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Developmental dysplasia of the hip

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Seminar

Developmental dysplasia of the hip

Carol Dezateux, Karen Rosendahl

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

need to know whether stable but mildly dysplastic hips in

childhood and adolescence have implications for hip
function and risk of osteoarthritis in adult life.

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

Lancet 2007; 369: 154152

Centre of Epidemiology for
Child Health, Institute of Child
Health, London, UK
(Prof C Dezateux FMedSci);
Section for Radiology,
University of Bergen, Bergen,
Norway; and Department of
Imaging, Great Ormond Street
Hospital for Children, London,
UK (Prof K Rosendahl PhD)
Correspondence to:
Prof Carol Dezateux
Medical Research Council Centre
of Epidemiology for Child Health,
Institute of Child Health,
University College London,
30 Guilford Street, London
WC1N 1EH, UK
c.dezateux@ich.ucl.ac.uk

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

Search strategy and selection criteria

We searched PubMed for articles published on developmental dysplasia of the hip during
the past 40 years (19662006) with MeSH terms hip dislocation and congenital. Our
search strategies were modied from those developed for a previous systematic review.1
We searched using the terms developmental dysplasia of the hip or (hip AND dysplas*),
and pooled the results. We referred to papers in databases that we had previously compiled
through research and associated systematic literature searches, and searched the reference
lists of other relevant published systematic reviews.15 In preparation of this Seminar, we
focused on publications from the past 10 years, but did not exclude commonly referenced
and highly regarded older publications. We also selected relevant papers from the
reference lists of articles identied by this search strategy. We have cited articles that meet
accepted quality standards for design and reportingnamely, randomised controlled trials
where available and relevant, or other well designed prospective observational studies
reporting prevalence, aetiology, or assessment of diagnosis, screening, treatment, or
clinical outcomes. Review articles and book chapters are also cited to provide readers with
more details and references than this Seminar allows.

1541

Seminar

Some form of neonatal screening for developmental

dysplasia of the hip is practised in most developed
countries throughout the world, and the Ortolani and
Barlow tests are the most common clinical tests. In the
Ortolani test, the examiner applies forward pressure to
each femoral head in turn, in an attempt to move a
posteriorly dislocated femoral head forwards into the
acetabulum: palpable movement suggests that the hip is
dislocated or subluxated, but reducible. In the Barlow
test, backward pressure is applied to the head of each
femur in turn, and a subluxatable hip is suspected on
the basis of palpable partial or complete displacement.4
Collectively these ndings are referred to as clinical hip
instability. Surprisingly, for such extensively used and
long-standing tests, interobserver agreement is poor and
experts disagree on criteria for an acceptable
examination.18,19 Although concern has been expressed
that repeated examinations with the Barlow test could
provoke dislocation in an otherwise normal hip,20
increased hip laxity has not been reported in repeatedly
examined infants.21,23 However, a study based on
examinations of an anatomical model showed that even
experienced examiners could use excessive force.22 Thus,
this concern cannot be entirely excluded.
The reported prevalence of clinical hip instability
detected in newborn infants ranges from 16 to 285 per
1000.2429 This variation could be due to dierences in the
inclusion of minor degrees of joint laxity, the expertise of
examiners, and the numbers of examiners used.
Prevalence of instability is also age-dependent, and
diminishes in the rst week of life as a consequence of
increased muscle tone.17 Although Ortolani and Barlow
tests become less reliable as neonates age, in some
countries they are used at 6 weeks.30 These tests might
also fail to detect bilateral irreducible dislocations in
neonates. Although examination for limited abduction
and asymmetrical skin creases has been proposed,
neither is a reliable sign of bilateral irreducible dislocation
in the neonate.3133 Similarly, isolated hip clicks in
newborn children are not thought to be of pathological
importance, although they are a frequent cause of
referral.34,35
Clinical diagnosis of hip displacement in older infants
and children is a challenge, because early features are
non-specic, and the disorder can be well advanced by
the time the diagnosis is suspected. Unilateral
displacement can be easier to ascertain, because
asymmetry prompts suspicion of abnormality. After the
initial relaxation of the newborn hip has disappeared,
usually within 3 months,17,36 limitation of abduction
(probably secondary to soft-tissue contractures of the
adductor muscles) is regarded as an important clinical
sign of hip pathology, especially if unilateral.16,17,33,37,38
Other clinical signs that have been reported to show hip
displacement include dierences in leg length,
attening of the buttock on the aected side, and
pronounced asymmetries of thigh-skin creases.39
1542

Cartilage roof triangle

Bony rim

Bony acetabular modelling

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.

