If the possibility of a
In a child with a discrepancy in shoe sizes, in neurogenic bladder is suspected – particularly if
which one foot is short with a high arch, the pos- there is some abnormality of the skin over the
sibility of muscular imbalance from nerve trac- sacral or lumbar spine – referral to a paediatric
tion should be considered. urologist is necessary for complete assessment.
Further Reading 255
Golden Rules
1. Paralysis and sensory loss nearly
always occur with a myelomeningo-
cele, except where it is low sacral.
2. In myelomeningocele, the meninges
are exposed to the air.
3. Perianal anaesthesia means that blad-
der function will be abnormal.
4. Motor loss is often more severe than
sensory loss.
5. Children with meningoceles or occult
spina bifida should be followed up
until after puberty since growth of the
spine with cord-tethering may produce
progressive neurological signs.
6. Cervical or high thoracic meningoce-
les generally do not cause severe neu-
rological deficits.
7. Skeletal deformity progresses with age
because of the imbalance in muscle
strength.
8. Paralysis leads to relatively shorter
legs with age because the growth of the
bones requires normal innervation and
active movement.
9. Paralysis and limb dependency lead to
poor venous return and lymphatic
oedema, producing puffy legs which,
with sensory loss, make the feet sus-
ceptible to pressure sores.
10. Recurrent meningitis: A sinus connect-
ing with the subarachnoid space should
be sought by careful examination of
the entire back and midline scalp.
Abnormalities of the Chest Wall
and Breast 19
There is a group of heterogeneous disorders or pectus carinatum) and (3) the rare deficiency
which present as abnormalities of the shape of deformities of the chest wall (which in themselves
the chest wall or breast. Chest wall deformities may be associated with a high protrusion defor-
include anterior lesions involving the ribs and mity). The term ‘deficiency’ denotes absence or
sternum, such as ‘funnel chest’ and ‘pigeon hypoplasia of one or more of the constituents of
chest’, and posterior lesions involving the verte- the chest wall: pectoral muscles, breast, ribs or
brae, such as kyphosis and scoliosis. Lesions of costal cartilages. In the depression and protrusion
the breast include congenital abnormalities, as groups, deformity may be symmetrical or asym-
well as acquired infections and hormonally metrical. The great majority are congenital or
induced enlargement. genetically determined and may increase or
All these problems are discovered when the decrease in severity with growth. The importance
clothes covering the trunk are removed and pres- of careful clinical appraisal relates to:
ent because the parents have noticed an abnor- 1. Identification of those with a protrusion defor-
mality or the child has become concerned about mity secondary to intrathoracic pathology
the way he or she looks. (e.g. tumour or congenital cystic lung).
2. Recognition of those with deficiency
deformities.
The Anterior Chest Wall 3. Accurate analysis of the components of the
deformity to assist in planning surgical correc-
Anterior deformities of the ribs and sternum can tion where this is required.
be separated readily into three major groups Chest wall deformities rarely cause symptoms
(Table 19.1): (1) the common depression deformi- but can be distressing from the psychological
ties (funnel chest or pectus excavatum), (2) the point of view. Severe depression deformities may
less common protrusion deformities (pigeon chest limit cardiac output with exercise in the erect
position, but this is virtually never a clinical
problem.
Table 19.1 Anterior chest wall deformities
Type Name Frequency
Depression Funnel chest Common
Clinical Assessment
(pectus excavatum)
Protrusion Pigeon chest Less common The initial clinical assessment is directed at
(pectus carinatum) establishing the type of chest wall deformity.
Deficiency Poland syndrome Rare This is achieved by determining the relationship
(of bone/muscle) of the sternum to its connecting costal cartilages.
Where the sternum is posterior to the adjacent the sterno-xiphisternal junction is placed nor-
costal cartilages, the deformity is called a depres- mally. In other situations, the site of maximal
sion deformity (Fig. 19.1), and where it is ante- protrusion is at the sterno-xiphisternal junction,
rior to them, it is described as a protrusion often with prominent adjacent costal cartilages
deformity (Fig. 19.2). In the latter case, one part on one or both sides and rotation of the sternum.
of the sternum may be involved to a greater Low sternal protrusion may be secondary to
degree than the rest. Where it is angulated for- intrathoracic pathology, for example, asthma,
wards in its upper part, it is termed a high protru- cystic fibrosis, cystic lung, congenital cardiac
sion. The sternum below this level may appear to disease or intrathoracic malignancy, and these
produce a depression, but this is only relative as conditions should be ruled out on history, exami-
nation and chest x-ray. In other types, the chest
wall deformity is the primary lesion, and there is
no associated intrathoracic pathology, although
±Kypho- Thin child
in Marfan syndrome, there may be a mild coex-
scoliosis isting depression deformity.
Once the type of deformity has been estab-
lished, the following features should be more
exactly defined: the vertical extent of the defor-
mity, the level of its greatest extent, the degree
of asymmetry, the rotation of the sternum, the
Deep depression
+ sternal
prominence of the costal cartilages, the degree
Lateral rotation of hollowing of the rib cage lateral to the sterno-
sulcus xiphisternal junction, the flaring of the lower
Pot belly costal cartilages and the degree of postural
Flared costal
margin kyphosis and scoliosis. Asymmetry occurs
when the sternum is rotated in its longitudinal
axis to make the costal cartilages prominent on
Fig. 19.1 Depression deformity (funnel chest) one side and recessive on the other (Fig. 19.3). In
Asymmetry
Sternal depression, rotation
(upper or lower)
Eversion and
Flaring “Boss” of
Thin High protrusion
costal margin
pectoral
fold
Kyphosis Absent
Scoliosis breast +
pectoral
Fig. 19.3 Features of chest wall deformities: asymmetry, muscles
sternal rotation, flaring of the costal margin and compen-
Syndactyly
satory spinal deformities
Yes No
Treatment if necessary
a b
Eye level
with iliac
crests
Correct tilt
with block
under foot
Fig. 19.7 The assessment of pelvic tilt caused by unequal (iii) the buttock folds. (b) From in front: 1. Stand the
leg length. (a) From behind: 1. Stand the patient on a firm patient on a firm surface with the examiner’s eyes level
surface with the buttocks level with the examiner’s eyes. with the iliac crests. 2. Ensure that the patient’s heels are
2. Confirm that the heels are together and weight-bearing. together and weight-bearing. 3. Press the thumbs firmly
3. Observe the relative levels of (i) the posterior iliac on the anterior iliac spines and assess whether they are
spines, (ii) the dimples overlying the sacroiliac joints and level
Kyphosis
No Yes
No Yes
No Yes
whereas in structural kyphosis, even strenuous supine on a firm surface. The examiner’s hand
muscular effort is unable to eliminate the can be placed beneath the patient’s lumber spine
deformity. to assess the degree of compensatory lordosis.
Scheuermann’s disease is the common struc- The right hand is then placed beneath the leg
tural abnormality leading to kyphosis, which immediately above the ankle and the leg elevated.
commences insidiously in early adolescence. Flexion of the knee is prevented by control of the
It affects the upper thoracic spine with resul- thigh with the left hand (Fig. 19.11).
tant limitation of flexion and extension. In more The angle to which the leg can be raised
severe cases, there is a compensatory lordosis decreases with age but in an adolescent should
with decreased range of movement of the lumbar reach 90°. Early detection and treatment of
spine. As most flexion occurs through the lumbar Scheuermann’s disease, with exercises or a brace,
spine rather than the thoracic spine, stiffness of may prevent progression of the deformity and
the lumbar region has a pronounced effect on the obviate the need for surgical fusion of the
boy’s ability to touch his toes. Straight leg-raising vertebrae.
is reduced because the lordosis causes a relative Other causes of kyphosis are rare in children
tightening of the hamstrings. The patient is placed but are important because they may need
The Posterior Chest Wall 263
Acute onset of pain suggests infection (osteomy- forward as far as usual suggests kyphosis or spon-
elitis/discitis) or trauma (wedge fracture), while dylolisthesis, both of which may be associated
night pain is suspicious of a tumour. The pres- with tight hamstring muscles.
ence of other abnormalities, such as fever (infec-
tion) or voiding difficulties (nerve compression),
may be helpful. Limitation of general activity
may indicate infection, while inability to bend The Breast
Areola
Fundi:
papilloedema Visual field defect
Ovarian tumour
Pubic hair
Menses
Visual
field loss
Lens
Optic
fundus
optic chiasma
Pituitary tumour
Pubertal
mastitis
Fat
Muscle
Gynaecomastia
Liver enlargement
Pubic hair
Testicular growth Penile development
Child Neonate
elevation of ribs horizontal ribs
increases thoracic cannot enlarge
volume thorax
Diaphragm
at birth Swallowed air Nasogastric
or T-O fistula decompression
lung compression
Fig. 20.2 The effect of excess air in the stomach on neonatal ventilation
collapse. The signs of respiratory distress are not palate (Pierre-Robin syndrome), which can be
specific for the cause. In some situations, the under- detected by inspecting the roof of the mouth
lying abnormality is immediately apparent, although using a torch. Sometimes, the larger defects are
a chest x-ray may be required to make a diagnosis. palpable by running the little finger across the
roof of the mouth, but this technique is less reli-
able and the diagnosis is made with greater cer-
Cause Obvious on External tainty if seen directly. The small jaw confines
Examination the tongue to the back of the mouth where it
may fill the cleft and cause obstruction of the
Examine the face and neck. Look for a small nasopharynx (Fig. 20.3). The infant is nursed
undercut lower jaw (micrognathia). This may be prone because in the supine position, the tongue
seen in association with a cleft of the secondary falls backwards and exacerbates the obstruction.
