Congenital dislocation of the hip in Ehlers-Danlos syndrome.
Nine patients with Ehlers-Danlos syndrome (EDS) were treated for bilateral congenital dislocation of the hip (CDH). CDH was diagnosed at six months of age in two patients and at two to five years of age in the remaining seven patients. The diagnosis of EDS, based on clinical criteria, was established considerably later. One patient with multiple deformities was only observed; the remaining eight patients were initially treated by closed reduction. Due to difficulties encountered in reduction and especially in stabilization, all hips were subsequently treated surgically, with a total of 42 procedures. Avascular necrosis of the femoral head developed in five hips, four of which had been treated with closed reduction with the patients under general anesthesia. At the follow-up evaluation, six patients had reached adulthood. Clinically, satisfactory results were obtained in 12 of 16 hips but roentgenographically in only six hips. Both femoral and innominate osteotomies are necessary to achieve and maintain the reduction in EDS patients with CDH.
Badelon
O
O
Department of Pediatric Orthopaedic Surgery, Hospital Robert Debré, Bichat University Medical School, Paris, France.
Bensahel
H
H
Csukonyi
Z
Z
Chaumien
J P
JP
D002648:Child; D002675:Child, Preschool; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D005260:Female; D006618:Hip Dislocation, Congenital / Q000150:complications* / Q000601:surgery / Q000628:therapy; D006801:Humans; D007223:Infant; D008297:Male; D012008:Recurrence; D012086:Reoperation; D014143:Traction