Budd–chiari syndrome as an extraintestinal manifestation of ulcerative colitis in a child

Background: Ulcerative colitis (UC) is a chronic inflammatory bowel disease with well-recognized extraintestinal manifestations. Budd–Chiari Syndrome (BCS), caused by hepatic venous outflow obstruction, is an exceptionally rare hepatic complication of pediatric UC and may remain clinically silent until advanced disease develops.

Methods: We report the case of a 12-year-old boy diagnosed with ulcerative colitis based on colonoscopy and histopathology. He initially presented with pancolitis and was treated with intravenous corticosteroids followed by oral steroids, mesalamine, and azathioprine, with good clinical response. Routine follow-up ultrasonography one year later revealed coarse hepatic echotexture and nodularity despite normal liver function tests and coagulation profile. Further evaluation included liver biopsy, contrast-enhanced CT abdomen, and Doppler studies.

Results: Liver biopsy demonstrated cirrhosis with lobular distortion, sinusoidal dilatation, and venous intimal fibrosis. Imaging revealed hepatomegaly, caudate lobe hypertrophy, narrowing of intrahepatic inferior vena cava and hepatic veins with collateral formation, confirming chronic Budd–Chiari Syndrome. Hypercoagulable work-up was negative except for low-titer ANA positivity. The child had compensated cirrhosis and was managed with anticoagulation. Interventional radiology-guided angioplasty was successfully performed, and the child remains under multidisciplinary follow-up.

Conclusion: Budd–Chiari Syndrome is a rare but serious extraintestinal manifestation of pediatric ulcerative colitis. Subtle hepatic imaging abnormalities, even in the presence of normal liver biochemistry, should prompt early Doppler evaluation. Early recognition and multidisciplinary management are crucial to prevent progression to hepatic decompensation.

Dr. Mounika Bazar, from India, has completed advanced training in Pediatric Gastroenterology, Hepatology, and Nutrition and is actively engaged in clinical practice and academic work. She has presented her research at national and international conferences and contributes to academic writing and clinical research. Her work reflects a commitment to academic excellence, with an evolving focus on healthcare systems, quality improvement, and medical education.