Surgical management of congenital malrotation presenting in adulthood

Abstract

Intestinal malrotation is characterised by positional and congenital fixation abnormalities resulting from a failure in embryonic development in the normal 270° rotation around the axis of the superior mesenteric artery. Intestinal malrotation is primarily thought to affect neonates with an incidence of 1 in 500, however, only 1 in 6000 live births are symptomatic, and these usually present within the first month of life in 40% of cases and within the first year in over 5% of cases as an obstructive pathology or volvulus. In adults, however, the incidence has been documented at 0.2%. These patients usually present with postprandial symptoms that are intermittent such as bilious emesis, abdominal pain and malabsorption. Rarely, adults with congenital malrotation may present with acute obstruction due to volvulus, however, less than 100 adult cases are described in the literature. A high index of suspicion is required to identify cases of malrotation in the adult patient which leads to delays in diagnosis and therefore increases in morbidity can be seen.