Reconstrução de hemiatrofia facial com hidroxiapatita de cálcio: relato de uma técnica segura e minimamente invasiva / Reconstruction of facial hemiatrophy using calcium hydroxylapatite filler: report of a safe and minimally invasive technique

Introdução: a hemiatrofia facial progressiva, também conhecida como síndrome de Parry Romberg, é uma forma rara de esclerodermia, cujo manejo de sequelas é desafiador. Objetivo: diante desse desafio, o objetivo deste estudo foi avaliar pela primeira vez na literatura a eficácia do preenchimento da hidroxiapatita de cálcio (CaHa) na reconstrução da mandíbula de um paciente com síndrome de Parry Romberg. Relato do caso: paciente do sexo feminino, 15 anos, com atrofia progressiva do lado direito da face por síndrome de Parry Romberg. Após o controle da doença, a principal queixa da paciente era assimetria facial, principalmente na região mandibular. Discussão: a CaHa é um preenchedor injetável biocompatível, que é gradualmente reabsorvido e substituído por estroma fibrovascular, formado principalmente por novo colágeno, em um processo que ocorre sem qualquer reação imunológica. Este perfil de segurança torna a CaHa uma boa escolha para a correção de sequelas de esclerodermia estável. Conclusão: este relato permite concluir que a biocompatibilidade do preenchimento de CaHa e a adequação para correção da mandíbula também são aplicáveis em procedimentos reconstrutivos para esclerodermia estável, de forma segura e minimamente invasiva, com ótimos resultados estéticos. O procedimento deve ser programado caso a caso, e um acompanhamento regular também é recomendado.

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.

Facial depression correction by using free anterolateral thigh adipofascial flap and human acellular dermal matrix / 中华医学美学美容杂志

Objective:To investigate the clinical effect of facial depression correction by using free anterolateral thigh adipofascial flap and human acellular dermal matrix.Methods:Nineteen facial depression patients (3 males and 16 females), age ranges from 16 to 56 years (average age: 29.1±10.37 years), caused by different reasons were selected in this research from Jan. 2008 to Dec. 2020. In the stage I operation, anterolateral thigh adipofascial flap was designed according to facial depression area and harvested to fill in the depression with vascular anastomosis; In the stage II operation, the human acellular dermal matrix was used to fill in the remained depression edge according to facial subunit.Results:All free anterolateral thigh adipofascial flap survived well. All patients revealed fascial subunit tissue atrophy of different degree in infraorbital, nasolabial sulcus and temporal areas which were repaired by using cellular dermal matrix and obtained satisfying effect during 1 to 8 years of follow-up with no obvious rejection. 18 of 19 patients showed flap hypertrophy and corrected by liposuction. All patients recovered well after 1 to 8 years of follow-up.Conclusions:Humana cellular dermal matrix could make up the limitations of treating fascial depression deformity when using anterolateral thigh adipofascial flap. The combination method of above approaches could obtain satisfying clinical effect, which is worthy of clinical promotion.

Adult linear scleroderma with ptosis: a case report and literature review / 中华神经科杂志

Objective:To discuss the clinical features, diagnosis and treatment of linear scleroderma (LS).Methods:A case of LS diagnosed in the Second Hospital of Shandong University in October 20, 2020, was reported and the clinical features and pathological documentation of the disease reported in the literature were reviewed.Results:A 24-year-old woman presented cicatricial alopecia on the left frontoparietal area and facial atrophy for about 10 years. Two years before, she began to suffer ptosis and neurological complaints. Clinical features of different stages of the disease are presented. All 15 patients reported in the literature were analyzed, with a median of 22 years and a male to female ratio of 9∶6. There were 4 cases of linear scleroderma with ipsilateral drooping eyelids and lateral contraction, 3 cases of linear scleroderma with demyelinating lesions, combined with lateral contraction, 3 cases of linear scleroderma combined with lateral atrophy, and 1 case of linear scleroderma with ipsilateral facial spasm. Two cases were with the chest sclerosing spot. Two cases of linear scleroderma were with epileptic seizure and white matter demyelination lesion. Six cases were treated with hormone, 2 cases were treated with methotrexate. One case was treated with both hormone and methotrexate. One case was treated with botulinum toxin. Three cases were treated with surgical correction of eyelid ptosis. One case was treated with ultraviolet A1 radiation phototherapy and 1 case was treated with vitamin therapy.Conclusions:Patients with scleroderma may have ipsilateral facial atrophy, blepharoptosis and facial spasm. Some patients involving the nervous system may have epilepsy and myelitis. And demyelinating lesions can be seen in magnetic resonance imaging. Localized scleroderma may develop into systemic scleroderma. Therefore, it is recommended to combine immunosuppressants as soon as possible to control the development of the disease if necessary.

