Abstract
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.
Highlights
Situs ambiguous, or heterotaxy, is a rare congenital abnormal position of organs and vessels
We present an interesting case of an adult patient with polysplenia syndrome with multiple abdominal abnormalities, including a congenital cloaca
Malrotation of the bowel is found in the majority of patients with heterotaxy syndrome [4,5,6,7,8,9,10]
Summary
Heterotaxy, is a rare congenital abnormal position of organs and vessels. Since there are many ways in which organs may be arranged, the clinical presentation of a heterotaxy syndrome can vary between patients. Besides left-sidedness, abdominal heterotaxy was present in 57% of the polysplenia patients. The presence of multiple spleens is the only anomaly [5,6].
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