PMID: 6965904May 1, 1980Paper

Osteopathia striata syndrome. Clinical, genetic and radiologic considerations

Clinical Pediatrics
H N BassB F Crandall

Abstract

Osteopathia striata, an autosomal dominant disorder, has been diagnosed in a 19-year-old mildly retarded woman. In addition, she has macrocephaly, a leonine facies, disfigurement of the lower jaw, a cleft palate and mixed hearing loss. Roentgenograms of the skull and long bones show thickening of the calvarium, particularly at the base, mandibular hyperplasia, and striations in the long bones and pelvis. Except for the cleft palate, which has not been previously reported, and the retardation, which appears to be quite uncommon in this condition, these findings are characteristic of osteopathia striata. Because the disorder may resemble several other conditions, the differential diagnosis should include osteopoikilosis, the autosomal dominant form of osteopetrosis, and hyperostosis corticalis generalisata.

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Citations

Jan 1, 1994·Pediatric Radiology·B B GayM Pasquali
Jan 1, 1986·Pediatric Radiology·G Currarino, J M Friedman
Aug 31, 2014·Atlas of the Oral and Maxillofacial Surgery Clinics of North America·Stephen A Sachs
Jul 13, 2005·European Archives of Oto-rhino-laryngology : Official Journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : Affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery·K Lüerssen, M Ptok
Aug 6, 2002·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·Leonard BerenholzMoshe Harrell
May 3, 1996·American Journal of Medical Genetics·R KönigS Fuchs
Sep 1, 1993·International Journal of Paediatric Dentistry·J R Goodman, C U Robertson

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