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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 Lancet 2007; 369: 1541–52
pathophysiology and natural history of the range of morphological and clinical disorders that constitute developmental Centre of Epidemiology for
dysplasia of the hip are poorly understood. Neonatal screening programmes, based on clinical screening examinations, Child Health, Institute of Child
Health, London, UK
have been established for more than 40 years but their effectiveness remains controversial. Whereas systematic
(Prof C Dezateux FMedSci);
sonographic imaging of newborn and young infants has afforded insights into normal and abnormal hip development Section for Radiology,
in early life, we do not clearly understand the longer-term outcomes of developmental hip dysplasia, its contribution University of Bergen, Bergen,
to premature degenerative hip disorders in adult life, and the benefits and harms of newborn screening. High quality Norway; and Department of
Imaging, Great Ormond Street
studies of the adult outcomes of developmental hip dysplasia and the childhood origins of early degenerative hip
Hospital for Children, London,
disease are needed, as are randomised trials to assess the effectiveness and safety of neonatal screening and early UK (Prof K Rosendahl PhD)
treatment. Correspondence to:
Prof Carol Dezateux
Introduction need to know whether stable but mildly dysplastic hips in Medical Research Council Centre
of Epidemiology for Child Health,
Developmental dysplasia of the hip is an important cause childhood and adolescence have implications for hip
Institute of Child Health,
of childhood disability. This disorder underlies up to 9% function and risk of osteoarthritis in adult life. University College London,
of all primary hip replacements and up to 29% of those 30 Guilford Street, London
in people aged 60 years and younger.6 Developments in Diagnosis WC1N 1EH, UK
c.dezateux@ich.ucl.ac.uk
ultrasound imaging have lent developmental hip Dislocation and subluxation can be diagnosed through
dysplasia a greater prominence in recent years: in several clinical examination, but some form of imaging is
European countries, all newborn infants routinely necessary for the diagnosis of stable acetabular dysplasia.10
undergo ultrasonography. One consequence of routine Distinction should be made between tests suitable for
ultrasonographic screening has been a pronounced screening whole populations of newborn infants, and
increase in treatment of neonates, which arises from those for assessment of individuals who are suspected,
clinical uncertainty about the management of on the basis of a clinical examination, to have
ultrasonography findings. The benefits and disadvantages developmental dysplasia. Only a few studies have
of different screening and treatment policies are difficult addressed the clinical validity of screening for hip
to address because of inconsistency in case definitions, instability separately from hip dysplasia.11,12
variation in methods of ascertainment, poor quality of
most studies, and absence of evidence from randomised Clinical examination
trials. We discuss the reliability of different screening Clinical hip instability in newborn infants was first
and diagnostic tests, before reviewing the epidemiology reported in 1879.13 A clinical test for assessment of hip
of developmental dysplasia of the hip; its prevalence, instability was described by Le Damany and Saiget in
aetiology, and natural history; treatment; late sequelae; 1910 and brought to prominence in 1937 by Ortolani.14,15
and secondary prevention through screening. We also Further tests to provoke dislocation or subluxation were
consider challenges for future research about this developed by Palmén in 1961 and by Barlow in 1962.16,17
disorder.
Developmental dysplasia of the hip refers to a range of
developmental hip disorders—from a hip that is mildly Search strategy and selection criteria
dysplastic, concentrically located, and stable, to one that We searched PubMed for articles published on developmental dysplasia of the hip during
is severely dysplastic and dislocated.7 Mild dysplasia the past 40 years (1966–2006) with MeSH terms “hip dislocation” and “congenital”. Our
might never manifest clinically, or might not become search strategies were modified from those developed for a previous systematic review.1
clinically apparent until adult life, whereas severe We searched using the terms “developmental dysplasia of the hip” or “(hip AND dysplas*)”,
dysplasia is most likely to present clinically in later and pooled the results. We referred to papers in databases that we had previously compiled
infancy or early childhood.8 In a dislocated or subluxated through research and associated systematic literature searches, and searched the reference
hip the femoral head is completely or partly displaced lists of other relevant published systematic reviews.1–5 In preparation of this Seminar, we
from the acetabulum: this disorder can be associated focused on publications from the past 10 years, but did not exclude commonly referenced
with secondary acetabular dysplasia, whether or not the and highly regarded older publications. We also selected relevant papers from the
dislocation or subluxation persists. In a stable dysplastic reference lists of articles identified by this search strategy. We have cited articles that meet
hip, the acetabulum is dysplastic but the femoral head is accepted quality standards for design and reporting—namely, randomised controlled trials
stable and not displaced. These disorders might share where available and relevant, or other well designed prospective observational studies
the same antecedents, but further research is needed, reporting prevalence, aetiology, or assessment of diagnosis, screening, treatment, or
especially to find out whether stable acetabular dysplasia clinical outcomes. Review articles and book chapters are also cited to provide readers with
in late adolescence is preceded by dysplasia or instability more details and references than this Seminar allows.
in infancy, or is modifiable by early treatment.9 We also

