scholarly journals Case of Rapid Progression of Hemiatrophy on the Face: A New Clinical Entity?

2015 ◽  
Vol 2015 ◽  
pp. 1-2 ◽  
Author(s):  
Hisashi Nomura ◽  
Shohei Egami ◽  
Tomoaki Yokoyama ◽  
Makoto Sugiura

A lot of diseases, including lupus profundus, morphea, lipodystrophy, and Parry-Romberg syndrome, may manifest progressive hemifacial atrophy. These diseases usually progress slowly and rapid progression of atrophy is extremely rare. We report a case of elderly-onset rapid progression of hemifacial atrophy only in three weeks. Our case did not meet variable differential diagnoses. We discuss the clinical character of the patient against the past of literature and suppose it may be a new clinical entity.

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Mesut Kaya ◽  
Ceyda Sel Yilmaz ◽  
Hanifi Kurtaran ◽  
Mehmet Gunduz

Progressive hemifacial atrophy, also known as Parry-Romberg syndrome, is a slowly advancing degenerative disease that mostly affects the cutaneous, subcutaneous fatty tissue, muscle tissue, and bone structures on one side of the face. We describe the chronological progression of this very rare syndrome from early childhood until adulthood in a patient who developed severe atrophy and lost one eye. We also discuss the aetiology and pathophysiology of this syndrome.


Author(s):  
Gabriel Queiroz Vasconcelos Oliveira ◽  
Leticia Almeida Cheffer ◽  
Pietry Dy Tarso Inã Alves Malaquias ◽  
Leonardo Morais Godoy Figueiredo ◽  
Thiago Felippe Oliveira Macêdo ◽  
...  

Introduction: progressive hemifacial atrophy, also known as Parry-Romberg syndrome, is a rare degenerative disease, etiology unclear. Is characterized by a slow and progressive atrophy that affects the face unilaterally. Although there is no treatment protocol to beestablished in these cases, however, it is known that surgery should only be made after stability condition. Objective: Report a case of Parry-Romberg syndrome and discuss a general characteristics, etiology, pathophysiology, differential diagnosis and treatment of progressive hemifacial atrophy. Case description: Female patient, 8 years old, with mild enophthalmos in the left eye, nose and chin deviation to the left side, crowding and mandibular anterior crossbite, in orthodontic treatment, two months ago, for crossbite correction and monitoring for evaluation of future therapeutic needs. Conclusion: The multidisciplinary approach of these patients is essential in order to promote a better quality of life, since the disease can cause a major facial deformity.


2021 ◽  
Vol 74 (11-12) ◽  
pp. 409-412
Author(s):  
Nurhan Kaya Tutar ◽  
Sinem Yazıcı Akkas ◽  
Sami Omerhoca ◽  
Nilufer Kale ◽  
Sabahattin Saip

Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a rare, slowly progressive disorder characterized by unilateral, painless atrophy of the skin and subcutaneous tissue of the face. Neurological manifestations such as epilepsy, migraine and trigeminal neuralgia are relatively common and accompany in 15-20% of cases. Various etiologies such as infection, trauma, embryonic developmental dysfunction, sympathetic dysfunction and autoimmune disorders have been suggested as possible causes. Here we describe a 37-year-old woman whose disease manifested with dynamic contrast enhanced white matter changes over a period of two years, suggesting a “relapsing-remitting” course. Besides the inflammatory activity, positive serum-autoantibodies, inflammatory findings in cerebrospinal fluid, and an overlapping systemic autoimmune disorder may further support the hypothesis of autoimmune-inflammatory mediated pathogenesis.


2020 ◽  
Author(s):  
Francesca Saldanha ◽  
Cory M. Resnick ◽  
Carolyn R. Rogers-Vizena

This final article of the two-part craniofacial series continues to provide the embryologic and developmental foundations necessary to understand congenital craniofacial pathology. Clinical characteristics and molecular genetics needed to make an accurate diagnosis and formulate a treatment plan will be detailed for atypical craniofacial clefts, Moebius Syndrome, Fibrous Dysplasia, Progressive Hemifacial Atrophy (Parry-Romberg syndrome) and Neurofibromatosis. Details of the operations applied in treatment of these disorders are discussed in separate Scientific American: Plastic Surgery (SAPS) articles as referenced. This review contains 3 tables, and 10 figures, and 42 references. Keywords: craniofacial, Tessier cleft, atypical facial cleft, Romberg syndrome, Parry-Romberg syndrome, Moebius syndrome, fibrous dysplasia, McCune Albright syndrome, neurofibromatosis


