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Objective To compare the agreement in judging the responsible vessels for hemifacial spasm (HFS) between the preoperative 3D Slicer 3D reconstruction and the actual intraoperative judgment of microvascular decompression (MVD). Methods A total of 62 patients with HFS who underwent MVD in Dong'e County People's Hospital of Shandong Province from July 2018 to January 2024 were included. 3D fast inflow with the steady state precession (3D-FIESTA) and 3D time-of-flight (3D-TOF) MRA were performed before surgery. 3D Slicer software was used to fuse 3D-FIESTA and 3D-TOF MRA images and 3D reconstruction to clarify the responsible vessels. The actual intraoperative judgment was taken as the "gold standard", and the agreement between preoperative 3D reconstruction and actual intraoperative judgment was compared. Results There were 61 cases whose responsible vessels were identified by preoperative 3D reconstruction, including anterior inferior cerebellar artery (AICA) in 47 cases, posterior inferior cerebellar artery (PICA) in 6 cases, vertebral artery (VA) in 5 cases, VA + AICA in 2 cases, and superior cerebellar artery (SCA) in one case. The 59 cases were consistent with actual intraoperative judgment. In one case, the preoperative 3D reconstruction was considered as VA compression, and the intraoperative evidence was VA + AICA compression; in one case, preoperative 3D reconstruction was AICA responsible vessels were found in one case, and the intraoperative evidence was arteriole compression. The accuracy of preoperative 3D reconstruction was 95.16% (59/62). Agreement test showed a high consistency between preoperative 3D reconstruction and actual intraoperative judgment (- = 0.886, P = 0.000). Conclusions It is very accurate to define the responsible vessels by 3D reconstruction before MVD in patients with HFS, which is helpful to make the surgical plan and provide the basis for the judgment of the responsible vessels during the operation. [ABSTRACT FROM AUTHOR]

Parry Romberg Syndrome (PRS) and en coup de sabre (ECDS) are head variants of linear morphea with functional and structural implications. This study describes the clinical course, autoimmune co-morbidities, complications, and treatment of adults with PRS/ECDS at a tertiary referral center. We retrospectively reviewed the records of all 34 adult patients with PRS/ECDS identified through billing code search and seen by dermatologists at our institution between 2015 and 2021. Eight patients (23.5%) had ECDS, 8 (23.5%) had PRS, and 18 (52.9%) had overlap. Twenty-six patients (76.5%) reported ocular, oral, and/or neurologic symptoms, and 8 (23.5%) had concomitant autoimmune/inflammatory conditions. Sixteen patients (47.1%) had a skin biopsy, and 25 (73.5%) had imaging. Forty-six MRIs were obtained, of which 6 (13.0%) reported intracranial findings and 25 (54.3%) reported disease-related connective tissue damage. Twenty-four patients (70.6%) underwent systemic treatment during their disease course per available clinical records. Seventeen patients (70.8%) had improved or stable disease upon treatment completion, with an average duration of 22.2 months. Ten patients (41.7%) reported recurrence of disease following the treatment course. To address changes to facial contour, 6 patients (17.6%) opted for procedural treatments. One patient (16.7%) experienced morphea reactivation following a filler injection performed off-immunosuppression. Compared to findings in children, our study suggests adults with PRS/ECDS are more likely to have oral and ocular complications but experience less severe neurologic symptoms. While systemic treatments appear beneficial in most adult patients with PRS/ECDS, disease may recur following discontinuation.

Parry-Romberg syndrome (PRS) is a rare neurocutaneous and craniofacial disorder characterized by progressive hemifacial wasting and atrophy that predominantly affects children and young adults, with an estimated prevalence of 1 in 700,000 individuals. Despite its rarity, PRS poses significant challenges for patients, their families, and healthcare providers due to its unpredictable course and potential functional and aesthetic impairments. The main aim is to provide a comprehensive overview of PRS, encompassing its clinical features, pathogenesis, and management techniques. We present a case of PRS in a 9-year-old female with pronounced facial asymmetry, with marked wasting and atrophy involving the entire right side of the face. CT scan revealed right sided hypoplasia of maxilla, mandible, and zygomatic arch with enophthalmos of right eye. MRI showed right temporalis, medial and lateral pterygoid, masseter, risorius, buccinator, zygomaticus major and minor, levator labii superioris, levatorangulioris and orbicularis oris muscles atrophy. The clinical presentation of PRS typically involves progressive facial atrophy, predominantly affecting the subcutaneous tissues, muscles and bones. Patients may experience various symptoms as the condition advances, including facial asymmetry, hemifacial pain, dental and ocular abnormalities and neurological manifestations. The exact etiology of PRS remains unknown, although autoimmune, genetic and vascular factors are likely contributors. Treatment of PRS needs a multidisciplinary approach involving dermatologists, plastic surgeons, neurologists, ophthalmologists, and dental specialists. Treatment options aim to alleviate symptoms, improve function and address cosmetic concerns. Surgical interventions such as autologous fat grafting, facial reconstructive procedures and orthognathic surgery have restored facial symmetry and function. Additionally, nonsurgical modalities, including botulinum toxin injections, prosthetic devices and dental interventions, may offer symptomatic relief and enhance overall quality of life.

