PARRY ROMBERG SYNDROME - A RARE CASE REPORT

The purpose of this report is to present a rare case of Parrv-Romberg syndrome (PRS), also called "progressive hemifacial atrophy". This rare degenerative condition is characterized by atrophic changes affecting one side of the face. The etiology of these changes remains idiopathic. Definitive diagnosis of Parry-Romberg syndrome is based on thorough clinical and radiological examination. Treatment is usually conservative while others include using alloplastic implants to improve facial disfigurement was suggested to the patient.

Clinical and imaging characteristics of 107 patients with linear morphea: a retrospective analysis / 中华皮肤科杂志

Objective:To analyze clinical, laboratory and imaging characteristics of different subtypes of linear morphea (LM) , and to propose an appropriate approach to the diagnosis and severity assessment of LM.Methods:Clinical data were collected from patients with clinically and/or pathologically confirmed LM in Department of Dermatology, West China Hospital, Sichuan University from January 2018 to December 2019, and retrospectively analyzed.Results:A total of 107 patients with LM were enrolled into this study, including 63 with LM of the limbs/trunk, 22 with morphea en coup de sabre, 11 with progressive hemifacial atrophy and 11 with eosinophilic fasciitis. Disease severity was evaluated by using the modified localized scleroderma skin severity index (mLoSSI) and localized scleroderma skin damage index (LoSDI) scores in 88 patients, with the mLoSSI scores ranging from 0 to 51 points, and the LoSDI scores ranging from 0 to 40 points. Routine blood examination of 10 patients with eosinophilic fasciitis showed increased eosinophil counts in 4 patients. Thirteen (14.8%) of 88 patients with LM were positive for antinuclear antibody, with titers of ≥ 1∶320. Magnetic resonance imaging (MRI) examination showed ipsilateral cerebral hemisphere atrophy and contralateral white matter hyperintensity on T2-weighted images in 2 out of 4 patients with progressive hemifacial atrophy, myofascial thickening in 26 out of 28 patients with LM of the limbs/trunk (92.9%) , subcutaneous septal and myofascial thickening in all 11 patients with eosinophilic fasciitis.Conclusions:The preliminary assessment of disease activity, severity and prognosis of LM can be made by mLoSSI and LoSDI. MRI examination is recommended for patients with clinical signs of involvement of subcutaneous structures.

Autologous Fat Transfer to Improve Aesthetic Appearance in Facial Asymmetry from Parry-Romberg Syndrome: A Case Report

@#<p><strong>OBJECTIVE: </strong>To present a case of progressive hemifacial atrophy in a young woman with Parry-Romberg Syndrome and the role of autologous fat transfer to improve her aesthetic appearance and lessen facial asymmetry.</p><p><strong>METHODS:</strong></p><p><strong>Design:</strong>           Case Report</p><p><strong>Setting:</strong>           Tertiary Government Training Hospital</p><p><strong>Patient:</strong>           One</p><p><strong>RESULTS: </strong>A 20-year old woman consulted because of drooping of the right eyelid and gradual thinning of right cheek muscles since age 16.  On examination, the right facial muscles were hypoplastic with prominent facial bony ridges. An MRI scan showed atrophy of the right medial pterygoid and masseter. She underwent autologous fat transfer on the right side of the face to augment the cheek, improve cosmetic appearance and lessen facial asymmetry.  </p><p><strong>Conclusion: </strong>Our patient is satisfied and happy with the outcome and cosmetic appearance of her autologous fat transfer and is ready to undergo the same procedure if the need arises in the future. Although no definite cure exists for Parry Romberg syndrome, our report illustrates the role of autologous fat transfer as an inexpensive, easily harvested and biocompatible material to improve facial asymmetry. The procedure yielded encouraging results, although long-term benefits remain uncertain.</p><p> </p><p><strong>Keywords:</strong> Parry-Romberg syndrome; progressive hemifacial atrophy; autologous transplantation</p>

Surgical manmgement with free flap in repairing hemifacial atrophy in 19 cases / 中华显微外科杂志

