scholarly journals PARRY ROMBERG SYNDROME

2016 ◽  
Vol 25 (4) ◽  
pp. 209-211
Author(s):  
Alexis-Virgil Cochino ◽  
◽  
Sigrid Covaci ◽  
Oana-Maria Farkas ◽  
◽  
...  
Keyword(s):  
Nervous System ◽  
Borrelia Burgdorferi ◽  
Subcutaneous Tissue ◽  
Rare Condition ◽  
Localized Scleroderma ◽  
Hemifacial Atrophy ◽  
The Face ◽  
Parry Romberg Syndrome ◽  
Facial Hemiatrophy

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.

scholarly journals Progressive hemifacial atrophy Parry Romberg syndrome characteristics of craniofacial morphology

2007 ◽  
Vol 54 (1) ◽  
pp. 52-61
Author(s):  
Angelina Nikodijevic-Latinovic
Keyword(s):  
Subcutaneous Tissue ◽  
Treatment Plan ◽  
Rehabilitation Program ◽  
Craniofacial Morphology ◽  
Dental Arch ◽  
Mandibular Ramus ◽  
Hemifacial Atrophy ◽  
Parry Romberg Syndrome ◽  
The Right ◽  
Facial Hemiatrophy

The aim of this case report was to analyze clinical finding in a patient with rare, Parry Romberg syndrome. The patient was diagnosed with progressive facial hemiatrophy that affected skin, subcutaneous tissue and underlying bony structures. Analysis of study models and cephalometric radiographs confirmed there was insufficient development of the facial right side: asymmetric narrow upper jaw, shorter mandibular ramus and corpus, upper frontal teeth asymmetry, crowding of the upper dental arch on the right side. An orthodontic treatment plan was proposed within the entire rehabilitation program that included collaboration with a maxillofacial and plastic surgeon.

scholarly journals Parry-Romberg Syndrome: A Case Study of Two Patients

2017 ◽  
Vol 10 (1) ◽  
pp. 14-16
Author(s):  
Shikhar Saxena
Keyword(s):  
Female Patient ◽  
Subcutaneous Tissue ◽  
Facial Asymmetry ◽  
Deviated Nasal Septum ◽  
Hemifacial Atrophy ◽  
Chief Complaints ◽  
The Face ◽  
Side Face ◽  
Parry Romberg Syndrome

ABSTRACT Introduction Parry-Romberg syndrome is a rare neurocutaneous disorder, i.e., characterized by a slow and progressive atrophy of facial tissues, including muscles, bones, and skin of one or occasionally both sides of the face. Atrophy may be preceded by cutaneous discoloration of the affected skin, such as hyperpigmentation or depigmentation. Observation A 30-year-old female patient came with a chief complaint of progressive deformity of left-side face since 7 to 8 years. Progressive atrophy on left half face was noticed first at the age of 23 years as an area of hyperpigmentation on left malar region, which progressed with atrophy of the underlying fat and subcutaneous tissue resulting in disfigurement. Head and neck examination confirmed the asymmetry with flattening and hyperpigmentation on left side of face. There was an evident loss of underlying fat, muscle, and subcutaneous tissue giving a sunken appearance. Computed tomography scan revealed generalized atrophy of the soft tissue of the left hemi face resulting in asymmetry on the left side with deviated nasal septum. Another female patient of 35 years old came to the Department of Otorhinolaryngology with marked facial asymmetry on right side. Conclusion Two cases of PRS from different families are discussed. A 30-year-old female who presented with chief complaints of left side hemifacial atrophy and blackish pigmentation of overlying skin whose clinical features were consistent with PRS and a 35-year-old female with right-sided facial hemifacial atrophy. How to cite this article Chandra M, Saxena S. Parry-Romberg Syndrome: A Case Study of Two Patients. Clin Rhinol An Int J 2017;10(1):14-16.

Parry-Romberg syndrome: is it a “relapsing-remitting” disease?

2021 ◽  
Vol 74 (11-12) ◽  
pp. 409-412
Author(s):  
Nurhan Kaya Tutar ◽  
Sinem Yazıcı Akkas ◽  
Sami Omerhoca ◽  
Nilufer Kale ◽  
Sabahattin Saip
Keyword(s):  
Subcutaneous Tissue ◽  
Autoimmune Disorder ◽  
Hemifacial Atrophy ◽  
Sympathetic Dysfunction ◽  
Contrast Enhanced ◽  
Relapsing Remitting ◽  
Progressive Hemifacial Atrophy ◽  
The Face ◽  
Parry Romberg Syndrome ◽  
Progressive Disorder

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.

