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Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency.
[kabuki syndrome]
Kabuki
syndrome
is
characterized
by
distinctive
facial
features
,
skeletal
anomalies
,
persisting
fingertip
pads
with
dermatoglyphic
abnormalities
,
postnatal
growth
deficiency
and
mental
retardation
.
This
report
reviews
our
results
in
the
operative
treatment
of
hip
dislocations
in
patients
with
Kabuki
syndrome
.
Between
2001
and
2009
,
seven
dislocated
hips
(
three
unilateral
and
two
bilateral
hips
)
in
five
patients
(
all
girls
)
were
operatively
treated
at
our
institution
.
The
operative
treatment
consists
of
open
reduction
,
femoral
derotation
varus
osteotomy
,
pelvic
osteotomy
(
Salter
in
one
and
incomplete
periacetabular
osteotomy
in
six
hips
)
and
capsular
plication
.
The
age
of
the
patients
at
the
time
of
surgery
ranged
from
2
.
4
to
5
.
7
years
,
with
an
average
of
3
.
6
years
.
The
follow-up
postoperative
period
ranged
from
3
.
2
to
6
.
3
years
,
with
an
average
of
5
.
0
years
.
At
the
final
follow-up
,
all
patients
reported
no
click
and
no
pain
,
and
showed
well-contained
hips
by
radiographs
.
All
seven
hips
were
graded
as
Severin
class
I
-
II
.
One
patient
presented
as
having
habitual
dislocation
of
the
hip
4
.
4
years
after
surgery
.
Computed
tomographic
(
CT
)
scans
revealed
posterior
acetabular
wall
deficiency
,
which
was
not
corrected
by
the
anterolaterally
directed
Salter
osteotomy
.
The
incomplete
periacetabular
osteotomy
provided
sufficient
posterolateral
coverage
of
the
acetabulum
.
Operative
treatment
combining
open
reduction
,
femoral
derotation
varus
and
incomplete
periacetabular
osteotomies
,
and
capsular
plication
provided
successful
results
in
patients
with
Kabuki
syndrome
who
had
the
characteristics
of
hip
instability
such
as
ligamentous
laxity
,
muscular
hypotonia
and
posterior
acetabular
wall
deficiency
.
Diseases
Validation
Diseases presenting
"pain"
symptom
achondroplasia
acute rheumatic fever
adrenal incidentaloma
adrenomyeloneuropathy
aniridia
aromatase deficiency
carcinoma of the gallbladder
cholangiocarcinoma
coats disease
congenital diaphragmatic hernia
congenital toxoplasmosis
cushing syndrome
cutaneous mastocytosis
cystinuria
dedifferentiated liposarcoma
dentin dysplasia
dracunculiasis
dystrophic epidermolysis bullosa
epidermolysis bullosa simplex
erdheim-chester disease
erythropoietic protoporphyria
esophageal adenocarcinoma
esophageal carcinoma
esophageal squamous cell carcinoma
fabry disease
familial mediterranean fever
focal myositis
hirschsprung disease
hodgkin lymphoma, classical
holt-oram syndrome
junctional epidermolysis bullosa
kabuki syndrome
kindler syndrome
lamellar ichthyosis
liposarcoma
locked-in syndrome
lymphangioleiomyomatosis
malignant atrophic papulosis
neuralgic amyotrophy
oligodontia
oral submucous fibrosis
papillon-lefèvre syndrome
phenylketonuria
pleomorphic liposarcoma
primary hyperoxaluria type 1
proteus syndrome
pyomyositis
scrub typhus
sneddon syndrome
systemic capillary leak syndrome
thoracic outlet syndrome
trochlear dysplasia
typhoid
von hippel-lindau disease
waldenström macroglobulinemia
well-differentiated liposarcoma
wolf-hirschhorn syndrome
This symptom has already been validated