„ ASPECTS OF CURRENT MANAGEMENT
Congenital hip disease in adults
T. Karachalios,
G. Hartofilakidis
From University of
Thessalia, Larissa
and the University of
Athens, Athens,
Greece
„ T. Karachalios, MD, DSc,
Associate Professor
Orthopaedic Department
Faculty of Medicine, School of
Health Sciences, University of
Thessalia, Larissa 41110,
Greece.
„ G. Hartofilakidis, MD, FACS,
Professor Emeritus
Orthopaedic Department
National and Kapodistrian
University of Athens, KAT
Hospital, Nikis 2 Street, Kifisia,
14561 Athens, Greece.
Correspondence should be sent
to Dr T. Karachalios; e-mail:
kar@med.uth.gr
©2010 British Editorial Society
of Bone and Joint Surgery
doi:10.1302/0301-620X.92B7.
24114 $2.00
J Bone Joint Surg [Br]
2010;92-B:914-21.
914
TERMINOLOGY, CLASSIFICATION, PRE-OPERATIVE PLANNING
AND MANAGEMENT
This paper reviews the current knowledge relating to the management of adult patients
with congenital hip disease. Orthopaedic surgeons who treat these patients with a total hip
replacement should be familiar with the arguments concerning its terminology, be able to
recognise the different anatomical abnormalities and to undertake thorough pre-operative
planning in order to replace the hip using an appropriate surgical technique and the correct
implants and be able to anticipate the clinical outcome and the complications.
The use of ultrasound as a screening test in variety of the underlying pathology.9-11 We
the newborn has allowed earlier diagnosis favour the use of the term ‘congenital hip dis-
and treatment with a better prognosis for con- ease’ and its classification in infants as dyspla-
genital disease of the hip.1 Orthopaedic sur- sia, subluxation or dislocation.4,8 In adults, we
geons who specialise in adult reconstructive have described three types of the disease,
surgery often face the problem of osteoarthri- namely, dysplasia, low dislocation and high
tis secondary to congenital hip disease in dislocation based on radiological and intra-
adult patients. These cases are the result of operative criteria.9,12
late diagnosis and treatment in areas where For a classification system to be useful in clin-
early screening and treatment were not effec- ical practice, it should precisely describe the
tive or in patients in whom treatment had underlying pathological anatomy. It should also
failed in childhood.2 be able to predict the outcome of different
Treatment of the young adult with congeni- methods of treatment and be simple and accu-
tal hip disease by periacetabular or inter- rate to use. The classification proposed by
trochanteric osteotomy is effective, but Crowe, Mani and Ranawat13 is the most com-
technically challenging.3-5 Acetabular osteot- monly used system for congenital hip disease.
omy may be used in older patients with The main element of this system is the degree of
advanced degenerative disease secondary to displacement and the migration of the femoral
congenital disease.6 The management of these head and describes four types of dislocation.
patients with a total hip replacement (THR) The displacement is calculated on an antero-
presents difficulties because most are young posterior pelvic radiograph by measuring the
with considerable demand on their implants vertical distance between the inter-teardrop line
and they may require complex reconstruction and the junction of the femoral head and medial
on both sides of the joint. side of the femoral neck. The amount of dislo-
cation is the ratio between this distance and the
Terminology and classification vertical diameter of the undeformed femoral
There is a need for an agreed terminology neck. However, displacement and migration do
which covers the entire spectrum of congenital not describe the underlying pathology, and this,
deformity of the hip and a classification of its in our opinion, limits the use of Crowe’s classi-
types, both in infancy and adulthood, in order fication for the purpose of documentation
to improve communication, the planning of only.10,11 Other commonly used classification
treatment and the evaluation of the results of systems14,15 are shown in Table I.
different treatments. The term ‘developmental When dealing with these cases in the
dysplasia of the hip’, which is widely used by 1970s, before modern imaging techniques
orthopaedic surgeons throughout the world, were available for pre-operative planning, we
does not reflect the congenital origin of the realised that not all of such hips were the
condition,7,8 while the indiscriminate use of same from an anatomical point of view and
the word ‘dysplasia’ does not accord with the that different reconstruction techniques and
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 CONGENITAL HIP DISEASE IN ADULTS 915