Respiratory Distress Relieved by Crying 271
Cleft palate
Tongue pushed
upwards and
backwards
Obstructs
aiway
Micrognathia
at birth, a fatal outcome may result. In complete A rare but potentially lethal cause of upper air-
bilateral choanal atresia, respiratory distress way obstruction is a pedunculated hamartoma of
develops rapidly and is severe. The child the nasopharynx. It causes intermittent obstruc-
becomes increasingly distressed and cyanotic, tion according to the posture of the infant and
but rapidly turns pink when crying, with relief may be seen at the back of the mouth as it pro-
of the obstruction as air is exchanged via the lapses into the oropharynx. Early diagnosis per-
mouth. The diagnosis of choanal atresia is sug- mits surgical removal before asphyxia occurs.
gested by the sudden improvement in the
infant’s condition on crying and the observa-
tion that the asphyxia is relieved by an oropha- The Frothy Baby in Respiratory
ryngeal airway. The diagnosis is confirmed by Distress
inability to pass a small but firm nasogastric
tube through either nasal passage. The tube Oesophageal atresia is a relatively common con-
should be directed posteriorly from the nostril genital anomaly where there is interruption of the
in a horizontal plane beneath the turbinates continuity of the oesophagus, associated in 85 %
(Fig. 20.5) and not upwards along the line of with a fistulous communication between the
the nose. An alternative method of demonstrat- lower trachea and distal oesophageal segment.
ing complete nasal obstruction is to drip saline The trachea and the oesophagus both form from
or Agarol into each nostril and note whether the foregut tube, and the fistula represents persis-
drainage occurs into the oropharynx. It is tence of this embryological connection.
imperative that the oral airway remains in place Swallowed saliva fills the proximal oesopha-
until surgery has established a patent nasal pas- gus, but the atresia prevents it from passing into
sage on each side. the stomach. As a consequence, saliva accumu-
Unilateral choanal atresia does not cause lates in the pharynx and mouth, giving the appear-
respiratory distress in the newborn and is detected ance of excessive salivation. The babies are often
in later years because of a persistent discharge described as being ‘mucousy’ or ‘drooling’, and
from the obstructed nostril. It must be distin- frothy saliva is seen dribbling from the side of the
guished from a nasal foreign body which usually mouth (Fig. 20.6). The fistula connecting the tra-
produces a purulent discharge. chea with the lower oesophagus may interfere
The Frothy Baby in Respiratory Distress 273
with effective ventilation if air passes preferen- In severe cases, cyanosis ensues. Frequently,
tially down the fistula into the stomach. there is a history of prematurity and maternal
Accumulation of air in the stomach may cause polyhydramnios.
abdominal distension with elevation of the dia- The diagnosis of oesophageal atresia is
phragm and exacerbate the respiratory difficulty. confirmed easily using one simple clinical
manoeuvre. A stiff 10-French-gauge catheter is
introduced gently through the mouth into the
upper oesophagus. In oesophageal atresia, its
progress is arrested between 9 and 13 cm from
the gums (Fig. 20.7). A narrow catheter should
not be used because it is likely to curl up in the
dilated upper oesophagus giving a false impres-
sion of oesophageal continuity (Fig. 20.8). Once
oesophageal atresia has been diagnosed using
Saliva this test, the presence of abdominal distension
bubbling and
dribbling and a resonant abdomen on percussion confirms
from mouth the existence of a distal tracheo-oesophageal
fistula.
If the diagnosis of oesophageal atresia has not
been made at birth, it becomes apparent at the
time of the first feed. The child gags and chokes
Fig. 20.6 Excessive neonatal salivation is highly sugges- and develops cyanotic respiratory distress with
tive of oesophageal atresia overflow of the milk from the oesophagus into
Tube passes
only 10 cm
from gums
Gently insert
10F tube
through mouth
the airway (Fig. 20.9). In oesophageal atresia, the atresia and should be avoided because of the
pharynx should be suctioned frequently to remove high risk of aspiration.
excessive saliva and avoid aspiration.
Oesophageal atresia often (50 %) is associated
with other congenital anomalies, particularly car- Onset of Severe or Progressive
diac, renal, anorectal, vertebral, radial and digital Respiratory Distress
(i.e. ‘VACTERL’ association). The infant must be
examined carefully for the presence of these Respiratory insufficiency shortly after birth in the
anomalies. Oesophageal atresia is seen also in absence of other conditions suggests a diagnosis
association with chromosomal trisomies, of of congenital diaphragmatic hernia. Development
which 13, 18 and 21 are the most common. of the transverse septum between the chest and
A chest x-ray should be obtained in every abdomen is defective, leaving a hole in the dia-
case. This will often show the dilated upper phragm – usually on the left side – through which
‘pouch’ of the oesophagus, and presence of air the abdominal viscera herniate into the chest.
below the diaphragm suggests a distal tracheo- This occurs before birth and inhibits pulmonary
oesophageal fistula. A lateral film may show development by compressing the lung buds,
the lower oesophagus filled with air from the which are hypoplastic. At birth, this produces
trachea. Absence of gas in the abdomen is respiratory distress because of (1) pulmonary
indicative of the rare defects of atresia without hypoplasia and (2) occupation of much of the
fistula or of atresia in association with a proxi- thoracic volume by abdominal contents. The
mal tracheo-oesophageal fistula. Overflow of degree of hypoplasia of the lungs is the ultimate
saliva, or reflux of gastric juice up the fistula determinant of survival.
into the trachea, may produce radiological Often, there is rapid development of respiratory
signs of aspiration pneumonia. The vertebral distress with cyanosis. The rapidity of onset and
column should be inspected for hemivertebrae severity of symptoms vary with the degree of hyp-
or other anomalies. A contrast study is not oplasia and the extent of interference with ventila-
needed to make the diagnosis of oesophageal tion. In the most severe cases, poor peripheral
Onset of Severe or Progressive Respiratory Distress 275
Milk feed
before diagnosis
Overflow of
saliva or milk
aspiration
Aspiration Diaphragm
Pneumonia pushed up by
air in stomach
Acid
reflux Aspiration
Scaphoid
abdomen
Respiratory
rate
Heart on “right”
Poor perfusion
perfusion and cardiovascular collapse occur within The chest is barrel-shaped and the abdomen is
minutes of birth. Apgar scores remain low, and, scaphoid because most of the bowel has herni-
without immediate resuscitation, these infants die ated through the defect in the diaphragm into the
quickly. Lesser degrees of lung compression pro- pleural cavity, leaving the abdomen empty of
duce a less dramatic clinical picture, and a few contents (Fig. 20.10). However, once air is swal-
children have no symptoms for days or months. lowed, this sign becomes less obvious. The
276 20 Respiratory Distress in the Newborn
Bowel and
stomach
Sudden Onset of Respiratory Distress
Heart
in chest
displaced
To right The sudden onset of severe and progressive
Liver symptoms of respiratory distress in an infant who
Deficient
diaphragm has been asymptomatic or is stable following
endotracheal intubation suggests the develop-
Fig. 20.11 The intrathoracic anatomy in a left-sided ment of a pneumothorax. The respiratory rate
congenital diaphragmatic hernia increases with marked sternal retraction, dimin-
ished air entry on auscultation and increased res-
presence of bowel and liver in the chest displaces onance on percussion on the side of the
the mediastinum to the contralateral side. pneumothorax. Shift of the mediastinum is
Swallowed air makes this worse. In a left-sided difficult to detect in this condition. The diagnosis
hernia, this produces apparent dextrocardia (with should always be considered in infants with a
the heart sounds most easily audible in the right pre-existing condition known to predispose to the
chest) and poor sounds on the left side (Fig. 20.11). development of a pneumothorax. The most criti-
In the uncommon right-sided hernia, the signs are cal of these is diaphragmatic hernia, but a pneu-
reversed. The classical sign of bowel sounds in mothorax may also occur following a difficult
one side of the chest on auscultation is not par- delivery or in an infant with hyaline membrane
ticularly reliable and may be difficult to elicit. disease, lung cyst and lobar emphysema. A plain
Where congenital diaphragmatic hernia is sus- radiograph of the chest confirms the diagnosis.
