title: The perinatal transition of the hepatobiliary cyst size provides information about the condition of bile flow in biliary cystic malformation cases.
abstract: OBJECTIVE
  The aim of this study was to understand the level of bile flow by perinatal transitions of the hepatobiliary cyst size in biliary cystic malformation (BCM) cases and to examine the association between the transitions of the cyst size, cholangiographic images of the intrahepatic bile ducts (IHBDs), and histological features of the liver specimens in BCM.
  
  
  METHODS
  We measured perinatal size of the hepatobiliary cyst in 7 BCM cases, composed of 1 case with choledochal cyst and 6 cases with biliary atresia (BA). Bile excretion was inferred from the transition of cyst size and the postoperative course. The IHBD cholangiographic images were also examined. In addition, histology of liver specimens obtained at the time of initial surgery was evaluated for IHBD maturity and fibrosis.
  
  
  RESULTS
  The size of the small cyst in 2 BA cases remained almost unchanged throughout gestation. These cases required longer postoperative period for the clearance of jaundice. Their IHBD images were indistinct, and histology showed severe fibrosis. The ratio of bile ducts to portal tracts was 0.5 in 1 of the cases. However, in other BCM cases where the cysts grew large perinatally, bile excretion was good postoperatively, and their jaundice cleared in shorter period. Their cholangiographic images demonstrated clearer periphery of the IHBDs, and their histology showed mild or moderate fibrosis, if any.
  
  
  CONCLUSIONS
  The perinatal transition of the cyst size in BCM cases can be helpful in assessing the level of bile flow. BCM with a small cyst during gestation can include some BAs with poorer bile flow, vaguer IHBD images, and prominent liver fibrosis. For the treatment of BCM cases, especially patients with a small biliary cyst, prompt response may be required after birth.
authors.affiliation: Department of Pediatric Surgery (E6), Graduate School of Medicine, Chiba University, Chuo-ku, Chiba 260-8670, Japan. takk@orion.ocn.ne.jp
authors.forename: Takeshi
authors.initials: T
authors.lastname: Saito
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Horie
authors.forename: Hideo
authors.initials: H
authors.lastname: Yoshida
authors.forename: Tadashi
authors.initials: T
authors.lastname: Matsunaga
authors.forename: Katsunori
authors.initials: K
authors.lastname: Kouchi
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Kuroda
authors.forename: Takao
authors.initials: T
authors.lastname: Etoh
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Higashimoto
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Kuriyama
authors.forename: Naomi
authors.initials: N
authors.lastname: Ohnuma
mesh_terms: D001646:Bile / Q000502:physiology; D001649:Bile Duct Diseases / Q000151:congenital / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D001656:Biliary Atresia / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D002758:Cholangiography; D015529:Choledochal Cyst / Q000175:diagnosis / Q000503:physiopathology / Q000601:surgery; D003560:Cysts / Q000151:congenital / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D004065:Digestive System Abnormalities / Q000175:diagnosis* / Q000503:physiopathology / Q000601:surgery; D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008099:Liver / Q000473:pathology; D008297:Male; D011247:Pregnancy; D016216:Ultrasonography, Prenatal