Table I. Details of classification systems for congenital hip disease

Author/s Types of increasing severity Characteristic feature

Hartofilakidis et al9-12 Dysplasia Low Low High High Description of anatomical
dislocation B1 dislocation B2 dislocation C1 dislocation C2 abnormalities
Crowe et al13 I, < 50%; II 50% to 75% III, 75% to 100% IV, > 100% Proximal migration/height of
femoral head
Eftekar14 Dysplasia Intermediate High dislocation Old unreduced Degree of subluxation
dislocation dislocation
Kerboull15 Anterior Intermediate Posterior Direction of subluxation

Fig. 1a Fig. 1b Fig. 1c

Diagram of the three main types of congenital hip disease in adults showing a) dysplasia, b) low dislocation and c) high dislocation.

materials were required for different cases. Notes on the

intra-operative appearance of the hip were kept and com-
pared with the radiographs in order to improve our pre-
operative planning using conventional imaging. The
whole spectrum of the disease was classified into three
types (Fig. 1).2,9-12 In dysplasia (Fig. 2), the femoral head
is contained within the true acetabulum, there is a supe-
rior segmental defect and the fossa is covered by an osteo-
phyte which makes the acetabulum shallow.9-12 In a low
dislocation (Fig. 3), the femoral head articulates with a
false acetabulum which partially (to a varying degree)
covers the true acetabulum. Apart from the superior seg-
mental defect of the true acetabulum, there is an anterior
segmental defect and a narrow opening of inadequate
depth. Increased anteversion is seen in most cases. Occa-
sionally there is a lack of posterior bone stock. 2,9-12 In a
high dislocation (Fig. 4), the femoral head migrates supe-
Fig. 2
riorly and posteriorly in relation to the hypoplastic, trian-
gular true acetabulum. There is a segmental defect of the Three-dimensional CT scan of a dysplastic left hip after removal
of the femoral head. The segmental defect in the superior wall
entire rim, and the acetabulum is shallow with a narrow and the osteophyte covering the fossa are shown by arrows.
opening. There is also an abnormal build-up of bone pos-
terosuperiorly and excessive anteversion of the acetabu-
lum. The iliac wing is hypoplastic and anteverted.2,9-12
The proximal part of the femur is normal in dysplasia, narrowing of the canal and thin cortices. Occasionally,
but the femoral neck is short in a low dislocation, and there is a residual angular deformity of the proximal
shorter still in a high dislocation with excessive antever- femur because of a previous osteotomy which can make
sion (Fig. 5). The diaphysis is hypoplastic with excessive the reconstruction much more challenging.

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916 T. KARACHALIOS, G. HARTOFILAKIDIS

Fig. 3 Fig. 4

Three-dimensional CT scan of a left hip with a low dislocation Three-dimensional CT scan of a right hip with a high dislo-
after removal of the femoral head. The partial cover of the cation after removal of the femoral head. The triangular true
true acetabulum by the false acetabulum and the narrow acetabulum and the posterior abnormally distributed bone
opening of the true acetabulum are shown by arrows. stock are shown by a circle and an arrow, respectively.

Fig. 5a

Figure 5a - The radiological appearance and the corresponding three-dimensional CT scan with and without the femoral head in two sub types of low
dislocation showing a hip with extended cover (type B1). The false acetabulum is marked by a star and the true acetabulum by an arrow.

We have validated this classification system as have
others,16-18 and were satisfied that we could predict any
local abnormalities from pre-operative radiographs. Later,
we recognised, as a result of intra-operative problems with
reconstruction, that both the low and the high dislocations
could each be subdivided into two groups.19 In the B1 sub-
type of low dislocation there is extended cover of the true
acetabulum by the false acetabulum and in the B2 subtype
there is limited cover (Fig. 5). In the C1 subtype of high dis-
location there is a false acetabulum high on the iliac wing
and in the C2 subtype the femoral head lies within the
abductor musculature (Fig. 6).
Pre-operative planning and reconstruction
Prior to surgery, an AP radiograph of the pelvis is obtained
and in cases of low and high dislocation a CT of the
involved hip is performed as well.19 The size and location of
the segmental defects are estimated, as are the diameter and
depth of the acetabulum (useful for the estimation of the
required size of the acetabular component), the distribution
of bone stock and the anteversion of the acetabulum and
the femoral neck. The proximal femur is assessed using
conventional radiographs and templates to estimate the
type and size of the femoral component to be used for
reconstruction.

THE JOURNAL OF BONE AND JOINT SURGERY

 CONGENITAL HIP DISEASE IN ADULTS 917

Fig. 5b

Figure 5b - The radiological appearance and the corresponding three-dimensional CT scan with and without the femoral head in the two subtypes of
low dislocation showing a hip with limited cover (type B2). The false acetabulum is marked by a star and the true acetabulum by an arrow.

