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Seminar
In its severest form, developmental dysplasia of the hip is one of the most common congenital malformations. The
pathophysiology and natural history of the range of morphological and clinical disorders that constitute developmental
dysplasia of the hip are poorly understood. Neonatal screening programmes, based on clinical screening examinations,
have been established for more than 40 years but their eectiveness remains controversial. Whereas systematic
sonographic imaging of newborn and young infants has aorded insights into normal and abnormal hip development
in early life, we do not clearly understand the longer-term outcomes of developmental hip dysplasia, its contribution
to premature degenerative hip disorders in adult life, and the benets and harms of newborn screening. High quality
studies of the adult outcomes of developmental hip dysplasia and the childhood origins of early degenerative hip
disease are needed, as are randomised trials to assess the eectiveness and safety of neonatal screening and early
treatment.
Introduction
Developmental dysplasia of the hip is an important cause
of childhood disability. This disorder underlies up to 9%
of all primary hip replacements and up to 29% of those
in people aged 60 years and younger.6 Developments in
ultrasound imaging have lent developmental hip
dysplasia a greater prominence in recent years: in several
European countries, all newborn infants routinely
undergo ultrasonography. One consequence of routine
ultrasonographic screening has been a pronounced
increase in treatment of neonates, which arises from
clinical uncertainty about the management of
ultrasonography ndings. The benets and disadvantages
of dierent screening and treatment policies are dicult
to address because of inconsistency in case denitions,
variation in methods of ascertainment, poor quality of
most studies, and absence of evidence from randomised
trials. We discuss the reliability of dierent screening
and diagnostic tests, before reviewing the epidemiology
of developmental dysplasia of the hip; its prevalence,
aetiology, and natural history; treatment; late sequelae;
and secondary prevention through screening. We also
consider challenges for future research about this
disorder.
Developmental dysplasia of the hip refers to a range of
developmental hip disordersfrom a hip that is mildly
dysplastic, concentrically located, and stable, to one that
is severely dysplastic and dislocated.7 Mild dysplasia
might never manifest clinically, or might not become
clinically apparent until adult life, whereas severe
dysplasia is most likely to present clinically in later
infancy or early childhood.8 In a dislocated or subluxated
hip the femoral head is completely or partly displaced
from the acetabulum: this disorder can be associated
with secondary acetabular dysplasia, whether or not the
dislocation or subluxation persists. In a stable dysplastic
hip, the acetabulum is dysplastic but the femoral head is
stable and not displaced. These disorders might share
the same antecedents, but further research is needed,
especially to nd out whether stable acetabular dysplasia
in late adolescence is preceded by dysplasia or instability
in infancy, or is modiable by early treatment.9 We also
www.thelancet.com Vol 369 May 5, 2007
Diagnosis
Dislocation and subluxation can be diagnosed through
clinical examination, but some form of imaging is
necessary for the diagnosis of stable acetabular dysplasia.10
Distinction should be made between tests suitable for
screening whole populations of newborn infants, and
those for assessment of individuals who are suspected,
on the basis of a clinical examination, to have
developmental dysplasia. Only a few studies have
addressed the clinical validity of screening for hip
instability separately from hip dysplasia.11,12
Clinical examination
Clinical hip instability in newborn infants was rst
reported in 1879.13 A clinical test for assessment of hip
instability was described by Le Damany and Saiget in
1910 and brought to prominence in 1937 by Ortolani.14,15
Further tests to provoke dislocation or subluxation were
developed by Palmn in 1961 and by Barlow in 1962.16,17
1541
Seminar
Bony rim
Figure 1: Grafs standard coronal section through the deepest part of the
acetabulum
Classication into Grafs types 14 is based on the appearances of the bony
acetabular modelling, the bony rim, and the cartilage roof triangle.
