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A Rare Case of Polysplenia Syndrome and Congenital Cloaca with Symptomatic Intestinal Non-rotation in an Adult Patient

Dorian Blommaert, Mark C. van Baal, David D Zimmerman, Barbara S Langenhoff

A 52-year-old women presented to our emergency department with nausea, vomiting and acute, progressive abdominal pain in the entire abdomen radiating to the back. Her history reported a congenital cloaca, for which she underwent reconstructive surgery twice during childhood. On physical examination normal bowel sounds were heard and no signs of peritoneal irritation were present. A contrast-enhanced computed tomography showed dilated small intestinal loops in the left upper quadrant and a bowel obstruction due to a non-rotation of the midgut. Furthermore, interruption of the vena cava inferior with azyguous continuation, polysplenia, short pancreas, bicornuate uterus and a small diaphragmatic hernia were seen. In this case report, these congenital anomalies are presented in the context of an unusual case of polysplenia syndrome.