Background: Congenital constriction ring syndrome (CCRS) is a rare condition diagnosed at birth characterised by deformation due to a constriction ring. The usual treatment for CCRS involves excision of the constriction ring and suture of the skin incorporating a Z-plasty to prevent scar contracture. A Z-plasty often results in an unsightly scar. In order to avoid this, we performed linear circumferential skin closure (LCSC). The aim of this paper is to report the outcomes of LCSC for CCRS. Methods: We retrospectively investigated all patients with CCRS who underwent LCSC between 2002 and 2020. Two linear incisions were placed proximal and distal to the constriction ring in parallel, and the constriction ring was excised carefully so as not to damage nerves or vessels. The deep subcutaneous and dermis layers were sutured. The skin was closed using adhesive tape. Two-stage surgery was performed in two patients with severe CCRS of the lower leg to avoid problems with distal circulation. Patients were followed up for at least 1 year and assessed for complications and quality of scar. Results: We performed LCSC for 31 sites in 19 patients, including one forearm, 14 fingers, 10 lower legs and six toes. The median age at the operation was 16 months (range: 4-175). The median follow-up period after surgery was 5.8 years (range: 1.9-16.0). The linear surgical scar had healed well in all patients and there were no complications. There was no recurrence of the constriction ring and no scar hypertrophy, though we did not perform fat mobilisation in all cases. None of the patients required additional surgery and the aesthetic outcome of the linear circumferential surgical scar was maintained at the final observation. Conclusions: Treatment for CCRS using LCSC resulted in no complications, no recurrence of constriction and an excellent aesthetic outcome. Level of Evidence: Level IV (Therapeutic).
Keywords: Congenital constriction rings syndrome; Deformity; Linear circumferential skin closure; Reconstruction; Z-plasty.