RESUMO
A case of a two month infant with complex congenital heart disease (aortic coarctation with ventricular septal defect) associated to a cellular brain migration failure is presented. The management strategy consisted on the correction of congenital heart disease by means of a two-stage surgery without a further preoperative evaluation of the neurological status. The patient developed several perioperative complications such as two episodes of cardiac arrest, reconnection to cardiopulmonary bypass, cardiac tamponade, chilothorax and septic shock. A neurological protocol consisting in electroencephalography, brain magnetic resonance and Single Photon Emission Computed Tomography (SPECT) was practiced during the postoperative period, which detected microgyria with paquigyria and a cellular brain migration failure was suspected. The final outcome was death due to multisystemic failure and the autopsy confirmed the neurological disease, as well as poor life function prognosis. Should the heart-brain binomial had been considered in an integral preoperative evaluation, the therapeutical approach could have been modified.
Assuntos
Anormalidades Múltiplas/diagnóstico , Encéfalo/anormalidades , Cardiopatias Congênitas/terapia , Encéfalo/patologia , Evolução Fatal , Humanos , Lactente , MasculinoRESUMO
Se presenta el caso de un lactante de dos meses con una cardiopatía congénita compleja del tipo coartación aórtica con comunicación interventricular asociada a un trastorno de migración celular cerebral. El manejo consistió en la corrección de la cardiopatía congénita en dos tiempos quirúrgicos sin haber profundizado en la evaluación neurológica preoperatoria. El paciente desarrolló múltiples complicaciones perioperatorias que incluyeron paro cardiaco en dos ocasiones, reconexiones consecutivas a circulación extracorpórea, tamponamiento cardiaco, quilotórax y choque séptico. Concomitante a las complicaciones postoperatorias, se realizó una evaluación neurológica secundaria bajo un protocolo de abordaje neurológico que consistió en electroencefalografía, resonancia nuclear magnética y tomografía por emisión de positrón (SPECT). De esta forma se detectó paquigiria con microgiria y se sospechó trastorno de migración celular cerebral. La evolución final fue hacia el deceso por falla multisistémica y la autopsia confirmó la patología neural, así como el pobre pronóstico para la función y la vida. De haberse considerado en el preoperatorio un estudio integral que incluyera el binomio corazón-cerebro, el planteamiento terapéutico podría haberse modificado.
A case of a two month infant with complex congenital heart disease (aortic coarctation with ventricular septal defect) associated to a cellular brain migration failure is presented. The management strategy consisted on the correction of congenital heart disease by means of a two-stage surgery without a further preoperative evaluation of the neurological status. The patient developed several perioperative complications such as two episodes of cardiac arrest, reconnection to cardiopulmonary bypass, cardiac tamponade, chilothorax and septic shock. A neurological protocol consisting in electroencephalography, brain magnetic resonance and Single Photon Emission Computed Tomography (SPECT) was practiced during the postoperative period, which detected microgyria with paquigyria and a cellular brain migration failure was suspected. The final outcome was death due to multisystemic failure and the autopsy confirmed the neurological disease, as well as poor life function prognosis. Should the heart-brain binomial had been considered in an integral preoperative evaluation, the therapeutical approach could have been modified.
