Polysplenia syndrome associated with multisystem malformation: A rare case report

Radiol Case Rep. 2024 Dec 30;20(3):1586-1590. doi: 10.1016/j.radcr.2024.12.037. eCollection 2025 Mar.

Abstract

Polysplenia syndrome is a rare congenital disease with multiple systemic developmental abnormalities. Their occurrence and development are closely related to embryonic development. The prognosis of the disease depends on its anatomical structure, and the presence or absence of concomitant cardiac malformations also has a prognostic impact. Here, we report a case of an adult female who showed the presence of epigastric pain and was diagnosed with polysplenia syndrome after an abdominal CT scan. Imaging further revealed the presence of developmental malformations in several systems, including cardiovascular, respiratory, and digestive systems. This case is rare and therefore has some clinical reference value.

Keywords: Azygos vein; Left IVC; Left Inferior Vena Cava; Polysplenia Syndrome.

Publication types

  • Case Reports