Racemose hemangioma presenting as a vitreous hemorrhage: case report / Hemangioma racemoso apresentando-se como hemorragia vítrea: relato de caso

ABSTRACT This report describes a case of retinal racemose hemangioma that first presented as a vitreous hemorrhage. The authors present the case of a 47-year-old woman with a sudden 5-day painless visual loss in her left eye. At the first visit, the best-correct visual acuities were 20/20 in the right eye and hand motions in the left eyes. Ultrasonography showed an attached retina and a massive vitreous hemorrhage. Pars plana vitrectomy was performed and a dilatation of large vessels was detected bulging from the optic disc. The best-correct visual acuities on day 30 postoperatively was 20/25 in the left eye. Fundus angiography and spectral-domain optical coherence tomography angiography showed anomalous arteriovenous communications with no intervening capillaries. The diagnosis was racemose hemangioma, an arteriovenous malformation of group 2 retina based on the Archer classification.

RESUMO Este relato descreve um caso de hemangioma racemoso da retina que se apresentou inicialmente como hemorragia vítrea. Os autores apresentam o caso de uma mulher de 47 anos com perda visual súbita e indolor 5 dias antes no olho esquerdo. Na primeira visita, a melhor acuidade visual corrigida foi de 20/20 no olho direito e movimentos das mãos no olho esquerdo. A ultrassonografia mostrou uma retina aderida e uma hemorragia vítrea maciça. Foi realizada vitrectomia pars plana, sendo detectada proliferação de grandes vasos salientes do disco óptico. A acuidade visual no dia 30 de pós-operatório foi de 20/25 no olho esquerdo. A angiografia de retina e a angiotomografia de coerência óptica de domínio espectral mostraram comunicações arteriovenosas anômalas sem capilares intermediários. O diagnóstico foi hemangioma racemoso, uma malformação arteriovenosa da retina do grupo 2 com base na classificação de Archer.

A 46-Year Retrospective Study of Oral Vascular Anomalies in a Brazilian Population / Estudio Retrospectivo de 46 Años de Anomalías Vasculares Orales en una Población Brasileña

A retrospective study was performed to describe the demographic and clinicopathological characteristics of cases of vascular anomalies in a Brazilian population. The data of cases diagnosed as vascular anomalies over a period of 46 years, were retrieved from the archives of the Pathological Anatomy Service of the Department of Dentistry of Federal University of Rio Grande do Norte. Information about sex, age, race, diagnosis, location, and clinical characteristics was extracted. A database was created for analysis in the SPSS system and description of the results. The sample consisted of 597 cases categorized as follows: pyogenic granuloma (n = 454), infantile hemangioma (n = 57), vascular malformation (n = 37), lymphangioma (n = 20), hemangiolymph angioma (n = 10), vascular thrombus (n = 7), hemangio endothelioma (n = 5), angiofibroma (n = 1), hemangioendothelio sarcoma (n = 1), and Kaposi sarcoma (n = 5). Overall, there was a predominance of females (64.2 %), white race (54.2 %), and patients in the 3rd and 4th decades of life. Most cases affected the gingiva/ ridge (44.75 %), had a red/reddish color (60.07 %), sessile implantation (52.4 %), soft consistency (52.7 %) and nodular appearance (53, 9 %), and were non-hemorrhagic (51.3 %) and asymptomatic (76.6 %). The demographic and clinicopathological findings obtained for most of the vascular lesions analyzed are similar to those reported in published studies involving other populations. Knowledge of the particularities of these disorders is important for appropriate diagnosis and treatment decision.

