Imaging of Congenital Anomalies of the Gastrointestinal

Tract
Arun Kumar G u p t a and B h u v n e s h G u g l a n i

Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India.

A b s t r a c t . The radiological imaging plays a vital role in the evaluatin of patients with congenital anomalies of the
gastrointestinal tract. The evaluation of these patients, most of which present early after birth, frequently requires the use of
various imaging modalities for making the correct diagnosis and planning surgical correction. This article reviews the common
congenital anomalies of the gastrointestinal tract including obstructive lesions, anomalies of rotation and fixation, anorectal
anomalies, and intestinal duplications. The plain radiograph is often diagnostic in neonates with complete gastric of upper
intestinal obstruction and further radiologic evaluation may be unnecessary. An upper gastrointestinal series should be
performed in all patients with incomplete intestinal obstruction. Sonography is useful in the evaluation of many congenital
anomalies affecting pediatric gastrointestinal tract especially hypertrophic pyloric stenosis, enteric duplication cysts, midgut
malrotation, meconium ileus and meconium peritonitis. Moreover, CT and MRI has assumed a greater importance as these
provide excellent anatomic details which may be necessary for correct diagnosis as well as treatment planning. This is
particularly true in evaluation of congenital anomalies such as esophageal/enteric duplications, vascular rings and anorectal
anomalies. It is important to be familiar with the role nad usefulness of the various imaging modalities so that these can be used
judiciously to avoid unnecessary radiation exposure while minimizing the patient discomfort.
[Indian J Pediatr 2005; 72 (5) : 403-414]

Key words : GIT; Congenital anomalies; Intestinal abnormalities

A wide spectrum of congenital anomalies m a y affect
gastrointestinal tract, some of which manifest early after
birth while others may not present till late childhood or
a d u l t h o o d . Imaging plays a very useful role in these
d e v e l o p m e n t a l lesions, most of which p r e s e n t with
o b s t r u c t i o n . In m o s t cases, plain r a d i o g r a p h y ,
gastrointestinal contrast study or Ultrasound (US) suffice.
C o m p u t e d t o m o g r a p h y (CT) and Magnetic Resonance
Imaging (MRI) are increasingly proving more useful to
provide an accurate diagnosis specially in difficult cases.
Developmental lesions of the neonatal gastrointestinal
tract can be grouped as follows: 1
Structural
Attributed to embryologic maldevelopment
9 Esophageal atresia with or without fistula
9 Antro-pyloric atresia
9 Antral diaphragm
9 Duodenal atresia
9 Duodenal stenosis
Intrinsic: windsock duodenum
Extrinsic: annular pancreas
9 Midgut malrotation with peritoneal bands
9 Duplication or mesenteric cyst
9 Anorectal atresia
Correspondence and Reprint requests : Dr. Arun Kumar Gupta,
Professor, Department of Radiodiagnosis, All India Institute of
Medical Sciences,New Delhi-110029.Fax : 91-26588641,26588663
Attributed to in utero vascular (ischemic) complication
9 Jejuno-ileal atresia
9 Colonic atresia or stenosis
9 Complicated meconium ileus
Functional
9 Meconium plug syndrome and its variants
9 Megacystis-microcolon-intestinal hypoperistalsis
Structural and Functional Combined
9 Hypertrophic pyloric stenosis
9 Midgut vovulus (complicating midgut
malrotation)
9 Uncomplicated meconium ileus
9 Colonic aganglionosis
In a n o r m a l n e o n a t e , s w a l l o w i n g b e g i n s a l m o s t
immediately after birth and gas should be present in the
stomach within few minutes. 3Within 3 hours of birth, the
entire small bowel usually contains gas while the sigmoid
colon is seen only after 8-9 hours. 4 Disruption of this
c o m m o n pattern is seen in obstruction or presence of
underlying illness such as brain damage, septicemia or
hypoglycemia.
Plain r a d i o g r a p h is a useful, s i m p l e and m o s t
inexpensive tool in the evaluation of the neonate with
gastrointestinal (GI) obstruction. Unlike adults and older
children, in neonates the small and large bowel usually

