A case series of congenital hepatic hilar cyst

Recommendations for diagnosis and management

Yaniv Berger*, Riccardo A. Superina, Andrew Zbar, Nora Balint-Lahat, Nir Horesh, Ron Bilik

*Corresponding author for this work

Research output: Contribution to journalArticle

2 Citations (Scopus)

Abstract

Background: Congenital hepatic hilar cysts are rare. Some are simple and do not require intervention, but some biliary cystic malformations impose the risk of morbidity and mortality.

Objectives: To assess a series of five patients presenting with congenital hepatic hilar cysts.

Methods: We retrospectively reviewed all cases presenting to our pediatric surgical service between January 2010 and December 2012 and found to have a congenital hepatic hilar cyst. Data regarding clinical, radiological, operative and pathological features were analyzed.

Results: Five children with congenital cyst of the hepatic hilum were identified; four of them were diagnosed prenatally. Four children had undergone surgical intervention: one with intrahepatic choledochal cyst, one with epidermoid cyst, and two with biliary atresia and an associated cyst of the common bile duct. In another case of choledochal cyst the treatment was conservative. All children except one had a good prognosis; one child with biliary atresia required liver transplantation.

Conclusions: The differential diagnosis of congenital hepatic hilar cyst includes a broad spectrum of pathologies. It is essential to diagnose biliary atresia as early as possible. Signs such as smaller cysts in association with a hypoplastic gallbladder and direct hyperbilirubinemia may be suggestive of biliary atresia.

Original languageEnglish (US)
Pages (from-to)32-36
Number of pages5
JournalIsrael Medical Association Journal
Volume17
Issue number1
StatePublished - Jan 1 2015

Fingerprint

Biliary Atresia
Cysts
Choledochal Cyst
Liver
Epidermal Cyst
Hyperbilirubinemia
Gallbladder
Liver Transplantation
Differential Diagnosis
Pediatrics
Pathology
Morbidity
Mortality

Keywords

  • Biliary atresia
  • Choledochal cyst
  • Epidermoid cyst
  • Hepatic hilar cyst
  • Kasai porto-enterostomy

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Berger, Yaniv ; Superina, Riccardo A. ; Zbar, Andrew ; Balint-Lahat, Nora ; Horesh, Nir ; Bilik, Ron. / A case series of congenital hepatic hilar cyst : Recommendations for diagnosis and management. In: Israel Medical Association Journal. 2015 ; Vol. 17, No. 1. pp. 32-36.
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abstract = "Background: Congenital hepatic hilar cysts are rare. Some are simple and do not require intervention, but some biliary cystic malformations impose the risk of morbidity and mortality.Objectives: To assess a series of five patients presenting with congenital hepatic hilar cysts.Methods: We retrospectively reviewed all cases presenting to our pediatric surgical service between January 2010 and December 2012 and found to have a congenital hepatic hilar cyst. Data regarding clinical, radiological, operative and pathological features were analyzed.Results: Five children with congenital cyst of the hepatic hilum were identified; four of them were diagnosed prenatally. Four children had undergone surgical intervention: one with intrahepatic choledochal cyst, one with epidermoid cyst, and two with biliary atresia and an associated cyst of the common bile duct. In another case of choledochal cyst the treatment was conservative. All children except one had a good prognosis; one child with biliary atresia required liver transplantation.Conclusions: The differential diagnosis of congenital hepatic hilar cyst includes a broad spectrum of pathologies. It is essential to diagnose biliary atresia as early as possible. Signs such as smaller cysts in association with a hypoplastic gallbladder and direct hyperbilirubinemia may be suggestive of biliary atresia.",
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Berger, Y, Superina, RA, Zbar, A, Balint-Lahat, N, Horesh, N & Bilik, R 2015, 'A case series of congenital hepatic hilar cyst: Recommendations for diagnosis and management', Israel Medical Association Journal, vol. 17, no. 1, pp. 32-36.

A case series of congenital hepatic hilar cyst : Recommendations for diagnosis and management. / Berger, Yaniv; Superina, Riccardo A.; Zbar, Andrew; Balint-Lahat, Nora; Horesh, Nir; Bilik, Ron.

In: Israel Medical Association Journal, Vol. 17, No. 1, 01.01.2015, p. 32-36.

Research output: Contribution to journalArticle

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T1 - A case series of congenital hepatic hilar cyst

T2 - Recommendations for diagnosis and management

AU - Berger, Yaniv

AU - Superina, Riccardo A.

AU - Zbar, Andrew

AU - Balint-Lahat, Nora

AU - Horesh, Nir

AU - Bilik, Ron

PY - 2015/1/1

Y1 - 2015/1/1

N2 - Background: Congenital hepatic hilar cysts are rare. Some are simple and do not require intervention, but some biliary cystic malformations impose the risk of morbidity and mortality.Objectives: To assess a series of five patients presenting with congenital hepatic hilar cysts.Methods: We retrospectively reviewed all cases presenting to our pediatric surgical service between January 2010 and December 2012 and found to have a congenital hepatic hilar cyst. Data regarding clinical, radiological, operative and pathological features were analyzed.Results: Five children with congenital cyst of the hepatic hilum were identified; four of them were diagnosed prenatally. Four children had undergone surgical intervention: one with intrahepatic choledochal cyst, one with epidermoid cyst, and two with biliary atresia and an associated cyst of the common bile duct. In another case of choledochal cyst the treatment was conservative. All children except one had a good prognosis; one child with biliary atresia required liver transplantation.Conclusions: The differential diagnosis of congenital hepatic hilar cyst includes a broad spectrum of pathologies. It is essential to diagnose biliary atresia as early as possible. Signs such as smaller cysts in association with a hypoplastic gallbladder and direct hyperbilirubinemia may be suggestive of biliary atresia.

AB - Background: Congenital hepatic hilar cysts are rare. Some are simple and do not require intervention, but some biliary cystic malformations impose the risk of morbidity and mortality.Objectives: To assess a series of five patients presenting with congenital hepatic hilar cysts.Methods: We retrospectively reviewed all cases presenting to our pediatric surgical service between January 2010 and December 2012 and found to have a congenital hepatic hilar cyst. Data regarding clinical, radiological, operative and pathological features were analyzed.Results: Five children with congenital cyst of the hepatic hilum were identified; four of them were diagnosed prenatally. Four children had undergone surgical intervention: one with intrahepatic choledochal cyst, one with epidermoid cyst, and two with biliary atresia and an associated cyst of the common bile duct. In another case of choledochal cyst the treatment was conservative. All children except one had a good prognosis; one child with biliary atresia required liver transplantation.Conclusions: The differential diagnosis of congenital hepatic hilar cyst includes a broad spectrum of pathologies. It is essential to diagnose biliary atresia as early as possible. Signs such as smaller cysts in association with a hypoplastic gallbladder and direct hyperbilirubinemia may be suggestive of biliary atresia.

KW - Biliary atresia

KW - Choledochal cyst

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KW - Kasai porto-enterostomy

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