Rotational anomalies occur as a result of an arrest of normal rotation of the embryonic gut. They are often associated with other gastrointestinal abnormalities, particularly those in which the intestines are located outside the coelomic cavity (eg, congenital diaphragmatic hernia or abdominal wall defects). However, intestinal malrotation can also occur in children and adults who have no associated anomalies.
An overview of the presentation, diagnosis, and treatment of intestinal malrotation will be presented here. Congenital diaphragmatic hernia and abdominal wall defects are discussed separately. (See "Obstetrical management of omphalocele".)
Rotational anomalies, which may or may not be symptomatic, are estimated to occur in between 1/200 and 1/500 live births [1,2]. Symptomatic malrotation is estimated to occur in 1/6000 live births . Traditionally, intestinal malrotation has been considered primarily a disease of infancy with infrequent occurrence beyond the first year of life. However, a series of 170 patients of all ages with intestinal malrotation managed at a single institution found the following age distribution :
●Infants under one year of age: 31 percent
●Children one to 18 years of age: 21 percent