Respiratory and craniofacial management in children with Apert syndrome.

Few studies have assessed the respiratory and craniofacial management of patients with Apert syndrome. The aim of the study was to report the clinical management at our national reference center over a 25-year-period. The multidisciplinary management of patients with Apert syndrome who had a respiratory polygraphy (PG) at our center was described. Twenty-eight patients with Apert syndrome had a first PG in our center at a mean age of 3.9 ± 5.4 (0.1-22.9) years old. Ten (36 %) patients had mild-to-severe obstructive sleep apnea (OSA), with 3 (11 %) having severe OSA. During the study period, 8 patients had severe OSA, 15 had upper airway surgery, 8 required CPAP, 2 a tracheostomy, and 27 (96 %) had at least one craniofacial procedure: 17 (61 %) patients had a posterior cranial vault decompression, 16 (57 %) a fronto-orbital advancement, 9 (32 %) a fronto-facial monobloc advancement, and 7 (25 %) a Le Fort 3 osteotomy. Our management protocol changed over time with systematic PGs, earlier cranial expansion and upper airway surgery, and later midface advancement. Multiple surgeries, combining upper airway, and craniofacial procedures, were necessary to treat upper airway obstruction. CPAP was effective to treat persistent OSA and wait for optimal timing of craniofacial surgery.