Intrapulmonary shunting in the biliary atresia/polysplenia syndrome: reversal after liver transplantation.

Abstract:

UNLABELLED:One hundred and seventy three children, including 93 with biliary atresia, received liver grafts at Addenbrooke's Hospital between 1983 and 1993. Of these, only seven developed cyanosis due to intrapulmonary shunting as a complication of their liver disease, and all seven of these had the biliary atresia/polysplenia syndrome. Intrapulmonary shunting was confirmed by a radioisotope scan in four children. Only one child with the syndrome did not have cyanosis when undergoing transplantation. Seven of the eight children are alive 6-54 months after transplantation, with normal pulmonary and hepatic function. Cyanosis recurred in one child who developed chronic rejection with liver failure. IN CONCLUSION:(a) there is a strong association between the biliary atresia/polysplenia syndrome and cyanosis due to intrapulmonary shunting; (b) intrapulmonary shunting is fully reversible after successful liver transplantation; and (c) cyanosis, once present, is progressive, and these children should be considered for liver transplantation as soon as it occurs.

journal_name

Arch Dis Child

authors

Fewtrell MS,Noble-Jamieson G,Revell S,Valente J,Friend P,Johnston P,Rasmussen A,Jamieson N,Calne RY,Barnes ND

doi

10.1136/adc.70.6.501

subject

Has Abstract

pub_date

1994-06-01 00:00:00

pages

501-4

issue

6

eissn

0003-9888

issn

1468-2044

journal_volume

70

pub_type

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