Se realizó un estudio retrospectivo para describir las características demográficas y clínico-patológicas de casos de anomalías vasculares en una población brasileña. Los datos de los casos diagnosticados como anomalías vasculares durante un período de 46 años, fueron obtenidos de los archivos del Servicio de Anatomía Patológica del Departamento de Odontología de la Universidade Federal de Rio Grande do Norte. Se analizó la información de sexo, edad, raza, diagnóstico, ubicación y características clínicas, y se creó una base de datos para análisis en el sistema SPSS y descripción de los resultados. La muestra consistió de 597 casos categorizados de la siguiente manera: granuloma piógeno (n = 454), hemangioma infantil (n = 57), malformación vascular (n = 37), linfangioma (n = 20), hemangiolinfangioma (n = 10), trombo vascular (n = 7), hemangioendotelioma (n = 5), angiofibroma (n = 1), hemangioendoteliosarcoma (n = 1) y sarcoma de Kaposi (n = 5). En general predominaron las mujeres de raza blanca (64,2 %), (54,2 %), y pacientes en la 3ª y 4ª década de la vida. La mayoría de los casos afectaban la encía / cresta (44,75 %), tenían un color rojo / rojizo (60,07 %), implantación sésil (52,4 %), consistencia blanda (52,7 %) y apariencia nodular (53,9 %), no hemorrágicos (51,3 %) y asintomáticos (76,6 %). Los hallazgos demográficos y clínico-patológicos de la mayoría de las lesiones vasculares analizadas son similares a los reportados en la literatura de otras poblaciones. El conocimiento de las particularidades de estos trastornos es importante para el diagnóstico y la decisión de tratamientos adecuados.

Abnormalities of the hallux skin and nail in the course of very rare arteriovenous malformation / Anormalidades de unha e pele do hálux no decorrer de malformação arteriovenosa muito rara

Abstract Arteriovenous malformations (AVMs) are usually found in the pelvic area and the brain. These vascular anomalies are rarely reported in the toes. AVMs in the toes may be asymptomatic, but can also cause atypical symptoms. Congenital AVMs can expand as patients age and manifest in adulthood. They may be provoked by injury. Acquired AVM might be caused by iatrogenic factors, venous or arterial catheterization, percutaneous invasive vascular procedures, surgery, or degenerative vascular disorders. An AVM can damage surrounding tissues and can cause destruction of skin, nails and bones. The course of the disease is often unpredictable and diagnosis is usually delayed as a result.

Resumo As malformações arteriovenosas (MAVs) são geralmente encontradas na região pélvica e no cérebro.. Essas anomalias vasculares raramente são relatadas nos dedos dos pés. A MAV nesse local pode ser assintomática ou apresentar sintomas atípicos. MAVs congênitas podem evoluir com a idade e se manifestar na idade adulta. O fator provocante pode ser uma lesão traumática. Uma MAV adquirida pode ser causada por fatores iatrogênicos, cateterismo venoso e arterial, procedimentos percutâneos vasculares invasivos, cirurgias e alterações degenerativas vasculares. A MAV pode danificar tecidos adjacentes e pode causar destruição de pele, unhas e ossos. O curso da doença é muitas vezes imprevisível, e como resultado, atrasar o diagnóstico.

Relato de caso: síndrome de Klippel-Trénaunay- Weber / Case Report: Klippel-Trénaunay-Weber syndrome

Introdução: A síndrome de Klippel-Trénaunay-Weber (SKTW) é caracterizada pelo conjunto de sinais que consiste em malformações capilares, malformações venosas com ou sem malformações linfáticas associado ao supercrescimento de membros. Na maioria das vezes, envolve apenas uma extremidade com malformação arteriovenosa e cerca de 75% dos pacientes manifestam antes dos 10 anos de idade. Relato de Caso: Relatamos um caso de Klippel-Trénaunay- Weber em um paciente de 7 meses em acompanhamento na enfermaria da Cirurgia Plástica do Hospital de Clínicas da Universidade Federal de Uberlândia para o qual foi proposto tratamento cirúrgico da lesão. Conclusão: Como a SKTW é uma doença com morbidade progressiva e grave, o paciente deve ser acompanhado em um centro de referência com experiência e arsenal terapêutico diversificado para atuar da melhor forma possível no tratamento.

Introduction: The Klippel-Trénaunay-Weber syndrome (KTWS) is characterized by several signs, including capillary malformations and venous malformations with or without lymphatic malformations associated with limb overgrowth. In most cases, only one extremity is involved with arteriovenous malformation, and approximately 75% of the patients manifest symptoms before 10 years of age. Case Report: We report a case of a 7-month-old patient with KTWS followed-up at the Plastic Surgery Service of the Hospital de Clínicas, Federal University of Uberlândia; surgical treatment of the lesion was proposed for the patient. Conclusion: Since KTWS is a progressive disease with severe morbidity, the patient must be followed-up at a reference center by experienced staff with diverse therapeutic arsenal.

