title: Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Orthopaedic Surgery, Saga Handicapped Children's Hospital, 2215-27 Kinryu, Kinryu-machi, Saga, 849-0906 Japan.
authors.forename: Akifusa
authors.initials: A
authors.lastname: Wada
authors.forename: Tomoyuki
authors.initials: T
authors.lastname: Nakamura
authors.forename: Toru
authors.initials: T
authors.lastname: Yamaguchi
authors.forename: Haruhisa
authors.initials: H
authors.lastname: Yanagida
authors.forename: Kazuyuki
authors.initials: K
authors.lastname: Takamura
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Oketani
authors.forename: Hideaki
authors.initials: H
authors.lastname: Kubota
authors.forename: Toshio
authors.initials: T
authors.lastname: Fujii
keywords: Hip dislocation; Kabuki syndrome; Open reduction; Operative treatment; Periacetabular osteotomy
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