Hip Int 2010 ; 20 ( 04 ): 559 - 561

CASE REPORT

Total hip arthroplasty in a patient with arthrogryphosis

and an ipsilateral above knee amputation
Michael Leonard, Paul Nicholson

Department of Orthopaedic Surgery, Adeladie and Meath Incorporating the National Childrens Hospital, Tallaght, Dublin
- Ireland
Department of Orthopaedic Surgery, Adeladie and Meath Incorporating the National Childrens Hospital, Tallaght, Dublin - Ireland

Abstract. The authors present the case of a young man with arthrogryphosis multiplex congenita and
an above knee amputation who underwent an ipsilateral total hip replacement. The unique aspects of
the case and technical difficulties are highlighted. Follow-up at five years revealed an excellent clinical
and radiological outcome.

Key Words: Above knee amputation, Arthrogryphosis, Total hip replacement

Accepted: September 17, 2010

Introduction CASE REPORT

The term ‘arthrogryphosis multiplex congenita’ represents A 36-year-old man with a right above knee amputation
a large heterogeneous group of conditions characterised presented with a 1 year history of disabling right hip pain.
by multiple joint abnormalities and contractures (1). The His pain was constant in nature, waking him from sleep
overall prevalence is reported as one in 3000 live births and impeding his ability to work. His pain was resistant
(2). The anatomical distribution and severity of the condi- to conservative measures.
tion can vary widely, with over 300 different disorders de- The patient had arthrogryphosis predominantly affecting
scribed (3). The etiology may result from decreased foetal his right lower limb. He had undergone several operations
movements secondary to various factors such as abnormal as a child and young adult including multiple corrective
connective tissue, neuropathic and myopathic processes, osteotomies, right knee and ankle arthrodeses, trifocal os-
maternal diseases and/or impaired foetal vascularity (4). teotomies and leg lengthening of 10 centimetres using a
Multiple corrective orthopaedic surgical procedures have circular frame (Fig. 1).
been described for both upper and lower limb deformities Unfortunately, he subsequently developed areas of pain-
(1). Acetabular dysplasia is a common finding (5). The re- ful ulceration in his pre-tibial area which proved unre-
ports of hip arthroplasty in this patient group consist main- sponsive to conservative care. An above knee amputa-
ly of small numbers within larger series of complex primary tion was performed and he was fitted with a prosthesis
arthroplasties undertaken for acetabular dysplasia (6). – a quadrilateral socket with a swing control knee and
There is little published information hip arthroplasty in multiaxsis foot in which he ambulated well prior to de-
patients with lower limb amputations. The altered anat- veloping his hip symptoms.
omy poses technical challenges to the surgeon includ- Examination of his right hip and stump revealed a hyp-
ing a reduced lever arm for dislocation/relocation, the oplastic thigh with multiple scars, all of which were well
amount of remaining bone length and bone fragility due healed. He had a fixed flexion deformity of 15 0 , flexion
to osteoporosis. to 700 and virtually no rotation. Plain radiographs re-

© 2010 Wichtig Editore - ISSN 1120-7000 559

Total Hip Arthroplasty in Above Knee Amputation

Fig. 1 - Full-length ra-

diograph of the lower
limbs showing evidence
of multiple previous ope-
rations and retained bro-
ken metal pins.

Fig. 2 - Pre-operative pelvic radiograph demonstrating a dysplastic

arthritic right hip.

Fig. 3 - Pelvic radiograph at 5 year follow-

vealed arthritis and acetabular dysplasia (Fig. 2). up.
Judet views confirmed advanced dysplasia and a comput-
erised tomography (CT) demonstrated that the remaining
femur was of sufficient diameter and length to accommo-
date available implant sizes.
Under epidural anaesthesia and in the lateral decubitus po-
sition an antero-lateral approach to the hip was performed.
The patient had a retained broken pin in the distal femur
from previous surgery. A 5 mm Steinman pin on a large
T-handle was inserted form the lateral side through a stab
incision proximal to this. The purpose of this construct was
to facilitate dislocation/relocation and to optimise rota-
tional control of the stump. The T-handle construct proved
adequate control to allow for dislocation and acetabular extrusion and adequate pressurisation. The use of a long
preparation, however intra-operative concern arose re- uncemented stem allowed us to address these issues suc-
garding the amount of torque required to allow for femoral cessfully. The modularity in the system also allowed us to
preparation. Subperiosteal dissection to the level of the optimise the implant height and version.
lesser trochanter was then performed to expose this area On the acetabular side a 46 mm uncemented shell plus
of more solid bone. A bone clamp was then placed at this two supplementary screws was combined with a 28 mm
level and this in addition to the T-handle pin construct was polyethelyene liner (Pinnacle bantam, Depuy Orthopae-
adequate to provide sufficient and safe torque to allow for dics, Warsaw, In, USA).
broaching of the femoral canal. The post-operative course was complicated by a significant
A modular uncemented SROM stem with a 28 mm metal amount of stump swelling. Once his incision had healed his
femoral head (Depuy Orthopaedics, Warsaw, In, USA) was swelling was successfully managed with a stump shrinker
used on the femoral side. The iatrogenic creation of a de- for two weeks. He then underwent successful rehabilitation
fect in the femoral cortex (with insertion of the Steinmann in his prosthesis. At follow-up 5 years after surgery he was
pin) and the defect in the distal femur (even in the pres- noted to be pain free, back at work and ambulating well. His
ence of a plug) raised concerns with regards to cement radiographs at latest follow-up were satisfactory (Fig. 3).

560 © 2010 Wichtig Editore - ISSN 1120-7000

Leonard and Nicholson
DISCUSSION
The reported incidence of hip arthritis in patients with low-
er limb amputations is between 5 and 61% (7, 8). It may
occur in either hip but is more frequent on the amputated
side, and is both more frequent and more severe in above
knee amputations (8). The literature on hip arthroplasty in
this patient population consists of a small number of case
reports and short series which are devoted to patients with
below knee amputations. In the single published article of
surgical intervention for hip arthritis in the presence of an
ipsilateral above knee amputation Gills et al reported suc-
cessful results with hemiresurfacing in four patients (9).
Appropriate implant positioning can be difficult in amputees
due to both altered anatomy and difficulties with exposure
(10). The successful use of the SROM prosthesis in difficult
primary total hip arthroplasties has been extensively re-
ported (6, 11). Its use in this case allowed us to both avoid
the hypothetical problems with cementation and to address
implant height and version which in this case proved invalu-
able due to the associated acetabular dysplasia.
Total hip arthroplasty in the presence of an ipsilateral
above knee amputation is a challenging procedure, par-
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© 2010 Wichtig Editore - ISSN 1120-7000
ticularly so in this case due to significant actetabular
dysplasia, arthrogryphosis and multiple previous opera-
tive procedures. The main challenge posed by the am-
putation was control of the lower extremity, in particular
the application of traction and/or rotation. We recom-
mend preparing and draping the entire extremity and in-
sertion of a pin in the distal femur to improve rotational
control. This can be further enhanced by placing a bone
clamp more proximally if required. The use of a modular
uncemented stem may avoid cementation problems and
allow for optimal implant positioning.
Informed written consent obtained from patient.
No financial support received.
No conflicts of interest.
Address for correspondence:
Michael Leonard
Department of Orthopaedic Surgery
Adeladie and Meath Incorporating the National Childrens Hospital
Tallaght
Dublin 24, Ireland
mikeleonard77@gmail.com
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