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Diseases

Genetic and Rare Diseases Information Center (GARD)

Progressive hemifacial atrophy


Other Names for this Disease
  • Parry-Romberg syndrome
  • Hemifacial atrophy, progressive
  • Romberg hemi-facial atrophy
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have a friend who is affected by progressive hemifacial atrophy. How is this condition treated? How can I locate doctors who are familiar with the management of this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

How might progressive hemifacial atrophy  be treated? 

At present there is no known specific treatment to stop the progression of progressive hemifacial atrophy. Since multiple systems may be affected by progressive hemifacial atrophy, a multidisciplinary team of physicians, surgeons, dentists, and psychologists may be needed to address all possible difficulties related to progressive hemifacial atrophy.[1][2][3][4]

In general, the success of a therapy is difficult to assess, as the disease stabilizes on its own and the period of time until stabilization is very unpredicatable (from 2-20 years). Since progressive hemifacial atrophy is believed to be related to linear scleroderma, physicians often try similar treatments with varying success as reported in published case studies. Suggested treatments include antimalarials, methotrexate, local or systemic steroids, tetracycline, and cyclophosphamide. Sympathectomy appears to have halted disease progression in some cases. Local therapeutic options include emollients (non-cosmetic skin softeners), vitamin D3 analogues (PUVA [Psoralen plus ultraviolet A]), and phototherapy. Hemicranial pain syndrome (can be in form of migraine or continuous severe headache, but on one side of the head) in progressive hemifacial atrophy has been treated successfully by repetitive local botulinum toxin A injections.[1]

Treatment of the eye and neurological involvement is symptomatic.[1][3]

After stabilization, surgical reconstruction using silicone implants, muscle flap grafts, galeal flaps, fat grafts, bone and cartilage grafts, or injectable dermal filler may be used to restore natural facial contours. These techniques can be successfully applied to treat eye difficulties as well. The most promising cosmetic results recently described are autologous fat grafting with adipose-derived stem cells.[1]

Last updated: 4/28/2016

How can I find physicians who are knowledgeable about progressive hemifacial atrophy?

The Romberg's Connection has a list of doctors who are knowledgeable about progressive hemifacial atrophy. We encourage you to contact this group for more information about this service. 

The Romberg’s Connection
Email: rombergs@hotmail.com
Web site: http://www.therombergsconnection.com/

The World Craniofacial Foundation maintians a list of craniofacial centers. Click here to access a listing of sites where these clinics are located. 
Last updated: 4/28/2016

References
Other Names for this Disease
  • Parry-Romberg syndrome
  • Hemifacial atrophy, progressive
  • Romberg hemi-facial atrophy
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.