Predictors of Enteral Autonomy in Children with Intestinal Failure: A Multicenter Cohort Study.

OBJECTIVES: In a large cohort of children with intestinal failure (IF), we sought to determine the cumulative incidence of achieving enteral autonomy and identify patient and institutional characteristics associated with enteral autonomy. STUDY DESIGN: A multicenter, retrospective cohort analysis from the Pediatric Intestinal Failure Consortium was performed. IF was defined as severe congenital or acquired gastrointestinal diseases during infancy with dependence on parenteral nutrition (PN) >60 days. Enteral autonomy was defined as PN discontinuation >3 months. RESULTS: A total of 272 infants were followed for a median (IQR) of 33.5 (16.2-51.5) months. Enteral autonomy was achieved in 118 (43%); 36 (13%) remained PN dependent and 118 (43%) patients died or underwent transplantation. Multivariable analysis identified necrotizing enterocolitis (NEC; OR 2.42, 95% CI 1.33-4.47), care at an IF site without an associated intestinal transplantation program (OR 2.73, 95% CI 1.56-4.78), and an intact ileocecal valve (OR 2.80, 95% CI 1.63-4.83) as independent risk factors for enteral autonomy. A second model (n = 144) that included only patients with intraoperatively measured residual small bowel length found NEC (OR 3.44, 95% CI 1.36-8.71), care at a nonintestinal transplantation center (OR 6.56, 95% CI 2.53-16.98), and residual small bowel length (OR 1.04 cm, 95% CI 1.02-1.06 cm) to be independently associated with enteral autonomy. CONCLUSIONS: A substantial proportion of infants with IF can achieve enteral autonomy. Underlying NEC, preserved ileocecal valve, and longer bowel length are associated with achieving enteral autonomy. It is likely that variations in institutional practices and referral patterns also affect outcomes in children with IF.

Natural history of pediatric intestinal failure: initial report from the Pediatric Intestinal Failure Consortium.

OBJECTIVE: To characterize the natural history of intestinal failure (IF) among 14 pediatric centers during the intestinal transplantation era. STUDY DESIGN: The Pediatric Intestinal Failure Consortium performed a retrospective analysis of clinical and outcome data for a multicenter cohort of infants with IF. Entry criteria included infants <12 months receiving parenteral nutrition (PN) for >60 continuous days. Enteral autonomy was defined as discontinuation of PN for >3 consecutive months. Values are presented as median (25th, 75th percentiles) or as number (%). RESULTS: 272 infants with a gestational age of 34 weeks (30, 36) and birth weight of 2.1 kg (1.2, 2.7) were followed for 25.7 months (11.2, 40.9). Residual small bowel length in 144 patients was 41 cm (25.0, 65.5). Diagnoses were necrotizing enterocolitis (71, 26%), gastroschisis (44, 16%), atresia (27, 10%), volvulus (24, 9%), combinations of these diagnoses (46, 17%), aganglionosis (11, 4%), and other single or multiple diagnoses (48, 18%). Prescribed medications included oral antibiotics (207, 76%), H2 blockers (187, 69%), and proton pump inhibitors (156, 57%). Enteral feeding approaches varied among centers; 19% of the cohort received human milk. The cohort experienced 8.9 new catheter-related blood stream infections per 1000 catheter days. The cumulative incidences for enteral autonomy, death, and intestinal transplantation were 47%, 27%, and 26%, respectively. Enteral autonomy continued into the fifth year after study entry. CONCLUSIONS: Children with IF endure significant mortality and morbidity. Enteral autonomy may require years to achieve. Improved medical, nutritional, and surgical management may reduce time on PN, mortality, and need for transplantation.

Derivação meso-porta (shunt Rex): tratamento atual da obstrução extra-hepática da veia porta / Meso-portal bypass (Rex shunt): current treatment of extrahepatic portal vein obstruction

Objetivo: Apresentar a caso de uma criança brasileira, provavelmente a primeira, portadora de obstrução extra-hepática da veia porta (OEHVP), submetida ao shunt Rex (derivação cirúrgica meso-porta) para tratamento da hipertensão porta pré-hepatica. Descrição: Menino de um ano e nove meses, 11 kg, previamente hígido, apresentou hematemese e melena. Foi realizada endoscopia digestiva alta, que demonstrou varizes de esôfago sangrantes grau III. Foi iniciado tratamento com propranolol e escleroterapia. Apresentou mais três episódios de sangramento importante e sinais laboratoriais de hiperesplenismo e aumento da amônia sérica. Realizado diagnóstico de cavernoma da veia porta. Foi indicado tratamento cirúrgico, após avaliação da coagulação e biopsia hepática normais. Foi feita opção pela derivação meso-porta ou shunt Rex, que consiste na colocação de um enxerto de veia jugular entre a veia mesentérica superior e o ramo esquerdo intra-hepatico da veia porta, restaurando o fluxo sanguíneo portal para o fígado. O paciente foi submetido com dois anos e seis meses, em março de 2004, à cirurgia no Children's Hospital de Chicago, sem intercorrências, tendo recebido alta no quinto dia pós-operatório. Retornou após um mês da cirurgia ao Brasil, onde seguiu fazendo controles periódicos. Atualmente, quase três anos apos o shunt Rex, o menino tem vida normal, com provas de função hepática normais, sem esplenomegalia, sem varizes e nem sinais de hipertensão porta. O shunt encontra-se pérvio e com bom fluxo pela ultra-sonografia. Conclusões: A cirurgia de derivação mesoporta e uma opção terapêutica recente e, muito provavelmente, tornar-se-á o método de escolha no manejo da hipertensão porta pré-hepática, por OEHVP. É superior aos outros procedimentos cirúrgicos, já que elimina totalmente a hipertensão porta e suas seqüelas.

Surgical restoration of portal flow corrects procoagulant and anticoagulant deficiencies associated with extrahepatic portal vein thrombosis.

Extrahepatic portal vein thrombosis (EHPVT) is associated with abnormal circulating procoagulants and anticoagulants. Eleven children with EHPVT and abnormal coagulation factors underwent a mesenterico-left portal vein bypass to restore portal flow. Coagulation factors had returned to normal by one year. The data suggest that portal venous flow is essential for maintaining normal coagulation.