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Topic: Hemifacial atrophy progressive


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In the News (Fri 11 Dec 09)

  
  Dorlands Medical Dictionary
atrophy in which the affected viscus assumes a brownish hue, due to intracellular accumulation of lipofuscin; it is seen chiefly in the heart, liver, and spleen of the elderly.
atrophy of an endocrine organ presumably caused by prolonged overwork of that organ.
atrophy of an organ in the course of infectious disease.
www.mercksource.com /pp/us/cns/cns_hl_dorlands.jspzQzpgzEzzSzppdocszSzuszSzcommonzSzdorlandszSzdorlandzSzdmd_a_72zPzhtm   (2415 words)

  
 DJO | Digital Journal of Ophthalmology
Progressive hemifacial atrophy (also known as Parry-Romberg syndrome (PRS)), can be associated with ophthalmic manifestations that involve mainly the ipsilateral orbit, adnexae and intraocular structures.
This patient with progressive hemifacial atrophy developed a combination of exudative neuroretinopathy and optic nerve dysfunction resulting in visual loss Thorough ophthalmologic examination in patients with progressive hemifacial atrophy (PRS) is essential.
A rare acquired progressive hemifacial atrophy that develops in the first decade of life, progresses rapidly, and may stabilize at any age is known as Parry-Romberg syndrome (PRS).
www.djo.harvard.edu /site.php?url=/physicians/oa/385   (1054 words)

  
 La Garangola - Casa Editrice ...
The onset is slow and progressive; Romberg’s disease usually begins during the first two decades of life and is more common in the female population (female-to-male ratio of 1.5 to 1).
The atrophy is unilateral in 95% of cases.
Progressive hemifacial atrophy can manifest in different grades of severity and the main symptoms usually are: wasting of subcutaneous fat (malar region, lateral mental region, brow, paramedian forehead, sclerodermia areas, pigmented and atrophic skin, bone hypoplasia (Rx), facial muscolature atrophy (e.g.
www.garangola.it /plasom.asp?ID=12   (318 words)

  
 Hemifacial Atrophy
Although the atrophy is usually confined to one side of the face and cranium, it may occasionally spread to the neck and one side of the body
The affected area extends progressively with the atrophy of the skin, subcutaneous tissue, the muscles, bones, cartilages, alveolar bone and soft palate on that side of the face.
Hemifacial atrophy remains almost as much an enigma today as it was when first reported by Romberg in 1846.
www.ispub.com /ostia/index.php?xmlFilePath=journals/ijds/vol1n1/hemifacial.xml   (2529 words)

  
 Medical Dictionary: Progressive Hemifacial Atrophy - WrongDiagnosis.com
Progressive Hemifacial Atrophy (medical condition): Wasting away of one side of the face.
Progressive Hemifacial Atrophy: Parry-Romberg syndrome is a rare disorder characterized by atrophy (wasting away) of the tissue on one side of the face.
The atrophy may also affect the tongue, soft palate, and mucous membranes of the gums.
www.wrongdiagnosis.com /medical/progressive_hemifacial_atrophy.htm   (169 words)

  
 Parry Romberg Syndrome
Affected areas may demonstrate shrinkage and atrophy of tissues beneath the skin (subcutaneous tissue), the layer of fat under the skin (subcutaneous fat), and underlying cartilage, muscle, and bone.
Parry-Romberg syndrome is characterized by slowly progressive atrophy of the soft tissues of half of the face (hemifacial atrophy).
In addition, due to progressive atrophic changes associated with the disorder, portions of the lower jaw bone (mandible) may be unusually short on the affected side and, in some cases, involved areas of the mandible may spontaneously fracture.
hw.healthdialog.com /kbase/nord/nord489.htm   (3535 words)

  
 Parry Romberg syndrome : newer concepts in pathophysiology. Aleem MA, Meikandan D, Raveendran S, Ramasubramanian D ...
Parry Romberg Syndrome (progressive facial hemiatrophy) is characterized by slowly progressive atrophy of subcutaneous tissue.
Progressive facial hemiatrophy is characterised by slowly progressive atrophy frequently involving only one side of the face, primarily affecting the subcutaneous tissue and fat.
Sometimes progressive hemifacial atrophy is treated with autotransplantation of thoracodorsal flaps, and other facial reconstructive surgery with free fatty tissue.
www.neurologyindia.com /article.asp?issn=0028-3886;year=1999;volume=47;issue=4;spage=342;epage=3;aulast=Aleem   (616 words)

