Parry-Romberg syndrome

Case contributed by Dr Mostafa Mahmoud El Feky

Presentation

Left enophthalmus, chronic facial asymmetry.

Patient Data

Age: 45
Gender: Female
CT

CT brain

Asymmetric left hemi-facial 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: enophthalmus (receded by 4 mm) associated with diminished orbital fat and tortious lax orbital segment of optic nerve. Atrophy of left lacrimal gland. The left eye lid 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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Case information

rID: 54866
Case created: 3rd Aug 2017
Last edited: 24th Aug 2017
Inclusion in quiz mode: Included

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