TECHNIQUE
Femoral Varus Derotation Osteotomy for the Treatment
of Habitual Subluxation and Dislocation of the Pediatric
Hip in Trisomy 21: A 10-Year Experience
Dominique M.A. Knight, MBBS, BSc, FRCS (Tr&Orth), Cristina Alves, MD, FEBOT,
and John H. Wedge, OC, MD, FRCSC
Background: Habitual hip subluxation and dislocation are
potentially disabling features of the trisomy 21 syndrome. We
describe outcomes after a femoral varus derotation osteotomy to
achieve and maintain hip stability and community ambulation.
Methods: All individuals with trisomy 21, who underwent hip
surgery at our institution between 1998 and 2008, were searched
using the hospital databases. The clinical notes and radiographs
were reviewed from presentation to final follow-up. Nine
children (16 hips) aged below 10 years, were identified. All
had a femoral varus derotation osteotomy with a target femoral
neck-shaft angle (NSA) of 105 degrees and external rotation of
<20 degrees of the distal fragment. All were performed by the
senior author.
Results: Mean age at first known hip dislocation was 4.6 years
(range, 4 to 5.2 y), mean age at surgery was 6.1 years (range, 5.2
to 7.0 y), and mean follow-up was 5.4 years (range, 3.8 to 7.1 y).
Mean NSA fell postoperatively to 106.0 degrees (range, 103.1 to
110.2 degrees) from 166.7 degrees (range, 162.2 to 171.1
degrees). In 2 hips, intraoperative instability remained, requiring
immediate periacetabular osteotomy and capsulorraphy. Post-
operatively, all patients demonstrated an asymptomatic wad-
dling gait, which persisted in 1 individual. Fourteen hips
developed peritrochanteric varus deformities with a mean center
of rotation and angulation of 21 degrees (range, 16 to 25
degrees). Two hips (12.5%) sustained implant-related fractures 4
and 8 years postoperatively. One hip (6.3%) developed arthritis
and none had redislocated at latest follow-up.
Conclusions: Sequelae from recurrent subluxation or dislocation
of hips in trisomy 21 may require surgery to prevent eventual
disability. We recommend a varus producing proximal femoral
osteotomy correcting the NSA to approximately 105 degrees.
This should be performed before the age of 7 years or a widened
or V-shape teardrop develops. After 2 implant-related fractures,
we recommend implant removal once the osteotomy has healed
From the Division of Orthopaedic Surgery, Hospital for Sick Children,
University Avenue, Toronto, Ontario, Canada.
Conflict of Interest and Investments: All authors declare that there are
neither competing interests nor investments and therefore have
nothing to declare.
Reprints: Dominique M.A. Knight, MBBS, BSc, FRCS (Tr&Orth),
Nuffield Orthopaedic Centre, Windmill Road, Headington, OX3
7LD, Oxford, Oxfordshire, OX3 8NE, UK. E-mail: dmaknight@
rcsed.ac.uk.
Copyright r 2011 by Lippincott Williams & Wilkins
638 | www.pedorthopaedics.com
and the hip stabilized. In our experience, this approach is
effective in maintaining hip stability.
Level of Evidence: A level 4 study, looking at a specific patient
population undergoing a particular procedure.
Key Words: trisomy 21, hip dislocation, hip subluxation,
surgical treatment, varus femoral derotation osteotomy
(J Pediatr Orthop 2011;31:638–643)
T risomy 21, originally described in 1866, is the most
common chromosomal abnormality with an incidence
of 1 in 795 live births.1 Improvement in management of
health issues associated with this disease, including
congenital heart defects, has contributed to a decreased
mortality of these children. The life expectancy of a
1-year-old child with trisomy 21 is between 43 and 55
years and is proportional to the degree of mental
disability.2,3 Increasing survival will inevitably be asso-
ciated with a rise in orthopaedic problems.
Musculoskeletal manifestations characteristic of
trisomy 21 include hip and patellar instability, genu
valgum, pes planus, metatarsus primus varus, atlanto-
axial instability, and scoliosis.4 These children demon-
strate hypotonia, ligamentous laxity, and hypermobile
joints in up to 76%,5 although some reports suggest an
incidence closer to 10%.6
Development of hip abnormalities in trisomy 21 is a
well known phenomenon. The spectrum includes habitual
dislocation (both voluntary and involuntary), progressive
subluxation, and acute dislocation. This hip pathology is
an entirely different entity to that of developmental
dysplasia of the hip. Acetabular dysplasia is uncommon
and typically these patients have an increased femoral
neck-shaft angle (NSA) producing a valgus femoral neck.
