Perioperative Pulmonary Optimization With Average Volume-Assured Pressure Support of a Pediatric Patient With Ullrich Congenital Muscular Dystrophy: A Case Report

Patients with preexisting respiratory compromise are at risk for perioperative respiratory failure. Adult literature has shown benefit with prophylactic postoperative use of noninvasive mechanical ventilation (NIMV). While pediatric literature has documented the increasing use of postoperative NIMV, there is no literature on prophylactic preoperative NIMV in patients with preexisting respiratory compromise. Further, surgical literature does not address preoperative prophylactic use of NIMV, as well as use of the newest modality of NIMV, average volume-assured pressure support (AVAPS). Here, we describe the first report of pre- and postoperative use of AVAPS in a pediatric patient with respiratory compromise from Ullrich disease.