Unilateral Neuroimaging Findings in Pediatric Craniofacial Scleroderma: Parry-Romberg Syndrome and En Coup de Sabre

2020 ◽  
Vol 35 (11) ◽  
pp. 753-762
Author(s):  
Ronald R. Seese ◽  
Daniel Glaser ◽  
Andre Furtado ◽  
Kavita Thakkar ◽  
Kathryn S. Torok
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
White Matter ◽  
Neurological Symptoms ◽  
Localized Scleroderma ◽  
Imaging Findings ◽  
History Of ◽  
Parry Romberg Syndrome ◽  
Clinical Associations

Objective: Parry-Romberg syndrome (PRS) and en coup de sabre (ECDS) are subtypes of craniofacial localized scleroderma. Systematic analyses of central nervous system imaging findings and their clinical associations in children are lacking. Here, we aim to characterize neuroimaging findings and associated neurological symptoms in these conditions. Methods: Neuroimaging and neurological symptoms of children evaluated at our institution with a diagnosis of PRS or ECDS were retrospectively reviewed. Laterality, location, stability, and number of lesion(s) were evaluated, as was the presence of susceptibility lesion(s) and contrast enhancement. History of seizures or headaches was noted. Results: From 2003 to 2019, 80 patients with PRS or ECDS were followed at our institution. Neuroimaging was completed in 73 and found to be abnormal in 25. In 12 (48%) of these 25 cases, headaches and/or seizures were present. In the vast majority of these cases (22/25, 88%), lesions were ipsilateral to skin findings. White matter was involved in 19 (76%) patients. MRI abnormalities preceded a rheumatological diagnosis in 7 (28%). Susceptibility lesions were noted in 11 (44%), and 8 (73%) of these patients endorsed a history of headaches. Most lesions were in the supratentorial compartment, did not enhance, and were stable at 1-year follow up imaging. Of those with progression, susceptibility findings were present at baseline. Conclusions: Neuroimaging findings in pediatric PRS and ECDS are often supratentorial, stable, unilateral, and ipsilateral to skin findings, and they can precede cutaneous findings.

scholarly journals Masson’s tumor of the pineal region: case report

2018 ◽  
Vol 128 (6) ◽  
pp. 1725-1730 ◽  
Author(s):  
Lefko T. Charalambous ◽  
Anirudh Penumaka ◽  
Jordan M. Komisarow ◽  
Amanda C. Hemmerich ◽  
Thomas J. Cummings ◽  
...  
Keyword(s):  
Nervous System ◽  
Case Report ◽  
Vascular Malformations ◽  
Subcutaneous Tissue ◽  
Pineal Region ◽  
Vascular Tumors ◽  
Intravascular Papillary Endothelial Hyperplasia ◽  
Masson’S Tumor ◽  
The Face ◽  
Papillary Endothelial Hyperplasia

Intracranial intravascular papillary endothelial hyperplasia (IPEH), also referred to as Masson’s tumor, is a condition that rarely occurs in the nervous system. IPEH most frequently occurs extracranially in the skin of the face, skull, neck, and trunk and can easily be mistaken clinically, radiologically, and histologically for angiosarcoma, organizing hematoma, or other vascular malformations. IPEH accounts for roughly 2% of all vascular tumors and is extremely rare intracranially, with only 23 reported cases compared with more than 300 cases of IPEH occurring in the skin and subcutaneous tissue. To date, it has never been reported to occur in the pineal region. The authors report the case of a patient with an IPEH in the pineal region who underwent complex resection and experienced reversal of neurological symptoms.

Early Onset and Severe Progression of Neuro-ophthalmological Manifestations in a Case with Parry–Romberg Syndrome

2019 ◽  
Vol 18 (05) ◽  
pp. 246-250
Author(s):  
Imen Zone Abid ◽  
Emna Jarrar ◽  
Jamel Feki ◽  
Fatma Kamoun ◽  
Chahnez Triki ◽  
...  
Keyword(s):  
Early Onset ◽  
Subcutaneous Tissue ◽  
Outer Nuclear Layer ◽  
Multidisciplinary Care ◽  
Hemifacial Atrophy ◽  
Left Brain ◽  
Brain White Matter ◽  
Patient Reported ◽  
Parry Romberg Syndrome ◽  
The Brain

AbstractParry–Romberg syndrome is a rare disorder characterized by unilateral facial atrophy affecting the skin, subcutaneous tissue, and muscles, and sometimes extending to the osteocartilaginous structures. Ophthalmological and neurological involvements are relatively rare. We present a case of an early onset of left hemifacial atrophy, a progressive severe left ocular impairment, and the presence of a neurological disorder in the left hemisphere of the brain. The neuro-ophthalmological manifestations on the left side include enophthalmos, mild pseudoptosis, moderate asymmetry of the eyebrows, diffuse chorioretinal atrophy, reduced visual acuity, reduced central retinal thickness (162 µm) with the interruption of the ellipsoid zone, and the alteration of the outer nuclear layer, with associated reduced responses in photopic and scotopic amplitudes. The patient reported high intensity left brain white matter and a brief clinical focal seizure. This case report adds to the repertoire spectrum of this rare syndrome. The presentation calls for a multidisciplinary care for patients like our case.