  
 A Description of Rombergs
HOLMES and A. Progressive hemifacial atrophy often referred to as Romberg's disease (or Parry Romberg Syndrome), is a pathological process involving progressive wasting of the skin, subcutaneous fat, muscle and occasionally, bones of the face.
The onset is slow and progressive and begins usually during the first two decades of life, more often between the ages of 5 and 15.
The progression of the atrophy usually lasts from 2 to 10 years, following which the process seems to enter a stable or "burn out" phrase.
www.geocities.com /rombergs/a_Description.html   (1711 words)

  
 Scleroderma (in children)
Progressive systemic sclerosis and CREST syndrome are potentially severe, life threatening illnesses in children...
This syndrome, described in the last century by Perry (1825) and Henoch and Romberg (1846), consists of slowly progressive atrophy of the soft tissues of essentially half the face, accompanied usually by contralateral Jacksonia epilepsy, trigminal neuralgia, and changes in the eyes and hair (Walsh, 1939; Wartenberg, 1945) Evidence of a mendelian basis is lacking...
long - standing debate on the relationship between progressive hemifacial atrophy and scleroderma, several authors stoutly defending the position that the coup de sabre - the strike of a sword - is a special type of progressive hemifacial atrophy.
ibis-birthdefects.org /start/sclerode.htm   (681 words)

  
 Parry-Romberg syndrome (www.whonamedit.com)
Facial hemiatrophy, facial trophoneurosis, hemiatrophia facialis, hemiatrophia faciei, hemiatrophia faciei progressiva, progressive facial hemiatrophy, progressive hemifacial atrophhy, trophoneurosis.
A rare disorder characterized by progressive atrophy of some or all tissues on one side of the face, occasionally extending to other parts of the body.
The disorder was again described by Romberg in 1846, and the descriptive title " progressive hemifacial atrophy" was coined in 1871 by the German neurologist Albert Eulenburg (1840-1917).
www.whonamedit.com /synd.cfm/1285.html   (435 words)

  
 Thieme-connect - Abstract
Although the etiology of progressive hemifacial atrophy, Romberg's disease, is still unknown, it typically manifests during emotionally salient times, such as the period before the age of 20 years.
Treatment for the atrophy is, in general, recommended after progression of the disease ceases; otherwise, many augmentations will be required after re-atrophy.
Even though disease progression could not be completely controlled in this case, free vascularized tissue transfer should be considered for mentally fragile young patients, because the free flap is the best among the procedures for Romberg's disease for maintaining volume.
www.thieme-connect.com /ejournals/abstract/jrm/doi/10.1055/s-2005-862774   (228 words)

  
 Neurology -- Correspondence for Pichiecchio et al., 59 (4) 606-608
Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry- Romberg disease): case report.
During this time, the patient associated clinical signs of progressive cortical (with partial clonic seizures in the right leg with jacksonian progression and secondary generalization) and cerebellar dysfunction (dysartrhia and ataxia).
Progressive facial hemiatrophy and epilepsy: a common underlying dysgenetic mechanism.
www.neurology.org /cgi/eletters/59/4/606   (435 words)

  
 Parry Romberg Syndrome
Parry-Romberg syndrome is a rare disorder characterized by slowly progressive degeneration (atrophy) of the soft tissues of half of the face (hemifacial atrophy).
Progressive tissue wasting can be on either side of the face.
In some cases, hair abnormalities may also appear on the affected side, including whitening (blanching) of the hair as well as abnormal bald patches on the scalp and loss of eyelashes and the middle (median) portion of the eyebrows (alopecia).
www.webmd.com /hw/raising_a_family/nord489.asp   (730 words)

  
 BioMed Central | Full text | Bilateral linear scleroderma "en coup de sabre" associated with facial atrophy and ...
The lesion was asymptomatic and not preceded by trauma.
In classic progressive facial hemiatrophy or Parry-Romberg syndrome (PRS), cutaneous inflammation, induration, and adhesion are absent or minimal, and atrophy usually involves the one entire side of the face [4-6].
Interestingly, atrophy was not preceded by clinical evidence of inflammation, discoloration of the skin, or sclerosis.
www.biomedcentral.com /1471-5945/1/9   (1792 words)

