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.
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