Segmental absence of intestinal musculature concerning a child with acute appendicitis

•The SAIM may be congenital and may only manifest after a triggering event such as an acute illness.•This diagnosis should be kept in mind during the laparotomy in order to avoid misdiagnosis.•Complete resection of intestinal segment with SAIM is curative.

Segmental absence of intestinal musculature (SAIM) is a rare histopathologic entity with few described cases, most of which in neonates. Rare reports of pediatric cases have been made. Pathogenesis is still uncertain. This report aims to highlight a pathologic condition that substantially affects the clinical evolution of an abdominal post-operative state as well as the therapeutic approach. We present the case of a 4-year old child, previously healthy, with a 4-day long intestinal obstruction. The exploratory laparotomy demonstrated peritonitis from a necrosed and perforated acute appendicitis. Post-operatory intestinal perforation led to a second laparotomy, with resection of a perforated ileal segment and one with vascular compromise. Pathologic analysis showed segmental absence of intestinal musculature of the small bowel. On a third operative time, resection of the macroscopically injured bowel segments and high protective enterostomy were made. A three years follow-up shows a healthy, well-developed child, who has already undergone enterostomy closure with no intercurrences. Etiology remains to be defined, possibly related to congenital or ischemic acquired defects. It usually manifests in neonates, but we present a case of a 4-year old child. Treatment consists of resection of the injured bowel segments.