Split notochord syndrome in an adult presenting with colonic duplication. Report of a case.

Abstract

A case of tubular colonic duplication, sigmoid colon diverticulum, and lumbar anterior spina bifida in a 28-year-old man was diagnosed as split notochord syndrome. After subtotal colectomy, including the duplicated colon, the patient recovered. Histologic study of the duplicated colon revealed multifocal, ectopic gastric fundic mucosa in a mosaic pattern. Since bleeding and perforation occur most frequently in conjunction with ectopic gastric mucosa within the duplication, we believe that complete resection of the duplicated bowel is the best procedure. Split notochord syndrome is a rare congenital anomaly. It is usually discovered in the first year of life, and the majority of reported cases have involved the cervical or thoracic region. Our patient is exceptional in having reached the age of 28 years without complaints and in that his case involved lumbar anterior spina bifida and tubular colonic duplication.