Objective To investigate the long-term effect of free flap in repairing hemifacial atrophy.Methods From April,1991 to April,2015,19 cases (5 males and 14 females) aged between 18 and 51 years (mean,26 years) with hemifacial atrophy were treated by free flap.Hemifacial atrophy affected the left side in 8 patients,and the right side in the remaining 11.Fifteen cases only had a hemifacial soft tissue deficit,the other 4 cases were associated with ipsilateral zygoma dysplasia,in which 1 had ipsilateral scar contracture.The latissimus dorsi muscle flap were applied in 12 cases (2 of which anastomose thoracodorsal nerve with the cervical branch of the facial nerve),the scapular flap in 2 cases,the tensor fascia latae dermal flap with dermal fat flap in 3 cases,and with iliac groin composite tissue flap and ilium groin dermal composite tissue flap in 1 case respectively.Among them,14 flaps had their blood vessels anastomosed to the superficial temporal artery and vein,and 5 to the facial artery and vein.All operations were successfuly accomplished and followed-up for 2-21 years.Results All patients had ideal clinical outcomes with significant improvement in facial symmetry and skin color,and no dysfunction happened.Conclusion The long-term effect of surgical management with free flap in repairing hemifacial atrophy is satisfactory.It is an effective method of clinical treatment.

Progressive hemifacial atrophy with ciliary body atrophy and ocular hypotony.

Progressive hemifacial atrophy (PHA) is a disease of unknown etiology affecting one‑half of the face. Ocular involvement is uncommon. Atrophy of iris is rare, with only a few cases of partial atrophy being reported in the literature. We report a case of total atrophy of iris and ciliary body with associated ocular hypotony in a 16‑year‑old girl with PHA. We believe this is the Access this article online Quick Response Code: Website: www.ijo.in DOI: 10.4103/0301-4738.151474 PMID: *** first reported case of complete atrophy of iris and ciliary body in PHA. Ocular hypotony in PHA was thought to be due to intra‑ocular inflammation. However in our case it appears to be secondary to severe atrophy of the ciliary body.

Síndrome de Parry-Romberg: achados nas sequências avançadas de ressonância magnética - relato de caso / Parry-Romberg syndrome: findings in advanced magnetic resonance imaging sequences - case report

Síndrome de Parry-Romberg é uma doença rara caracterizada por atrofia hemifacial progressiva associada a outras alterações sistêmicas, dentre elas, neurológicas. Atualmente, são poucos os trabalhos que exploraram sequências avançadas em ressonância magnética nesta enfermidade. Neste artigo, relatamos o caso de um paciente com 45 anos e descrevemos os achados de ressonância magnética estrutural e em sequências avançadas, correlacionando com dados fisiopatológicos.

Parry-Romberg syndrome is a rare disease characterized by progressive hemifacial atrophy associated with other systemic changes, including neurological symptoms. Currently, there are few studies exploring the utilization of advanced magnetic resonance sequences in the investigation of this disease. The authors report the case of a 45-year-old patient and describe the findings at structural magnetic resonance imaging and at advanced sequences, correlating them with pathophysiological data.

Abordagem terapêutica da síndrome de parry-romberg baseada em um sistema de classificação de gravidade / Therapeutic Approach To The Parry-Romberg Syndrome Based On A Severity Grading System

Introdução: Síndrome de Parry-Romberg (SPR) é caracterizada pela atrofia hemifacial progressiva que, muitas vezes, resulta em graves distúrbios estéticos e funcionais. Embora existam escalas de gravidade, nenhuma delas é completamente ideal para auxiliar na abordagem terapêutica destes pacientes. O objetivo deste estudo foi delinear as estratégias cirúrgicas para o tratamento da SPR baseado em um novo sistema de classificação de gravidade da doença. Método: Trata-se de uma análise retrospectiva dos pacientes com SPR operados em 2005-2011. As abordagens cirúrgicas foram individualizadas de acordo com a escala de gravidade clínica baseada na evolução da doença: tipos I (envolvimento da epiderme, derme e tecido subcutâneo); II (tipo I + envolvimento muscular); e III (tipo I + II + envolvimento ósseo). Quatro (28,57%) pacientes com SPR tipo I, 6 (42,85%) tipo II e 4 (28,57%) tipo III foram incluídos. Resultado: Um total de 47 procedimentos foi realizado. Gordura livre foi enxertada em todos os pacientes. Todos os pacientes do tipo II e 1 (25%) do tipo III foram submetidos a enxertos dermogordurosos. Enxertos ósseos com retalhos de fáscia têmporo-parietal foram aplicados no tratamento de todos os pacientes do tipo III. Um (25%) paciente do tipo III foi submetido à cirurgia ortognática. Houve melhora global na aparência facial em todos os pacientes, sem complicações relacionadas aos procedimentos. Conclusão: O sistema de classificação de gravidade proposto para a SPR pode facilitar a decisão terapêutica e resultados parcialmente satisfatórios podem ser alcançados com a combinação de técnicas cirúrgicas de acordo com a gravidade da doença.

