Please choose a workflow. A standard workflow allows you to browse the repository with full case detail; the academic workflow allows you to browse the repository with limited case detail revealed. Double click on the images to launch image viewer.
Osteopathia Striata
Case Detail
| Anatomy: Musculoskeletal |
 Joseph Junewick, MD FACR |
| Diagnostic Category: Genetic or Congenital |
|
| Created: over 3 years ago |
|
| Updated: over 2 years ago |
|
| Tags:
PEDS
|
|
| Modality/Study Types:
CT
|
|
Activities:  PDF  ImageJA |
History
9 year old female with hearing loss
Case Images
Diagnosis
Osteopathia Striata with Cranial Sclerosis -Voorhoeve Disease -Hyperostosis Generalisata with Striation.
Findings
Macrocephaly frontal bossing.
Depressed nasal bridge.
Hypertelorism.
Poorly aerated sinuses.
Linear, regular longitudinal metaphyseal bands.
Fan-like striations of iliac bones.
Occasional exostoses.
Discussion
Osteopathia striata with cranial sclerosis is an autosomal dominant disorder with variable intrafamilial expression first describe by Voorhoeve. Females are more often affected but males are more severely affected. Perhaps there is a common genetic link between osteopathia striata, melorheostosis, and osteopoikilosis. Striations develop during childhood and are not progressive. Facial nerve palsies, ophthalmoplegia, hearing loss occur as a result of cranial sclerosis. Osteopathia striata without cranial sclerosis is probably a mild form of this disease.