RESUMO
OBJECTIVE: To determine the influence of the new onset of esophageal variceal hemorrhage (EVH) on transplant-free survival in children with biliary atresia and to examine variables that predicted survival after the onset of EVH. METHODS: Retrospective chart review of 134 patients with biliary atresia who underwent portoenterostomy between 1973 and 1992 at a single institution; 29% had EVH. RESULTS: The risk of death or need for liver transplantation was 50% at 6 years after the initial episode of EVH. Patients with a serum bilirubin concentration < or =4 mg/dL at the first episode of EVH had transplant-free survival of >80% for 4 years after this episode, those with bilirubin levels >4 to 10 mg/dL had 50% survival at 1 year, and those with bilirubin levels >10 mg/dL had 50% survival at 4 months. The risk of death or transplant for a child with EVH and total serum bilirubin levels >10 mg/dL was 12.0 (95% CI: 6.0, 24.1), 4 to 10 mg/dL was 7.2 (3.1, 16.7), and < or =4 mg/dL was 0.6 (0.1, 3.1) times the risk of a same-aged child who did not have EVH. CONCLUSIONS: Children with biliary atresia and first EVH episode have a variable prognosis related to total serum bilirubin concentration at the time of the episode.
Assuntos
Atresia Biliar/cirurgia , Varizes Esofágicas e Gástricas/mortalidade , Hemorragia Gastrointestinal/mortalidade , Transplante de Fígado , Atresia Biliar/complicações , Bilirrubina/sangue , Criança , Pré-Escolar , Varizes Esofágicas e Gástricas/complicações , Feminino , Humanos , Lactente , Masculino , Estudos Retrospectivos , Risco , Taxa de SobrevidaRESUMO
Increased portal pressure is the product of both increased resistance to splanchnic flow through the liver and increased blood flow in the portal circuit. Although portal hypertension in children is less common than in adults, the important clinical end results are the same, ie, esophageal variceal hemorrhage, ascites, and hypersplenism. The etiology of portal hypertension in children is very different from adults in whom cirrhosis (most commonly secondary to alcohol) is the predominant cause. In children, extrahepatic obstruction due to portal vein thrombosis is the most common cause. However, as children survive longer with biliary atresia, cystic fibrosis, and other liver diseases, the incidence of intrahepatic obstruction causing portal hypertension is increasing. The treatment has also undergone a dramatic evolution over the last decade with the near extinction of portosystemic shunt procedures and their replacement with endoscopic treatment of esophageal varices and liver transplantation.
Assuntos
Hipertensão Portal , Criança , Pré-Escolar , Varizes Esofágicas e Gástricas , Hemorragia Gastrointestinal , Hemostase Endoscópica , Humanos , Lactente , Transplante de Fígado , EscleroterapiaRESUMO
The Registry provides information about 904 children with biliary atresia from more than 100 institutions. There was a 1.4 to 1 female predominance; racial distribution was 62% caucasian, 20% black, 11% Hispanic, 4.2% asian, and 1.5% American Indian. Eight hundred sixteen (90%) underwent corrective surgery (median age at operation, 69 days). Intraoperatively, 70% had totally obliterated extrahepatic bile ducts, 22% had patency of the gallbladder and distal common duct, whereas only 8% had "correctable" biliary atresia (proximal duct patency). A variety of reconstructions were used, but the majority of patients had a Roux-en-Y portoenterostomy with or without exteriorization. Follow-up was available for 670 children (74%) with average length of follow-up of 5 years (range, 1 to 16 years). Five-year actuarial survival was 48% following Kasai's operation, but was less than 10% (at 3 years) if no operative correction was done. Survival was unaffected by sex, type of reconstruction, or cholangitis. Predictors of a bad outcome were (1) caucasian race; (2) operative age greater than 60 days; (3) presence of cirrhosis at initial biopsy; (4) totally nonpatent extrahepatic ducts; (5) absent ducts at the level of transection in the liver hilus; and (6) subsequent development of varices or ascites. Identification of factors predictive of the ultimate outcome provide a basis for either continued efforts with management of Kasai's operation or for early referral for liver transplantation.