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Seminar

Some form of neonatal screening for developmental


dysplasia of the hip is practised in most developed Cartilage roof triangle

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 Bony rim

Collectively these findings are referred to as clinical hip


instability. Surprisingly, for such extensively used and Bony acetabular modelling
long-standing tests, interobserver agreement is poor and
experts disagree on criteria for an acceptable Figure 1: Graf’s standard coronal section through the deepest part of the
examination.18,19 Although concern has been expressed acetabulum
Classification into Graf’s types 1–4 is based on the appearances of the bony
that repeated examinations with the Barlow test could acetabular modelling, the bony rim, and the cartilage roof triangle.
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 However, because less pronounced asymmetries of
examinations of an anatomical model showed that even these skin creases are seen in about 30% of all infants
experienced examiners could use excessive force.22 Thus, with normal hip joints and are not always present in
this concern cannot be entirely excluded. those with abnormal hip joints, this test is
The reported prevalence of clinical hip instability unreliable.16,40
detected in newborn infants ranges from 1·6 to 28·5 per Parents whose children are subsequently diagnosed
1000.24–29 This variation could be due to differences in the with dislocation report a range of early features, such as
inclusion of minor degrees of joint laxity, the expertise of limping, walking on tiptoe, difficulty in crawling,
examiners, and the numbers of examiners used. dragging one leg, evident asymmetry of thigh creases,
Prevalence of instability is also age-dependent, and hip abduction or leg length, difficulty in putting on
diminishes in the first week of life as a consequence of nappies, and difficulty in sitting astride an adult’s knee
increased muscle tone.17 Although Ortolani and Barlow or a bicycle.8 Although dislocation is conventionally
tests become less reliable as neonates age, in some associated with delayed walking, in one study no
countries they are used at 6 weeks.30 These tests might important differences in walking age were reported.41
also fail to detect bilateral irreducible dislocations in Shortening of the limb and a limping or rolling gait are
neonates. Although examination for limited abduction later features of persistent dislocation, but are not
and asymmetrical skin creases has been proposed, invariable, and cases first diagnosed in late adulthood or
neither is a reliable sign of bilateral irreducible dislocation at post mortem have been reported.42
in the neonate.31–33 Similarly, isolated hip clicks in
newborn children are not thought to be of pathological Ultrasonographic assessment
importance, although they are a frequent cause of Ultrasound imaging of the newborn hip to detect
referral.34,35 developmental dysplasia was first proposed in 1980,43 and
Clinical diagnosis of hip displacement in older infants was regarded as an important advance because it was
and children is a challenge, because early features are more informative than radiography for depiction of the
non-specific, and the disorder can be well advanced by cartilaginous infant pelvis and did not necessitate exposure
the time the diagnosis is suspected. Unilateral to ionising radiation. Ultrasound imaging has subsequently
displacement can be easier to ascertain, because been used to screen unselected populations of newborn
asymmetry prompts suspicion of abnormality. After the infants, to examine neonates with specified risk factors for
initial relaxation of the newborn hip has disappeared, developmental dysplasia of the hip, to diagnose or exclude
usually within 3 months,17,36 limitation of abduction the disease in those with positive findings on clinical
(probably secondary to soft-tissue contractures of the screening, to monitor response to treatment, and to
adductor muscles) is regarded as an important clinical determine treatment duration. Various techniques and
sign of hip pathology, especially if unilateral.16,17,33,37,38 criteria have been used for assessment of hip morphology
Other clinical signs that have been reported to show hip and stability.44–46 The method of ultrasonography proposed
displacement include differences in leg length, by Graf44 assesses hip morphology, but also takes account
flattening of the buttock on the affected side, and of hip stability. An ultrasound image obtained in the
pronounced asymmetries of thigh-skin creases.39 standard plane is used to assign hips to one of four main

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Figure 2: Graf types 1–4:


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 ossification (types 2a–2c); and so-called decentering hips with highly deficient bony modelling, rounded/flattened bony rim and displaced cartilage roof triangle
(D). Types 3 and 4 comprise eccentric hips with poor bony modelling, flattened bony rim and a displaced cartilage roof triangle.

