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.

Developmental Foot Deformities in Patients with Connective Tissue Disorders

“…The most common foot deformity in patients with LS is talipes equinovarus (clubfoot) deformity 44,45,51,52 . Diagnosis of clubfoot requires very detailed clinical assessment and is commonly managed with the Ponseti method of serial casting followed by percutaneous Achilles tenotomy, whereas surgical correction is reserved for patients who fail conservative management 44,51-54 . LS-related clubfoot deformity carries a high risk of recurrence after conservative management, making the decision to undergo repeat casting or surgical correction a crucial one to optimize patient outcomes 54 .…”

“…Although research is limited, patients with LS-related clubfoot deformities are commonly managed with the Ponseti method of serial casting followed by percutaneous Achilles tenotomy, whereas surgical correction is reserved for patients who fail conservative management 44,51-54 . Performing posteromedial soft tissue release in patients with LS that fail conservative management has demonstrated satisfactory results with plantigrade feet and no difficulty in ambulation 51 . Although the evidence on the management of LS-related skewed foot deformity is sparse, bilateral capsulotomy can be considered to achieve correction in these patients 55 .…”

“…However, in utero ultrasound and postnatal radiographs (anteroposterior, dorsiflexion lateral) may be required in some instances. Although research is limited, patients with LS-related clubfoot deformities are commonly managed with the Ponseti method of serial casting followed by percutaneous Achilles tenotomy, whereas surgical correction is reserved for patients who fail conservative management 44,51-54 . Performing posteromedial soft tissue release in patients with LS that fail conservative management has demonstrated satisfactory results with plantigrade feet and no difficulty in ambulation 51 .…”

Developmental Dysplasia of the Hip in Patients with Connective-Tissue Disorders

“…If hip stability is not maintained or achieved with the harness, closed reduction with spica casting also has been associated with good results 31 . Conversely, Matar and Garg, in a case report on a patient with Larsen syndrome with dislocated hips and knees and severe clubfeet, recommended first treating the knee dislocations in order to later facilitate the use of a Pavlik harness or spica cast 32 .…”

Management Dilemma of a Neuropathic Knee in a Known Case of Larsen Syndrome: A Case Report

“…Various non-operative management includes pharmacological therapy, casting, and bracing. The operative management includes external fixator, fusion, and arthroplasty [ 6 ]. Considering the rarity of LS and its orthopedic manifestation, the existing literature regarding the optimal operative management is very rare and evidence for best treatment with long-term follow-up is limited.…”