Ando H, Ito T, Nagaya M, Watanabe Y, Seo T, Kaneko K
Department of Surgery, Branch Hospital, Nagoya University School of Medicine, Japan.
J Pediatr Surg. 1995 Dec;30(12):1658-62. doi: 10.1016/0022-3468(95)90445-x.
Pancreaticobiliary maljunction (PBM) usually is associated with choledochal cyst. PBM without dilatation of the common bile duct is rare in infants and children. This rare type of the anomaly may lead to the development of malignancy of the bile duct in later life. The authors report the clinical presentation and surgical treatment of seven pediatric patients with PBM. Symptoms and signs included repeated episodes of abdominal pain (7 of 7), nausea and vomiting (6 of 7), intermittent jaundice (3 of 7), and acholic stools (2 of 7). Some patients presented with high levels of serum and urinary amylase. These symptoms and signs might have been related to the temporary obstruction of bile flow in the common channel, where endoscopic retrograde cholangiopancreaticography (ERCP) disclosed a protein plug in four of the patients. The common bile duct proximal to the junction of the main pancreatic duct was excised, and a Roux-en-Y hepaticojejunostomy reconstruction was performed. To prevent iatrogenic injury of the main pancreatic duct, repeat cholangiography was performed with the aid of radiopaque silver clips placed on the line of dissection to evaluate the distance between the site of dissection and the main pancreatic duct. All patients have been free of symptoms since the surgery. PBM without dilatation of the common bile duct can be detected more frequently if ERCP is performed on every patient who has repeated episodes of abdominal pain refractory to conventional therapy. Complete excision of the common bile duct and gallbladder followed by hepaticojejunostomy is recommended for PBM, while the goals of decreasing the high risk of carcinoma of the bile duct and preventing recurrent symptoms.
胰胆管合流异常（PBM）通常与胆总管囊肿相关。在婴幼儿和儿童中，胆总管无扩张的PBM较为罕见。这种罕见类型的异常可能导致后期胆管恶性肿瘤的发生。作者报告了7例小儿PBM患者的临床表现及手术治疗情况。症状和体征包括反复腹痛（7例中的7例）、恶心和呕吐（7例中的6例）、间歇性黄疸（7例中的3例）及无胆汁粪便（7例中的2例）。部分患者血清和尿淀粉酶水平升高。这些症状和体征可能与共同通道中胆汁流动的暂时受阻有关，在内镜逆行胰胆管造影（ERCP）检查中，4例患者发现有蛋白栓。切除主胰管汇合处近端的胆总管，并进行Roux-en-Y肝空肠吻合术重建。为防止医源性主胰管损伤，在解剖线上放置不透X线的银夹以辅助重复胆管造影，评估解剖部位与主胰管之间的距离。所有患者术后均无症状。对于每一位经传统治疗后仍反复腹痛的患者，如果进行ERCP检查，胆总管无扩张的PBM可能更容易被发现。对于PBM，建议完整切除胆总管和胆囊，随后行肝空肠吻合术，目的是降低胆管癌的高风险并防止症状复发。
