What is the treatment for Parry Romberg syndrome?

Treatment of Parry-Romberg Syndrome

There is no definitive cure for Parry-Romberg syndrome, but treatment should focus on symptom management, disease stabilization, and reconstructive surgery once the condition has stabilized.

Disease Overview

Parry-Romberg syndrome (PRS), also known as progressive hemifacial atrophy, is a rare degenerative disorder characterized by:

• Unilateral facial atrophy affecting skin, subcutaneous tissue, muscles, and sometimes osteocartilaginous structures
• Typically begins in childhood and progresses gradually over several years
• Stabilization can take up to 20 years
• Often associated with neurological, ophthalmologic, and maxillofacial manifestations

Treatment Approach

1. Disease Stabilization Phase

• Immunomodulatory Therapy

  • Corticosteroids are the first-line treatment during active disease phase 1
  • Other immunomodulators may be considered in steroid-resistant cases 1

• Neurological Symptom Management

  • Anticonvulsants for seizure control in patients with neurological involvement 1
  • Antimigraine medications for patients with headaches

2. Monitoring for Systemic Involvement

• Neurological Assessment

  • Brain MRI to evaluate for white matter abnormalities and calcifications 1, 2
  • EEG for patients with seizures or neurological symptoms

• Ophthalmologic Evaluation

  • Regular eye examinations for glaucoma and other ocular manifestations 3
  • Treatment of specific eye conditions as needed

3. Reconstructive Phase

• Timing of Intervention

  • Reconstructive surgery should only be performed after disease progression has stabilized (typically 1-2 years without progression) 4
  • Premature intervention may lead to poor outcomes due to ongoing tissue atrophy

• Surgical Options

  • Autologous fat grafting for mild to moderate soft tissue defects
  • Free tissue transfer for severe cases
  • Bone reconstruction for patients with significant skeletal involvement

Special Considerations

Overlapping Conditions

• En Coup de Sabre

  • Often coexists with PRS 3
  • May require additional treatment considerations

• Linear Scleroderma

  • When present, may benefit from more aggressive immunosuppressive therapy

Multidisciplinary Care

Due to the diverse manifestations of PRS, treatment should involve coordination between:

• Neurologists
• Plastic surgeons
• Ophthalmologists
• Rheumatologists
• Maxillofacial surgeons

Prognosis

• Disease typically progresses for 2-10 years before stabilizing
• Early intervention with immunomodulatory therapy may help limit disease progression
• Reconstructive outcomes are generally better when performed after complete disease stabilization
• Long-term follow-up is essential due to potential for neurological complications even after facial atrophy has stabilized

Common Pitfalls

• Premature surgical intervention before disease stabilization
• Failure to monitor for neurological and ophthalmologic complications
• Inadequate psychological support for patients dealing with facial asymmetry
• Overlooking the need for long-term follow-up even after apparent disease stabilization

While there are no established guidelines specifically for PRS management, the treatment approach should focus on controlling inflammation during the active phase, monitoring for systemic complications, and reconstructive procedures only after the disease has stabilized.