Indian Journal of Pediatrics, Volume 72--May, 2005 403

 Arun Kumar Gupta and Bhuvnesh Guglani
cannot be distinguished. The gas is distributed
throughout the small and large bowel where a little fluid
is present resulting in sharp bowel-air interfaces which
appear as multiple closely a p p o s e d r o u n d e d or
polyhedral structures on plain radiograph. 2 The precise
level of obstruction may be evident in high GI obstruction
but is difficult to determine in a low gut obstruction. High
GI obstruction occurs most commonly at the level of
duodenum and proximal jejunum and plain radiography
alone is often diagnostic.
Bilious vomiting indicates obstruction distal to the
ampulla of Vater. Although there is little role of contrast
examintion in high gut obstruction, 5 GI contrast
examination should be performed in obstructions
presenting beyond first few days of life to rule out
malrotation and midgut volvulus. The stomach is
emptied through a nasog'astric tube before upper GI
contrast study is undertaken.
Barium suspension is not used in cases of suspected
perforation or if there is a risk of barium inspissations.
Aspiration into lungs shoufd be avoided while using
contrast agents because commonly used high osmolality
ionic contrast may produce severe pulmonary edema.
Hypertonic water-soluble ionic contrast media may be
useful in relieving obstruction in meconium ileus by
drawing water into the bowel lumen. However, this may
cause fluid and electrolyte imbalance. Therefore, infant
should be hydrated and serum electrolytes monitored
before the procedure. Non-ionic low osmolality contrast
media are preferred in most circumstances. Barium is
preferred in cases of suspected Hirschsprung's disease or
other conditions where delayed films have diagnostic
value.
High Intestinal Obstruction: The usual presentation is
vomiting which may be bile stained if obstruction is distal
to ampulla of Vater. The plain radiograph is often
diagnostic and further radiological evaluation may be
unnecessary. However, an upper gastrointestinal series
must be performed in all patients with incomplete
intestinal obstruction. The causes of high obstruction
include pyloric atresia, duodenal atresia, malrotation with
midgut volvulus or Ladd's bands and proximal jejunal
atresia. 2 Partial obstruction results from jejunal stenosis,
peritoneal bands, duplication cyst, malrotation and
Meckel's diverticulum. 4
Low Intestinal Obstruction: Failure to pass meconium in
the first 24-48 hours of life may be due to structural or
functional reasons. The causes include ileal or colon
atresia, Anorectal malformations, Hirschsprung's disease,
meconium plug syndrome, and neonatal small left colon
syndrome.
Ultrasonography (US) is often the first modality to be
used in investigation of child with abdominal lump or
suspected hypertrophic pyloric stenosis. US is highly
accurate in the diagnosis of hypertrophic pyloric stenosis
and extremely useful in the investigation of mass lesions
such as enteric duplication cysts and mesenteric or
404
omental cysts}
US is the modality of choice for prenatal screening, but
occasionally additional imaging information is needed. 6
MRI is being increasingly used for prenatal imaging of
congenital anomalies. The use of fast sequences like single
shot fast spin echo and echoplanar imaging has enabled
successful prenatal MR imaging. Esophageal, duodenal,
or small bowel atresia can be diagnosed on antenatal MRI.
On MRI, the signal intensity differences of the dilated
bowel may provide additional information comple-
mentary to the US in identifying the site of obstruction.
The proximal small bowel (fluid content) appears
hyperintense on single-shot fast spin echo (SSFSE) and
hypointense on T l - w e i g h t e d fast spin echo (FSE)
imaging. In contrast, the distal small bowel and colon
appear hypointense on SSFSE and hyperintense on T1-
weighted FSE imaging due to presence of meconium. 7~
ESOPHAGUS
Esophageal Atresia (EA) and Tracheo-Esophageal
fistula (TEF)
The presence of polyhydramnios, reduced intrahiminal
liquid in the fetal gut and inability to detect the fetal
stomach on prenatal ultrasound may provide an early
clue to the possibility of esophageal atresia. 9
The chest radiographs (AP and Lateral) demonstrate
proximal esophageal pouch filled with air. The abdomen
is always included in the radiograph to look for air in the
gastrointestinal tract. The absence of gas in stomach or
intestinal tract is suggestive of pure esophageal atresia
(EA) without fistula or EA with proximal tracheo-
esophageal fistula. The position of aortic arch should be
conveyed to the surgeon as it may help determine the
surgical approach. When plain radiographs fail to
indicate the side of the arch, and as a preoperative work-
up, echocardiography should be performed before
surgery to prognosticate and plan successful outcome} ~
Chest radiograph may show consolidation due to
aspiration pneumonia especially in the dependant upper
lobes in supine position.
Radiographically, there is inability to pass a red rubber
catheter into the stomach. The proximal pouch may be
outlined due to swallowed air (Fig la). Gentle injection of
air via the tube may distend the proximal pouch. The
distance between proximal and distal esophageal pouch
can be assessed only after a gastrostomy in a staged
procedure. Isolated long gap esophageal atresia is known
to be associated with 13 pair of ribs} 2
The diagnosis is frequently delayed in H-type fistula.
The fistula is more precisely termed as N-type fistula as it
courses obliquely from the esophagus upwards to the
trachea (Fig lb). Most H-fistulas are at thoracic inlet (T2-
3). Tube esophagogram under fluoroscopic guidance with
patient in the lateral or steep prone oblique position is the
best way to demonstrate H-type fistula. The nasogastric
tube placed at GE junction is gradually withdrawn while
Indian Journal of Pediatrics, Volume 72--May, 2005
 Imaging of Congenital Anomalies of the Gastrointestinal Tract
Fig la. Oesophageal Atresia with distal Tracheosophageal fistula.
Lateral radiograph shows air filled dilated proximal
oesophageal pouch. The presence of air in the stomach and
the bowel indicate distal tracheosophageal fistula.
injecting contrast at various levels? ~ A small amount of
isotonic non-ionic contrast medium or properly diluted
barium should be used. The contrast should be aspirated
back immediately after the study.
Esophageal stenosis and webs may be associated with
tracheoesophageal fistula? 3,14Up to one quarter of patients
w i t h EA & TEF h a v e associated w i t h GI a n o m a l i e s
including pyloric stenosis, duodenal/small bowel atresia,
anorectal malformations. There should be high index of
suspicion for associated anomalies. The pattern of air
distribution on plain radiographs may provide clue to
these anomalies. Air confined to the stomach and or
d u o d e n u m suggests associated duodenal atresia. Follow
Indian Journal of Pediatrics, Volume 72--May, 2005
Fig lb. H-type tracheoesophageal fistula. The contrast study
demonstrates the superiorly angulated fistula (arrow) from
the oesophagus to the trachea.
up radiographs may be obtained if initial findings are
suspicious.
Associated anomalies are f r e q u e n t l y seen in such
individuals including features of the VACTREL spectrum
(vertebral anomalies, anorectal malformation,
cardiovascular malformation, tracheo-esophageal fistula
w i t h e s o p h a g e a l atresia, renal a n o m a l i e s a n d limb
defects). The other recognized associations are trisomy 18,
trisomy 21, CHARGE syndrome, Potter syndrome and
presence of 13 pair of ribs. 3,15
The dilatation of upper pouch may persist for months
f o l l o w i n g r e p a i r of atresia. P a t i e n t s w i t h r e p a i r e d
esophageal atresia show a high incidence of abnormal
peristalsis in the e s o p h a g u s , w h i c h m a y cause
d y s p h a g i a . 3,16 A n a s t o m o t i c s t r i c t u r e s a n d gastro-
esophageal reflux occur c o m m o n l y after EA and TEF
repair. The incidence of stricture increases in patients with
long gap EA with a delayed primary repair 17 and reflux.
STOMACH
Gastric Atresia, Antro-pyloric Membranes: Congenital
gastric o b s t r u c t i o n is rare~ Isolated pyloric atresia,
p y l o r i c / p r e p y l o r i c m e m b r a n e , or antral webs are
uncommon causes of gastric obstruction in the newborn.
Pyloric atresia is classified into three types: (a) complete
atresia with no connection b e t w e e n the stomach and
d u o d e n u m , (b) complete atresia with a fibrous b a n d
405
 Arun Kumar Gupta and Bhuvnesh Guglani

