Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency
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  • 作者:Akifusa Wada (1)
    Tomoyuki Nakamura (2)
    Toru Yamaguchi (2)
    Haruhisa Yanagida (2)
    Kazuyuki Takamura (2)
    Yutaka Oketani (1)
    Hideaki Kubota (1)
    Toshio Fujii (1)
  • 关键词:Kabuki syndrome ; Operative treatment ; Hip dislocation ; Open reduction ; Periacetabular osteotomy
  • 刊名:Journal of Children's Orthopaedics
  • 出版年:2012
  • 出版时间:August 2012
  • 年:2012
  • 卷:6
  • 期:4
  • 页码:261-267
  • 全文大小:593KB
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    2. Kuroki Y, Suzuki Y, Chyo H, Hata A, Matsui I (1981) A new malformation syndrome of long palpebral fissures, large ears, depressed nasal tip, and skeletal anomalies associated with postnatal dwarfism and mental retardation. J Pediatr 99:570-73 CrossRef
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    5. Schrander-Stumpel C, Meinecke P, Wilson G, Gillessen-Kaesbach G, Tinschert S, K?nig R, Philip N, Rizzo R, Schrander J, Pfeiffer L et al (1994) The Kabuki (Niikawa-Kuroki) syndrome: further delineation of the phenotype in 29 non-Japanese patients. Eur J Pediatr 153:438-45 CrossRef
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  • 作者单位:Akifusa Wada (1)
    Tomoyuki Nakamura (2)
    Toru Yamaguchi (2)
    Haruhisa Yanagida (2)
    Kazuyuki Takamura (2)
    Yutaka Oketani (1)
    Hideaki Kubota (1)
    Toshio Fujii (1)

    1. Department of Orthopaedic Surgery, Saga Handicapped Children’s Hospital, 2215-27 Kinryu, Kinryu-machi, Saga, 849-0906, Japan
    2. Fukuoka Children’s Hospital, Fukuoka, Japan
文摘
Purpose 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. Conclusion 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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