Introduction: The Parry-Romberg Syndrome (PRS) is characterized by progressive hemifacial atrophy that often leads to severe esthetic and functional difficulties. Although there are systems for grading disease severity, none have proven ideal in optimizing the therapeutic approach to these patients. This study aimed to establish the surgical strategies for the treatment of PRS based on a new system for severity grading of the disease. Methods: This retrospective study included PRS patients undergoing surgery between 2005 and 2011. The surgical strategies were adapted for each patient according to a clinical severity grading system based on disease progression: type I, affecting the epidermis, dermis, and subcutaneous tissue; type II, type I + muscle involvement; and type III, Types I+ II + bone involvement. The sample included four patients (28.57%) with PRS type I, six patients (42.85%) with PRS type II, and four patients (28.57%) with PRS type III. Results: Forty-seven procedures were performed. Free-fat grafts were used in all patients. Dermal fat grafts were used in all type II patients and one type III patient (25%). Bone grafts with temporoparietal fascia flaps were performed for the treatment of all type III patients. One type III patient (25%) underwent orthognathic surgery. All patients were improved in their overall facial appearance and there were no procedure-related complications. Conclusion: Our proposed system for grading PRS severity can facilitate the choice of therapeutic approaches and with a combination of surgical techniques based on the severity of the disease partially satisfactory outcomes can be attained.

Presentación atípica de morfea tipo malformación vascular símil con síndrome Parry-Romberg secundario / Atypical presentation of vascular malformation morphea simile Parry-Romberg syndrome secondary

La Morfea es una forma de presentación clínica de la esclerodermia localizada, que generalmente se manifiesta como una placa eritematosa con un anillo lila que evoluciona a placa atrófica. Existen presentaciones atípicas de morfea en que al inicio la piel afectada simula ser una malformación vascular tipo nevus flameus o mancha en vino de oporto. El Síndrome Parry-Romberg corresponde a una morfea localizada que se caracteriza por hemiatrofia facial que afecta la funcionalidad de la cara y que puede comprometer piel, tejido muscular y hueso. El objetivo de este artículo es presentar, por medio de un caso clínico, una entidad rara de morfea que al inicio simula ser un nevusflameus. El nevus flameus adquirido es raro y requiere tiempo para que se inicien los cambios escleróticos. Se presenta el caso de un niño de 8 años que consultó por un cuadro atípico sospechoso de nevus flameus y que al cabo de 3 años evolucionó como una hemiatrofia facial progresiva. Los pacientes con diagnóstico de nevus flameus adquirido deberían monitorizarse en busca de signos de evolución a morfea y si la ubicación es en la cara siempre debe sospecharse un Síndrome Parry-Romberg para el inicio temprano de terapia y disminuir las consecuencias asociadas.

Morphea is a clinical presentation of the localized scleroderma, which usually manifests as an erythematous plaque with a purple halo that evolves to an atrophic plaque. There are atypical presentations of the morphea that, at the beginning, the affected skin simulates being a vascular malformation port wine stain simil. The Parry-Romberg Syndrome corresponds to a localized morphea that characterizes for showing a facial hemiatrophy that affects the facial functionality and may compromise the skin, muscular tissue and bones. The objective of this paper is to present, through a clinical case, a rare entity of the morphea that at the beginning simulates being a port wine stain. The acquired port wine stain is rare and requires some time to show sclerotic changes. A clinical case of an 8 years old patient that consulted for a suspicion of an atypical clinical picture of port wine stain and that after 3 years evolved in a progressive facial hemiatrophy is presented. The patients with an acquired port wine stain diagnosis should be monitored to find signs of an evolution to morphea and if it is located on the face there should always be a suspicion of a Parry Romberg syndrome in order to start earlier the therapy and reduce the associated consequences.

Selecting flap repaire hemifacial atrophy by digital technology / 中华显微外科杂志

ObjectiveTo explore clinical application of selecting flap by using of digital technique in treatment of hemifacial atrophy.MethodsSeven patients with hemifacial atrophy were selected,preoperative CT angiography was performed,deformity and flap three-dimensional reconstruction and design bone and soft tissues using Mimics 14.3 software, latissimus muscular flap or femur anterolateral flap were selected,according to tilt donor area and recipient area blood vessel diameter. Defect model were printed using rapid prototyping. ResultsHemifacial atrophy had a good postoperative shape,and the flaps survived in all the 7 cases.Follow-up 3 years,the flaps look well and the patients get expecting results. ConclusionsThe digital technique was a relatively useful tool that can assist surgeons with reconstruction of the flap,and accurate marking of the extent of the flap to be harvested.Therefore avoiding intraoperative injuries to the blood vessels to better survival of the flaps.