Manejo quirúrgico de pacientes adultos con anomalías vasculares: serie de casos / Surgical management of adult patients with vascular anomalies: series of cases

Introducción: Las anomalías vasculares son un grupo heterogéneo de patologías, que considera tanto tumores como malformaciones vasculares. Son, habitualmente, de diagnostico y manejo durante la infancia, algunas lesiones persisten hasta la edad adulta. El tratamiento habitual considera terapias poco invasivas, siendo la alternativa resecctiva un opcion infrecuente. Objetivos: El objetivo de este estudio es presentar el manejo quirúrgico de pacientes portadores de anomalías vasculares realizado por el equipo de Cirugía Plástica y Reconstructiva del Hospital Clínico de la Universidad de Chile. Material y Métodos: Serie de casos retrospectiva de pacientes con diagnóstico de anomalias vasculares, sometidos a manejo quirúrgico por el equipo de Cirugía Plástica y Reconstructiva del Hospital Clínico de la Universidad de Chile. Se incluyó todos los pacientes con diagnóstico de anomalías vasculares intervenidos entre los años 2010 ­ 2016. Se realizó una revisión del historial clínico de cada paciente elaborando un análisis descriptivo. Resultados: Serie de 9 pacientes, 6 (66,6%) de sexo femenino, con edad promedio de 34,88 ±15,70 años. De los cuales 6 (66,67%) presentaron malformaciones vasculares y 2 (22,22%) tumores vasculares. Se observo compromiso de cabeza en 6 (66,67%) casos y 2 (22,22,%) genital. Se utilizo colgajos locales de avance en 6 (66,67%) y colgajos de transposición en 2 (22,22%) casos. Conclusiones: El manejo quirúrgico es una alternativa terapéutica segura que ofrece resultados buenos a las lesiones complejas. Y por tanto, creemos que es necesario un trabajo multidisciplinario entre pediatras, dermatólogos, radiólogos fisiatras, psicólogos y cirujanos plásticos para poder ofrecer mejores resultados a nuestros pacientes.

Introduction: Vascular anomalies are a heterogeneous group of pathologies, which considers tumors and vascular malformations. Usually diagnosed and treated during childhood, some lesions persist into adulthood. The usual treatment considers non-invasive therapies, being the respective alternative an uncommon option. Objectives: Present the surgical management of adults patients with vascular anomalies performed by the Plastic and Reconstructive Surgery team of the Clinical Hospital of the University of Chile. Material and methods: Retrospective case series of patients with diagnosis of vascular anomalies submitted to surgical management by the Plastic and Reconstructive Surgery team of the Clinical Hospital of the University of Chile. All patients with diagnosis of vascular anomalies operated between the years 2010 - 2016 were included. A review of the clinical history of each patient was carried out and a descriptive analysis was done. Results: Series of 9 patients, 6 (66.6%) female, with a mean age of 34.88 ± 15.70 years. Of these, 6 (66.67%) had vascular malformations and 2 (22.22%) had vascular tumors. Head involvement was observed in 6 (66.67%) cases and 2 (22.22%) genital. Local flaps were used in 6 (66.67%) and transposition flaps in 2 (22.22%) cases. Conclusions: Surgical management is a safe therapeutic alternative that offers beneficial results to complex lesions. Therefore, we believe a multidisciplinary work between pediatricians, dermatologists, radiologists.

Hemorragia gastrointestinal baja por malformación arteriovenosa yeyunal / Gastrointestinal bleeding due to arteriovenous malformation of the jejunum

Paciente varón de 21 años, sin antecedentes de importancia que ingresa a Emergencia por presentar deposiciones rojo oscuras, tendencia al sopor e hipotensión, con anemia importante procediéndose a realizar sucesivamente transfusión sanguínea, endoscopia digestiva alta, angiotomografía abdominal y arteriografía que, con diagnóstico clínico de lesión de Dieulafoy, motivó laparotomía exploratoria encontrándose una lesión vascular a nivel yeyunal con sangrado activo. Se discute el manejo de hemorragia digestiva severa con compromiso hemodinámico y la causa poco común de malformación arteriovenosa a nivel yeyunal hallada por patología.