connecting the stomach and duodenum, (c) and a gastric

membrane or diaphragm2 8,19The congenital antro-pyloric
webs can be diagnosed on UGI contrast study where
they are seen as linear defects resulting in' pseudo double
bulb' appearance, as the inflowing barium outlines the
space between the antrum and pylorus first and then the
duodenal bulb. 2~The membrane may be detected on
sonography if the stomach is filled with clear fluid.
Congenital Hypertrophic Pyloric Stenosis (CHPS):
CHPS is characterized by hypertrophy of the circular
muscles. Recent work suggests that impaired neuronal
nitric oxide synthase synthesis in the myenteric plexus is
an important contributing factor in the pathogenesis of
infantile hypertrophic pyloric stenosis as well as of
achalasia, diabetic gastroparesis, Hirschsprung's disease,
and Chaga's disease. 21 The mainstay of diagnosis remains
palpation of the hypertrophied pylorus in a quietly
feeding baby. Ultrasonography is the primary imaging
method, if required, and has replaced contrast evaluation.
There is controversy over exact measurements of the
Fig 2b. Hypertrophicpyloric stenosis (HPS): On tranverse view,
gastric outlet in normal and hypertrophic pylorus. The hypertrophied pylorus has a doughnut appearance. The
thickness of the muscle is the most discriminating and mucosa is echogenicwith surroundinghypoechoicmuscle
accurate criterion for the diagnosis of hypertrophic layer (betweencursors).
pyloric stenosis. 22
Ultrasonographically the antro-pyloric muscle of each pylorospasm may overlap those accepted as positive for
patient is measured in the midlongitudinal plane (Fig 2a). hypertrophic pyloric stenosis. In pylorospasm, there is
The stomach is usually well distended allowing easy considerable variability in measurement or image
identification of the antro-pyloric region. The water or appearance with time during the s t u d y . 24 This is an
glucose solution may be given in cases of inadequate important clue for diagnosing pylorospasm. The
distention but should be removed after examination to sonographic double-track sign can be seen in cases of
prevent vomiting and aspiration. The antro-pyloric pylorospasm as well as hypertrophic pyloric stenosis. It is
muscle less than 2 mm thick should be considered not pathognomonic for hypertrophic pyloric stenosisY
unequivocally normal. 23The muscle thickness of 3 mm or
A barium study may be performed if ultrasound is
more is abnormal (Fig 2b) and diagnostic for pyloric inconclusive or if gastro-oesophageal reflux is suspected
stenosis, whereas muscle thickness from 2mm to less than (Fig 2c). The hypertrophied muscle mass causes
3 mm is abnormal but not specifically diagnostic for elongation and narrowing of pyloric canal ('string sign')
pyloric stenosis. 24 The sonographic measurements in as well as a bulge in the distal antrum with streak of

Fig 2c. Hypertrophicpyloricstenosis(HPS):Upper GIbariumstudy

Fig 2a. Hypertrophicpyloricstenosis (HPS):Longitudinalview on shows narrowed and elongated pyloric canal. The
sonographyshows elongatedpyloriccanal w~h thickened hypertrophied muscle mass produces indentation on the
hypoechoeicmuscle (arrow). duodenal cap and the antrum.