However, because less pronounced asymmetries of

these skin creases are seen in about 30% of all infants
with normal hip joints and are not always present in
those with abnormal hip joints, this test is
unreliable.16,40
Parents whose children are subsequently diagnosed
with dislocation report a range of early features, such as
limping, walking on tiptoe, diculty in crawling,
dragging one leg, evident asymmetry of thigh creases,
hip abduction or leg length, diculty in putting on
nappies, and diculty in sitting astride an adults knee
or a bicycle.8 Although dislocation is conventionally
associated with delayed walking, in one study no
important dierences in walking age were reported.41
Shortening of the limb and a limping or rolling gait are
later features of persistent dislocation, but are not
invariable, and cases rst diagnosed in late adulthood or
at post mortem have been reported.42

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
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Seminar

Figure 2: Graf types 14:

Type 1 comprises a normal hip with a good bony modelling (large arrow), a sharp bony rim (arrowhead) and a narrow, covering cartilage roof triangle (small arrow). Type
2 includes physiologically immature hips with a satisfactory bony modelling, a rounded bony rim and a covering cartilage roof triangle (type 2a); hips with pathologically
delayed ossication (types 2a2c); and so-called decentering hips with highly decient bony modelling, rounded/attened bony rim and displaced cartilage roof triangle
(D). Types 3 and 4 comprise eccentric hips with poor bony modelling, attened bony rim and a displaced cartilage roof triangle.

categories, on the basis of features of the acetabulum, the

bony modelling, and the cartilage roof (gures 1 and 2).
However, these morphological appearances represent a
continuum from normal to severe dysplasia, rather than
four distinctive groups. Further, indications or thresholds
for treatment remain controversial: some investigators
monitor children with type 2a hips and treat those with 2c
or D hips, whereas others follow cases of type 2c and D
hips and treat types 3 and 4.11,4751
Despite these limitations, Grafs method has gained
acceptance throughout most of Europe, albeit with
modications. For example, Rosendahl and colleagues
have adapted Grafs method so that it assesses hip
morphology and hip stability separately, to distinguish
their relative importance for treatment outcome.52
Harcke,45 Novick,53 and their co-workers have reported a
dynamic multiplanar approach, that emphasises hip
stability and the positional relation between the femoral
head and the acetabulum, and classies each hip as
normal, subluxated, or dislocated. Morin46 has proposed a
technique for assessment of the extent of lateralisation of
the femoral head to show the proportion of the femoral
head covered by the bony acetabulum. Terjesen54,55 has
modied this method to measure the so-called bony-rim
percentage, later termed the femoral-head coverage.
Technique or grading
system

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

Morphology and instability

(modied Graf)

3613

836

130

27

07

199760

Morphology (Graf)

4648

501

448

45

06

199454

Femoral head coverage

(modied Morin)

4459

959

29

12

199461

Bony rim percentage

(Harcke/Morin)

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

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1543

Seminar

A
Perkins line

Acetabular
Index
Hilgenreiners line

Figure 3: Radiological classication of hips using the acetabular index

(A) The acetabular index measures acetabular inclination before ossication of
the triradiate cartilage, and is dened as the angle between Hilgenreiners line
and a line from the inferior margin of the iliac bone through the acetabular bony
rim. (B) Radiograph of a 5 month-old girl, showing a normal right hip with a well
formed acetabulum, and a dysplastic left hip with a attened acetabular edge
(arrow) and a centred femoral head.

