A new variant of type III jejunoileal atresia

Abstract

Jejunoileal atresia (JIA) is a congenital defect that can result in significant loss of bowel length. The traditional classification of JIA was first proposed by Grosfeld and includes 4 subtypes. Among these, type IIIB, or apple-peel atresia, is characterized by a proximal atretic jejunum and a distal segment of spiraled bowel that terminates at the cecum. Owing to this anatomy, patients with type IIIB JIA are at increased risk for short bowel syndrome and intestinal failure. In this report, we described the case of a neonate with a prenatal diagnosis of JIA. At exploration, she was initially found to have a type IIIB atresia. However, instead of terminating at the cecum, the distal spiraled segment was followed by 75 cm of normal small bowel and mesentery. Surgical correction proceeded with minimal resection and primary anastomosis. She recovered well from this procedure, tolerated full enteral nutrition by mouth, and displayed good weight gain at outpatient follow-up. Owing to the unique anatomy of the gastrointestinal tract in this case report, we propose the addition of a new class of JIA, type IIIC, to better reflect its prognostication and surgical management.