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Imaging Case Book

Neonatal type 1 choledochal cyst

Journal of Perinatology volume 27, pages 453–454 (2007)Cite this article

The initial abdominal sonogram demonstrated a large RUQ cyst in the porta hepatis. The main intrahepatic biliary ducts were dilated. A gallbladder was identified. The mass displaced the hepatic artery anteriorly. Echogenic debris was present in the dependent portion of the cyst. Given this appearance, a choledochal cyst was considered most likely. The infant was taken to the operating room where the cystogram demonstrated a choledochal cyst without biliary atresia (BA) as contrast flowed freely antegrade into the duodenum and retrograde into the slightly dilated intrahepatic biliary tree.

Choledochal cysts were reportedly described first by Abraham Vater in 1723.1 Choledochal cysts are cystic dilatations of the extrahepatic and intrahepatic biliary tree or both. They are approximately three times more common in women than in men. Although the etiology is not known, it has been shown that in at least 94% of choledochal cysts there are anomalous insertions of the common bile duct into the pancreatic duct.2 It is believed that this allows reflux of pancreatic enzymes into the biliary tree leading to ductal dilatation and scarring. Among the various classification schemes, that of Todani is widely used. The cystic dilatation in this patient is that of Todani type I. This comprises 80–90% of all choledochal cysts.1 Todani type II are diverticular dilatations of the common bile duct. Todani type III is a choledocele, a malformation of the ampulla of Vater that does not have a high insertion of the pancreatic duct and common bile duct. Type IV is a choledochal cyst associated with other intrahepatic and extrahepatic cysts or Caroli's disease. Type V is intrahepatic cystic biliary dilatation or Caroli's disease.

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References

  1. Savader SJ, Benenati JF, Venbrux AC, Mitchell SE, Widlus DM, Cameron JL et al. Choledocahl cysts: classification and cholangiography appearance. A J Roentgenol 1991; 156: 327–331.

    Article  CAS  Google Scholar 

  2. Kimura K, Ohto M, Ono T, Tsuchiya Y, Saisho H, Kawamura K et al. Congenital cystic dilatation of the common bile duct: relationship to anomalous pancreaticobiliary duct union. Am J Roentegenol 1977; 128: 571–577.

    Article  CAS  Google Scholar 

  3. Yoshida H, Itai Y, Minami M, Kokubo T, Ohtomo K, Kuroda A . Biliary malignancies occurring in choledochal cysts. Radiology 1989; 173: 389–392.

    Article  CAS  Google Scholar 

  4. Iwa N, Deguchi E, Yanagihara J, Iwai M, Matsuo H, Todo S et al. Cancer arising in a choledochal cyst in a 12-year-old girl. J Pediatr Surg 1990; 25: 1261–1263.

    Article  Google Scholar 

  5. Bass EM, Cremin BJ . Choledochal cysts: clinical and radiological evaluation of 21 cases. Pediatr Radiol 1976; 5: 81–85.

    Article  CAS  Google Scholar 

  6. Suita S, Shono K, Kinugasa Y, Kubota M, Matsuo S . Influence of age on the presentation and outcome of choledochal cysts. J Pediatr Surg 1999; 34: 1765–1768.

    Article  CAS  Google Scholar 

  7. Tsuchida Y, Kawarasaki H, Iwanaka T, Uchida H, Nakanishi H, Uno K . Antenatal diagnosis of biliary atresia (Type I cyst) at 19 weeks gestation: differential diagnosis and etiologic implications. J Pediatr Surg 1995; 30: 697–699.

    Article  CAS  Google Scholar 

  8. Saito T, Horie H, Yoshida H, Matsunaga T, Kouchi K, Kuroda H et al. The perinatal transition of the hepatobiliary cyst size provides information about the condition of bile flow in biliary cystic malformation cases. J Pediatr Surg 2006; 41: 1397–1402.

    Article  Google Scholar 

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  1. St Louis Children's Hospital Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, MO, USA

    T E Herman & M J Siegel

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  1. T E Herman
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  2. M J Siegel
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Correspondence to T E Herman.

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Herman, T., Siegel, M. Neonatal type 1 choledochal cyst. J Perinatol 27, 453–454 (2007). https://doi.org/10.1038/sj.jp.7211759

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