Síndrome de Noonan y quilotórax

Resumen

El quilotórax en la edad pediátrica generalmente se desarrolla en complicaciones posquirúrgicas de cirugía cardiotorácica y raramente se debe a malformaciones del sistema linfático asociado a síndromes dismórficos. Presentamos dos casos de Síndrome de Noonan con desa-rrollo de quilotórax neonatal. Ante pacientes con fenotipo Noonan y derrame pleural que se desarrolle en el período neonatal, sin antece-dente de trauma obstétrico, está indicado descartar malformación linfá-tica congénita y estudiar el derrame pleural para realizar tratamiento inicial conservador con dieta. Radiografía de tórax, ecografía y TC to-rácico muestran el derrame pleural y patrón parenquimatoso compatibles con quilotórax y linfangiectasias.

Abstract

Chylothorax during childhood usually develops as a result of postoperative complications following cardiothoracic surgery. It is rarely due to the malformations of the lymphatic system associated with dys-morphic syndrome. We report two cases of Noonan syndrome involving neonatal development of chylothorax. In children with the Noonan phenotype who develop pleural effusion during the neonatal period in the absence of obstetric trauma, it is advisable to rule out the presence of congenital lymphatic malformation and study the pleural effusion, initially introducing conservative treatment with dietary therapy. Chest radiography, ultrasound and computed tomography reveal the presence of the pleural effusion and parenchymal pattern compatible with chylothorax and lymphangiectasis.