Management of Rare Causes of Pediatric Chronic Respiratory Failure

The need for long-term noninvasive positive pressure ventilation (NiPPV) in children with chronic respiratory failure is rapidly growing. This article reviews pediatric-specific considerations of NiPPV therapy. Indications for NiPPV therapy can be categorized by the cause of the respiratory failure: (1) upper airway obstruction, (2) musculoskeletal and/or neuromuscular disease, (3) lower respiratory tract diseases, and (4) control of breathing abnormalities. The role of NiPPV therapy in select rare conditions (spinal muscular atrophy, congenital central hypoventilation syndrome, cerebral palsy, scoliosis, and Chiari malformations) is also reviewed.