P0801 - Cruveilhier- Baumgarten Syndrome

Carlos M. Ramos-Pachon, MD¹, Andrew Dam, DO¹, Humberto M. Rios, MD¹, Micaella Kantor, MD², Sarah M. Arvaneh, MD¹, Luis M. Nasiff, MD, FACG^1
1Larkin Community Hospital, Hialeah, FL; ²Larkin Community Hospital, Miami, FL

Introduction: Cruveilhier- Baumgarten syndrome CB) is an uncommon condition, the term is used for cases of liver cirrhosis with portal hypertension in which a recanalization of umbilical and paraumbilical veins causes a loud venous hum that can be heard over the abdomen.

Case Description/Methods: A 51 year old male with a past medical history of alcohol abuse, alcoholic liver cirrhosis and esophageal varices presented to the emergency room complaining of coffee ground emesis and dark-colored stools. On a physical exam, a small umbilical venous dilatation with a low-pitched continuous hum was noted on auscultation. Abdominal ultrasound demonstrated hepatofugal flow. CT abdomen and pelvis without contrast showed findings consistent with cirrhotic liver, mild ascites, splenomegaly and umbilical vein recanalization. The patient underwent an EGD, which was negative for any esophageal or gastric varices.

Discussion: In 1833 Pegot reported a case of portal hypertension in which a loud venous hum was heard at the umbilicus. In 1883, Sappey described accessory portal veins in the suspensory ligament entering the liver capsule through different locations, such as the vessels located at the falciform ligament through which the anterior parietal veins communicate with the left branch of the portal

vein. These vessels play an important role in the origin of transhepatic portosystemic shunts. Paraumbilical vessels may anastomose with the superior epigastric or internal thoracic veins and drain into the superior vena cava or anastomose with the inferior epigastric vein and then drain into the IVC through the external iliac vein. In CB syndrome, the umbilical portion of the

left portal vein feeds the paraumbilical vein that then leaves the liver and extends toward the umbilicus. Our case describes a patient with liver cirrhosis who was found to have a continuous umbilical venous hum on a physical exam with imaging findings consistent with a recanalized paraumbilical vein. It is hypothesized that the recanalization of the paraumbilical vein in portal

hypertension acts to decrease hepatofugal flow, thus becoming a preventative factor in the formation of esophageal varices. Although uncommon, the presence of recanalization of the paraumbilical vein implies a variation in the prognosis in those with decompensated liver cirrhosis, thus, warrants further investigation.
We presented a case of CB syndrome, a condition in which a transhepatic portosystemic shunt can prevent the formation of esophageal varices.


Disclosures:


Carlos M. Ramos-Pachon, MD¹, Andrew Dam, DO¹, Humberto M. Rios, MD¹, Micaella Kantor, MD², Sarah M. Arvaneh, MD¹, Luis M. Nasiff, MD, FACG¹. P0801 - Cruveilhier- Baumgarten Syndrome, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.