CLINICAL ORTHOPAEDICS AND RELATED RESEARCH
Number 457, pp. 247252
CASE REPORTS
Acetabular Dysplasia with Hip Subluxation in Trevors
Disease of the Hip
Alexandre Arkader, MD*; Jared E. Friedman, BA*; Leslie Moroz, BA*; and
John P. Dormans, MD*,
Dysplasia epiphysealis hemimelica (Trevors disease) is a
rare developmental bone disorder characterized by single or
multiple epiphyseal intraarticular lesions that are usually
unilateral and restricted to one side of the limb. Although the
process often is limited to the lower extremity, hip involve-
ment is not common. When it occurs it is more commonly
limited to the femoral side. We report a 14-year-old boy with
Trevors disease involving the hip, femoral and acetabular
sides, and with progressive dysplasia. We describe a new
surgical approach to this rare condition using a two-stage
procedure consisting of intraarticular resection of the lesion
followed by a modified shelf acetabuloplasty in a second
stage to prevent compromise of the proximal femur vascu-
lature, and to allow preservation and improvement of range
of motion of the hip. At 36 months followup the patient is
pain-free, has full range of motion, improved gait, and no
signs of recurrence. Although technically challenging, the
two-stage approach to Trevors disease seems a reasonable
and safe method.
Received: June 19, 2006
Revised: September 20, 2006
Accepted: November 10, 2006
From the *Division of Orthopaedics, The Childrens Hospital of Philadel-
phia, Philadelphia, PA; and the University of Pennsylvania School of Medi-
cine, Philadelphia, PA.
Each author certifies that he or she has no commercial associations (eg,
consultancies, stock ownership, equity interest, patent/licensing arrange-
ments, etc) that might pose a conflict of interest in connection with the
submitted article.
The authors certify that their institution has approved the reporting of this
case, that all the investigations were conducted in conformity with ethical
principles of research, and informed consent was obtained.
Correspondence to: John P. Dormans, MD, Division of Orthopaedic Surgery,
The Childrens Hospital of Philadelphia, 2nd Floor Wood Center, 34th &
Civic Center Blvd, Philadelphia, PA 19104-4399. Phone: 215-590-1534;
Fax: 215-590-1501; E-mail: dormans@email.chop.edu.
DOI: 10.1097/BLO.0b013e31802ea479
247
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2006 Lippincott Williams & Wilkins
Trevors disease is a rare developmental bone disorder
characterized by single or multiple epiphyseal, intraarticu-
lar lesions involving half the local joint area. These lesions
are usually unilateral and restricted to one side of the
limb.2,4,5,13 It usually is limited to the lower extremi-
ties, however, upper extremity lesions have been re-
ported.1,10,16,22,23,27,30 Patients typically present with a
painless swelling or mass, or less frequently with limb
deformity and asymmetric gait because of localized over-
growth of cartilage.14,25 Pain sometimes is present and can
be caused by degenerative joint disease or osteoarthritis
(OA) and mechanical impingement. Boys are affected
more frequently than girls.4,14,17
We report a 14-year-old boy with Trevors disease, in-
volving the hip (femoral and acetabular sides) causing
progressive hip dysplasia and describe a new method of
surgical treatment for this rare condition. We also provide
a concise review of the literature.
Case Report
A 14-year-old boy was referred with a 9-year history of
intermittent but progressive right hip pain and limp. The
patient reported experiencing pain during 50% to 60% of
his waking hours that ranged from 3 to 6 on a subjective
pain scale of 10. He previously was diagnosed with Legg-
Calv-Perthes disease at another institution where nonop-
erative treatment included observation and physical
therapy. The patient was engaged in competitive soccer,
but a recent increase in the severity of pain led him to
curtail this activity and seek medical advice.
At the time of his first visit, the patient walked with a
noticeable limp and had a positive Trendelenburg sign.
