Intestinal Rotational Abnormalities


Intestinal rotation abnormalities (IRA) is the current term to describe conditions which were commonly referred to as "intestinal malrotation". The term IRA is more inclusive and is slowly penetrating the pediatric surgical vocabulary.

IRA is a congenital anatomical condition that predisposes a significant portion of intestine to twisting with subsequent obstruction and ischemic necrosis. Midgut volvulus is a feared pediatric surgical emergency as the loss of small intestine and the ensuing risk of short gut syndrome and intestinal failure can cause significant morbidity and mortality. The surgical treatment for malrotation is the Ladd procedure.

What is the difference between malrotation and volvulus?

Malrotation of the intestine is the failure of the intestine to rotate normally during the development of the embryo. One of the dangers of malrotation of the intestine is that the bowel may be obstructed by abnormal bands or twist on its own blood supply. Malrotation of the intestine is usually not apparent until the intestine becomes obstructed or twisted, generally in infants or in early childhood. Symptoms may be as indistinct as chronic abdominal pain or severe with signs of obstruction including bilious emesis. Malrotation can be discovered incidentally and the management of asymptomatic malrotation remains an area of controversy.

Volvulus is the abnormal twisting of a portion of the gastrointestinal tract, usually the midgut, which can cause obstruction and impair blood flow. Volvulus can lead to ischemia, necrosis, perforation and the loss of the involved segment of the gastrointestinal tract. Malrotation of the bowel during fetal development can predispose the patient to a volvulus which often has a sudden onset. The symptoms and signs of a volvulus include pain and bilious emesis. Initially the abdominal exam may be essentially normal with the signs of peritoneal irritation occurring later. Volvulus requires emergency surgery to relieve the obstruction and ensure normal blood flow to the bowel. .

midgut volvulus
Descriptive text is not available for this image
Intraoperative picture of a five day old with midgut volvulus and necrotic bowel. Note the appendix adherent to the proximal jejunum.

What is the outcome if the diagnosis of volvulus is delayed?

A catastrophic outcome of a delay in diagnosis and intervention is the total loss of the midgut if the volvulus occurs along the vascular distribution of the superior mesenteric artery (proximal jejunum to mid ascending colon). This would leave the child with only centimeters of viable small bowel and a very limited portion of colon. Their immediate postoperative course is often complicated by sepsis and other issues while their long-term health is compromised by the sequelae of "short gut syndrome."

see also Ladd Procedure for Malrotation

Content in this topic is referenced in SCORE Malrotation overview

There's more to see -- the rest of this topic is available only to subscribers.

Last updated: January 18, 2022