Parry-Romberg syndrome (progressive hemifacial atrophy)

Epidemiology

• rare disorder
• more common in females
• onset between ages of 5 and 15

Pathology

• slowly progressive hemifacial atrophy, generally left sided
• initial pathology involves tissues above the maxilla or within the nasolabial fold
• pathology progresses to the angle of the mouth, areas around the eye, the brow, the ear, & the neck
• the tongue, soft palate, & gums may be affected
• facial muscles may atrophy
• bone loss in the facial bones may occur
• retinal & optic nerve atrophy may occur

Clinical manifestations

• the eye & cheek of the affected side may become sunken
• alopecia
• hyperpigmentation
• vitiligo
• seizures
• trigeminal neuralgia
• progression of the atrophy often lasts from 2 to 10 years, then enters a stable phase

Management

• no specific treatment
• prognosis is variable
  • no cure
  • in some cases, the atrophy ceases before entire face is affected
  • in mild cases, cosmetic problem only

More general terms

• facial disorder/injury/anomaly
• developmental disorder

Patient information

Parry-Romberg syndrome patient information