A 23-year old male patient with no history of importance was admitted to Emergency because of dark red stools, tendency to lethargy and hypotension, with significant anemia proceeding to perform on blood transfusion, upper endoscopy, abdominal angiotomography and arteriography with clinical diagnosis of Dieulafoy lesion motivated exploratory laparotomy finding injury vascular, the jejunum with active bleeding. The management of severe gastrointestinal bleeding with hemodynamic compromise and uncommon cause of arteriovenous malformation in the jejunum is discussed.

Dieulafoy's disease of the bronchial tree: a case report / Doença de Dieulafoy da árvore brônquica: relato de caso

ABSTRACT CONTEXT: Dieulafoy's disease of the bronchial tree is a very rare condition. Few cases have been reported in the literature. It can be asymptomatic or manifest with massive hemoptysis. This disease should be considered among heavy smokers when recurrent massive hemoptysis is present amid otherwise normal findings. The treatment can be arterial embolization or surgical intervention. CASE REPORT: A 16-year-old girl was admitted to the emergency department due to hemoptysis with an unknown lesion in the bronchi. She had suffered massive hemoptysis and respiratory failure one week before admission. Fiberoptic bronchoscopy revealed a lesion in the bronchus of the right lower lobe, which was suspected to be a Dieulafoy lesion. Segmentectomy of the right lower lobe and excision of the lesion was carried out. The outcome for this patient was excellent. CONCLUSION: Dieulafoy's disease is a rare vascular anomaly and it is extremely rare in the bronchial tree. In bronchial Dieulafoy's disease, selective embolization has been suggested as a method for cessation of bleeding. Nevertheless, standard anatomical lung resection is a safe and curative alternative.

RESUMO CONTEXTO: A doença de Dieulafoy da árvore brônquica é uma condição muito rara, poucos casos foram descritos na literatura. Pode ser assintomática ou manifestar-se com hemoptise maciça. Esta doença deve ser considerada em fumadores pesados quando eles têm recorrentes hemoptises maciças sem outros achados anormais. O tratamento pode ser tanto embolização arterial como intervenção cirúrgica. RELATO DE CASO: Uma menina de 16 anos foi admitida no Serviço de Urgências devido a hemoptise com uma lesão nos brônquios de origem desconhecida. Havia sofrido hemoptise maciça e insuficiência respiratória uma semana antes da admissão. A broncoscopia de fibra óptica relevou lesão no brônquio do lobo inferior direito, com suspeita de ser lesão de Dieulafoy. Foi realizada uma segmentectomia do lobo inferior direito com excisão da lesão. O resultado da paciente foi excelente. CONCLUSÃO: A doença de Dieulafoy é uma anomalia vascular rara, sendo extremamente rara na árvore brônquica. Na doença de Dieulafoy bronquial, embolização seletiva tem sido sugerida como método para cessação do sangramento; no entanto, a habitual resseção anatômica do pulmão é uma alternativa segura e curativa.

Giant scalp arteriovenous malformation / Malformação arteriovenosa gigante de escalpo

SUMMARY Arteriovenous malformations (AVMs) of the scalp are rare lesions. The clinical picture presents with complaints of increased scalp, scalp disfigurement, pain and neurological symptoms. Its origin can be congenital or traumatic. We present a case of giant scalp AVMs and its management, followed by a brief literature review on the subject. The diagnosis of scalp AVMs is based on physical examination and confirmed by internal and external carotid angiography or computed tomographic angiography (CTA). Surgical excision is especially effective in scalp AVMs, and is the most frequently used treatment modality.

RESUMO Malformações arteriovenosas (MAV) do couro cabeludo são lesões raras. O quadro clínico apresenta-se com queixas de aumento do couro cabeludo, desfiguração do couro cabeludo, dor e sintomas neurológicos. A origem pode ser congênita ou traumática. Apresentamos um caso de MAV gigante de couro cabeludo e o tratamento adotado, seguindo-se uma breve revisão da literatura. O diagnóstico das MAV de couro cabeludo baseia-se no exame físico e é confirmado pela angiografia carótida interna e externa ou angiografia por tomografia computadorizada. A excisão cirúrgica é especialmente eficaz em MAV de couro cabeludo e é a modalidade de tratamento mais frequentemente utilizada.

