155 Clinical Course | Crouzon AN and its treatment. OSA was present in 14 patients, 10 of whom had a tracheotomy. Noninvasive therapy was given to 9 patients, and adenotonsillectomy was performed in 13 patients. Four patients had developed papilledema during their lifetimes. Twelve patients developed an FOHR ≥ 0.4, 6 of whom had hydrocephalus. The MRI and/or CT scans of 16 children were available, and we found that 10 children showed tonsillar herniation (≥ 5 mm). Table 1. Characteristics of patients with Crouzon syndrome and acanthosis nigricans. Characteristics No. Of Patients Total 19 Sex Male 5 (26%) Female 14 (74%) Age in years at confirmation FGFR3 mutation (median, IQR) 0.98 (6.12) Follow up age (median, IQR) 10.3 (17.8) Closed Sutures pattern at birth Unilateral coronal 1 Bicoronal 4 Bicoronal metopic 2 Bicoronal partial lambdoid 2 Bicoronal bilambdoid 2 Pansynostosis 3 Unknown 5 Expression pattern Exorbitism 15 Midface hypoplasia 16 Hypertelorism 8 Upper Airways Choanal Atresia 6 Apertura Piriformis stenosis 9 Obstructive sleep apnea 14 mild 0 moderate 1 severe 13 Treatment Obstructive sleep apnea O2 2 Optiflow 1 BiPAP/CPAP 6 Tracheotomy 10 Adenotonsillectomy 13 ICH factors Papilledema 4 Ventriculomegaly 12 Progressive ventriculomegaly 6 Tonsillar herniation 10 BIPAP = bilevel positive airway pressure; CPAP = continuous positive airway pressure; FU = follow-up 9
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