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Choledochal Cysts

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Fundamentals of Pediatric Surgery

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Abstract

Choledochal cyst is a rare congenital anomaly that left untreated can progress to cholangitis and biliary cirrhosis. Most commonly grouped by the Todani classification, five types of choledochal cysts have been described with types I and IV being the most common. Signs at presentation may include jaundice, fever, abdominal pain, and a right upper quadrant mass. Because of the widespread use of perinatal ultrasound, these biliary anomalies may also be diagnosed antenatally. Surgical management of a choledochal cyst should proceed in a timely fashion to avoid cholangitis, pancreatitis, and the small risk of cirrhosis. The goal of surgical intervention is complete cyst excision and biliary bypass. Cyst excision with roux-en-Y hepaticojejunostomy is generally effective for types I, II, and IV choledochal cysts. Type III cysts (choledochocele) may be treated using endoscopic sphincterotomy. Patients with type V cysts (Caroli’s disease) require partial hepatectomy or transplantation depending on the extent of biliary abnormalities.

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Authors and Affiliations

  1. Department of Pediatric Surgery, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA

    Inna N. Lobeck MD & Gregory M. Tiao MD

Authors
  1. Inna N. Lobeck MD
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  2. Gregory M. Tiao MD
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Correspondence to Gregory M. Tiao MD .

Editor information

Editors and Affiliations

  1. Department of General Surgery, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA

    Peter Mattei

  2. Division of Pediatric Surgery Department of Surgery, University of Wisconsin, Madison, Wisconsin, USA

    Peter F. Nichol

  3. Primary Children’s Hospital Department of Surgery, University of Utah, Salt Lake City, Utah, USA

    Michael D. Rollins, II

  4. Departments of Surgery and Pediatrics, Alpert Medical School of Brown University, Providence, Rhode Island, USA

    Christopher S. Muratore

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Lobeck, I.N., Tiao, G.M. (2017). Choledochal Cysts. In: Mattei, P., Nichol, P., Rollins, II, M., Muratore, C. (eds) Fundamentals of Pediatric Surgery. Springer, Cham. https://doi.org/10.1007/978-3-319-27443-0_81

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  • DOI: https://doi.org/10.1007/978-3-319-27443-0_81

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-319-27441-6

  • Online ISBN: 978-3-319-27443-0

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