What is Parry-Romberg syndrome?
Parry-Romberg syndrome, also known as Romberg’s disease or progressive hemifacial atrophy, is a rare atrophic disorder characterized by a progressive deterioration of the skin and soft tissues of half the face. It typically affects the left side of the face, and is more common in females than in males.
How do you treat Parry-Romberg syndrome?
There is no cure and there are no treatments that can stop the progression of Parry-Romberg syndrome. Reconstructive or microvascular surgery may be needed to repair wasted tissue.
Is Parry-Romberg syndrome an autoimmune disease?
Background. Parry–Romberg syndrome (PRS) is a rare disease characterized by progressive unilateral facial atrophy. Its etiology is not well-understood, but the involvement of autoimmunity has been suggested.
Is Parry Romberg hereditary?
Mostly no. To date, there is very little evidence that Parry Romberg Syndrome is genetic. There are a few published studies that point towards it being an “autosomal dominant” genetic disorder (Anderson et al. 2005, Lewkonia & Lowry 1983).
How do you know if you have Parry-Romberg syndrome?
What are the symptoms of Parry-Romberg syndrome?
- the appearance that the mouth and nose are shifting or leaning toward one side.
- a sunken-in appearance of both the eye, as well as the cheek, on the affected side of the face.
Does Parry-Romberg affect the brain?
Between 10-20% of individuals with Parry-Romberg syndrome may have changes on brain MRI scans. These changes can include atrophy of the brain on the same side as the facial changes, and sometimes inflammation within the brain itself.
What causes facial hemiatrophy?
The cause of progressive hemifacial atrophy is unknown. Due to its similarity to localized, it has been considered as an autoimmune disorder. Other causes or triggers that have being suggested include: inflammation of the brain (encephalitis) or blood vessels (vasculitis)
Who can diagnose Parry-Romberg syndrome?
How is Parry-Romberg syndrome diagnosed? Doctors at Boston Children’s Hospital are able to diagnose this disease based on: a detailed review of your child’s complete medical history. an extensive physical examination, looking for signs of progressive loss of skin, fat, muscle, and bone.
How do I test Parry-Romberg?
MRI allows better characterization of the soft tissue and bony changes when present. Between 10-20% of individuals with Parry-Romberg syndrome may have changes on brain MRI scans. These changes can include atrophy of the brain on the same side as the facial changes, and sometimes inflammation within the brain itself.
Parry-Romberg syndrome is a rare, acquired disorder characterized by slowly progressive shrinkage (atrophy) of the skin and soft tissues of half of the face (hemifacial atrophy). In rare cases, both sides of the face are affected. In some people, atrophy may also affect the limbs, usually on the same side of the body as the facial atrophy.
What is progressive hemifacial atrophy (Parry-Romberg syndrome)?
Parry-Romberg syndrome, or progressive hemifacial atrophy, is a rare disorder of unknown etiology. Patients present with unilateral atrophy of skin that may progress to involve underlying fat, muscle, and osseocartilaginous structures. Neurologic complications are common.
Which diagnostic tests are indicated in the workup of Parry Romberg syndrome?
A diagnostic lumbar puncture and serum test for autoantibodies may also be indicated in people who present with a seizure disorder of recent onset. Oligoclonal bands and an elevated IgG index may be found in 50% of the patients. A 3D, soft tissue reconstruction of a CT scan of a 17-year-old girl with Parry Romberg syndrome.
What tissues of the mouth are affected by Parry-Romberg syndrome?
The tissues of the mouth, including the tongue, gingiva, teeth and soft palate are commonly involved in Parry–Romberg syndrome. 50% of affected individuals develop dental abnormalities such as delayed eruption, dental root exposure, or resorption of the dental roots on the affected side.