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ABC of Pediatric Surgical ImagingBiliary Atresia

ABC of Pediatric Surgical Imaging: Biliary Atresia Surgeon: A. Alexander A Biliary Atresia Radiologist: L. Tebogo Hlabangana Clinical Diff erential Diagnosis Clinical Insights Alagille syndrome Biliary atresia is a cholangio-destructive Caroli disease disease of the bile ducts of unknown Cholestasis aetiology. Cystic fi brosis The most common, surgically treatable Neonatal hepatitis cause of cholestasis encountered during Alpha 1 antitrypsin defi ciency the newborn period. Infections (toxoplasmosis, rubella, If untreated, the bile ducts obliterate cytomegalovirus infection, herpes leading to cholestasis, cirrhosis and liver simplex virus infection, syphilis) failure. Choledochal cyst Type I involves obliteration of the com- mon bile duct only (good prognosis but rare). Type II is atresia of the common hepatic duct. Type III (>90% of patients) involves atresia of the extra-hepatic ducts to the level of porta hepatis. Following Kasai portoenterostomy, 70% may clear jaundice, but complications include cholangitis (50%), portal hypertension (>60%) and progressive cirrhosis that requires transplantation. Warnings Jaundice that is progressive or persisting S beyond the fi rst 2 weeks of life should always be investigated for an obstructive The four common types of biliary atresia component. The success of portoenterostomy is reduced if the operation is delayed more than 60 days. Clinical investigations should be expedited. 14 Biliary Atresia – http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png

ABC of Pediatric Surgical ImagingBiliary Atresia

Editors: Andronikou, Savvas; Alexander, Angus; Kilborn, Tracy; Millar, Alastair J. W.; Daneman, Alan
Alexander, A.; Hlabangana, L. Tebogo
Springer Journals — Jan 1, 2010
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Publisher
Springer Berlin Heidelberg
Copyright
© Springer-Verlag Berlin Heidelberg 2010
ISBN
978-3-540-89384-4
Pages
14 –15
DOI
10.1007/978-3-540-89385-1_7
Publisher site
See Chapter on Publisher Site

Abstract

Surgeon: A. Alexander A Biliary Atresia Radiologist: L. Tebogo Hlabangana Clinical Diff erential Diagnosis Clinical Insights Alagille syndrome Biliary atresia is a cholangio-destructive Caroli disease disease of the bile ducts of unknown Cholestasis aetiology. Cystic fi brosis The most common, surgically treatable Neonatal hepatitis cause of cholestasis encountered during Alpha 1 antitrypsin defi ciency the newborn period. Infections (toxoplasmosis, rubella, If untreated, the bile ducts obliterate cytomegalovirus infection, herpes leading to cholestasis, cirrhosis and liver simplex virus infection, syphilis) failure. Choledochal cyst Type I involves obliteration of the com- mon bile duct only (good prognosis but rare). Type II is atresia of the common hepatic duct. Type III (>90% of patients) involves atresia of the extra-hepatic ducts to the level of porta hepatis. Following Kasai portoenterostomy, 70% may clear jaundice, but complications include cholangitis (50%), portal hypertension (>60%) and progressive cirrhosis that requires transplantation. Warnings Jaundice that is progressive or persisting S beyond the fi rst 2 weeks of life should always be investigated for an obstructive The four common types of biliary atresia component. The success of portoenterostomy is reduced if the operation is delayed more than 60 days. Clinical investigations should be expedited. 14 Biliary Atresia –

Published: Jan 1, 2010

Keywords: Bile Duct; Cystic Fibrosis; Portal Hypertension; Biliary Atresia; Choledochal Cyst

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