Malformación arterio venosa cervical compleja sintomática / Complex arteriovenous malformation of the neck

Background: Arteriovenous malformation (AVM) is an abnormal connection between arteries and veins, bypassing the capillary system and forming arterial, venous and capillary nests. Case report: We report a female consulting for the first time at the age of 15 for an AVM located in the left posterior region of the neck. The patient was operated at that time, performing an extensive excision of the lesion and covering the defect with a skin flap. The lesion relapsed three years later. Vascular imaging revealed the malformation with afferent arteries from the subclavian, vertebral and left occipital arteries and a great posterior cervical nest that drained through a great anomalous vein to the subclavian vein. A surgical ligation of all the anomalous branches of the subclavian vein was performed, to perform a local excision in a second intervention. The patient got pregnant, delaying the intervention and had a normal delivery at the age of 19. At 21 years of age, she consulted again due to a great growth of her AVM, with repeated bleeding episodes, requiring transfusions. The patient was treated with embolization of the nest and the afferent arteries. Finally at 23 years of age, the lesion was excised again and the defect was covered with a skin flap. The patient had a good postoperative evolution.

Introducción: Las Malformaciones Arterio-Venosas (MAV) son alteraciones estructurales congénitas del desarrollo del sistema vascular en que se observan comunicaciones anómalas arterio-venosas conformando un "nido" arterio-venoso-capilar. Caso Clínico: Mujer que consulta a los 15 años de edad por una MAV en la región cervical posterior izquierda desde su nacimiento. Se efectúa una resección amplia de la lesión hasta el plano aponeurótico cubriendo el defecto con un colgajo de rotación cutáneo-adiposo. Evoluciona bien, pero recidiva luego de tres años. Un estudio vascular a los 18 años de edad evidencia la MAV con arterias aferentes desde la arteria subclavia, vertebral y occipital izquierdas, un gran "nido" cervical posterior que drena por una gran vena anómala hacia la vena subclavia izquierda. Se efectúa una ligadura quirúrgica de todas las gruesas ramas anómalas de la arteria subclavia, para efectuar una nueva resección local en un segundo tiempo. Sin embargo, esto se pospone pues la paciente se embaraza y tiene un parto normal a los 19 años. Vuelve a consultar a los 21 años por un gran crecimiento de su MAV con hemorragias a repetición, algunas muy profusas que obligan a transfundirla. Una AngioRNM confirma la MAV con un gran nido cervical posterior con nuevas aferencias. Se efectúa embolizaciones directas locales con espuma de polidocanol y micropartículas, y finalmente con alcohol. Una vez reducido el nido se emboliza además las principales aferencias por vía endovascular y se efectúa a los 23 años una amplia resección de la MAV cubriendo el defecto con un colgajo miocutáneo de dorsal ancho izquierdo. Ha evolucionado bien hasta ahora. Conclusión: El diagnóstico y tratamiento de una MAV compleja representa un gran desafío al equipo médico tratante que acompaña y atiende al paciente en el transcurso del tiempo, y a veces por el resto de la vida.

Malformación arteriovenosa del íleon medio, identificada por cápsula endoscópica: reporte de caso / Ileal vascular malformation, identified by capsule endoscopy: case report

Forty-nine year-old-man, diabetes mellitus Type 2, bone marrow transplantation 10 years ago due to chronic myelocytic leukemia, deep vein thrombosis and placement of cava vein filter 6 years ago, chronic use of Aspirin (100 mg). He presents with melena and abdominal pain. Upper endoscopy, colonoscopy, angio- CT and angiography were negative. Capsule endoscopy (PillCamTMSB) shows two segments of vascular malformation with active bleeding from the second segment in the middle-ileum. Laparotomy was performed founding at the level of the middle-ileum two vascular lesions of variceal type in the intestinal wall. Surgical resection of 15 cm of middle ileum and a primary anastomosis was performed. Histopathology reports, submucosal arteriovenous vascular malformation (AVM).

