The Romberg's Connection 2009 Survey Results

Affected Person Medically Diagnosed and Age

Page 11. I. General Information cont.: Diagnostic Information
1. At what age was the affected person diagnosed:
Age Responses
% of Number responding
69 1
57 1
50 - 51 2
45 - 49 6
40 - 44 7
35 - 39 4
30 - 34 8
25 - 29 11
20 - 24 11
15 - 19 20
10 - 14 23
5 - 9 22
1 - 4 8
Number responding = 124 / percent responding = 87% of the total respondents (143).

Page 11. I. General Information cont.: Diagnostic Information
Calculated: Number of Years Until Diagnosed:
Years Responses
% of Number responding
55 - 59 1
50 - 54 1
45 - 49 5
40 - 44 5
35 - 39 8
30 - 34 4
25 - 29 8
20 - 24 9
15 - 19 4
10 - 14 17
5 - 9 25
1 - 4 23
0 14
Number responding = 124 / percent responding = 87% of total respondents (143).

Page 11. I. General Information cont.: Diagnostic Information
2. Please select the type of specialist who made the diagnosis:
Specialist Responses
% of Number responding
Acupuncturist 0
Dentist 7
Dermatologist 18
Ear, Nose
and Throat
Geneticist 3
Neurologist 21
Opthamologist 5
Rheumatologist 7
Other (Specify) 20
Number responding = 128 / percent responding = 90% of the total respondents (143).
Note: This was a multiple choice question.
This chart has a link to additional information.

Page 11. I. General Information cont.: Diagnostic Information
3. Please select the procedure used to make the diagnosis:
Procedure Responses
% of Number responding
ANA (Antinuclear
Antibody test for
level of inflammation)
Biopsy for
Biopsy for
sclerotic content
CAT (computed
axial tomography
(CAT or CT scan))
MRI (magnetic
resonance imaging)
Ultrasound 1
Other (Specify) 17
Number responding = 127 / percent responding = 89% of the total respondents (143).
Note: This was a multiple choice question.
This chart has a link to additional information.

