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)

Serie de Casos: Síndrome de Parry Romberg, Resolución Quirúrgica / Case Series: Parry Romberg Syndrome, Surgical Resolution

INTRODUCCIÓN: El síndrome de Parry Romberg o atrofia hemifacial progresiva es una patología caracterizada por causar atrofia facial unilateral con afectación de piel, tejido celular subcutáneo y músculo. Su prevalencia mundial es de 1/700 000, tiene predominio por el sexo femenino con una proporción mujer ­ hombre de 3:2. Clínicamente se manifiesta como una esclerodermia lineal en golpe de sable, y afecta con mayor frecuencia a hemicara izquierda. El tratamiento es de tipo quirúrgico e incluye técnicas como injertos, colgajos o prótesis. CASO CLÍNICO: A continuación se presenta una serie de casos que incluye a tres pacientes que consultaron por alteraciones de la anatomía y el desarrollo facial; quienes posterior a un examen físico minucioso y a la realización de exámenes de imagen, fueron diagnosticados de síndrome de Parry Romberg. EVOLUCIÓN: En todos los casos el tratamiento fue quirúrgico; optándose por la colocación de un injerto dermograso en la zona afectada, el mismo que fue obtenido de la cavidad abdominal. La evolución clínica y la adherencia del injerto fue satisfactoria y sin complicaciones. CONCLUSIÓN: Los resultados obtenidos de la colocación de injertos dermograsos para el tratamiento de la atrofia hemifacial progresiva fueron excelentes a corto, mediano y largo plazo.(au)

BACKGROUND: Parry Romberg syndrome or progressive hemifacial atrophy is an entity characterized by unilateral facial atrophy with involvement of skin, subcutaneous tissue and muscle. Its worldwide prevalence is 1/700 000, with a female - male ratio of 3:2. Clinically it manifests as linear scleroderma and most frequently affects the left side of the face. The treatment is surgery and includes techniques such as grafts, flaps or prostheses. CASE REPORT: The following is a case series that includes three patients, who consulted for anatomic and developmental alterations of the face; who after a meticoluos physical examination and imaging tests were diagnosed with Parry Romberg syndrome. EVOLUTION: In all cases, the treatment was surgery; opting for the placement of a skin graft in the affected area, which was obtained from the abdominal cavity. The clinical evolution and adherence of the graft was satisfactory and without complications. CONCLUSION: The results obtained from the placement of skin graft as the treatment of progressive hemifacial atrophy were excellent in short, medium and long term.(au)

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>

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.

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.

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.

Validación de la prueba de Romberg Modificada para la determinación del tiempo de propiocepción inconciente en adultos sanos / Validation of a Modified Romberg's Test to determine normal adult lower limb unconscious proprioception times

Introducción La propiocepción es la capacidad que tiene el cuerpo para detectar el movimiento y la posición articular, lo cual tiene grandes implicaciones en el control neuromuscular. Actualmente no hay una prueba clínica estandarizada que permita determinar objetivamente la normalidad en su evaluación en la población sana con un patrón de marcha maduro. En el año 2012 se diseñó un signo clínico denominado prueba de Romberg modificada, que permite caracterizar los valores propioceptivos de la población normal en los miembros inferiores. Materiales y métodos Estudio de cohorte transversal ciego de 163 pacientes. Se excluyeron pacientes con algún tipo de lesión osteoarticular o neuropatía periférica en los miembros inferiores. Estos individuos se sometieron a la prueba de Romberg modificada, registrando su valor en segundos en el miembro inferior derecho e izquierdo, y comparándolas con la edad, lateralidad y el sexo. Resultados Al estratificar los sujetos evaluados por grupos etarios, se observó una relación inversamente proporcional entre la prueba de Romberg modificada y la edad. La lateralidad y el sexo no presentaron una diferencia significativa (p=0,53).. Discusión La prueba de Romberg modificada, es una prueba útil para la determinación de la propiocepción normal en los adultos, la cual disminuye con la edad sin presentar diferencias con respecto a la lateralidad o el sexo del individuo. Nivel de evidencia IV

