Chronic budd–chiari syndrome in a child with ulcerative colitis: A rare extraintestinal manifestation

Background: Ulcerative colitis (UC), a chronic inflammatory bowel disease (IBD), frequently presents with extraintestinal manifestations (EIMs), especially involving the joints, skin, and hepatobiliary system. While primary sclerosing cholangitis and autoimmune hepatitis are known hepatic EIMs, Budd–Chiari Syndrome (BCS)—a condition caused by hepatic venous outflow obstruction—is exceedingly rare, particularly in children with IBD.

Case Presentation: A 12-year-old boy diagnosed with UC based on colonoscopy and biopsy presented with chronic diarrhea, blood-streaked stools, abdominal pain, and weight loss. He improved on corticosteroids and was maintained on mesalamine and azathioprine. During routine follow-up one year later, ultrasound revealed coarse hepatic echotexture and surface nodularity. Liver function and coagulation profiles were normal. Liver biopsy showed cirrhosis with sinusoidal dilatation and venous fibrosis. Doppler and CT imaging confirmed narrowing of the intrahepatic IVC and hepatic veins with collateral formation, consistent with chronic BCS. Hypercoagulable workup was unremarkable, except for a low-titer positive ANA. The patient was referred for hematology evaluation and is being followed for possible angioplasty and stenting.

Conclusion: BCS is a rare but serious complication of UC in children. Chronic inflammation, endothelial dysfunction, and immunological factors may contribute to its pathogenesis, even in the absence of identifiable thrombophilia. This case highlights the importance of routine hepatic surveillance in pediatric IBD patients and the need for a multidisciplinary approach to management. Early recognition of hepatic vascular complications can prevent progression to hepatic decompensation and allow timely intervention.

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