title: Management of joint dislocations of the lower limb in Larsen syndrome: practical approach.
abstract: INTRODUCTION Larsen syndrome is an autosomal-dominant osteochondrodysplasia characterised by large joint dislocations and craniofacial anomalies. CASE HISTORY We present a rare case of Larsen syndrome with bilateral dislocated hips and knees and severe clubfeet at 7-year follow-up. We undertook bilateral open reduction of both hips at age 8 months. This procedure was preceded by open reduction and left-knee V-Y quadricepsplasty at age 4 months following a failed trial of closed reduction of the left knee. Both feet had a severe deformity (Pirani score of 5.5 and 6.0) and were treated using the Ponseti method, but the left foot relapsed at 24 months and required posteromedial release. CONCLUSIONS We tried to address the difficult questions on the timing and sequence of surgical interventions by treating clubfeet and dislocated knees early using Ponseti casts which included the knees. Open reduction of hips was done later, and further interventions were guided by functional needs.
authors: Alder Hey Children's Hospital , UK.
authors: H E
authors: HE
authors: Matar
authors: Alder Hey Children's Hospital , UK.
authors: N K
authors: NK
authors: Garg
mesh_terms: D002648:Child; D003025:Clubfoot / Q000601:surgery*; D005260:Female; D006618:Hip Dislocation, Congenital / Q000601:surgery*; D006801:Humans; D031221:Knee Dislocation / Q000151:congenital / Q000601:surgery*; D010009:Osteochondrodysplasias / Q000150:complications*; D012008:Recurrence; D016896:Treatment Outcome
keywords: Congenital knee dislocation; Larsen syndrome; Ponseti casts; V–Y Quadricepsplasty