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Hamartoma of the Tuber Cinereum
Joseph Junewick, MD FACR
over 7 years ago
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ARSt Case Repository

Osteopathia Striata

Case Detail

Anatomy: Musculoskeletal
Junewick
Joseph Junewick, MD FACR
Diagnostic Category: Genetic or Congenital
Created: over 8 years ago
Updated: over 8 years ago
Tags: PEDS
Modality/Study Types: CT
Activities:
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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.



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