Síndrome de Parry-Romberg: hemiatrofia facial progresiva. Reporte de caso / Parry-Romberg Syndrome: progressive facial hemiatrophy case report

INTRODUCCIÓN: el Síndrome de Parry-Romberg es un trastorno neurocutáneo, muy raro, caracterizado por hemiatrofia facial progresiva, su primer signo clínico es la asimetría facial, se presenta frecuentemente de manera simultánea con la esclerodermia lineal. PRESENTACIÓN DE CASO: femenina de 54 años de edad, con antecedente de hemiatrofia facial y alteración oftalmológica izquierda desde los 14 años. DISCUSIÓN: el Síndrome de Parry-Romberg inicia entre las primeras décadas de la vida con hiperpigmentación, atrofia de la dermis, grasa subcutánea, músculo y huesos subyacentes. La atrofia afecta la hemicara siguiendo la distribución del nervio trigémino y sus ramas; este caso coincidió con la edad de inicio, la lesión se limitó a la hemicara izquierda. CONCLUSIÓN: debe tenerse presente como diagnóstico diferencial en pacientes con alteración en la simetría facial de larga evolución sin causa aparente, el diagnóstico temprano evita las complicaciones graves, se recomienda un manejo multidisciplinario para mejorar la calidad de vida de los pacientes.

INTRODUCTION: parry-Romberg Syndrome is a neurocutaneous disorder, very rare and characterized by progressive facial hemiatrophy, the first clinical sign is facial asymmetry. SPR occurs simultaneously with high frequency with linear scleroderma. CASE PRESENTATION: a 54-year-old female, with a history of left facial hemiatrophy since she was 14 years old, presented a left ophthalmological alteration. DISCUSSION: Parry-Romberg Syndrome begins in the first decades of life with hyperpigmentation, atrophy of the dermis, subcutaneous fat, muscle and underlying bones. Atrophic affects the hemicara with distribution of the trigeminal nerve and its branches; this case coincided with the age of onset and the lesion was limited to the left hemicra. CONCLUSION: it should be borne in mind as a differential diagnosis in patients with long-standing facial symmetry alteration without apparent cause, early diagnosis avoids serious complications, multidisciplinary management is recommended to improve the quality of life of patients.

Morfeas con manifestaciones cefálicas / Morpheas with cephalic manifestations

Introducción: La hemiatrofia facial progresiva (HFP) o síndrome de Parry-Romberg y la morfea en golpe de sable (MGS) forman parte de las morfeas lineales cefálicas. Son enfermedades inflamatorias crónicas de la piel y tejidos subyacentes, que se caracterizan por esclerosis y atrofia cutánea. Objetivos: Describir las características clínicas, manifestaciones asociadas, histología, laboratorio, estudios complementarios y tratamientos instaurados. Diseño: Estudio retrospectivo descriptivo. Materiales y métodos: Se revisaron las historias clínicas de pacientes con morfea evaluados en el Sector Colagenopatías desde julio de 2010 hasta diciembre de 2016. Resultados: De 56 pacientes, 11 cumplieron con los criterios de inclusión, 7 con diagnóstico de HFP, 2 con MGS y 2 con ambas patologías. El 64% fueron mujeres. Las manifestaciones extracutáneas se presentaron en el 64% de los casos. El tratamiento utilizado en todos los pacientes fue el metotrexato, asociado o no, al uso de corticosteroides sistémicos. Conclusiones: La mayoría de nuestros resultados concuerdan con la bibliografía consultada, excepto las manifestaciones asociadas. Destacamos el tratamiento asociado de metotrexato y pulsos de corticosteroides intravenosos con resultados satisfactorios y bien tolerado. (AU)

