$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.