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01-08-2012 | Original Clinical Article

Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency

Authors: Akifusa Wada, Tomoyuki Nakamura, Toru Yamaguchi, Haruhisa Yanagida, Kazuyuki Takamura, Yutaka Oketani, Hideaki Kubota, Toshio Fujii

Published in: Journal of Children's Orthopaedics | Issue 4/2012

Abstract

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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Title: Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency
Authors: Akifusa Wada
Tomoyuki Nakamura
Toru Yamaguchi
Haruhisa Yanagida
Kazuyuki Takamura
Yutaka Oketani
Hideaki Kubota
Toshio Fujii
Publication date: 01-08-2012
Publisher: Springer Berlin Heidelberg
Published in: Journal of Children's Orthopaedics / Issue 4/2012
Print ISSN: 1863-2521
Electronic ISSN: 1863-2548
DOI: https://doi.org/10.1007/s11832-012-0426-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Surgical treatment of hip dislocation in Kabuki syndrome: use of incomplete periacetabular osteotomy for posterior acetabular wall deficiency

Abstract

Purpose

Methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Purpose

Methods

Results

Conclusion

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