pected on clinical grounds, an immediate plain The development of a tension pneumothorax
chest x-ray must be obtained. The film should is particularly hazardous to the infant and is sug-
include the abdomen so that the distribution of gested by continued progression of symptoms as
bowel gas can be determined. The main radio- a result of further mediastinal shift, contralateral
logical features include: lung compression and interference of venous
1. Loops of bowel in the chest on the side of the return. Cyanosis and cardiovascular collapse are
defect later signs. The diagnosis of tension pneumotho-
2. Hemidiaphragm not visible rax is confirmed on chest x-ray, but in some cases,
3. Mediastinal shift to the contralateral side needle aspiration or tube thoracostomy are
4. Abnormal distribution of bowel gas within the required as life-saving measures before an x-ray
abdomen can be obtained. The best position in which to
There are a number of uncommon conditions insert a chest drain is through the fourth or fifth
which can have a similar radiological appearance intercostal spaces in the anterior axillary line.
Sudden Onset of Respiratory Distress 277
Obstruction of the bowel at birth is a relatively Table 21.2 Causes of neonatal bowel obstruction:
pathology
common abnormality and has a large number of
possible causes, most of which are rare individu- Pathogenesis Disease
ally (Table 21.1). The common forms of obstruc- Failure of gut development Oesophageal atresia
tion in the neonatal period are Hirschsprung Failure of gut canalization Duodenal atresia
disease and necrotizing enterocolitis. Less fre- Ischaemic involution Small bowel atresia
(thromboembolus, volvulus,
quent conditions include small bowel atresia, intussusception)
either of the ileum or jejunum; malrotation or Functional obstruction
incomplete rotation of the bowel, with superven- Inflammation Necrotizing enterocolitis
ing volvulus (twisting of the bowel); duodenal Defective nerves Hirschsprung disease
atresia or stenosis; various abnormalities of the Meconium viscosity Meconium ileus (cystic
anorectal region, including imperforate anus; fibrosis)
meconium ileus (as the first manifestation of cys-
tic fibrosis); and perforation of the bowel, either
prenatally (causing a sterile meconium peritoni-
tis) or postnatally (causing septic peritonitis). or increased viscosity of the contents (Table 21.2).
The pathological processes which lead to bowel Such a classification has some use in the practical
obstruction at birth can be divided into three assessment since the symptoms and signs vary
groups: an intrinsic structural abnormality of the with the three groups, and the groups carry differ-
bowel; an atresia secondary to occlusion of the ent prognostic implications for the baby.
blood vessels in utero; and a functional obstruc-
tion caused by inflammation, defective innervation
The Causes of Bowel Obstruction
Table 21.1 Causes of neonatal bowel obstruction
Disease Frequency Hirschsprung Disease
Hirschsprung disease More common
Necrotizing enterocolitis The ganglion cells of the intramural plexuses in
Small bowel atresia Less common the bowel control the coordination of contraction
Malrotation with volvulus and relaxation of normal peristalsis. If these gan-
Duodenal atresia/stenosis glion cells are missing, the bowel appears normal
Imperforate anus macroscopically, but it is obstructed functionally
Meconium ileus Uncommon because of lack of coordinated peristalsis. The
Prenatal perforation degree of resting contractility in the ‘aganglionic’
(meconium peritonitis) segment of bowel is variable, and it may be atonic
or spastic. Meconium is unable to pass through potential for overgrowth of pathogenic organisms
the aperistaltic segment and leads to a functional in the dilated and obstructed proximal colon to
obstruction with secondary proximal dilatation. produce a fulminating enterocolitis, with rapid
The dilatation occurs in the normally innervated progression to generalized sepsis and death,
proximal bowel and, in the past, led to the term makes Hirschsprung disease an important condi-
‘congenital megacolon’ (Fig. 21.1). The length of tion to diagnose early.
colon affected by this deficiency of ganglion cells
is variable but in most patients extends from the
anus proximally as far as the rectosigmoid junc- Necrotizing Enterocolitis
tion. Pathological confirmation of Hirschsprung
disease is obtained when biopsy of the mucosa Necrotizing enterocolitis is a serious, acquired
just inside the anal canal shows absence of gan- abnormality of the bowel which appears to be
glion cells in the submucous (and myenteric) caused by mucosal hypoxia and ischaemia in
plexus and overgrowth of presynaptic neurofibrils association with bacterial invasion of the gut wall
in the mucosa and submucosa (Fig. 21.2). The by necrotizing and gas-forming organisms
(Fig. 21.3). Conditions which produce perinatal
stress (e.g. prematurity, obstetric complications,
sepsis, cyanotic heart disease and metabolic dis-
Dilated turbances) predispose to its development. The
‘Transition’ normal bowel ischaemic mucosa is colonized by certain patho-
zone
(partial innervation)
genic bacteria which include strains of
Clostridium. These organisms multiply within
the layers of the bowel wall and produce large
gas bubbles known as ‘pneumatosis intestinalis’
‘Aganglionic’ Levator muscles or ‘intramural gas’. Further progression of the
bowel infection and ischaemia produces gangrene and
absent nerves perforation of the bowel, which leads rapidly to
generalized sepsis and peritonitis.
Fig. 21.1 Hirschsprung disease
Suction rectal
biopsy
a b
Fig. 21.2 Pathological
diagnosis of Hirschsprung
disease from a suction rectal
biopsy stained with acetylcho-
linesterase (a) or H & E (b).
Acetylcholinesterase staining Submucosa
reveals numerous extrinsic
autonomic neurofibrils
ramifying in the submucosa
and lamina propria (the
extrinsic nerves overgrow
because they cannot find an
Numerous
intrinsic nerve to form a Muscularis neurofibrils Absent
synapse). H & E shows that mucosa ganglia
the intrinsic enteric nerves,
known as ganglion cells, are Acetylcholinesterase H&E
absent from the rectal wall stain stain
The Causes of Bowel Obstruction 281
a Gap
b
Cord
Web
c
Fig. 21.4 The pathogenesis
of small bowel atresia from
vessel occlusion (a), fetal
intussusception (b) or fetal
volvulus (c)
282 21 Neonatal Bowel Obstruction
c
Narrow
Wide base bile base of
of mesentery mesentery
Duodenal
obstruction
Midgut
ischaemia
a b c
the abnormality varies, ranging from a simple with imperforate anus include identification of
membranous covering over an otherwise rela- the site of a fistula, if present, and determination
tively normal anal canal to complete agenesis of of the lowest level of the normal bowel in relation
the anorectum (Fig. 21.7). In most patients, there to the pelvic floor muscles and anal sphincter
is a fistulous connection from the bowel to the (Fig. 21.8). In addition, patients with imperforate
perineal skin or urogenital tract, which represents anus commonly manifest multiple anomalies in
failure of complete separation of the gut from the other parts of the body (e.g. oesophageal atresia,
urogenital tract. The clinical problems in children cardiac defects) and significant anomalies in
284 21 Neonatal Bowel Obstruction
adjacent structures (e.g. urinary tract anomalies abdominal X-ray by irregular calcification in the
and sacral agenesis). peritoneal cavity. The underlying abnormality
may be any vascular or mechanical event which
produces perforation, including (1) meconium
Meconium Ileus ileus with volvulus of heavily laden loops which
perforate, or with rupture of the dilated proximal
Meconium ileus is a functional disorder of the ileum; (2) ischaemia and infarction of the bowel
bowel in which the highly viscous meconium wall secondary to vascular occlusion or fetal
becomes impacted and then dehydrated, to hypoxia; and (3) intussusception with gangrene
obstruct the terminal ileum with dried meconium and perforation in utero. The timing of the perf-
pellets (Fig. 21.9). It is a manifestation of the oration in relation to birth is variable, and occa-
common recessive disorder of cystic fibrosis and sionally the perforation is secondary to fetal
is its first presentation in 20 % of cases. The mucus hypoxia which may have occurred within the last
produced in these patients is tenacious and sticky few days of pregnancy. When the abnormality has
and makes the meconium sticky, causing obstruc- occurred in the fetus at an earlier stage, the perf-
tion of the distal small bowel lumen. Proximal to oration seals and healing by scar formation may
the obstruction, there is massive dilatation of the result in secondary bowel atresia (Fig. 21.10).
ileum, which may undergo secondary volvulus
leading to perforation and/or atresia (Fig. 21.9).
