Surgical Treatment of Active Amniotic Band Syndrome (ABS) by Z-plasty and Radical Excision of the Overgrown Tissue: A Report of 2 Cases With Progressive Lymphedema Causing Vascular Insufficiency.

UNLABELLED: The study describes 2 children at risk of limb amputation due to lower extremity active amniotic band syndrome, in which the constriction bands were released surgically using the extensive approach. Both patients presented almost the same clinical appearance: a deep constriction band localized in one third of the distal part of the leg and a pseudoconstriction at the ankle joint level resulting from the tightening of the retinaculum of extensors. In both the cases, after birth amniotic band syndrome caused progressive enlargement of the distal part of the foot, which was associated with edema and vascular insufficiency. Case 1, at the age of 3 months, underwent a primary excision and Z-plasty of the proximal constriction band, and after 6 weeks a secondary excision was performed covering the soft tissue bulk from the dorsal part of the foot formed due to vascular insufficiency progression. In contrast, case 2 underwent a more radical 1-staged surgery at the age of 4 weeks, that is, an excision and Z-plasty of the proximal constriction and the same radical excision of the soft tissue bulk. In addition, in both patients decompression fasciotomy was performed. A follow-up after surgery, respectively, at the age of 20 and 5 years, revealed a fully functional foot and restoration of blood supply. Thus, the 1-staged radical excision of constrictions combined with removal of the overgrown skin and pathologic soft tissue can be recommended for similar cases. LEVELS OF EVIDENCE: Level IV-case series.