Maximising Kasai portoenterostomy in the treatment of biliary atresia: Medical and surgical options

TY - JOUR

T1 - Maximising Kasai portoenterostomy in the treatment of biliary atresia

T2 - Medical and surgical options

AU - Grieve, A.

AU - Davenport, Mark

PY - 2012/11

Y1 - 2012/11

N2 - Biliary atresia (BA) remains one of the most challenging conditions in paediatric surgery. It has several possible causes, resulting in a range of different clinical scenarios. The current therapeutic approach is almost entirely surgical with an initial attempt to restore bile flow and preserve the native liver using a Kasai-type portoenterostomy. Liver transplantation (cadaveric or living donor) is usually reserved for failure or for infants presenting late with end, stage cirrhosis. The role of adjuvant medical therapy is unclear and evidence of benefit is lacking. Nonetheless, the use of post-operative steroids, prophylactic antibiotics and choleretic agents such as ursodeoxycholic acid is common. Ideally, the entire pathway should be complementary and seamless with few infants dying of end-stage liver, disease or uncorrectable associated congenital malformations. Experience from high-volume centres suggests that clearance of jaundice can be achieved in 50 - 60% of infants, with 10-year native liver and real survival rates of 45% and 90%, respectively.

AB - Biliary atresia (BA) remains one of the most challenging conditions in paediatric surgery. It has several possible causes, resulting in a range of different clinical scenarios. The current therapeutic approach is almost entirely surgical with an initial attempt to restore bile flow and preserve the native liver using a Kasai-type portoenterostomy. Liver transplantation (cadaveric or living donor) is usually reserved for failure or for infants presenting late with end, stage cirrhosis. The role of adjuvant medical therapy is unclear and evidence of benefit is lacking. Nonetheless, the use of post-operative steroids, prophylactic antibiotics and choleretic agents such as ursodeoxycholic acid is common. Ideally, the entire pathway should be complementary and seamless with few infants dying of end-stage liver, disease or uncorrectable associated congenital malformations. Experience from high-volume centres suggests that clearance of jaundice can be achieved in 50 - 60% of infants, with 10-year native liver and real survival rates of 45% and 90%, respectively.

KW - CHOLESTATIC LIVER-DISEASE

KW - URSODEOXYCHOLIC ACID

KW - SURGERY

U2 - 10.7196/SAMJ.6120

DO - 10.7196/SAMJ.6120

M3 - Literature review

SN - 0256-9574

VL - 102

SP - 865

EP - 867

JO - SAMJ SOUTH AFRICAN MEDICAL JOURNAL

JF - SAMJ SOUTH AFRICAN MEDICAL JOURNAL

IS - 11

ER -