Cruveilhier-Baumgarten syndrome

Introduction

Syndrome characterized by:

• hepatic cirrhosis
• portal hypertension
• congenital patency of umbilical or periumbilical veins

Clinical manifestations

• hematemesis
• ascites
• splenomegaly (hypersplenism)
• esophageal varices
• large tortuous veins in the abdominal wall
  • caput medusae
• venous hum near xiphoid process may be present
  • often accompanied by thrill
  • hum may be increased or decreased by Valsalva maneuver

More general terms

• cirrhosis
• developmental disorder syndrome (multisystem disorder)