A Rare Case of Banti’s Syndrome Cured by Autosplenectomy

Clarisse Enria B. Porras, MD; Alex Alcasid, MD
Southern Philippines Medical Center

Background: Banti’s Syndrome is marked by elevated portal pressure without liver disease or portal vein obstruction. Its cause remains unclear, though factors like arsenic exposure and hypervitaminosis A are implicated. This case highlights diagnostic and management challenges in a young patient with gastrointestinal bleeding.

Clinical Presentation: A 23-year-old male with no known co-morbidities presented with melena. Physical examination revealed a large palpable mass in the left upper quadrant, with no signs of chronic liver disease. Endoscopy showed grade 3 esophageal and gastric varices. Work up indicated preserved liver function and pancytopenia. Investigations revealed F0 in Fibroscan, and no thrombosis on Doppler. CT showed a normal liver, extrahepatic portosystemic shunt, gastric varices, and minimal ascites. Liver biopsy was normal, and tests for autoimmune, hypercoagulable, and myeloproliferative disorders, as well as schistosomiasis, were negative. Findings confirmed Banti’s Syndrome. Splenectomy and devascularization was advised however the patient declined. Hence, Balloon-Occluded Retrograde Transvenous Obliteration with splenic artery embolization was performed. Gastrorenal shunt was not identified, hence splenic artery embolization was proceeded. The procedure was successful with no development of splenic abscess. Two months later, CBC was normal and endoscopy revealed significant resolution of the varices. The patient had no complaints thereafter.

Conclusion: This case highlights the complexity of Banti’s Syndrome, a rare cause of non-cirrhotic portal hypertension. Absence of hepatic fibrosis and exclusion of secondary causes were essential in establishing the diagnosis. Multidisciplinary management is crucial for optimal outcomes. Further research is needed to better understand the pathophysiology and refine treatment strategies for Banti’s Syndrome.

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