Introduction: Progressive facial hemiatrophy (PFH) or Parry-Romberg Syndrome and morphea en coup de sabre are cephalic linear morpheas. They are chronics inflammatories diseases of the skin and underlying tissues, characterized by cutaneous atrophy and sclerosis. Objectives: To describe clinical features, associated extracutaneous manifestations, histological and laboratory findings, imaging and diagnostic modalities and treatments established in patients with diagnosis of HFP, MGS, or both, evaluated in our Department. Design: Retrospective descriptive study. Materials and methods: We included medical histories of patients diagnosed with morphea evaluated in Collagenopathy Sector from July 2010 up to December 2016. Results: Of 56 patients, 11 met the inclusion criteria, 7 with diagnosis of PFH, 2 with morphea en coup de sabre and 2 with both pathologies. 64% were women. 64% showed extracutaneous manifestations. The treatment used in all of the patients was methotrexate, associated or not, with the use of systemic corticosteroids. Conclusions: Most of our results agree with the bibliography consulted, with the exception of the associated manifestations. We emphasize the associated treatment of methotrexate and intravenous corticosteroid pulses with satisfactory results and well tolerated. (AU)

Changes of facial volume maintenance rate after single autologous fat grafted for repaired progressive facial hemiatrophy / 中华医学美学美容杂志

Objective To measure the change of facial volume maintenance rate after autologous fat grafted for repaired progressive facial hemiatrophy by using three-dimensional digital technology.Methods 3D scanner was used to acquire facial data in 10 patients with progressive facial hemiatrophy before operation;Mimics 17.0 software was used to reconstruce patients' facial 3D model and to calculate the volume of facial tissue defect;autologous fat was grafted to repair facial deformity.The facial volume maintenance rate was calculated in all the patients 3 months and 6 months after operation.Results We had performed facial 3D data acquisition and facial repaired with autologus fat grafted in 10 patients;patients' facial morphology was improved.The mean facial volume maintenance rate was (35.80±3.44)％ in 3 months and (27.82±3.80)％ 6 months after surgery.Conclusions The mean facial volume maintenance rate in postoperative 3 months is inferior to that in 6 months in single autologous fat grafted for repairing progressive facial hemiatrophy.

Lipofilling no tratamento de casos leves de hemiatrofia facial / Lipofilling for the treatment of mild cases of Facial Hemiatrophy

A hemiatrofia facial ou síndrome de Parry-Romberg é caracterizada por atrofia progressiva dos tecidos da hemiface, acometendo a pele e o subcutâneo. Suas manifestações clínicas são muito variadas. As estratégias terapêuticas de eleição para o tratamento da síndrome ainda não foram completamente estabelecidas. Os autores descrevem um caso de hemiatrofia facial leve, em que o paciente foi submetido a lipofilling (lipoenxertia autóloga), evoluindo com resultado satisfatório. As alternativas de tratamento, suas vantagens e desvantagens, são amplamente discutidas no estudo, sendo que a lipoenxertia autóloga desponta como excelente opção terapêutica, por sua segurança, baixo custo e reprodutibilidade.

Facial hemiatrophy, or Parry-Romberg syndrome, is characterized by the progressive atrophy of the tissues of half of the face, affecting skin and subcutaneous tissue. Its clinical manifestations are very varied. Preferred therapeutic strategies for the treatment of the syndrome were not yet completely established. The authors describe a case of mild facial hemiatrophy, in which the patient underwent lipofilling (autologous fat grafting), progressing with satisfactory result. Treatment alternatives, its advantages and disadvantages are widely discussed in the study, with fat grafting emerging as an excellent therapeutic option due to its safety, low cost and replicability.

Parry-Romberg Syndrome Augmented by Hyaluronic Acid Filler

Parry Romberg Syndrome (PRS), also known as idiopathic progressive hemifacial atrophy, is a rare neurocutaneous disorder characterized by loss of skin and subcutaneous fat of face, muscles, and bones causing unilateral atrophy. Most patients require only soft tissue augmentation although syndrome has varying grades of severity. In the majority of reported cases, it has been treated with surgical flap or autologous fat transplantation. However, these treatments need complicated surgical skills which take a lot of time and cost. Herein we report the first case of PRS augmented by hyaluronic acid (HA) filler in a 42-year-old female patient to suggest that HA filler could be a safe, simple, and even rational economic alternative to surgical treatment.

