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

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.

Neglected subluxation of the hip after trauma: A case report

“…The posterior instability of the hip caused by a defect in the posterior acetabular wall was treated by IPO. IPO involves pelvic osteotomy developed for hip dislocation in children with strong defects of the posterior acetabular wall, and it enables proper acetabular roof coverage of the lateral and posterior sides [ 9 ]. However, at 2 years after surgery, a difference of 21 mm in the length of the legs persisted ( Fig.…”

Diagnosis and Management of Hip Dislocation in Patients with Kabuki Syndrome

“…The hip dislocations in Kabuki syndrome were managed in a similar manner to neglected developmental dysplasia of the hip and the results were satisfactory. Wada et al16) also reported the treatment outcomes of five Kabuki syndrome children with seven hip dislocations. Five hip dislocations were diagnosed before 10 months of age, and nonoperative treatment (closed reduction) resulted in successful reduction and maintenance.…”

“…The major pathology of hip dislocation in Kabuki syndrome is ligamentous laxity16) as in Down syndrome, Ehlers-Danlos syndrome, and Larsen syndrome. According to the literature, hip dislocations of Down syndrome are associated with ligamentous laxity, excessive femoral anteversion, and posterior acetabular wall deficiency 20).…”

“…They suggested that hip dislocation associated with ligamentous laxity requires more bony stability of the hip joint. Wada et al16) reported that the acetabular deficiency in Kabuki syndrome patients was more posterior and performed an incomplete periacetabular osteotomy to provide sufficient posterolateral coverage. We agree that if the hip joint remains unstable or dysplastic, bony stabilization is indicated.…”

“…The incidence of hip dislocations in Kabuki syndrome ranges from 18% to 62% 515). Some studies showed that most of the hip dislocations in Kabuki syndrome could be treated successfully with surgical interventions 1516). In this study, we investigated the diagnostic process for Kabuki syndrome and hip dislocation and the outcomes of treatment of hip dislocation in patients with Kabuki syndrome.…”