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Parry-Romberg syndrome (progressive hemifacial atrophy)
Epidemiology:
1) rare disorder
2) more common in females
3) onset between ages of 5 and 15
Pathology:
1) slowly progressive hemifacial atrophy, generally left sided
2) initial pathology involves tissues above the maxilla or within the nasolabial fold
3) pathology progresses to the angle of the mouth, areas around the eye, the brow, the ear, & the neck
4) the tongue, soft palate, & gums may be affected
5) facial muscles may atrophy
6) bone loss in the facial bones may occur
7) retinal & optic nerve atrophy may occur
Clinical manifestations:
1) the eye & cheek of the affected side may become sunken
2) alopecia
3) hyperpigmentation
4) vitiligo
5) seizures
6) trigeminal neuralgia
7) progression of the atrophy often lasts from 2 to 10 years, then enters a stable phase
Management:
1) no specific treatment
2) prognosis is variable
a) no cure
b) in some cases, the atrophy ceases before entire face is affected
c) in mild cases, cosmetic problem only
General
facial disorder/injury/anomaly
developmental disorder
References
- NINDS Parry-Romberg Syndrome Information Page
https://www.ninds.nih.gov/disorders/all-disorders/parry-romberg-information-pagem