Surgical Considerations in the Management of Constriction Ring Syndrome.

Abstract

Surgical management of constriction ring syndrome (CRS) is individualised due to the heterogenic presentation of the condition. CRS includes constriction rings, acrosyndactyly, nubbins and short digits. Involvement of more than one limb is common and children often need multiple surgeries. Each limb may need staged surgeries. If the child has vascular or lymphatic compromise secondary to a constriction ring, the ring needs to be excised and released in the first few days of life. The rings are released using multiple big Z-plasties in one or two stages. Nerve palsy associated with the rings need early intervention. Tendon transfers may be advised when nerve procedures like neurolysis and nerve reconstruction fail. Acrosyndactyly can be corrected with separation of the fused fingertips. At times, the web is more distal than usual and requires web deepening using partial syndactyly separation techniques and may need full thickness skin grafting. Complex type of acrosyndactyly is difficult to treat as it needs proper planning and staged surgical correction to achieve a five-digit hand. Separation of the syndactyly with web deepening gives more functional length and independence to the digits. The short fingers, especially the thumb, need reconstruction. Non-vascularised toe phalangeal transfer or a microvascular toe transfer can reconstruct a missing digit. Level of Evidence: Level V (Therapeutic).