Cruvelier-Baumgarten Syndrome Type II in Combination with Arteriovenous Splenic Artery Fistula: Literature Review and Clinical Case

Abstract

Cruvelier-Baumgarten syndrome is a rare disease that causes portal hypertension in association with unclogged umbilical or paraumbilical veins, hypoplasia of the intrahepatic portal system, and liver atrophy with minimal or no fibrosis. Patients with this condition experience the classic signs of portal hypertension such as gastrointestinal bleeding, ascites, and encephalopathy. Frequent clinical manifestations are the symptom of "jellyfish head" and venous murmur over the umbilical region due to the presence of large umbilical and paraumbilical veins. In the presented clinical case of Cruvelier-Baumgarten syndrome type II in combination with splenic artery arteriovenous fistula, pathological changes were not pronounced. There was no "jellyfish head" symptom, but a venous murmur was heard in the umbilical region. The patient complained of moderate pain in the epigastrium, right hypochondrium. Examination of the patient, which revealed, in addition to the typical manifestations of this disease, also multiple SAVFs, as well as angiography data regarding pressure in the umbilical vein - 5 mm Hg, determined the tactics of treatment in the volume of endovascular occlusion of the splenic artery branches, which was successfully completed. As a result, there was a decrease in pressure in the portal system and a sharp decrease in discharge through the recanalized umbilical vein into the basin of the inferior vena cava, which made it possible to level out complaints. At the control examination two months after treatment, the patient had no complaints. A review of the literature on the Cruveilhier-Baumgarten syndrome and disease is presented.