Résumé
OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.
Sujets)
Humains , Mâle , Femelle , Nouveau-né , Nourrisson , Atrésie des voies biliaires/chirurgie , Hépato-porto-entérostomie/méthodes , Facteurs temps , Atrésie des voies biliaires/mortalité , Atrésie des voies biliaires/anatomopathologie , Brésil/épidémiologie , Hépato-porto-entérostomie/mortalité , Taux de survie , Études rétrospectives , Facteurs âges , Transplantation hépatique/méthodes , Transplantation hépatique/mortalité , Résultat thérapeutique , Estimation de Kaplan-Meier , Ictère néonatal/chirurgie , Ictère néonatal/anatomopathologie , Foie/chirurgie , Foie/anatomopathologieRésumé
O presente trabalho tem por objetivo alertar para a importância do diangóstico e tratamento precoce da hiperbilirrubinemia direta, por atresia de vias biliares, devido a sua grande complicaçäo que é a cirrose hepática e conseqüente morte do lactente. O médico dispöe de pouco tempo entre o diagnóstico e o tratamento quando possível, sendo que após 2 a 3 meses, terá danos irreversíveis ao lactente. Quando houver quadro icterico, com acolia fecal, colúria, e predomínio da bilirrubina direta, sempre ter na hipótese diagnóstica a atresia de vias biliares. Por ser uma patologia de baixa incidência, muitas vezes poderá passar desapercebido,levando o lactente a condenaçäo