aspects of current management congenital hip disease in … · true acetabulum by the false...

914 THE JOURNAL OF BONE AND JOINT SURGERY

ASPECTS OF CURRENT MANAGEMENT

Congenital hip disease in adultsTERMINOLOGY, CLASSIFICATION, PRE-OPERATIVE PLANNING AND MANAGEMENT

T. Karachalios, G. Hartofilakidis

From University of Thessalia, Larissa and the University of Athens, Athens, Greece

T. Karachalios, MD, DSc, Associate ProfessorOrthopaedic DepartmentFaculty of Medicine, School of Health Sciences, University of Thessalia, Larissa 41110, Greece.

G. Hartofilakidis, MD, FACS, Professor EmeritusOrthopaedic DepartmentNational and Kapodistrian University of Athens, KAT Hospital, Nikis 2 Street, Kifisia, 14561 Athens, Greece.

Correspondence should be sent to Dr T. Karachalios; e-mail: [email protected]

©2010 British Editorial Society of Bone and Joint Surgerydoi:10.1302/0301-620X.92B7. 24114 $2.00

J Bone Joint Surg [Br] 2010;92-B:914-21.

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 inthe newborn has allowed earlier diagnosisand treatment with a better prognosis for con-genital disease of the hip.1 Orthopaedic sur-geons who specialise in adult reconstructivesurgery often face the problem of osteoarthri-tis secondary to congenital hip disease inadult patients. These cases are the result oflate diagnosis and treatment in areas whereearly screening and treatment were not effec-tive or in patients in whom treatment hadfailed in childhood.2

Treatment of the young adult with congeni-tal hip disease by periacetabular or inter-trochanteric osteotomy is effective, buttechnically challenging.3-5 Acetabular osteot-omy may be used in older patients withadvanced degenerative disease secondary tocongenital disease.6 The management of thesepatients with a total hip replacement (THR)presents difficulties because most are youngwith considerable demand on their implantsand they may require complex reconstructionon both sides of the joint.

Terminology and classificationThere is a need for an agreed terminologywhich covers the entire spectrum of congenitaldeformity of the hip and a classification of itstypes, both in infancy and adulthood, in orderto improve communication, the planning oftreatment and the evaluation of the results ofdifferent treatments. The term ‘developmentaldysplasia of the hip’, which is widely used byorthopaedic surgeons throughout the world,does not reflect the congenital origin of thecondition,7,8 while the indiscriminate use ofthe word ‘dysplasia’ does not accord with the

variety of the underlying pathology.9-11 Wefavour the use of the term ‘congenital hip dis-ease’ and its classification in infants as dyspla-sia, subluxation or dislocation.4,8 In adults, wehave described three types of the disease,namely, dysplasia, low dislocation and highdislocation based on radiological and intra-operative criteria.9,12

For a classification system to be useful in clin-ical practice, it should precisely describe theunderlying pathological anatomy. It should alsobe able to predict the outcome of differentmethods of treatment and be simple and accu-rate to use. The classification proposed byCrowe, Mani and Ranawat13 is the most com-monly used system for congenital hip disease.The main element of this system is the degree ofdisplacement and the migration of the femoralhead and describes four types of dislocation.The displacement is calculated on an antero-posterior pelvic radiograph by measuring thevertical distance between the inter-teardrop lineand the junction of the femoral head and medialside of the femoral neck. The amount of dislo-cation is the ratio between this distance and thevertical diameter of the undeformed femoralneck. However, displacement and migration donot describe the underlying pathology, and this,in our opinion, limits the use of Crowe’s classi-fication for the purpose of documentationonly.10,11 Other commonly used classificationsystems14,15 are shown in Table I.

When dealing with these cases in the1970s, before modern imaging techniqueswere available for pre-operative planning, werealised that not all of such hips were thesame from an anatomical point of view andthat different reconstruction techniques and

CONGENITAL HIP DISEASE IN ADULTS 915

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materials were required for different cases. Notes on theintra-operative appearance of the hip were kept and com-pared with the radiographs in order to improve our pre-operative planning using conventional imaging. Thewhole spectrum of the disease was classified into threetypes (Fig. 1).2,9-12 In dysplasia (Fig. 2), the femoral headis 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 lowdislocation (Fig. 3), the femoral head articulates with afalse 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 anteriorsegmental defect and a narrow opening of inadequatedepth. Increased anteversion is seen in most cases. Occa-sionally there is a lack of posterior bone stock. 2,9-12 In ahigh dislocation (Fig. 4), the femoral head migrates supe-riorly and posteriorly in relation to the hypoplastic, trian-gular true acetabulum. There is a segmental defect of theentire rim, and the acetabulum is shallow with a narrowopening. 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,but the femoral neck is short in a low dislocation, andshorter still in a high dislocation with excessive antever-sion (Fig. 5). The diaphysis is hypoplastic with excessive

narrowing of the canal and thin cortices. Occasionally,there is a residual angular deformity of the proximalfemur because of a previous osteotomy which can makethe reconstruction much more challenging.

