Hip Dislocation in Patient with Down
syndrome: Case Report and Literature Review
Zaidman M1*, Katzman A2 and Eidelman M2
1Hadassah Medical Center, Jerusalem, Israel
2Rambam Medical Center, Jerusalem, Israel
Submission: March 15, 2019;Published: April 15, 2019
*Corresponding author: Zaidman M, Hadassah Medical Center, Jerusalem, Israel
How to cite this article: Zaidman M, Katzman A, Eidelman M. Hip Dislocation in Patient with Down syndrome: Case Report and Literature Review. Open Access J Surg. 2019; 10(3): 555789. DOI: 10.19080/OAJS.2019.10.555789.
Down syndrome or trisomy of chromosome 21 is considered to be the most common chromosome abnormality with the incidence of 1.7 of every 1000 live births [1,2]. The orthopaedic disorders are the next only to the congenital heart disease . Since the effective treatment of heart problems increased survival and life expectancy of the patients with Down syndrome [2-5] the management of orthopaedic disorders became of growing importance. The purpose of the article is to share our experience in treatment of fixed anterior hip dislocation with severe acetabulum dysplasia in child with Down syndrome and to summarize the literature data referred to the incidence of hip dislocation, predisposing factors, hip joint morphology, natural history, conservative and surgical treatment options and their outcomes.
Keywords: Down Syndrome; Hip Dislocation; Salter Osteotomy; Femoral Osteotomy.
9 years old girl with Down syndrome was admitted to our institution due to fixed right hip anterior dislocation. The girl’s mother stated her daughter had right hip dislocation at list once a day but was able to reduce it by her own. She was never referred to the doctor because of her hip dislocations before as it seemed not to cause any disability. Her medical history was remarkable for hypothyroidism treated with eltroxin.
Physical examination revealed prominent limping on ambulation, positive Galeazzi sign (Figure 1), femoral head could be palpated in the right groin region, and the girl was pain free throughout her right hip range of motions. She had generalized join laxity and muscle hypotonia. Radiological examination confirmed right hip dislocation. CT scan demonstrated anterior position of femoral head, shallow acetabulum with severe
anterior and posterior wall deficiency (Figures 2 & 3), and signs of soft tissue interposition. Acetabulum index was measured as 33 degree (Figure 4) and femoral anteversion as 70 degree.
As the attempt of closed reduction under general anaesthesia
was unsuccessful because of residual femoral head subluxation
and gross instability the girl underwent open reduction with
capsule plication, femoral varus derotational osteotomy (VDRO)
with one centimetre femoral shortening, Salter osteotomy and
one and a half hip spica application. In addition, the bone graft
strips were put on the capsule around the acetabulum (Figure 5).
Femur was derotated for thirty degree for femoral anteversion
correction. Neck shaft angle was ninety five degree after the
varus osteotomy. Paediatric locking hip plate (Synthes) was used
for femoral osteotomy fixation. The spica cast was removed in 8
weeks after the surgery. The hip joint remained stable on the last
follow up visit 12 month following the surgery.
Trisomy of chromosome 21 is considered to be the most
common chromosome abnormality with the incidence of 1.7 of
every 1000 live births [1,2]. Down syndrome is a multi-system
disorder. Forty to sixty per cent of patients sustain congenital
heart abnormalities .
The orthopaedic disorders are the next only to the congenital
heart disease  and mostly include cervical spine instability,
hip subluxation or dislocation, patellar instability, scoliosis,
metatarsus varus, pes planus [2-4,6]. Since operative survival
for congenital heart disorders and life expectancy dramatically
increased in recent years [2-5] the paediatric orthopaedic
surgeon more often has to face with the management of
orthopaedic problems which are usual cause of compromised
Shaw et al.  examined 114 patients with Down syndrome
with age ranged from ten to 43 years and found 7.9% of overall
incidence of hip abnormalities including dysplasia, avascular
necrosis or slipped capital femoral epiphysis. 2.9 % of the
patients sustained hip joint dysplasia or dislocation. Wolf et al.
 described the incidence of 1%-7% of hip instability in trisomy
21 patients. Bennet et al.  examined 220 institutionalised
patients aged 5 to 62 years (average 26 and a half years) and
reported the incidence of 4.5 per cents of hip dislocation. Hresko
et al.  found hip abnormality in 28% of 65 adults with Down
syndrome what was statistically correlated with ambulation
ability. 18% had hip dysplasia. The authors followed up by serial
examinations the subgroup of 18 patients and observed that hip
instability could occur in adulthood after skeletal maturity and
became worse with time.
