Pancreatic cysts causing biliary obstruction in von Hippel Lindau syndrome.
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.
Gupta
Rahul
R
Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Chettri
Deepak
D
Sharma
Arun
A
Duseja
Ajay
A
Dhiman
Radha Krishan
RK
Chawla
Yogesh Kumar
YK
Kalra
Naveen
N
Gupta
Amod
A
Behera
Arunanshu
A
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*