Table I. Details of classification systems for congenital hip disease

Author/s Types of increasing severity Characteristic feature

Hartofilakidis et al9-12 Dysplasia Lowdislocation B1

Low dislocation B2

High dislocation C1

High dislocation C2

Description of anatomicalabnormalities

Crowe et al13 I, < 50%; II 50% to 75% III, 75% to 100% IV, > 100% Proximal migration/height of femoral head

Eftekar14 Dysplasia Intermediatedislocation

High dislocation Old unreduced dislocation

Degree of subluxation

Kerboull15 Anterior Intermediate Posterior Direction of subluxation

Fig. 1b

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

Fig. 1a Fig. 1c

Fig. 2

Three-dimensional CT scan of a dysplastic left hip after removalof the femoral head. The segmental defect in the superior walland the osteophyte covering the fossa are shown by arrows.

916 T. KARACHALIOS, G. HARTOFILAKIDIS

THE JOURNAL OF BONE AND JOINT SURGERY

We have validated this classification system as haveothers,16-18 and were satisfied that we could predict anylocal abnormalities from pre-operative radiographs. Later,we recognised, as a result of intra-operative problems withreconstruction, that both the low and the high dislocationscould each be subdivided into two groups.19 In the B1 sub-type of low dislocation there is extended cover of the trueacetabulum by the false acetabulum and in the B2 subtypethere is limited cover (Fig. 5). In the C1 subtype of high dis-location there is a false acetabulum high on the iliac wingand in the C2 subtype the femoral head lies within theabductor musculature (Fig. 6).

Pre-operative planning and reconstructionPrior to surgery, an AP radiograph of the pelvis is obtainedand in cases of low and high dislocation a CT of theinvolved hip is performed as well.19 The size and location ofthe segmental defects are estimated, as are the diameter anddepth of the acetabulum (useful for the estimation of therequired size of the acetabular component), the distributionof bone stock and the anteversion of the acetabulum andthe femoral neck. The proximal femur is assessed usingconventional radiographs and templates to estimate thetype and size of the femoral component to be used forreconstruction.

Fig. 3

Three-dimensional CT scan of a left hip with a low dislocationafter removal of the femoral head. The partial cover of thetrue acetabulum by the false acetabulum and the narrowopening of the true acetabulum are shown by arrows.

Fig. 4

Three-dimensional CT scan of a right hip with a high dislo-cation after removal of the femoral head. The triangular trueacetabulum and the posterior abnormally distributed bonestock 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 lowdislocation showing a hip with extended cover (type B1). The false acetabulum is marked by a star and the true acetabulum by an arrow.


VOL. 92-B, No. 7, JULY 2010

The major technical difficulties encountered during aTHR for congenital hip disease are reconstruction of theacetabulum in cases of low and high dislocation, implanta-tion of the femoral component in a very narrow diaphysisand in patients with a residual angular deformity of thefemur. We suggest that an osteotomy of the greater tro-chanter is included in the approach to the joint. This notonly makes access easier, but may also restore the bio-mechanics if the trochanter is advanced distally therebyincreasing the power of the abductor mechanism.20 Mostsurgeons currently use trochanteric osteotomy only inselected 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 dislocationsand in some stiff hips with dysplasia, it helps to avoid seri-

ous complications and gives a better result. For mechanicalreasons, we favour placing the acetabular component at thelevel of the true acetabulum (Fig. 7).21,22 However, it is notalways possible to achieve cover of the acetabular compo-nent with host bone at this level. In cases in which thereamed acetabulum can provide osseous cover of at least80% (as it is estimated inter-operatively) we prefer anuncemented metal-backed acetabular component (Fig. 7).The size of these components is often small, 40 mm to42 mm, and in order to avoid problems with thin poly-ethylene liners,23 (including TK) some authors currentlyadvocate the use of monobloc components or alternativebearing surfaces such as ceramic-on-ceramic (Fig. 8a).24,25

Good medium-term results have recently been reportedusing resurfacing arthroplasty in patients with dysplasia.26

Fig. 5b

Figure 5b - The radiological appearance and the corresponding three-dimensional CT scan with and without the femoral head in the two subtypes oflow 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 dislocationshowing type-C1 high dislocation in which the false acetabulum is denoted by a star and the true acetabulum by an arrow.



