Hiperplasia estromal pseudoangiomatosa (HEP) de mama: a propósito de un caso / Pseudoangiomatous stromal hyperplasia (PASH) of the breast: about a case

La hiperplasia pseudoangiomatosa estromal de la mama es una patología benigna de rara aparición en mujeres, que hoy en día sigue generando incertidumbre en cuanto a su manifestación y al tratamiento definitivo. Nuestro objetivo será detallar el manejo y los resultados obtenidos luego de tratar a una paciente con esta patología atendida en hospital público durante la pandemia, que presentó gigantomastia bilateral a expensas de crecimiento y simetrización de mama contralateral afectada por HEP durante su estado gravídico.

Pseudoangimatous stromal hyperplasia of the breast, is a pathology of rare appearance, in women, which today continues to generate uncertainty regarding its manifestation and definitive treatment. Our objective will be to detail the management and results obtained after treating a patient with this pathology in a public hospital during a pandemic. who presented bilateral gigantomastia at the expense of growth and symmetrization of the contralateral breast affected by HEP during her pregnancy.

Angiomatosis intraorbitaria / intraorbital angiomatosis

Introducción: Los angiomas cavernosos son entidades frecuentes, dentro de los tumores orbitarios, la presencia de 2 o más es infrecuente dentro de una órbita, la topografía extraconal es la más común, cuando son múltiples pueden ser parte de angiomatosis cerebral en algún momento de su evolución, por ello la necesidad de controles evolutivos. Descripción del caso: Paciente mujer, 30 años que debuta con proptosis derecha, dolor retrocular, diplopía, agudeza visual normal.Tac se visualizan dos lesiones intraorbitarias derechas, intraconales, solidas, bien definidas, retrooculares, que contacta (la más interna) el nervio óptico y provoca una rotación externa del globo ocular. RMN: las lesiones se manifiestan más difusas y heterogéneas, con signos de hemosiderina. Resultados: Video explicativo del caso, y pasos estructurados de la técnica. Se utilizó orbitomía lateral derecha, incisión ciliar con extensión hacia la porción lateral de la órbita, osteotomía del reborde lateral orbitario, luego con drill se avanza la resección de la pared lateral hasta el borde externo de la hendidura esfenoidal. Punto de tracción sobre la glándula lagrimal, permitiendo rotación del globo ocular y tensar periorbita. Se abre periorbita, identificando músculo recto externo y por su borde superior se avanza en la disección profunda reconociendo vaina del nervio óptico, localizando los angiomas cavernosos, disecándolos circunferencialmente y extrayéndolos. Reconstrucción de la pared orbitaria y reborde orbitario, cierre por planos con criterio cosmético, no hubo déficit óculo-motor. Conclusión: La exéresis quirúrgica es el gol estándar de tratamiento. La orbitotomía lateral es un abordaje mínimamente invasivo, seguro a esta patología.

Introduction: Cavernous angiomas are common entities, within orbital tumors, the presence of 2 or more than two is uncommon within an orbit, extraconal topography is the mostcommun, when they are multiple, they can be part of brain angiomatosis at some point in their evolution, therefore the need for evolutionary controls. Case Description: Pte. woman, 30 years who debuts with right proptosis, retrocular pain, diplopia, normal visual acuity. Tac two right, intracontal, solid, well-defined, retroocular intraorbital lesions are displayed that contacts (the innermost) the optic nerve and causes an external rotation of the eyeball. MRI: lesions appear more diffuse and heterogeneous, with signs of hemosiderin. Results: Explanatory video of the case, and structured steps of the technique.Right lateral orbitomia, ciliary incision extending to the lateral portion of the orbit, osteotomy of the right orbital lateral rim was used, then with drill the resection of the lateral wall is advanced. Traction points on the tear gland, allowing rotation of the eyeball and tensioning periorbita. It opens periorbita, identifying external straight muscle and by its upper edge advances in the deep dissection, recognizing sheath of the optic nerve, locating the cavernous angiomas, dissecting them circumferentially and removing them. Reconstruction of the orbital wall and orbital rim, closure by planes with cosmetic criteria, there was no oculo-motor deficit. Conclusion: Surgical resection is the standard treatment goal. The lateral orbitotomia was a minimally invasive approach, safe to this pathology.

