A new approach to amniotic band disease

Background/Aim: Amniotic band disease (ABD) is a rare condition, generally associated with three types of anomalies: malformations, deformities and amputations. Several theories have been put forward, the extrinsic theory being the most widely adopted. The aim of the study was to clarify the pathophysiology of the disease, to propose a new classification and to precise the therapeutic indication through a radio-clinical description of seventy-five amniotic bands in ten patients.Methods: This was a descriptive radio-clinical and surgical study of 75 amniotic bands in amniotic disease. The clinical features were used to describe the type of amniotic band: deep (impeding vascular circulation, compressing soft tissues and bone) or superficial cutaneous with a good prognosis. The shape of the band was described as semicircular, circular, heliocoidal or complex figure of two linked circles shaping the number 8 flat/bended. There were 5 mild constrictions without lymphoedema, 28 moderate (constrictions without lymphoedema in 7 cases and severe constrictions with lymphoedema in 21 cases) and 41 amputations (one leg and 40 fingers and toes). Surgical treatment involved 16 deep constrictions and three feet.Results: Resection of the amniotic bridges gave an acceptable cosmetic result, but lymphoedema persisted. Surgery on both feet enabled walking with plantigrade support. However, the Ponseti technique was used on a child with a severe and complex heliocoid constriction. He developed ischaemia, requiring immediate removal of the cast. Digital separation of the fingers restored a functional hand in particular the pauci-digital pincer, but the aesthetics were judged insufficient by the parents.Conclusions: Based on presented findings, amniotic limb disease should be classified into three types: 'Type A' with a good prognosis, 'Type B' subdivided into six sub-types and "Type C' defined by circular or complex constriction. The classification provides a therapeutic orientation. Depending on the type of the constriction band of the disease, different strategies can be envisaged: therapeutic abstention, cosmetic surgery or emergency limb salvage surgery.