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DEVELOPMENTAL DYSPLASIA OF THE HIP

The term developmental dysplasia of the hip (DDH) is necessarily general and
encompasses the many facets of the condition. DDH is variable at presentation and is
defined as an abnormal formation of the hip joint occurring between organogenesis
and maturity as a result of instability. It is intentionally non-specific to include the
entire spectrum of the disorder both in time and severity. This spectrum does not
include hip abnormalities caused by other diseases, such as cerebral palsy or
myelodysplasia. As in many other conditions delay in detection leads to more severe
consequences for treatment and prognosis. However, not all individuals at the same
age of presentation have the same degree of abnormality. Therefore the concept of
DDH as a spectrum in time and severity should be useful in organising one's
understanding of this rather enigmatic problem.
CASE REPORT
NS., a two and a half year old girl, born full term, via spontaneous vaginal delivery
with a birth weight of 2.7kg. She was the second child in the family. Once she started
to walk at the age of one and a half years old, her parents noticed that she was
walking with a limping gait. Clinically, she was a healthy child and walked with a
Trendelenberg gait. The left lower limb was shortened. There was limited abduction
and marked internal rotation of the left hip. Plain radiographs showed that the left hip
joint dislocated superiorly and the acetabulum was dysplastic.
Left adductors release, open reduction of the left hip and left femoral derotation
osteotomy was performed. Anterior(Smith-Paterson) approach was used. The findings
were the adductors were tight, the left hip had dislocated superiorly, the capsule was
markedly thickened with hourglass constriction, the labrum was thickened and
inverted, the transverse acetabular ligament was also thickened, the acetabulum was
filled with pulvinar and the femoral head anteverted by about 60 degrees. One third

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tubular plate was used to hold the osteotomy site. Postoperatively hip spice was
applied.
Patient discharged well. The hip spica was changed twice, that was 3 weeks and 6
weeks after the operation. When she was reviewed four months postoperatively in the
clinic, the left hip was stable with full flexion and there was slight left lower limb
shortening. The surgical wound healed well. The plate over the left femur was
removed eleven months postoperatively. The osteotommy site had united with no
fracture gap seen.
DISCUSSION
DDH was formally known as congenital dislocation of the hip. The disease did not
change but there are some very good reasons to adapt the new terminology. The
change from congenital to developmental is crucial because clear evidence has shown
that not all cases are diagnosable at birth. Hips that are found to be normal at birth
(and even in the first few months of life) can subsequently be found to be abnormal
later. In addition, this terminology acknowledges that with the passage of time
changes occur.
In general the incidence in white neonates is 1% for dysplasia and 0.1% for dislocated
hips. Incidence varies with race. There is four-fold increase likelihood in females and
the left side is involved in 60% of cases (right 20%, bilateral 20%). Associated birth
factors include breech presentation, torticolis, talipes equinovarus, metatarsus
adductus and being first born. These factors are believed to be "packaging" issues.
Although 2-3% of all babies are breech presentations, 16-25% of the DDH patients
are born breech. Risk is relative to intrauterine position with cephalic 0.7%, footling
breech 2% and frank breech 20%.
Three theories have been postulated to account for the disease process of DDH, (i)
mechanical, (ii) primary acetabular dysplasia and (iii) ligamentous laxity. The
mechanical theory helps to explain the increase incidence in first born and breech
position as well as the association with the other packaging problems. It implies

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abnormal intrauterine positioning as a cause. The theory of primary acetabular