should be followed up.76,77 Such a policy would have

substantial implications because of the high proportion of
newborn infants with immature hips, the moderate
reliability of ultrasound for assessment of immature hips,
and uncertainty about treatment thresholds. The
probability of spontaneous resolution in slightly dysplastic,
or Graf type 2c or D hips is unclear because few hips in
these categories are left untreated.70,72,78 However, the
ndings of one small randomised trial of abduction
splinting in infants with stable but dysplastic hips
suggested spontaneous improvement with growth and
without intervention.79

Radiological assessment
Radiography does not depict the pelvis as clearly as
ultrasound in young infants, who have largely cartilaginous
1544

hips, but is more useful in later infancy when ultrasound

might not be reliable.80 Pelvic radiographs have been used
to screen selected high-risk infants aged 4 months.81
However, pelvic radiographs are more commonly used to
assess dislocation, subluxation, or dysplasia for those in
whom developmental dysplasia of the hip is suspected
clinically; to monitor hip development after early treatment;
and to assess longer-term outcomes. There has been little
assessment of the radiological exposure consequent on
repeated pelvic radiological examinations, although one
study has suggested small but measurably increased risks
of fatal leukaemias and reproductive defects in patients
surgically treated for hip dysplasia who are presumably in
receipt of a high number of pelvic radiographs.82
The most commonly used radiological classication
system in infants and young children is the acetabular
index, which measures acetabular inclination before
ossication of the triradiate cartilage (gure 3).83,84
Age-related standards for this index have been reported by
several investigators.85 Because the acetabular index varies
according to the position of the pelvis, additional measures
aid assessment of the acetabular depth and the location
and shape of the femoral head by making adjustment for
the degree of lateral rotation and pelvic tilt (gure 3).85,86
After fusion of the triradiate cartilage, the centre edge of
Wiberg, or centre-edge angle, is commonly used to assess
the degree of lateral coverage of the femoral head.84,87
Dierent classication systems for developmental
dysplasia of the hip, based on age at presentation, have
been described.84,8890
In early adult life, pain and functional limitation are a
poor guide to anatomical abnormalities, which are believed
to predict later degenerative change.9193 However, the
prognostic implications of specic radiological appearances
are not well dened. One of the most widely used measures,
originally described by Severin in 1941,87 is based on
radiological appearance of the hip at skeletal maturity; it
classies hips on the basis of acetabular depth and femoral
head location. This technique has been used to assess the
longer-term anatomical outcomes of hip dislocation and
its treatment.91,9396 However, interobserver reliability has
been reported to be low, and the validity and interpretation
of studies that report and predict longer-term outcome
have been questioned.94,97,98 Alternative classication
schemes have been proposed: the Crowe classication99
assigns hips to four categories according to the extent of
subluxation, and that of Hartolakidis100 includes an
assessment of acetabular abnormality. Both these
classications have been used preoperatively to assess the
outcome of total hip replacement and determine the
severity of dysplasia in adults.99,101 The interobserver and
intraobserver reliability of both classication systems are
good, but adoption of one standard method has been
advocated to allow comparison of outcomes between
studies.102
Treatment for developmental dysplasia of the hip aims to
ensure normal growth and development of the acetabulum
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Seminar

