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Cruveilhier-Baumgarten syndrome
Syndrome characterized by:
1) hepatic cirrhosis
2) portal hypertension
3) congenital patency of umbilical or periumbilical veins
Clinical manifestations:
1) hematemesis
2) ascites
3) splenomegaly (hypersplenism)
4) esophageal varices
5) large tortuous veins in the abdominal wall
- caput medusae
6) venous hum near xiphoid process may be present
a) often accompanied by thrill
b) hum may be increased or decreased by Valsalva maneuver
General
cirrhosis
developmental disorder syndrome (multisystem disorder)
References
DeGowin & DeGowin's Diagnostic Examination, 6th edition,
RL DeGowin (ed), McGraw Hill, NY 1994, pg 876