Komura J, Yano H, Tanaka Y, Tsuru T
Department of Pediatric Surgery, Kurume University School of Medicine, Fukuoka, Japan.
Eur J Pediatr Surg. 1993 Aug;3(4):244-7. doi: 10.1055/s-2008-1063553.
We report the first known case of an annular pancreas associated with pancreaticobiliary maljunction without cholangiectasis in an infant, aged 2 years and 5 months in Japan. Only two other cases have been reported in Japan both of which were in adults. In our case, the main clinical features were abdominal pain, vomiting and an increasing level of plasma amylase. Endoscopic retrograde cholangiopancreatography (ERCP) was not successful in demonstrating the pancreaticobiliary maljunction due to duodenal stenosis. At operation, a complete type of annular pancreas was found with no enlargement of the common bile duct. We could visualize the pancreaticobiliary maljunction using cholangiopancreatography from the gallbladder during the operation. We then performed duodeno-duodenostomy (side-to-side anastomosis, diamond anastomosis) and portal jejunostomy (Roux en Y anastomosis). The infant was discharged in a good condition at 13 days after the operation. The pattern of the pancreatic ducts was Millbourn's 2a and the type of the duct in the annular portion was Yumura's type I. These results correspond to Lecco's hypothesis that the ring formation originates from the ventral pancreas. It has been further suggested that the cacogenesis and/or dysplasia of the ventral pancreas plays a role in the development at the stage of the pancreaticobiliary maljunction.
我们报告了日本首例已知的2岁5个月婴儿环形胰腺合并胰胆管合流异常且无胆管扩张的病例。日本仅另有两例相关报道，均为成人病例。在我们的病例中，主要临床特征为腹痛、呕吐及血淀粉酶水平升高。由于十二指肠狭窄，内镜逆行胰胆管造影（ERCP）未能成功显示胰胆管合流异常。手术中发现为完全型环形胰腺，胆总管无增粗。术中通过胆囊胰胆管造影可观察到胰胆管合流异常。随后我们进行了十二指肠-十二指肠吻合术（侧侧吻合、菱形吻合）和门静脉空肠吻合术（Roux-en-Y吻合）。婴儿术后13天情况良好出院。胰管形态为米尔本2a型，环形部分的导管类型为汤村I型。这些结果符合莱科的假说，即环状结构起源于腹侧胰腺。进一步表明腹侧胰腺的致癌和/或发育异常在胰胆管合流异常阶段的发育过程中起作用。