and femoral head, but such development is often perturbed

in the course of surgical or non-surgical treatment in
childhood. The resulting disturbances of growth in the
proximal femur are dened radiologically as avascular or
aseptic necrosis of the femoral head, although there is little
pathological evidence for a vascular mechanism.103 Growth
disturbances are a recognised complication of surgery, and
an iatrogenic complication of abduction splinting.96,104 In
children with unilateral hip dysplasia, disturbance of
growth in the proximal femur has been reported in the
initially normal contralateral hip.96,104 Radiological scoring
systems for avascular necrosis include Kalamchi and
McEwens widely used classication, which is based on an
assessment of damage to the ossic nucleus and the
physis.105 One study has reported that this classication is
highly reliable, and another has reported that it can predict
poor function at skeletal maturity.106,107 The frequency of
avascular necrosis in hips after operative procedures
ranges from 5 to 60%, and after non-surgical treatment,
from 0 to 14%.96,108 However, these ndings might not be
directly comparable, since several studies did not have
adequate length of follow-up, and the studies diered in
losses to follow-up and in the exact denition and scoring
system used.109
Despite the limitations of the Severin score, it has been
used in the few studies of adult outcomes in surgically
treated developmental dysplasia of the hip.91,9395 Both the
acetabular index, assessed 2 or more years after hip
reduction, and the Severin classication at skeletal maturity
have been reported to predict future need for total hip
replacement or arthrodesis.95,96,110 These ndings could be
interpreted as supportive of a link between early acetabular
remodelling, residual dysplasia at skeletal maturity, and
the long-term risk of degenerative change, but
methodological limitations include small sample size, low
reliability of the outcome measure, and in some studies
absence of blinded assessment. Thus, cautious
interpretation is needed.
The limitations of two-dimensional assessments of the
hip and femur have increasingly been recognised, not least
because of the challenges of total hip replacement in
severely dysplastic hips.111,112 In some centres, computed
tomography is used to dene proximal femoral deformity
and the extent of femoral neck anteversion and acetabular
anteversion before total hip replacement in young
adults.111,113115 This technique is not recommended for
young children as the necessary doses of ionising radiation
are greater than those used in radiography.84 Magnetic
resonance imaging can allow assessment of hip and
acetabular alignment, and depiction of hip anatomy, and
although at present preschool-aged children must be
sedated for this method, it is potentially useful in older
children.

Epidemiology
The prevalence of developmental dysplasia of the hip
varies with age and method of case ascertainment. In
www.thelancet.com Vol 369 May 5, 2007

unscreened populations the median prevalence of

persistent and clinically diagnosed hip dysplasia is
estimated to be 13 per 1000 (range 084 to 15) on the
basis of a rigorous review of studies from 44 unscreened
populations of predominantly northwest European
ancestry living in Australia, the USA, Canada, Scandinavia,
and the UK.24 The prevalence of neonatal clinical hip
instability detected through the Ortolani and Barlow
manoeuvres is higherit ranges from 16 to 285 per
1000.24 Thus, neonatal hip instability, ascertained through
clinical screening programmes, is considerably more
common than clinically diagnosed hip dysplasia. This
nding is consistent with suggestions that hip instability
is transient and resolves spontaneously in the rst few
weeks of life. The prevalence of ultrasonographically
detected hip dysplasia in newborn populations in which
ultrasound has been used as a primary screening test is
even higher; it ranges between 340 and 603 per 1000 for
studies that apply a morphological classication.11,54,55,61
The variation in prevalence, together with information on
the natural history of these ultrasonographic appearances,
emphasises that developmental dysplasia of the hip
cannot be diagnosed on the basis of one neonatal clinical
or sonographic screening assessment, since both are
associated with false-positive diagnoses.
Developmental dysplasia of the hip is most common in
girls.116 Ethnic variation in the prevalence of dislocation
and subluxation in unscreened populations has been
reported, with notably higher rates in Japanese, Turkish,
Amerindian, and Lapp populations.24,117119 The acetabular
dimensions of Japanese people are shallower than those
of British people, and their rates of osteoarthritis are also
lower.120
In the past, without detection by screening,
developmental dysplasia of the hip usually presented
clinically after walking age, and at least 50% of patients
started treatment by 5 years of age.121 The recognised
longer-term complications of untreated developmental
dysplasia of the hip include pain in the hip, knee, and
lower back; disturbances of gait; and degenerative
changes in the hip joint. However, the risk of such
complications is not well dened. Some reports suggest
that, without treatment, functional impairment due to
developmental dysplasia of the hip is common, and that
it increases with age but is not inevitable.42,121,122 When
followed up for an average of 50 years, 1141% of those
with untreated dislocation remained free of pain.42,121,122
Some evidence suggests that patients with untreated
subluxation or a false acetabulum have a poor functional
prognosis, with onset of pain on average 10 years earlier
with a false acetabulum than with untreated subluxation
alone.42,123,124 However, the outcome of untreated acetabular
dysplasia in the absence of dislocation or subluxation is
dicult to assess since ascertainment is likely to be
incomplete. Dysplasia has been reported in those with
degenerative hip-joint disease, but childhood dysplasia
does not invariably persist.117
1545