Effect of autologous fat transplantation and anastomotic vascular anterolateral fascial fat flap transplantation for PHFA facial soft tissue defect / 中国医师杂志

Objective To evaluate the efficacy and value of autologous fat transplantation and anesthesia of the anterolateral femoral fascial flap (ALT) in the clinical treatment of progressive hemifacial atrophy (PFHA).Methods From April 2014 to June 2017,42 patients with progressive hemi-facial wretched malformations who were treated in our hospital were randomly divided into observation group (using autologous fat transplantation).And 21 cases with anastomotic anterolateral femoral fascial fat flap transplantation were enrolled in the control group.Then the therapeutic effect was compared between the two groups.Results The rate of facial asymmetry in the observation group and the control group were (10.96 ±1.35) ％ and (11.98 ± 1.37) ％ in the 6 months after operation,significantly lower than that before operation [(20.13 ± 1.59)％ and (24.65 ± 1.81)％],and the difference was statistically significant (P ＜0.05);While there was no significant difference between the two groups (P ＞ 0.05).The volume of facial defect [(14.16 ±2.98)ml and (8.95 ±4.31)ml] in the observation group and the control group 6 months after operation was significantly lower than that before the operation [(43.17 ± 12.15)ml and (46.83 ±16.21)ml],with statistically significantthe difference (P ＜ 0.05);While the difference between the two groups was not statistically significant (P ＞ 0.05).The operation time and hospital days in the observation group were significantly lower than in the control group,and the number of operation times was more than that of the control group (P ＜ 0.05).The volume retention rate of the actual transplanted tissue in the observation group was lower than that of the control group [(28.12 ± 6.11) ％ vs (77.21 ± 5.33) ％],with statistically significant difference (P ＜ 0.05).There was no significant difference in the maximum and average 3-D deviation,as well as the the postoperative complications between the two groups at 6 months after operation (P ＞ 0.05).In the observation group,the satisfaction rate of the patients was significantly better than that of the control group (P ＜ 0.05),but there was no significant difference between the satisfaction degree of doctors and the third party (P ＞ 0.05).Conclusions Autologous fat granule transplantation in the clinical treatment of severe PFHA patients can effectively restore the patient's facial symmetry.At the same time,it has the characteristics of simple operation,easy operation,rich material,quick recovery of surgical trauma and no rejection.It is worth widely spreading in clinical practice.

Forehead reconstruction with a custom-made three-dimensional titanium implant in a Parry-Romberg syndrome patient

Parry-Romberg syndrome is a rare neurocutaneous syndrome characterized by progressive shrinkage and degeneration of the tissues usually on only one side of the face. It is usually difficult to restore the facial contour due to skin tightness. In this case report, we report a forehead reconstruction with custom-made three-dimensional (3D) titanium implant of a Parry-Romberg syndrome patient who was treated with multiple fat grafts but had limited effect. A 36-year-old man presented with hemifacial atrophy. The disease progressed from 5 to 16 years old. The patient had alopecia on frontal scalp and received a surgery using tissue expander. The alopecia lesion was covered by expanded scalp flap done 22 years ago. Also, he was treated with fat grafts on depressed forehead 17 years ago. However, it did not work sufficiently, and there was noted depressed forehead. We planned to make 3D titanium implant to cover the depressed area (from the superior orbital rim to the vertex). During the operation, we confirmed that the custom-made 3D implant accurately fit for the depressed area without any dead spaces. Previously depressed forehead and glabella were elevated, and the forehead contour was improved cosmetically. A custom-made 3D titanium implant is widely used for skull reconstruction and bring good results. In our case, the depressed forehead of a Parry-Romberg syndrome patient was improved by a 3D titanium implant.

