Budd-Chiari syndrome presenting as fulminant hepatic failure

Area of Science:

• Hepatology
• Vascular Surgery

Background:

• Budd-Chiari syndrome (BCS) is a rare hepatic vascular disease characterized by obstruction of hepatic venous outflow.
• Fulminant hepatic failure (FHF) is a severe complication of liver disease with high mortality.
• Timely diagnosis and intervention are critical for managing BCS presenting with FHF.

Observation:

• Two cases of BCS presenting with encephalopathy within eight weeks of symptom onset, meeting the criteria for FHF, are described.
• One patient, initially suspected of non-A, non-B hepatitis, developed grade 4 coma and died 12 days post-presentation.
• The second patient, misdiagnosed with intra-abdominal malignancy, experienced exacerbated encephalopathy after exploratory laparotomy, leading to death three weeks later.

Findings:

• Necropsy confirmed Budd-Chiari syndrome as the underlying diagnosis in both cases.
• Neither patient underwent non-invasive investigations like ultrasound or isotope scanning, delaying diagnosis.
• Delayed diagnosis precluded consideration for orthotopic liver transplantation, a potentially life-saving treatment for severe BCS-FHF.

Implications:

• This case series highlights the critical importance of considering BCS in patients presenting with FHF.
• Non-invasive diagnostic modalities should be employed early in the diagnostic workup of suspected FHF.
• Prompt diagnosis and consideration for liver transplantation can significantly improve outcomes in severe Budd-Chiari syndrome.