Carter et al.  investigate the incidence of generalised joint
laxity in patients with congenital hip dislocation and in normal
schoolchildren. They found the generalised joint laxity in 7 per
cent of normal schoolchildren. The per cent was considerably
higher in those with congenital hip dislocation. Based on the
received data the authors inferred that generalised joint laxity
which is often familiar was an important predisposing factor
to congenital hip dislocation in boys but less important in
girls with exception of familiar cases. Joint laxity and muscles
hypotonia are well recognized features of Down syndrome. Most
of the authors postulated the generalized joint laxity and muscle
hypotonia to be the major predisposing factors of the most
of orthopaedics disorders including hip dislocation in Down
Livingstone et al.  in his report based on examination
of 39 children with Down syndrome related the increased
incidence of orthopaedic problems to muscle hypotonia rather
than to generalize joint laxity. Applying strict Carter and
Wilkinson criteria the authors didn’t reveal generalized joint
laxity in children with Down’s syndrome in their series. Shaw
et al.  suggested the joint laxity to be not predisposing but
causative factor. The authors believe that also patients with
Down syndrome may have hip dysplasia but more often the joint
laxity allows hip subluxation resulting in consequence dysplasia
and habitual or persistent dislocation.
Shaw et al.  studied the roentgenographic anatomy of
hip joints in 114 patients. The morphological features they
described were deeply seated in acetabulum femoral head, the
acetabulum roof – more horizontal. The neck – shaft angle was normal but femoral anteversion was moderately increased. One
can expect such anatomical features of hip joint to contribute to
femoral head stability in patients with Down syndrome.
Bennet et al.  also found the acetabulum of patients with
trisomy 21 to be deeper as compared with normal child. Wolf et
al.  described two cases of bilateral acetabular posterior wall
deficiency noted on three-dimensional reconstruction computed
tomography. Aprin et al.  considered that moderate to severe
femoral neck anteversion and increased neck shaft angle are
common feature of the hips in patients with Down syndrome.
The natural history of untreated hips in patients with
Down syndrome suggests progressive function deterioration
and secondary degenerative arthritis in adulthood. Untreated
unstable hips ultimately end up with fixed dislocation in the late
teens or early twenties [5,6].
That corresponds with the results of Bennet et al.  based
on examination of 220 patients with Down syndrome. According
to their data the hips were stable but hyper mobile until the age
of two years. Between two till ten years-habitual hip dislocation
may occur. Leaving untreated the instability may lead to
acute dislocation after the age of seven or eight years. Acute
dislocation could be easily reduced under anaesthesia. With time
containments is reduced and acetabulum dysplasia progressed.
Between late teens of early twenties fixed dislocation occurs but
pain usually is not a prominent feature .
Hresko et al.  reported increased incidence of hip
instability in adults with Down syndrome which progressed from
mild to severe subluxation or dislocation and can occur after
skeletal maturity. Turra et al.  described two untreated cases
of habitual monolateral hip dislocation in Down syndrome. In
one case the year after the onset of hip dislocation the posterior
wall smoothing was already obvious on CT scan. In the other case
habitual hip dislocation progressed from subluxation to fixed
dislocation at the end of 9 years follow up. Increased per cent
of hip instability and dislocation observed in adults with Down
syndrome  supports the progressive feature of the disorder.
Bennet et al.  adviced all children with trisomy 21
should be regularly tested for hip instability. The authors
considered closed reduction and hip spica immobilisation was
only occasionally successful. If the hip joint remained unstable
after 6 weeks of hip spica treatment the authors suggested the
operative treatment to be carried out. They recommended to
full fill capsular plication combined with pelvic or/and femoral
osteotomy. Also, the operative treatment was successful only
in fifty per cent of patients; the successfully treated patients
were doing much better than those left untreated. The best
results were if the operation was done at the stage of habitual
dislocation. If fixed dislocation was the case, the authors offered
leaving it alone as the results of operative treatment at that
stage were poor. If the pain was an issue in the phase of fixed
dislocation, the authors suggested the Schantz procedure to be
done. As a complication of surgical treatment – infection may be
Wolf et al.  described two cases of posterior acetabular
wall deficiency with hip dislocation in patients with Down
syndrome successfully addressed by modified Pemberton
osteotomy to provide better posterior wall coverage of femoral
head. The joints left stable more than ten years on the follow
up. Packer  reported 3 cases of recurrent hip dislocation
successfully treated by innominate osteotomy. Immobilisation
alone has not provided hip stabilization. One patient had Down’s
syndrome and all three children had generalised joint laxity.
Gore  treated the posterior hip dislocation in patient
with Down’s syndrome by capsule plications with a posterior
approach after failed attempts of spica cast immobilization.
