Parry Romberg Syndrome – Pictures, Symptoms, Treatment
What is Parry Romberg Syndrome?
Parry Romberg Syndrome is otherwise termed as facial hemiatrophy or progressive facial hemiatrophy or hemifacial microstomia or Romberg syndrome. It is an etiologically and clinically heterogeneous kind of syndrome. It involves the subcutaneous fat, dermis, muscle and the bone underlying it. It is rarely involves the Contralateral or ipsilateral side of the person’s face.
This ipsilateral dilation can be seen in studies of cranial imaging examination. While other experts define it as a rare kind of disorder that has a characteristic of a slow progression of the atrophy or wasting of the person’s soft tissue which is located half portion of the face or the so called hemifacial atrophy which is commonly the left area of the face.
Other experts would define it as a disorder that is uncommon and that has a characteristic of skin hyperpigmentation. It is accompanied by abnormalities by the neurological area which includes episodes of severe pain in the facial region and seizures.
It can be classified with the syndromes of the neurocutaneous system. The onset usually begins at around the age 5 to 15 years of age. The progression last often from 2 to 10 years. It is commonly reported in women than in men. It also is known to burn out in a few years but will have a bigger chance to leave asymmetrical facial features which may be difficult to cure.
History
The first person, who describes the Parry Romberg Syndrome, in the year 1815, was Caleb Parry who was an English Physician. The description was elaborated thoroughly by Moritz Romberg during the year 1846. The first one who used the descriptive syndrome was Albert Eulenburg, who was a German neurologist during the year 1871.
Parry Romberg Syndrome Symptoms, Signs
Persons who have a diagnosis of Parry Romberg syndrome have the following clinical features:
- Facial pain
- Migraine
- Focal seizures
- Hemianopia
- Cognitive impairment
- Hemiparesis
- Nausea
- Vomiting
- Enopthalmos
- Heterochromia iridis
- Refractive error
- Hemiatrophy of salivary gland and tongue
- Electrical disturbances which have a characteristic of fast muscle spasm
- Hemifacial atrophy
- Coup de sabre or the lesions or scar of the linear area of the scleroderma on the person’s scalp and forehead or strike of the sword kind of appearance
- Achromia of the hair or the lacking of pigmentation or normal color of the person’s hair
- Hyperchromia of hair or an increase in the pigmentation of the person’s hair
- Telangiectatic nevus which is a skin condition that has a characteristic of deep pink, flat localized areas of dilation of the capillaries
- Gaunt face
- Wrinkled and thin skin
- White or absent hair
- Atrophic sebaceous gland
- Facial Paresthesias
- Contralateral Jacksonian epilepsy
- Underdevelopment of the skull’s base
- Facial Bone is small
- Hemiarthrophy of the lips
- Absent malar bone
- Vitiligo or the person’s skin having dark and light patches
- Aplasia or absence or Hypoplasia or incomplete development of the person’s external ear
- Delayed eruption of the teeth on the person’s affected side
- Functioning of the nervous system is defective
Causes & Risk Factors
There are research studies that show that Parry Romberg Syndrome is caused by autoimmune kind of disorder. There are persons who have the Parry Romberg Syndrome have been found to posses antinuclear antibodies found in serum. It is known to be an autosomal dominant kind of inheritance pattern.
There are also reports of familial occurrences. There are other studies that show that in monozygotic kind of twins, it is only one of the said twins was positively known to be affected. Meanwhile, there are other theories that suggest that it is of cell mediated inflammatory process of the person’s blood vessels, trigeminal neuritis, cranial neural crest disorders, borrelia or viral infection or probably sympathetic nervous system alteration are the etiological cause of why person’s have this kind of syndrome.
Diagnosis
Persons with Parry Romberg syndrome are diagnosed by the physician by conducting the following examinations:
- Medical history examination
- Physical examination
- MRI or magnetic resonance imaging scan of the brain
- CT scan
- Lumbar puncture
- Autoantibodies serum test or examination
Treatment
The physicians usual treat Parry Romberg syndrome with the following therapies or treatment:
Pharmacological treatment
The physicians usually prescribe immunosuppressive drugs such as corticosteroids, azathioprine, methotrexate, and cyclosphosphamide. Other medications are given to treat symptoms like antiseizure and migraine pain relief.
Surgical treatment
Physicians, who have patients diagnosed with Parry Romberg syndrome, will suggest them to undergo surgeries such as microsurgical reconstructive surgical procedure which will help in restoring the normal facial contour. Meanwhile, persons who have the severe form of the syndrome which may need additional surgical procedures like cartilage grafts, othognathic surgery, bone grafts, bone distraction, and pedicled temporal fascia flaps. The best time for the surgical procedure usually depends on the preference of the physician. Some physician would prefer early surgical intervention and others would prefer to wait awhile prior to doing the surgical procedure.
