Biliary and gastric drainage in advanced pancreatic serous cystadenoma and portal hypertension in Von Hippel-Lindau syndrome.
Von Hippel-Lindau is a genetic syndrome, comprising several variant mutations on chromosome 3, that predisposes patients to the development of benign and malignant tumors. Tough relatively common, Von Hippel-Lindau syndrome (VHL) with associated hepato-biliary and gastric outlet obstruction, and portal hypertension consequent to the mass effect of a pancreatic serous cystadenoma is a rare scenario. This manuscript reports a 41-year-old female with the aforementioned presentation who successfully underwent a palliative cholecysto- and a gastro-jejunostomy. To the knowledge of the authors, this is the only report, describing a palliative biliary decompression for VHL-related pancreatic serous cystadenoma and portal hypertension.
1 University of Maryland School of Medicine, Division of General and Oncologic Surgery, Baltimore, MD 21201, USA ; 2 University of Maryland Baltimore Washington Medical Center, 301 Hospital Drive, Glen Burnie, MD 21061, USA.
Daniel C
DC
Medina
1 University of Maryland School of Medicine, Division of General and Oncologic Surgery, Baltimore, MD 21201, USA ; 2 University of Maryland Baltimore Washington Medical Center, 301 Hospital Drive, Glen Burnie, MD 21061, USA.
Ricardo
R
Osorno
1 University of Maryland School of Medicine, Division of General and Oncologic Surgery, Baltimore, MD 21201, USA ; 2 University of Maryland Baltimore Washington Medical Center, 301 Hospital Drive, Glen Burnie, MD 21061, USA.
Cherif N
CN
Boutros
Cholecysto-jejunostomy; bile leak; gastro-jejunostomy; jaundice; pancreatic serous cystadenoma; von Hippel-Lindau syndrome (VHL)