PARRY ROMBERG SYNDROME

Parry Romberg Syndrome or Progressive Hemifacial Atrophy is a rare disease. It starts at an early age ranging from the age of 5 up to 15 years. The syndrome is characterized as as a slowly progressing impairment (atrophy or deterioration) of the skin and tissues of the half portion of the face (or hemifacial atrophy) which usually happens in the left area. In some rare cases, it can be that both parts of the face are affected, or the atrophy might also affect the lower and upper limbs. Mostly, individuals suffering from Parry-Romberg Syndrome see some of the symptoms at the age of 20. Women are prone to this type of syndrome.

The progression period of the atrophy lasts from 2 up to 10 years, and then after that, it will proceed to a stable stage or phase by which the atrophy slows down. If the disease occurs around the eye, complications in the retina or the optic nerves might happen.

Parry-Romberg Syndrome’s atrophy varies from mild to crucial changes. In some cases, if the syndrome started at childhood, the progression instantly increases. In some mild cases, it doesn’t cause a person to be disabled except for the cosmetic effect. Different prognosis happens in different individuals. Atrophy stops before it affects the whole part of the face in some cases.

SYMPTOMS

The skin, soft tissues, muscles, cartilages, and bones become thin and shrinking.

Physical changes include:

  • The mouth and nose can be seen shifting to one side.
  • Sunken eyes and cheeks
  • Change in skin color: hyperpigmentation (darkly pigmented) and vitiligo (several patches of unpigmented skin)
  • Whitening and loosing of facial hair
  • Damage in muscle or facial bone

Systemic manifestations include:

  • Neurological oddity (like seizures and migraine)
  • Trigeminal neuralgia (or series of severe facial pain)
  • Ophthalmologic and orthodontic modifications

Causes

  • Bacterial Infections
  • Viral Infections
  • Autoimmune conditions, wherein the immune system mistakenly destroys some of the nerves or tissues.
  • Swelling of the brain
  • Physical related trauma
  • Abnormalities in the sympathetic nervous system

DIAGNOSIS

The diagnosis is based on its symptoms, a patient’s history, and a series of clinical and specialized examinations. An MRI (magnetic resonance imaging) is used to produce images of tissues and organs to see if atrophy or inflammation is happening in the brain, or if there are changes in the facial structures. Then, surgical removal and a biopsy (or a microscopic examination) in the affected area in the skin may be done.

TREATMENT

There is still no treatment for Parry-Romberg Syndrome, but reconstructive or microvascular surgery is used in some cases. Also, muscle grafts or bone implants, and silicone or fat injections are considered as alternative treatments for the syndrome.

In some cases, specific treatments are directed towards the symptoms of the syndrome. Procedures are done by the coordination of different health-related professionals, from pediatricians, surgeons, dentists, dermatologists, neurologists, and other health-related specialists.

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