A case report of amniotic band syndrome complicated by extensive body wall defect

• Published in: Journal of Japan Society of Perinatal and Neonatal Medicine Vol. 60; no. 2; pp. 240 - 244
• Main Authors: Kanno, Masayuki, Shimizu, Masaki, Wakamatsu, Hiroaki
• Format: Journal Article
• Language: Japanese
• Published: Japan Society of Perinatal and Neonatal Medicine 2024; 一般社団法人 日本周産期・新生児医学会
• Subjects: amniotic band syndrome; body wall defects; constriction ring; meningocele; scoliosis
• ISSN: 1348-964X; 2435-4996
• DOI: 10.34456/jjspnm.60.2_240

Amniotic band syndrome is a syndrome including multiple malformations of the extremities, cranium, face, and trunk caused by the amniotic band that is formed when the amnion ruptures in the fetal period. The type of malformations depend on the timing of amniotic rupture. In case of early rapture, the symptoms become more severe: such as cranial, facial, and thoracoabdominal wall defects. In the present case, a meningocele and scoliosis were noted at fetal period. After birth, we found extensive body wall defects adjacent to the meningocele, and the liver, spleen, and lungs were visible through the peritoneal-like membrane. Considering the risk of injury and infection of the exposed organs, the body wall defect was covered using an urgent flap surgery and skin grafting. The constriction ring of toe and clubfoot were found, therefore we diagnosed the body wall defect as a symptom of amniotic band syndrome. In this case, there was also a finding suspected of chondrodysplasia punctata brachytelephalangic type, and although the contribution to amniotic band syndrome was not clear, the influence of maternal anti-TNF-alpha antibody preparations was speculated. Amniotic band syndrome should be considered in cases with various malformations of different developmental stages and with characteristic findings such as constriction rings.