406 Indian Journal of Pediatrics, Volume 72--May, 2005

 Imaging of Congenital Anomalies of the Gastrointestinal Tract

barium pointing towards pyloric channel ('beak sign').
The b a r i u m m a y outline c r o w d e d mucosal folds as
parallel lines ('double/triple track sign'). These findings,
if transient, should be interpreted with caution. The
i m p o r t a n c e of clinical e x a m i n a t i o n and test feed is
e m p h a s i z e d especially in cases w i t h equivocal
sonographic or barium findings. This will reduce the risk
of false-positive diagnoses and negative laparotomies. 2~
DUODENUM
Duodenal Atresia/Stenoses
Complete duodenal obstruction is much more common
than congenital gastric obstruction. Vomiting is usually
bilious as obstruction is often distal to the ampulla of
Vater. The plain r a d i o g r a p h of the a b d o m e n shows
'double bubble' sign corresponding to air in stomach and
dilated proximal duodenum with absence of air in distal
gastrointestinal tract. This condition may be diagnosed
with antenatal ultrasound showing fluid filled double-
bubble and polyhydramnios. Fetal karyotype should be
o b t a i n e d as up to 30% of patients have D o w n ' s
syndrome. 9 There is a frequent association with other
a n o m a l i e s in up to 50% of cases. Partial d u o d e n a l
obstruction is caused by annular pancreas, Ladd's bands,
m i d g u t volvulus, p r e - d u o d e n a l portal vein and
duplication cyst.
A pre-duodenal portal vein (persistent left vitelline
vein) results from n o r m a l situs a s y m m e t r y , and is
c o m m o n l y f o u n d in patients w i t h h e t e r o t a x y . The
resultant portal vein courses anterior to the pancreas and
d u o d e n u m . The diagnosis is made by identifying the
prepancreatic course of the portal vein on sonography, CT
or MR imaging. It is now believed that in most cases of
duodenal obstruction associated with preduodenal portal
vein, the obstruction is due to a primary, obstructing
duodenal lesion such as intraluminal membrane or web
and such a lesion should be suspected in these patients if
duodenal obstruction is present. 2,27
Jejuno-ileal atresia results from vascular occlusion and
ischemic injury to the developing gut. Jejuno-ileal atresia
is classified into four types. Type l is a simple intraluminal
d i a p h r a g m c o m p o s e d of m u c o s a l and s u b m u c o s a l
elements. No interruption of the muscularis is present.
Type II represents an atresia with solid fibrous cord (all
the layers of intestinal wall interrupted) connecting the
blind ends of the bowel. Type III is an atresia with
complete separation of the blind ends and an associated
mesenteric defect. The familial form of multiple atresias is
considered as Type IV. 1~3o
The d i s p r o p o r t i o n a t e d i l a t a t i o n of the bowel
immediately proximal to the atresia results in a bulbous
c o n t o u r s u g g e s t i v e of c o n g e n i t a l small bowel
obstruction. 31Plain radiography is usually diagnostic and
further radiological "evaluation (upper GI series) is not
necessary (Fig. 3). In chses of partial obstruction little
amount of distal gas is usually present. A small amount of
air may be injected through a nasogastric tube to confirm
complete or partial jejunal obstruction. In isolated
proximal atresia of the duodenum or jejunum, the colon
is of normal size because the remaining bowel distal to
the atresia produces sufficient intestinal secretions to
produce a normal caliber colon? In ileal atresia, the colon
has a n o r m a l location b u t the caliber is r e d u c e d
(functional microcolon) 32. In low intestinal obstruction
contrast enema should be done to exclude colonic lesion,
meconium ileus or Hirschsprung's disease especially

SMALL BOWEL

Jejunal/ileal atresia
Intestinal atresias account for about one third of all cases
of n e o n a t a l intestinal obstruction. Prenatal
ultrasonography is more reliable in detection of duodenal
atresia than the more distal lesions. The survival rate has
improved significantly in most of the series with the
operative mortality being less than 1%. More distal the
atresia better is the survival. An increased mortality is
observed in multiple atresias, 'apple peel' syndrome, and
w h e n atresia is associated w i t h m e c o n i u m ileus,
meconium peritonitis and gastroschisis.28Intestinal atresia
occurs in approximately 10% to 20% of neonates with Fig 3. Small bowel atresia: Plain radiograph demonstratesmultiple
gastroschisis and may be missed at the initial closure if a dilated bowel loops and fluid levels.Peritonealcalcifications
thick 'peel' obscures the bowel. 29 are seen as a result of meconiurnperitonitis.

