BACKGROUND: Acute neonatal liver failure is a rare condition that is often fatal. Liver transplantation (LTx) in affected neonates may be life saving, but there are only few data on the long-term outcome of neonatal LTx. PATIENTS AND METHODS: We conducted a retrospective study of 11 LTx performed in 10 pediatric patients with acute liver failure in the first month of life. Median age at LTx was 15 days (range: 7-31 days) and median weight was 3.25 kg (range: 2-4 kg). The reasons for liver failure were neonatal hemochromatosis (n=5), hemangioendothelioma (n=2), infection caused by echovirus type 11 (n=1), mitochondrial disorder (n=1), unknown (n=1), and primary nonfunction after LTx (n=1). In 10 patients, LTx organs of deceased donors were used (reduced size n=5, split n=5), and living donor LTx was performed in one neonate. The patients were evaluated with respect to survival, graft function, perioperative complications, and neurodevelopmental outcome. RESULTS: After a median follow-up time of 5 years (range: 1-14 years), 8 of 10 patients (80%) were alive. Seven of them were in good clinical condition and had normal liver function tests. One patient had to undergo retransplantation because of primary nonfunction and another is currently listed for retransplantation because of chronic graft dysfunction. Neurodevelopment was normal in 75% of the surviving patients. CONCLUSIONS: Liver transplantation provides good short- and medium-term results in neonatal acute liver failure.
BACKGROUND: Acute neonatal liver failure is a rare condition that is often fatal. Liver transplantation (LTx) in affected neonates may be life saving, but there are only few data on the long-term outcome of neonatal LTx. PATIENTS AND METHODS: We conducted a retrospective study of 11 LTx performed in 10 pediatric patients with acute liver failure in the first month of life. Median age at LTx was 15 days (range: 7-31 days) and median weight was 3.25 kg (range: 2-4 kg). The reasons for liver failure were neonatal hemochromatosis (n=5), hemangioendothelioma (n=2), infection caused by echovirus type 11 (n=1), mitochondrial disorder (n=1), unknown (n=1), and primary nonfunction after LTx (n=1). In 10 patients, LTx organs of deceased donors were used (reduced size n=5, split n=5), and living donorLTx was performed in one neonate. The patients were evaluated with respect to survival, graft function, perioperative complications, and neurodevelopmental outcome. RESULTS: After a median follow-up time of 5 years (range: 1-14 years), 8 of 10 patients (80%) were alive. Seven of them were in good clinical condition and had normal liver function tests. One patient had to undergo retransplantation because of primary nonfunction and another is currently listed for retransplantation because of chronic graft dysfunction. Neurodevelopment was normal in 75% of the surviving patients. CONCLUSIONS: Liver transplantation provides good short- and medium-term results in neonatal acute liver failure.
Authors: Shikha S Sundaram; Estella M Alonso; Michael R Narkewicz; Song Zhang; Robert H Squires Journal: J Pediatr Date: 2011-05-31 Impact factor: 4.406