Abstract

PILONA RUIZ, Sergio et al. Thoracoabdominal síndrome. A case report. Rev Ciencias Médicas [online]. 2007, vol.11, n.1, pp. 56-64. ISSN 1561-3194.

A Caucasian male newborn, first born, delivered at 39 weeks of pregnancy in an eutocic labor was admitted at the Intensive Neonatal Care Unit, presenting an osseous defect and aponeurotic muscle covered by skin of the distal median third of the sternum extended to the navel. He was a carrier of a Thoracoabdominal Ectopia Cordis, a minor Omphalocele and a Patent Ductus Arteriosus without hemodynamic repercussion evolving favorably with the placement of a synthetic material in the thorax (Gorotex) as a protection. The patient was discharged from the hospital at 16 days old and readmitted at 2 months old to restore the Omphalocele, dying of multiple organ failure due to sepsis. The phenotype observed on the propositus was compatible with the Thoracoabdominal Syndrome, which has a dominant trait pattern linked to X -chromosome (MIM: 313850) reported by carmi et al. In 1990. This was the second sporadic case reported in Pinar del Río, Cuba, in two families which were not related.

Keywords : CONGENITAL HEART DISEASE; DUCTUS ARTERIOSUS PATENT; INFANT NEWBORN.

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