Grieb, Dominik and Feldkamp, Axel and Lang, Thomas and Melter, Michael and Stroszczynski, Christian and Brassel, Friedhelm and Meila, Dan (2014) Caroli Disease Associated With Vein of Galen Malformation in a Male Child. PEDIATRICS, 134 (1). E284-E288. ISSN 0031-4005, 1098-4275
Full text not available from this repository. (Request a copy)Abstract
We report the first case of a male child with both Caroli disease and vein of Galen malformation. The neonate presented to our department with congestive heart failure as a result of the intracranial arteriovenous high-flow shunt. Over time, several endovascular embolizations led to a complete angiographic occlusion of the shunt. Additionally, the diagnosis of Caroli disease was made at the age of 2 months. He developed choledocholithiasis necessitating endoscopic sphincterotomy and stone extraction. As a prolonged medical treatment he received ursodeoxycholic acid and antibiotics. A coincidence of Caroli disease and vein of Galen malformation has not yet been described. Both diseases are very rare, leading to the question of whether there is a link in the pathogenesis. Based on the few previously described underlying mechanisms, we develop hypotheses about the relationship between both rare diseases. We consider overexpression of vascular endothelial growth factor and its receptors as a possible common molecular mechanism in their pathogenesis. We also highlight the critical role of increased expression of the Notch ligand Jagged 1 both in the development of cerebral arteriovenous malformations in general and in the formation of dilated intrahepatic bile ducts (eg, in Caroli disease).
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | INTRAHEPATIC BILE-DUCTS; ANEURYSMS; NOTCH; vein of Galen malformation; Caroli disease; intracranial aneurysms; VEGF |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Kinder- und Jugendmedizin Medicine > Lehrstuhl für Röntgendiagnostik |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 17 Oct 2019 11:05 |
| Last Modified: | 17 Oct 2019 11:05 |
| URI: | https://pred.uni-regensburg.de/id/eprint/9957 |
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