Cruveilhier-Baumgarten disease in Japan--on the basis of our own case

Area of Science:

• Hepatology
• Vascular Surgery
• Gastroenterology

Background:

• Cruveilhier-Baumgarten disease is a rare condition characterized by the recanalization of the umbilical vein, leading to portal hypertension.
• Portal hypertension can cause significant complications, including esophageal varices and splenomegaly.

Observation:

• A 59-year-old patient presented with a history of anemia, splenomegaly, abnormal liver function, and esophageal varices.
• Laparoscopy revealed a distended vein in the falciform ligament, confirmed by angiography and ultrasonography.
• Liver biopsy showed minimal fibrosis and no cirrhosis, despite evidence of portal hypertension.

Findings:

• The patient exhibited Cruveilhier-Baumgarten disease with patent umbilical vein and portal hypertension.
• Esophageal varices and hypersplenism were present, consistent with portal hypertension.
• The umbilical vein dilatation was likely congenital and not the primary cause of active portal hypertension.

Implications:

• This case underscores the importance of diagnosing Cruveilhier-Baumgarten disease even with minimal liver histology changes.
• Understanding the prevalence of specific symptoms like esophageal varices and splenomegaly in different regions, such as Japan, is crucial for clinical diagnosis.
• Further research into the congenital versus acquired nature of umbilical vein dilatation in portal hypertension is warranted.