scholarly journals Morphea en coup de sabre and hemifacial atrophy in an interdisciplinary approach

2020 ◽  
Vol 3 (2) ◽  
pp. 97-107
Author(s):  
Agnieszka Wróblewska ◽  
Adriana Polańska ◽  
Aleksandra Dańczak‑Pazdrowska ◽  
Ryszard Żaba ◽  
Zygmunt Adamski ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Interdisciplinary Approach ◽  
Psychosocial Problems ◽  
Active Phase ◽  
Functional Disorders ◽  
Developmental Patterns ◽  
Hemifacial Atrophy ◽  
Parry Romberg Syndrome ◽  
Therapeutic Protocols

The increase in the degree of fluctuation asymmetry is accompanied, among others, by diseases such as morphea en coup de sabre (morphea ECDS) or Parry‑Romberg syndrome (PHA). Patients suffering from them struggle not only with dermatological defects, but also with neurological, rheumatological, orthopedic, ophthalmological and dental symptoms. Morphological and functional disorders and craniofacial deformities related to them often generate psychosocial problems. The complexity of the issues to be solved proves the necessity of undertaking interdisciplinary actions aimed at developing objective diagnostic and therapeutic protocols, restoring (especially in pediatric patients) as close as possible to the correct developmental patterns, inhibiting the active phase of the disease and undertaking effective and aesthetically satisfying measures.

Craniofacial Syndromes Part II: Atypical Facial Clefts, Romberg Syndrome, Moebius Syndrome, Fibrous Dysplasia, and Neurofibromatosis

2020 ◽  
Author(s):  
Francesca Saldanha ◽  
Cory M. Resnick ◽  
Carolyn R. Rogers-Vizena
Keyword(s):  
Fibrous Dysplasia ◽  
Treatment Plan ◽  
Moebius Syndrome ◽  
Hemifacial Atrophy ◽  
Mccune Albright Syndrome ◽  
Facial Clefts ◽  
Progressive Hemifacial Atrophy ◽  
Parry Romberg Syndrome ◽  
Albright Syndrome ◽  
Craniofacial Syndromes

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

scholarly journals Parry Romberg Syndrome with contralateral iridocorneal endothelial syndrome: a unique case

2021 ◽  
Vol 8 (12) ◽  
pp. 1873
Author(s):  
Usha K. Raina ◽  
Shruti Bhattacharya ◽  
Prateeksha Sharma ◽  
Varun Saini
Keyword(s):  
Corneal Edema ◽  
Rare Disorder ◽  
Specular Microscopy ◽  
Unique Case ◽  
Hemifacial Atrophy ◽  
Normal Side ◽  
Ocular Manifestations ◽  
Iridocorneal Endothelial Syndrome ◽  
Progressive Hemifacial Atrophy ◽  
Parry Romberg Syndrome

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.

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 Unilateral Papillitis as the Initial Presentation of Hemifacial Atrophy: Case Report and Review of Literature

2020 ◽  
Vol 10 (1) ◽  
pp. 97-101
Author(s):  
Akhlaque Hossain Khan ◽  
Nazmin Ahmed ◽  
Vijay Kumar Raut
Keyword(s):  
Neurologic Manifestations ◽  
Review Of Literature ◽  
Hemifacial Atrophy ◽  
Plastic And Reconstructive Surgery ◽  
Visual Improvement ◽  
Progressive Hemifacial Atrophy ◽  
Threatening Condition ◽  
Parry Romberg Syndrome ◽  
The Right ◽  
Fundoscopic Examination

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

scholarly journals Parry– Romberg Syndrome: A Case Report

2014 ◽  
Vol 4 (1) ◽  
pp. 67-70 ◽  
Author(s):  
P Devani ◽  
Hira Lal ◽  
A Thakral
Keyword(s):  
Rare Case ◽  
Final Diagnosis ◽  
Rare Entity ◽  
Review Of Literature ◽  
Mri Findings ◽  
Hemifacial Atrophy ◽  
Radiological Features ◽  
Progressive Hemifacial Atrophy ◽  
Parry Romberg Syndrome ◽  
Ct And Mri

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

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.

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.

TREATMENT OF PROGRESSIVE HEMIFACIALATROPHY (PARRYROMBERG SYNDROME) WITH POLYMETHYL METHACRYLATE: A CASE REPORT.

2020 ◽  
pp. 1-2
Author(s):  
Roberto Chacur ◽  
Honório Sampaio Menezes ◽  
Nívea Maria Bordin da Silva Chacur ◽  
Danuza Dias Alves ◽  
Rodrigo Cadore Mafaldo ◽  
...  
Keyword(s):  
Case Report ◽  
Polymethyl Methacrylate ◽  
Hemifacial Atrophy ◽  
Aesthetic Result ◽  
Tissue Augmentation ◽  
Progressive Hemifacial Atrophy ◽  
Subcutaneous Layer ◽  
Parry Romberg Syndrome ◽  
Good Aesthetic Result

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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