Upper Gastrointestinal Bleeding Due to Ruptured Gastric Varices: A Diagnostic Challenge

Gastric variceal (GV) bleeding is a life-threatening complication of portal hypertension, most commonly associated with cirrhosis. However, non-cirrhotic portal hypertension (NCPH) is an important and under-recognized cause, with portosinusoidal vascular disorder (PSVD) being a key entity, particularly in patients exposed to chemotherapy. A 39-year-old man with a history of childhood neuroblastoma treated with dacarbazine, cyclophosphamide, doxorubicin, and vincristine presented with hematemesis and syncope. On admission, he was hypotensive and tachycardic. Laboratory tests revealed anemia (hemoglobin 6.9 g/dL), preserved liver function, and normal coagulation. Endoscopy showed a fundal GV with a "white nipple" sign. He received prompt vasoactive therapy, endoscopic cyanoacrylate injection, blood transfusion, and prophylaxis with ceftriaxone. Despite initial stabilization, bleeding recurred after terlipressin discontinuation, requiring a new endoscopic intervention. Common causes of cirrhosis were excluded. Imaging showed no parenchymal or vascular abnormalities. Elastography revealed normal liver stiffness (5.3 kPa) and elevated splenic stiffness (33.8 kPa). Hepatic angiography determined a portosystemic gradient of 15 mmHg. Liver biopsy demonstrated vascular changes consistent with PSVD. Extensive workup excluded other etiologies, leading to a final diagnosis of NCPH due to PSVD likely secondary to prior chemotherapy. The patient was started on a non-selective beta-blocker, with no further bleeding during follow-up. This case highlights the diagnostic and therapeutic challenges of GV bleeding in NCPH and underscores the importance of a systematic and multidisciplinary workup to identify PSVD. A detailed history, including prior chemotherapy exposure, is crucial for appropriate management.