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

study of adults with hip osteoarthritis or with a need for

total hip replacement might help to nd out whether the
genetic factors implicated in predisposition to
developmental dysplasia of the hip might dier from
those that aect the severity of its later sequelae.136,137

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
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not require direct surgery to the hip joint to more invasive

procedures, such as acetabuloplasty and femoral or pelvic
osteotomies. Sometimes more than one operation is
needed. These various approaches are used on the basis
of expert opinion and of selected small case series from
single centres that report the radiological outcomes of
specic procedures.1

Outcomes of developmental dysplasia of the hip

Long-term follow-up to skeletal maturity and beyond is
needed to identify the functional outcomes of
developmental dysplasia of the hip and its treatment,
since a hip that is anatomically and radiologically poor
can function well during childhood and adolescence but
later become symptomatic.91,96 The few reports of small
selected case series that provide information about adult
outcomes of treatment are, by denition, unrepresentative
of contemporary approaches to early detection and
management.109
Reported outcomes of abduction splinting are dicult
to interpret in the absence of a randomised control group,
and most observational studies have methodological
weaknesses.1,2,4,5 In a large prospective observational study
of 221 children with 12% loss to follow-up, 12 (5%) of
those treated with abduction splinting needed surgery,
and by 5 years of age, 7 (3%) had signs of acetabular
dysplasia, and 1 (1%) signs of avascular necrosis.144 These
estimates are comparable to those of other case series
reviewed in a recent decision analysis, but signicant
biases in assessment cannot be excluded.1 These caveats
also apply to reports of outcome after surgical procedures.
Radiological evidence of osteoarthritis was noted in more
than half those treated 1050 years earlier, of whom half
had developed hip pain by 40 years of age.121 In this study,
clinical and radiological outcome was assessed as good or
better in 78% of unilateral and 51% of bilateral
dislocations, but in only 28% of those treated after 3 years
of age. The prognosis for those treated by open procedures
seems to have improved over time: in the earliest series
of hips treated by open reduction some 20 years
previously, only a fth were radiologically normal and
slightly more than half were free of avascular necrosis,
compared with 5178% and 98% respectively in more
recent case series.30 In two cohorts followed up for a
mean duration of 30 and 33 years, respectively, the
outcomes were reportedly similar95,96 One study, of a
cohort of 119 patients diagnosed and treated between
1938 and 1969, and followed up at a mean age of 31 years,
noted disturbed proximal femoral growth in 91 patients
(60%), degenerative hip disease in 65 (43%), and total hip
replacements in 17 (11%) of 152 hips.96 In another cohort,
of 147 patients diagnosed and treated between 1947 and
1965 and followed up at a mean age of 36 years, the
ndings were similar: moderate to severe osteoarthritis
in 78 (41%) and total hip replacement or arthrodesis in 27
(14%) of 191 hips.95 Features associated with a poorer
prognosis included older age at operation, high
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dislocation, subluxation, and evidence of growth

disturbance on follow-up.
Proximal-femoral growth disturbance, dened
radiologically as avascular necrosis, aects up to 60% of
treated hips in some series.108 Some investigators suggest
that the risk of this complication is reduced by better
preoperative management, including the use of
preoperative skin traction and the so-called human
position during postoperative immobilisation.145,146
However, the eectiveness of preoperative skin traction
has not been rigorously tested or proven, despite
widespread adoption of this practice in the USA and
other countries.103
Another way to assess the longer-term outcome of
developmental dysplasia of the hip is to quantify its
contribution to the need for total hip replacement within
a population, especially in young adults. However,
obvious limitations include selection biases in relation to
demand for, or access to, treatment. Data from the
Norwegian Arthroplasty Register,6 which includes
information on 72 301 hip replacements undertaken over
a 15-year period, show that about 10% and 76% of all
hip replacements resulted from congenital hip disorders
or dysplasia respectively. In people younger than 60 years,
these proportions were greater: about 48% for congenital
hip disorders and 240% for dysplasia, with a female
excess at all ages.6

