Spinal muscular atrophy (SMA), a lower motor neuron anterior horn cell disease, causes significant respiratory morbidity and mortality in children. Acute respiratory distress syndrome (ARDS) accounts for 1-4% of all Pediatric Intensive Care Unit (PICU) admissions. Management outcomes for ARDS in patients with SMA have not been described. We present the case of a 5-year-old boy with Type II SMA and ARDS requiring invasive mechanical ventilation. He improved with meticulous management of mechanical ventilation, airway clearance, fluid/nutrition, and sedation/analgesia. He was successfully extubated after 14 days of invasive mechanical ventilation and discharged home after a 20 day hospitalization.
Keywords: Acute Respiratory Distress Syndrome (ARDS); acute lung injury (ALI); mechanical ventilation; spinal muscular atrophy.
© 2015 Wiley Periodicals, Inc.