Hemimasticatory spasm (HMS) is a rare movement disorder characterized by paroxysmal spasms or twitches of the unilateral jaw-closing muscles. This study aimed to comprehensively evaluate the clinical features of patients with HMS. Data from 17 patients newly diagnosed with HMS (12 females and 5 males; mean age at onset: 46.7 years) who visited our department were retrospectively analyzed, and a literature search based on electronic medical databases from their inception until November 30, 2023, was conducted. A manual search was conducted for articles cited in the related literature. A total of 117 cases (72 females and 45 males; mean age at onset: 37.1 years) from 57 studies were analyzed. The muscles involved were the masseter (97.4%), temporalis (47.9%), and medial pterygoid (6%). Morphea or scleroderma was observed in 23.9% of the patients, and facial hemiatrophy in 27.4%. In 17.9% of the cases, Parry--Romberg syndrome was either complicated or suspected. Typical electromyographic findings included the absence of a silent period during spasms (23.9%) and irregular brief bursts of multiple motor unit potentials. Oral medicines, such as clonazepam or carbamazepine, alleviated the symptoms for some patients but were often unsatisfactory. Botulinum toxin therapy was effective in most cases. Recently, microvascular decompression surgery is increasingly being used, resulting in complete relief in some cases. In conclusion, highly effective modalities are currently available, and it is necessary to raise awareness of HMS to ensure that it can be diagnosed and treated accurately by both medical and dental professionals. [ABSTRACT FROM AUTHOR]

Background: This study aimed to evaluate the use of fat grafting enriched with platelet-rich plasma through a computerized-assisted mapping for esthetic restoration in progressive hemifacial atrophy (Parry-Romberg disease). Methods: This prospective study was conducted on 53 patients presented by Parry-Romberg disease and was corrected by facial fat grafting (FFG) enriched with platelet-rich plasma (PRP). A computerized software program was used to design a detailed map to achieve clinical symmetry with fat grafting application, as the anatomical subunits direct 3-dimensional volumetric symmetric, and compartments direct isolated recipient-specific grafting. Also, volumetric asymmetry was assessed through outlines of facial contour, projection, and proportions for both sides and comparing the mirror image of unaffected side as a template. Results: Objective ultrasound and photogrammetric measurements showed a significant improvement in facial symmetry postoperatively compared to preoperative (P < 0.05), with no significant differences between 12 and 18 months after surgery. The FACE-Q score regarding comparison before surgery and 18 months after surgery showed a statistically significant improvement in all modules (P < 0.001) with overall satisfaction concerning outcome of 82.7 ± 0.8. Conclusion: Enriched fat grafting with platelet-rich plasma (PRP) is a good alternative to reconstruct soft tissue defects for patients with progressive hemifacial atrophy (Parry-Romberg disease) with a minimally invasive approach and low complications. It provides volumetric replacement, enhancement of skin texture, and improvement of hyperpigmentation with restoration of facial contour for an esthetic pleasing appearance. Isolated replacement in accordance with the anatomical facial subunits and fat compartments allows three-dimensional reconstruction and maximizes fat retention. [ABSTRACT FROM AUTHOR]

Parry Romberg syndrome also known as progressive hemifacial atrophy is an uncommon degenerative condition, characterized by unilateral, slow, and progressive atrophy of face. Patient presents with loss of facial symmetry and neurological manifestations. After the degenerative process settles, reconstructive surgeries are performed to address facial asymmetry. For accurate assessment of volume deficit, laser scanning and three- dimensional printing can be used which offers the advantage of precise surgical planning and good aesthetic outcome. We present a case of soft tissue reconstruction in Parry Romberg syndrome with anterolateral thigh flap with use of three- dimensional laser scanning. [ABSTRACT FROM AUTHOR]