Dental care challenges in Sturge-Weber syndrome: a case report / Desafios do tratamento odontológico na síndrome de Sturge-Weber: relato de caso

Introduction: Sturge-Weber syndrome (SWS) is a rare condition characterized by facial capillary malformation, involves ocular, neurological, and cutaneous alterations. Associated with unilateral characteristic port-wine stains, gingival growth and purple-red coloration. Aim: his case aims to report dental treatment challenges in patients with SWS and importance of oral health maintenance in these individuals. Case report: a 20-year-old woman with an established diagnosis of SWS, presented bad breath and spontaneous gingival bleeding, with gingival growth and reddish-purple spots spread to labial and alveolar mucosa, tongue, and palate. Conditioning of the patient's oral environment by supra and subgingival scraping, dental unit extraction was performed. A conservative treatment plan was adopted for management adequacy of oral environment owing to possible complications inherent to the condition. Conclusion: it is important to emphasize the importance of dental surgeon's performance in relation to a multidisciplinary health team, as well as cooperation of patient, to obtain better results from the proposed therapy.

Introdução: a síndrome de Sturge-Weber (SSW) é uma condição rara caracterizada por malformação capilar facial, envolve alterações oculares, neurológicas e cutâneas. Associada a manchas unilaterais características do vinho do porto, crescimento gengival e coloração vermelho-púrpura. Objetivo: este caso tem como objetivo relatar desafios do tratamento odontológico em pacientes com SSW e a importância da manutenção da saúde bucal nesses indivíduos. Relato de caso: paciente do sexo feminino, 20 anos, com diagnóstico estabelecido de SSW, apresentou mau hálito e sangramento gengival espontâneo, com crescimento gengival e manchas roxas avermelhadas espalhadas pela mucosa labial e alveolar, língua e palato. Condicionamento do ambiente oral do paciente por raspagem supra e subgengival, foi realizada extração da unidade dental. Foi adotado um plano de tratamento conservador para adequação do manejo do ambiente bucal devido a possíveis complicações inerentes à condição. Conclusão: é importante enfatizar a importância do desempenho do cirurgião-dentista em relação a uma equipe multidisciplinar de saúde, bem como a cooperação do paciente, para obter melhores resultados com a terapia proposta.

Fractura patológica de cadera en edad pediátrica. Revisión de la angiomatosis quística ósea / Pathological hip fracture of pediatric age. Revision of the cystic angiomatosis of the bone

Resumen: Introducción: La angiomatosis quística ósea es una enfermedad con solamente 200 casos descritos basados en la proliferación no maligna angiomatosa en tejido óseo y vísceras. Se observa más en la pelvis, los huesos largos y la cintura escapular. La evolución clínica varía de formas autolimitantes a osteólisis agresiva masiva (Gorham-Stout). Su diagnóstico es por exclusión, con datos clínico-radiológicos (imágenes líticas) y resultados histopatológicos no específicos. Caso clínico: Nuestro estudio se basa en el caso clínico de un paciente de 14 años diagnosticado con angiomatosis quística ósea, con afección ósea diseminada, que vino a nuestro centro por el dolor y la impotencia funcional en la cadera derecha, diagnosticando una fractura pertrocantérea. Se le hizo una osteosíntesis con tornillo deslizante de la placa VERSA de cuatro agujeros. Se utilizó un injerto alogénico de hueso esponjoso de la cabeza femoral. La evolución fue satisfactoria con ambulación a plena carga por mes sin requerir medicación analgésica y control de rayos X a los ocho meses, los cuales mostraron signos de completa integración del injerto. La escala analgésica del dolor (EVA) mostró una puntuación de 2/10 por mes. Discusión: Hay controversia con respecto al tratamiento de estas fracturas por la osteosíntesis con el injerto del hueso. Nos enfrentamos a un caso sin criterios clínicos serios, pero con múltiples focos de osteólisis. Se decidió no intervenir profilácticamente la cadera contralateral, ya que es asintomática y hay diferentes patrones de evolución descritos en la literatura y los riesgos en la cirugía

