| A rare intestinal disease characterized by a single, focal intestinal perforation, associated with hemorrhagic necrosis, typically occurring at the terminal ileum, involving antimesenteric border. It may also occur in the jejunum or colon. It predominantly affects very (or extremely) low weight infants (birth weight less than 1500 g) mostly in the first week of life. Patients have healthy bowel apart from the perforation site, they present with bluish discoloration and gasless abdomen in the absence of pneumatosis intestinalis. Urinary tract infection can also be present. However they do not manifest necrotizing enterocolitis, bowel obstruction or prodromal clinical symptoms. |
