Malrotation syndrome resulting in fatal ileus in children.

Background: This report deals with two deaths of children involving intestinal volvulus, i. e. a pathological knotting and twisting of the mesenterial root on the basis of congenital malrotation followed by obstruction and extensive ischaenia.

Case reports: (1) A 10-year-old girl (premature, 25th week) with severe disability, amaurosis and epilepsy was admitted to hospital due to general agitation and a bloated abdomen without peristaltic sounds, but died some hours later. Autopsy revealed a volvulus of 2/3 of the small intestine based on congenital malrotation with additional clamping of the intestine underneath adhesions (previous appendectomy). The abdominal cavity showed beginning peritonitis as the cause of death. (2) A 2-month-old premature male baby (surviving twin, 29th week) with a persisting ductus arteriosus was hospitalised for four weeks after failed vascular surgery due to acute general deterioration. Radiological diagnostics using a contrast medium revealed a vascular anomaly (right-sided aortic arch). Around 10 hours later, the infant developed an acute abdomen with ileus symptoms. Emergency surgery showed infarction of the entire small intestine due to an anti-clockwise 180⁰-volvulus, with death occurring 24h later. Further examination showed a malrotation as anomaly. Apparently, the volvulus had been caused by extensive use of contrast medium resulting in increased intestinal mobility.