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 Â years , with an average of 3 .6 Â years .The follow-up postoperative period ranged from 3 .2 to 6 .3 Â years , with an average of 5 .0 Â 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 Â 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 .

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Diseases presenting "follow-up postoperative period" symptom

kabuki syndrome

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