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

children with false-positive screening results, and with

failures of screening, diagnosis, and treatment in those
who do have developmental dysplasia.153
Much uncertainty also surrounds the clinical
eectiveness and safety of screening programmes based
on ultrasound examination. Two randomised trials have
addressed primary ultrasound screening but neither has
compared it with a strategy of no screening.11,71 In a study
comparing clinical screening alone with a combination
of clinical screening and universal or targeted ultrasound,
Rosendahl and colleagues reported higher rates of
abduction splinting and follow-up in the universal
ultrasound group due to inconclusive early ultrasound
ndings.11 Although fewer children screened with
universal ultrasound presented with late subluxation or
dislocation, this dierence was not statistically signicant.
Holen and colleagues71 reported similar ndings in a trial
comparing clinical screening and universal ultrasound
with clinical screening and targeted ultrasound, and
recommended that ultrasound should be used only for
those with clinical hip instability or recognised risk
factors.
The prevalence of later surgical treatment for
developmental dysplasia of the hip in those not detected
by screening has been used as a surrogate measure of
outcome of screening. The rate of later surgical treatment
ranges from 007 to 179 per 1000 (median 045) in
dierent countries with clinical screening programmes.24
This variation probably indicates methodological
dierences in denition of surgical treatment,
completeness of ascertainment and duration of follow-up,
and dierences between populations and in the quality
of screening programmes.9,36,140,154156 The equivalent
estimate for screening programmes based on primary
ultrasound has been less extensively reported. A study
from Germany that used similar methods to the UK
MRC study of clinical screening, showed an
ascertainment-adjusted rst operative procedure rate of
026 per 1000 livebirths (95% CI: 022032) after
ultrasonographic screening, compared with the
equivalent estimate of 078 (072084) after clinical
screening.48,140
However, with one notable exception,69 ultrasound
screening programmes are associated with 4070 fold
increases in abduction splinting rates relative to the
prevalence of developmental dysplasia of the hip before
screening was introduced.11,148 The long-term outcome for
those who are treated for a disorder they do not have is an
important consideration, since iatrogenic avascular
necrosis of the femoral head aects up to 1% of treated
children, might aect normal hips as well as those that
are initially abnormal, and, in its severest form, might
lead to premature osteoarthritis.108,157 Other adverse
consequences of abduction splinting include femoral
nerve palsies and pressure sores, diculties in handling
and positioning the infant in an abduction splinting
device, and parental anxiety.143,158
1548

Several studies have examined the cost-eectiveness of

dierent primary screening strategies for developmental
dysplasia of the hip, but with one exception29 these have
not assessed longer-term outcomes, and none has reported
quality-adjusted life years as an outcome.4 In a decision
analysis based on UK data, Brown and co-workers29
suggested that uncertainties in the cost- eectiveness of
screening mirrored uncertainties in the evidence regarding
its clinical eectiveness. Cost- eectiveness was aected
by assumptions about the long-term outcomes of surgical
treatment and the proportion of infants treated with
abduction splinting appliances. The most robust economic
analysis was based on a randomised trial in the UK,139 and
assessed costs associated with the use of ultrasonography
for the diagnosis and management of neonatal hip
instability, and concluded that this use of ultrasonography
was unlikely to impose an increased cost burden and
could reduce costs to health services and families.159

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.
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Seminar

Trials that entail random assignment to treatment with

abduction splinting or watchful waiting for infants in the
borderline ultrasound categories might prove acceptable
and might not only provide useful information about the
natural history of these appearances, but also help to
assess the eectiveness and disadvantages of treatment.29,162
In the meantime, we suggest that extension of clinical
screening to include universal ultrasound is not justied
scientically or ethicallya position endorsed earlier this
year by the US Preventive Services Task Force.162,163

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.

23

Conict of interest statement

We declare that we have no conict of interest.
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