Progressive hip subluxation or habitual dislocation tends
to develop after learning to walk, a milestone often
delayed but reached within the first decade.7 If sympto-
matic, these hips will not only affect patients’ quality of
life but their ability to live independently in the commu-
nity as adults.8
Management of the habitually subluxating and
dislocating hip in trisomy 21 aims to prevent degenerative
arthritis and long-term disabling pain. Hresko et al8
J Pediatr Orthop  Volume 31, Number 6, September 2011
J Pediatr Orthop  Volume 31, Number 6, September 2011
found that as hips progressed from mild-to-severe
subluxation or dislocation, the deterioration in walking
often precluded community-based living. In an institu-
tionalized population of 65 patients with trisomy 21, they
found 28% had radiographic abnormalities in one or
both hips.8
We describe the features of habitual hip dislocation
in children with trisomy 21 and report a series of
surgically treated trisomy 21 hips in a population aged
below 10 years. Our technique primarily addresses
femoral neck valgus, the main pathologic feature we
believe to be responsible for hip instability in those with
habitual dislocation.
All children were treated by the senior author by a
proximal femoral varus derotation osteotomy (VDRO),
correcting the NSA to approximately 105 degrees. The
amount of derotation performed was never more than 20
degrees. Typically, it was much less even if the estimated
intraoperative increase in anteversion was greater as these
children tend to walk with an externally rotated gait due
to hypotonia and ligamentous laxity. This technique was
successful in restoring and maintaining hip stability in all
16 hips, and therefore maintained the patients’ ability to
remain community ambulators.
METHODS
All individuals with trisomy 21, who had undergone
hip surgery at our institution between 1998 and 2008,
were identified using the hospital databases.
Sixteen involved hips in 9 individuals aged below 10
years were reviewed. Children older than 9 years had not
been recommended for surgery as they have incongruent
or lateralized hips secondary to triradiate physeal growth
arrest. These older patients have been found, in unpub-
lished study, to have unfavorable outcomes of even
combined pelvic and femoral osteotomies. In our patient
cohort, 1 individual, patient 4, turned 10 while waiting for
surgery.
The clinical notes and radiographs, from initial
presentation to final follow-up, were reviewed. Presenting
symptoms were hip “clicking” or “popping,” which
represented voluntary or involuntary habitual hip sub-
luxation, dislocation, and spontaneous dislocation.
From all available radiographs, the NSA, center-
edge angle, acetabular index, and Smith ratio were
calculated preoperatively and postoperatively. All com-
plications were recorded.
Surgical Technique
After administration of a general anesthetic, the
patient was positioned supine on a radiolucent operating
table. Under fluoroscopy, the hip was screened to
determine the rotational profile. This confirmed the
degree of true femoral neck valgus and anteversion.
Further, with the hip abducted and internally rotated, it
also helped to determine the amount of correction to
restore Shenton arc and centralize the femoral head. A
lateral subvastus femoral approach exposed the proximal
femur and osteotomy site. A 90-degree angled blade plate
r 2011 Lippincott Williams & Wilkins
Femoral Varus Derotation Osteotomy
was used to create a varus producing osteotomy. The
target NSA was 105 degrees; however, with leg rotation
the true value may be between 100 and 110 degrees. This
amount of varus of the distal fragment was sufficient to
centre the femoral head within the acetabulum.
After the osteotomy fixation, hip stability was
intraoperatively evaluated. If subluxation persisted, a
periacetabular osteotomy and capsulorraphy were per-
formed and cancellous bone placed around the capsule to
induce fibrosis. In this series, 2 hips remained unstable
intraoperatively after the VDRO. This was due to
posterior acetabular deficiency and immediate stabiliza-
tion with a periacetabular osteotomy and capsulorraphy
was achieved.
All patients were immobilized postoperatively in a
double hip spica or Petrie cast for 6 to 8 weeks, after
which all restrictions on activity were removed. This
immobilization maintained hip stability and aimed to
break the behavioral habit of dislocating; although at the
conclusion of surgery, no hips could be dislocated
manually.
Statistical Methods
Continuous variables were assessed for normality
with the d’Agostino Pearson test. Normally distributed
variables are presented as means with 95% confidence
intervals.
Preoperative and postoperative means were com-
pared using the Student t test for paired samples.
RESULTS
Seven male and 2 female patients with 16 hips were
treated (Table 1). The mean age at first known hip
dislocation was 4.6 years (range, 4 to 5.2 y). All patients
were referred with habitual dislocation (Fig. 1A, Fig. 2A),
either voluntary or involuntary, and 1 spontaneous acute
hip dislocation reduced by the father.