categories, on the basis of features of the acetabulum, the Values below 47% for boys and 44% for girls are regarded
bony modelling, and the cartilage roof (figures 1 and 2). as pathological.54,55
However, these morphological appearances represent a The method used to assess ultrasound images from
continuum from normal to severe dysplasia, rather than screening of unselected neonates affects the reported
four distinctive groups. Further, indications or thresholds prevalence of various features of hip; these are summarised
for treatment remain controversial: some investigators in the table.54,56–62 Variation between studies might stem
monitor children with type 2a hips and treat those with 2c from differences in diagnostic criteria; in age and
or D hips, whereas others follow cases of type 2c and D population studied; and in image acquisition, processing,
hips and treat types 3 and 4.11,47–51 and analysis. The interobserver and intraobserver
Despite these limitations, Graf’s method has gained reliability of the Graf classification has been reported as
acceptance throughout most of Europe, albeit with moderate to good for normal hips, but as poor for
modifications. For example, Rosendahl and colleagues abnormal and borderline abnormal hips, especially in
have adapted Graf’s method so that it assesses hip studies in which the clinical findings and history associated
morphology and hip stability separately, to distinguish with such cases were not revealed to examiners.23,54,63–68
their relative importance for treatment outcome.52 Whether reliability can be improved by standardised
Harcke,45 Novick,53 and their co-workers have reported a training is unclear, since in one study performance was
dynamic multiplanar approach, that emphasises hip unrelated to previous training or experience.66
stability and the positional relation between the femoral Controversy about the natural history of these findings
head and the acetabulum, and classifies each hip as relates to the inadequate follow-up of screened infants in
normal, subluxated, or dislocated. Morin46 has proposed a all categories and the scarcity of well designed prospective
technique for assessment of the extent of lateralisation of studies. Hips regarded as normal in the neonatal period
the femoral head to show the proportion of the femoral are likely to remain normal,11,69–71 and more importantly,
head covered by the bony acetabulum. Terjesen54,55 has more than 90% of immature hips will have improved by
modified this method to measure the so-called bony-rim 3 months of age.72–75 Despite these findings, some
percentage, later termed the femoral-head coverage. investigators argue that sonographically immature hips

Technique or grading Number of infants 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
system screened or normal (%) head coverage (%) lower femoral head coverage (%) lowest femoral head coverage (%)
198656 Morphology (Graf) 1001 – 2·3 – 0·2
198857 Morphology (Graf) 615 84·7 13·0 2·0 0·3
199158 Morphology (Graf) 1292 40·1 56·6 3·3*
1996†59 Morphology and instability 3613 83·6 13·0 2·7 0·7
(modified Graf)
1997‡60 Morphology (Graf) 4648 50·1 44·8 4·5 0·6
1994§54 Femoral head coverage 4459 95·9 2·9 1·2 –
(modified Morin)
199461 Bony rim percentage 14 050 93·9 6·0 – 0·09
(Harcke/Morin)
199562 Anterior Dynamic 4430 – 1·0 – 0·09

*Graf 2c or D and type 3 or 4. †A dislocatable femoral head was found in 0·1% of the normal and 0·6% 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 different types varied substantially between the six different 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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hips, but is more useful in later infancy when ultrasound


A
Perkins’ line
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
Acetabular whom developmental dysplasia of the hip is suspected
Index clinically; to monitor hip development after early treatment;
and to assess longer-term outcomes. There has been little
Hilgenreiner’s line
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 classification
B
system in infants and young children is the acetabular
index, which measures acetabular inclination before
ossification of the triradiate cartilage (figure 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 (figure 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
Different classification systems for developmental
dysplasia of the hip, based on age at presentation, have
been described.84,88–90
In early adult life, pain and functional limitation are a
poor guide to anatomical abnormalities, which are believed
to predict later degenerative change.91–93 However, the
prognostic implications of specific radiological appearances
Figure 3: Radiological classification of hips using the acetabular index are not well defined. One of the most widely used measures,
(A) The acetabular index measures acetabular inclination before ossification of originally described by Severin in 1941,87 is based on
the triradiate cartilage, and is defined as the angle between Hilgenreiner’s line
and a line from the inferior margin of the iliac bone through the acetabular bony
radiological appearance of the hip at skeletal maturity; it
rim. (B) Radiograph of a 5 month-old girl, showing a normal right hip with a well classifies hips on the basis of acetabular depth and femoral
formed acetabulum, and a dysplastic left hip with a flattened acetabular edge head location. This technique has been used to assess the
(arrow) and a centred femoral head. longer-term anatomical outcomes of hip dislocation and
its treatment.91,93–96 However, interobserver reliability has
should be followed up.76,77 Such a policy would have been reported to be low, and the validity and interpretation
substantial implications because of the high proportion of of studies that report and predict longer-term outcome
newborn infants with immature hips, the moderate have been questioned.94,97,98 Alternative classification
reliability of ultrasound for assessment of immature hips, schemes have been proposed: the Crowe classification99
and uncertainty about treatment thresholds. The assigns hips to four categories according to the extent of
probability of spontaneous resolution in slightly dysplastic, subluxation, and that of Hartofilakidis100 includes an
or Graf type 2c or D hips is unclear because few hips in assessment of acetabular abnormality. Both these
these categories are left untreated.70,72,78 However, the classifications have been used preoperatively to assess the
findings of one small randomised trial of abduction outcome of total hip replacement and determine the
splinting in infants with stable but dysplastic hips severity of dysplasia in adults.99,101 The interobserver and
suggested spontaneous improvement with growth and intraobserver reliability of both classification systems are
without intervention.79 good, but adoption of one standard method has been
advocated to allow comparison of outcomes between
Radiological assessment studies.102
Radiography does not depict the pelvis as clearly as Treatment for developmental dysplasia of the hip aims to
ultrasound in young infants, who have largely cartilaginous ensure normal growth and development of the acetabulum