dysplasia has two main proponents, Faber and Wynne-Davis. They believe that there
is a primary acetabular dysplasia that predisposes to dislocation and an increased rate
of acetabular dysplasia in biologic parents of patients with late DDH. Howorth and
Massie support the theory of ligamentous laxity. The hormonal effects of relaxin on
the collagenous structures of the infant are similar of those to the mother. Novacheck
(1996) believes that combination of ligamentous laxity with mechanical factors from
malpositioning can cause the femoral head to move out of the confines of the
acetabulum. The changes that occur in the acetabulum are secondary to these primary
problems and are the result rather than the cause of the dysplasia.
The pathologic changes in patients with DDH are the basis for understanding DDH as
a spectrum in time and severity. Changes begin in the hip joint capsule. Laxity of the
structure allows the femoral head to begin to migrate out of the acetabulum.
Redundancy is present superolaterally. The labrum is a cartilaginous structure that
normally enlarges the capacity of the bony acetabulum. With subluxation the labrum
everts, and the ligamentum teres elongates. At this point the femoral head is
subluxated or subluxatable. With further laxity, the femoral head dislocates and the
labrum inverts. At this point, the intraarticular structures, including the ligamentum
teres and the pulvinar (fibrofatty tissue in the floor of the acetabulum) may begin to
hypertrophy. The labrum become pathologic in size and shape is then referred to as
the limbus. With these changes, the femoral head can no longer be relocated because
of the obstruction to reduction caused by the soft tissues. The psoas tendon coursing
across the front of the joint capsule courses a narrowing at the isthmus. The transverse
acetabular ligament, capsule, limbus, pulvinar and ligamentum teres all may have a
role in preventing relocation of the femoral head and at this point the dislocation is
fixed. With the passage of time, contractures can develop in the iliopsoas and hip
adductors, further prohibiting positioning of the leg and efforts to reduce the hip.
Ultimately, because the growth forces have been abnormal, the cartilage models of the
proximal femur and acetabulum deform with several possible consequences, including
flattening of the femoral head, valgus neck-shaft angle, excessive femoral
anteversion, and acetabular dysplasia.

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Because of the differences in severity of the dysplasia and the progressive changes
that occur over time, presentation and the method of confirming the diagnosis are
variable. Before walking age, the diagnosis is based on physical examination.
Initially, the examination in the new-born nursery is the critical screening tool to
detect loose, dislocatable, and dislocated hips. Detection up until approximately 4 to 6
months of age still depends primarily on the Barlow and Ortolani manoeuvres as part
of routine well-baby examinations. At approximately 6 months of age, the family may
notice a difference in the range of motion with some difficulty diapering.
Occasionally the family may notice or the examination may indicate a leg length
difference or an asymmetric appearance of the two legs (asymmetric skinfolds). The
importance of frequent, routine screening hip examinations until walking age by an
experienced examiner cannot be overemphasised. With the onset of walking, gait
asymmetry (generally related to leg discrepancy) is the hallmark for identifying an
abnormal hip. Occasionally, asymmetric intoeing or outtoeing is the clue. If the hip
abnormality remains undiagnosed, it will likely be the onset of pain or more
noticeable gait asymmetry that draws attention to the hip as the child becomes older,
larger, and more well developed. (Novacheck, 1996)
Physiologic flexion, abduction and external rotation contractures are normal findings
in neonatal hip examinations. Knee flexion contractures are also typical. These of
course are related to normal intrauterine positioning. An asymmetric examination may
be a subtle clue to the presence of hip dysplasia. Barlow provocative tests and
Ortolani reduction manoeuvre is the mainstay screening examination. A helpful trick
to remember is that the "O" in Ortolani stands for the hip is "out". The examination is
a reduction manoeuvre to place it back to a normal position. The Barlow's provocative
test then detects the subluxatable or dislocatable hip. The key to detection is not a
"click" but rather a "clunk". One tries to detect abnormal movements between the
femoral head and the acetabulum. Novacheck (1996) recommends minimisation of
movements of abduction and adduction and to emphasis the push of the Barlow's test
to try to move the femoral head posteriorly and the pull of the Ortolani reduction
manoeuvre to draw the femoral head anteriorly back into place. The child should be at

