Parry–Romberg syndrome

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Parry–Romberg syndrome (also known as progressive hemifacial atrophy) is a rare neurocutaneous syndrome characterized by progressive shrinkage and degeneration of the tissues beneath the skin, usually on only one side of the face (hemifacial atrophy) but occasionally extending to other parts of the body. An autoimmune mechanism is suspected, and the syndrome may be a variant of localized scleroderma, but the precise etiology and pathogenesis of this acquired disorder remains unknown. It has been reported in the literature as a consequence of sympathectomy. The syndrome has a higher prevalence in females and typically appears between 5 – 15 years of age.
In addition to the connective tissue disease, the condition is often accompanied by significant neurological, ocular and oral signs and symptoms. The range and severity of associated symptoms and findings are highly variable.
Epidemiology
There may be slightly increased prevalence in females. It is thought to be of sporadic onset.
Clinical presentation
It typically presents in childhood. The overlying craniofacial skin in the affected region may be hyperpigmented.
Associations
The condition is often accompanied by:
seizures
migraines
trigeminal neuralgia
Other possible associations include:
Rasmussen encephalitis 8
Radiographic features
CT/MRI brain
may show atrophy on one side of the face +/- scalp
high (T2/PD) signal of white matter within the ipsilateral brain has also been reported on MRI 8
Management
Medical
Medical management may involve immunosuppressive drugs such as methotrexate, corticosteroids, cyclophosphamide, and azathioprine. No randomized controlled trials have yet been conducted to evaluate such treatments, so the benefits have not been clearly established.
Surgicals
Affected individuals may benefit from autologous fat transfer or fat grafts to restore a more normal contour to the face. However, greater volume defects may require microsurgical reconstructive surgery which may involve the transfer of an island parascapular fasciocutaneous flap or a free flap from the groin, rectus abdominis muscle (Transverse Rectus Abdominis Myocutaneous or “TRAM” flap) or latissimus dorsi muscle to the face. Severe deformities may require additional procedures, such as pedicled temporal fascia flaps, cartilage grafts, bone grafts, orthognathic surgery, and bone distraction. The timing of surgical intervention is controversial; some surgeons prefer to wait until the disease has run its course while others recommend early intervention.

 

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