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and femoral head, but such development is often perturbed unscreened populations the median prevalence of
in the course of surgical or non-surgical treatment in persistent and clinically diagnosed hip dysplasia is
childhood. The resulting disturbances of growth in the estimated to be 1·3 per 1000 (range 0·84 to 1·5) on the
proximal femur are defined radiologically as avascular or basis of a rigorous review of studies from 44 unscreened
aseptic necrosis of the femoral head, although there is little populations of predominantly northwest European
pathological evidence for a vascular mechanism.103 Growth ancestry living in Australia, the USA, Canada, Scandinavia,
disturbances are a recognised complication of surgery, and and the UK.24 The prevalence of neonatal clinical hip
an iatrogenic complication of abduction splinting.96,104 In instability detected through the Ortolani and Barlow
children with unilateral hip dysplasia, disturbance of manoeuvres is higher—it ranges from 1·6 to 28·5 per
growth in the proximal femur has been reported in the 1000.24 Thus, neonatal hip instability, ascertained through
initially normal contralateral hip.96,104 Radiological scoring clinical screening programmes, is considerably more
systems for avascular necrosis include Kalamchi and common than clinically diagnosed hip dysplasia. This
McEwen’s widely used classification, which is based on an finding is consistent with suggestions that hip instability
assessment of damage to the ossific nucleus and the is transient and resolves spontaneously in the first few
physis.105 One study has reported that this classification is weeks of life. The prevalence of ultrasonographically
highly reliable, and another has reported that it can predict detected hip dysplasia in newborn populations in which
poor function at skeletal maturity.106,107 The frequency of ultrasound has been used as a primary screening test is
avascular necrosis in hips after operative procedures even higher; it ranges between 34·0 and 60·3 per 1000 for
ranges from 5 to 60%, and after non-surgical treatment, studies that apply a morphological classification.11,54,55,61
from 0 to 14%.96,108 However, these findings might not be The variation in prevalence, together with information on
directly comparable, since several studies did not have the natural history of these ultrasonographic appearances,
adequate length of follow-up, and the studies differed in emphasises that developmental dysplasia of the hip
losses to follow-up and in the exact definition and scoring cannot be diagnosed on the basis of one neonatal clinical
system used.109 or sonographic screening assessment, since both are
Despite the limitations of the Severin score, it has been associated with false-positive diagnoses.
used in the few studies of adult outcomes in surgically Developmental dysplasia of the hip is most common in
treated developmental dysplasia of the hip.91,93–95 Both the girls.116 Ethnic variation in the prevalence of dislocation
acetabular index, assessed 2 or more years after hip and subluxation in unscreened populations has been
reduction, and the Severin classification at skeletal maturity reported, with notably higher rates in Japanese, Turkish,
have been reported to predict future need for total hip Amerindian, and Lapp populations.24,117–119 The acetabular
replacement or arthrodesis.95,96,110 These findings could be dimensions of Japanese people are shallower than those
interpreted as supportive of a link between early acetabular of British people, and their rates of osteoarthritis are also
remodelling, residual dysplasia at skeletal maturity, and lower.120
the long-term risk of degenerative change, but In the past, without detection by screening,
methodological limitations include small sample size, low developmental dysplasia of the hip usually presented
reliability of the outcome measure, and in some studies clinically after walking age, and at least 50% of patients
absence of blinded assessment. Thus, cautious started treatment by 5 years of age.121 The recognised
interpretation is needed. longer-term complications of untreated developmental
The limitations of two-dimensional assessments of the dysplasia of the hip include pain in the hip, knee, and
hip and femur have increasingly been recognised, not least lower back; disturbances of gait; and degenerative
because of the challenges of total hip replacement in changes in the hip joint. However, the risk of such
severely dysplastic hips.111,112 In some centres, computed complications is not well defined. Some reports suggest
tomography is used to define proximal femoral deformity that, without treatment, functional impairment due to
and the extent of femoral neck anteversion and acetabular developmental dysplasia of the hip is common, and that
anteversion before total hip replacement in young it increases with age but is not inevitable.42,121,122 When
adults.111,113–115 This technique is not recommended for followed up for an average of 50 years, 11–41% of those
young children as the necessary doses of ionising radiation with untreated dislocation remained free of pain.42,121,122
are greater than those used in radiography.84 Magnetic Some evidence suggests that patients with untreated
resonance imaging can allow assessment of hip and subluxation or a false acetabulum have a poor functional
acetabular alignment, and depiction of hip anatomy, and prognosis, with onset of pain on average 10 years earlier
although at present preschool-aged children must be with a false acetabulum than with untreated subluxation
sedated for this method, it is potentially useful in older alone.42,123,124 However, the outcome of untreated acetabular
children. dysplasia in the absence of dislocation or subluxation is
difficult to assess since ascertainment is likely to be
Epidemiology incomplete. Dysplasia has been reported in those with
The prevalence of developmental dysplasia of the hip degenerative hip-joint disease, but childhood dysplasia
varies with age and method of case ascertainment. In does not invariably persist.117