Abstract: Introduction: Bone cystic angiomatosis is a disease with only 200 cases described, based on angiomatous nonmalignant proliferation, in bone tissue and viscera. It focuses on pelvis, long bones and scapular waist. Clinical evolution ranges from self-limiting forms to massive aggressive osteolysis (Gorham-Stout).Its diagnosis is of exclusion, with nonspecific clinical, radiological (lytic images) and histopathological findings. Clinical case: Our study is based on the clinical case of a 14-year-old man diagnosed with bone cystic angiomatosis, with disseminated bone involvement, who came to our center for pain and functional impotence in the right hip, diagnosing a pertrochanteric fracture. It was operated by open reduction and osteosynthesis with four-hole VERSA sliding plate screw. An allogeneic graft of cancellous bone was used from femoral head. The evolution was satisfactory, with ambulation at full load per month without requiring analgesic medication and control X-ray at eight months that showed signs of complete integration of the graft. The analgesic pain scale (visual analogue scale) showed a score of 2/10 per month. Discussion: There is controversy regarding the treatment of these fractures by osteosynthesis with bone graft. We are facing a case without serious clinical criteria, but with multiple foci of osteolysis. It was decided not to intervene prophylactically the contraleteral hip, since it is asymptomatic and there are different patterns of evolution described in the literature and risks in surgery.

Outcomes after surgical resection of primary non-myxoma cardiac tumors

Abstract Objective: Primary cardiac tumors are rare lesions with different histological type. We reviewed our 17 years of experience in the surgical treatment and clinical results of primary non-myxoma cardiac tumors. Methods: Between July 2000 and February 2017, 21 patients with primary cardiac tumor were surgically treated in our institution. The tumors were categorized as benign non-myxomas and malignants. Data including the demographic characteristics, details of the tumor histology and grading, cardiac medical and surgical history, surgical procedure of the patients were obtained from the hospital database. Results: Eleven patients were diagnosed with benign non-myxoma tumor (male/female:7/4), ranging in age from 10 days to 74 years (mean age 30.9±26.5 years). Papillary fibroelastoma was the most frequent type (63.6%). There were two early deaths in benign group (all were rhabdomyoma), and mortality rate was 18%. The mean follow-up period was 69.3±58.7 months (range, 3 to 178 months). All survivals in benign group were free of tumor-related symptoms and tumor relapses. Ten patients were diagnosed with malignant tumor (sarcoma/lymphoma:8/2, male/female:3/7), ranging in age from 14 years to 73 years (mean age 44.7±18.9 years). Total resection could be done in only three (30%) patients. The mean follow-up period was 18.7±24.8 months (range, 0-78 months). Six patients died in the first 10 months. Conclusion: Complete resection of the cardiac tumors, whenever possible, is the main goal of surgery. Surgical resection of benign cardiac tumors is safe, usually curative and provides excellent long-term prognosis. On the contrary, malignant cardiac tumors still remain highly lethal.

Síndrome de Sturge Weber: relato de caso / Sturge-Weber Syndrome: a case report

A Síndrome de Sturge-Weber é uma síndrome rara, não hereditária e de etiologia desconhecida. É caracterizada por proliferações vasculares harmatomatosas, nos tecidos do cérebro e face e ocorre em cerca de 1/50.000 nascimentos. Este trabalho relata o caso de uma paciente de 7 anos com Síndrome de Sturge-Weber e aborda as etapas de seu tratamento junto com uma equipe multidisciplinar na APAE-Santo Ângelo, de 2001 até 2009(AU)

The Sturge-Weber syndrome is a rare, nonhereditary syndrome of unknown etiology. It is characterized by hamartomatous vascular proliferations in the brain and face tissues and occurs in about 1/50,000 live births. This paper reports the case of a 7-year-old patient with Sturge-Weber syndrome, and addresses the steps for its treatment with a multidisciplinary team in the APAE-Santo Ângelo from 2001 until 2009(AU)