Objective Parry⁃Romberg syndrome is a rare disease characterized by unilateral facial progressive atrophy accompanied by ipsilateral intracranial lesions and/or cerebral atrophy, this article is aimed to analyze the clinical, radiological and pathological features of patients with Parry ⁃ Romberg syndrome and explore the microvascular mechanism in the pathogenesis of the disease, to provide reference for understanding the nature of disease. Methods and Results The age of onset was 27 and 17 years old of the 2 patients respectively. Case 1 initially presented with left⁃sided progressive hemifacial atrophy and developed paroxysmal pain, twitch and numbness on the left face. Case 2 presented with a band ⁃ like alopecia in the left fronto⁃parietal area with cutaneous atrophy, and sudden right limb mild hemiplegia. The MRI of Case 1 revealed a space⁃occupying lesion in the left frontal and insular lobes without enhancement. The MRI of Case 2 showed recent lacunar cerebral infarction in left posterior limb of internal capsule. The histopathological examination of Case 1 showed hemorrhage, microvascular thrombosis, fibrinoid necrosis of vascular walls and mild peri⁃microvascular chronic inflammatory cell infiltration, who was irresponsive to prednisone. Case 2 received aspirin and remained free from any cerebrovascular event at his one⁃year⁃ follow up. Conclusions The microvascular mechanism may be the pathogenesis of Parry ⁃ Romberg syndrome, and the antiplatelet therapy may be an optional schedule for stroke prevention and treatment in patients with Parry⁃Romberg syndrome.

Purpose: Parry-Romberg syndrome (PRS) is a rare disorder characterized by slowly progressive hemifacial atrophy. Although the underlying etiology is unknown, proposed disease mechanisms which include autoimmune, infection, trauma, and other causes have been proposed as underlying disease mechanisms. Approximately, 10–20% of PRS patients have neurologic manifestations such as epilepsy, headaches, or associated vascular malformations. There are reports of PRS responsive to immunosuppressive medications, supporting the autoimmune hypothesis. Currently, the neuropathologic findings in patients with PRS are not well documented. Methods: Herein, we present a case of a 19-year-old female with PRS, who underwent partial frontal lobectomy for intractable epilepsy. Results: The resection specimen showed multifocal active lymphocytic (T-cell mediated) arteritis in midsized cortical arterioles, with the adjacent meninges showing fibrosis involving both the meningeal tissue and its vascular network. In addition, neuronal loss and gliosis were evident in the cortex, likely due to the associated vascular injury. Conclusions: This report is the first to demonstrate active cerebral vasculitis in a patient with PRS, supporting the previous suspicion of inflammatory etiology in this disease. In addition, the widespread vascular fibrosis in the meningeal vessels and an area of cortical ischemia support the presence of previous inflammatory vascular processes in the area. [ABSTRACT FROM AUTHOR]

Background: Parry–Romberg syndrome (PRS) is an enigmatic and acquired degenerative condition. It is characterized by a gradual and progressive facial hemiatrophy, after which facial deformities appear unexpectedly in a stable state. The degree of disfigurement depends on tissue involvement, illness onset, and duration. Nowadays, non-invasive treatments with lower morbidity, such as structured fat grafting (SFG) are preferred over traditional operations such as free flaps and bone grafts. However, for severe cases of PRS, multiple sessions of SFG are required. Case study: Despite the therapeutic failure that a 20-year-old lady had suffered after five previous conventional surgical procedures, the outcome was successful after only one surgical therapy employing a particular combination of SFG and malar silicone elastomers implant (SI). Conclusion: The author highlights the interest in a combination of non-invasive procedures using SFG and malar augmentation with SI to repair large volume loss while improving symmetry and skin texture. Otherwise, the role of adipose-derived stem cells in fat survival is crucial and should be the focus of future studies and the key to technological advancement. [ABSTRACT FROM AUTHOR]

Rheumatic diseases have very heterogeneous manifestations and diagnostic criteria. Rheumatic diseases have a wide range of involvement, including systemic (joints and internal organs), as well as skin, mucosa, hair, and nails. Some of these symptoms can cause severe comorbidities and significantly impair the quality of life. Skin findings are critical for early recognition or reinforcing the diagnosis of rheumatic diseases. At the same time, some skin lesions have particular importance as they may be the first and/or most serious comorbid symptom of the disease. Although most of these findings are not specific for rheumatic diseases (such as facial telangiectasia in scleroderma or nonscarring alopecia seen in systemic lupus) some findings may be disease-specific (e.g. discoid lesions in discoid lupus, malar rash in systemic lupus, and Gottron papules in dermatomyositis). Thanks to the contributions of dermatology, the skin findings of rheumatic diseases have become clearer in recent years, thus enabling the classification, phenotyping, and early treatment of rheumatic diseases. Considering all these, each dermatological finding should be taken into consideration and evaluated on a case basis in terms of suspected condition, diagnosis, treatment, and management of post-treatment comorbidities. In conclusion, both rheumatologists and dermatologists have a great responsibility in detailed anamnesis and dermatological examination for detecting the condition, classifying and phenotyping as when necessary, and developing early treatment options. [ABSTRACT FROM AUTHOR]

