title: Obstructive sleep apnea successfully treated by mandibular distraction osteogenesis in a rare skeletal dysplasia.
abstract: Osteopathia striata with cranial sclerosis (OSCS) is a rare X-linked skeletal dysplasia characterized by linear striations of the long bones, osteosclerosis of the cranium, and extraskeletal anomalies. We report a female infant with OSCS diagnosed radiographically with molecular confirmation in the first year of life. The patient presented at 5 months with severe obstructive sleep apnea, which had progressed rapidly after the neonatal period and which responded favorably to mandibular distraction osteogenesis. This procedure has not previously been reported in association with OSCS, which is a rare cause of Pierre Robin sequence with dysmorphic features.
authors.affiliation: Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia. mandie.griffiths@rch.org.au
authors.forename: Amanda Louise
authors.initials: AL
authors.lastname: Griffiths
authors.forename: Andrew
authors.initials: A
authors.lastname: Heggie
authors.forename: Sarah
authors.initials: S
authors.lastname: Holman
authors.forename: Stephen P
authors.initials: SP
authors.lastname: Robertson
authors.forename: Susan M
authors.initials: SM
authors.lastname: White
mesh_terms: D005260:Female; D006801:Humans; D007223:Infant; D019857:Osteogenesis, Distraction / Q000379:methods*; D010855:Pierre Robin Syndrome / Q000150:complications / Q000601:surgery*; D020181:Sleep Apnea, Obstructive / Q000209:etiology / Q000601:surgery*