A case series of congenital hepatic hilar cyst: recommendations for diagnosis and management.
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.


OBJECTIVE
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.
Yaniv
Y
Berger
Riccardo A
RA
Superina
Andrew
A
Zbar
Nora
N
Balint-Lahat
Nir
N
Horesh
Ron
R
Bilik
D001656:Biliary Atresia / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D015529:Choledochal Cyst / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D003560:Cysts / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000151:congenital / Q000175:diagnosis* / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000379:methods; D012189:Retrospective Studies