The Presentation of Bowel
Obstruction
Prenatal Perforation
Three cardinal symptoms herald obstruction of
Perforation of the bowel in utero produces a dis- the bowel in the neonate: (1) vomiting, (2)
tinctive form of sterile peritonitis. Leakage of abdominal distension and (3) failure to pass, or
meconium through a perforation initiates a chem- delay in the passage of, meconium.
ical inflammation in the peritoneal cavity and This triad of symptoms is similar to the pre-
metabolic conditions favourable to calcification. sentation of bowel obstruction at other times of
Thus, meconium peritonitis is recognized on life. The important principle in management is
The Presentation of Bowel Obstruction 285
Unused
‘microcolon’ Perforation
Pellets
2° atresia
lncidence
5 10
Age at presentation (days)
In a neonate with bile-stained vomiting, with symptoms or signs at birth or within the first
abdominal distension and failure to pass meco- 48 h, whereas the functional obstructions, such as
nium, the apparent urgency of the clinical situa- Hirschsprung disease and necrotizing enterocoli-
tion may lead to the infant’s transfer to a surgical tis, usually present after the first 48 h (Fig. 21.11)
centre before a complete initial evaluation has since they do not cause obstructions in utero.
been undertaken. However, the clinician should Details of the presenting signs and symptoms
elicit several important points from the obstetric may be useful. Vomiting of milk which after a
history: (1) details of obstetric problems, includ- period becomes bile-stained, is more likely to be
ing antepartum haemorrhage or pre-eclamptic associated with obstruction of the small bowel or
toxaemia, as these may predispose to fetal colon than when bile-stained vomiting is the ini-
hypoxia and secondary gut perforation; (2) details tial feature. In this latter situation, the diagnosis
of prenatal screening tests, particularly ultra- favours duodenal obstruction, with the important
sonography in the second trimester, which may cause being malrotation with volvulus. In fact,
have detected structural abnormality; and (3) the any child who presents with green vomitus as the
presence or absence of hydramnios, since swal- initial symptom should be regarded as having
lowed amniotic fluid is absorbed in the distal malrotation with volvulus until proven otherwise
jejunum. If there is a congenital obstruction in (Fig. 21.12).
the proximal jejunum, duodenum or oesophagus, Abdominal distension, when present at birth,
there will be significant polyhydramnios. suggests small bowel obstruction and is typical of
Obstruction distal to the midjejunum is unlikely meconium ileus (Fig. 21.13). High obstructions,
to be associated with polyhydramnios. such as oesophageal atresia, duodenal atresia or
A family history of other children with cystic malrotation with volvulus and secondary duode-
fibrosis, an earlier baby with meconium ileus or a nal obstruction, in most instances are not associ-
history of Hirschsprung disease in siblings or ated with abdominal distension. More distal
parents is further useful information. The timing causes of obstruction, such as imperforate anus,
of onset of symptoms provides a clue to the diag- Hirschsprung disease or necrotizing enterocoli-
nosis since most anatomical obstructions present tis, do not present with abdominal distension at
The Presentation of Bowel Obstruction 287
±”Double
bubble”
Duodenum not
greatly enlarged
±Distension
(closed loop
”Cork-screw’’on obstruction)
barium meal
±Signs of
peritonitis
(midgut infarction)
Abdominal distension
birth but after a few days. There are two excep- distal tracheo-oesophageal fistula, particularly if
tions to these general principles. First, in oesoph- the neonate is premature and is ventilated for
ageal atresia, marked gaseous abdominal respiratory distress. Positive airway pressure
distension may occur from passage of air down a forces gas down the fistula into the stomach and
288 21 Neonatal Bowel Obstruction
Epigastric
fullness
Scaphoid
Green lower abdomen
vomitus
b c
Web
Double bubble
Distal
No distal gas
gas
small bowel, and the abdomen rapidly becomes is done by collecting all the historical evidence
tympanitic and distended. The second exception and looking for physical signs of obstruction.
is malrotation with volvulus, where abdominal Early and extreme abdominal distension, associ-
distension developing after the volvulus causes ated with failure to pass meconium, suggests long-
obstruction of both the duodenal and colonic ends standing and complete obstruction. By contrast,
of the midgut. Vascular insufficiency and ischae- moderate or mild distension appearing some days
mia cause fluid to accumulate in the midgut after birth suggests either functional or incomplete
lumen, which becomes dramatically distended obstruction. Where the clinical evidence indicates
and exhibits signs of peritoneal irritation. a bowel obstruction, the next step is to determine
The time of first passage of meconium after the level of obstruction, as this will provide an
birth is an important indicator not only of the con- important clue to the underlying cause.
tinuity of the gastrointestinal tract but also of its General examination of the baby should include
functional competence. One of the cardinal symp- inspection of the vomitus or nasogastric aspirate to
toms of Hirschsprung disease is failure to pass determine whether it contains bile or blood. Acute
meconium within the first 24 h of birth. In 90 % of gastritis caused by accumulation of acid in high
normal children, meconium is passed within 12 h, obstructions (e.g. duodenal atresia) may present
while in 99 %, meconium is passed by 24 h. In pre- with blood in the vomitus. Observation of the abdo-
mature or very sick neonates, passage of meconium men will determine the degree, extent and location
may be delayed, but even in these the diagnosis of of distension. Duodenal obstruction produces epi-
Hirschsprung disease should be considered. gastric fullness, with a scaphoid lower abdomen
(Figs. 21.12 and 21.14). Hirschsprung disease may
present with dilatation of the colon several days
Physical Examination before the small bowel becomes dilated (Fig. 21.15).
Volvulus with duodenal obstruction alone presents
When one of the three cardinal signs of bowel with a scaphoid abdomen, while distension of the
obstruction is present, the clinician’s first role is to ischaemic small bowel causes generalized disten-
determine whether there is true obstruction. This sion. Unrelated abnormalities, such as massive
Physical Examination 289
Progressive
distension
Mottled
periphery
O esophageal atresia
R adial T racheo-oesophageal
aplasia fistula
Variable
R enal
anomalies
V ertebral anomalies
(e.g. hemivertebrae)
Imperforate
A nus
a b
Microcolon
Disorders of sex development (DSD) present as a stimulates the Wolffian or ‘male’ duct to develop
wide spectrum of physical appearances ranging into a vas, epididymis and seminal vesicle: in its
from normal female to normal male. This chapter absence, the Wolffian duct regresses. Műllerian-
provides a practical approach to the assessment inhibiting substance (MIS/AMH) causes active
and diagnosis at first presentation rather than regression of the Műllerian or ‘female’ ducts.
emphasizing the pathological classification. The Without a Y chromosome and SRY gene, the
most important DSD is congenital adrenal hyper- gonad develops into an ovary which secretes low
plasia (CAH) because of its frequency (about levels of oestrogen during early development. In
50 % of cases) and its association with life- the absence of Műllerian-inhibiting substance,
threatening vomiting and dehydration shortly the Műllerian ducts form the fallopian tubes, the
after birth (1-3 weeks). uterus and upper third of the vagina – the latter
two by fusion of the caudal ends of the ducts. In
the female embryo, the Wolffian duct undergoes
Normal Sexual Development passive regression because androgen is absent
(Fig. 22.1).
All embryos are formed with the capacity to dif- The early development of the external genita-
ferentiate into either a male or a female. At lia is the same in both sexes (Fig. 22.2). The cloa-
8 weeks of gestation, the urogenital ridge con- cal membrane is surrounded by an inner genital
tains a primitive gonad and two ducts. The meso- fold and a genital tubercle anteriorly. Lateral to
nephric duct (Wolffian duct) forms the vas the inner genital folds are the outer genital folds,
deferens, and the paramesonephric duct which form either the scrotum or the labia majora.
