Amniotic constriction band syndrome resulting in amputation caused by septate uterus: a case report

Abstract

Amniotic band syndrome is an unusual congenital condition characterized by manifestations of disfigurement and disablement. Patients with this condition may experience an array of clinical deformities, including constriction rings, digital defects, and even visceral defects. Although this disease has been identified for centuries, its etiology is still unknown. The present male patient was born by cesarean section at 34 weeks and 4 days of gestation. At birth, an amniotic band that encircled and constricted his right upper limb was observed. Four hours after the amniotic band was cut off, amputation was performed because the right limb remained insensate. The patient suffered from amniotic band syndrome and presented with a gangrenous limb leading to amputation at birth, which is extremely rare. Moreover, the patient's mother suffered from a uterine septum, which has not been previously reported in this situation. Timely surgical treatment avoided further tissue necrosis threating the patient's life. This rare case of amniotic band syndrome provides new clinical evidence for the "extrinsic theory".

Keywords: Amniotic constriction band syndrome; amputation; extrinsic theory; misoprostol; neonate; septate uterus.

Figure 1.

At birth, the distal limb showed cyanoderma, edema of the extremity, and focal peeling (a). After 4 hours, the right limb continued to show signs of hypoperfusion and more edema without any compromise (b). X-ray result before surgery (c). The residual limb after surgery (d). Postoperative X-ray image (e).