What is a Parry Romberg Syndrome?
Parry Romberg Syndrome is also known as Progressive hemifacial atrophy. It is a condition recognized by slow and progressive atrophy. It generally occurs at the unilateral facial tissues including muscles, bones, and skin. Parry Romberg Syndrome brings much more functional and psychological trouble. A symmetric face gradually loses its natural identity. There is regional atrophy of the skin, subcutaneous tissue and musculature. In this case, the underlying bone and cartilage may also be involved. There is a sharply demarcated line between normal and abnormal skin developed. The scenario of the involved area is different from one another. The area varies from a discrete lesion to a widespread, excessive malformation. For this malformation, the mouth and nose are affected. The person suffering from Parry Romberg Syndrome may also suffer from the unilateral exposition of teeth when lips are involved.
There are some synonyms of Parry Romberg Syndrome. These synonyms are as follows:
- Hemifacial atrophy (HFA)
- Progressive hemifacial atrophy
- Progressive facial hemi atrophy
- Romberg Syndrome.
Parry Romberg Syndrome is a rare syndrome. For some people, atrophy may also affect the limbs generally on the same side of the body as the facial atrophy. The symptoms and the severity of Parry Romberg Syndrome are variable from one person to another. Some people also feel some neurological abnormalities. These abnormalities affect the eyes and teeth. The majority of people with Parry Romberg Syndrome experience this particular syndrome before the age of 20. There is no definite cause for this specific syndrome. It appears to occur randomly for so many unknown reasons.
Symptoms of Parry Romberg Syndrome
The overall symptoms of Parry Romberg Syndrome vary from person to person. It can be from mild to severe. However, the most common syndrome is the thinning and shrinkage of the skin, tissues, muscles, cartilage, and bones. Being a progressive condition, the overall symptoms worsen by the time a person enters into a stable phase. The degeneration of the muscle and tissue includes areas including nose, mouth, tongue, eyes, ear, eyebrow and neck. There are some physical changes that a person affected with Parry Romberg Syndrome may notice:
- It may have occurred that the mouth and the nose are shifting or leaning towards one side.
- The affected person may see a sunken-in appearance of both eyes, as well as the neck and on the other affected side of the face.
- The Parry Romberg Syndrome patient may suffer from changing in skin color which is also called pigmentation. It can also be lightening which is called hypopigmentation and darkening which is called hyperpigmentation.
- The affected patient’s facial hair may turn white and fall out which is called alopecia.
- The patient suffering from Parry Romberg Syndrome may suffer from facial bone and muscle loss.
Some others signs that may also be noticed in some Parry Romberg Syndrome patients are:
- Presence of some neurological symptoms like seizures, migraine or some excessive facial pain which is known as trigeminal neuralgia.
- The ophthalmologic manifestations can also occur in many cases.
- The affected patients may also notice some orthodontic changes.
Causes of Parry Romberg Syndrome
Parry Romberg Syndrome commonly happens in girls. The exact reason for this particular disease is still unknown. However, there are some factors which considered as the cause of Parry Romberg Syndrome. These include:
- The Parry Romberg Syndrome patients suffer from viral and bacterial infections.
- The affected patients may also suffer from autoimmune diseases.
- The patient may suffer from nervous system abnormalities.
- There can be inflammation of brain or meninges, which is the lining of the skull.
- The affected patient may suffer from physical trauma which might give rise to Parry Romberg Syndrome.
Onset of Parry Romberg Syndrome
Parry Romberg Syndrome generally begins between the ages of 5 and 15 years. The gradual progression of the atrophy often lasts from 2 to 10 years. After that particular time, the process seems to enter a stable phase. The muscle in the facial portion may experience atrophy, and there may be facial bone loss. When the disease surrounds the eye portion, then the affected patient may suffer from the retina and optic nerve damage.
Prognosis of Parry Romberg Syndrome
The prognosis of Parry Romberg Syndrome for individuals may vary. In many cases, the atrophy ends before the entire face is affected by the syndrome. In many mild cases, the disorder generally causes no disability more than cosmetic effects.
Diagnosis of Parry Romberg Syndrome
The diagnosis of Parry Romberg Syndrome is based upon the identification of individual characteristic symptoms. It includes many steps such as detailed patient history, thorough clinical evaluation and a wide range of specialized tests. The specific test is used to identify which symptoms are present and which one occurs first. For many cases, an MRI may be used in individuals showing neurological symptoms. An MRI is a magnetic radio wave which is used to produce cross-sectional images of the particular organs and bodily tissues. The affected patient may also go through a surgical process and microscopic examination which is called biopsy. This may be used in individuals with linear scleroderma en coup de saber.
Many people have sustained injuries or burns to their faces may also develop an appearance similar to the Parry Romberg Syndrome. The affected patient needs a proper diagnosis and treatment which is essential for management of Parry Romberg Syndrome.
Treatment of Parry Romberg Syndrome
There is no cure for Parry Romberg Syndrome. No medications can stop the progression of Parry Romberg Syndrome. The affected patient may need some reconstructive and microvascular surgery to repair the wasted tissue.
Therapies for Parry Romberg Syndrome
The whole treatment or therapy may occur in the presence of pediatricians, surgeons (plastic surgeons), dentists, ophthalmologists, neurologists, and other healthcare professionals. They all work as a strong team to treat the Parry Romberg Syndrome patient. Many surgical techniques have been used to improve the cosmetic appearance of the affected individuals. Surgery may leave scar tissues. The anticonvulsant drug therapy may help to prevent, reduce and control the seizures. Some medications and the surgical process may be beneficial in some cases.