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rest and not struggling against the examiner. The presence of asymmetric skinfolds is
a sensitive but non-specific indicator of abnormality. If the folds are symmetric than it
would be very unlikely that the hips are abnormal. Asymmetric is common and is
present in 30% of all infants. It can be used as an indicator to raise one's index of
suspicion, As time passes and secondary soft tissue contractures develop the Barow
and Ortolani manoeuvres become less dependable as the hip becomes fixed in its
normal position. Limited abduction, skinfold asymmetry, and the Galeazzi sign
(relative shortening of the femoral segment) become more important. Bilateral hip
disease is especially difficult to identify because no asymmetry is present. Findings on
examination include widening of the perineum, symmetric limited abduction (less
than 45o) and the appearance of abnormally short thigh segments for the child's
overall size. After walking age hyperlordosis and a waddling gait are the classic
findings.
Radiographs before 4 months of age can be fraught with problems and may provide a
false sense of security if they seem normal. Because the secondary centre of
ossification of the femoral head has not yet developed, an abnormal relationship
between the upper end of the femur and the acetabulum may not be apparent. In
addition, unless the femoral head lies in an abnormal position at rest, the radiograph
may provide false-negative information. Therefore, the only way that the radiograph
can be useful is if it is abnormal. A normal radiograph before 4 months of age cannot
rule out pathology.

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Figure 1. Radiographic signs of subluxation and dysplasia on an AP view. The proximal


femoral ossification center has been drawn but may be absent before age 6 months (H,
Hilgenreiner's line; P, Perkins' line; S, Shenton's line (broken on left); AI, acetabular index).
Note the subluxation in the left hip.

( i ) The acetabular index (AI). It is an indicator of the slope of the acetabulum. The
upper limit of normal values is 30o (up to 1 year), 25o (1-3 years) and 20o (3
years to adult).
( ii ) The vertical line of Perkin's (P) is an extremely valuable determinant because it
can identify with considerable accuracy the relationship of the proximal femur to
the acetabulum. It consists of a vertical line drawn through the outermost tip of
the ossified acetabular roof and perpendicular to the horizontal Y-line of
Hilgenreiner. If the proximal metaphysis of the femur lies lateral to this line, it is
considered to be dysplastic or subluxated (or even dislocated) hip.
( iii ) Shenton's line (S). This is a line drawn between the medial border of the femoral
neck and the superior border of the obturator foramen. A normal hip reveals a
continuous line while a dislocated hip reveals a disrupted line.
( iv ) Centre-edge (CE) angle of Wiberg is also useful in assessment of the future of
the joint. This is measured from the centre of the head to the superolateal edge of
the acetabulum. Any angle less than 20o indicates lateral subluxation of the hip.

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( v ) Von-Rosen's view - Von Rosen's lines; with the hips adducted 45o and in internal
rotation, the femoral shaft should point into the acetabulum.
Ultrasonography is usually used whether as the first imaging study for infants in
whom physical examination of the hip is abnormal or as a screening examination for
infants who have an increased risk of dislocation of the hip. Compared with x-rays of
the pelvis, ultrasound examination has several advantages ( i ) it is a sensitive indicator of malposition, instability and lack of acetabular
development;
( ii ) it is more accurate than a radiograph in defining abnormality of the hip because
it shows the cartilaginous components of the acetabulum and the proximal part
of the femur, which are not visualised on routine radiographs;
( iii ) with dynamic techniques, it accurately describes stability and can show
subluxation with or without dislocability; in dislocated hips, examination by
ultrasound assesses reducibility;
( iv ) with ultrasound, the child is not exposed to ionising radiation.
Ultrasound is invaluable in managing a child undergoing treatment particularly in the
first few months of life (Benson, 1998). Taylor (1997) in his study concluded that
ultrasound monitoring has led to an acceptably low level of intervention, a high
reduction and minimal iatrogenic complications. Mosert et al. (2000) in their study on
the results of Pavlik harness treatment for neonatal hip dislocation as related to Graf's
sonographic classification, concluded that this method has become an indispensable
tool in guiding their treatment policy in dislocated hips in children younger than 26
weeks of age.
An important issue is whether the sensitivity of ultrasonography leads to unnecessary
treatment. Harcke et al. (1991) in their experience have indicated that, in infants who
are younger than 30 days, and in whom laxity of the hip is seen on ultrasound
examination but the results of clinical examination are normal, the hip almost always
becomes normal without treatment.