All patients had a VDRO (Fig. 1B; Fig. 2B). Mean
age at surgery was 6.1 years (range, 5.2 to 7 y) and mean
follow-up was 5.4 years (range, 3.8 to 7.1 y). No hips
redislocated by the time of last follow-up.
The mean preoperative NSA was 167 degrees
(range, 162 to 171 degrees) and the mean postoperative
NSA was 107 degrees (range, 103 to 110 degrees). The
mean Smith ratio was 0.8 preoperatively (range, 0.7 to
0.9) and postoperatively 0.8 (range, 0.8 to 0.8). The mean
preoperative acetabular index was 18 degrees (range, 15
to 21 degrees) and postoperatively 19 degrees (range, 16
to 21 degrees).
Complete hip dislocation was present in 2 hips
preoperatively. In those not dislocated, the mean center-
edge angle was 22 degrees preoperatively (range, 13 to 31
degrees) and 30 degrees (range, 26 to 33 degrees)
postoperatively.
In 2 hips, intraoperative instability persisted due to
posterior acetabular wall deficiency. In these, a periace-
tabular osteotomy and capsulorraphy with cancellous
bone placed around the capsule to induce fibrosis were
also done.
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 TABLE 1. Patient Data
Knight et al

Preop/ Preop/Postop Preop/ Symptoms Secondary

Pt. Age at Presenting FU Postop/ Acetabular Postop at Last Surgical
No. Sex Surgery Side Complaint Surgery Year Final NSA Index Smith Ratio Follow-up Complications Interventions
1 M 7 Left Habitual Open Reduction, 9 L: 180/122/ L: 30/24 L: 1/ 0.78 Left Left Trendelenburg None
dislocation VDRO+acetabulo- 131 Trendelen- Left mild proximal femoral
plasty burg bowing
2 M 5 Bilateral Habitual VDRO 9 L: na/110/ L: na/ 16 L: na/ 0.81 None Superficial wound infection ORIF for left
dislocation— 108 R: na/ 22 R: na/ 0.87 Rx proximal
failed brace R: na/ with antibiotics. Bilateral femur implant

640 | www.pedorthopaedics.com
106/135 proximal femoral bowing. related
Left proximal femoral plate
fracture fracture+right
8 years after the index hardware
surgery, proximal to blade removal
plate
3 M 5 Bilateral Habitual VDRO 9 L: 166/101/ L: 17/25 L: 0.7/ 0.83 None Superficial wound infection Bilateral hardware
dislocation 100 R: 14/22 R: 0.73/ Rx with PO antibiotics. removal
R: 161/ 0.78 Proximal femoral bowing.
106/108 Prominent hardware
4 F 10 Left Habitual Open Reduction, 8 L:168/115/ L:21/49.2 L: 0.93/ 0.75 Pain Left hip arthritis Referred for hip
dislocation— VDRO+acetabulo- 100 arthroplasty
failed brace plasty
5 M 3 Bilateral Right hip pain. VDRO 8 L: 162/100/ L: 15/14 L: 0.73/ 0.83 Right knee Left mild proximal femoral None
Left habitual 145 R: 15/18 R: 0.82/ pain bowing
dislocation R: 159/ 0.78
112/150
6 F 5 Bilateral Habitual VDRO 5 L: 176/104/ L: 14/24 L: 0.83/0.88 Pain over GT Prominent hardware None
dislocation 111 R: 11/9.8 R: 0.75/ Proximal femoral bowing
R: 175/ 0.75
104/100
7 M 6 Bilateral Habitual VDRO 4 L: 168/104/ L: 21.2/22 L: 0.66/ 0.64 None Bilateral proximal femoral ORIF for left
J Pediatr Orthop

dislocation 137 R: 18/22 R: 0.65/ varus bowing+left proximal



R: 160/96/ 0.64 proximal femoral fracture, femur implant

122 4 years after related
the index surgery fracture+right

r
hardware
removal
8 M 4 Bilateral Habitual VDRO 2 L: na/103/ L: na/14.4 L: na/ 0.82 None Proximal femoral varus None
dislocation 112 R: na/12.4 R: na/ 0.78 bowing
R: na/
101/111
9 M 5 Bilateral Spontaneous VDRO 2 L: 162/114/ L: 15/17.8 L: 1/ 0.87 None Prominent hardware None
dislocation 103 R: 22/13.7 R: 0.73/ Proximal femoral varus
R: 163/ 0.77 bowing
108/110
F indicates female; GT, greater trochanter; L, left; M, male; NSA, neck-shaft angle; ORIF, open reduction and internal fixation; Postop, postoperative; Preop, preoperative; PO, per oral; Pt, patient; R, right; VDRO,
varus derotation osteotomy.