Determining the Cause of Natural Death: A Case of Previously Unknown Sturge-Weber Syndrome / 대한법의학회지

In some cases, it is difficult to determine a single cause of death even after conducting full autopsy and additional tests. A 49-year-old man, reportedly having diabetes mellitus, was found unconscious by his mother and revealed to be dead. He had several contusions all over his body, including the right periocular area, but they did not appear fatal. A focal area of polymicrogyria and cortical dysplasia was found on the right preoccipital notch, accompanied with dystrophic calcification and leptomeningeal angiomatosis. These findings were considered indicative of Sturge-Weber syndrome, a rare neurocutaneous disorder, of atypical type without facial lesions. Blood level of beta-hydroxybutyrate was 859 microg/mL, implying that he also had diabetic ketoacidosis. His ketoacidosis may not have been corrected appropriately because of status epilepticus in association with brain lesion, resulting in his death, but neither direct evidence nor statement was obtained. In cases with several apparent causes of death, the examiner's assumption should be based not on imagination but on evidence, and logic should not be overlooked. It is more helpful for the investigators or the bereaved to obtain more detailed information rather than come to a hasty conclusion.

Breast hamartoma: a clinicopathologic analysis of 27 cases and a literature review

OBJECTIVES: Breast hamartoma is an uncommon breast tumor that accounts for approximately 4.8% of all benign breast masses. The pathogenesis is still poorly understood and breast hamartoma is not a well-known disorder, so its diagnosis is underestimated by clinicians and pathologists. This study was designed to present our experience with breast hamartoma, along with a literature review. METHOD: We reviewed the demographic data, pathologic analyses and imaging and results of patients diagnosed with breast hamartoma between December 2003 and September 2013. RESULTS: In total, 27 cases of breast hamartoma operated in the Ankara University Medicine Faculty's Department of General Surgery were included in the study. All patients were female and the mean age was 41.8±10.8 years. The mean tumor size was 3.9±2.7 cm. Breast ultrasound was performed on all patients before surgery. The most common additional lesion was epithelial hyperplasia (22.2%). Furthermore, lobular carcinoma in situ was identified in one case and invasive ductal carcinoma was observed in another case. Immunohistochemical staining revealed myoid hamartoma in one case (3.7%). CONCLUSION: Breast hamartomas are rare benign lesions that may be underdiagnosed because of the categorization of hamartomas as fibroadenomas by pathologists. Pathologic examinations can show variability from one case to another. Thus, the true incidence may be higher than the literature indicates. .

Hiperplasia pseudoangiomatosa nodular gigante de la mama / Pseudoangiomatous stromal hyperplasia (pash) of the breast

La hiperplasia pseudoangiomatosa (PASH) es una lesión proliferativa benigna de la mama, poco frecuente, caracterizada por la existencia de lagos pseudovasculares embebidos en una gran proliferación del estroma mamario. Probablemente, el desarrollo de la PASH tenga una influencia hormonal, por lo que típicamente se diagnostica en mujeres en edad fértil. La PASH es un hallazgo histopatológico casual en las piezas quirúrgicas y biopsias mamarias realizadas por otra patología. La presentación clínica en forma de masa palpable es poco frecuente. El principal diagnóstico diferencial debe realizarse con el angiosarcoma de bajo grado. El tratamiento de la PASH nodular es una correcta exéresis quirúrgica asegurando borde sano amplio. El pronóstico es excelente, con un mínimo riesgo de recidiva si se realiza una adecuada cirugía. Se presenta el caso de una mujer de 37 años que acude a consulta por un nódulo mamario de crecimiento rápido.

Pseudoangiomatous stromal hyperplasia (PASH) of the breast is a rare benign proliferative mesenchymal lesion characterized by the presence of open slit like spaces embedded in a hyalinized fibrous stroma. The development of PASH is probably subject to hormonal influence so it typically affects women in the reproductive age group. Pseudoangiomatous stromal hyperplasia is frequently an incidental histologic finding in breast surgeries or biopsies performed for other injuries. In rare cases, it presents as a localized breast mass. The most important differential diagnosis is low-grade angiosarcoma. Tumorous PASH is treated by local surgical excision with clear margins. The prognosis is excellent, with minimal risk of recurrence after adequate surgery. The presented case was a 37-years-old woman who was admitted with a rapidly growing breast tumor.