  
 Romberg Syndrome
The hemifacial atrophy typically progresses over approximately three to five years and then ceases.
Progressive tissue wasting is limited to one half of the face, usually the left side
Atrophy of half of the upper lip and tongue as well as abnormal exposure, delayed eruption, or wasting of the roots of certain teeth on the affected side
www.thedoctorsdoctor.com /diseases/romberg_syndrome.htm   (334 words)

  
 eMedicine - Morphea : Article by Michael Girardi, MD   (Site not responding. Last check: 2007-10-03)
Linear morphea is the most common morphea subtype in children and adolescents and includes the en coup de sabre and progressive hemifacial atrophy variants (see Image 2).
Progressive hemifacial atrophy (Parry-Romberg syndrome) may be a form of linear morphea.
Disabling pansclerotic morphea of children begins with the extensor extremities and progresses to involve the trunk, the flexor extremities, the face, and the scalp, with sparing of the fingertips and the toes.
www.emedicine.com /derm/topic272.htm   (5374 words)

  
 Localized Scleroderma: Linear, by the International Scleroderma Network (ISN)
Treatment of atrophies secondary to trilinear scleroderma en coup de sabre by autologous tissue cocktail injection.
Progressive hemifacial atrophy (Parry Romberg Syndrome) is usually sporadic but a few familial cases have been reported suggesting a possible hereditary influence and many investigators believe it to be a localized form of scleroderma.
A case of progressive hemifacial atrophy occurring in a 30-year-old woman is reported, in which the aetiology is thought to be Lyme disease, a borrelial infection.
www.sclero.org /medical/about-sd/types/linear.html   (3959 words)

  
 Parry-Romberg Information Page: National Institute of Neurological Disorders and Stroke (NINDS)
Parry-Romberg syndrome is a rare disorder characterized by slowly progressive deterioration (atrophy) of the skin and soft tissues of half of the face (hemifacial atrophy), usually the left side.
Initial facial changes usually involve the tissues above the upper jaw (maxilla) or between the nose and the upper corner of the lip (nasolabial fold) and subsequently progress to the angle of the mouth, areas around the eye, the brow, the ear, and the neck.
The progression of the atrophy often lasts from 2 to 10 years, and then the process seems to enter a stable phase.
www.ninds.nih.gov /disorders/parry_romberg/parry_romberg.htm   (509 words)

  
 Scleroderma "en coup de sabre": pathological evidence of intracerebral inflammation -- Stone et al. 70 (3): 382 -- ...
The Parry Romberg syndrome of progressive facial hemiatrophy and linear scleroderma en coup de sabre.
MR of brain involvement in progressive facial hemiatrophy (Romberg disease): reconsideration of a syndrome.
Progressive intracranial aneurysmal disease in a child with progressive hemifacial atrophy (Parry-Romberg disease).
jnnp.bmjjournals.com /cgi/content/full/70/3/382   (1856 words)

  
 Progressive hemifacial atrophy: a case report -- Zafarulla 69 (7): 545 -- British Journal of Ophthalmology
Progressive hemifacial atrophy: a case report -- Zafarulla 69 (7): 545 -- British Journal of Ophthalmology
A case of a 28-year-old Caucasian female with progressive hemifacial
atrophy and atrophy of the rhomboid muscles on the ipsilateral side is
bjo.bmjjournals.com /cgi/content/abstract/69/7/545   (76 words)

  
 AACPS In-Service Exam
She says that she has had progressive loss of soft-tissue volume on the right side of the face since age 10 years that became stabilized four years ago.
The correct response is Option C. Hemifacial microsomia is the most common craniofacial anomaly; affected patients have unilateral malformations of the mandibular ramus and facial paralysis.
Goldenhar syndrome, a variant of hemifacial microsomia, is characterized by vertebral or hemivertebral anomalies, lipodermoids, and epibulbar dermoids.
www.aacplasticsurgery.org /member4/newcore2003/2002/craniofacial02.html   (1323 words)

  
 Arch Neurol -- Abstract: Progressive hemifacial atrophy: report of three cases, including one observed over 43 years, ...   (Site not responding. Last check: 2007-10-03)
Arch Neurol -- Abstract: Progressive hemifacial atrophy: report of three cases, including one observed over 43 years, and computed tomographic findings, January 1982, Asher and Berg 39 (1): 44
Progressive hemifacial atrophy: report of three cases, including one observed over 43 years, and computed tomographic findings
Three cases of progressive hemifacial atrophy were studied.
0-archneur.ama-assn.org.library.lanecc.edu /cgi/content/abstract/39/1/44   (101 words)