When it is not possible to use an uncemented acetabularcomponent, the cotyloplasty technique is a good alterna-tive.9,10 This involves medialisation of the acetabular floorby the creation of a cemented comminuted fracture of theentire medial wall, impaction of autogenous bone graft andthe implantation of a small, cemented all-polyethyleneacetabular 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 beensuggested (Fig. 7b).27,28 Although the short-term results ofthis technique are excellent, a high failure rate has beenreported after approximately 12 years.28 This may berelated to the complex pathological anatomy of the trueacetabulum 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 givessatisfactory results when at least 70% of the acetabularcomponent 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 thistechnique is that with the acetabular component at thislevel, the lever arm for the body-weight is much longer thanthat of the abductors, and causes excessive loading of thehip. Also, the shearing forces acting on the acetabular com-ponent can lead to early loosening. In unilateral cases ahigh acetabular component does not correct leg-length andleaves the patient with a limp.

We initially used a Charnley cemented femoral compo-nent exclusively for the reconstruction of the proximal

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 dislocationshowing a type-C2 high dislocation with no false acetabulum; the femoral head is free-floating within the gluteal muscles. The true acetabulum ismarked by an arrow.

Fig. 7a

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

Fig. 7b Fig. 7c


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femur. Later, various small cemented components were alsoused (Figs 7 and 8). Several authors prefer uncemented fem-oral components (Figs 7 and 8).31,32 In our opinion, theprinciples and goals of uncemented fixation with optimalfit and fill of the canal, initial stability and adequate bonegrowth are not easily achieved in a narrow femoral canalwith a thin cortex.33 One author (TK) currently performsthe same procedure using a short cementless conical distalbearing component (Fig. 8a). Custom-made femoral com-ponents have also been used.34

For hips with a high dislocation we favour reduction ofthe components by shortening the femur with progressiveresection of bone from the femoral neck. We argue againstleaving the greater trochanter in place and shortening thefemur by subtrochanteric osteotomy35-37 because in mostcases of high dislocation the greater trochanter lies abovethe centre of rotation of the femoral head. This makes tro-chanteric osteotomy and advancement essential. Subtro-chanteric osteotomy may also lead to undesirablecomplications. Despite this, some surgeons continue to per-form shortening osteotomies (either subtrochanteric or dia-physeal) with or without a trochanteric osteotomy. Thesetechniques have been practiced over the years without clearindications for one or the other based on comparativeresults. It seems that detachment of the trochanter withshortening of the proximal metaphysis is indicated forproximal 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 pelvisand lower limbs is essential to measure the femora accu-rately and thus the true leg-length discrepancy. It has beenfound 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 toreduce the joint and equalise leg-lengths.

Clinical outcomeTHR is the last resort for the treatment of an adult withosteoarthritis secondary to congenital hip disease. Theyoung age of the patients combined with the altered mor-phology of the hip, and consequent lack of bone stock mayresult in a higher rate of a failure39 and a worse functionaloutcome.29 It should be stressed that the interpretation ofthe published results is difficult because most series includepatients with many different types of hip disease.

Charnley and Feagin40 stated that THRs should beavoided in patients with congenital dislocation of the hipand inadequate bone stock. Despite this early discourage-ment, surgeons have attempted this procedure, developedtheir technique and reported their results. Overall, mid- andlong-term failure rates vary from 7.7% to 44.8% in non-homogeneous series.13,27,31,41-47 In five homogeneous serieswith high dislocations there was a failure rate of 25% in agroup of 87 patients at a mean follow-up of 10 years,48 of17% in a smaller group of 28 patients at a mean follow-upof 9.4 years,49 of 10% in a group of 83 patients with amean follow-up of eight years,35 and of 95% in a group of52 patients with a mean follow-up of 12.3 years.32 In arecent study of 28 patients with Crowe type-IV hips treatedwith 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 of4.8 years.50 In our original series of 229 THRs, dysplastichips performed as well as primary osteoarthritic hips with asurvival rate of 90% at 15 years whereas in hips with lowand high dislocation the survival rate was only 75% at the15-year follow-up.12,51 Femoral components performedbetter in patients with a low dislocation while acetabular

Fig. 8a

Radiographs of alternative reconstructive techniques showing a) the use of a monobloc tantalum trabecular metalacetabular 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 femoralcomponent and c) cotyloplasty (modern version) with a monobloc tantalum trabecular metal acetabular component com-bined with an ordinary cementless femoral component.

Fig. 8b Fig. 8c



components performed better in those with a high disloca-tion.12 Chougle, Hemmady and Hodgkinson52 reportedsimilar findings for the long-term behaviour of the acetab-ular component in patients with a high dislocation.

Recent studies from centres in which these proceduresare performed in sufficient numbers show satisfactory long-term clinical results with limited complication rates, evenfor 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 techniqueand implants and finally be able to anticipate the clinicaloutcome and avoid complications.

Supplementary materialA figure showing anatomical abnormalities seen inhigh 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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aspects of current management congenital hip disease in … · true acetabulum by the false...

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