Intestinal malrotation is a congenital disorder of incomplete rotation of the intestine during fetal growth. Because of this incomplete rotation the intestine lies in a peculiar location in the abdomen (Figure 1) that is at higher risk for twisting off the blood supply to the intestine (midgut volvulus). (Figure 2) Children with this disorder often present during the first six months of life with difficulties of intolerance to feedings and associated vomiting, often of a bilious, yellow or green nature. Intestinal malrotation by itself does not cause intestinal problems however it is the twisting, or volvulus, that causes the ischemia of the intestine and the noted blockage.
Whether symptomatic during the newborn phase, infancy or later in life, the identification of feeding intolerance, vomiting of a bilious nature that is not related to an actual illness raises the suspicion of the medical provider to this condition.
With appropriate level of concern for intestinal malrotation or the presence of bilious emesis, a medical provider will first order an evaluation of the abdomen called an upper gastrointestinal contrast series or UGI. This test allows the child to swallow contrast and monitor its progress through the intestinal tract. By evaluating the first portion of the intestine as contrast leaves the stomach radiologists are able to determine if the presence of malrotation is indeed the cause of the child’s symptoms. In cases of midgut volvulus there is a complete obstruction in the duodenum, the portion just past the stomach, due to the twist. (Figure 3) This test is all that is often needed in order to make the diagnosis. There are very subtle findings of some cases of intestinal malrotation without volvulus were delayed films or follow-up studies will be required.
Aside from the difficulties with regards to vomiting and intolerance to feedings, there may be no other clinical findings evident in the patients care. The abdomen may or may not become distended during evaluation and it may or may not show evidence of tenderness. The later finding of tenderness would be highly unlikely but of a significant enough finding as to warrant emergent evaluation and potential surgery to minimize ongoing ischemia to the intestines.
If the diagnosis of intestinal malrotation has been made by radiologic assessment, surgery is the only course for treatment. Any signs of volvulus, or twisting of the intestine, necessitate immediate surgical intervention. However if a child with malrotation and no evidence of volvulus is identified and having intermittent difficulties, scheduling for surgical intervention can be done on a more elective basis.
The operation for intestinal malrotation is to merely separate the intestinal components of the small and large intestine so that the chance for volvulus does not occur. Normal rotation can not be achieved with surgery but at the completion of the operation (Ladd procedure) the colon is fully separated from the small intestine to opposite sides of the abdomen. (Figure 4) Additionally an appendectomy is performed to avoid any difficulties in future diagnosis of appendicitis (as the appendix would be on the left side) and minimizing any difficulties with regards to potential future appendicitis.