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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.

Treatment

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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

References
  1. Bucher F, Fricke J, Neugebauer A, Cursiefen C, and Heindl LM. Ophthalmological manifestations of Parry-Romberg syndrome. Surv Ophthalmol. April 1 2016; 6257(15):30073-4. http://www.ncbi.nlm.nih.gov/pubmed/27045226.
  2. Lee Y-J, Chung K-Y, Kang H-C, Kim HD, and Lee JS. Parry-Romberg syndrome with ipsilateral hemipons involvement presenting as monoplegic ataxia. Korean Journal of Pediatrics. 2015; 58(9):354-357. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4623455/.
  3. Aydin H, Yologlu Z, Sargin H, and Metin MR. Parry-Romberg syndrome: Physical, clinical, and imaging features. Neurosciences. 2015; 20(4):368-371. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4727625/.
  4. Panda AK, Gopinath G, and Singh S. Parry-Romberg syndrome with hemimasticatory spasm in pregnancy; A dystonia mimic. Journal of Neurosciences in Rural Practice. 2014; 5(2):184-186. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4064192/.


Clinical Trials & Research for this Disease

  • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
  • ClinicalTrials.gov lists trials that are studying or have studied Progressive hemifacial atrophy. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
  • Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition. 
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.