(Műllerian duct) forms the Fallopian tube, uterus Fusion of the central region of the inner genital
and upper vagina. folds divides the cloaca into an anterior urogeni-
The two sexes do not form equally: develop- tal membrane and a posterior anal membrane.
ment of the female internal and external genitalia Development from 8 to 10 weeks’ gestation
begins in the absence of fetal hormones, whereas differs between the sexes and is dependent on the
development of male structures is controlled presence of testosterone. If testosterone is present
actively by the hormones secreted by the testes (i.e. male or abnormal female), the inner genital
(Fig. 22.1). When a Y chromosome (containing folds fuse superficially over the urogenital sinus
the SRY gene) is present, the gonad develops into to form the male urethra, while fusion of the outer
a testis at 8 weeks of gestation and begins to genital folds forms the scrotum. Enlargement of
secrete two hormones, an androgen (testosterone) the genital tubercle forms the penis. The degree
and a glycoprotein (Műllerian-inhibiting sub- of fusion of inner and outer genital folds is
stance or anti-Műllerian hormone). Testosterone directly related to the level of androgens. In the
Undifferentiated
XX gonad XY
Ovary
Testis
Passive Active
E Wolffian Műllerian
MIS
duct duct Testosterone
regression regression
Műllerian
duct Wolffian
duct
Fig. 22.1 Sexual differentiation of the internal genital ducts under the influence of oestrogen (E), Műllerian-inhibiting
substance (MIS/AMH) or testosterone
female, where androgen levels are extremely low, 1. Any degree of virilization of the external geni-
the urogenital membrane breaks down to form talia is proof of androgen secretion, which
the urethral and vaginal openings, and the genital may be normal or abnormal.
tubercle does not enlarge. The inner genital folds 2. The presence of a vas or epididymis is proof
remain separated to form the labia minora, while of androgen secretion at an early stage of
the outer folds enlarge to form the labia majora. development since this implies that the
The genital tubercle, which forms the clitoris, Wolffian duct has been actively stimulated.
remains small and is usually hidden by growth of 3. Regression of the Műllerian duct is proof of
the genital folds. Although oestrogenic hormones the presence of functional testicular tissue,
are present in the female embryo, they are not which has produced Műllerian-inhibiting sub-
important in the initial development of the exter- stance (MIS/AMH).
nal genitalia. Therefore, if testosterone is absent
or unable to function, the external genitalia will
have a female appearance even in a genetically Abnormal Sexual Development:
male fetus. Ambiguous Genitalia
Because male development is actively con-
trolled, several important principles useful in The common forms of DSD which cause ambig-
clinical practice can be derived: uous genitalia are shown in Table 22.1.
Abnormal Sexual Development: Ambiguous Genitalia 295
Genital
tubercle
Cloaca
Inner
genital fold
Outer genital
fold
Urogenital
membrane
Anal
membrane
and leads to severe dehydration, which usually Dysplastic Ovaries (Turner Syndrome;
presents in the second or third week of life with 45,X DSD)
sudden collapse (the so-called salt-wasting cri-
sis). Failure of cortisol secretion produces an The other DSD common in females is failure of
infant with low resistance to stress, so that even ovarian development caused by Turner syn-
trivial infections will induce severe vomiting, drome, but in these children the external genita-
diarrhoea and dehydration. Occasionally, less lia are normal female because external genitalia
common enzyme deficiencies lead to excess pro- will develop a female appearance in the absence
duction of deoxycorticosterone and induce hyper- of any hormone. Babies with Turner syndrome
tension (Fig. 22.3). may be identified clinically by growth retarda-
tion and a webbed neck. The fetal lymphatics in
Table 22.1 Common causes of abnormal sexual Turner syndrome are abnormal: lymphangioma-
development tous development, which occurs in the cervical
Congenital adrenal hyperplasia (CAH) (46,XX DSD) lymphatics, leads to the redundant folds of skin
Dysplastic ovaries (Turner’s syndrome) (45,X DSD) of the neck. Lymphangiomatous maldevelop-
Dysplastic testes ment elsewhere in the fetus with Turner syn-
Symmetrical (46,XY DSD) drome may be seen on the dorsum of the hands
Asymmetrical (mixed chromosome DSD) and feet. Therefore, a baby born with oedema-
Androgen insensitivity, syndrome (AIS) (46,XY DSD) tous or puffy feet should be investigated for
Complete (CAIS)
Turner syndrome if the genitalia are normal
Incomplete (partial AIS)
female in appearance.
X Hypothalamus
CRF
Pituitary
Androgens
Cortisol Aldosterone
Virilization
Fig. 22.3 Biochemical
abnormalities in the common Addisonian Dehydration
forms of congenital adrenal crisis Na K
hyperplasia (CAH)
The Clinical Presentation of DSD 297
Dysplastic Testes (46,XY DSD) complete syndrome (CAIS), the external genita-
lia are completely female in appearance.
Disorders of sex development may be produced Commonly, the clue that gonads are really testes
by dysplastic testes. One or both testes may be is their discovery in inguinal herniae. The normal
affected. When both testes are dysplastic, defici- secretion and response to Műllerian-inhibiting
ency of androgen may cause incomplete viriliza- substance (MIS/AMH) leads to complete regres-
tion of the external genitalia, and deficiency of sion of the Műllerian duct, while inability to
Műllerian inhibiting substance may allow preser- respond to androgens prevents the development
vation of Műllerian duct structures (i.e. uterus of the Wolffian duct into a vas. Thus, there is an
and tubes). Usually, both gonads are undescended absence of internal genital ducts, but persistence
because they are unable to secrete the hormone(s) of the lower vagina. The failure of the tissues to
necessary for testicular descent. respond to testosterone prevents complete descent
of the testes. Infants with incomplete androgen
sensitivity (partial AIS) have incomplete viriliza-
Ambiguous Asymmetrical Genitalia tion of the external genitalia, but the testes are
palpable usually in the groin or labioscrotal
In mixed chromosome DSD, there may be asym- folds.
metrical dysplastic testes (known as mixed
gonadal dysgenesis, and usually with 45,X/46,XY
chromosomes), one gonad may be a reasonable The Clinical Presentation of DSD
testis which has descended with preservation of
the Wolffian duct on that side and local regres- Babies with a DSD present with three basic types
sion of the Műllerian duct. The more dysplastic of ambiguous genitalia at birth: predominant
gonad usually remains in the abdomen and is female appearance but with an enlarged clitoris;
nonfunctional (a so-called streak gonad), and indeterminate genitalia which appear half female/
which is unable to cause regression of its half male; and predominantly male appearance,
Műllerian duct or preservation of its Wolffian but with hypospadias and impalpable gonads
duct. The external genitalia show some viriliza- (Table 22.2). Infants with obviously ambiguous
tion with one palpable testis. Children with mixed genitalia have the LEAST risk of an error being
gonadal dysgenesis may appear to be males with made in the neonatal period because the attending
‘severe hypospadias’ and one palpable testis. It is physician is unable to assign a sex and urgently
an important abnormality to identify since the refers the child for further investigation. The chil-
dysplastic testes have a significantly increased dren at greatest risk are those with enlargement of
risk of malignant degeneration. the clitoris, where the anatomy appears to be
‘female’, and those with ‘hypospadias and unde-
scended testes’, where the anatomy appears to be
Androgen Insensitivity (CAIS or Partial ‘male’. In these two groups, there is strong pres-
AIS; 46,XY DSD) sure on the attending physician to assign the sex
which is closest to the appearance of the genitalia
Infants with a 46,XY chromosome pattern but rather than refer to a regional centre for further
with insensitivity to androgens have a genetic
abnormality in the androgen receptor system.
Table 22.2 Clinical presentations of DSD
This prevents the normal target tissues, such as
the Wolffian duct and external genitalia, from ‘Clitoral enlargement’
responding to androgen stimulation. The abnor- ‘Ambiguous genitalia’
mality may be partial or complete, and in the ‘Hypospadias with impalpable testes’
298 22 Ambiguous Genitalia: Is It a Boy or a Girl?