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Aetiology study of adults with hip osteoarthritis or with a need for


Limb-bud differentiation and hip-joint cleavage happen total hip replacement might help to find out whether the
by the 8th week of intrauterine life: the importance of genetic factors implicated in predisposition to
normal developmental progression in fetal position and developmental dysplasia of the hip might differ from
lower-limb rotation by the start of the final trimester of those that affect the severity of its later sequelae.136,137
pregnancy has been extensively documented.39 The risk
of developmental hip dysplasia is increased by factors Treatment
associated with intrauterine mechanical constraint and The primary aim of treatment is to achieve concentric
abnormal position in the final trimester, but is also reduction of the hip, thereby increasing the chances of a
related to the postnatal environment and genetic functionally and anatomically good outcome. Avoidance
predisposition. Factors associated with mechanical of surgery through early detection and non-surgical
constriction of the fetus, including large birthweight management is an important secondary goal, not least
for gestational age, breech presentation, and because surgery is associated with a substantial risk of
oligohydramnios, are more common in those with avascular necrosis. Non-surgical treatment secures the
developmental hip dysplasia, but the most important and hip in a flexed and abducted position using a splint
potentially avoidable perinatal risk factor is vaginal appliance. Such equipment differs in size, shape, ease of
delivery of breech-presenting babies.116,125,126 Both breech removal, and constraints on mobility. In one UK survey,138
presentation and mode of delivery of breech infants are 15 different splint appliances were used to treat dislocated
reported to be significant risk factors for developmental and dislocatable hips, and treatment duration ranged
dysplasia of the hip. In one case control study, vaginal from 2 to 52 weeks. This variation has arisen in the
delivery of a breech presentation was associated with a absence of evidence from randomised trials to assess the
17-fold increased risk of developmental dysplasia of the clinical effectiveness and safety of abduction splinting
hip, compared with a seven-fold increased risk for breech devices.2,4,5
babies delivered by elective caesarean section.116 The use of ultrasound to inform decisions about
Avoidance of postnatal mechanical constriction has initiation of treatment with abduction splinting devices
been advocated for prevention of developmental hip has been assessed in a randomised trial: infants with
dysplasia. Postnatal swaddling practices, such as the use clinical hip instability who were randomly assigned to
of cradle boards in American Indian populations and ultrasound assessment were less likely to be given
tight swaddling in Japan, which both cause long periods abduction splinting devices, but no more likely to need
of extension and adduction of the thighs, have been surgery later, than those randomly assigned to receive
implicated in the high rates of developmental dysplasia only clinical assessment.139 This finding suggests that
of the hip recorded in these populations.118,127 A public infants with clinical hip instability that is detected by
health campaign to alter infant swaddling practices in screening can be safely left untreated on the basis of
Japan was associated with a subsequent fall in prevalence subsequent ultrasound appearances.139 The trial did not
of hip dysplasia.118,127 Theoretically, modern methods of assess ultrasound used in all newborn infants, or in those
caring for infants in developed countries, such as long who have stable hips but also have recognised risk factors
periods spent in baby seats and the use of very slim for developmental dysplasia of the hip; the value of early
disposable nappies which do not abduct the hips as detection and treatment for such infants remains
widely, could also affect hip development. The joint laxity unclear.
that predisposes individuals to developmental dysplasia Early detection and treatment does not entirely avoid
of the hip could also be affected by the maternal hormonal the need for subsequent surgery, and surgery is needed
milieu, but this disorder has not been associated with by up to 5% of infants treated with abduction
changes in concentrations of urinary oestrogen, serum splinting.24,48,140,141 In the UK Medical Research Council
β-estradiol, or serum or cord blood relaxin.128–131 (MRC) study of clinical screening, a fifth of all children
The familial risk for developmental dysplasia of the hip who needed a first operative procedure for developmental
is well recognised. Early studies suggested that both dysplasia of the hip by the age of 5 years had been treated
acetabular dysplasia and joint laxity were heritable, and previously with an abduction splinting device.140 Whether
more recently the disorder has been associated with age at which abduction splinting is initiated can predict
ultrasonographic evidence of severe dysplasia.132,133 In a subsequent need for surgery after splinting is unclear
study based on the Norwegian Twin Registry, the odds because of confounding by severity and mode of
ratio for prevalence of hip dysplasia was reported to be detection. The part played by parental adherence to
much higher for mothers than for siblings, fathers, and abduction splinting treatment is also unknown, as
offspring, which suggests a maternal effect.134 Familial parental responses to screening and treatment have only
joint laxity, associated with joint hypermobility, has been recently received attention.142,143 Children with irreducible
identified as a risk factor for developmental dysplasia of dislocations detected by screening or those diagnosed
the hip, and the heritability of joint hypermobility has after clinical presentation usually need surgical
been estimated at 70% in female adult twins.135 Systematic intervention, which can range from procedures that do