Examination of the right hip showed full range of flexion-
extension (11010), 25 internal rotation, external ro-
 Clinical Orthopaedics
248 Arkader et al and Related Research

tation to 15, and abduction to 25. There was some atro-

phy of his right thigh musculature. Pain was elicited with
palpation of the joint and greater trochanter region and
with extremes of range of motion (ROM). There was no
numbness, tingling, weakness, or other indications of neu-
rologic compromise. The patient was otherwise healthy
and denied history of fever, malaise, weight loss, chills,
night sweats, or pain during the night.
Plain radiographs showed an eccentric irregularly ossi-
fied mass located adjacent to the right femoral head, re-
sulting in an incongruent hip with a break of Shentons
line and insufficient acetabular coverage. However, the
joint space was maintained with no evidence of degenera-
tive joint disease (Fig 1).
A skeletal survey showed a similar eccentric mass with Fig 2AB. (A) Anteroposterior and (B) lateral radiographs of
irregularity and fragmentation arising from the right proxi- the right knee show irregularity and several bony fragments of
mal lateral epiphysis of the tibia, and also from the lateral the lateral distal femoral epiphysis and of the proximal lateral
distal femoral epiphysis (Fig 2). epiphysis of the tibia.
Frontal plane magnetic resonance (MR) images showed
an enlarged femoral head with a partially ossified and eccentric chondroid mass arising from the lateral proximal
femoral epiphysis (Fig 3). The MR images also confirmed
the acetabular dysplasia with a well-maintained joint
space. Axial computer tomography (CT) images showed a
cleavage plane on the posterior aspect of the epiphysis and
confirmed acetabular involvement by the process (Fig 4).
Although it seems that the acetabulum was directly in-
volved by the disease process, it is possible that those
changes were secondary to the femoral head deformity.
Because of the patients worsening pain despite nonop-
erative treatment and limited ROM attributable to femoral
head deformity, we recommended operative treatment an-

Fig 1AB. (A) Anteroposterior and (B) frog-lateral radiographs

of the hips show an irregularly ossified mass located on the Fig 3. A coronal MRI image shows a large cartilaginous mass
right femoral head, an incongruent hip, and insufficient ace- arising from the lateral proximal femoral epiphysis and con-
tabular coverage of the right femoral head. firms insufficient coverage of the femoral head.

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Number 457
April 2007
Fig 4. An axial CT image shows the cleavage plane on the
posterior aspect of the femoral epiphysis (arrow) and acetab-
ular involvement by the process with subluxation of the en-
larged femoral head.
ticipating progressive disease. We planned a two-stage
surgery to decrease the risks associated with the proximal
femur vasculature, and to allow recovery of function and
ROM. During the first stage a partial capsulotomy without
dislocation of the femoral head was performed through a
posterior approach, taking care to protect the vascular
structures of the proximal femur (ie, medial femoral cir-
cumflex and lateral ascending arterial branches). There
was a large amount of tumor exuding from the posterior
and lateral femoral head as noted from the imaging. The
diagnosis of dysplasia epiphysealis hemimelica was con-
firmed intraoperatively based on the gross and micro-
scopic (frozen section) appearance of the tumor. There
was substantial abutment and impingement of the Trevors
bone on the posterior rim of the acetabulum with extremes
of ROM including abduction and extension, which we
thought most likely were related to the patients pain. All
abnormal cartilage was removed with care to protect the
remaining normal acetabular articular cartilage. The joint
was inspected to remove any sequestrated cartilaginous
bodies. Careful capsular repair subsequently was done.
Immediately after surgery, a continuous passive motion
machine was used and continued for 4 weeks, during
which time the patient was allowed only toe-touch weight-
bearing. Six weeks after the first stage, he was fully
weightbearing, was essentially pain-free, and had im-
proved ROM.
The patient was pain-free approximately 6 months after
the initial stage. However, radiographs continued to show
evidence of severe dysplasia of the right hip with a center-
edge angle of 0 (Fig 5).