Hamartoma e hiperplasia estromal pseudoangiomatosa de la mama, diagnósticos diferenciales infrecuentes de cáncer de mama / Hamartoma and nodular pseudoangiomatous stromal hyperplasia: differential diagnosis

Introducción: Los hamartomas y la hiperplasia estromal pseudoangiomatosa nodular (PASH) son entidades infrecuentes en la patología tumoral mamaria, sin embargo, pueden simular al cáncer de mama. La única herramienta certera preoperatoria es la biopsia. El tratamiento quirúrgico es curativo con bajo índice de recurrencia. Objetivo: El objetivo de este trabajo es exponer un caso de hamartoma mamario con PASH asociado cuya presentación inicial fue sugerente de probable patología maligna. Caso Clínico: Mujer de 44 años, ingresa a controles por probable patología mamaria maligna, la mamografía fue informada como BIRADS 0, la ecografía mamaria como BIRADS US: 5, la biopsia CORE informó PASH. Se realizó mastectomía parcial objetivando en la biopsia diferida un hamartoma mamario con extensa hiperplasia estromal pseudoangiomatosa. Discusión: Los hamartomas y la PASH son patologías sin presentación clínica específica, los hallazgos radiológicos del hamartoma pueden ser patognomónicos, no así en la PASH; en el caso expuesto el estudio anatomopatológico demostró una asociación de estas patologías, la cual se describe desde un 16 por ciento a un 71 por ciento de los casos de hamartoma mamario. El análisis de toda la pieza quirúrgica es fundamental para establecer un diagnóstico definitivo.

Introduction: Hamartoma and nodular pseudoangiomatous stromal hyperplasia (PASH) are inusual breast tumor entities, however, can simulate breast cancer. The only accurate tool is preoperative biopsy. Surgical treatment is curative with low recurrence rate. Objective: To present a case of mammary hamartoma associated with PASH whose initial presentation was suggestive of probable malignancy. Case Report: Woman, 44 years old, admitted to controls because of probable malignant breast disease, mammography was reported as BIRADS 0, breast ultrasound as BIRADS U.S: 5, CORE biopsy reported PASH. Partial mastectomy was performed. On delayed biopsy mammary hamartoma with extensive stromal hyperplasia pseudoangiomatosa was diagnosed. Discussion: PASH and hamartomas are diseases with no specific clinical presentation, radiological findings may be pathognomonic of hamartoma, while not in PASH. In the reported case the pathological study showed an association of these conditions, described in a 16% to 71% breast hamartoma cases. The analysis of the entire surgical specimen is essential for a definitive diagnosis.

Tumoral Pseudoangiomatous Stromal Hyperplasia of Male Breast: A Case Report

Tumoral form of pseudoangiomatous stromal hyperplasia (PASH) of male breast is a rare disease entity. We report on a case of PASH that presented as a palpable subareolar breast mass in a 46-year-old male who has been on hemodialysis due to chronic renal failure. To the best of our knowledge, no case of PASH in a male with chronic renal failure has yet been reported. We describe its mammographic and sonographic findings with correlative histopathologic features, confirmed by surgical excision.

A Case of Diffuse Dermal Angiomatosis / 대한피부과학회지

Diffuse dermal angiomatosis is an acquired, benign vascular proliferation with poorly circumscribed, violaceous, and livedoid plaques with frequent ulceration. Histologically, there is diffuse and interstitial proliferation of CD31-positive endothelial cells and myofibroblasts within the dermis. Endothelial atypia, mitoses, and vasculitis are lacking. Here we describe a 52-year-old male who presented with erythematous and purpuric patches on the trunk and lower extremities for 2 years. Histologic examination demonstrated a dense proliferation of endothelial cells in the papillary and reticular dermis. Small vascular spaces and extravasated erythrocytes were noted. Immunohistochemical staining using anti-CD31 antibody was positive.