In this report, we describe an unusual case of progressive hemifacial atrophy or Parry-Romberg syndrome in a 10-year-old girl with progressive hemifacial microsomia and limb anomalies who had brain magnetic resonance imaging (MRI) findings of white matter hyper-intensities. Patients typically present with neurological manifestations such as epilepsy, facial pain, and migraines and ophthalmological symptoms in conjunction with white matter lesions. The patient demonstrated normal cognition and psychomotor development despite the presence of white matter lesions in her frontal lobe that is commonly associated with neurological symptoms. This report brings attention to the complicated relationship between facial, limb and brain imaging findings in Parry-Romberg syndrome and differentiates it from hemifacial microsomia syndrome.

Objective Parry-Romberg syndrome is a rare disease characterized by unilateral facial progressive atrophy accompanied by ipsilateral intracranial lesions and/or cerebral atrophy, this article is aimed to analyze the clinical, radiological and pathological features of patients with Parry - Romberg syndrome and explore the microvascular mechanism in the pathogenesis of the disease, to provide reference for understanding the nature of disease. Methods and Results The age of onset was 27 and 17 years old of the 2 patients respectively. Case 1 initially presented with left -sided progressive hemifacial atrophy and developed paroxysmal pain, twitch and numbness on the left face. Case 2 presented with a band - like alopecia in the left fronto-parietal area with cutaneous atrophy, and sudden right limb mild hemiplegia. The MRI of Case 1 revealed a space-occupying lesion in the left frontal and insular lobes without enhancement . The MRI of Case 2 showed recent lacunar cerebral infarction in left posterior limb of internal capsule. The histopathological examination of Case 1 showed hemorrhage, microvascular thrombosis, fibrinoid necrosis of vascular walls and mild peri -microvascular chronic inflammatory cell infiltration, who was irresponsive to prednisone. Case 2 received aspirin and remained free from any cerebrovascular event at his one - year - follow up. Conclusions The microvascular mechanism may be the pathogenesis of Parry - Romberg syndrome, and the antiplatelet therapy may be an optional schedule for stroke prevention and treatment in patients with Parry-Romberg syndrome. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study is to compare three-dimensional cephalometric measurements of the skeleton and dentoalveolar region between Turkish individuals and individuals from other populations. Methods: This is a single-center, retrospective study using cone beam computed tomography images (CBCT). Individuals have no missing teeth, are between the ages of 18 and 30, have a balanced profile, and have an Angle Class I relationship. The CBCT images were obtained in DICOM format and analyzed in DOLPHIN 11.8 software. 61 parameters were measured on the CBCT images, and 14 parameters were contrasted with individuals from other populations. Results: Mandibular values were more protrusive in Korean and Chinese populations than in Turkish and Cypriot individuals. Similarly, upper facial height (N- ANS distance) was found to be much higher. It was found that the length of the mandibular corpus was shorter and the angle of convexity was higher in Turks and Cypriots than in Koreans and Chinese populations. Conclusion: Ethnic facial and skeletal characteristics play a fundamental role in the outcome and course of orthodontic treatment. Gender differences also play an important role when facial sizes and proportions are compared in different societies. [ABSTRACT FROM AUTHOR]

We present the clinical course of a 9-year-old female patient with Bloch–Sulzberger syndrome and severe neurological deficit that met the major (classic cutaneous signs) and minor (dental anomalies and retinal pathology) diagnostic criteria of Landy and Donnai. Longitudinal multidisciplinary follow-up was carried out from birth to adulthood. Neurological involvement was assessed with electroencephalographic (EEG) and neuroimaging tests at different times during the patient's life. Cranio-maxillofacial involvement was evaluated using lateral skeletal facial and cephalometric analyses. The right and left facial widths were measured through frontal face analysis and using the vertical zygomatic–midline distance. Oral rehabilitation was performed through orthodontic treatment and major dental reconstruction using composite resins. This treatment aimed to improve the occlusion and masticatory function, relieve the transversal compression of the maxilla, and reconstruct the fractured teeth. We believe that, due to significant neurological and cognitive impairment, orthognathic surgery was not the best option for restoring function and improving oral health-related quality of life. [ABSTRACT FROM AUTHOR]

