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Related Experiment Videos

Osteopathia striata with cranial sclerosis

B B Gay1, L J Elsas, J B Wyly

  • 1Department of Radiology, Emory University School of Medicine, Atlanta, Georgia.

Pediatric Radiology
|January 1, 1994
PubMed
Summary

Osteopathia striata with cranial sclerosis (OS-CS) is a rare bone dysplasia affecting females more often. This review details clinical and radiologic findings in a new patient, highlighting skeletal abnormalities and complications.

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Area of Science:

  • Genetics
  • Radiology
  • Pediatrics

Background:

  • Osteopathia striata with cranial sclerosis (OS-CS) is a rare genetic bone dysplasia.
  • It presents with distinctive facial and neurological abnormalities.

Observation:

  • This case review follows a patient from birth to 20 years, believed to be the 35th reported case.
  • OS-CS is more common in females and can be autosomal dominant or a sporadic mutation.
  • Patients present with hypertelorism, flat nasal bridge, frontal bossing, large head, hypoplastic maxilla, palate anomalies, chronic otitis media, hearing deficits, nasal obstruction, and neurological deficits.

Findings:

  • Distinctive skeletal abnormalities include skull base and calvarium sclerosis, linear striated densities in long bones and pelvis.
  • Poor development of mastoid and sinus air cells is noted.

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  • Bone scans show increased bone turnover, supported by elevated pyridinoline excretion.

    • Implications:

    • Major complications arise from the constriction of skull base foramina.
    • While not life-threatening, OS-CS can lead to significant disability.
    • Early diagnosis and management are crucial for mitigating complications.