Indian Journal of Pediatrics, Volume 72--May, 2005 407

 Arun Kumar Gupta and Bhuvnesh Guglani
when dilated small bowel or colon cannot be
differentiated on plain radiography.4,10Sonography may
be useful in differentiating meconium ileus and ileal
atresia. In meconium ileus, the dilated bowel loops are
filled with echogenic material, whereas in ileal atresia the
bowel contents are echo-poor.33
The 'apple peel' s y n d r o m e is thought to follow
intrauterine occlusion of the distal superior mesenteric
artery (SMA) resulting in a proximal jejunal atresia with
absence of mid-small bowel and the dorsal mesentery.
The distal bowel derives its blood supply from the
proximal SMA. The distal small bowel spirals around its
single vascular supply and resembles an "apple peel. "34,35
Intestinal stenoses are much less common than
intestinal atresias. Stenoses result from three major
causes: external compression of the bowel lumen,
intramural narrowing resulting from rests of heterotopic
tissue, and incompletely perforated intraluninal webs. l~
Stenoses caused by extrinsic impression on the bowel
lumen are almost entirely confined to the duodenum as
a result of annular pancreas ~ or peritoneal bands.
Malrotation
In the fetal life, the primitive midgut herniates into the
extraembryonic celom and later returns into the
abdominal cavity between 6-10 weeks. During this
process, the primitive midgut rotates anticlockwise
through 270 degrees around the axis of the superior
mesenteric artery. Both, duodeno-jejunal junction and
caecal pole undergo 270 degree anti-clockwise rotation
followed by fixation of duodeno-jejunal junction in the
left upper quadrant and the ileo-caecal junction in the
right lower quadrant. Interruption of this process at any
point results in a narrow base of small bowel mesentery
with predisposition to volvulus.2 The term 'malrotation'
or 'Nonrotation' is a misnomer as it results from lack of
complete intestinal rotation The Ladd's bands or
hyperfixation bands can form due to faulty peritoneal
attachments as a result of malfixation and malrotation of
the intestine. Rotational abnormalities are seen in
approximately 70% of patients with situs ambiguous
(heterotaxy).
A neonate with bilious vomiting on the first day of life
and radiographic evidence of complete d u o d e n a l
obstruction does not require further radiological
evaluation. On the other hand a neonate with bilious
vomiting who has been normal for first few days or with
radiographic evidence of incomplete obstruction
(presence of gas in distal part of gut) requires further
evaluation.4 An upper gastrointestinal series is usually
performed initially to demonstrate the level and nature of
obstruction. On a supine radiograph the normal C- loop
crosses the midline and duodeno-jejunal junction lies to
the left of the left vertebral pedicles at the level of the
duodenal bulb. The normal duodeno-jejunal junction may
be mobile and easily displaceable in infancy, especially in
children less than 4 months of age. 37
408
Any duodenal obstruction that occurs after immediate
postnatal period should be assumed to be midgut
volvulus until proved otherwise. 38 The upper gastro-
intestinal examination shows a typical corkscrew appea-
rance of the duodenum and proximal jejunum (Fig 4). The
distal jejunum lies to the right of midline. The Ladd's
bands result in Z-shaped duodeno-jejunal configuration
and usually obstruct at the third portion of the duodenum
but can obstruct more distally.ByA barium meal should be
done for suspected malrotation since a normal barium
enema does not exclude malrotation. The position of
caecum may be normal in a significant number of patients
with malrotation. 2~The UGI examination remains the
gold standard for the diagnosis of malrotation
Fig 4. Midgutvolvulus:Upper GI barium study demonstratesthe
classic 'corkscrew' appearance of the duodenum and
proximaljejunum.
Superior mesenteric vein (SMV) normally lies to the
right of superior mesenteric artery (SMA). Malrotation
may be suggested on US if the SMV is to the left or
anterior to the artery. However, this is neither a sensitive
nor a specific sign. The sonographic "whirlpool sign" is
objective and a definite sign as volvulus, is shown at color
Doppler US as clockwise spiraling of the mesentery and
SMV around the SMA.~, 4~Recent literature is replete with
many diagnostic criteria for various imaging modalities.
The multiplicity of the findings is a testimony to the
unsatisfactory nature of their yield.
Meconium peritonitis
Meconium peritonitis results from an in-utero bowel
perforation proximal to a complete obstruction. Although
commonly associated with bowel atresias, it may be
present in asymptomatic neonates with in-utero sealed
bowel perforation. Plain abdominal radiograph may
Indian Journal of Pediatrics, Volume 72--May, 2005
 Imaging of Congenital Anomalies of the Gastrointestinal Tract

show peritoneal calcifications due to calcified meconium

(Fig 5). The small bowel loops may be dilated in cases of
associated bowel atreasia. These calcific densities may be
seen in the scrotum also if the processus vaginalis is
patent. On ultrasound, the calcifications are seen as
echogenic densities'outside the bowel wall.

Fig 6. Sonogram of a distal ileal duplication cyst. An anechoic

lesion with typical wall characteristics is seen-inner
echogenicmucosal stripe and outer hypoechoic muscle layer
(arrow).

length of the gut where they lie along the mesenteric

b o r d e r and share a c o m m o n muscle wall and blood
supply. The duplications commonly occur in the distal
ileum (Fig 7) and esophagus. Duplication cysts are usually
s p h e r i c a l lesions and less often t u b u l a r . The
communication with the adjacent bowel is uncommon in
spherical lesions and is more likely to occur with tubular
duplications. 43

Fig 5. Meeonium ileus: plain radiograph shows multiple dilated

bowel loops (low intestinal obstruction) and 'soap bubble'
appearance in the right lower quadrant.

Meconium ileus
Meconium ileus is a low intestinal obstruction caused by
inspissated meconium usually in the terminal ileum and
is almost always associated with cystic fibrosis. The plain
abdominal radiograph usually shows low small bowel
obstruction with dilated small bowel loops but absent or
scant fluid levels. Although the absence of air-fluid levels
strongly suggests meconium ileus, the presence of air
fluid levels does not e x c l u d e it as some cases do
d e m o n s t r a t e air-fluid levels, e s p e c i a l l y those w i t h
associated complications such as volvulus and stenosis or
atresia.lS,42Volvulus of a meconium laden heavy bowel
loop is c o m m o n and can lead to intestinal stenoses,
atresias, gangrene and perforation. The "soap bubble"
appearance caused by admixture of air and meconium is Fig 7. Hirschsprung's disease (recto-sigmoid): Contrast enema
frequently seen (Fig 6). It can, however, also be seen with (lateral radiograph) shows abnormal rectosigmoid index and
ileal atresia, colon atresia, Hirschsprung's disease and the transition zone with narrow caliber of the rectosigmoid and
meconium plug syndrome. TMCalcifications or pseudocyst dilated proximal ganglionated sigmoid colon. The extensive
may be seen due to meconium peritonitis and localized ulcerations are due to enterocolitis.
ascitis Ultrasound is the imaging modality of choice for the
Enteric Duplication Cyst evaluation of an abdominal mass in the neonate. The most
common clinical manifestation of intra-abdominal enteric
Enteric duplication cysts can occur anywhere along the duplication cysts is intestinal obstruction. Occasionally,