Objective: This study investigates crown and root anomalies in patients with Parry-Romberg Syndrome. Design: This is a retrospective review of patients with Parry-Romberg Syndrome who were evaluated at a tertiary care center from 1980-2020. Setting: Patients seen in the dental unit from 1980-2020. Patients, Participants: Seventeen patients with documented Parry-Romberg Syndrome were referred for dental evaluation. Main Outcome Measures: All dental anomalies were documented. Root anomalies were assessed using panoramic radiographs and cone beam CT (CBCT) scans to evaluate buccal-lingual, mesio-distal, and axial measurements of hypoplastic teeth, which were compared to those of contralateral teeth. Results: Findings included agenesis (29%, n = 5), hypoplastic teeth (29%, n = 5), delayed canine eruption (24%, n = 4), and mulberry molars (12%, n = 2). Of the five patients with tooth hypoplasia, four had CBCT records and the fifth had panoramic radiographs available for assessment. Axial length was always shorter in hypoplastic teeth relative to contralateral teeth, with differences ranging from 1.2-9.2 mm. Differences in crown size of hypoplastic versus contralateral teeth were unpredictable but always present. Conclusions: Patients with Parry-Romberg Syndrome can have hypoplastic roots with atypical crown morphology. A patient's specific dental anomaly will influence planning and treatment. [ABSTRACT FROM AUTHOR]

Parry--Romberg syndrome (PRS) is a rare disorder characterised by progressive hemifacial atrophy, along with various neurological and ophthalmological manifestations. Patients with PRS may undergo reconstructive procedures such as fat grafting to address the facial contour deformities. Anaesthesiologists may face significant challenges due to the asymmetric craniofacial structures and the potential presence of neurological deficits, including hemiparesis. We report a case of an 18-year-old male with left-sided hemifacial atrophy and PRS with wasting of one side of the lobe of the thyroid gland who underwent fat grafting under general anaesthesia. The challenges faced during the anaesthetic management of this patient are airway management, positioning and monitoring of neurological deficits. PRS has been reported to coexist with other autoimmune diseases, including thyroid disorders. However, thyroid involvement in PRS is rare. This case used ultrasound to evaluate the airway and assess PRS impact. This case highlights the importance of ultrasound airway, which can serve as an effective tool for identifying associated conditions (like thyroid atrophy) in patients with PRS and can assist in the diagnosis and management of this rare and complex disease. [ABSTRACT FROM AUTHOR]

Introduction: Progressive Facial Hemiatrophy, also known as Parry Romberg Syndrome, is a rare form of linear scleroderma. The management of facial atrophy sequelae is challenging. Objective: This study aims to evaluate for the first time in literature the effectiveness of the calcium hydroxylapatite (CaHa) filler in the jawline reshaping of a patient with Parry Romberg Syndrome. Case report: A 15-year-old woman with progressive atrophy of the right side of the face due to Parry Romberg Syndrome. After disease control, the patient’s main complaint was facial asymmetry, mainly in the jawline region. Discussion: CaHa is a biocompatible injectable filler that is gradually resorbed and replaced by fibrovascular stroma, mainly formed for new collagen, in a process that occurs without any immunological reaction. This safety profile makes CaHa a good choice for correcting stable scleroderma defects. Conclusion: This report concludes that CaHa filler biocompatibility and suitability for the jawline correction are also applicable in reconstructive procedures for stable scleroderma, safely and minimally invasively, with optimal aesthetic results. The method must be programmed case-by-case, and a regular follow-up is also recommended.

We present the clinical course of a 9-year-old female patient with Bloch–Sulzberger syndrome and severe neurological deficit that met the major (classic cutaneous signs) and minor (dental anomalies and retinal pathology) diagnostic criteria of Landy and Donnai. Longitudinal multidisciplinary follow-up was carried out from birth to adulthood. Neurological involvement was assessed with electroencephalographic (EEG) and neuroimaging tests at different times during the patient’s life. Cranio-maxillofacial involvement was evaluated using lateral skeletal facial and cephalometric analyses. The right and left facial widths were measured through frontal face analysis and using the vertical zygomatic–midline distance. Oral rehabilitation was performed through orthodontic treatment and major dental reconstruction using composite resins. This treatment aimed to improve the occlusion and masticatory function, relieve the transversal compression of the maxilla, and reconstruct the fractured teeth. We believe that, due to significant neurological and cognitive impairment, orthognathic surgery was not the best option for restoring function and improving oral health-related quality of life.

