Polysplenia Syndrome With Persistent Left Superior Vena Cava: Case Report and Review of the Literature

Abstract

Polysplenia syndrome is an uncommon condition associating several splenic nodules (sometimes polylobed spleen and cases of normal spleen have been described) with a number of malformations that appear between the fourth and sixth week of embryonic development. Although it has been suggested that genetic, teratogenic, and embryogenic factors may be at fault, the exact etiology remains unclear. Clinically, it is generally asymptomatic or mildly symptomatic. The authors report the case of an 11-months-old infant from a poorly monitored pregnancy. He was admitted to the emergency room for respiratory discomfort in a context of apyrexia. A thoraco-abdominal CT scanner revealed a polysplenia syndrome.

Adult intestinal malrotation presenting as caecal volvulus with incidental findings of duplicate inferior vena cava and other rare abnormalities: case report

“…The described abnormalities of our case follow the pattern of polysplenia syndrome which is defined as two or more spleens accompanying congenital malformations in the abdomen or thorax that arise between weeks 4–6 of embryogenesis [ 13 , 14 ]. IVC abnormalities and intestinal malrotation are known abnormalities associated with polysplenia syndrome [ 14 ]. However, polysplenia is rare in adulthood as most cases also have severe congenital cardiac malformations, which result in 75% mortality by 5 years old [ 15 ].…”