Indian Journal of Pediatrics, Volume 72--May, 2005 409

 Arun Kumar Gupta and Bhuvnesh Guglani
haemorrhage or gastrointestinal bleeding may occur due
to ectopic gastric mucosa which is seen in 10-20% of cases
which makes it visible with technetium-99m
pertechnetate scanning and may be of diagnostic value.
The cyst shows the echogenic inner rim of mucosa and
hypoechoic outer rim of the muscle layer (double wall
sign). This is most easily identified in the dependent
portion of the cyst. 44Peristalsis of the cyst wall when
visible, is another useful sign.45The cyst is fluid filled,
usually unilocular and anechoic. Occasionally, internal
echoes and septations may be seen due to hemorrhage or
inspissated mucoid material. Rarely, the cyst is
completely echogenic and gives false impression of a
solid lesion. Multiple duplication cysts may be present in
15-20% of cases. 46
Esophageal duplication cyst: duplications of the
esophagus are the second most common duplication of
the gastrointestinal tract after ileal duplications. 43,47An
esophageal duplication cyst may present with pressure
symptoms such as dysphagea, stridor, or breathing
difficulty. On chest radiograph, duplication cyst is seen as
posterior mediastinal mass. Ultrasound may demonstrate
double-layered wall especially in large cysts abutting
posterior chest wall. CT and MRI demonstrate well
delineated outlines with non-enhancing attenuation
values of water. Endoscopic US can accurately diagnose
duplication cyst by demonstrating contiguity of the
muscularis propria of the esophagus with the muscle
layer of the cyst wall.~
Duodenal duplication cyst is a rare congenital anomaly
and usually presents with symptoms of obstruction, but
may cause biliary obstruction and pancreatitis. 49The
differential diagnosis of duodenal duplication includes a
choledochocele, a pancreatic pseudo.cyst and intralurninal
dlverticulum.5~
Mesenteric Cyst (Lymphangioma)
Mesenteric cyst is a congenital malformation arising due
to sequestration of lymphatic vessels. 51These are usually
seen in the mesentery and less often in omentum and
retroperitoneum. Children usually present in the first
decade, with increasing abdominal girth or a palpable
abdominal mass. Sonography reveals a thin-walled
unilocular or multilocular cystic lesion, whereas CT and
MRI demonstrate variable characteristics of the cyst
contents (usually water-to fat) depending upon whether
fluid is chylous, infected or haemorrhagic. Ultrasound is
particularly useful to demonstrate the thin septations
which may not be well seen on CT. Rarely, a mesenteric
lymphangioma may contain calcification mimicking a
mesenteric teratoma. 52
LARGE BOWEL
Colonic Obstruction
Colonic atresia is quite rare and often indistinguishable
410
from obstruction of the distal ileum. The right colon is
most commonly affected. 53 Plain radiograph
demonstrates features of a low intestinal obstruction with
air-fluid levels or mottled pattern due to retained
meconium. Contrast enema shows a distal microcolon
and complete obstruction at the level of atresia. On US,
dilatation of the distal small bowel and proximal colon
with echogenic contents due to retained meconium may
be seen. 4
Hirschsprung's Disease
Approximately one-fifth of neonatal intestinal
obstructions are due to Hirschsprung's disease.2In about
three quarters of these cases, the area of aganglionosis is
limited to the rectum and sigmoid (short segment
disease). 54 Long segment disease involves a variable
portion of the colon proximal to the sigmoid and in total
aganglionosis coli the entire colon and a part of the
terminal ileum2~ is involved. Total colonic aganglionosis
and 'ultra short segment' disease is rare.
The majority of patients present within the first 6
weeks in life. Neonates present with failure to pass
meconium, abdominal distention, vomiting or
enterocolitis. Enterocolitis is the leading cause of death in
Hirschsprung's disease. 55Enterocolitis and perforation is
more common in the long segment disease. The
perforation occurs most commonly in the ascending colon
or in the appendixfi6,s7
Imaging Features: Plain radiograph shows features of
low bowel obstruction with colonic dilatation out of
proportion to the small bowelY There may be absence of
rectal gas or small amount of gas may be seen in rectum
(especially on prone films) on plain radiograph. However,
the absence of rectal gas is not specific for Hirschsprung's
disease, being more commonly seen in infants with sepsis
and necrotizing enterocolitis. 59
Digital examination of the rectum or enemas,in
patients scheduled for barium studies should not be done
as it may distort the findings.1~For the same reason, the
study (enema) is never performed with an inflated Foley
catheter in the rectum. TM Barium enema is performed
carefully on an unprepared patient by inserting a straight-
tipped catheter to a point just beyond the anal sphincter.
The patient should be placed in lateral position and
barium infused slowly as rapid infusion can distend and
mask the transition zone. The contrast agent should be
prepared with normal saline to avoid possibility of water
absorption from the large surface area of dilated colon?
The diagnostic feature in short segment disease is funnel
shaped transition zone and reversal of the recto-sigmoid
ratio (Fig 7). Normally, the rectum is the most distensible
portion of the bowel and has a diameter greater than that
of the sigmoid colon (recto: sigmoid ratio >1). 2~The
radiological transition zone is commonly found distal to
the pathological transition zone.6~There may be irregular,
uncoordinated contractions in the aganglionic segment
(20% of cases) which may be mistaken for mucosal
Indian Journal of Pediatrics, Volume 72--May, 2005
 Imaging of Congenital Anomalies of the Gastrointestinal Tract