The hip stayed stable during long term follow up. However, the
author could observe subsequent the femoral head subluxation
with secondary degenerative arthritis development in 20 years
Kirkos et al.  described multidirectional habitual hip
dislocation with no signs of hip dysplasia in a 3 years old girl with
Down syndrome successfully treated by hip spica application
for 12 weeks. The hip remained stable on the 12 months follow
up after spica removal. Greene  advocated for non-surgical
method of treatment of hip dislocation in patient with Down’s
syndrome by using the principle of prolong immobilization or
casting. He reported his experience of treatment of two children
(three hips) at the stage of bilateral habitual hip dislocation by
abduction orthosis full time for 6 months followed by night time
treatment for additional 4 months (the first case) and complete
unilateral hip dislocation addressed by closed reduction
and spica cast immobilization for 4 months and ambulatory
abduction orthosis for 8 month (the second case). The author
believed that non operative management could be successful and
avoids such complication as infection which is rather common in
patients with trisomy 21.
The increased infection rate after operative treatment was
mentioned by Berghof et al.  and occurred in 2 cases out of
7. The second or third operation was needed for consequent hip
instability following the primary surgery in the author’s series.
Bettuzzi et al.  described two cases of successful treatment
of habitual hip dislocation in 4 years old girl and symptomatic
hip subluxation in 11 years old girl with Down syndrome.
The authors recommended capsule plication, femur varus
derotational osteotomy combined with Pemberton osteotomy.
Aprin et al.  treated six patients with Down syndrome
(10 hips) with hip dislocation. The authors suggested closed
reduction and spica cast immobilization for the first dislocation
and operative treatment – for recurrent instability. If the
acetabulum was sufficient – the authors recommended capsule plication combined with varus derotational femoral osteotomy,
if the acetabulum was dysplastic – Salter Innominate osteotomy
should be added. Beguiristain et al.  offered femoral
derotational osteotomy with capsule plication as a treatment of
choice for early stages of hip dislocation in Down syndrome .
Trisomy 21 is the most common chromosome abnormality.
Due to improved management of heart problems life expectancy
of children with Down syndrome dramatically increased.
Orthopaedic surgeon has to face with management of orthopaedic
disorders mostly related to generalized joint laxity and muscle
hypotonia. One of the most significant abnormalities which can
compromise ambulating ability and to lead to painful condition
is hip joint instability. The natural history of hip instability is
often progression to habitual hip dislocation which ends up with
fixed hip dislocation and degenerative painful osteoarthritis.
Hip dysplasia is relatively rare. Although a few cases of
posterior wall insufficiency were reported, the acetabulum
tends to be deeper in patients with Down syndrome.
No one of the authors has submitted big enough amount
of clinical cases for evidence based conclusion and treatment
options recommendations. Most of the authors suggested
the attempt of conservative treatment by immobilization to
be carried out which could be successful in some cases. If
conservative treatment failed – the operative treatment should
be undertake and usually included pelvic and proximal femur
osteotomy with capsule plication. Better results were described
before the fixed hip dislocation occurred. High infection rate and
re-dislocation could be an issue.
We described the fixed anterior hip dislocation followed the
stage of habitual hip dislocation in 9 years old girl with Down
syndrome and classical presentation of generalized joint laxity
and muscle hypotonia. The challenge was combined severe
anterior and posterior acetabular wall deficiency. Anterior
direction of hip dislocation advocated for improving the anterior
coverage of femoral head, what could be achieved by innominate
osteotomy combined with varus derotational femoral osteotomy.
Because of posterior wall deficiency the main issues of
concern were not to create posterior femoral head instability
and dislocation by acetabulum reorientation with innominate
osteotomy and by over correction of femoral anteversion.
However, because of the anterior direction of hip dislocation
and gross anterior instability we decided to perform the Salter
osteotomy. We corrected 30 degree of anteversion. The posterior
hip stability was checked intraoperative. The operated hip
remained stable on the last follow up 12 months following the
Nine years old girl with Down syndrome sustained fixed
anterior hip dislocation combined with anterior and posterior
wall deficiency was successfully treated by Salter osteotomy,
VDRO, open reduction with capsule plication and placing the
“bone chips” around the capsule. We believe that chosen option
could be a treatment of choice for such complicated presentation.
The placement of the “bone chips” on anterior and posterior
capsule around the acetabulum could contribute to joint stability
in the cases of prominent acetabulum insufficiency. The femoral
shortening might be useful in the case of fixed hip dislocation.
The case discussion contributes to gained knowledge and
experience of hip joint instability management in patients with