Copyright of Pamukkale Medical Journal is the property of Pamukkale Journal of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Clinical Neuroscience / Ideggyógyászati Szemle is the property of LifeTime Media Kft. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Although international attention is focused on the direct effects of the coronavirus disease 2019 (COVID-19), the healthcare consequences of inadequate medical care of non--COVID-19-related diseases should also be considered. We therefore aimed to describe the indirect effects of the COVID-19 epidemic on pediatric ambulatory healthcare associated with fear from COVID-19. Methods: Epidemiologic data on number of visits to a multidisciplinary outpatient clinic, which provides care for children with chronic illnesses, were collected during a two-month period of the COVID-19 outbreak. The characteristics of patients whose avoidance of planned visits resulted in an adverse health consequence were described. Results: During the study period, a 34% decrease in ambulatory care visits was seen, compared with the corresponding period in 2019. Forty patients avoided a scheduled essential visit. After staff intervention 36 were rescheduled and received essential care, however, four patients (4/1200 total visits, 0.33%) with a median age of 8.5 years (range: 2.5-18) remained unconvinced and avoided the visit. Their background illness included DiGeorge syndrome, Parry-Romberg syndrome, uncontrolled epilepsy and Crohn disease. Despite good compliance to follow-up prior to the outbreak, all avoided an essential outpatient visit that resulted in worsening of their chronic medical condition. Their shared reason for no-show was the fear of contracting COVID-19. Conclusion: This series highlights the possible adverse health outcomes among chronic pediatric patients resulting from accessibility barriers posed by the fear of COVID-19. It provides another important aspect of the overall health impacts of the COVID-19 pandemic that requires targeted interventions. [ABSTRACT FROM AUTHOR]

Parry-Romberg syndrome (PRS) is an uncommon degenerative craniofacial condition characterized by a slow, progressive, and unilateral atrophy of facial tissues, including muscles, cartilage, nerves, bones, and skin. This article reports a severe case of PRS in a 22-year-old adult female, exhibiting facial asymmetry, hypoplasia of the right side of the face, areas of skin hyper pigmentation, and oral changes involving the mandible and teeth in whom orthodontic intervention is being done.

Copyright of Polish Otorhinolaryngological Review / Polski Przegląd Otorynolaryngologiczny (Index Copernicus) is the property of Index Copernicus International and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Ege Journal of Medicine is the property of Ege University, Faculty of Medicine and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Purpose: We describe a case of Parry-Romberg syndrome (PRS) presenting with panuveitis and retinal vasculitis.Methods: We conducted a retrospective review of our patient's case and related literature published through May 2019.Results: A 26-year-old woman with history of PRS was diagnosed with panuveitis and retinal vasculitis. Intraocular inflammation was controlled with local and systemic corticosteroids. The relationship between PRS and intraocular inflammation is discussed with references to the relevant on literature.Conclusions: Our findings and the accompanying literature review suggest that the patient's ocular involvement included multiple fundus lesions, retinal vascular disorder, and unilateral poliosis - all of which may be attribute to trigeminal neuro vasculitis. As the Varicella-zoster virus may contribute to the onset of the autoimmune processes associated with PRS, this requires further exploration. This report confirms the utility of multimodal imaging in the study, screening, and follow-up of intraocular inflammation in patients with PRS. [ABSTRACT FROM AUTHOR]

Acquired facial lipoatrophy is a rare disease with an unclear etiology and pathological pathway. The distinct causative factors of this disease have been not elucidated, but it is suspected to be associated with immune systemrelated diseases, most notably AIDS. Although the management of facial lipoatrophy is very important for patients' social life and mental health, no treatment framework has been developed due to the unknown nature of the disease manifestation. The present case report was designed to provide sequential imaging to visualize the disease progression. The clinical backgrounds of the patients are also introduced, helping characterize this disease entity more clearly for maxillofacial specialists. [ABSTRACT FROM AUTHOR]

Background: Parry-Romberg syndrome is characterized by progressive hemiatrophy of the skin, subcutaneous tissue, muscle and bones of the skull. Its incidence is low, with a progressive and slow course. Its etiology is unknown, but it has been associated with several factors. Its clinical presentation involves dermatological, musculoskeletal and neuropsychiatric manifestations. The treatment consists of medical and surgical strategies. The use of steroids, alone or in combination with immunomodulators, has the objective of slowing down progression. The surgical treatment lies in facial reconstruction or volumetric regeneration, to correct the appearance and function of facial structures. The objective is to show three cases of Parry-Romberg syndrome with the representative characteristics of the disease. Clinical cases: 1) A 41-year-old woman with atrophy and right supraciliary hypochromia associated with seizures and headache. 2) A 43-year-old woman with parietal deformation and right supraciliary atrophy, associated with facial paralysis, depression and headache. 3) A 36-year-old woman with right hemifacial atrophy associated with ocular involvement and headache. The indicated treatment was based on a systemic steroid plus a cytostatic agent. The surgical treatment was evaluated according to the affection of facial structures. Conclusions: Parry-Romberg syndrome is a rare disease, characterized by progressive atrophy. Clinical manifestations determine the diagnosis and early start of medical and surgical treatment. [ABSTRACT FROM AUTHOR]

