Pentalogía de Cantrell, diagnóstico incompleto. Reporte de caso

espanolLa pentalogia de Cantrell (PC) es un sindrome poco frecuente, descrito por Cantrell, Haller y Ravitch en 1958. En el existen defectos de la pared del abdomen, del esternon, del diafragma, del pericardio y del corazon. Pueden presentarse los cinco defectos o darse una combinacion de ellos. La PC puede acompanarse de anomalias en miembros, cara, organos, vias urinarias y genitales. Se presenta el caso de un nino nacido por cesarea, quien exhibio defecto del torax en la cara anterior, abdomen con anomalia supra umbilical y como anormalidad asociada, en la mano derecha tenia solo tres dedos y con deformidad tipo pinza. Hacia la porcion media e inferior del esternon se visualizo higado, intestino y corazon contenidos en saco de peritoneo parietal que protruia a traves del defecto citado. La radiografia de torax en posicion anteroposterior mostro el defecto esternal en tercio medio e inferior. El neonato fue admitido en la unidad de cuidados perinatales donde fallecio al tercer dia de su ingreso. El informe de anatomia patologica reporto: PC. Cerebro con edema y congestion leuco meningea. Congestion pulmonar y hepatica. EnglishThe pentalogy of Cantrell (PC), described in 1958 by Cantrell, Haller, and Ravitch, is a rare syndrome. It is conformed by defects in the abdominal wall, sternum, diaphragm, pericardium and heart. Those defects can occur together or given their combination. The PC can be accompanied by abnormalities in the face, members, bodies, urinary tract and genitals. We present the case of a child born by cesarean section, who submitted a defect in the anterior thorax; abdominal wall, above the umbilical region, and associated abnormalities in the right hand, which only had three fingers and a defect in the form of caliper. Towards the half bottom of the sternum we look the liver, the intestine and the heart; they all were contained in a bag of parietal peritoneum, that peeked through the defect cited. A chest X-ray in anteroposterior position, showed the sternal defect in bottom and middle third. The new born was admitted to the perinatal care unit, where he died the third day of admission.The anatomopathology report explained: PC. Edematous brain and leukomeningeal congestion. Pulmonary and hepatic congestion.