Ultrasonographic assessment
Ultrasound imaging of the newborn hip to detect
developmental dysplasia was rst proposed in 1980,43 and
was regarded as an important advance because it was
more informative than radiography for depiction of the
cartilaginous infant pelvis and did not necessitate exposure
to ionising radiation. Ultrasound imaging has subsequently
been used to screen unselected populations of newborn
infants, to examine neonates with specied risk factors for
developmental dysplasia of the hip, to diagnose or exclude
the disease in those with positive ndings on clinical
screening, to monitor response to treatment, and to
determine treatment duration. Various techniques and
criteria have been used for assessment of hip morphology
and stability.4446 The method of ultrasonography proposed
by Graf44 assesses hip morphology, but also takes account
of hip stability. An ultrasound image obtained in the
standard plane is used to assign hips to one of four main
www.thelancet.com Vol 369 May 5, 2007
Seminar
Number of infants
screened
Values below 47% for boys and 44% for girls are regarded
as pathological.54,55
The method used to assess ultrasound images from
screening of unselected neonates aects the reported
prevalence of various features of hip; these are summarised
in the table.54,5662 Variation between studies might stem
from dierences in diagnostic criteria; in age and
population studied; and in image acquisition, processing,
and analysis. The interobserver and intraobserver
reliability of the Graf classication has been reported as
moderate to good for normal hips, but as poor for
abnormal and borderline abnormal hips, especially in
studies in which the clinical ndings and history associated
with such cases were not revealed to examiners.23,54,6368
Whether reliability can be improved by standardised
training is unclear, since in one study performance was
unrelated to previous training or experience.66
Controversy about the natural history of these ndings
relates to the inadequate follow-up of screened infants in
all categories and the scarcity of well designed prospective
studies. Hips regarded as normal in the neonatal period
are likely to remain normal,11,6971 and more importantly,
more than 90% of immature hips will have improved by
3 months of age.7275 Despite these ndings, some
investigators argue that sonographically immature hips
Graf type 1 or stable Graf type 2a or low femoral Graf type 2c or D or dislocatable or Graf type 3 or 4 or dislocated or
or normal (%)
head coverage (%)
lower femoral head coverage (%) lowest femoral head coverage (%)
198656
Morphology (Graf)
1001
23
02
198857
Morphology (Graf)
615
847
130
20
03
199158
Morphology (Graf)
1292
401
566
33*
199659
3613
836
130
27
07
199760
Morphology (Graf)
4648
501
448
45
06
199454
4459
959
29
12
199461
14 050
939
60
009
199562
Anterior Dynamic
4430
10
009
*Graf 2c or D and type 3 or 4. A dislocatable femoral head was found in 01% of the normal and 06% of the immature hips, while a dislocatable or dislocated femoral head was seen in 62% of the minor
dysplastic and in 100% of the major dysplastic hips. The distribution of dierent types varied substantially between the six dierent hospitals involved in the study.
Table: Studies reporting prevalence of ultrasonographic hip abnormalities from universal screening programmes in unselected newborn populations
1543
Seminar
A
Perkins line
Acetabular
Index
Hilgenreiners line
Radiological assessment
Radiography does not depict the pelvis as clearly as
ultrasound in young infants, who have largely cartilaginous
1544
Seminar
Epidemiology
The prevalence of developmental dysplasia of the hip
varies with age and method of case ascertainment. In
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Seminar
Aetiology
Limb-bud dierentiation and hip-joint cleavage happen
by the 8th week of intrauterine life: the importance of
normal developmental progression in fetal position and
lower-limb rotation by the start of the nal trimester of
pregnancy has been extensively documented.39 The risk
of developmental hip dysplasia is increased by factors
associated with intrauterine mechanical constraint and
abnormal position in the nal trimester, but is also
related to the postnatal environment and genetic
predisposition. Factors associated with mechanical
constriction of the fetus, including large birthweight
for gestational age, breech presentation, and
oligohydramnios, are more common in those with
developmental hip dysplasia, but the most important and
potentially avoidable perinatal risk factor is vaginal
delivery of breech-presenting babies.116,125,126 Both breech
presentation and mode of delivery of breech infants are
reported to be signicant risk factors for developmental
dysplasia of the hip. In one case control study, vaginal
delivery of a breech presentation was associated with a
17-fold increased risk of developmental dysplasia of the
hip, compared with a seven-fold increased risk for breech
babies delivered by elective caesarean section.116
Avoidance of postnatal mechanical constriction has
been advocated for prevention of developmental hip
dysplasia. Postnatal swaddling practices, such as the use
of cradle boards in American Indian populations and
tight swaddling in Japan, which both cause long periods
of extension and adduction of the thighs, have been
implicated in the high rates of developmental dysplasia
of the hip recorded in these populations.118,127 A public
health campaign to alter infant swaddling practices in
Japan was associated with a subsequent fall in prevalence
of hip dysplasia.118,127 Theoretically, modern methods of
caring for infants in developed countries, such as long
periods spent in baby seats and the use of very slim
disposable nappies which do not abduct the hips as
widely, could also aect hip development. The joint laxity
that predisposes individuals to developmental dysplasia
of the hip could also be aected by the maternal hormonal
milieu, but this disorder has not been associated with
changes in concentrations of urinary oestrogen, serum
-estradiol, or serum or cord blood relaxin.128131
The familial risk for developmental dysplasia of the hip
is well recognised. Early studies suggested that both
acetabular dysplasia and joint laxity were heritable, and
more recently the disorder has been associated with
ultrasonographic evidence of severe dysplasia.132,133 In a
study based on the Norwegian Twin Registry, the odds
ratio for prevalence of hip dysplasia was reported to be
much higher for mothers than for siblings, fathers, and
ospring, which suggests a maternal eect.134 Familial
joint laxity, associated with joint hypermobility, has been
identied as a risk factor for developmental dysplasia of
the hip, and the heritability of joint hypermobility has
been estimated at 70% in female adult twins.135 Systematic
1546
Treatment
The primary aim of treatment is to achieve concentric
reduction of the hip, thereby increasing the chances of a
functionally and anatomically good outcome. Avoidance
of surgery through early detection and non-surgical
management is an important secondary goal, not least
because surgery is associated with a substantial risk of
avascular necrosis. Non-surgical treatment secures the
hip in a exed and abducted position using a splint
appliance. Such equipment diers in size, shape, ease of
removal, and constraints on mobility. In one UK survey,138
15 dierent splint appliances were used to treat dislocated
and dislocatable hips, and treatment duration ranged
from 2 to 52 weeks. This variation has arisen in the
absence of evidence from randomised trials to assess the
clinical eectiveness and safety of abduction splinting
devices.2,4,5
The use of ultrasound to inform decisions about
initiation of treatment with abduction splinting devices
has been assessed in a randomised trial: infants with
clinical hip instability who were randomly assigned to
ultrasound assessment were less likely to be given
abduction splinting devices, but no more likely to need
surgery later, than those randomly assigned to receive
only clinical assessment.139 This nding suggests that
infants with clinical hip instability that is detected by
screening can be safely left untreated on the basis of
subsequent ultrasound appearances.139 The trial did not
assess ultrasound used in all newborn infants, or in those
who have stable hips but also have recognised risk factors
for developmental dysplasia of the hip; the value of early
detection and treatment for such infants remains
unclear.