Background Proprioception is considered the ability of the body to detect movement and joint position, and has great implications in neuromuscular control. There are currently no standardised clinical tests to objectively determine how much should be considered normal in its evaluation in the healthy population with a mature gait pattern. In 2012, a clinical sign called the modified Romberg test was designed to determine the proprioceptive values in the lower limbs of the normal population. Materials and methods A cross-sectional blind study was conducted on a cohort of patients with no history of osteoarticular injury in the lower limbs and no peripheral neuropathy. The subjects underwent the modified Romberg test, with a record made of its value in seconds in the lower right and left limbs, and comparing them with age, laterality, and gender. Results The study included 163 patients. On stratifying the subjects evaluated according to age groups, a tendency to decrease the modified Romberg test result with increased age was observed. According to the analysis, it can be observed that the proprioception of a limb does not have a significant difference (P=.53) with respect to the contralateral limb (laterality) or gender, but there is with respect to age. Discussion The modified Romberg test result of 20seconds is a useful tool for the determination of normal proprioception in adults. This value decreases with age, without showing differences as regards the laterality or gender of the subject. Evidence Level IV.

Validación de la prueba de Romberg Modificada para la determinación del tiempo de propiocepción inconciente en adultos sanos / Validation of a Modified Romberg's Test to determine normal adult lower limb unconscious proprioception times

Introducción La propiocepción es la capacidad que tiene el cuerpo para detectar el movimiento y la posición articular, lo cual tiene grandes implicaciones en el control neuromuscular. Actualmente no hay una prueba clínica estandarizada que permita determinar objetivamente la normalidad en su evaluación en la población sana con un patrón de marcha maduro. En el año 2012 se diseñó un signo clínico denominado prueba de Romberg modificada, que permite caracterizar los valores propioceptivos de la población normal en los miembros inferiores. Materiales y métodos Estudio de cohorte transversal ciego de 163 pacientes. Se excluyeron pacientes con algún tipo de lesión osteoarticular o neuropatía periférica en los miembros inferiores. Estos individuos se sometieron a la prueba de Romberg modificada, registrando su valor en segundos en el miembro inferior derecho e izquierdo, y comparándolas con la edad, lateralidad y el sexo. Resultados Al estratificar los sujetos evaluados por grupos etarios, se observó una relación inversamente proporcional entre la prueba de Romberg modificada y la edad. La lateralidad y el sexo no presentaron una diferencia significativa (p=0,53).. Discusión La prueba de Romberg modificada, es una prueba útil para la determinación de la propiocepción normal en los adultos, la cual disminuye con la edad sin presentar diferencias con respecto a la lateralidad o el sexo del individuo. Nivel de evidencia IV

Background Proprioception is considered the ability of the body to detect movement and joint position, and has great implications in neuromuscular control. There are currently no standardised clinical tests to objectively determine how much should be considered normal in its evaluation in the healthy population with a mature gait pattern. In 2012, a clinical sign called the modified Romberg test was designed to determine the proprioceptive values in the lower limbs of the normal population. Materials and methods A cross-sectional blind study was conducted on a cohort of patients with no history of osteoarticular injury in the lower limbs and no peripheral neuropathy. The subjects underwent the modified Romberg test, with a record made of its value in seconds in the lower right and left limbs, and comparing them with age, laterality, and gender. Results The study included 163 patients. On stratifying the subjects evaluated according to age groups, a tendency to decrease the modified Romberg test result with increased age was observed. According to the analysis, it can be observed that the proprioception of a limb does not have a significant difference (P=.53) with respect to the contralateral limb (laterality) or gender, but there is with respect to age. Discussion The modified Romberg test result of 20seconds is a useful tool for the determination of normal proprioception in adults. This value decreases with age, without showing differences as regards the laterality or gender of the subject. Evidence Level IV.

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.

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.