Embryonic biliary atresia in a very-low-birth-weight premature infant.
Two major forms of biliary atresia, the embryonic and perinatal type, are considered to have different pathogeneses and distinct prognoses. Embryonic biliary atresia is associated with worse prognosis. We report a case of embryonic biliary atresia in a preterm male infant of 31 weeks of gestation and weighing 1375 g, with the initial manifestation of intermittent acholic stool 5 days after birth. Kasai portoenterostomy was per-formed at the age of 51 days when he weighed 2164 g. Nevertheless, poorly restored bile flow and progressive cholestasis led to early liver transplantation at the age of 8 months. Liver function had recovered to normal levels by the age of 12 months. Diagnosis of biliary atresia in preterm infants is difficult and requires a high index of suspicion and careful workup. This case illustrates the poor outcome of embryonic biliary atresia and that early liver transplantation may be necessary to improve the prognosis.
Chen
Hung-Wen
HW
Department of Pediatrics, National Taiwan University Hospital, National Taiwan University College of Medicine, Taipei, Taiwan.
Hsu
Wen-Ming
WM
Chang
Mei-Hwei
MH
Chen
Chien-Yi
CY
Chou
Hung-Chieh
HC
Tsao
Po-Nien
PN
Hsieh
Wu-Shiun
WS
D001656:Biliary Atresia / Q000196:embryology* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D007234:Infant, Premature; D019102:Infant, Very Low Birth Weight; D016031:Liver Transplantation; D008297:Male