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Volume 31, Number 6, September 2011
J Pediatr Orthop  Volume 31, Number 6, September 2011
FIGURE 1. A, Habitual dislocation of the left hip. B, Same
patient 8 years after varus derotation osteotomy.
During the postoperative follow-up, 14 hips (88%)
developed an acquired varus deformity in the proximal
femur (Fig. 2C). This center of rotation and angulation
was peritrochanteric, at the level or just proximal to the
blade of the plate, with a mean center of rotation and
angulation of 21 degrees (range, 16 to 25 degrees). This
was a distinct entity from the surgical varus created at the
osteotomy site.
Complications
Two hips (12.5%) sustained an implant-related
fracture, one 62 months postoperatively and the second 55
months postoperatively. Both cases had prominent hard-
ware, a varus femoral bow proximal to the plate and cystic-
like lesions within the proximal femur (Fig. 2D, Fig. 3A, B).
After open reduction and internal fixation using locked
plates, recovery was uneventful (Fig. 2E).
r 2011 Lippincott Williams & Wilkins
Femoral Varus Derotation Osteotomy
Two hips (12.5%) developed superficial wound
infections successfully treated with a short course of oral
antibiotics.
All patients developed a waddling gait postopera-
tively secondary to the femoral varus osteotomy, which
shortened and weakened the abductor mechanism. This
gait pattern was anticipated and improved over 24 to 36
months postoperatively in all but 1 who was asympto-
matic at the time of final review despite a mild waddling
gait. Patient 4 developed hip arthritis (6.3%).
DISCUSSION
Children with trisomy 21 have 1% to 7% risk of
recurrent hip instability.9,10 If untreated, these hips
progress to subluxation, chronic dislocation, and acet-
abular dysplasia. The natural history is development of a
fixed dislocation in the late teens or early 20s.7,11 After
maturity, the trisomy 21 hip may become unstable.
Hresko et al8 reported a 36% incidence (4 of 11 patients)
of late presenting hip subluxation in previously
normal hips.
Cristofaro and Donovan12 reported a 10% inci-
dence of osteoarthritis in an adult population with
trisomy 21. Kioschos et al13 performed total hip
arthroplasties on 6 such patients, with a mean age of 36
years, all had disabling arthritis. These studies highlight
the importance of early intervention to prevent an
unfavorable outcome.
Prevention of dislocating or recurrently subluxing
hips in trisomy 21 patients should include surgery to
increase hip containment. There is, however, very little
information regarding the management of these challen-
ging hips available in the literature.
Various osteotomies with variable outcomes have
been used to manage hip instability in patients with
trisomy 21 syndrome.6,7,14 These children have character-
istic findings on pelvic radiography including broad
acetabular wings, reduced iliac, and acetabular angles
with well-formed deep acetabulae.15 Shaw et al10 found
that these patients have acetabulae that are deeper and
more horizontal than the normal population. They also
found an increase in coexisting hip disease, including
Perthes, slipped capital femoral epiphysis, avascular
necrosis, habitual dislocation, and dysplasia. An in-
creased range of hip motion, particularly external
rotation was believed to contribute to this increased
incidence10 and was a consistent feature in our patients.
Children with trisomy 21 are sometimes braced or
treated with hip spicas to prevent ongoing habitual
dislocation. Greene2 reports successful use of an ambula-
tory abduction orthosis after 8 and 10 months of treatment
in 2 patients (3 hips). One hip required closed reduction and
hip spica immobilization for 4 months before application
of the brace. All hips were stable at review, 2 years and
10 years after treatment. This technique may certainly assist
behavior modification but has not been shown in a larger
series to prevent recurrence.
www.pedorthopaedics.com | 641
Knight et al
FIGURE 2. A, Patient 7 preoperative radiographs, (B) One year postoperative, (C) Proximal femoral bow 3 years after varus
derotation osteotomy. D, Left implant-related fracture. E, Fracture fixation with a locked plate.