Page 11. I. General Information cont.: Diagnostic Information
4. Comments for the Affected person's diagnostic information:
I did not receive informations about the disease, I looked on the internet.
A team of New York dermatologists provided the diagnosis.
After everything else was ruled out (M.S., Lups, Scleroderma, Lyme disease...others), noticed an indentation on my forehead. That lead to the diagnosis.
There were numerous neurologic, rheumatic and ophthamology tests.
My initial symptom was en coup de sabre, visually diagnosed by a dermatologist when I was 2 or 3. The phrase Parry Romberg Syndrome was not used until much later, by the plastic surgeon who performed several surgeries and procedures when I was in high school. However, he did not *at that time* call it PRS to me or (as far as they can recall) to my parents. Years later, as a married adult expecting my first child, I had called him to ask him some questions and that was when I first heard the term PRS.
Team of dermatologists (approximately 25) confirmed diagnosis.
It took 8 years to finally find someone who knew what PRS was.
Started as a small quarter size blemish in the affected area - left side check.
I self diagnosed via Internet research more than one year before professional diagnosis.
I have had a couple of 3D Cat Scans which have monitored the progression since I was diagnosed. The blood work came back normal.
In 2001 at the Childrens Hospital in down town Houston, Texas, I was diagnosed by a "Team of Doctors" they specialized in diagnosing and treating children with cleft palates and other child hood deformities caused by some syndrome or illness. I had a CAT scan and X -ray done on my skull. I was asked a few questions by 10 different doctors who practiced different specialties. Finally They told me I had Parry Rombergs and that I was was the 4th person who came in that had Parry Romberge Syndrome.
My ENT doctor said that he had heard of Parry-Romberg syndrome, but had never seen a case of it, until he saw me.
I don't know exactly how to answer this but, when I was first diagnosed, they called it hemifacial atrophy, then about a year and a half ago, I went to a cosmatologist and he told me the correct name was: Parry Romberg Syndrome, and that it was affecting the right side of my face now. Gave me some kind of cream and told me that there was nothing that he could do for me. So, I haven't been seen by no one else.
"Trigger, looking back appears to have been a knock on the forehead above my left eye during a car accident, at age 14. After that, I began to notice the tissue around my left eye began to disappear around the eye, including the lid area showing more eyelid on the left eye. I also noticed I would rather have photos taken on the left, due to being thinner on that side, under my chin and in the neck area. I had extremely severe headaches beginning at age 15 beginning early Saturday AM after a Friday night football game. I noticed it was the first time my mother gave me 3 adult aspirin at a time. I had severe headaches until June 2002, by then I found out they were migraines. During my teens I had symptoms of stroke, and tias, but didn't know what they were. I had my vision in my right eye cut out on me or become very darkly shaded. Also, a period of bright lights that made me lose vision in both eyes. Upon physical activity, I had a spatial orientation problem on my right side, that did get better playing raqcuetball, but stumbled a great deal over my right toe, while playing, even walking. My left leg is shorter than the right one. Upon review of my Social Security Disability Doctor's review of me, and obtaining a copy of her report, she brought up the stationary Romberg Test. Since many Rombergers are affected by Central Vestibular Dysfunction, basically, interfering in their sense of balance, and spatial orientation. This causes falling at best and neurally mediated or neurocardiogenic syncope (fainting) at worst, or other types of Vertigo, positional or Peripheral. This is basically due to having become more single sensory oriented, while walking forward, with each change in vision, and sense of the height of the step or slope, the brain has to recalculate the height, position, with each step. The other senses necessary to for balance become disconnected causing the Central Vertigo. Due to a TIA with Dysphasia (inability to speak clearly), but able to think clearly in July 2002, I went for an MRI of the brain, that clearly showed the results of years of migraines, as well as old plaques in both hemispheres in the brain. The SSDI Drs.' report wasn't received until May of 2003, and upon review by my ENT, we didn't discover that I had no left cheek muscle. The report from NORD, verified other neurological, myclonic seizures, focal or sensory seizures from plaques in the right brain. The asymmetry was measured, and noted from the face down the body, and under the left rootline of the gum. Audio testing and ENG testing verified Left Peripheral Vertigo, but the Cawthorne's head exercises that should have only taken weeks to cure the problem and put me back on balance, only exacerbated the Vertigo. That is when my ENT realized my gait, floaters in my right eye, and a black spot in my right eye, found while doing the coin toss exercise, and the inability to determine where sound is coming from resulted in the diagnosis of Central Vertigo. Since then, in 2004, I've had another TIA, and an atypical stroke of sorts, with plaques in the gray matter of the brain that the neurologists have missed. Also, in addition to severe clotting factors, my Primary Care and Rheumotologist found a genetic fluctuating platelet elevated level. The Venostat that was conducted in the late 1990s, showed inflammation of the blood vessels, that causes Peripheral Neuropathy (Inflammation and Constriction being an issue for people with Rombergs), but I didn't have the level of Primary Care in Internal Medicine that I have now. So, by looking forward and backward, we've put the pieces together, for the full diagnosis of Progressive Hemi-Facial Atrophy, beginning at around the age of 14, and continuing, with further atrophy until the present, at age 54."
The ENT doctor said he had never seen a case of PR syndrome, but had heard about one case.
Very little was given except the effect of wasting of the soft tissue.
I was born with Parry Rombergs, I first went to see a surgeon at the age of 17. I went back to a different surgeon at the age of 37, who gave me a diagnosis on the 3rd visit.
"Diagnosed by biopsy with 'localized scleroderma'. Pediatric dermatologist and pediatric rheumatologist both diagnosed it as PRS. Pediatrician called it Linear Morphea."
Orginally started with hair loss at 15, and later had a reconstructive flap done, which caused some infection and poor healing. Later diagnosed with Linear Scleroderma. Wasn't until after pregnacy that the drastic symptoms came about, diagnosed by Dr. Seibert, for a reconsructive microvascular surgery attempted, which repeatedly got infected probably the first mersa.
Went to GP (General Practitioner) who didnt know what was wrong so referred to plastic surgeon who had seen one case before and diagnosed with Rombergs.
The plastic surgeon took x-rays & photos along with him to a medical convention and came back with my diagnosis as Rombergs.
She also received an MRI and CT scan to establish no neurological effects.
Diagnosed 15 years after surgery to remove affected area on revision of file.
Initially a plastic surgeon said that I had a birth defect when I was examined in my early teens. Then at 16, I was examined be a surgeon at Stanford Medical Center who put my history of seizures together with my facial deformities and diagnosed Rombergs.
