Twisting of the Spleen Observed in a Case of Polysplenic Disorder Associated with Situs Viscerum Inversus and Kartagener's Syndrome

Abstract

The authors describe a rare example of polysplenic disorder observed in a thirteen year-old girl known to be affected by situs viscerum inversus and Kartagener's syndrome. The spleen consisted of ten "mini spleens" positioned along the gastric curve. The twisting of one of these was the cause of the surgical intervention which led to the discovery of the splenic malformation. Grossly, each spleen had its own peduncle depending on a single major peduncle, representing the splenic hilus. The overall weight was 260 g. The twisted spleen was blackish in colour, filled with blood and weighed 100 g. Histopathologically, no particular finding was observed. Although the preoperative diagnosis of polysplenic disorder is difficult, the knowledge of a basic malformative condition should lead to the search for this possibly associated malformation because of its clinical implications.