Osteopathia striata with cranial sclerosis
Osteopathia striata with cranial sclerosis
Definition
Osteopathia striata with cranial sclerosis (OS-CS) is a bone dysplasia characterized by longitudinal striations of the metaphyses of the long bones, sclerosis of the craniofacial bones, macrocephaly, cleft palate and hearing loss.
Also known as hyperostosis generalisata with striations, osteopathia striata with cranial sclerosis, Osteopathia striata with cranial sclerosis, X-linked dominant, Robinow-Unger syndrome — per MONDO