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Parry-Romberg syndrome

Case contributed by Mostafa Elfeky
Diagnosis almost certain

Presentation

Left enophthalmus, chronic facial asymmetry.

Patient Data

Age: 45 years
Gender: Female

CT brain

ct

Asymmetric left hemifacial atrophy affecting mainly the soft tissues of the peri-orbital, masticator and temporal soft tissues. Left orbital ridge shows mild thinning compared to the right one.

The left globe shows enophthalmos (receded by 4 mm) associated with diminished orbital fat and tortuous lax orbital segment of optic nerve. Atrophy of left lacrimal gland. The left eyelid is atrophic and edematous with calcifications.

Mild atrophic changes of the left cerebellar hemisphere.

The left parotid gland is absent (agenesis).

Left hemi-tongue atrophic changes.

Case Discussion

Parry-Romberg syndrome (progressive facial hemiatrophy) is a rare progressive craniofacial disorder with facial hemiatrophic changes involving skin, soft tissues, cartilaginous and bony structures with slight female predilection. The tongue, gums and teeth can be also affected.

Differential diagnoses include hemifacial microsomia (congenital and non-progressive condition), post-traumatic atrophy and partial lipodystrophy (bilateral and involves primarily the adipose tissue).

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