Urogenital
opening
Empty
Labio-scrotum
Incomplete
fusion
Empty
hemiscrotum
Androgens
Low Very high
excess pigmentation, which is common in con- degree of fusion of the inner genital folds to form
genital adrenal hyperplasia because of the excess a urethra; and (3) the degree of outer genital fold
melanin-stimulating hormone produced by the fusion to form a scrotum, with its characteristi-
pituitary. The pigmentation is commonly in the cally wrinkled skin (Fig. 22.8). Any degree of
genital or perianal skin, nipples, palmar creases virilization indicates exposure to androgenic hor-
and knuckles. The body may show a general mones, but does not prove that the child is a
increase in melanin pigmentation, including genetic male, even if masculinization is extreme.
freckles. Darkly pigmented genitalia are espe- The phallus is lifted up to expose its ventral sur-
cially suggestive of adrenal hyperplasia, allowing face, and the genital folds are spread apart with
for racial characteristics. the fingers: the position of the urethral or urogen-
The external genitalia should be examined to ital orifice can be identified and the degree of uro-
determine (1) the size of the phallus; (2) the genital fusion estimated (Fig. 22.9).
The Clinical Presentation of DSD 301
Vomit
Dehydration ±Pigmentation
high levels of androgens are produced at the time or even laparotomy and gonadal biopsy may be
of sexual differentiation, the external genitalia required. The sex of the child needs to be deter-
may be completely masculinized, producing a mined quickly, but this can be done only after
very large phallus identical to a penis (and con- consultation and proper investigation.
taining a penile urethra) and a short upper vagina
which opens into the posterior urethra. The only
clue to the underlying DSD is the absence of the Absent Vagina
testes. The common form of anatomy in children
with congenital adrenal hyperplasia is shown in This is a frequent concern of mothers of normally
Fig. 22.12. The urogenital fusion causes partial feminized infants: during washing or a change of
covering of the introitus beneath which there is a nappy, the mother notices that the introitus can-
normal uterus and vagina. not be seen. She assumes that the girl has an
absent vagina and seeks immediate medical opin-
ion. The usual diagnosis in this circumstance is
Investigation labial adhesion, although true atresia of the vagina
does occur.
Any child born with a genital anomaly should be Labial adhesion is secondary to inflammation
referred for immediate investigation, including and ulceration of the labia minora in an infant
chromosome analysis, electrolyte estimation (for with ammoniacal dermatitis. When the labia re-
evidence of low sodium and high potassium con- epithelialize, the two sides of the introitus become
centrations in the serum), blood glucose accidentally stuck together by bridging of the
concentrations (hypoglycaemia is commonly new epithelium. It is seen in infancy or early
associated with adrenal hyperplasia) and childhood but not at birth. The fusion of the labia
17-hydroxyprogesterone concentrations in order is quite different from the fusion seen in females
positively to identify children with the common exposed to virilizing androgens. The labia can be
form of adrenal hyperplasia. Urinary gas-liquid separated by simple traction or by pushing a ther-
chromatography may show some of the less com- mometer or paper clip posteriorly between them.
mon forms of abnormal steroid production. In There are several points which enable the
addition, an urgent urogenital sinugram, with or common acquired adhesion of the labia to be dis-
without endoscopy, should be performed to delin- tinguished from congenital vaginal or hymenal
eate the anatomy of the urethra and possible atresia (Fig. 22.13). In labial adhesions, the labia
vagina. A pelvic ultrasound scan confirms the cover the posterior half of the introitus like two
presence of a uterus. Occasionally, laparoscopy curtains joined in the midline. The posterior rim
Absent Vagina 303
a
2. Do not assign the sex of a child born
with ambiguous genitalia. Perform
complete physical examination, investi-
gations and urgent referral to an expert.
Referral must be on the first day of life.
3. Virilization of the external genitalia is
proof of androgen secretion (normal or
abnormal): the clitoris enlarges, the
labia minora fuse to create a male ure-
thra, and the labia majora unite as a
b scrotum and become wrinkled.
4. The presence of a vas or epididymis is
proof of androgen secretion at an early
stage of development.
5. Any regression of the Műllerian duct
indicates functional testicular tissue
producing Műllerian-inhibiting sub-
stance (MIS/AMH).
6. Congenital adrenal hyperplasia must
be diagnosed as soon as possible (even
if there is no salt losing) because viril-
ization progresses with age and, if cor-
rectly treated, a girl with congenital
adrenal hyperplasia may be fertile.
Fig. 22.13 The different appearance of the vulva in labial 7. Vomiting, diarrhoea and dehydration
adhesions in infancy (a) and vaginal atresia of a neonate in the neonatal period in a phenotypic
(b) ‘male’ may indicate a girl with con-
genital adrenal hyperplasia.
of the labia is absent around the site of the 8. Perineal pigmentation is suggestive of
fourchette. In vaginal atresia (Fig. 22.13b), the congenital adrenal hyperplasia when
condition presents at birth or puberty (times of high amounts of melanin-stimulating
exposure to hormone stimulation) rather than in hormone have been produced by the
infancy. The hymen bulges out between an obvi- pituitary gland.
ous rim of the labia minora, which is continuous 9. The presence of Műllerian structures
posteriorly around the fourchette. can be determined clinically on rectal
examination by palpation of the cervix
and uterus.
10. The presence of two testes indicates a
genetic male, even if the external geni-
Golden Rules talia are feminized, but does not indi-
1. The diagnosis and sex of rearing need to cate the correct sex of rearing. This is
be determined as soon as possible after determined after referral and complete
birth, to avoid the salt-losing crisis of investigation.
adrenal hyperplasia and to prevent 11. A baby with ‘hypospadias’ should be
undue psychological and social stress assumed to be male only when both
on parents, relatives or the child. testes are palpable in a fused scrotum.
304 22 Ambiguous Genitalia: Is It a Boy or a Girl?
Further Reading
12. In discussion with parents, avoid emo-
tionally disturbing terms, such as ‘her- Hutson JM, Warne GL, Grover SR (eds) (2012) Disorders
of Sex Development: An integrated approach to man-
maphrodite’, ‘pseudohermaphrodite’
agement. Springer, Heidelberg.
or even ‘intersex’. ‘Genital anomaly’
or ‘disorders of sex development’ is an
acceptable label while awaiting
definitive diagnosis and deciding the
sex of rearing.