Fig. 6a

Figure 6a - The radiological appearance and the three-dimensional CT scan with and without the femoral head in the two subtypes of high dislocation
showing type-C1 high dislocation in which the false acetabulum is denoted by a star and the true acetabulum by an arrow.

The major technical difficulties encountered during a
THR for congenital hip disease are reconstruction of the
acetabulum in cases of low and high dislocation, implanta-
tion of the femoral component in a very narrow diaphysis
and in patients with a residual angular deformity of the
femur. We suggest that an osteotomy of the greater tro-
chanter is included in the approach to the joint. This not
only makes access easier, but may also restore the bio-
mechanics if the trochanter is advanced distally thereby
increasing the power of the abductor mechanism.20 Most
surgeons currently use trochanteric osteotomy only in
selected cases, mainly difficult primary and revision THRs.
However, in our opinion, in patients with congenital dis-
location of the hip, especially in low and high dislocations
and in some stiff hips with dysplasia, it helps to avoid seri-
ous complications and gives a better result. For mechanical
reasons, we favour placing the acetabular component at the
level of the true acetabulum (Fig. 7).21,22 However, it is not
always possible to achieve cover of the acetabular compo-
nent with host bone at this level. In cases in which the
reamed acetabulum can provide osseous cover of at least
80% (as it is estimated inter-operatively) we prefer an
uncemented metal-backed acetabular component (Fig. 7).
The size of these components is often small, 40 mm to
42 mm, and in order to avoid problems with thin poly-
ethylene liners,23 (including TK) some authors currently
advocate the use of monobloc components or alternative
bearing surfaces such as ceramic-on-ceramic (Fig. 8a).24,25
Good medium-term results have recently been reported
using resurfacing arthroplasty in patients with dysplasia.26

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918 T. KARACHALIOS, G. HARTOFILAKIDIS

Fig. 6b

Figure 6b - The radiological appearance and the three-dimensional CT scan with and without the femoral head in the two subtypes of high dislocation
showing a type-C2 high dislocation with no false acetabulum; the femoral head is free-floating within the gluteal muscles. The true acetabulum is
marked by an arrow.

Fig. 7a Fig. 7b Fig. 7c

Radiographs of alternative hip reconstructive techniques showing a) cementless acetabular and femoral components with high
placement of the cup, b) cementless components with anatomical placement of the cup and the use of structural bone autograft
and c) a cementless acetabular component (anatomical position) combined with a thin cemented femoral component.

When it is not possible to use an uncemented acetabular
component, the cotyloplasty technique is a good alterna-
tive.9,10 This involves medialisation of the acetabular floor
by the creation of a cemented comminuted fracture of the
entire medial wall, impaction of autogenous bone graft and
the implantation of a small, cemented all-polyethylene
acetabular component (Figs 8b and 8c).9,10
Augmentation of superior segmental defects with struc-
tural autograft or allograft and the placement of the acetab-
ular component in the anatomical position have been
suggested (Fig. 7b).27,28 Although the short-term results of
this technique are excellent, a high failure rate has been
reported after approximately 12 years.28 This may be
related to the complex pathological anatomy of the true
acetabulum and the abnormal distribution of stresses com-
bined with the unfavourable long-term behaviour of struc-
tural grafts. It has been suggested that this technique gives
satisfactory results when at least 70% of the acetabular
component is supported by the host bone.29 High place-
ment of the component in the region of the false acetabu-
lum has also been proposed.27,30 The problem with this
technique is that with the acetabular component at this
level, the lever arm for the body-weight is much longer than
that of the abductors, and causes excessive loading of the
hip. Also, the shearing forces acting on the acetabular com-
ponent can lead to early loosening. In unilateral cases a
high acetabular component does not correct leg-length and
leaves the patient with a limp.
We initially used a Charnley cemented femoral compo-
nent exclusively for the reconstruction of the proximal

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 CONGENITAL HIP DISEASE IN ADULTS 919

Fig. 8a Fig. 8b Fig. 8c

Radiographs of alternative reconstructive techniques showing a) the use of a monobloc tantalum trabecular metal
acetabular component combined with a short conical cementless femoral component, b) cotyloplasty (the original tech-
nique) with the use of an all-polyethylene offset-bore acetabular component combined with a thin cemented femoral
component and c) cotyloplasty (modern version) with a monobloc tantalum trabecular metal acetabular component com-
bined with an ordinary cementless femoral component.