Sclerosing angiomatoid nodular transformation of the spleen: report of five cases and review of literature / 中华医学杂志(英文版)

Sclerosing angiomatoid nodular transformation (SANT) of the spleen, a newly defined primary lesion of the spleen, is very rare. Immunohistochemistry is the only way to confirm the diagnosis of SANT. We present the clinical characteristics and postoperative outcomes of five SANT cases that underwent splenectomy from January 2007 to October 2010. Although SANT had specific imaging findings, differential diagnosis from other splenic tuomrs or malignant lesions preoperatively was difficult. The hand-assisted laparoscopic splenectomy was a useful and effective technique for the management and postoperative diagnosis of SANT. All SANT patients had good prognosis without recurrence after splenectomy.

Sturge Weber syndrome: a case report / Síndrome de Sturge Weber: um relato de caso

PURPOSE: The Sturge-Weber syndrome (SWS) is a rare congenital disorder that occurs sporadically and features lesions of both skin and nervous system. We report a case of 7 year-old girl with Sturge-Weber syndrome and discuss its clinicopathological features, differential diagnosis and also emphasize the importance of its diagnosis in the clinical oral practice. CASE DESCRIPTION: The classic pathognomonic manifestations include angioma of the leptomeninges extending to cerebral cortex with ipsilateral angiomatous lesions, unilateral facial nevus affecting a division of trigeminal nerve, hemiparesis, intracranial calcification, mental retardation and refractory epilepsy. The most apparent indication of SWS is a facial birthmark or "Port Wine Stain" present at birth and typically involving at least one upper eyelid and the forehead. Management of a patient with Sturge-Weber Syndrome may be challenging due to risk of hemorrhage. CONCLUSION: The dentists should focus on comprehensive therapy, starting with behavior management and stress on preventive measures.

OBJETIVO: A síndrome de Sturge-Weber (SWS) é uma rara doença congênita que ocorre esporadicamente e apresenta lesões de pele e do sistema nervoso. Este artigo é um relato de caso de uma menina de 7 anos com SWS para discutir suas características clinicopatológicas, diagnóstico diferencial e enfatizar a importância do seu diagnóstico na prática clínica odontológica. DESCRIÇÃO DO CASO: As manifestações clássicas patognomônicas incluem angioma das leptomeninges que se estendem para o córtex cerebral com lesões angiomatosas ipsilaterais, nevo facial unilateral, que afetam uma divisão do nervo trigêmeo, hemiparesia, calcificação intracraniana, retardo mental e epilepsia refratária. A indicação mais evidente de SWS é uma marca de nascença facial ou "mancha vinho do porto" presente no nascimento e tipicamente envolvendo pelo menos uma pálpebra superior e a testa. O controle clínico de um paciente com SWS pode ser desafiador devido ao risco de hemorragia. CONCLUSÃO: Os dentistas devem adotar uma abordagem terapêutica abrangente, começando com o controle de comportamento e de estresse com medidas preventivas.

Malformaciones Vasculares / Vascular malformation

Las malformaciones vasculares son anomalías presentes siempre desde el nacimiento que, al contrario de los hemangiomas, nunca desaparecen; pueden crecer durante toda la vida por hipertrofia. Según la clasificación de la ISSVA, las malformaciones vasculares se dividen en función del vaso afectado en capilares o venulares, venosas, linfáticas, arterio-venosas y combinadas o complejas. Cada una de ellas, con unas peculiaridades clínicas y hemodinámicas definitorias.

Vascular malformations are anomalies always present at birth that, contrary to hemangiomas, never regress; and may hypertrophy during lifetime. According to the ISSVA classification, vascular malformations are divided, depending on the affected vessel, into capillary or venular, venous, lymphatic, arteriovenous and combined or complex; each with certain defining clinical and haemodynamic peculiarities.

