[Respiratory complications in children with Chiari malformation type II associated with myelomeningocele].

Management of life-threatening respiratory complications of Chiari malformation type II (CM II) is important in patients with myelomeningocele (MMC). The objective of this study is to determine the clinical features and outcome of respiratory complications in MMC.The study was a retrospective chart review of 50 patients with MMC who were treated from birth between 2002 and 2013 at the National Center for Child Health and Development, Tokyo Japan. Respiratory complications were divided into three types; upper airway obstruction, sleep-disordered breathing (SDB) and prolonged expiratory apnea with cyanosis (PEAC). SDB was further divided into two subtypes: sleep apnea type and central hypoventilation type.Twelve (24%) of the 50 MMC patients had respiratory complications. Among them, most had at least two types of complications; six had upper airway obstruction, 10 had SDB, and 10 had PEAC. The respiratory complications appeared during the first six months in most patients. Surgical decompression was performed 11 of the patients during 10 and 60 days after respiratory symptoms appeared; of which, four required invasive respiratory support in spite of decompression surgery. Three patients with central hypoventilation type SDB required ventilator support with tracheostomy, and one with upper airway obstruction needed tracheostomy. In the patients with PEAC, the frequency of apneic spells decreased over time. There was no death in the patients with respiratory complications of MMC.In addition to surgical decompression for CM II, management of respiratory complications may improve mortality outcome. Such screening should be performed in patient with MMC particularly in the first six months.