Síndrome de Parry Romberg y epilepsia refractaria al tratamiento / Parry Romberg Syndrome And Pharmacoresistant Epilepsy

Resumen El Síndrome de Parry Romberg es una enfermedad idiopática, caracterizado por la atrofia de tejido celular subcutáneo. Su signo clínico principal es la hemiatrofia facial progresiva con inclusión de piel, tejido subcutáneo, nervios, músculo y/o hueso. Se han propuesto diversas teorías, entre ellas el posible origen hereditario y genético de esta patología, debido a que se ha manifestado algunas veces en miembros de una misma familia. Se presenta un caso de una paciente con síndrome de Parry Romberg de inicio tardío, que cursó con atrofia de hemicara derecha desde los 20 años de edad asociada a epilepsia refractaria al tratamiento y con antecedente patológico familiar de un hermano que presenta igualmente atrofia en hemicara derecha y una marcada esclerodermia en golpe de sable.

Abstract Parry Romberg syndrome is an idiopathic disease, characterized by subcutaneous tissue atrophy. Its main clinical sign is progressive facial hemiatrophy including: skin, subcutaneous tissue, nerves, muscle and/or bone. Several theories have been mentioned, among them the possible hereditary and genetic origin of this disorder, as the manifestation of this disease sometimes is seen in members of the same family. We present a case: a patient with late presentation Parry Romberg, with right side face atrophy associated with refractory epilepsy and family history of a brother who presents right side face atrophy as well and a marked scleroderma in coup of saber.

Implicaciones anestésicas del Síndrome de Parry-Romberg; reporte de un caso / Anesthetic implications of Parry Romberg Syndrome: A case report

Introduction: Parry-Romberg Syndrome is a rare degenerative disease characterized by unilateral atrophy affecting the skin, connective tissue, muscle and bone. The end result is facial asymmetry associated with other skin, dental, visual, cardiovascular, and neurological disorders. Clinical findings, diagnostic evaluation and interventions: The case of a patient with Parry-Romberg Syndrome programmed for frontonasal flap remodeling is discussed. The patient's history includes trigeminal neuralgia, epilepsy, and two previous surgical interventions. Uneventful endotracheal intubation with the Glideoscope® video laryngoscope was performed, upon adequate pre-oxygenation followed by anesthetic induction. Conclusion: The phenotypical characteristics of Parry Romberg Syndrome are severe facial hemiatrophy and craniofacial anomalies that require careful preoperative evaluation and management of a potentially difficult airway. Consequently, the use of video laryngoscopes is a first-line approach. Due to the syndrome's associated disorders, it is essential to maintain hemodynamic stability and prevent any potential seizures.

Introducción: El Síndrome de Parry-Romberg es una enfermedad degenerativa poco común, caracterizada por una atrofia unilateral que afecta la piel, el tejido conjuntivo, el músculo y el hueso. El resultado final es una asimetría facial cursando con otras alteraciones cutáneas, dentales, oculares, cardiovasculares y neurológicas. Hallazgos clínicos, evaluación diagnóstica e intervenciones: Presentamos un caso de un paciente con Síndrome de Parry-Romberg programado para remodelación de colgajo frontonasal. Entre sus antecedentes destacan neuralgia del trigémino, epilepsia y dos intervenciones quirúrgicas previas. Tras una adecuada preoxigenación y posterior inducción anestésica, se realiza una intubación endotraqueal sin incidencias mediante el videolaringoscopio Glideoscope®. Conclusión: El Síndrome de Parry Romberg presenta como características fenotípicas hemiatrofia facial grave y anomalías craneofaciales, que requieren una cuidadosa evaluación preoperatoria y el manejo de una vía aérea potencialmente difícil. Es por esto que los videolaringoscopios resultan una alternativa de primera línea. Debido a sus trastornos asociados, es esencial mantener la estabilidad hemodinámica y la prevención de posibles crisis convulsivas.

Treatment of hemifacial spasm in patient with hemifacial atrophy using combination therapy (ultrasound therapy and TENS): a case report

A 19-year-old male patient presented with facial hemi-atrophy with unilateral spasms of the masseter and temporalis muscles. Ultrasound therapy and Transcutaneous Electric Nerve Stimulation therapy, known as combination therapy, were given on alternate days for 2 weeks. At the end of 2 weeks of combination therapy the patient reported a drastic reduction in the number of episodes of muscle spasm. The visual analog scale score for tenderness of the masseter and temporalis was also markedly reduced. No one has previously used combination therapy for the treatment of facial hemi-atrophy with hemi-facial spasms. The encouraging results of the combination therapy has prompted us to document this study.