ulcerations. Delayed films after 24 hrs may demonstrate

prolonged retention of barium in the sigmoid colon (not
rectum which normally holds barium for 24 hours). This
is a strong indicator of Hirschsprung's disease, even when
the enema findings have been inconclusive. Rectal biopsy
is always necessary for confirmation.
The diagnosis of total colonic aganglionosis is much
more difficult. The radiological findings viz.
foreshortening, rounding of the colonic flexures and small
caliber colon2are relatively nonspecific. The prolonged
retention of barium on delayed films should alert one to
suspect the diagnosis. 61Presence of skip lesions in
Hirschsprung's disease is extremely rare (controversial).
Awareness of this variant aids in the interpretation of the
barium enema in children with signs and symptoms of
aganglionosis, especially when the rectal biopsy is
normal. ~2The associations of Hirschsprung's disease
include Down's syndrome, ileal and colon atresia and
neuro-cristopathies.~
Anorectal A n o m a l i e s
Fig 8. Lateralviewof distalcologramshowsrectourethralfistulain
The anorectal malformations are divided into high and a maleinfantwitha highanorectalmalformation.Associated
low types depending upon whether the termination of sacral dysgenesisis seen.
h i n d g u t is above or below the puborectalis sling.
Anorectal anomalies are often associated with fistulous
determining the level of atresia. 68The use of invertogram
tracts opening into the genitourinary system. Although
is to be discouraged as it causes unnecessary stress.
the distinction between high and low lesions can be made
During invertogram, the baby usually keeps crying
clinically, the radiologic study is required to evaluate the
causing contraction of puborectalis sling and obliteration
internal anatomy. This is usually accomplished using
of the lower rectum. Also, the rectum may be pulled in the
contrast studies. In both male and female infants with low
cephalic direction resulting in error of classification. In
lesions, there is usually a visible perineal opening and the
those anomalies, which are associated with fistulae with
communication with the genitourinary tract is absent 1~176
the urinary or genital tract, the fistula becomes the highest
However, there are cases with imperforate anus with
point of rectum in an invertogram and gas may escape
external signs of a low lesion with demonstrable fistula
resulting in less distension of the rectum. In prone
into the anterior urethra either at surgery or by
position, the fistula is lowest and therefore rectum is
radiographic studies. 66These cases should be considered
better distended causing adequate delineation of the
an intermediate form of imperforate anus with external
rectal gas 6s. The pubococcygeal line and M line are
signs of a low lesion but with fistula characteristically to
imaginary lines used to represent the level of puborectalis
the membranous or bulbous urethra. 65High lesions are
sling on lateral radiographs. The "M" line is a better
treated with initial diverting colostomy and definitive
anatomic correlate of puborectalis sling. The "M" line
repair at a later age. A pressure augmented distal
passes horizontally through the junction of the lower third
colostogram is recommended prior to definitive repair,
and upper two thirds of the ischium.
both to confirm the level of rectal atresia and to define any
Ultrasonography can assess the distance of the rectal
associated fistulous communication. 67 Water- soluble
pouch in relation to the perineal surface. A pouch perineal
contrast medium is injected under mild pressure through
distance of < 1.5 cm is indicative of low anomalies, while
a Foley's catheter with its balloon inflated to occlude the
a pouch that terminates above the base of the bladder
stoma. The fistulous tract not identified on the
(superior extent of urogenital diaphragm) is indicative of
conventional contrast colostogram (Fig 8) may be
a high lesion. 69However, the distinction between high
delineated when an augmented-pressure modification of
and intermediate anomaly cannot be made out with this
the technique is utilized. 66Micturating cystourethro-
technique. Recently, anal endosonography has been used
graphy (MCU) and/or retrograde urethrography (RGU)
as an accurate alternative to MRI in the assessment of
are also useful to demonstrate fistula and presence of
anorectal malformations after repair, y~
vesicoureteric reflux.
CT and MRI are the modalities of choice to delineate
The easily available plain radiograghy is simple and is
pelvic anatomy including puborectalis muscle and
often the preliminary investigation to be used in
external sphincter. Congenital anorectal malformations
evaluation of these patients. Prone cross table lateral view
are found in many forms, and are frequently associated
with babies kept in genu-pectoral position is useful in