2021 ◽  
Vol 8 (12) ◽  
pp. 1873
Author(s):  
Usha K. Raina ◽  
Shruti Bhattacharya ◽  
Prateeksha Sharma ◽  
Varun Saini

Parry-Romberg syndrome (PRS) is a rare disorder which causes progressive hemifacial atrophy, with ocular manifestations like hypotony, enophthalmos and corneal edema on the ipsilateral atrophic side. This is a report of a unique case of PRS with contalateral manifestations like ectropion uvea, correctopia and endothelial deposits, along with polymegathism and pleomorphism seen on specular microscopy suggestive of Iridocorneal Endothelial (ICE) Syndrome. ICE syndrome and PRS have not been reported together in any literature so far. This case highlights the importance of a thorough glaucoma workup and corneal examination on the atrophic facial side as well as on the apparently normal side in all cases of PRS.


2016 ◽  
Vol 25 (4) ◽  
pp. 209-211
Author(s):  
Alexis-Virgil Cochino ◽  
◽  
Sigrid Covaci ◽  
Oana-Maria Farkas ◽  
◽  
...  

Parry-Romberg syndrome, which is also known as progressive facial hemiatrophy, is a variant of localized scleroderma. This is a rare condition (OMIM # 141300), characterized by a progressive but self-limited atrophy of the skin and subcutaneous tissue on one side of the face. We present the case of a 9 year old boy with a typical presentation of hemifacial atrophy, with Borrelia burgdorferi infection without any changes of the nervous system.


2020 ◽  
Vol 10 (1) ◽  
pp. 97-101
Author(s):  
Akhlaque Hossain Khan ◽  
Nazmin Ahmed ◽  
Vijay Kumar Raut

Papillitis is a vision threatening condition, characterized by inflammation of the optic disc which often mimicked the features of papilledema; hence these patients often referred to the Neurosurgeon to exclude any intracranial pathology. This entity is associated with a number of intracranial, as well as extracranial pathologies. Among them, Parry–Romberg syndrome, also known as progressive hemifacial atrophy (PHA) possesses multiple ophthalmologic and neurologic manifestations. Here we report the case of a 14-year-old girl, who presented with the feature of progressive dimness of vision involving the right eye. Thorough physical examination demonstrated features of PHA overlapping with papillitis on fundoscopic examination. The patient treated with steroids, following which there was visual improvement. After evaluation in our facility, she was referred to department of plastic and reconstructive surgery for aesthetic improvement. Bang. J Neurosurgery 2020; 10(1): 97-101


2014 ◽  
Vol 4 (1) ◽  
pp. 67-70 ◽  
Author(s):  
P Devani ◽  
Hira Lal ◽  
A Thakral

The purpose of this report is to present radiological features of a rare entity called Parry-Romberg syndrome (also known as Progressive hemifacial atrophy). The authors report one rare case of a 18 year old female patient with Parry-Romberg syndrome, accompanied by a brief review of literature and various radiological features of this entity. Final diagnosis of a Parry-Romberg syndrome was made on clinical and radiological grounds. Radiologists should be familiar with various radiographic, CT and MRI findings observed in this disorder. DOI: http://dx.doi.org/10.3126/njr.v4i1.11571 Nepalese Journal of Radiology, Vol.4(1) 2014: 67-70


2020 ◽  
pp. 1-2
Author(s):  
Roberto Chacur ◽  
Honório Sampaio Menezes ◽  
Nívea Maria Bordin da Silva Chacur ◽  
Danuza Dias Alves ◽  
Rodrigo Cadore Mafaldo ◽  
...  

Progressive hemifacial atrophy, also called Parry-Romberg Syndrome (PRS), is a craniofacial disorder that typically involves the subcutaneous layer of one side of the face.This article We reports a case of a patient treated with polymethylmethacrylate for tissue augmentation and facial volume recovering asymmetry. Polymethylmethacrylate is effective and safe for treating progressive hemifacial atrophy and can leads a good aesthetic result.


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