Progressive facial hemiatrophy


Progressive facial hemiatrophy

Description, Causes and Risk Factors:

Progressive facial hemiatrophy 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. 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.

The etiology of this rare syndrome still remains unclear. Etiologic factors accused for the disease are familial tendency which was suggested to be autosomally dominant but not much supported in literature, viral infections, peripheral trigeminal neuritis, lenfositic neurovasculitis, localized scleroderma and endocrine autoimmune disorders. In literature it is emphasized that progressive facial hemiatrophy accompanied by segmental vitiligo may be due to autoimmunity.

The pathologic etiology of progressive facial hemiatrophy is poorly understood; trauma, infection with virus, immunologic abnormality, cranial vascular malformation, connective tissue disorder, and disturbance of fat metabolism have been proposed.In addition, progressive facial hemiatrophy may be congenital in nature.Because the cells participating in the formation of the frontonasal bud and the cells that give rise to the cerebral hemisphere have common progenitors when one side of the rostral neural tube is affected, it can cause ipsilateral cerebral and facial lesions later in life.

Symptoms:

    Progressive facial hemiatrophy is a craniofacial disorder characterized by progressive shrinking and deformation of one side of the face with atrophy of the subcutaneous connective and fatty tissues.The atrophy often progresses slowly for many years and then stabilizes. The main morphologic features are unilateral facial atrophy and ipsilateral enophthalmos and deviation of the mouth and nose toward the affected side.

  • Neurologic abnormalities occur in 15% of patients and include focal seizures, visual loss due to involvement of the retina and optic nerve, facial pain due to trigeminal neuralgia, and migraine.

  • Neuroradiologic features include ipsilateral cerebral hemiatrophy and white matter hyperintensity on magnetic resonance imaging and intracortical calcifications.

  • Trophic malformation of the cervical sympathetic trunk leading to sympathetic dysfunction has been implicated in the pathogenesis of progressive facial hemiatrophy.

Diagnosis:

Diagnosis can be made solely on the basis of history and physical examination in people who present with only facial asymmetry. For those who report neurological symptoms such as migraine or seizures, MRI scan of the brain is the imaging modality of choice. A diagnostic lumbar puncture and serum test for autoantibodies may also be indicated in people who present with a seizure disorder of recent onset.

Treatment:

In the treatment of progressive facial hemiatrophy; immunosuppressive medicines such as cyclophosphamide, prednisolone, methotrexate are used, as LSCS treatment. In cases, where an advanced atrophy exists, cosmetic plastic surgery is applied. For this reason, mostly grafts together with dermis and fatty tissue are preferred.

Affected individuals may benefit from microsurgical reconstructive surgery to restore a more normal contour to the face. Such surgery often involves 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.

NOTE: The above information is for processing purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.

DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.

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