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Omphalocele is one of the abdominal wall defects. It can be associated with other malformations including imperforate anus, agenesis of the colon, bladder exstrophy and cardiovascular disease, frequently leading to non-viable fetuses. A case of a female newborn who was prematurely delivered (35.3 weeks) in Belize with low birth weight (1860 g) is presented. This first-born child required emergency surgery at ten hours of life for ruptured giant omphalocele associated with tetralogy of Fallot, hypoglycemia, and severe general malaise. The aim of this report is to present the diagnostic and therapeutic procedures in such cases. Given the appropriate prenatal diagnosis implemented in Cuba, it is unusual for Cuban doctors, except those working abroad, to treat these patients; hence, this paper may be useful in the teaching context.

El onfalocele es uno de los defectos de la pared abdominal. Puede asociarse a otras malformaciones como el ano imperforado, agenesia del colon, extrofia vesical y afecciones cardiovasculares, que a menudo devienen fetos no viables. Se presenta el caso de una recién nacida en Belize, de parto pretérmino (35,3 semanas), con bajo peso (1860 g) y primogénita, que requirió cirugía de urgencia a las diez horas de vida por onfalocele gigante roto, asociado a tetralogía de Fallot, hipoglucemia y gran toma del estado general. El objetivo del informe es exponer los procederes diagnósticos y terapéuticos en este tipo de casos. Dado el adecuado diagnóstico prenatal que existe en Cuba, es inusual que los médicos cubanos, salvo aquellos que colaboran en el extranjero, asistan a estos pacientes; por ello, este trabajo puede ser útil en el contexto docente.

A case of a 30 year-old black, female patient, born in Angola, Africa with past history of multiple miscarriages is presented. She stated that the last pregnancy she had had abdominal pain, as well as some other less common discomfort. A great umbilical hernia defect was detected which affected the pregnant uterus and other abdominal viscera. It was necessary to admit the patient into the hospital during the last 10 weeks of pregnancy until the cesarean surgery and the hernia reparation was performed. The mother and infant developed satisfactorily and were discharged from the hospital. They were followed up in consultations to which they never assisted. The low frequency of occurrence of this association which was corroborated by means of national as well as international bibliographical revision. This led us to prepare this report.