femur. Later, various small cemented components were also
used (Figs 7 and 8). Several authors prefer uncemented fem-
oral components (Figs 7 and 8).31,32 In our opinion, the
principles and goals of uncemented fixation with optimal
fit and fill of the canal, initial stability and adequate bone
growth are not easily achieved in a narrow femoral canal
with a thin cortex.33 One author (TK) currently performs
the same procedure using a short cementless conical distal
bearing component (Fig. 8a). Custom-made femoral com-
ponents have also been used.34
For hips with a high dislocation we favour reduction of
the components by shortening the femur with progressive
resection of bone from the femoral neck. We argue against
leaving the greater trochanter in place and shortening the
femur by subtrochanteric osteotomy35-37 because in most
cases of high dislocation the greater trochanter lies above
the centre of rotation of the femoral head. This makes tro-
chanteric osteotomy and advancement essential. Subtro-
chanteric osteotomy may also lead to undesirable
complications. Despite this, some surgeons continue to per-
form shortening osteotomies (either subtrochanteric or dia-
physeal) with or without a trochanteric osteotomy. These
techniques have been practiced over the years without clear
indications for one or the other based on comparative
results. It seems that detachment of the trochanter with
shortening of the proximal metaphysis is indicated for
proximal malformations which are difficult to treat; a mid-
diaphyseal osteotomy is indicated when there is a concom-
itant severe valgus deformity of the knee, while a subtro-
chanteric shortening osteotomy has broader indications.29
When planning a shortening osteotomy, a CT of the pelvis
and lower limbs is essential to measure the femora accu-
rately and thus the true leg-length discrepancy. It has been
found that in up to 30% of cases of low and high disloca-
tion the affected femur is longer than that on the opposite
side.36,38 Rarely, a two-level osteotomy is required to
reduce the joint and equalise leg-lengths.
Clinical outcome
THR is the last resort for the treatment of an adult with
osteoarthritis secondary to congenital hip disease. The
young age of the patients combined with the altered mor-
phology of the hip, and consequent lack of bone stock may
result in a higher rate of a failure39 and a worse functional
outcome.29 It should be stressed that the interpretation of
the published results is difficult because most series include
patients with many different types of hip disease.
Charnley and Feagin40 stated that THRs should be
avoided in patients with congenital dislocation of the hip
and inadequate bone stock. Despite this early discourage-
ment, surgeons have attempted this procedure, developed
their technique and reported their results. Overall, mid- and
long-term failure rates vary from 7.7% to 44.8% in non-
homogeneous series.13,27,31,41-47 In five homogeneous series
with high dislocations there was a failure rate of 25% in a
group of 87 patients at a mean follow-up of 10 years,48 of
17% in a smaller group of 28 patients at a mean follow-up
of 9.4 years,49 of 10% in a group of 83 patients with a
mean follow-up of eight years,35 and of 95% in a group of
52 patients with a mean follow-up of 12.3 years.32 In a
recent study of 28 patients with Crowe type-IV hips treated
with an uncemented implant and a subtrochanteric osteot-
omy, there was only one failure of the acetabular compo-
nent but a high complication rate after a mean follow-up of
4.8 years.50 In our original series of 229 THRs, dysplastic
hips performed as well as primary osteoarthritic hips with a
survival rate of 90% at 15 years whereas in hips with low
and high dislocation the survival rate was only 75% at the
15-year follow-up.12,51 Femoral components performed
better in patients with a low dislocation while acetabular

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920 T. KARACHALIOS, G. HARTOFILAKIDIS
components performed better in those with a high disloca-
tion.12 Chougle, Hemmady and Hodgkinson52 reported
similar findings for the long-term behaviour of the acetab-
ular component in patients with a high dislocation.
Recent studies from centres in which these procedures
are performed in sufficient numbers show satisfactory long-
term clinical results with limited complication rates, even
for patients with a high dislocation.12,31,32,35,36,39,43,46,47
Orthopaedic surgeons who treat adult patients with con-
genital hip disease should be familiar with its terminology,
be able to recognise the different anatomical abnormalities,
be able to carry out thorough pre-operative planning,
reconstruct the hip using the appropriate surgical technique
and implants and finally be able to anticipate the clinical
outcome and avoid complications.
Supplementary material
A figure showing anatomical abnormalities seen in
high dislocation is available with the electronic ver-
sion of this article on our website at www.jbjs.org.uk
No benefits in any form have been received or will be received from a commer-
cial party related directly or indirectly to the subject of this article.
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