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not require direct surgery to the hip joint to more invasive dislocation, subluxation, and evidence of growth
procedures, such as acetabuloplasty and femoral or pelvic disturbance on follow-up.
osteotomies. Sometimes more than one operation is Proximal-femoral growth disturbance, defined
needed. These various approaches are used on the basis radiologically as avascular necrosis, affects up to 60% of
of expert opinion and of selected small case series from treated hips in some series.108 Some investigators suggest
single centres that report the radiological outcomes of that the risk of this complication is reduced by better
specific procedures.1 preoperative management, including the use of
preoperative skin traction and the so-called human
Outcomes of developmental dysplasia of the hip position during postoperative immobilisation.145,146
Long-term follow-up to skeletal maturity and beyond is However, the effectiveness of preoperative skin traction
needed to identify the functional outcomes of has not been rigorously tested or proven, despite
developmental dysplasia of the hip and its treatment, widespread adoption of this practice in the USA and
since a hip that is anatomically and radiologically poor other countries.103
can function well during childhood and adolescence but Another way to assess the longer-term outcome of
later become symptomatic.91,96 The few reports of small developmental dysplasia of the hip is to quantify its
selected case series that provide information about adult contribution to the need for total hip replacement within
outcomes of treatment are, by definition, unrepresentative a population, especially in young adults. However,
of contemporary approaches to early detection and obvious limitations include selection biases in relation to
management.109 demand for, or access to, treatment. Data from the
Reported outcomes of abduction splinting are difficult Norwegian Arthroplasty Register,6 which includes
to interpret in the absence of a randomised control group, information on 72 301 hip replacements undertaken over
and most observational studies have methodological a 15-year period, show that about 1·0% and 7·6% of all
weaknesses.1,2,4,5 In a large prospective observational study hip replacements resulted from congenital hip disorders
of 221 children with 12% loss to follow-up, 12 (5%) of or dysplasia respectively. In people younger than 60 years,
those treated with abduction splinting needed surgery, these proportions were greater: about 4·8% for congenital
and by 5 years of age, 7 (3%) had signs of acetabular hip disorders and 24·0% for dysplasia, with a female
dysplasia, and 1 (1%) signs of avascular necrosis.144 These excess at all ages.6
estimates are comparable to those of other case series
reviewed in a recent decision analysis, but significant Screening
biases in assessment cannot be excluded.1 These caveats Screening of newborn infants is based on the premise
also apply to reports of outcome after surgical procedures. that if developmental dysplasia of the hip is not diagnosed
Radiological evidence of osteoarthritis was noted in more clinically until after walking age, it is likely to need
than half those treated 10–50 years earlier, of whom half complex surgical treatment and the outcome is likely to
had developed hip pain by 40 years of age.121 In this study, be less favourable than if it were diagnosed earlier. This
clinical and radiological outcome was assessed as good or rationale also assumes that early diagnosis and treatment
better in 78% of unilateral and 51% of bilateral can promote normal hip development and prevent
dislocations, but in only 28% of those treated after 3 years premature osteoarthritis associated with clinical
of age. The prognosis for those treated by open procedures presentation of developmental dysplasia of the hip.
seems to have improved over time: in the earliest series Systematic clinical screening of neonates has been
of hips treated by open reduction some 20 years practised now for more than half a century in European
previously, only a fifth were radiologically normal and countries,16,17 with ultrasound screening of whole
slightly more than half were free of avascular necrosis, populations introduced in Austria and Germany in the
compared with 51–78% and 98% respectively in more 1980s and 1990s.147,148 In some centres, ultrasound is used
recent case series.30 In two cohorts followed up for a to assess those with clinically detected hip instability or
mean duration of 30 and 33 years, respectively, the with recognised specific risk factors for developmental
outcomes were reportedly similar95,96 One study, of a dysplasia of the hip, such as breech delivery and a positive
cohort of 119 patients diagnosed and treated between family history.149–151
1938 and 1969, and followed up at a mean age of 31 years, The challenges that arise from the scarcity of robust
noted disturbed proximal femoral growth in 91 patients evidence to inform screening policies for developmental
(60%), degenerative hip disease in 65 (43%), and total hip dysplasia of the hip are well rehearsed. The outcomes of
replacements in 17 (11%) of 152 hips.96 In another cohort, clinical screening have never been compared in a
of 147 patients diagnosed and treated between 1947 and randomised trial with those of clinical diagnosis or of no
1965 and followed up at a mean age of 36 years, the screening.9,152 Furthermore, the effectiveness of screening
findings were similar: moderate to severe osteoarthritis programmes cannot be assessed directly, because we do
in 78 (41%) and total hip replacement or arthrodesis in 27 not have a diagnostic test to distinguish infants who are
(14%) of 191 hips.95 Features associated with a poorer truly affected from those who are not. Thus, clinical
prognosis included older age at operation, high screening is associated with potential overtreatment of

