Síndrome diencéfalico como causa de desnutrición severa

El sindrome diencefalico es un complejo de sintomas y signos causados por disfuncion de esta area del encefalo caracterizado por una marcada desnutricion aun cuando la ingesta calorica es normal. Se presentan dos casos, el primero de ellos una nina de 13 meses de edad con antecedentes de un fallo de medro a partir del tercer mes de vida, que ingreso en este servicio para el estudio de una desnutricion proteico energetica severa que presento en el transcurso de su evolucion un apetito inestable y al mes de ingresada un evento paroxistico. Se le realizo resonancia magnetica nuclear y se comprobo imagen hipodensa, redondeada, que media aproximadamente 3 x 3 cm en region supraselar; fue intervenida quirurgicamente en 2 ocasiones, se realizo exeresis del tumor, y se confirmo anatomopatologicamente un astrocitoma pilocitico de bajo grado. El segundo paciente, un lactante que ingreso con el diagnostico confirmado de tumor intracraneal para estudio, semejante al caso presentado anteriormente, mostraba una marcada desnutricion proteico energetica, se le realizo tomografia axial computarizada en la que se pudo apreciar una extensa masa tumoral supraselar con dilatacion del sistema ventricular. Durante su evolucion presento marcada anorexia con perdida de peso progresiva, por lo que se realizo gastrostomia. A los 59 dias fallecio como consecuencia de una pancitopenia, y la necropsia concluyo: astrocitoma pilocitico de bajo grado. Diencephalic syndrome is a set of symptoms and signs caused by dysfunction in this area of the encephalon and characterized by marked malnutrition despite adequate intake of calories. Two cases were reported in this paper. The first one was a 13-years old girl with a history of medro failure since her 3rd month of life, who was admitted to this service for the study of her severe protein/energy malnutrition. In the course of her hospitalization, she presented with unstable appetite and suffered a paroxistic event one month after being hospitalized. The infant girl was then performed magnetic resonance imaging test which revealed a 3 cm x 3cm rounded hypodense image in the suprasellar region. She was operated on twice, the tumor was excised and the anatomic and pathological analysis yielded the presence of low grade pilocytic astrocytoma. The second patient was an small infant who was admitted to the service with a confirmed diagnosis of intracranial tumor for study, very similar to the case previously presented. He suffered an acute protein/energy malnutrition, so he was performed a computerized tomography which showed a broad tumoral mass in the suprasellar region with dilated ventricular system. During the hospitalization, he presented with marked anorexia, progressive loss of weight and he finally underwent gastrotomy, but he died 59 days after admission as a result of pancytopenia. The result of necropsy was low grade pilocytic astrocytoma.