Complete absence of bile and pancreatic ducts in a newborn: a new entity of congenital anomaly in hepato-pancreatic development

Abstract

We examined a newborn who had no bile and pancreatic ducts. Hydrops was evident after 29 weeks of gestation and she died shortly after birth, weighing 1,368 g. One of her siblings had died of hydrops at about six months of gestation, and there were two more miscarriages of unknown cause. At autopsy on the newborn, the liver had an abnormally round shape and the pancreas was not in the normal position. There was an ectopic small pancreas with normally developed islets. Histological analysis revealed the complete absence of extra-and intra-hepatic bile and pancreatic ducts. Immunostaining of these tissues showed no positive bile duct marker staining using epithelial membrane antigen and cytokeratin 19 in the liver. Albumin and a-fetoprotein staining was positive in the liver, and insulin and glucagon staining was positive in the remaining islets. Thus, this case is characterized by complete absence of bile and pancreatic ducts. These findings suggest the existence of a gene linked to the development of bile and pancreatic ducts.

A case of metachronous biliary carcinoma

“…The origin of DCBD could be related to a random subdivision of the hepatic diverticulum during the first week of embryogenesis or due to differences in recanalisation of the hepatic primodium [6,7]. In this patient DCBD was suspected on CT and confirmed during surgery for the cholangiocarcinoma in the distal common bile duct.…”

Type Va extrahepatic bile duct duplication: a case report

“…Our case consists of two long extrahepatic bile ducts joined distally to form a single short common bile duct with the cystic duct draining into the right hepatic duct without the presence of a common hepatic duct. Mechanisms involved in this developmental anomaly include disturbance in the recanalization of the hepatic primordium, random subdivision of the hepatic diverticulum during the first week of embryogenesis, and early disruption of the development of CBD, resulting in the persistence of an extrahepatic accessory duct that is present in early embryogenesis and regresses with normal development [4, 8, 9]. The first description of DCBD was done by Vesalius in 1543 [1].…”

Duplication of the extrahepatic bile duct

“…2002). Mechanisms involved in this developmental anomaly include disturbance in the recanalization of the hepatic primordium (Nakamura et al. 2003), random subdivision of the hepatic diverticulum during the first week of embryogenesis (Knisely 2003), and early disruption of development, resulting in the persistence of an extrahepatic accessory duct that is present in early embryogenesis and regresses with normal development (Choi et al.…”

“…Free flow of the contrast into the duodenum (curved arrow) was noted during the examination. bs_bs_banner involved in this developmental anomaly include disturbance in the recanalization of the hepatic primordium (Nakamura et al 2003), random subdivision of the hepatic diverticulum during the first week of embryogenesis (Knisely 2003), and early disruption of development, resulting in the persistence of an extrahepatic accessory duct that is present in early embryogenesis and regresses with normal development (Choi et al 2007). Goor and Ebert (1972) first classified this anomaly, which was subsequently modified by Saito et al (1988) and recently by Choi et al (2007) to include recently described rare types.…”