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The routine use of ultrasound screening of all newborn infants has become a
controversial issue. Clarke et al. (1989) showed that the screening of all infants who
had risk factors (for example, breech delivery, positive family history, deformity of
the foot or torticolis) and of all infants who had abnormality on physical examination
did not reduce the prevalence of late cases in the practice. Paton et al. (1999) from
their study results also suggest that selective screening of infants with "at-risk" factors
in isolation is of little value in reducing the incidence of late dislocation. However,
they found that screening clinically unstable hips alone or associated with "at-risk"
factors has a high rate of detection.
The essential, basic goals of treatment are the same regardless of the age at diagnosis.
The treatment necessary to achieve those goals varies and depends on the age at
diagnosis and the degree of abnormality. The goals are hierarchic and are as follows1. The hip must be concentrically reduced. The cartilage of the surface of the
femoral head must lie directly on the cartilage of the floor of the acetabulum.
There can be no intervening space or soft tissue between them.
2. When step 1 is achieved, stability must be obtained so that when the leg is allowed
to move freely without support, it does not move away from the socket floor
(subluxate) or dislocate.
3. All of the dysplastic features of bone and cartilage must resolve. These are the
most long-lasting changes and the slowest to correct. For resolution to occur, the
femoral head must be stable and located in the acetabulum. These dysplastic
features include flattening of the femoral head, a misshapen acetabulum, and
abnormal shape of the proximal femur (excessive femoral anteversion and
increased neck-shaft angle).
All of these steps must be accomplished while avoiding complications. The two most
important complications are failure to achieve all of these goals and aseptic necrosis
(loss of the blood supply to the femoral head). Aseptic necrosis may be common, and
its outcome can be worse than the initial problem. Failure to achieve the three goals
also leads to a poor long-term outcome. Because of the progressive changes that occur

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over time, treatment options are frequently divided into age categories, but treatment
pertinent to the case would be discussed.
Persistent Dislocation: 18 months to the Age Limit
In this age group, closed reduction is less likely to succeed; most orthopaedist would
proceed straight to arthrography and open reduction.
Before the procedure of arthrography and open reduction, a period of traction will
help loosen the tissue and bring the femoral head down opposite the acetabulum.
However, some researchers have advocated femoral shortening rather than
preoperative skeletal traction, as the later has been associated with high incidence of
avascular necrosis, redislocation and poor results. Schoenecker et al. (1984) in their
study of 39 hips using the method of femoral diaphyseal shortening, first described by
Ombredane on 1923, reported no significant avascular necrosis or resultant deformity,
low rate of redislocation (8%) and less stiffness in the hip joint. They clearly
demonstrated that femoral diaphyseal shortening was preferable to traction as an aid
in the operative reduction of a DDH in children who were more than 3 years old.
Galpin et al. (1989) concluded that in the child who is 2 years or older, the treatment
of DDH with one stage femoral shortening, open reduction, capsulorraphy, and if
necessary pelvic osteotomy can produce reliable results and without an increase in the
risk of avascular necrosis. The limb-length discrepancy that is produced by the
shortening does not appear to cause a clinical problem.
In arthrography, a diluted radio-opaque contrast is injected into the unanaesthesised
child's hip. The arthrogram will clarify the anatomy of the hip. It may demonstrate an
obstruction to reduction. Typically, this is an enfolded limbus; however, a tight
inferior transverse ligament and capsular constriction, secondary to encroaching
iliopsoas, may be involved.

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The major indications of open reduction are ( i ) An older age. The likelihood of concentric reduction of an untreated dislocation,
without interposition of soft tissue, decreases and the prevalence of avascular
necrosis increases with closed treatment after a child has begun to walk.
Therefore, attempts at closed reduction should be limited to children who are
less than 3 years old.
( ii ) The failure of closed treatment to achieve or maintain a concentric reduction at
any age.
( iii ) The failure of previous open reduction.
There are a wide variety of operative approaches used in open reduction of DDH and
are found to be successful. These include medial and anterior approaches. They may
be combined with femoral shortening (as mentioned above), femoral derotation and
pelvic osteotomy.
There are two ways to approach the hip form the medial side: anteromedially and
posteromedially. The difference is the direction that the dissection takes with respect
to the adductor brevis muscle. The anteromedial approach proceeds anterior to the
adductor brevis muscle and either anterior or posterior to the pectinus, to expose the
iliopsoas and hip capsule. Typically a branch of the medial circumflex artery must be
ligated during the anteromedial approach. The posteromedial approach develops in the
interval between the adductor brevis and adductor magnus muscles directly to the hip
capsule.
The advantages of medial approach are ( i ) Simple, minimum dissection, bloodless access to the medial and inferior aspects
of the hip joint.
( ii ) Direct approach to the common obstacles to reduction before the child has begun
to walk, which are the psoas tendon, capsular constriction and transverse
acetabular ligament.