Fat hypertrophy is a less commonly known complication of autologous fat transfer. We present a 32-year-old female with left hemifacial atrophy associated with systemic sclerosis, who was treated with 7 fat transfer procedures to correct the facial asymmetry. A total of 236.5 mL of fat was injected to the hemiface over a 4-year period to achieve good symmetry. A progressively enlarging, painless, soft mass over the left parotid region was noted at 3 months after the final fat transfer procedure. Magnetic resonance imaging showed a markedly enlarged bulk of subcutaneous fat over the left cheek with no evidence of necrosis, edema, or pathologic enhancement. Concurrent weight gain was noted secondary to additional nutritional input. The patient's aesthetic, symptomatic, and functional concerns led to the subsequent removal of 115 mL fat by liposuction.Level Of Evidence:5: [ABSTRACT FROM AUTHOR]

To provide an update on the current management, including evaluation and treatment, and the available diagnostic tools for linear scleroderma of the head, i.e., Parry-Romberg Syndrome and en coup de sabre (PRS/ECDS). A rapid scoping review of the literature was conducted to include manuscripts published in English between 2010 and 2019. Literature searches were performed in PubMed and EMBASE databases. The were analyzed for descriptive statistic reporting. This study reviewed 215 manuscripts reporting these 1430 patients. Surgical reports comprised the majority of the reviewed literature. Most PRS/ECDS did not appear to receive comprehensive multisubspecialty evaluation for extracutaneous manifestations; 21% of cases noted neurological screening, 4% noted dental screening, and 3% noted ophthalmologic screening. Methotrexate and glucocorticoids remain the most frequent choice for immunosuppressive treatment, though fewer than 7% of patients reported receiving systemic medical therapies. Surgical procedures for cosmetic or functional improvement were common (59%) among the reported patients. Autologous fat grafting was the most frequently utilized cosmetic treatment (50% of procedures) followed by free flap transfers (24% of procedures). There is ongoing need for standardized evaluation, monitoring, and treatment to prevent morbidity in PRS/ECDS, especially in children. When these patients are managed by rheumatologists, methotrexate, and steroids remain the first-line treatment, but a review of the published literature reflects that this may be a minority. Most PRS/ECDS patients are not evaluated in a multidisciplinary fashion. We propose comprehensive evaluations across subspecialties at the baseline and follow-up levels to monitor disease activity and record extracutaneous manifestations, treatment algorithms, and surgical intervention considerations. [ABSTRACT FROM AUTHOR]

We present the case of a 4-year-old patient, with the appearance of a hyperchromic macula in the right region malar with skin retraction, since the first year of life. The clinicopathological correlation suggested the diagnosis of localized scleroderma or morphea. We also present a brief review of the literature about localized scleroderma and its variants, with the objective of contributing to the knowledge of the first levels of care and favoring the timely diagnosis and a positive impact in the management and prognosis of this sickness. In conclusion, the localized scleroderma or morphea is a disease that affects the quality of life of the patient, since it is not limited to the skin with a spectrum of cutaneous manifestations, but also some compromise variants extradermic. As an early diagnosis, allows to establish an appropriate treatment and follow-up avoiding the development of complications. [ABSTRACT FROM AUTHOR]

Objective: To study the clinical characteristics and prognosis of morphea in Thai adult patients. Methods: The medical records of 81 morphea adult patients who visited Siriraj Hospital, Thailand, between 2006 and 2015, were retrospectively reviewed. The demographics, clinical features, treatments and outcomes were analyzed. Clinical improvement was categorized as excellent (>80%), partial (1%-80%), and no response. Results: Circumscribed morphea was the most common subtype (34, 42%) of the 81 patients, followed by linear morphea (21, 26%), generalized morphea (14, 17%), en coup de sabre (11, 14%) and Parry-Romberg syndrome (1, 1%). Systemic treatment and ultraviolet A (UVA)1 phototherapy had satisfactory outcomes in 83% and 79% of cases, respectively, providing a partial to excellent response. For overall remission, one year after the treatment, 30% of patients achieved a partial to excellent response. After 2 years, this proportion rose to 50%. The median time to clinical response was 24 months. Conclusion: Morphea is a difficult-to-treat dermatosis, with the majority of the patients having a partial clinical response and a high recurrent rate. Combination of treatment might be a worthy option. [ABSTRACT FROM AUTHOR]