Parry-Romberg Syndrome Associated with Hypothyroidism

PURPOSE: We report a case of Parry-Romberg syndrome associated with right enophthalmos, hypotropia, up-gaze limitation, en coup the sabre, morphea, and hypothyroidism. CASE SUMMARY: A 41-year-old woman presented with slowly progressive right enophthalmos that started 5 years previous. With a Hertel exophthalmometer, the right eye measured 10 mm, and the left eye 13 mm. Right enophthalmos and atrophy of subcutaneous tissues of right side orbit were observed. Axial scan of computerized tomography shows atrophy of right side orbital subcutaneous tissues without bony abnormality. At primary gaze, 4 prism diopter hypotropia was observed in the right eye. Up-gaze was limited in both eyes. Visual acuity was 1.0 in both eyes. No specific sign was found in her anterior segment. The patient had experienced right side alopecia 20 years prior and had undergone biopsy, which proved morphea. Linear en coup de sabre morphea was found on the right forehead and scalp. Hypothyroidism due to Hashimoto's thyroiditis was diagnosed based on a blood test, which also showed positive antinuclear antibody. Thus, the patient was diagnosed with Parry-Romberg syndrome associated with hypothyroidism. CONCLUSIONS: Clinicians need to consider the rare disease Parry-Romberg syndrome when a patient with ophthalmologic symptoms such as enopthlamos or eye movement disorders is accompanied by dermatologic symptoms such as linear en coup de sabre morphea or alopecia.

A case report of Dyke-Davidoff-Masson syndrome / 基础医学与临床

Dyke-Davidoff-Masson syndrome ( DDMS) is a rare epilepsy syndrome which is characterized by cerebral hemiatrophy with ipsilateral compensatory skull changes and contralateral hemiplegia recurrent .Here we reported a case of which the curative effect turned out to be unsatisfied after conservative treatment , physical examination re-vealed mental retardation , facial asymmetry , mild right hemiparesis , MRI scan of the head showed left cerebral hemiatrophy , calvaria thickening , PET-CT showed less functional left cerebral hemisphere .The patient finally un-derwent multi-lobe disconnection to relieve recurrent seizure , whose seizure was well controlled in the following up of one year .

Parry-Romberg syndrome in a pediatric patient: a case report / Síndrome de Parry-Romberg en paciente pediátrico: informe de caso

The Parry-Romberg syndrome is a rare degenerative disease of unknown etiology that has dental implications. It is characterized by a progressive hemifacial atrophy that appears in the early stages of life. It causes aesthetic, functional and psychological alterations, and has social implications for the patient. There is no definitive treatment for the Parry-Romberg syndrome. Systemic and immune alterations produce oral and maxillofacial manifestations, which need to be managed by specialized dental professionals. The aim of this paper is to do a literature review of the Parry-Romberg syndrome and describe the oral and clinical characteristics of this condition in a 12-year-old male pediatric patient, who had a history of neurological disorders and facial asymmetry on the left side. Dentists require an adequate knowledge of the clinical and dental characteristics of this syndrome. With early diagnosis and appropriate surgical and orthodontic treatment at an early age, they can improve the quality of life of patients and minimize invasive long-term effects...

El síndrome de Parry-Romberg es una rara enfermedad degenerativa de etiología desconocida con implicaciones odontológicas. Se caracteriza por una atrofia progresiva que aparece a temprana edad, generalmente hemifacial. Tiene alteraciones estéticas, funcionales, psíquicas y sociales, no existiendo un tratamiento definitivo. Las manifestaciones orales y maxilofaciales de este síndrome son parte de una afectación sistémica e inmunológica, lo cual resalta la necesidad de su conocimiento y manejo por parte de los profesionales de la odontología. El objetivo de este reporte es describir las características clínicas y orales del Síndrome de Parry-Romberg en un paciente pediátrico masculino de 12 años de edad con antecedentes neurológicos que presenta una asimetría facial izquierda, presentado en conjunto una revisión bibliográfica de este síndrome. Las características clínicas y dentales de este síndrome como parte de una afectación sistémica requieren de un adecuado conocimiento por parte del profesional dental, el cual con un diagnóstico precoz y un adecuado tratamiento quirúrgico y ortodontico a temprana edad para mejorar la calidad de vida del paciente y minimizar los efectos invasivos a largo plazo...