Hombre de 49 años, con antecedentes de diabetes mellitus tipo 2, trasplante de médula ósea hace 10 años por leucemia mieloide crónica, trombosis venosa profunda e instalación de filtro de vena cava inferior hace 6 años, usuario crónico de Aspirina® 100 mg/día. Presenta melena y dolor abdominal difuso. Es transfundido y se realizan estudios de endoscopia alta, colonoscopia, AngioTC y angiografía sin hallazgos del sitio de sangrado. Paciente ingiere cápsula endoscópica (PillCamTMSB) que muestra dos segmentos en intestino delgado, íleon medio y medio distal con malformaciones vasculares tipo várices, identificando sangrado activo en el segundo segmento. Se realiza laparotomía y a nivel del íleon medio se observan dos paquetes varicosos en la pared intestinal. Se realiza resección quirúrgica de 15 cm de íleon medio, y anastomosis primaria término-terminal. El paciente evolucionó satisfactoriamente sin recurrencia de sangrado. El estudio histopatológico e histoquímico con tinción de Van Gieson-elástica reporta malformación vascular arteriovenosa (AVM) submucosa de la pared del intestino delgado.

Malformación arteriovenosa pulmonar: características clínicas, diagnóstico y rol del tratamiento quirúrgico en pacientes tratados con cirugía resectiva pulmonar / Pulmonary arteriovenous malformation: clinical features, diagnosis and role of surgical management in patients with lung resection surgery

Introducción: Las malformaciones arteriovenosas pulmonares (MAVP) son infrecuentes y la cirugía tiene un rol en casos seleccionados. Objetivos: Describir las características clínicas, métodos diagnósticos y rol del tratamiento quirúrgico en pacientes tratados con cirugía por MAVP. Materialy Método: Revisión retrospectiva, incluyendo todos los pacientes con diagnóstico de MAVP en quienes se realizó cirugía en el Instituto Nacional del Tórax, desde febrero de 2005 a febrero de 2010. El seguimiento fue por control médico o contacto telefónico. Resultados: Se analizó 8 pacientes, seis mujeres (relación 3:1), edad entre 16 y 68 años. Los síntomas y signos más frecuentes fueron: disnea, cianosis y acropaquia. La localización más frecuente fue el lóbulo inferior derecho. Cuatro pacientes tenían MAVP múltiples y cuatro cumplían con criterios de Enfermedad de Rendu-Osler-Weber Seis tenían policitemia y dos anemia. La radiografía fue anormal en todos los casos y la tomogrqfía computada definió la anatomía en siete. Angiogrqfia se realizó en tres casos. Dos tenían ecocardiograma con contraste y cuatro cintigrama. La cirugía más frecuente fue la lobectomía. La indicación de cirugía fue: tamaño de la MAVP en cinco, falla de embolización en dos y hallazgo intra operatorio en uno, intervenido por otra razón. Uno presentó hemorragia post-operatoria. La estadía post-operatoria fue entre 2 y 10 días. No hubo mortalidad. En el último control todos los pacientes estaban asintomáticos. Conclusión: Las MAVPpueden presentarse en un amplio espectro clínico y anatómico. Pueden generar síntomas y complicaciones graves, por esto se recomienda tratarlas. El estudio pre-operatorio se basa en demostrar el shunt y en determinar las características anatómicas de la lesión. En algunos casos seleccionados está indicada la cirugía resectiva pulmonar.

