Mandraka F, Zuelke C, Lock G
Klinik und Poliklinik für Innere Medizin I, Klinikum der Universität Regensburg.
Dtsch Med Wochenschr. 2003 May 2;128(18):984-8. doi: 10.1055/s-2003-38955.
A 54-year-old patient with painless jaundice and vomiting had been diagnosed with a Peutz-Jeghers syndrome 20 years before.
The blood analysis showed a cholestatic constellation as well as increased transaminases. Sonographic, radiological, endoscopic and histological findings indicated multiple hamartomatous polyps of the Peutz-Jeghers' type in the entire small and large bowel with occlusion of the papilla of Vater and the superior gastrointestinal tract by a big polyp.
After an initial percutaneous transhepatic cholangiographic drainage, Whipple's operation and a segmental resection of small and large bowel were performed. A highly differentiated adenocarcinoma of the duodenum was found in the resected specimen.
This case demonstrates the potentially severe complications of a Peutz-Jeghers syndrome that had been neglected for years.
一名54岁无痛性黄疸和呕吐患者20年前被诊断为佩-吉综合征。
血液分析显示胆汁淤积及转氨酶升高。超声、放射学、内镜及组织学检查结果表明,全小肠和大肠存在多发佩-吉型错构瘤性息肉,一个大息肉阻塞了 Vater 壶腹和上消化道。
最初行经皮经肝胆管造影引流,之后进行了惠普尔手术及小肠和大肠部分切除术。切除标本中发现十二指肠高分化腺癌。
该病例显示了多年来被忽视的佩-吉综合征可能引发的严重并发症。