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The disadvantages are ( i ) Poor access to the acetabulum and its internal structures, such as the neolimbus,
ligamentum teres and pulvinar.
( ii ) In ability to plicate the capsule (capsular repair).
( iii ) Reconstructive procedure on the pelvis or femur cannot be accomplished.
This approach is not used in children who have begun to walk and when a dislocated
femoral head has migrated proximally.
The anterior approach (Smith-Patterson approach) is a popular, comprehensive
approach to the hip joint that provides superior exposure of the acetabulum, access to
intraarticular structures, ability to plicate the capsule, and access to the pelvis if pelvic
osteotomy is to be performed concurrently.
Dissection proceeds between the sartorius and tensor fascia late muscles, with release
of the sartorius form the anterior superior iliac spine and of the straight head of the
rectus from the inferior spine, to gain access to the hip capsule. This approach is
appropriate for any child of any age. The disadvantages are ( i ) Increased amounts of dissection and blood loss.
( ii ) More difficult in obtaining access to the iliopsoas and in exposure of the
transverse acetabular ligament.
After open reduction, the affected hip is immobilised in a cast for a period of 3
months or so. The affected hip is usually maintained in 90o of flexion and 30o of
abduction. A degree of internal rotation is usually required for congruous reduction.
During open reduction if excessive femoral anteversion (> 50 o) mandates substantial
internal rotation (typically more than 20-30o) to maintain the femoral had in the stable
zone, proximal femoral osteotomy may be performed. The procedure may be
combined with femoral shortening in children who are less than 3 years old.
Derotation osteotomy is virtually always combined with femoral shortening in older
children. Klisic et al. (1988) described this proceed in the study of long-term results of

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open reduction combined with femoral shortening, iliac osteotomy, reorientation of


upper femur and medial iliopsoas transfer in older children (ranging from 7-15 years).
They reported good results in two third of their patients. This procedure is not advised
after the appearance of pubertal signs.
Concomitant pelvic osteotomy to redirect the acetabulum in children who are older
than 18 months has been advocated. This procedure will increase the stable zone
particularly in patients who have substantial acetabular dysplasia. Pelvic osteotomy
combined with open reduction is an accepted practice and the major indication is the
need to increase the stable zone of the hip and accelerate the development of the
acetabulum towards normal.
Reduction of dislocation above the "age limit" (age where reduction of dislocation is
unwise as the risk of avascular necrosis is greatly increased) in unilateral dislocation
is about 10 years. The untreated hip is mobile, the patient limps but has little pain
until mid life (late third or early fourth decade). With bilateral dislocation, the
deformity and the waddling gait is symmetrical and therefore not so noticeable, thus
the risk of operative intervention is greater because failure on one side turns this into
asymmetrical deformity. So the "age limit" in this group is about 6 years. The
untreated patient as reported by Crawford et al. (1999) achieved a better quality of life
then many patients of similar age who have undergone surgical treatments aimed at
reducing such late presenting hip dislocations.
In conclusion, DDH represents a spectrum in time and severity, which helps to
explain the disease process and improve one's ability to properly screen for hip
abnormalities. Regardless of age and diagnosis the goals of treatment remain the
same: the femoral head must be reduced, it must be stable in the reduced position, and
all dysplastic features of bone and cartilage must resolve (while complications are
avoided). Early detection is important because these goals are easier to achieve at
younger ages with less intervention and a lower likelihood of complications.

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