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children with false-positive screening results, and with Several studies have examined the cost-effectiveness of
failures of screening, diagnosis, and treatment in those different primary screening strategies for developmental
who do have developmental dysplasia.153 dysplasia of the hip, but with one exception29 these have
Much uncertainty also surrounds the clinical not assessed longer-term outcomes, and none has reported
effectiveness and safety of screening programmes based quality-adjusted life years as an outcome.4 In a decision
on ultrasound examination. Two randomised trials have analysis based on UK data, Brown and co-workers29
addressed primary ultrasound screening but neither has suggested that uncertainties in the cost- effectiveness of
compared it with a strategy of no screening.11,71 In a study screening mirrored uncertainties in the evidence regarding
comparing clinical screening alone with a combination its clinical effectiveness. Cost- effectiveness was affected
of clinical screening and universal or targeted ultrasound, by assumptions about the long-term outcomes of surgical
Rosendahl and colleagues reported higher rates of treatment and the proportion of infants treated with
abduction splinting and follow-up in the universal abduction splinting appliances. The most robust economic
ultrasound group due to inconclusive early ultrasound analysis was based on a randomised trial in the UK,139 and
findings.11 Although fewer children screened with assessed costs associated with the use of ultrasonography
universal ultrasound presented with late subluxation or for the diagnosis and management of neonatal hip
dislocation, this difference was not statistically significant. instability, and concluded that this use of ultrasonography
Holen and colleagues71 reported similar findings in a trial was unlikely to impose an increased cost burden and
comparing clinical screening and universal ultrasound could reduce costs to health services and families.159
with clinical screening and targeted ultrasound, and
recommended that ultrasound should be used only for Future directions
those with clinical hip instability or recognised risk In 1984, screening for developmental dysplasia of the hip
factors. was famously described in the pages of the Lancet as a
The prevalence of later surgical treatment for “mess”.160 Some 20 years later the authors of a systematic
developmental dysplasia of the hip in those not detected review concluded that “general screening of newborn
by screening has been used as a surrogate measure of infants for [developmental dysplasia of the hip] provides
outcome of screening. The rate of later surgical treatment us with a good example of how early acceptance of an
ranges from 0·07 to 1·79 per 1000 (median 0·45) in intervention or technology can inhibit or even preclude
different countries with clinical screening programmes.24 good quality research, resulting in long term—if not
This variation probably indicates methodological permanent—uncertainty”.5 This conclusion is consistent
differences in definition of surgical treatment, with the findings of other systematic reviews of clinical
completeness of ascertainment and duration of follow-up, and ultrasound screening.1,2–5,29 However, no countries
and differences between populations and in the quality have abandoned their established clinical screening
of screening programmes.9,36,140,154–156 The equivalent programmes, although some have decided not to add
estimate for screening programmes based on primary universal ultrasound screening.2,4,161
ultrasound has been less extensively reported. A study Where will we be 20 years from now? We will probably
from Germany that used similar methods to the UK know more about the genetic control of acetabular
MRC study of clinical screening, showed an development and hip stability and its implications for
ascertainment-adjusted first operative procedure rate of adult hip health. An essential prerequisite for this
0·26 per 1000 livebirths (95% CI: 0·22–0·32) after knowledge will be careful cross-sectional and longitudinal
ultrasonographic screening, compared with the phenotypic assessment of patients recruited to large,
equivalent estimate of 0·78 (0·72–0·84) after clinical well designed, multicentre studies. Advances in
screening.48,140 three-dimensional imaging modalities might enable better
However, with one notable exception,69 ultrasound characterisation of hips according to variables such as
screening programmes are associated with 40–70 fold femoral anteversion. However, such techniques will need
increases in abduction splinting rates relative to the to avoid use of ionising radiation to assess normative
prevalence of developmental dysplasia of the hip before changes related to childhood growth and development.
screening was introduced.11,148 The long-term outcome for Will there be a trial of primary screening? Although
those who are treated for a disorder they do not have is an various trial designs have been proposed,4,5,24,158 they might
important consideration, since iatrogenic avascular not be feasible in countries where clinical and ultrasound
necrosis of the femoral head affects up to 1% of treated screening are already established. Medicolegal concerns,
children, might affect normal hips as well as those that combined with widely held beliefs about the effectiveness
are initially abnormal, and, in its severest form, might of screening and treatments, probably preclude the
lead to premature osteoarthritis.108,157 Other adverse equipoise needed for a trial. Introduction of a screening
consequences of abduction splinting include femoral service in a stepped-wedge design has been suggested, but
nerve palsies and pressure sores, difficulties in handling good information systems will be needed if such ecological
and positioning the infant in an abduction splinting comparisons are to link longer-term outcomes to screening
device, and parental anxiety.143,158 arm at birth.