Caracterização miofuncional orofacial na síndrome de Parry-Romberg / Orofacial myofunctional characterization in Parry-Romberg syndrome

Objetivo Caracterizar os aspectos miofuncionais orofaciais de pacientes acometidos pela síndrome de Parry-Romberg, por meio de protocolos clínicos padronizados e da Eletromiografia de Superfície (EMGs) dos músculos mastigatórios. Métodos A amostra foi composta por quatro pacientes com síndrome de Parry-Romberg e quatro indivíduos normais, separados em Grupo Pesquisa (GP) e Grupo Controle (GC), respectivamente, pareados por gênero e idade. Os grupos foram comparados em relação ao desempenho na avaliação clínica fonoaudiológica - Protocolo de Avaliação Miofuncional Orofacial com Escores Expandido (AMIOFE-E), registro das medidas de antropometria facial e de movimentos mandibulares e na avaliação instrumental - Eletromiografia de Superfície (EMGs) dos músculos mastigatórios. Para todas as comparações, foi utilizado o nível de significância de 5%. Resultados Observou-se diferença significativa entre os grupos nas variáveis postura/posição e escore total do AMIOFE-E. Além disso, a análise das variáveis categóricas do AMIOFE-E indicou diferença significativa entre os grupos para palato - altura e largura - e comportamento da língua na deglutição e função mastigatória. A análise das medidas antropométricas indicou diferença significativa entre os grupos somente para e lateralidade mandibular à direita. Não foram observadas diferenças para os dados eletromiográficos. Conclusão O presente estudo é o primeiro a avaliar o Sistema Miofuncional Orofacial de indivíduos acometidos pela síndrome de Parry-Romberg, por meio de protocolos padronizados. Os resultados indicam que esses indivíduos apresentam alterações, principalmente quanto à mobilidade mandibular e mobilidade de língua, na deglutição e na função de mastigação. .

Purpose To characterize the orofacial miofunctional aspects of patients with Parry-Romberg syndrome, using standardized clinical protocols and Surface Electromyography (sEMG) of the masticatory muscles. Methods Participants were four patients with Parry-Romberg syndrome and four healthy individuals, paired by age and gender, divided in two groups: Study Group (SG) and Control Group (CG), respectively. The groups were compared regarding performance during clinical examination - Orofacial Myofunctional Evaluation with Expanded Scores (OMES-E) and record of facial anthropometric measures and of jaw movements, and during an instrumental evaluation - Surface Electromyography (sEMG) of the masticatory muscles. Results Statistically significant differences between the groups were observed for the following variables: 1) numerical: mandibular lateral movement to the right, OMES-E posture/position and total score, 2) categorical: palate, behavior of the tongue during swallowing and chewing function. At sEMG no statistically significant differences were observed. Conclusion The present study is the first to evaluate the orofacial myofunctional system of individuals with Parry-Romberg syndrome using standardized protocols. Results indicate that these individuals present alterations specially when considering mandibular movements, tongue mobility during swallowing and chewing function. .

Síndrome Parry-Romberg / Parry-Romberg syndrome

El síndrome de Parry-Romberg, también conocido como hemiatrofia facial progresiva, es un síndrome neurocutáneo raro de etiología desconocida. Las principales características son la atrofia de los tejidos blandos, y algunas veces del hueso, en una mitad de la cara o parte frontal de la cabeza, sin debilitamiento facial. Estudios recientes plantean la posibilidad hereditaria de esta patología, ya que se recogen afectados en una misma familia. En este trabajo se describe un sujeto afectado que viene con su hija con una asimetría facial. Es importante el seguimiento de estos pacientes y su familia para realizar un diagnóstico que permita actuar precozmente.