Index
Cirrhosis, 238–241 F
Cleft lip, 11, 139, 145–147, 149 Face
Cleft palate, 13, 139, 142, 145–148 abnormalities, 132
clinical variations, 148 development, 133
Clitoral enlargement, 297, 298 Faeces, 23, 38, 76, 78, 82, 91, 95, 96, 155, 170,
Cloaca 216, 217, 227, 229, 232–234
development, 283, 293 Femoral hernia, 55, 56
‘Club foot’, 14, 186, 250 Fingertip laceration, 168
Colic, 23–26, 35–38, 91–99, 145, 230, 235, 236, 282 Flail chest, 157, 159
Congenital adrenal hyperplasia (CAH), 72, 293, Flat feet, 190–191
295–296, 298–303 Foot deformity, 185–187, 202
Congenital anomalies, 9, 10, 18, 102, 138, 141, 209, 212, Foreign body
269, 274 inhaled, 181–182
Consciousness, 29, 127, 138, 161–162, 165, 249–251 swallowed, 180–182
Constipation, 19, 37, 38, 59, 91, 95–96, 216, 233, 234 Foreskin
Corrosive adherent, 63, 65, 67
swallowed, 182 non-adherent, 64–65
Craniosynostosis, 123, 131 Four stages of development, 10
Cremasteric reflex, 44–45 Fractures
Cremaster muscle, 44, 46 capitellar, 174–175
Crohn’s disease, 236 clavicle, 172
Cystic hygroma, 107, 108, 110, 113, 143, femur, 152, 176, 197, 199
149, 227, 271 forearm, 173–176
lower limb, 163, 176
supracondylar, 173–174, 183
D upper limb, 173–175
Dentigerous cyst, 139, 143, 144 Frequency, 12, 19, 25, 32, 35, 49, 53, 59, 63, 69, 83, 85,
Depressed fracture, 165–167 92–94, 116, 123, 128, 132, 148, 156, 162–163,
Dermatomes, 250, 251 185, 191, 195, 199, 203–204, 209, 210, 213,
Dermoid cyst, 116, 117, 131–132, 134, 139, 142–143, 222, 227, 229, 234, 246, 247, 254, 257, 263,
255 265, 267, 273, 274, 279, 281, 293, 302
Diagnosis, 1, 11, 19, 41, 63, 76–83, 91, 101–102, 123, Frothy baby, 272–274
139, 151, 185, 210, 215, 232, 237, 246, 261, Fundi, 161, 163, 266
269, 280, 293 Funnel chest, 257, 258
Diaphragmatic hernia, 274–277
Diarrhoea, 19, 20, 24, 26, 33–35, 37, 39, 85, 93, 94, 99,
213, 231, 233, 234, 236, 296, 303 G
Discharging vagina, 169, 171 Gallstones, 242
Disorders of sex development (DSD), 70, 72, 73, Gastric peristalsis, 83, 86, 87, 89
293–302, 304 Gastroenteritis, 19, 24–26, 37, 84, 85, 91, 93, 99, 231,
clinical examination, 298–302 236, 301
clinical presentation, 297–298 Gastro-oesophageal reflux, 26, 83–86, 203
Dorsal hood, 14, 70, 71, 73 Gastroschisis, 12–13, 76, 78, 80, 82
Down syndrome, 141 Generalized peritonitis, 34, 280
DSD. See Disorders of sex development (DSD) General principles, 1–7, 151, 287
Duodenal atresia, 141, 279, 282, 283, 286–289 Genitalia, 14–16, 26, 41, 45, 59, 63, 69–70, 72, 169, 172,
Duodenal injury, 156 197, 207, 267, 293–304
Dysmorphism, 11 Germ layer, 9, 10
Dysuria, 32, 69, 203–204, 210, 213 Gestational age, 14–17
Glasgow coma scale, 161–162, 165
Glomerulonephritis, 211, 213–214
E Goal-oriented examination, 2–4, 6, 151
Ears, 11–15, 79, 123, 127, 132, 134–138, 141, 172 Gowers’ test, 190, 191
Encephalocele, 123, 132–134, 245 Groin lump
Epididymitis, 49–53 causes, 55
Epigastric hernia, 81 Guarding, 23, 27–30, 32–35, 38, 39, 95, 155, 170, 171,
Exomphalos, 75, 76, 78–80, 82, 141 216, 219, 235–236, 289
External angular dermoid, 131–134 Gums
External inguinal ‘ring’, 42, 43, 57, 301 lesions, 144, 146
Extradural haematoma, 162, 165, 166 Gynaecomastia, 264, 267–268
Index 307
H In-toeing, 187–189
Haematocele, 53, 55 Intracerebral haematoma, 126, 166
Haematuria, 38, 96, 97, 156, 182, 203, 204, 212–214, 223 Intraperitoneal bleeding
causes, 213, 214 signs, 155
Haemolytic uraemic syndrome, 211 Intra-thoracic trauma
Haemorrhagic disease of the newborn, 233, 236 consequences, 157
Haemothorax, 157–159 Intussusception, 19, 20, 24–26, 35–36, 38, 78, 83,
Handlebar injury, 151, 153 91–99, 145, 149, 216–217, 219, 235, 236,
Hard palate 279, 281, 284, 285
development, 13, 147 mass, 20, 26, 94, 95, 97, 216–217, 219, 235
Head injuries, 159–169, 183 signs, 20, 94, 95, 99, 216, 235, 236
minor, 165 symptoms, 20, 93, 95, 99
neurological assessment, 161 Irritable hip, 195, 196
severe, 162, 165
Hemihypoplasia
face, 103–105, 121 J
Henoch-Schönlein purpura, 19, 38, 91, 92, 96, 97, 191, Jaundice, 13, 224–228, 237–243, 267
211, 213, 214, 236 neonatal, 224, 237–240
Hernia potential causes, 237
indirect, 55
strangulated inguinal, 19, 51, 59, 61
Hip joint, 185, 194, 196–198, 200, 201 K
Hirschsprung disease, 212, 279–280, 285–289, 292 Kidney, 13, 23, 41, 154–157, 204, 209, 216–218, 220,
History, 1, 20, 44–45, 66, 81, 83, 94, 103, 127, 148, 151, 221, 223–226, 295–296
187, 204, 216, 231, 258, 273, 285, 298 Knock knees (Genu Valgum), 189
Horseshoe kidney, 221, 223 Kyphoscoliosis, 252
Hydatid of Morgagni, 49–52, 61 Kyphosis, 257–259, 261–264, 268
Hydroceles, 41, 42, 51, 53–56, 58, 59, 61 causes, 261, 263
diagnosis, 54
encysted hydrocele, 41, 42, 55, 56, 58, 59, 61
Hydrocephalus, 123–127, 138, 245, 246, 250, 252 L
clinical signs, 124, 125 Labial adhesion, 302, 303
presentations, 126, 127 Labial laceration, 172
Hydronephrosis, 217, 220, 221, 225, 226, 230, 289 Lacerations, 154–156, 158, 159, 166–172, 176, 183
Hypertension, 26, 82, 159, 209, 211, 221, 236, 238, Lap-belt injury, 152, 156
240–242 Lateral neck swellings, 107
causes, 211, 236, 240 Limp
Hypospadias, 14, 63, 69–73, 297–299, 303 causes, 195–198, 200
abnormalities, 70 clinical examination, 198–201
types, 195, 198, 199
Lingual thyroid, 118, 120, 139, 141, 142, 149
I Lips
Idiopathic scrotal oedema, 49, 50, 52, 53, 72 lesions, 144, 146
Imperforate anus, 11, 52, 225, 279, 282, 283, 286–287, Liver failure, 241, 267
289, 291 Localized peritonitis, 34, 38, 180
Indirect inguinal hernia, 55 Loin pain, 23, 50, 51, 203, 204, 213
Inflammation Lymphatic drainage
signs, 215, 219 head, 108
Inguinal hernia Lymphatic malformation, 13, 107, 110, 113, 121, 139,
contents, 58, 59 141, 227, 271
irreducible, 57–59 Lymphoma, 36, 107, 216, 217, 241
ovary, 59
Inguinal region
surface anatomy, 43 M
Inhalation injury, 179 Macroglossia, 139–141, 147
Injured child causes, 141
assessment, 151–153 MAIS. See Mycobacterium avium intracellulare
priorities, 153 scrofulaceum (MAIS)
Internal femoral torsion, 187–189 Malrotation, 11, 24–26, 233, 235, 236, 279, 281–282,
Internal tibial torsion, 187–188 285–288, 292
308 Index
Marfan syndrome, 258 Occult spina bifida, 187, 189, 245, 253–255
Masses presentations, 253, 254
abnormal abdominal, 216–217 Oesophageal atresia, 10, 272–275, 277, 279, 283–284,
normal, 216 286, 287, 289
normal abdomen, 23 diagnosis, 273, 274
right iliac fossa, 35–36, 236 Oesophageal varices, 26, 236, 240, 241
tumour, 217–221 Open pneumothorax, 157, 159
Mastitis, 264, 265, 267 Optic disc, 128, 129
pubertal, 264, 265, 267 Orbital, 132–134
Meatal stenosis, 63, 69, 72 Orbital cellulitis, 132
Meatal ulcer, 63, 68–69, 72, 213 Organogenesis, 9, 10
Meckel’s diverticulum, 75, 77, 91–92, 231, 235, 236 Oropharynx
Meconium ileus, 238, 279, 284–287 lesions, 147–149
Meconium peritonitis, 279, 285 Ortolani test, 185, 186, 202
Medical interview, 3 Osgood-Schlatter disease, 197–198
