DOI: 10.36347/sjmcr.2023.v11i08.034

Pages: 1564-1567

Polysplenia syndrome is a rare congenital disorder belonging to the large spectrum of situs ambigus syndromes. It is characterized by the association of multiple spleens with other vascular, cardiac, intestinal and biliary anomalies. We report the case of an 8 year-old-girl suffering of chronic abdominal pain resistant to symptomatic treatment. Radiological investigations showed right-sided stomach midline liver, two spleens in the retro-gastric region, absence of retrohepatic inferior vena cava and direct drainage of the hepatic veins to the right atrium. No cardiac anomaly was detected. This disorder is often diagnosed incidentally during imaging studies for unrelated medical conditions or when patients present with symptoms of associated anomalies. The aim of this case report is to increase the awareness of this rare entity in pediatric groups presenting common digestive symptoms but with functional impact on daily life.