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.

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