Biopsy came back as "localized scleroderma" but all specialists say PRS.
"Have several of the
- atrophy of the sucutaneous muscle on half of my face, lip and tongue.
- skin overlying affected areas has become darkly pigmented (hyperpigmentation) with, areas of hyperpigmentation and patches of unpigmented skin (vitiligo).
- onset of the disease began at the age of 9."
Upon the recommendation of my dentist, I went to eye doctor for difference in pupils (and dents in my head). (I had visual problems since the age of 7). It was very fortunate for me that the eye doctor knew exactly what PRS was.
After getting the diagnosis, we went to a rheumatologist and she did the ANA test and other auto-immune tests.
The specialist (visual) recognized PRS at our first visit to the hospital. We visited the hospital because our son was suffering from headaches.
Half of her face is affected. She was treated by Dr. Thomas Reese in New York University. She still suffers with the condition. It was first diagnosed as Progressive Hemi Atrophy.
There were physical tests involving electrodes, there was some system for ruling out scleroderma, but it was so long ago that I don't remember. Don't have the records.
Plastic Surgery
Took some time for diagnosis and was made by a team of doctors at Children's Hospital.
Was first diagnosed with Lupus (later found out that was an incorrect diagnosis). Was then refered to reconstructive surgeon who made positve diagnosis of PRS.
"Facial asymmetry was first noticed by orthodonist (though did not know PRS), then primary care physician also noticed (though did not know PRS) and she referred us to craniofacial specialist at Children's Hospital in Boston where son was diagnosed. This was confirmed at Massachusetts General Hospital, also in Boston. I was unaware of other types of procedures to diagnose PRS."
My PRS showed already as a child - but I did not ask for diagnosis until I was 32.
It took a long time as only the eye doctor knew what it was. Then I was referred to a plastic surgeon.
"On the left side of my face I had:
coup de sabre
droopy eye lid
cleft in chin
hollow cheek
droopy lip."
"I had a CT Scan, 2 MRIs of the brain, EMG, and multiple blood tests to rule out other diagnoses. Finally saw both a neurologist and plastic surgeon at the University of Pennsylvania in the fall of 2007 and both diagnosed Parry-Romberg."
I went to see my GP concerned that one side of my face was slightly swollen. My GP thought it was the other way around, that one side of my face had started to atrophy. He then went away to research what it might be, which was when he found out about Rombergs. I then had an MRI to see whether the muscles and/or soft tissues of my head had been affected.
Dermatologist + Neurologist in cooperation (University Clinic)
It took two years for the condition to present itself in a form where the diagnosis could be made. Prior to that, it ranged from "nothing serious" to mast cell disease, dermatomyositis, appearance of Sturge-Webber, AVM.
I was referred by my regular doctor to a group of plastic surgery doctors.
"Atrophy to the L hand side of the face, trunk, and back. Hyperpigmentation on affected areas."
Dr. Mintz ran a lot of test, including bone density and a special x-ray that took an hour with radioactive dye that I don't kow the name of prior to surgery.
The only symptom I had was a 7th cranial nerve palsy (Bell's palsy) the investigations to find the cause of this were CT and MRI scans. PRS was diagnosed off brain findings on MRI.
"Ten years between onset and diagnosis
age 24 onset
age 34 reconstruction surgery."
1. first saw dentist. She realized something was not right. She thought she was developing a cleft lip but knew that cannot happen. She referred us to see a plastic surgeon and also asked if it was ok to send her teeth x-rays to other doctors. Plastic surgeon diagnosed her and then ordered a CT scan. He then referred us to rheumatology and he further diagnosed her with the scleroderma. And he referred us to a genetic doctor, mris, eegs, neurology, audiology, and an allergist. And also a counselor.
Depression and lost of fat / muscle at affected area.
Blood tests did not confirm the diagnosis however, we were told that this is probably an onset of PRS.
Left side of the face, cheek, chin, lip. Not the forehead.
Visited Dr. Kenneth E. Salyer in Dallas after learning about PRS and he confirmed the self-diagnosis. Didn't recommend surgery as the case at the time, was relatively "minor."
My diagnosis was made by an ear-nose-throat plastic/reconstructive surgeon. He took one look at me and told us what I have. He's worked on PRS patients before.
I was only 15 years old when they did my biospy. I do not know what type it was but they removed two sections of my skull the size of a quarter.
Dr. Grillion just said it.
Immediately after giving birth to my son at age 23, my left side of my face went flat. I saw a dermatologist who referred me to a plastic surgeon.
Diagnosed with PR at age 29 by dentist (TMJ specialist). Afflicted with PR since around age 11. Diagnosed with scleroderma (not linear) at age 35 by rheumatologist (subsequent to first pregnancy). Likely had scleroderma for many years as well, however, both PR and scleroderma flared immediately following pregnancy.
Visual diagnosis was also used - Neurologist looked at pictures of me at an earlier age. He was also willing to look at the information I brought from the NORD site. It's important to get a doctor who will listen and also do a bit of research. Dermatologist diagnosed me with another disease, that I as a lay person knew enough about to know that it was inaccurate - she was very dismissive.
"ANA originally negative; a year later, ranged between 80/speckled, 40/speckled, 160/speckled
Doctors go back and forth--say it looks like LS but acts like PRS--elements of both
Say mild so far, but have seen changes over 3 years, so don't know what "mild" means; is it just starting? How do you know if it will stop? Child also showing signs at same time"
Don't really know much about diagnosis as I was a child- can remember having EEG, ECG and being "on display" for medical students in the public hospital system- not good memories.
Initial diagnosis by plastic surgeon was that of "hemifacial atrophy." Follow up exams at Mayo, but do not recall a specific reference to "Rombergs." One endocrinologist there mentioned linear scleroderma; when I asked about that, she said it was the same thing.
I was referred to Dr. Joseph Murray at Peter Bent Brigham Hospital (now Brigham and Women's) in1973 by a Plastic Surgeon. Dr. Murray diagnosed me, and told me that there were only 50 known cases in the country.
Number responding = 63 / percent responding = 45% of total respondents (143).

Disclaimer: Please be advised that everyone's experiences may be different and appropriate treatments may vary. Any medical information that you find on The Romberg's Connection website must NOT serve as a substitute for a consultation with one's personal physician. Our visitors should discuss any specific questions or concerns they may have about Rombergs with health care professionals who are familiar with the specifics of their special case.
As a support group, The Romberg's Connection is unable to offer medical advice to anyone.

Throughout this survey, when we use the term "Rombergs" or "Romberg" we are referring to: "Parry Romberg", "Parry Rombergs", "Parry Romberg Syndrome" and "PRS".

This is an unscientific survey designed to gather information from those who must deal with Rombergs on a daily basis.