Resumo

GRECO A, Junior; GOMEZ F, Claudia  e  ASTIGARRAGA F, Norma. Pentalogy of Cantrell: a Case Report. Pediatr. (Asunción) [online]. 2012, vol.39, n.3, pp.193-197. ISSN 1683-9803.

Introduction: The pentalogy of Cantrell is characterized by ectopia cordis, defects of the lower sternum, anterior diaphragm, supra-umbilical portion of the anterior abdominal wall, and pericardium, as well as heart defects. Its incidence is approximately 1 in 100,000 births, and affects males disproportionately, at a ratio of 2 to 1. Generally, the reported cases of pentalogy of Cantrell have demonstrated poor prognosis. Survival is the exception, and is dependent on the degree and complexity of defects of the heart and abdominal wall as well other anomalies present. Case Report: We report the case of the male newborn of 40 weeks gestation, delivered vaginally, weighing 2.750 kg, with an Apgar score of 8/8. At 24 hours the patient was polypneic, with lower chest retraction, saturation of 95% without oxygen, and acyanotic, with asymmetrical chest expansion causing bulging in part of the chest and upper abdomen and excursion accompanying respiration. The abdomen was asymmetric due to thoracic, epigastric, and umbilical bulging, with palpable heart beat and bowel loops visible and palpable. Apex beat was located in the epigastric region, peripheral pulse was palpable, capillary refill good, and S1 and S2 sounds were normal without murmurs or gallop rhythm. Echocardiography reported an 18 mm atrial septal defect without hemodynamic repercussion. At age 5 months correction of the omphalocele was done and the diaphragm dome was checked and found to be unaffected. Diagnosis was completed with computerized tomographic angiography which observed partial thoracic-abdominal ectopia cordis, absence of the distal portion of the sternum, defects of the pericardium, dysraphism of the sacral spinal cord, rib fusion, and anomalies of the left carotid. Discussion: The pentalogy of Cantrell is a complex condition requiring interdisciplinary team intervention to reach an exact diagnosis and determine the ideal treatment.

Palavras-chave : Pentalogy of Cantrell; Cantrell's syndrome; Cantrell-Haller-Ravitchs syndrome; thoracoabdominal syndrome; newborn; congenital.

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