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Background: It is necessary to understand the mechanism of trigeminal neuralgia (TN) and hemifacial spasm (HFS) in order to seek for an effective noninvasive remedy. As previous studies implied that inflammatory cytokines induced by demyelination following the nerve injury may be the initiated factor causing neuropathic pain, we attempt to analyze the correlation between cytokines and these hyperactive cranial nerve disorders. Method: The consecutive patients whose diagnosis were confirmed by microvascular decompression surgery as primary TN or HFS caused by vascular compression and healthy volunteers between March and May 2018 in XinHua Hospital Shanghai JiaoTong University School of Medicine were recruited. Preoperatively, venous blood was collected and the protein concentrations of IL-1β, IL-2, IL-6, IL-8, IL-10, TNF-α and IFN-γ were determined with ELISA. Each cytokine was compared between the patients and healthy volunteers. Results: Ultimately, 28 healthy volunteers as well as 44 TN and 47 HFS patients were enrolled in this investigation. The serum levels of IL-1β, IL-6, IL-8 and TNF-α in either HFS or TN patients were significantly higher than that in healthy volunteers (p < 0.05), yet which were similar between TN and HFS patients (p > 0.05). Besides, there was a significantly correlation between IL-6 concentration and severity of HFS (r = 0.933, p < 0.05) or TN (r = 0.943, p < 0.05). Discussion: Vascular compression of trigeminal or facial nerve roots may induce a rise in variety of cytokines, and IL-6 may play an important role in the signaling pathways to generate ectopic impulses from these cranial nerves. [ABSTRACT FROM AUTHOR]

This correction notice addresses an error in the original publication of an article titled "Neuropathologic findings in a patient with Parry-Romberg syndrome: active cerebral vasculitis and brain injury." The incorrect consent for publication statement stated that no written consent was obtained from the patient, but the correct information is that written informed consent for publication of the patient's clinical details and clinical images was obtained. The authors of the article are Edina A. Wappler-Guzzetta, Brian W. Hanak, Firas Bannout, Sheri L. Harder, and Jeremy K. Deisch. [Extracted from the article]

Removal of an eye may be indicated in cases of congenital abnormality, severe trauma, or disease such as an infection, tumor, or malignancy. The disfigurement associated with a loss of an eye is often accompanied with physical problems, psychological trauma, and a poor quality of life. A prosthetic replacement is the treatment of choice to return the individual to his normal vocation by producing an acceptable and life-like appearance. This article describes prosthetic rehabilitation of a 19-year-old male suffering from facial hemiatrophy with the loss of his left eye due to retinoblastoma when he was 2-year-old using medically graded silicone material. The technique used is simple, cost effective, and easy way for fabrication and rehabilitation of an orbital defect using silicone prosthesis where retention is achieved by a combination of silicone adhesives and tapes, and to a very small extent by bony and soft tissue undercut, hence providing better esthetic and psychological outcome. The acrylic part of the prosthesis was adhered to the socket with the help of a two-way silicon adhesive tape. Since the patient had lost his eye when he was 2-year-old, the development of eye and periorbital tissue on the defect side lead to hemiatrophy; in our approach, we have attempted to build the prosthesis in par with the normal side so that the fullness on the defect side was restored to that of the contralateral side. The fabricated facial prosthesis was durable, esthetic, and had good retention.

A case study is presented of a 43-year-old right-handed man with evaluation of progressive gait difficulty. His cranial nerves examination revealed an impairment in up gaze and visual acuity was reduced in the left eye, and his vitamin E level was undetectable in the serum which was tested on two separate occasions. He was treated for dystonia and ataxia syndrome.

Parry-Romberg syndrome (PRS) or progressive facial hemiatrophy is a developmental craniofacial disorder of unknown etiology characterized by a slowly progressive unilateral facial atrophy. It is associated with different systemic manifestations particularly, maxillofacial, neurologic and ophthalmologic abnormalities. Dentists must be aware of PRS to identify this invalidating disorder. In this article, we review the etiology, clinical features (especially craniofacial and dental manifestations) and treatment of PRS. We searched in PubMed line using specific words such as PRS from 2008 to 2014 (August). We identify 14 papers have described oral manifestations of this syndrome. We excluded all the article papers that did not indicate to oral manifestations of PRS.