Décès consécutif à des anomalies congénitales de rotation intestinale : à propos de deux cas concernant un enfant et un jeune adulte

We report the cases of two males 6 and 16 years old, who died of bowel obstruction due to anomalies of malrotation. Both diagnoses could only be made at the time of autopsy. These two case reports are remarkable as a literature review found only two cases of death due to such anomalies, and because they were older than usual as they are generally diagnosed in the early neonatal period. These are the first lethal cases reported after the age of one. Anomalies of midgut rotation, also called common mesentery, are seldom symptomatic after this age, and even more exceptionally in adulthood. Adult clinicians who hardly know anything about this life-threatening pathology generally do not make the diagnosis. Most of the malrotations are diagnosed early in the first months of life because of the occurrence of bilious vomiting. An upper gastrointestinal contrast study confirms the anomaly. When such a case is suspected in much older patients, because of an acute bowel obstruction or chronic abdominal pains, a Doppler ultrasonography and preferably an abdominal computed tomography will confirm the diagnosis. Treatment of choice remains chirurgical (Ladd procedure), but must be performed in emergency even in front of a chronic symptoms to avoid bowel obstruction by volvulus, which could lead to mesenteric infarction, peritonitis and death. In spite of the fact that such observations remain exceptional, the severity of prognosis, the hypothesis of intestinal malrotation must be kept in mind when dealing with a patient who present unexplained and persistent digestive disorder, whatever his age is.