Parry-Romberg Syndrome with En Coup de Sabre

Parry-Romberg syndrome (PRS) is a relatively rare degenerative disorder that is poorly understood. PRS is characterized by slowly progressing atrophy affecting one side of the face, and is frequently associated with localized scleroderma, especially linear scleroderma, which is known as en coup de sabre. This is a report of the author's experiences with PRS accompanying en coup de sabre, and a review of the ongoing considerable debate associated with these two entities. Case 1 was a 37-year-old woman who had right hemifacial atrophy with unilateral en coup de sabre for seven years. Fat grafting to her atrophic lip had been conducted, and steroid injection had been performed on the indurated plaque of the forehead. Case 2 was a 29-year-old woman who had suffered from right hemifacial atrophy and bilateral en coup de sabre for 18 years. Surgical corrections such as scapular osteocutaneous flap and mandible/maxilla distraction showed unsatisfying results.

Progressive Hemifacial Atrophy Treated by Orthodontic Surgery / Oral Science International

Progressive hemifacial atrophy (PHA) is a self-limited atrophy of subcutaneous tissues (and less frequently of hard tissues) on one side of the face. It is a sporadic, slowly progressing disease whose pathogenesis is still unknown. As a rule the asymmetry caused by PHA (usually of soft tissue) is treated by volume augmentation that involves free tissue grafting or a pedicled flap transfer.We describe a rare case of hard tissue PHA. The atrophic changes occurred in the left molar tooth, maxilla and mandible and were associated with moderate soft tissue atrophy. The left side of the patient's mouth was higher on the right side, and the occlusal plane was severely inclined in spite of normal occlusion. After no alteration and no progression of the atrophy were established, the patient was treated with orthodontic surgery. To correct the inclined occlusal plane and asymmetry profile, we performed a Le Fort I and intraoral vertical ramus osteotomy. In the 3-year follow-up, there were good occlusal balance and improved symmetrical profile without relapse or recurrence of the atrophy. Thus, orthodontic surgery was effective as a first procedure to treat hard tissue atrophy that appeared with moderate soft tissue atrophy.

A Case of Severe Scleroderma en Coup de Sabre with Ipsilateral Body Atrophy

Linear scleroderma is a variant of localized scleroderma which may occur in the head, trunk and extremities, affecting variously the skin, subcutaneous tissue, muscle, cartilage and bone. Particularly, scleroderma en coup de sabre is the descriptive term denoting linear scleroderma of the frontoparietal area of the scalp and face. We report a case of severe scleroderma en coup de sabre with ipsilateral body atrophy(total hemiatrophy) who underwent multidisciplinary assessment and required both two-jaw surgery and groin free flap to correct the underlying skeletal deformities and augment the facial soft tissues. The reason for classifying this as a case of scleroderma en coup de sabre in preference to Parry-Romberg syndrome is because of the striking clinical features seen in this patient.

A Case of Parry-Romberg Syndrome with Intractable Seizure / 대한소아신경학회지

Parry-Romberg syndrome, first described in 1825 by Parry and in 1846 by Romberg, is a rare disorder characterized by a progressive hemifacial atrophy of the skin and adipose tissue and atrophy of muscle, cartilage, and underlying bony structures. It is sometimes accompanied with such complications as ophthalmologic abnormality, localized alopecia and neurologic symptoms, for example, contralateral Jacksonian epilepsy, trigeminal neuralgia, migrane and hemiplegia. The onset is slow and progressive, starting at the first or second decade of life and lasting for 2-10 years, ending with a face being "burned out". It is often associated with epilepsy but the link between these two conditions is poorly understood. In patients with progressive hemifacial atrophy, a high incidence of abnormal neuroradiologic findings in the brain has been reported. Brain MRI findings include cerebral hemiatrophy, cortical calcification, unilateral focal infarction in the corpus callosum, diffuse deep and subcortical white matter signal changes and mild cortical thickening. We report a case of Parry-Romberg syndrome in a 5-year-old boy who had a progressive Rt. facial hemiatrophy with intractable epilepsy and basal ganglia calcification from brain MRI.