Price Leslie, Kozarek Richard, Agoff Nicholas
Virginia Mason Medical Center, 1100 9th Ave., Seattle, WA 98101, USA.
Dig Dis Sci. 2008 Oct;53(10):2822-5. doi: 10.1007/s10620-007-0182-x. Epub 2008 Feb 15.
Choledochal cysts are rare congenital or acquired cystic dilatations of the intra- or extrahepatic bile ducts. Their exact pathophysiology remains unclear. Extensive complications exist with untreated cysts, including biliary stasis and inflammation, with resultant stricture and stone formation, cholangitis, pancreatitis, and malignant degeneration. Most commonly reported malignancies include cholangiocarcinoma, adenocarcinoma, and gallbladder cancer. We report the case of a 41-year-old female with a history of chronic calcific pancreatitis, choledocholithiasis, and a large type I choledochal cyst, who presented with flu-like symptoms followed by painless jaundice and weight loss. Endoscopic retrograde cholangiopancreatography and direct cholangioscopy revealed a mass at the biliary bifurcation, a 4-cm choledochal cyst with multiple calculi, absence of anomalous pancreaticobiliary ductal union, and multiple calcifications in the pancreatic head. Pathology demonstrated a synchronous choledochal cyst and squamous cell carcinoma, an infrequently reported phenomenon. We report the associated rare finding of chronic calcific pancreatitis, without anomaly of the pancreatic biliary junction. This suggests that the formation of calculi within the choledochal cyst may contribute to chronic calcific pancreatitis, possibly by virtue of papillary stenosis.
胆总管囊肿是肝内或肝外胆管罕见的先天性或后天性囊性扩张。其确切的病理生理学仍不清楚。未经治疗的囊肿存在广泛的并发症，包括胆汁淤积和炎症，继而导致狭窄和结石形成、胆管炎、胰腺炎和恶变。最常报道的恶性肿瘤包括胆管癌、腺癌和胆囊癌。我们报告一例41岁女性病例，有慢性钙化性胰腺炎、胆总管结石和一个大型I型胆总管囊肿病史，该患者最初出现类似流感的症状，随后出现无痛性黄疸和体重减轻。内镜逆行胰胆管造影和直接胆管镜检查显示在胆管分叉处有一个肿物、一个4厘米的胆总管囊肿伴有多发结石、无胰胆管合流异常，且胰头有多发钙化。病理显示为同时存在胆总管囊肿和鳞状细胞癌，这是一种很少报道的现象。我们报告了伴有慢性钙化性胰腺炎这一罕见发现，且无胰胆管交界处异常。这表明胆总管囊肿内结石的形成可能导致慢性钙化性胰腺炎，可能是由于乳头狭窄所致。
