Table of Contents
Washington University Experience | DEVELOPMENTAL MALFORMATIONS | Hydromyelia | 2A0 Case 2 History
This patient was a 14 year old male with 16p deletion and TTN mutation associated with medically refractory epilepsy s/p L frontal lobectomy and corpus callosotomy, diplegic cerebral palsy, and moderate intellectual disability. He was admitted to the PICU following unwitnessed ‘out of hospital’ cardiac arrest with return of spontaneous circulation achieved after arrival to outside hospital ED. Through the course of the day, he demonstrated profound neurologic dysfunction including profound dilated cardiomyopathy with severely dilated left ventricle, severely decreased systolic function (LV SF%=14%, Z-score=-9.7), and significant aortic insufficiency with holodiastolic flow reversal in the descending aorta. The family elected to redirect care towards comfort measures only and further wished to pursue organ donation with donation after cardiac death.