scholarly journals Chronologic Presentation of a Severe Case of Progressive Hemifacial Atrophy (Parry-Romberg Syndrome) with the Loss of an Eye

2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Mesut Kaya ◽  
Ceyda Sel Yilmaz ◽  
Hanifi Kurtaran ◽  
Mehmet Gunduz
Keyword(s):  
Early Childhood ◽  
Muscle Tissue ◽  
Severe Case ◽  
Fatty Tissue ◽  
Hemifacial Atrophy ◽  
Progressive Hemifacial Atrophy ◽  
Subcutaneous Fatty Tissue ◽  
The Face ◽  
Parry Romberg Syndrome ◽  
Bone Structures

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.

Atypical papillitis: An isolated manifestation of Lyme disease

2019 ◽  
Vol 30 (5) ◽  
pp. NP46-NP52
Author(s):  
Lucia Mata-Moret ◽  
Carolina Garcia-Villanueva ◽  
Clara Monferrer-Adsuara ◽  
Veronica Castro Navarro ◽  
Enrique Cervera-Taulet
Keyword(s):  
Nervous System ◽  
Lyme Disease ◽  
Borrelia Burgdorferi ◽  
Skin Rash ◽  
Endemic Area ◽  
Rare Condition ◽  
Indirect Test ◽  
Physician Consultation ◽  
Early Stages

Lyme disease is a rare condition caused by the bacterium Borrelia burgdorferi. Despite typical symptoms including fever, headache, fatigue, and a characteristic skin rash, sometimes we cannot find those due to the lack of physician consultation in those early stages. If this disease is left untreated, infection could spread to the nervous system causing neuroborreliosis, an atypical and complicated manifestation of this disease. We present the case of an atypical papillitis, probably caused by this bacterium. We suspected this because of the results on the indirect test bloods and the improvement of the symptoms after treatment. This entity should be considered as a possible diagnosis of atypical optical neuropathies, particularly if it occurs in an endemic area.

scholarly journals ASPECTOS CLÍNICOS DA SÍNDROME DE PARRY-ROMBERG: RELATO DE CASO

2014 ◽  
Vol 5 (1) ◽  
Author(s):  
Gabriel Queiroz Vasconcelos Oliveira ◽  
Leticia Almeida Cheffer ◽  
Pietry Dy Tarso Inã Alves Malaquias ◽  
Leonardo Morais Godoy Figueiredo ◽  
Thiago Felippe Oliveira Macêdo ◽  
...  
Keyword(s):  
Multidisciplinary Approach ◽  
Treatment Protocol ◽  
Facial Deformity ◽  
Disease Etiology ◽  
Hemifacial Atrophy ◽  
Progressive Hemifacial Atrophy ◽  
The Face ◽  
Parry Romberg Syndrome ◽  
Therapeutic Needs

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.

scholarly journals Progressive facial hemiatrophy (Parry-Romberg syndrome): short case report

2019 ◽  
Vol 25 (3) ◽  
pp. 28
Author(s):  
Alexandre Puidupin ◽  
Aline Desoutter ◽  
Sophie Deneuve ◽  
Lara Nokovitch ◽  
Anne-Gaëlle Chaux-Bodard
Keyword(s):  
Early Childhood ◽  
Case Report ◽  
Social Life ◽  
Mosaic Disease ◽  
Localized Scleroderma ◽  
Unknown Aetiology ◽  
Adult Age ◽  
Extraoral Implants ◽  
Parry Romberg Syndrome ◽  
Facial Hemiatrophy

Introduction: The Parry Romberg syndrome (PRS) is a mosaic disease of unknown aetiology which mostly affects women. The facial hemiatrophy generally begins during the early childhood. It has a great impact on social life, and aesthetic rehabilitation is a major challenge. Observation: A 38 years-old-female patient presented with an enucleation of the left eye, due to multiple hamartomas and progressive facial hemiatrophy. The placement of 2 extraoral implants was proposed to bear an ocular epithesis. Comments: PRS develops between the 2nd and 20th year of life, and stabilizes at the adult age. Its main characteristic is a progressive facial hemiatrophy, which involves skin, fat tissues, muscles and osteocartilaginous tissues. 20% of patients have neurological symptoms, and epilepsy is often described as a side effect of the disease. Conclusion: Diagnosis of localized scleroderma has to be eliminated, as these 2 entities are very similar. Major facial reconstructive surgery is often proposed to restore aesthetics.

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