Background: Pulmonary arteriovenous malformations (PA VM) are rare and surgery has a role only in selected cases. Our objectives are to describe clinical features, diagnostic methods and role of surgical treatment in patients with PA VM. Methods: Retrospective review of all patients with PA VM, in whom surgery was performed in our institution, from February 2005 to February 2010. The follow up controls were done through physician or telephone contact. Results: 8 patients, six females (3:1), aged between 16-68 years were analyzed. Most common sigiis and symptoms were dyspnea, cyanosis and clubbing. Right lower lobe was the most frequent location. Four had multiple PA VM and four met criteria for Rendu-Osler- Weber disease. Six patients had polycythemia and two anemia. Radiography was abnormal in all and computed tomography defined anatomy in seven. Angiography was performed in three, two had contrasted echocardiography and four had scintigraphy. Most common surgical treatment was lobectomy. Indications for surgery were the size of PA VM in five cases, failure of embolization in two and one because of intra-operative findings, without a previous diagrwsis. One had postoperative bleeding. Discharge was between day 2 and 10 days after surgery. There was no mortality. At their last control all patients were asymptomatic. Conclusion: PA VM presents a wide and varied range of clinical and anatomical findings. They can cause major symptoms and serious complications, which justify their treatment. The preoperative study is based primarily on demonstrating the shunt and determining the anatomical characteristics of the lesion. In selected cases lung resection surgery is indicated.

Manejo de malformaciones arterio venosas en servicio de neurocirugía Hospital Escuela Lenin Fonseca / Management of arterio venous malformations in neurosurgery service Lenin Fonseca Hospital School

El objetivo del trabajo es conocer el manejo de las malformaciones arteriovenosas en Nicaragua .queremos demostrar la vigencia del tratamiento quirúrgico de esta patología. Se analizo un periodo de cinco año del 2004 al 2008, se excluyeron todas las malformaciones epicraneales , cavernomas y telengectasia. Se recogió la información de 62 expedientes, llenando una ficha previamente elaborada, se analizaron los resultados en epiinfo versión 3.5.1. Se encontró un ligero predominio del sexo masculino, la edad predominante fue entre los 15 a 40 año . La principal forma de presentación fue la hemorragia intracraneal, la escala de espetzler predominante fue el grado III, la principal complicación postquirúrgica fue la hemorragia intracerebral, 24 de los 62 pacientes se trato de forma conservadora, la principal complicación transquirurgica fue la hemorragia masiva, las principales complicaciones se asociaron a la mala condición clínica al ingreso y escala de espetzler III, IV, V. Se recomienda protocolizar el tratamiento de este tipo de patología según la escala de espetzler...

Microsurgical approach of arteriovenous malformations in the central lobule / Abordagem microciúrgica para malformações arteriovenosas no lóbulo central

Arteriovenous malformations (AVM) are neurovascular disorders that occur mainly in young adults. Their clinical presentation is variable and depends on its location, size and occurrence of bleeding. They can represent incidental findings in neuro radiological exams. The treatment of these lesions when located in eloquent areas, namely around the central sulcus, is controversial, with different therapeutical approaches presented in the literature. We consider that surgical extirpation of many of these lesions is feasible in selected cases , when supported by profound anatomical knowledge and refined microsurgical technique, achieving cure with minimal aditional deficit. In the present article, we elaborate a surgical technique for the approach of AVMs located in the central sulcus, specially in finding the topographic lesion location and craniotomy.

Malformações arteriovenosas (MAV) são uma entidade patológica que ocorre mais frequentemente em adultos jovens. Sua manifestação clínica é variável e depende de sua localização, tamanho e ocorrência ou não de sangramento. Podem também ser diagnoticadas como achados incidentais em investigações neurorradiólogicas . O tratamento destas lesões, quando localizadas em áreas eloqüentes, em particular aquelas lesões localizadas próximas ao sulco central do cérebro, é controverso e diferentes abordagens terapêuticas são relatadas. Consideramos que a ressecção cirúrgica destas lesões é viável em casos selecionados, quando realizada sob profundo conhecimento anatômico e técnica microcirúrgica refinada, pois deste modo, as lesões podem ser curadas sem agregar morbidade ao paciente. No presente artigo, discutimos a técnica cirúrgica para MAVs localizadas nas adjacências do sulco central do cérebro, especialmente a localização topográfica da lesão e craniotomia.

