Gupta Rahul, Chettri Deepak, Sharma Arun, Duseja Ajay, Dhiman Radha Krishan, Chawla Yogesh Kumar, Kalra Naveen, Gupta Amod, Behera Arunanshu
Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
JOP. 2008 May 8;9(3):313-6.
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.
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.
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.
在冯·希佩尔-林道综合征患者中，胰腺囊肿继发肝外胆管梗阻较为罕见。我们描述了一名患有冯·希佩尔-林道综合征的患者，该患者因胰腺囊肿导致胆管梗阻，最初接受内镜治疗，随后接受了手术治疗。
一名女性患者，十年前因胰腺和肾囊肿以及视网膜血管瘤被诊断为冯·希佩尔-林道综合征，出现了持续两个月的胆汁淤积性黄疸。经检查，发现她因胰腺囊肿导致低位胆管梗阻。该患者最初接受了内镜逆行胆管造影及置入7法式/10F/12F胆道塑料支架治疗。她的胆汁淤积症状有所改善，但由于支架堵塞需要频繁更换支架；她最终接受了肝空肠吻合术，随访情况良好。
该病例突出了这样一个事实，即导致胆管梗阻的胰腺受累情况，虽然不常见，但在冯·希佩尔-林道综合征患者中可能发生。内镜下胆道支架置入和手术对这些患者有帮助。