In those individuals younger than 7 years with
congruent hips, a VDRO with adequate varization is
often sufficient to address the femoral deformity. Under
fluoroscopic screening, we found the main femoral neck
deformity to be in the coronal rather than the sagittal
plane, therefore a valgus osteotomy was the primary
correction required. We aimed for an intraoperative NSA
of 105 degrees accepting 100 to 110 degrees to be a
realistic goal. This allowed for the difficulties in accurate
NSA measurement secondary to thigh positioning pre-
operatively, intraoperatively, and during follow-up
assessment. If excessive anteversion was present, correc-
tion of this was undertaken cautiously and in all cases
<20 degrees of external rotation was required. This may
seem counter-intuitive in a hip with apparent increased
external rotation. However, much is due to hypermobility
and ligamentous laxity in the joints and soft tissues and
not from excessive retroversion of the femoral neck. If too
much external derotation is performed to the distal
fragment, the hip then becomes at risk of subluxating or
dislocating posteriorly due to the varying presence of
posterior acetabular deficiency. Reducing the femoral
NSA to this range, with minimal derotation was found in
our series to stabilize the hip in most cases (88%).
Those in whom intraoperative instability persists,
an immediate periacetabular osteotomy and capsulorra-
phy may be performed. Cancellous bone placed around
the capsule induces fibrosis, preventing capsular restretch-
ing; this has not caused excessive stiffness in our
experience. In our series, 2 of 16 hips required this
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J Pediatr Orthop  Volume 31, Number 6, September 2011
additional stabilization due to posterior acetabular
deficiency. These patients were noted to be older, aged 7
and 10 years, and their postoperative NSAs were 115 and
122 degrees, the least achieved in our series. Varus
correction to >110 degrees (our mean was 106 degrees) is
likely to be insufficient as the neck valgus recurs with
growth. Alternatively, creating an NSA of <90 degrees
significantly alters hip biomechanics, which may be
poorly tolerated. The child will have a marked waddle,
poor walking distance due to abductor fatigue, and this
amount of varus does not remodel.
In children aged 7 years and older, repeated trauma
from habitual dislocation damages the triradiate growth
center and the hip becomes increasingly lateralized. This
incongruence will not remodel, causes abnormal loading,
early osteoarthritis, and pain in adolescence. Early
surgical intervention should be considered because after
hip congruence is lost, a solitary femoral osteotomy is
insufficient. This was reflected in our series, with a pelvic
procedure required in those patients who presented for
surgery aged 7 years or older. The outcome here became
less predictable. After 10 years of age, the teardrop has
often become grossly deformed and we have had little
success with combined femoral and acetabular osteo-
tomies.
Aprin et al14 reviewed 6 patients with trisomy 21,
although the series was small with a short follow-up, our
findings reflect theirs. They found that patients aged
below 5 years had normal acetabulae and a varus femoral
osteotomy was sufficient to achieve excellent or good
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J Pediatr Orthop  Volume 31, Number 6, September 2011
FIGURE 3. A, Proximal femoral bow and intraosseous cystic
lesions. B, Postimplant removal.
results. Those aged 9 to 13 years had dysplastic
acetabulae and required acetabular procedures, with no
excellent results. We agree this surgery should be under-
taken before 10 years of age and for a solitary femoral
osteotomy, we believe ideally before 7 years of age.
During routine review, we observed a secondary
varus deformity in the proximal femur in 14 of the 16 hips
and in some, cystic-like lesions of the proximal femur. In
2 hips, a fracture of the femur proximal to the entry point
of the blade plate occurred. These were both treated with
removal of the blade plate and fixation of the fracture
with a locking plate (Fig. 2D, E) followed by elective
removal of the contralateral blade plate, if present
(Fig. 3B).
After our implant-related fractures, we now recom-
mend routine removal of the femoral plates with
restricted activities and wheelchair use for 6 weeks.
Removal is delayed until the hip is stabilized and the
osteotomy healed, approximately 9 to 12 months from
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Femoral Varus Derotation Osteotomy
surgery. Removing the implant aims to eliminate the
major factor, which may cause a pathologic fracture.
Patients with trisomy 21 have reduced bone density
and bone turnover is increased, when compared with the
general population.16 This, alongside the known multiple
endocrine abnormalities effecting bone, may explain the
postoperatively acquired femoral bow and intraosseous
cystic lesions observed in the presence of a retained implant
in some of our cohort. Further study is needed to assess this
within this subgroup of the pediatric population.
We believe that management of the subluxated or
dislocated trisomy 21 hip should include surgery for a
predictable result. We recommend a varus producing
proximal femoral osteotomy to correct the NSA to 100 to
110 degrees. This should optimally be performed before
7 years of age or signs of a widened or V-shape teardrop
develop. In our experience, this has been effective in
maintaining hip stability.
Little is known about the natural history of habitual
hip dislocation in these children. Patients with trisomy 21
are living longer with hips that are at ongoing risk.
Thought should be given to the potential benefits of
periodic screening of this treatable hip condition in the
trisomy 21 population at least until skeletal maturity.
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