We performed the second stage to correct the right hip
dysplasia and prevent development of early OA. We per-
formed a modified shelf arthroplasty through an anterolat-
eral approach (curvilinear bikini-type skin incision). The
rectus femoris and its two heads were identified. The in-
direct head was Z-lengthened after being freed from the
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Trevors Disease of the Hip 249
Fig 5. An anteroposterior radiograph of the pelvis shows right
acetabular dysplasia with a center-edge angle of 0, compared
with 35 on the left (normal) side.
capsule. The capsule was further identified and a minicap-
sulotomy was performed to find the joint. The C-arm like-
wise was brought in and this iliac osteotomy-slot was
made using a combination of drills and high-speed burr.
An iliac crest bone graft was taken from the right iliac
crest and consisted of large cancellous sheets and cortical
sheets. These were placed into the acetabular slot such that
the concave side was facing down on the capsule to
achieve as much coverage as possible. The surgery was
uneventful. Radiographs taken 12 weeks after the second
stage showed good coverage of the femoral head and heal-
ing of the bone graft (Fig 6).
At the latest followup, 36 months after the second stage,
the patient had a much improved gait pattern with no limp
or Trendelenburg sign, was pain-free, and had improved
ROM (full extension and flexion, 30 abduction, 35 in-
ternal rotation, and 25 external rotation). Radiographs
showed no signs of recurrence of the lesion and confirmed
good coverage of the femoral head (Fig 7).
DISCUSSION
The French surgeons Mouchet and Belot first described an
intraarticular lesion characteristic of Trevors disease in
1926. They reported a case of tarsal bone involvement and
therefore called it tarsomegalie.20 In 1950, Trevor de-
scribed eight patients with ankle involvement and called
the condition tarso-epiphyseal aclasis.28 The term dyspla-
sia epiphysealis hemimelica, which explicitly describes
the characteristic involvement of either the medial or lat-
eral half of a single limb, was derived by Fairbank in 1956
after his review and report of Trevors patients.7
The incidence of Trevors disease is approximately one
in 1 million.17,18 However, this estimate may be artificially

250 Arkader et al
Fig 6AB. (A) Anteroposterior and (B) frog-lateral radiographs
of the hips 6 weeks after a shelf procedure on the right show
good containment of the femoral head.
low since many cases may go unrecognized because of a
similar histologic appearance to osteochondroma or be-
cause the lesion may be asymptomatic. The disease affects
males three times more frequently than females.4,14 Most
patients are diagnosed between the ages of 2 and 14
years.2,5,14
The etiology of the condition remains unknown. Theo-
ries about a genetic basis are speculative; there is no de-
finitive evidence of familial or hereditary transmis-
sion.4,7,14,17 The pathologic process is characterized by
asymmetric, abnormal proliferation of the cartilaginous re-
gion of the epiphysis. Because of the similar patho-
anatomy and histologic features of Trevors disease and
osteochondroma, the decision to classify a lesion as
Trevors disease is based primarily on the intraarticular
location of a lesion.2,7,13,19 The lesion or lesions typically
involve approximately 12 the epiphysis, although they may
involve the entire joint surface. In most patients the dis-
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Clinical Orthopaedics
and Related Research
Fig 7AB. (A) Anteroposterior and (B) frog-lateral radiographs
of the hips 36 months after a modified shelf procedure of the
right hip show correction has been maintained with good con-
tainment of the femoral head and no signs of recurrence.
ease is confined to one limb.7,8,13,14,19 The disorder usu-
ally is limited to the lower extremity, predominating
around the knee11,14 and ankle,14 however, there are iso-
lated reports of involvement of carpal bones,15,22,23,27,30
shoulder,5 wrist,23 hip,5,19,25 sacroiliac joint,24 patella,6
and calcaneous.13 At our institution, 15 children have been
diagnosed with Trevors disease during the past 20 years,
and the lower extremities were involved in all cases.
Although the diagnosis of Trevors can be presumed
based on history, physical examination, and imaging stud-
ies, a biopsy may be necessary for definitive diagnosis.