Meningocele, 132, 229, 245, 246, 248–249, 255 Osteomyelitis, 168, 172, 185, 191–195, 202, 264
Mesenteric adenitis, 19, 20, 31, 36–37 signs and symptoms, 192
‘Mesenteric’ cysts, 225–227, 229 Ovarian cyst, 35, 36, 224–226, 229, 230, 288–289
Metatarsus adductus, 186–188 Ovarian teratomas (dermoids), 230
Micrognathia, 140, 142, 146–147, 270, 271
Micrognathia (Pierre-Robin syndrome), 142
Mixed chromosome DSD, 296–299 P
Mixed gonadal dysgenesis, 297–299 Pain
Mouth characteristics, 20, 24, 93
lesions in the floor, 141–144 location, 20, 22–24, 263
pathological conditions, 139 Papilloedema, 127–129, 138, 161, 163, 266
Mucus retention cyst, 139, 143–145 Paraphimosis, 63, 64, 66–68, 72
Murphy’s sign, 242 Parotid, 107, 108, 132, 135–138
Mycobacteria, 107, 111 Pedestrian injury, 151, 152, 160
Mycobacterium avium intracellulare scrofulaceum Pelvic, 11, 20, 21, 23–26, 32–35, 39, 169, 199, 204, 212,
(MAIS), 107, 111, 112, 137 221, 223, 224, 227–230, 251, 253, 261, 283,
Myelomeningocele, 186, 202, 234, 245–250, 255 302
Penetrating spike injury, 169–170
Penis
N circumcised, 64, 69
Neck Percussion tenderness, 28, 31, 32, 34, 39, 193, 204, 206,
midline swellings, 116–121 289
Necrotizing enterocolitis, 232, 279–281, 285–287, Perforation
289, 290, 292 prenatal, 279, 285, 289
Neonatal bowel obstruction Perianal reflex, 253
causes, 279–284 Perineal injuries, 169–171, 183, 212
pathology, 279 differential diagnosis, 171
Neonatal examination, 51 Peritonitis, 13, 19, 20, 22–25, 27–32, 34, 35, 37–39, 58,
Neonatal jaundice 82, 94, 95, 155, 170, 180, 181, 232, 235–236,
assessment, 237–240 279, 280, 284, 285, 289
Nephroblastoma, 223 Perthes’ disease, 195–197, 199, 200
Neural tube Peutz-Jeghers syndrome, 97, 99, 145
embryology, 245 Peyer’s patches, 36, 92–94
Neuroblastoma, 107, 113, 116, 191, 211, 215, 217, 218, Phimosis, 63–66, 68, 72, 211–213
222, 224–226, 229, 241 Pierre-Robin syndrome, 139–142, 146, 270, 271
Neurogenic bladder, 6, 210–212, 214, 253, 254 Pigeon chest, 257, 258
Neurological assessment, 161–164, 200 Pinna, 13–15, 134–137
Neurological development, 16–17, 209 Plagiocephaly, 103, 105, 123, 131
Normal sexual development, 293–294 cause, 105
Pneumothorax, 13, 157–159, 276
Poland syndrome, 257, 259, 260, 265
O Polydactyly, 11, 187, 202
Obstruction Polyp, 91–92, 97, 145, 213, 234–236
presentation of bowel, 284–288 Portal hypertension, 26, 82, 236, 238, 240–242
Index 309
Posterior urethral valve, 78–79, 208, 211, 212 Semimembranosus bursa, 201–202
Pre-auricular sinuses, 132, 134, 135 Sensory deficit
Precocious puberty assessment, 168, 250–251
examination, 264, 266, 267 Septic arthritis, 24, 191–195, 202
Premature thelarche, 264, 265 Sexual abuse, 169–172, 182
Problem-oriented, 1, 2, 285 Shock, 31, 95, 99, 154, 155, 158, 159, 161–163,
Pseudohaematuria, 214 178–179, 182, 230, 231, 289
Pupils, 129, 161–163, 165, 166 Sialectasis, 107, 132, 136–138
fixed, dilated, 162, 165, 183 Skull fracture, 165, 167
Pyelonephritis, 205, 211, 213 Slipped upper femoral epiphysis, 195, 197
Pyloric stenosis, 26, 83–89 Small bowel atresia, 279, 281, 284
clinical features, 86 Smegma, 63–64, 66, 67
Pyloric ‘tumour’, 86–89 Sole creases, 14, 15
Spider naevus, 242
Spina bifida
Q neonatal assessment, 245–255
Quinsy, 139, 148–149 occulta, 212, 245, 253–255
Squint, 101–102, 260
Sternomastoid, 101–104, 107–110, 114, 115,
R 120, 121
Radius muscle, 101, 103, 107–110, 120, 121
subluxation, 175 tumour, 101, 103, 104, 107, 108
Raised intracranial pressure, 127, 129–130, 138, 160, Straddle injury, 169–171, 183
266 Subdural haematomata, 124, 163, 166
Ranula, 139, 141–144, 149 Submandibular duct calculus, 107, 143
Rebound tenderness, 31–33, 39 Suprapubic
Rectal examination, 20, 23, 30, 32–34, 72, 95, 170, aspiration, 203, 204, 207
227, 229, 230, 232, 234, 235, 289, 300, Swellings
301, 303 midline neck, 116–121
Rectal prolapse, 98, 234, 235, 251, 252 Swollen penis
Referred pain, 23, 24, 50–51 red, 66–68, 72
Respiratory distress, 13, 142, 146–147, 157–159, 179, Syndactyly, 187, 202, 259
269–277, 287 Syndrome, 11, 12, 18, 38, 59, 79, 96, 97, 99, 136, 138–142,
progressive, 274–276 145, 146, 149, 173, 187, 202, 211, 213, 254,
signs, 269, 270 257–260, 265, 270, 271, 288, 289, 296, 297
Retina
examination, 127
Retractile testis T
criteria, 44 Talipes equinovarus, 186–189
Retroperitoneal haematomata, 19, 38, 155 Tenderness, 13, 24, 28–37, 39, 50–52, 54, 55, 61, 80–82,
Rigidity 95, 107–109, 111, 116, 144, 148, 154–156,
decerebrate (extensor), 163 170, 173–176, 191–195, 197, 198, 200, 202,
decorticate, 163 204–206, 214, 216, 218, 219, 222, 227, 229,
231–233, 235, 236, 242, 243, 263, 265, 267,
289, 290
S Tension pneumothorax, 157, 158, 276
Salivary duct calculus, 143–144 Testicular descent
Sausage-shaped mass, 95, 98, 216 embryology, 41
Scaphocephaly, 131 Testicular tumour, 53, 55
Scheuermann’s disease, 262–264 Testis
Scoliosis, 104, 106, 204, 205, 257–262, 268 dysplastic testes, 296–298, 301
causes, 204, 259, 260, 268 impalpable, 42, 48–49, 61, 72, 297–299
clinical approach, 262 Thomas’ test, 198, 200, 201
Scrotal development, 16 Thoracic injuries, 156–158
Scrotum Thyroglossal cyst, 116–119, 121
acutely painful, 49–51 Thyroid, 108, 114, 116–118, 120–121, 139, 141, 142,
examination, 45 149, 151, 238, 239
physical examination, 45, 51–53, 55 Tongue-tie, 139, 140, 149
swollen, 50, 51, 53–55 Tonsillitis, 24, 115, 139, 141, 147–148
310 Index
Torsion V
referred pain, 51 VACTERL, 291
Torticollis, 101–103, 106–108, 121, 123, 131, VACTERL association, 11, 274, 289, 291
132, 259, 260, 262 Vaginal atresia, 302, 303
differential diagnosis, 101, 102, 136 Varicocele, 53–55, 215, 221, 223
Torticollis (wry-neck), 101–105 Vascular malformations, 107, 113, 114, 121, 139, 245
Trigger thumb, 187, 202 Ventriculoperitoneal shunt, 127, 129–130
Turner syndrome, 296 assessing, 129–130
Virchow’s node, 116
Virilization
U degree of, 72, 294, 300, 301
Umbilical hernia, 13, 76, 80–82, 241 Visual clues, 4, 5, 7
Umbilicus Visual field deficit
discharge of urine, 78 examination, 266
discharging faeces, 78, 82 Vitelline duct, 75–77
mucus discharge, 78 Volvulus, 20, 24–26, 80, 232, 233, 235–237, 279,
spreading cellulitis, 81–82 281–282, 284, 288, 292
Undescended testis, 42–48, 51, 55–56, 61, 70, 72, 297, 298 malrotation, 24–26, 233, 235, 236, 238, 279,
landmarks, 42–44 281–282, 285–288, 292
Upper lip frenulum, 144–145 midgut, 24, 25, 232, 288
Urachal malformations, 77 Vomiting
Urachus, 76–79, 222 non-bile-stained, 83–89
Ureter Vomitus
ectopic ureter, 211, 212, 214 cause, 26, 85, 286
Urethral obstruction, 65, 79, 208, 213 coffee-ground, 85, 231
Urinary
extravasation, 156, 169, 170, 183
tract damage, 156 W
tract infection, 19, 38, 52, 84, 203, 204, 208, Weber’s test, 135
209, 213, 214 Weeping umbilicus, 76–78, 82
presentation, 203 Wetting
Urine causes, 209–212, 214
bag specimen, 207–208 Wilms’ tumour, 211, 213–217, 219–228, 241
collecting a specimen, 205–209 Wry-neck
midstream, 204, 206, 208, 210 causes, 101