Indian Journal of Pediatrics, Volume 72--May, 2005 411

 Arun Kumar Gupta and Bhuvnesh Guglani
with other anomalies, especially of the spine, spinal cord,
and urogenital system. 71 Magnetic resonance imaging
has proven to be the single stop modality to answer all the
crucial questions such as level and type of malformation,
t y p e of fistula, d e v e l o p m e n t a l state of the s p h i n c t e r
m u s c l e c o m p l e x , a n d the p r e s e n c e of a s s o c i a t e d
anomalies. 71-7~MRI has contributed to a better insight in
the m o r p h o l o g y a n d p a t h o g e n e s i s of such c o m p l e x
congenital malformations.YL
Spinal r a d i o g r a p h s m u s t be examined carefully for
abnormalities, because spinal pathology has profound
effect on the o u t c o m e . N o r m a l r a d i o g r a p h i c a n d
sonographic appearance of spinal a n a t o m y in children
with anorectal malformation makes MRI superfluous, but
if r a d i o g r a p h s or u l t r a s o u n d are u n i n f o r m a t i v e /
a b n o r m a l , MRI s h o u l d be u s e d to a c c u r a t e l y d e p i c t
possible intraspinal pathology. TM S o n o g r a p h y of the
urinary tract for associated renal anomalies is essential in
all patients with anorectal malformation.
Megacystis-Microcolon-Intestinal Hypoperistalsis
Syndrome (Berdon Syndrome) 7s
This s y n d r o m e is a rare congenital cause of intestinal
obstruction. The abdominal distention is due to dilated
bladder and dilated short bowel in absence of organic
obstruction. There is hypomotility of small bowel with
retrograde peristalsis on upper GI contrast study while an
e n e m a s h o w s a microcolon. U l t r a s o u n d will s h o w a
dilated bladder and bilateral hydrourteronephrosis. The
anterior abdominal wall musculature is thin, similar to
prune belly syndrome.
Meconium Plug Syndrome
I m m a t u r e left colon is a form of functional obstruction
associated with p r e m a t u r i t y , maternal d r u g ingestion
(magnesium preparations or high doses of sedatives),and
in babies of diabetic mothers. 4,76 The affected infants
p r e s e n t w i t h distal b o w e l o b s t r u c t i o n a n d d e l a y e d
meconium passage. However, the bowel distention is less
severe than with a mechanical obstruction. The contrast
enema typically shows a mildly dilated right and
transverse colon and a small left colon with an abrupt
transition at the level of splenic flexure. The meconium
plugs are seen as multiple elongated filling defects in
dilated colon. The contrast enema using water-soluble
contrast media is both diagnostic as well as therapeutic. If
clinical improvement does not follow over next few hours
or days, suction rectal biopsy is recommended to rule out
Hirschsprung's disease.
CONCI~USION
The goal of imaging is to provide an accurate diagnosis
and other r e l e v a n t i n f o r m a t i o n to the clinician while
minimizing patient discomfort and radiation exposure.
The j u d i c i o u s choice of v a r i o u s i m a g i n g m o d a l i t i e s
r e q u i r e s a c o n c e r t e d effort of the clinician a n d the
412
radiologist aimed at providing best information and help
to the treating clinician to p l a n out the m o s t suitable
treatment efficaciously.
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Book Review

Hyperalgesia: Molecular Mechanism and Clinical Implications

I read the book "Hyperalgesia: Molecular Mechanism and Clinical Implications" with a great interest.
Pain is the terrible fear of mankind and it is more terrible than death itself. Pain relief is the most important
task of a physician. There are various theory and treatment associated with acute and chronic pain, still chronic
pain most of the time and in most of the world remain untreated, may be because of lack of understanding of
its etiology and mechanism. Before treating pain at each level it is important to understand mechanism of action
at peripheral and central level.
This book consists of 26 contributions submitted by renowned researchers from different parts of the world.
The book is divided into five sections, the first section discusses the basic question and defined very well about
hyper excitability. This section also gives very good insight about which nomenclature should be used in various
conditions of pain.
Second section describes the exiting n e w developments in molecular research and c o m m o n p a t h w a y
emerging to understand peripheral mechanism of hyperalgesia like discovery of the capsaicin receptor on the
u n d e r s t a n d i n g of p e r i p h e r a l sensitization, contribution of tetrodotoxin-resistant s o d i u m channels in
development of peripheral hyperalgesia, VR-1 receptors or TRPV1 receptors is a key substrate in hyperalgesia.
Third section describes transudation mechanism of nociceptors following the pathological process. For
example, mechanism of cold hyperalgesia induced by inflammatory process, visceral pain and hyperalgesia,
ASIC3 is responsible for development of mechanical hyperalgesia following muscle injury. This section also
explains w h y it is difficult to adequately treat pain in cancer patients.
The fourth section describes the role of central nervous system in hyperalgesia and explains very well the
molecular mechanism of central hyperalgesia and its neurobiological features.
The fifth section explains that m a n y brain specific functions also contribute to hyperalgesia and pain
chronicity.
It is a good reference book for clinician and basic scientist working in the area of molecular biology,
neurobiology, molecular pharmacology.
It is also a good reference book for people working in the area of genetics and trying to find out the cause of
pain at genetic level. In addition, it is a useful reference book for pharmaceutical industries involved in making
analgesic/and doing pain research.
For general practitioner and clinician it m a y be of limited use as the main emphasis is on molecular aspect
of pain and its mechnism.

Dr. Sushma Bhatnagar,

Associate Professor,
Unit of Anaesthesiology
Dr BRAIRCH, AIIMS,
New Delhi.

414 Indian Journal of Pediatrics, Volume 72--May, 2005