[First orthotopic liver transplantation in patient with biliary atresia and situs inversus in spain].
Biliary atresia is the most common indication for liver transplantation in the pediatric age group. The Kasai portoenterostomy has become established as the primary treatment for biliary atresia. If portoenterostomy fails, death before 2 years of age is likely without liver transplantation. The most common multiple malformation syndrome associated with biliary atresia is polysplenia syndrome, which forms a constellation of defects of body symmetry, splenic development and vascular anomalies, including situs inversus, polysplenia and others. The situs inversus was formerly considered an absolute contraindication for liver transplantation. Recently however, several case reports have been published suggesting that neither situs inversus nor this particular subset of vascular abnormalities should be considered contraindications to liver transplantation. We present one case of liver transplantation performed in patient with biliary atresia, situs inversus and polysplenia. This is the first report described in Spain for a liver transplant in a child with biliary atresia plus situs inversus.
Asensio Llorente
M
M
Grupo de Trasplante Hepático y Cirugía Pediátrica, Hospital Materno Infantil Vall d'Hebron, 119-129, Barcelona.
López Espinosa
J A
JA
Ortega López
J
J
Sánchez Sánchez
L M
LM
Castilla Valdez
M P
MP
Ferrer Blanco
C
C
Margarit Creixell
C
C
Iglesias Berengue
J
J
D000015:Abnormalities, Multiple / Q000601:surgery*; D001656:Biliary Atresia / Q000150:complications / Q000601:surgery*; D006801:Humans; D007223:Infant; D016031:Liver Transplantation / Q000379:methods*; D008297:Male; D012857:Situs Inversus / Q000150:complications / Q000601:surgery*; D013030:Spain; D013154:Spleen / Q000002:abnormalities*; D013577:Syndrome; D016896:Treatment Outcome