Early detection and treatment does not entirely avoid
the need for subsequent surgery, and surgery is needed
by up to 5% of infants treated with abduction
splinting.24,48,140,141 In the UK Medical Research Council
(MRC) study of clinical screening, a fth of all children
who needed a rst operative procedure for developmental
dysplasia of the hip by the age of 5 years had been treated
previously with an abduction splinting device.140 Whether
age at which abduction splinting is initiated can predict
subsequent need for surgery after splinting is unclear
because of confounding by severity and mode of
detection. The part played by parental adherence to
abduction splinting treatment is also unknown, as
parental responses to screening and treatment have only
recently received attention.142,143 Children with irreducible
dislocations detected by screening or those diagnosed
after clinical presentation usually need surgical
intervention, which can range from procedures that do
www.thelancet.com Vol 369 May 5, 2007
Seminar
Screening
Screening of newborn infants is based on the premise
that if developmental dysplasia of the hip is not diagnosed
clinically until after walking age, it is likely to need
complex surgical treatment and the outcome is likely to
be less favourable than if it were diagnosed earlier. This
rationale also assumes that early diagnosis and treatment
can promote normal hip development and prevent
premature osteoarthritis associated with clinical
presentation of developmental dysplasia of the hip.
Systematic clinical screening of neonates has been
practised now for more than half a century in European
countries,16,17 with ultrasound screening of whole
populations introduced in Austria and Germany in the
1980s and 1990s.147,148 In some centres, ultrasound is used
to assess those with clinically detected hip instability or
with recognised specic risk factors for developmental
dysplasia of the hip, such as breech delivery and a positive
family history.149151
The challenges that arise from the scarcity of robust
evidence to inform screening policies for developmental
dysplasia of the hip are well rehearsed. The outcomes of
clinical screening have never been compared in a
randomised trial with those of clinical diagnosis or of no
screening.9,152 Furthermore, the eectiveness of screening
programmes cannot be assessed directly, because we do
not have a diagnostic test to distinguish infants who are
truly aected from those who are not. Thus, clinical
screening is associated with potential overtreatment of
1547
Seminar
Future directions
In 1984, screening for developmental dysplasia of the hip
was famously described in the pages of the Lancet as a
mess.160 Some 20 years later the authors of a systematic
review concluded that general screening of newborn
infants for [developmental dysplasia of the hip] provides
us with a good example of how early acceptance of an
intervention or technology can inhibit or even preclude
good quality research, resulting in long termif not
permanentuncertainty.5 This conclusion is consistent
with the ndings of other systematic reviews of clinical
and ultrasound screening.1,25,29 However, no countries
have abandoned their established clinical screening
programmes, although some have decided not to add
universal ultrasound screening.2,4,161
Where will we be 20 years from now? We will probably
know more about the genetic control of acetabular
development and hip stability and its implications for
adult hip health. An essential prerequisite for this
knowledge will be careful cross-sectional and longitudinal
phenotypic assessment of patients recruited to large,
well designed, multicentre studies. Advances in
three-dimensional imaging modalities might enable better
characterisation of hips according to variables such as
femoral anteversion. However, such techniques will need
to avoid use of ionising radiation to assess normative
changes related to childhood growth and development.
Will there be a trial of primary screening? Although
various trial designs have been proposed,4,5,24,158 they might
not be feasible in countries where clinical and ultrasound
screening are already established. Medicolegal concerns,
combined with widely held beliefs about the eectiveness
of screening and treatments, probably preclude the
equipoise needed for a trial. Introduction of a screening
service in a stepped-wedge design has been suggested, but
good information systems will be needed if such ecological
comparisons are to link longer-term outcomes to screening
arm at birth.
www.thelancet.com Vol 369 May 5, 2007
Seminar
18
Contributors
Both authors searched the literature. C Dezateux wrote the rst draft, and
K Rosendahl contributed to this draft and prepared the tables and gures.
Both authors revised the manuscript and agreed and approved the nal
version.
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Seminar
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