Rapidly Growing Bilateral Pseudoangiomatous Stromal Hyperplasia of the Breast

A tumoral pseudoangiomatous stromal hyperplasia (PASH) that causes huge breast enlargement is very rare. Only two cases of huge tumoral PASHs have been reported in the English medical literature. We report here on a surgically confirmed case of bilateral huge tumoral PASH in a 47-year-old woman, and we present the imaging and histopathology findings. We also review the relevant medical literature.

Aspectos clínicos da síndrome de Sturge-Weber:[revisão] / Clinical features of Sturge-Weber syndrome: [review]

Introdução: A Síndrome de Sturge-Weber é uma rara condição de desenvolvimento congênita, sendo caracterizada por uma desordem neurocutânea com angiomas envolvendo as leptomeninges e a pele da face, preferencialmente no trajeto do ramos oftálmico (V1) e maxilar (V2) do nervo trigêmeo. Objetivo: Realizar uma revisão de literatura sobre a Síndrome de Sturge-Weber, enfatizando aspectos atuais. Método: Foram consultadas as bases de dados on-line EMedicine, Encyclopedia of Medicine, Find Articles, LILACS, MEDLINE, Merkmanuals On-Line Medical Library e SciELO, aplicando à pesquisa os termos: Síndrome de Sturge-Weber, síndromes neuro-cutâneas, angiomatose encefalotrigeminal, nevo flamíneo, para artigos publicados entre 1991 e 2007. Revisão de Literatura:O achado clínico mais característico é a presença, já ao nascimento, do nevo flamíneo que atinge, geralmente, uma metade da face podendo estender-se até o pescoço; além disso, outras manifestações clínicas podem estar presentes, como a angiomatose corticocerebral, calcificações cerebrais, epilepsia, afecções oculares, bucais e retardo mental.O diagnóstico é estabelecido por meio da pesquisa de alterações neurológicas e oftálmicas em pacientes com o nevo flamíneo característico, aliando-se aos dados clínicos exames complementares como Tomografia Computadorizada.O tratamento consiste, basicamente, em controlar as manifestações clínicas já evidentes e prevenir o surgimento de outras alterações, principalmente orais e oculares. Conclusão: Esta síndrome, apesar de pouco freqüente, precisa ser precocemente diagnosticada, visto que acarreta uma série de complicações aos seus portadores quando não tratada, principalmente por atingir o Sistema Nervoso Central.Os profissionais da saúde devem, portanto, estar aptos a reconhecer seus sinais e sintomas característicos, e assim melhorar a qualidade de vida desses pacientes.

Introduction: The Syndrome of Sturge-Weber is a rare condition of congenital development, and is characterized by a neurocutaneous disorder with angiomas wrapping the leptomeninges and the face skin, mainly in the course of ophthalmic (V1) and maxillary (V2) branches of the trigeminal nerve. Objective: To review the literature about the Sturge-Weber Syndrome with emphasis on the current aspects. Method: The following databases were searched: EMedicine, Encyclopedia of Medicine, FindArticles, LILACS, MEDLINE, Merck Manuals On-Line Medical Library and Scielo, and the searches applied the terms: Sturge-Weber Syndrome, neurocutaneous syndromes, encephalo-trigeminal angiomatosis, nevus flammeus, in articles published between 1991 and 2007. Literature's Review: The most characteristic clinical statement is the presence, since the birth, of nevus flammeus, that generally reaches one half of the face and may stretch out up to the neck; in addition, other clinical manifestations may be present, like the corticocerebral angiomatosis, cerebral calcifications, epilepsy, ocular and buccal affections and mental retardation. The diagnosis is established by means of the inquiry of neurological and ophthalmic alterations in patients with a characteristic nevus flammeus, allied to the clinical data of complementary exams such as Computerized Tomography. The treatment consists basically of controlling the already confirmed clinical manifestations and preventing from the appearing of other alterations, mainly buccal and ocular. Conclusion: This syndrome is not much frequent, but it needs to be early diagnosed, since it brings a series of complications to its carriers when not treated, specially because of reaching the Nervous Central System. The health professionals have to be suitably able to recognize its characteristic signs and symptoms, and so improve the quality of life of these patients.