title: Pancreatic cysts causing biliary obstruction in von Hippel Lindau syndrome.
abstract: BACKGROUND
  Extrahepatic biliary obstruction secondary to pancreatic cysts is rare in patients with von Hippel Lindau syndrome. We describe a patient with von Hippel Lindau syndrome who had biliary obstruction due to pancreatic cysts who was initially managed endoscopically and then surgically.
  
  
  METHODS
  A female patient with von Hippel Lindau syndrome which had been diagnosed ten years earlier based on the presence of pancreatic and renal cysts with retinal hemangiomas, presented with cholestatic jaundice of two months duration. On investigation, she was found to have lower end biliary obstruction caused by pancreatic cysts. The patient was initially managed with endoscopic retrograde cholangiography and a 7 French/10F/12F biliary plastic stent placement. Her cholestatic symptoms improved but required frequent stent exchange due to stent block; she finally underwent a hepaticojejunostomy and is doing well on follow-up.
  
  
  CONCLUSIONS
  This case highlights the fact that pancreatic involvement leading to biliary obstruction, although uncommon, can occur in patients with von Hippel Lindau syndrome. Endoscopic biliary stent placement and surgery are helpful in these patients.
authors.affiliation: Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
authors.forename: Rahul
authors.initials: R
authors.lastname: Gupta
authors.forename: Deepak
authors.initials: D
authors.lastname: Chettri
authors.forename: Arun
authors.initials: A
authors.lastname: Sharma
authors.forename: Ajay
authors.initials: A
authors.lastname: Duseja
authors.forename: Radha Krishan
authors.initials: RK
authors.lastname: Dhiman
authors.forename: Yogesh Kumar
authors.initials: YK
authors.lastname: Chawla
authors.forename: Naveen
authors.initials: N
authors.lastname: Kalra
authors.forename: Amod
authors.initials: A
authors.lastname: Gupta
authors.forename: Arunanshu
authors.initials: A
authors.lastname: Behera
mesh_terms: D000328:Adult; D002779:Cholestasis / Q000209:etiology* / Q000601:surgery; D005260:Female; D006391:Hemangioma / Q000150:complications / Q000175:diagnosis; D006801:Humans; D041781:Jaundice, Obstructive / Q000209:etiology; D010181:Pancreatic Cyst / Q000150:complications* / Q000601:surgery; D019572:Retinal Neoplasms / Q000150:complications / Q000175:diagnosis; D006623:von Hippel-Lindau Disease / Q000150:complications*