Budd-Chiari syndrome: two cases with different courses
Shinjiro Inomata1, Yasuaki Takeyama, Takashi Tanaka
1Department of Gastroenterology and Medicine, Fukuoka University Faculty of Medicine, Fukuoka, Japan.
Insights
This report details two Budd-Chiari syndrome cases with atypical presentations. Effective treatment for one patient with inferior vena cava obstruction highlights the need for careful monitoring in all cases.
Area of Science:
- Hepatology
- Vascular Medicine
- Internal Medicine
Background:
- Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction.
- Diagnosis and management can be challenging due to diverse clinical presentations.
- Understanding atypical BCS presentations is crucial for timely intervention.
Observation:
- Case 1: A 57-year-old male with inferior vena cava (IVC) obstruction, antiphospholipid syndrome, hepatitis C, and alcohol abuse presented with edema and varices.
- Case 2: A 73-year-old female with membranous IVC obstruction presented with abdominal pain but no edema or varices.
- Both cases displayed atypical features, with liver biopsy findings not correlating directly with clinical presentation or obstruction severity.
Findings:
- Case 1: Percutaneous transluminal angioplasty and anticoagulation ameliorated symptoms, with no restenosis at 9 months.
- Case 2: Membranous IVC obstruction required no intervention, with preserved liver function despite congestion.
- Histopathological findings in both cases were atypical relative to clinical and imaging data.
Implications:
- These cases underscore the importance of integrating clinical, imaging, and histopathological data for accurate BCS diagnosis and management.
- Atypical presentations necessitate vigilant follow-up for potential complications like hepatocellular carcinoma.
- Successful intervention in complex BCS cases can lead to significant symptom improvement and prevent disease progression.
Abstract:
We report two cases of Budd-Chiari syndrome. Case 1: A 57-year-old man presented with leg edema and esophageal varices. Cavography showed obstruction of the inferior vena cava with antiphospholipid syndrome. Further, the patient showed positive serology for hepatitis C virus and consumed large quantities of alcohol. Percutaneous transluminal angioplasty was performed on this patient and anticoagulants administered; leg edema and esophageal varices were ameliorated although liver biopsy showed cirrhosis without evident congestion. More than 9 months since the diagnosis, restenosis of the inferior vena cava has not occurred. Case 2: A 73-year-old woman presented abdominal pain but no edema or varices. Cavography showed membranous obstruction of the inferior vena cava which required no therapy. Manifestation of portal hypertension was not present and liver function was maintained although liver biopsy showed obvious congestion. These cases showed untypical features against histopathology, and careful observation will be required for emergence of hepatocellular carcinoma.
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