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Trials that entail random assignment to treatment with 18 Bialik V, Fishman J, Katzir J, Zeltzer M. Clinical assessment of hip
abduction splinting or watchful waiting for infants in the instability in the newborn by an orthopedic surgeon and a
pediatrician. J Pediatr Orthop 1986; 6: 703–05.
borderline ultrasound categories might prove acceptable 19 El-Shazly M, Trainor B, Kernohan WG, et al. Reliability of the
and might not only provide useful information about the Barlow and Ortolani tests for neonatal hip instability. J Med Scr
natural history of these appearances, but also help to 1994; 1: 165–68.
20 Moore FH. Examining infants’ hips–can it do harm?
assess the effectiveness and disadvantages of treatment.29,162 J Bone Joint Surg 1989; 71-B: 4–5.
In the meantime, we suggest that extension of clinical 21 Jones DA. Neonatal hip stability and the Barlow test.
screening to include universal ultrasound is not justified J Bone Joint Surg 1991; 73-B: 216–18.
scientifically or ethically—a position endorsed earlier this 22 Chow YW, Turner I, Kernohan WG, Mollan RA. Measurement of
the forces and movements involved in neonatal hip testing.
year by the US Preventive Services Task Force.162,163 Med Eng Phys 1994; 16: 181–87.
Contributors 23 Andersson JE. Neonatal hip instability: normal values for
Both authors searched the literature. C Dezateux wrote the first draft, and physiological movement of the femoral head determined by an
K Rosendahl contributed to this draft and prepared the tables and figures. anterior-dynamic ultrasound method. J Pediatr Orthop 1995; 15:
Both authors revised the manuscript and agreed and approved the final 736–40.
version. 24 Leck I. Congenital dislocation of the hip. In: Wald N, Leck I, eds.
Antenatal and neonatal screening. 2nd edn. Oxford: Oxford
Conflict of interest statement University Press, 2000: 398–424.
We declare that we have no conflict of interest. 25 Krikler SJ, Dwyer NSP. Comparison of results of two approaches
to hip screening in infants. J Bone Joint Surg 1992; 74-B: 701–03.
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