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$Unique_ID{BRK04088}
$Pretitle{}
$Title{Parry-Romberg Syndrome}
$Subject{Parry-Romberg Syndrome Progressive Hemifacial Atrophy Romberg Disease
Romberg Hemifacial Atrophy Romberg Trophoneurosis Facial Hemiatrophy
Trigeminal Neuralgia Jacksonian Epilepsy Horner Syndrome Scleroderma}
$Volume{}
$Log{}
Copyright (C) 1987, 1989 National Organization for Rare Disorders, Inc.
489:
Parry-Romberg Syndrome
** IMPORTANT **
It is possible the main title of the article (Parry-Romberg Disease) is
not the name you expected. Please check the SYNONYMS listing on the next
page to find alternate names, disorder subdivisions, and related disorders
covered by this article.
Synonyms
Progressive Hemifacial Atrophy
Romberg Disease
Romberg Hemifacial Atrophy
Romberg Trophoneurosis
Facial Hemiatrophy
Information on the following diseases can be found in the Related
Disorders section of this report:
Scleroderma
Trigeminal Neuralgia
Jacksonian Epilepsy
Horner Syndrome
General Discussion
** REMINDER **
The information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or
treatment purposes. If you wish to obtain more information about this
disorder, please contact your personal physician and/or the agencies listed
in the "Resources" section of this report.
Parry-Romberg Syndrome is a slowly progressive wasting (atrophy) of the
soft tissues of the face. The disorder is usually limited to half of the
face. Pain may occur in the affected area, and changes of the eyes and
facial hair can accompany soft tissue changes. Parry-Romberg Syndrome may
also be associated with other disorders. It can begin without warning,
usually during the second decade of life. Facial atrophy may cease abruptly
or progress slowly and then become stationary.
Symptoms
Parry-Romberg Syndrome is characterized by soft tissue wasting (atrophy)
limited to one side of the face which usually begins without warning. Pain
can occur in short episodes on the affected side, similar to the pain
associated with Trigeminal Neuralgia. Sensory impairment, abnormally
diminished or increased sweating, and tear duct dysfunction may also involve
the affected area. Facial features may be shifted toward the affected side, a
painless furrow may appear in the brow or cheek, the eye and cheek may become
sunken, and the eyebrows and facial hair turn white and then fall out.
Atrophy rarely affects muscle or bone, but can spread to the soft palate,
tongue, and mucous membranes of the gums. Some individuals with Parry-Romberg
Syndrome may be affected by Jacksonian (focal) Epilepsy.
Causes
The exact cause of Parry-Romberg Syndrome is not known. It was first
identified by Doctors Parry in 1825 and Romberg in 1846. Scientists believe
the disorder may be related to irritation, injury, or nerve inflammation
(neuritis) in the peripheral sympathetic nervous system. A lesion of the
trigeminal nerve of the face may also cause this disorder. Some cases are
thought to be a form of Scleroderma.
Affected Population
Parry-Romberg Syndrome is a rare disorder which affects males and females in
equal numbers.
Related Disorders
Symptoms of the following disorders can be similar to those of Parry-Romberg
Syndrome. Comparisons may be useful for a differential diagnosis:
Scleroderma is a group of chronic disorders characterized by fibrosis,
degenerative changes, vascular abnormalities and an excess of collagen in
the skin. Scleroderma is the chronic hardening and shrinking of the
connective tissues of any part of the body, although the term literally
means "hardening of the skin". Many forms of this disorder exist. Some
scientists believe Parry-Romberg Syndrome, which affects only the face and
neck, may be a form of Scleroderma. (For more information on this disorder,
choose "Scleroderma" as your search term in the Rare Disease Database).
Horner Syndrome, also known as Cervical Sympathetic Paralysis Syndrome, is
characterized by contraction of the pupils of the eyes (miosis), prominent
eyeballs (exophthalmos), partially drooping eyelids (ptosis), and absence of
sweating (anhidrosis) limited to the face and neck. The exact cause of some
cases of Horner Syndrome are not known, although scientists believe it is
either inherited or the result of injury to the neck (cervical sympathetic
ganglia). Similar symptoms may affect nerves of the face and neck in some
patients with Parry-Romberg Syndrome.
The following disorders may be associated with Parry-Romberg Syndrome as
secondary characteristics. They are not necessary for a differential
diagnosis:
Trigeminal Neuralgia, also known as Tic Douloureux, is a nerve disorder
characterized by attacks of acute pain at the side of the mouth and nose,
along distribution of the trigeminal nerve. Pain occurs in intense,
extremely short episodes (usually only a few seconds), and may be triggered
in affected individuals by brushing teeth, chewing, and/or extreme heat or
cold. Often, symptoms are limited to one side of the face. Excessive
salivation, tearing of the eyes or flushing of the skin may signal episodes.
This disorder can occur in conjunction with some cases of Parry-Romberg
Syndrome. (For more information on this disorder, choose "Trigeminal
Neuralgia" as your search term in the Rare Disease Database).
Jacksonian Epilepsy is a form of partial epilepsy, beginning with an
isolated disturbance of nerve function such as twitching of a limb, and
progressing along the limb muscles usually from the hand or foot toward the
trunk of the body. (For more information on this disorder, choose "Jacksonian
Epilepsy" as your search term in the Rare Disease Database).
Therapies: Standard
Treatment of Parry-Romberg Syndrome usually involves reconstructive and/or
microvascular surgery. An injection of fat cells obtained by liposuction
may be useful in some cases. Silicone implants can be used as well. Muscle
or bone grafts are other procedures that may be helpful in some cases.
Vision disturbances can be treated by an ophthalmologist. Other treatment
is symptomatic and supportive.
Therapies: Investigational
This disease entry is based upon medical information available through
April 1989. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate. Please check with the agencies listed in the Resources section
for the most current information about this disorder.
Resources
For more information on Parry-Romberg Disease, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
FACES
National Association for the Craniofacially Handicapped
P.O. Box 11082
Chattanooga, TN 37401
(615) 266-1632
Society for the Rehabilitation of the Facially Disfigured, Inc.
550 First Avenue
New York, NY 10016
(212) 340-5400
National Craniofacial Foundation
3100 Carlisle Street, Suite 215
Dallas, TX 75204
(800) 535-3643
NIH/National Institute of Neurological Disorders & Stroke (NINDS)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
(800) 352-9424
NIH/National Institute of Dental Research (NIDR)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4261
References
MENDELIAN INHERITANCE IN MAN, 7TH Ed.: Victor A. McKusick; Johns Hopkins
University Press, 1986. P. 294.
PROGRESSIVE HEMIFACIAL ATROPHY (PARRY-ROMBERG DISEASE): M.T. Miller, et
al.; J Pediatr Ophthalmol Strabismus (January-February 1987, issue 24(1)).
Pp. 27-36.
LIPOSUCTION FAT GRAFTS IN FACE WRINKLES AND HEMIFACIAL ATROPHY: A
Chajchir, et al.; Aesthetic Plast Surg (1986, issue 10(2)). Pp. 115-117.
THE USE OF FREE REVASCULARIZED GRAFTS IN THE AMELIORATION OF HEMIFACIAL
ATROPHY: M.J. Jurkiewicz, et al.; Plast Reconstr Surg (July 1985, issue
76(1)). Pp. 44-55.
HEMIFACIAL ATROPHY. A REVIEW OF AN UNUSUAL CRANIOFACIAL DEFORMITY WITH A
REPORT OF A CASE: D.D. Dedo; Arch Otolaryngol (September 1978, issue
104(9)). Pp. 538-541.