INTRAPULMONARY SHUNTING IN THE BILIARY ATRESIA POLYSPLENIA SYNDROME -REVERSAL AFTER LIVER-TRANSPLANTATION

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