Pediatric sigmoid volvulus of an extremely long sigmoid colon with hypoganglionosis: a case report.

Sigmoid volvulus is an extremely rare cause of intestinal obstruction in pediatric patients. This condition occurs when a redundant sigmoid loop with a narrow mesenteric base of attachment to the posterior abdominal wall rotates around its mesenteric axis. This situation might result in vascular occlusion and large bowel obstruction. There are only a few predisposing factors of sigmoid volvulus, such as a long-term history of constipation or pseudo-obstruction with an excessive sigmoid colon. Underlying hypoganglionosis can also lead to large bowel obstruction. There have only been two reported cases of hypoganglionosis with sigmoid volvulus, and both were in adults. Sigmoid volvulus usually presents with abdominal pain, nausea, vomiting, constipation and abdominal distension, an absence of stool, or the presence of melenic stool in the rectum. Initial treatment options are non-surgical for stable patients, although surgical management might be necessary. If sigmoid volvulus is not recognized and resolved, it may lead to serious complications and death. Pediatric sigmoid volvulus is frequently the fulminant type, and therefore, a decision about treatment must be prompt. We present an unusual pediatric case of an extremely long sigmoid colon with hypoganglionosis, which twisted and caused obstruction. This condition was resolved with surgical resection.