Parry-Romberg syndrome, also known as progressive hemifacial atrophy, is a weird neurocutaneous syndrome of unknown etiology. The main characteristics are atrophy of soft tissues, and sometimes the bone, in one half of the face or the frontal side of the head, without facial weakening. Current studies consider this pathology likely hereditary, since affected people are known belonging to a same family. This work describes an affected individual coming with his daughter also with a facial asymmetry. It is important the following of these patients and their families to complete a diagnosis permitting to precautiously take measures.

Alterações do sistema miofuncional orofacial na síndrome de Parry-Romberg: revisão crítica da literatura / Alterations of the myofunctional orofacial system in the Parry-Romberg syndrome: a critical literature review

INTRODUÇÃO Esta revisão qualitativa da literatura analisou publicações científicas internacionais sobre possíveis alterações miofuncionais orofaciais em pacientes acometidos pela Síndrome de Parry-Romberg, por meio da base de dados PubMed. MÉTODOS: O levantamento realizado limitou-se a seres humanos, de qualquer faixa etária, no idioma inglês, entre os anos 2002 e 2012. As publicações sem acesso completo, repetidas por sobreposição das palavras-chave, revisões de literatura, cartas ao editor e as não relacionadas diretamente ao tema foram excluídas. RESULTADOS: Foram identificados 719 estudos, sendo 21 dentro dos critérios estabelecidos. Com base nos estudos selecionados, pacientes acometidos pela Síndrome de Parry-Romberg podem apresentar alterações dos tecidos mole e duro, tais como atrofia dos músculos esternocleidomastoideo, masseter e pterigoideos; atrofia na região da bochecha e depressão da prega nasolabial; desvio dos lábios e nariz; atrofia unilateral da língua; atrofia do ângulo da boca; reabsorção progressiva do osso da maxila e da mandíbula; atrofia do arco zigomático, do osso frontal e assimetria facial; desenvolvimento atrófico das raízes ou reabsorção patológica dos números de dentes permanentes; redução da mandíbula e erupção atrasada dos dentes superiores e inferiores. CONCLUSÃO: Apesar do crescente interesse pelo diagnóstico e pela descrição sintomatológica de indivíduos com Síndrome de Parry-Romberg, a escassez de publicações que abordem tratamentos funcionais e interdisciplinares é evidente. Verifica-se a necessidade da realização de estudos mais específicos que visem à melhoria da qualidade de vida desses pacientes.

INTRODUCTION This qualitative literature review analyzed international scientific publications on possible orofacial myofunctional alterations in patients with Parry-Romberg syndrome by using PubMed. METHODS: The survey was conducted in English, between 2002 and 2012, and was limited to human beings of any age. Publications without full access, duplicated by overlapping keywords, literature reviews, letters to the editor, and those not directly related to the research topic were excluded. RESULTS: We identified 719 studies, of which 21 were within the established criteria. Based on the selected studies, patients with Parry-Romberg syndrome may show changes in soft and hard tissues such as atrophy of the sternocleidomastoid, masseter, and pterygoid muscles; atrophy in the cheek region and depression of the nasolabial fold; deviation of the lips and nose; unilateral tongue atrophy; atrophy of the mouth angle; progressive resorption of the maxilla and mandible bone; atrophy of the zygomatic arch and frontal bone, and facial asymmetry; atrophic root development or pathological resorption of permanent tooth numbers; and jaw reduction and delayed eruption of the upper and lower teeth. CONCLUSION: Despite the growing interest in the diagnosis and symptomatic description of individuals with Parry-Romberg syndrome, publications that address functional and interdisciplinary treatments are scarce. Therefore, specific studies aimed at improving the quality of life of these patients are needed.

Dyke-davidoff-masson syndrome- a rare cause of refractory epilepsy.

DDMS is a rare syndrome characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation. The characteristic radiologic features are cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. Dyke-Davidoff- Masson Syndrome (DDMS) is one among the syndromes associated with refractory epilepsy. We report a case of DDMS in an 18 year old female who presented with seizures, hemiparesis of the right side and mental retardation. Computed tomography on this patient assisted in making a diagnosis of DDMS.