Arteriovenous malformation, complications, and perioperative anesthetic management

Arteriovenous malformations [AVMs] are the most common intracranial vascular malformation, with an estimated occurrence of 1:5000-1:2000 persons. The repair requires careful embolization, often followed by stereotactic radiosurgery and can also include open craniotomy. Preoperatively, patients may be healthy or dramatically unstable, as 30-50% of these cases present with acute cerebral hemorrhage. One of the most important considerations for the anesthesiologist should be attempting to achieve hemodynamic stability in the face of potential increased intracranial pressure and subsequent vulnerability of the tissues to ischemic insult. Knowledge of the risks and hazards of the procedure and collaboration with specialists, including neuroradiologists, critical care physicians, and potentially neurosurgeons, ultimately form the basis for appropriate management. AVM's can lead to potentially fatal ischemic or hemorrhagic complications that may occur in up to 8% of cases. The protection of the airway, adequate monitoring, and maintaining cardiovascular and neurological stability, are critical. Further, in the setting of a radiological suite, peripheral location considerations involving equipment, monitors, and appropriate drugs and sustaining the patient's immobility during the radiological procedures, while managing potential perioperative complications are all essential

Malformación arteriovenosa del páncreas: caso clínico / Pancreatic arteriovenous malformation: Report of one case

Las malformaciones arteriovenosas del páncreas (MAVP) son poco frecuentes y generalmente asintomáticas, por lo que existen escasos reportes en la literatura. Se pueden complicar con sangrado, dolor o hipertensión portal. El diagnóstico preciso se logra con la angiografía, que también puede ser usada como terapia inicial. El tratamiento definitivo es la cirugía. Se presenta el caso de un hombre de 35 años que consultó por dolor epigástrico intenso con escasa respuesta a analgésicos. El estudio con tomografía computada, resonancia y angiografía de abdomen mostró una MAVP. Se le realizó una pancreatoduodenectomía con desaparición de los síntomas en forma completa.

Pancreatic arteriovenous malformations are uncommon and generally asymptomatic. Their complications can be bleeding, pain or portal hypertension. Angiography gives the definitive diagnosis and also can be used as the initial treatment. Surgery is the definitive treatment. We report a 35 years old with epigastric pain. Abdominal CAT scan, magnetic resonance and angiography showed a pancreatic arteriovenous malformation. He was subjected to a pancreatoduodenectomy with complete resolution of symptoms.

Aneurisma cirsoideo gigante de cuero cabelludo / Giant scalp cirsoid aneurysm

Objetivo: describir un nuevo caso de aneurisma cirsoideo gigante de cuero cabelludo. Descripción: una mujer de 34 años de edad presentó una historia de cefaleas y deformación del cuero cabelludo pulsatil que aumentaba de tamaño con la maniobra de Valsalva. La resonancia magnética, la angiorresonancia y la angiografía digital mostraron una malformación arteriovenosa de cuero cabelludo alimentada exclusivamente por la arteria carótida externa derecha. Intervención: se realizó primero un clipado transitorio de las principales arterias eferentes para reducir la masa. Luego se hizo un colgajo cutáneo exponiendo y extirpando la malformación. Se preservó la vascularización del cuero cabelludo. La evolución postoperatoria fue favorable, retrogradando los síntomas. Conclusión: existen controversias en el manejo de malformaciones complejas del cuero cabelludo pero, pensamos que la elección de la técnica quirúrgica fue apropiada para el caso presentado. Palabras clave: aneurisma cirsoideo, cuero cabelludo, malformación arteriovenosa

Objective: to describe a new case of giant cisoid aneurysm of the scalp. Description: a woman, 34 years-old, presented a history of headaches and a pulsating scalp deformity that increased in size with the Valsalva manoeuvre. Magnetic resonance, angioresonance and digital angiography showed an arteriovenous malformation of the scalp feeded exclusively by the right external carotid artery. Intervention: We performed first clipping of the main efferent arteries, reducing the flow and the mass. Through a cutaneous flap of malformation was exposed and resected. Scalp vascularisation was preserved. Postoperative outcome was uneventful and the symptoms desappeared. Conclusion: There are many controversies in the managment of scalp complex malformations but we think that the surgical technique performed was appropiate for this case. Key words: arteriovenous maformation - cirsoid aneurysm - scalp.

Malformación vascular arteriovenosa intraventricular - caso clínico / Intraventricular arteriovenous vascular malformation - clinical case

Se expone el caso clínico de una paciente de 16 años con una malformación vascular intraventricular y se discute acerca del manejo de las MAV intraventriculares.