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Recém-nascido, sexo feminino, com diagnóstico de ectopia cordis e onfalocele; realizada cesariana e com 6 horas correção da parede abdominal e da ectopia cordis. Evoluiu com grave instabilidade hemodinâmica, sendo reoperada para liberação dos pontos do esterno. Apresentou melhora progressiva, extubação no 15º e alta no 40º dia. Aos oito meses foi reinternada para fechamento do esterno e reconstrução abdominal (neoonfaloplastia). Houve boa evolução, com alta no 5º dia. Com 4 anos de evolução, encontra-se assintomática, sem medicações ou restrições físicas. Female neonate diagnosed with ectopia cordis and onphalocele was submitted to an operation at 6 hours of age to reconstruct the abdominal wall and place the heart in the left pleural space. After initial hemodinamic instability the patient was reoperated to open the sternum; with progressive recovery, extubation ocurred on 15th postoperative day and she was discharged in 40th day. With 8 months of life a new operation was successfully performed to close the sternum and reconstruct the abdominal wall. After four years, the child is asymptomatic with no necessity of medications or physical restrictions

Correção de ectopia cordis e onfalocele em recém-nascido: relato de caso operado com sucesso