  
 [No title]   (Site not responding. Last check: 2007-10-03)
Parry-Romberg syndrome is a rare disorder characterized by atrophy (wasting away) of the tissue on one side of the face.
There is no treatment to stop the progression of Parry-Romberg syndrome.
In some cases, the atrophy stops before the entire face is affected.
www.noranclinic.com /mi_parryromberg.htm   (168 words)

  
 Progressive hemifacial atrophy with agenesis of the head of the caudate nucleus -- Leao and da Silva 31 (12): 969 -- ...
Progressive hemifacial atrophy with agenesis of the head of the caudate nucleus -- Leao and da Silva 31 (12): 969 -- Journal of Medical Genetics
Progressive hemifacial atrophy with agenesis of the head of the caudate nucleus
left motor seizures, and mild atrophy of the left arm.
jmg.bmjjournals.com /cgi/content/abstract/31/12/969   (150 words)

  
 Hemifacial Atrophy
Hemifacial Atrophy: A Case Report And Review Of Literature.
Locicalised Hemifacial Atrophy (le),Bumb RA, Singhi MK: IJDVL
Parry-Romberg Syndrome is a rare disorder characterized by: Hemifacial atrophy Distinctive
health.cancer-help.org /web/Hemifacial_Atrophy.html   (410 words)

  
 Duane's retraction syndrome with facial hemiatrophy (a case report). Sharma DC, Parihar PS, Kumawat DC, Ramakrishnan S, ...
Facial hemiatrophy of Parry-Romberg syndrome is characterised by progressive wasting of some or all the tissues of one side of face[18].
It is a disorder of uncertain aetiology, though much attempts have been made to classify this disorder as a form of lipodystrophy[1].
Muscle atrophy in this syndrome is postulated to be secondary to disappearance of fat and connective tissues.
www.jpgmonline.com /article.asp?issn%3D0022-3859%3Byear%3D1990%3Bvolume%3D36%3Bissue%3D1%3Bspage%3D51%3Bepage%3D3%3Baulast%3DSharma   (1330 words)

  
 Microsurgery can correct facial deformities
There are three phases that generally accompany scleroderma en coup de sabre: the initial inflammatory phase; induration; and subsequent atrophy of the skin and underlying tissues, including fat and fascia (fiberlike connective tissue).
In severe forms of this disorder, especially those originating in childhood, muscle atrophy, cartilage changes, and impaired bone development in the area of the lesion may occur as well (Lakhani and David, 1984; Mayes, 1999).
I interviewed 13 women for my study, one of whom had scleroderma en coup de sabre with hemifacial atrophy and served as a participant in the pilot study; 12 of whom had contracted the systemic form of this illness and had lived with scleroderma-related facial changes for at least two years.
www.scleroderma.org /medical/facial_articles/wood_2002_1_1a.htm   (2670 words)

  
 Microsurgery can correct facial deformities   (Site not responding. Last check: 2007-10-03)
Tending to involve deep layers of skin and underlying subcutaneous fat in a segmental distribution, linear SD may cause one side of the face to look different from the other (facial asymmetry).
Romberg's disease, also known as progressive hemifacial atrophy, in its earlier stages usually involves the skin and subcutaneous tissue, and later may involve the facial muscles and skeleton.
The classic teaching with facial atrophy was to let it progress until it stabilized, then wait two years before intervening with surgery.
www.scleroderma.org /medical/facial_articles/Shulman_2000.shtm   (1213 words)

  
 Facial Neuralgia Resources: Template Conditions
The names Progressive Facial Hemiatrophy, Progressive Hemifacial Atrophy, Romberg Syndrome, PRS, HFA are synonyms for this disorder
Parry-Romberg Syndrome is a rare disorder characterized by slowly progressive wasting (atrophy) of the soft tissues of half of the face (Hemifacial atrophy.) It was described in the last century by Parry (1825) and Henoch and Romberg (1846).
The resulting wasting can sometimes result in distinctive changes of the eyes and hair; and neurological abnormalities including episodes of uncontrolled electrical disturbances in the brain (seizures) and episodes of severe pain in tissues supplied by the fifth cranial nerve (trigeminal nerve) including the mouth, cheek, nose.
facial-neuralgia.org /conditions/romberg.html   (480 words)

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