Actually, there is no current cure that could stop the progressive Parry Romberg syndrome. Studies have shown that the disease will be periodically progressive and the treatment is directed towards symptomatic relief. There is also a need for follow up in a long term process for disorders which are regarding the somatic form and preventing the problems that regards the psychological aspect.
Prognosis
The prognosis for persons with Parry Romberg Syndrome varies from one person to another. In some persons, the atrophy will end before the person’s entire face gets affected. Meanwhile, persons with milder cases, there is no need to be alarmed because there is only cosmetic disability.
Complications
Complications that would fall under the Parry Romberg Syndrome include:
- Keratitis
- Iritis
- Optic nerve atrophy
- Facial palsy
- Trigeminal neuralgia
- Neurological complications such as epilepsy
- Psychiatric complications
Pictures
Source – wikimedia.org
credit – isciii.es
Image source – juniordentist.com
please help me
please help me. I live in Canada and have spent my own my money (while in excruciating pain for over a year…) to travel to Cedar Sinai hospital in LA, USA to be diagnosed with Parry Rombergs as no specialists in Calgary could figured out what was going on. CAN SOMEONE THERE HELP ME just helpe me FIGURE OUT WHERE TO GO FOR HELP IN CANADA FOR TREATMENT??? I would be really grateful. Thank you. Nicole.
Hi Nicole,
I can’t seem to find anyone to diagnose me in Toronto. You had to go to LA? Who did you see in Calgary (what kind of specialists?) No one seems to know anything about it here and there is no coverage for any reconstructive/plastic surgeries or fat grafts for it. I am also very scared and sad.
After 7 years, I was finally diagnosed yesterday with Facial Hemiatrophy by Dr. Tai, Hy’s Centre, Edmonton. He had done an EMG that came back normal but diagnosed the Perry Romberg Syndrome. I can’t tell you how much I’ve been passed around, made to feel like it was all in my head, been living a waking nightmare for the past 7 years.
Linda, What were your symptoms? What other tests did you have done? Thanks. 🙂
Worst of all is that this has slowly progressed so that now about 90% of the time my eye is closed, this affects my vision, the left side of my face, my eyebrow droops totally closing my eye, my nose pulls to the left and the corner of my mouth pulls up and my face tightens around the mouth and nose area. It now feels like my face is falling off making my cheek bones hurt. I have severe, frequent pain in my right temple, severe pain in the back of my head and neck/shoulder area. I have had vision distrubances, noise and light are amplified. Perhaps worst of all the the fogginess in my brain, I can’t think clearly and can’t wait for most days to end so I can have hope of waking up the next morning maybe feeling a little better. My eye are almost constantly dilated, I choke on food,, my tongue swells so that I can’t close my mouth, I wake up with facial edema, bad, and I can’t think now but I have a small brain aneurysm and there is calcification in the basal ganglia. Have had EMG, MRI, CT, can’t remember what else, bloodwork, etc.
LINDA
I also have lymphedema in my face. I have never found anyone else with Romberg’s that has lymphedema. I have a hard time swallowing and hearing at times too.
Does this parry-domdreg syndrome always makes ur faces become that way or are they people who had it and it didnt effect them at all.
So sorry you are going through this. Hopefully the disease process will burn itself out soon! Meanwhile and then these OTC nutritional supps may help the body do its repairs.
NIACIN the flushy kind relaxes blood vessels. Work up to 500 mg/dose several times a day. Once you get used to the tingle it can actually feel pleasant. “Reversible loss of eyebrows and lashes along with other ocular side effects, possibly due to use of niacin to treat hyperlipidemia, has been reported.[105]”
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3358936/
Vitamin B12 may help with neuropathy. Try 1000 mcg daily, more won’t hurt.
Vitamin E softens and minimizes scar tissue. 800IU – 1000IU daily.
Vitamin C keeps tissues resilient, which is good for everything. 2000mg-3000mg (2 gm-3 gm) daily divided.
A multivitamin with at least 25mg of the B vitamins.
The body repairs itself with protein, so keep it high.
Chronic pain can be so hard. Feelings of love generate endorphins, our body’s natural opiates. Love anything, tell youself, “I love you So much” in the mirror. Love the soft feel of the bed at night. Love a tree, a cat, dog, horse, the feel of grass on bare feet, a work of art, anything you can. And know although we have not met I also love you and send healing energies your way.