Histologically, the lesions are similar to those of solitary
osteochondromas or the exostosis seen in multiple heredi-
Number 457
April 2007
tary osteochondromatosis; however, Trevors disease
characteristically involves the epiphyses. Once the ossifi-
cation process begins the lesions are readily identified on
plain radiographs.
Trevors disease is characterized by a spectrum of
symptoms caused by the intraarticular location of the le-
sions. Patients may be asymptomatic or experience mild
discomfort, and walk with nearly normal gait or a limp. In
some cases an obvious deformity, limb-length discrep-
ancy, or decreased ROM are present.3,14,17,25 Articular
surface irregularity leading to early secondary OA, and
premature closure of the physis also have been de-
scribed.4,12,14
Successful imaging will identify the location and size of
the intraarticular mass. For most patients, plain radio-
graphs are sufficient to provide this information, however,
MRI and CT are helpful when the lesions have not begun
the process of ossification. Magnetic resonance imaging
also provides additional information regarding the extent
of the lesion, its relationship to the bone and soft tissues,
and the condition of the articular surface.11,21
A system for classifying Trevors disease was proposed
by Azouz et al.2 Their system differentiated localized
cases (single bone involvement) from classic (more than
one bone in one limb involved) and generalized cases
(entire limb involved).
The differential diagnoses are broad and include Olli-
ers disease, dysplasia epiphysealis multiplex, multiple he-
reditary exostosis, primary aseptic necrosis, aseptic necro-
sis, and chondrosarcoma, among others.5,1214 The inclu-
sion of Perthes disease in the differential diagnosis of
Trevors disease has not been addressed in the literature.
Bowen and Schmidt3 reported three cases of intraarticular
osteochondromas of the femoral neck in patients with
Legg-Calv-Perthes disease; however, the reported pa-
tients did not meet the clinical criteria for Trevors disease.
All lesions reported in their paper diminished in size dur-
ing the late phase of reossification and did not require
treatment.3 Our patient was diagnosed with Legg-Calv-
Perthes disease before his first visit at our institution. Ad-
ditional imaging and biopsy confirmed the diagnosis of
Trevors disease.
The treatment of children with Trevors disease should
be individualized and varies from observation to surgical
excision. Surgery is indicated for patients with increasing
pain, deformity, incongruence, intraarticular loose body
and decreased function.12,17 As reported by Herring, the
shelf arthroplasty, first described by Gill and later modi-
fied and popularized by Staheli et al,26 has been used for
the past several decades for the treatment of hip dysplasia,
Perthes, and other conditions to achieve improvement of
femoral head coverage by acetabular augmentation.9 This
Copyright Lippincott Williams & Wilkins. Unauthorized reproduction of this article is prohibited.
Trevors Disease of the Hip 251
procedure, however, has not been described for hip recon-
struction in patients with Trevors disease.
Tschauner et al29 described good results using the Pem-
berton acetabuloplasty to obtain femoral head coverage in
a 7-year-old child with Trevors disease. They stated MRI
should be used for diagnosing the early loss of contain-
ment and deformity of the femoral head. After a 4-year
followup, the femoral head remained contained with sec-
ondary congruency. Despite a decreased ROM, the patient
was fully participating in daily activities for his age. The
downside lies in the fact that the Pemberton osteotomy is
a volume-decreasing procedure and may not be indicated
for all cases of Trevors disease because often there is
femoral head deformity with development of coxa magna.
A two-stage approach seemed reasonable and safe for
our patient because it allowed him to fully recover from
the intraarticular resection of the disease. At followup this
enabled confirmation of an acetabuloplasty (ie, Trendelen-
burg gait and radiographic evidence of insufficient acetab-
ular coverage of the femoral head) that was performed in
a second stage. Although technically challenging, opera-
tive removal of an intraarticular mass was achieved in our
patient. Residual hip dysplasia was managed successfully
with a modified shelf arthroplasty.
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