I wonder if cannabis can help with this? With NIDA the NIH and FDA, being so recalcitrant to let us find out, we won’t know much anytime soon.
i smoked on the daily and i think i have this condition. thats why im scared to stop i do get tension on my left temple and some pain on thy neck area well more like a tightness going to try to get it looked at
It seems that more and more people are saying the use of weed helps with the pain and inflammation of this disease. I have read studies that say someone with the disease stopped and soon felt the symptoms quickly return. Have you tried CBD Oil?
hi … I recently note that my son with this kind of issue, he is 13 only. After consult with all kind of specialist, one of it say it was the Parry Romberg Syndrome …. now so worry , could any one help give some advise.
Hi frnds I am from India I am suffering from parry syndrome I think this at start at 3 years before and now my age 20 please any one knows about this disease help me how to cure nd where I want take treatment please please please help me
Hello i have a child HeS ten years old he has been suffering of parry romberg for seven years i think that is important to treat early this afecction with revascularitazion of the face iam a general surgery and live un cuba
I’m Mishelle from Oklahoma. I’ve suffered from this for 8 years. I’ve seen every specialist this state has to offer. I’ve even travelled to Colorado and still no dice. I have horrible migraines, problems with my jaw and severe pain and pressure, in and around my eye. I’m so desperate for a solution and feel absolutely helpless. Please help in any way you can.
I also have Parre Romberg , Mine has slowly progressed over thirty years. I had very severe trigeminal neuralgia and was treated with Botox injections which worked really well for me. Hope this helps
i have had perry romberg for 23 yeas little was know then- many mds did not even know the name when i was told i had it…they gave me high doses of steroids – thats it it did help me breathe- there is a national organization of rare disorders and nih that has some good info on it. i suffer terribly and notice difficultly thinking even simple things i can not concentrate very well and get confused. does anyone know what kind of specialist i should see or meds that will help i have no family to help me
i have had perry romberg for 23 yeas little was know then- many mds did not even know the name when i was told i had it…they gave me high doses of steroids – thats it it did help me breathe- there is a national organization of rare disorders and nih that has some good info on it. i suffer terribly and notice difficultly thinking even simple things i can not concentrate very well and get confused. does anyone know what kind of specialist i should see or meds that will help i have no family to help me
It’s a multiscpecialty disease. You have to explain to your Dr’s what you have. Get a neurologist, ENT, plastic surgeon and dermatologist. There are a few drugs used to slow the progression. I’m leaning more and more about it myself. Having autologous fat transfer in a few months.
I think I have PR I am seeing an ENT and have chronic sinus … but the right side of my face is losing fat and my eye is sinking … when I smile it looks like a mild stroke symptom of the right side … who do you see and what kind of fat transfer are you getting and by whom? Thank you so much … I just found this site tonight and I think I have PR
I too have dermal problem and have parry romberg on the right half. I am a male. I don’t have facial as well as scalp hair at all on the right part of the face. Can a dermatologist help me?
My skull wasn’t affected. Only cosmetic problems. But the biggest problem was having no hair on the entire right scalp and face.
I’m visiting so doctor in Dallas, TX this Thursday to see if he thinks I have PRS. Left sunken cheek, sagging eyelid, odd intention on top left forehead/ skull, constantly unbalanced and dizzy, pressure in back of left eye… any opinions? Suggestions? Other things I should know?
I am 17 years old male. I was diagnosed with parry romberg syndrome 9 years ago. It is mild and my right side tissues were affected and not my skull. But it also affected my hair growth. My right side of head doesn’t have hair at all. Just imagine you seeing a guy half face with hair and half without hair. People calling you “Harvey two faced”. They just make fun of me whenever they see me. It is not only my hair, my chin and lips have also sunken. It was so painful. In my childhood I was normal. One day I fell down badly and hurt my chin. It was from then on that this syndrome started showing its presence in me. Is there anyone else with parry romberg syndrome and no hair(facial and head scalp hair)?
I have a missing band of hair but I can cover it up with other hair. Mine is from someone doing a cannon ball on my head in the pool when I was 10..
I have parry romber syndrome and I know how you feel
I live in Portland Oregon am 44 and was diagnosed when I was 19. I had fat grafting surgery but my body absorbed it. My new insurance won’t cover fillers even though they helped with pain. Any suggestions with how to get through to insurances would be greatly appreciated. Thanks
hi i m suffering from this disease from 7 years bone of my front forehead is invaginate from center.plzzzz tell me treatment.