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.

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.

Malformación arteriovenosa en cavidad oral: a propósito de un caso y revisión de la literatura / Arteriovenous malformation in the oral cavity: a case report and a literature review

Las malformaciones arteriovenosas (malformaciones A-V) de alto flujo en la cavidad oral no son una patología frecuente, existen pocos datos publicados sobre su tratamiento y sigue siendo muy controvertido debido a la alta tasa de complicaciones vitales que conlleva y la alta tasa de recurrencia. El único tratamiento curativo es la resección radical, con las consecuencias no sólo vitales, sino también desfigurativas que supone. Presentamos un caso de malformación A-V congénita en región submandibular, suelo de boca derecho y lengua, que tras varios episodios de dolor y aumento de tamaño, sin realizar ninguna medida agresiva, en el último control se observa trombosis espontánea de la misma. En este caso, la evolución con un tratamiento expectante sin cirugía, ha sido la trombosis con mejoría clínica, sin presentar nuevo episodio de aumento de tamaño, ni sangrado doce meses después, a pesar de seguir con una lesión de gran tamaño en el suelo de la boca.

he high-flow arteriovenous malformations (A-V malformations) in oral cavity are not a common disease, there are few published data on treatment and it remains highly controversial because of the high rate of vital complications and the high rate of recurrence. The only curative treatment is radical resection, with not only vital consequences but also the disfigurement involved. We present a case of congenital A-V malformation in right submandibular region, floor of the mouth and tongue. After several episodes of pain and enlargement, in the last control without any aggressive action, the lesion presented spontaneous thrombosis. In this case, despite following with a large lesion on the floor mouth, the evolution with an expectant non-surgical treatment has been the thrombosis with clinical improvement, without presenting new episode of enlargement, nor bleeding twelve months later.

Manejo odontológico da Síndrome de Rendu-Osler-Weber: relato de caso / Dental management of Rendu-Osler-Weber Syndrome: case report

A Telangiectasia Hemorrágica Hereditária (THH) ou Síndrome de Rendu-Osler-Weber (SROW) é uma displasia fibrovascular sistêmica que apresenta alterações na camada muscular da parede dos vasos sanguíneos. Isto faz com que os vasos sejam sujeitos a rupturas frequentes.Este diagnóstico pode ficar a cargo do cirurgião dentista visto que os primeiros sinais podem ser observados em boca. Podem ser identificados telangiectasias e hemorragias na mucosa bucal.Por segurança, o manejo odontológico deste paciente deve ser focado na sua condição de saúde geral. Assim, este artigo tem por objetivo a descrição de um caso clínico de tratamento odontológico de uma paciente com SROW, esclarecendo as principais características dessa síndrome e relatando as peculiaridades do seu manejo.

The Rendu-Osler-Weber Syndrome or Hereditary Hemorrhagic Telangiectasia (HHT) is a rare systemic fibrovascular dysplasia, which causes a defect in the elastic and muscle layer of the blood vessel wall, turning them more vulnerable to traumas and spontaneous ruptures. The dentists can play an important role in this diagnostic because the first signs often appear in the mouth, such as telangiectasia and recurrent bleeding in the oral mucosa. The management of this patient should be appropriate to its systemic profile to ensure the safety and effectiveness of dental treatment. This work aims to describe the main characteristics of this syndrome, its peculiarities and limitations of management during dental treatment, followed by the presentation of a clinical case.

Malformación arteriovenosa uterina: a propósito de un caso / Arteriovenous uterine malformation: a case report

Paciente de 37 años que acudió a urgencias por metrorragia, G3P1A2, el último de ellos un aborto tardío en gestación gemelar monocorial biamniótica de 20 semanas hacía dos meses. En la ecografía destaca en zona miometrial un área de marcada vascularización de 67 x 45 mm. Tras descartar proceso residual gestacional y ante la sospecha de malformación arteriovenosa uterina se solicitó resonancia magnética nuclear y angiotac, tras confirmar la malformación vascular se programó para embolización de ambas arterias uterinas con el fin de preservar la fertilidad.

A 37-year-old patient came to the emergency due to metrorrhagia, G3P1A2, the last of them a late abortion in a 20 weeks monochorionic diamniotic twin pregnancy two months ago. Ultrasound revealed an intramiometrial area of 67 x 45 mm with increased vascularization area. A diagnosis of an arteriovenous malformation was considered. In order to confirm the diagnosis pelvic magnetic resonance and angiogram was performed. Once the vascular malformation was confirmed the patient was scheduled for transcatheter arterial embolization in order to preserve fertility.

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.

Arterio Venous Malformation of Tongue Presenting with Bleeding in Pregnancy.

Arterio venous malformation (AVM) of the tongue is a rare craniofacial vascular anomaly. It occurs due to the failure of complete involution of fetal capillary bed leading to the development of abnormal connections between arteries and veins. It can present with diff erent clinical presentations and radiographic findings. It may be overlooked at birth due to its innocent appearance. Progression of the AVMs is commonlyinduced by puberty, trauma, and pregnancy. Furthermore, some forms of treatment, including ligation of arterial feeders, partial excision, incomplete arterial embolization, and laser treatment can trigger progression of quiescent AVMs. Progression of AVMs in pregnancy can sometimes lead to torrential hemorrhage. Th is can endanger the life of the mother and fetus. We report a case of a 22-year-old primigravida referred of our institution at 35 weeks 3 days gestation with intrauterine fetal death and severe anemia, due to profuse bleeding from an AVM of the tongue.

Malformación arterio-venosa uterina y hemorragia obstétrica: diagnóstico y manejo en tres casos clínicos / Uterine arteriovenous malformation and obstetric hemorrhage: diagnosis and management in three cases

Las malformaciones vasculares uterinas son muy infrecuentes y se presentan asociadas a metrorragia con riesgo vital. Su incidencia no es conocida porque las series son de pocos casos. Presentamos tres pacientes con hemorragia obstétrica cuyo estudio diagnóstico con ultrasonido y angiografía, demostró malformación arteriovenosa uterina. En un caso, dada la extensión de la lesión, el tratamiento fue con embolización bilateral de arterias uterinas seguida de histerectomía. Otras dos pacientes, por preservación de fertilidad, fueron sometidas solo a embolización. La evolución clínica y las imágenes confirmaron que los procedimientos fueron exitosos. Se discute la importancia de la interpretación correcta de las imágenes para el diagnóstico, la adaptación de los métodos terapéuticos al caso individual y el manejo multidisciplinario.

The uterine vascular malformations are very infrequent and associated to maternal hemorrhage with vital risk. The incidence is unknown because the published series are little with few cases. We described three cases with obstetrical hemorrhage and the image study with ultrasound and angiography showed an arteriovenous malformation. One patient, for the extensive lesion, was treated with bilateral embolization of uterine arteries and then, histerectomy. The others patients, for preservation her fertility, were treated with embolization only. The clinical evolution and the vascular images study confirm the success of the procedures. We discuss the importance of images analysis for the diagnosis, the individual application of therapeutic methods and a multidisciplinary approach for this scope.

An Arteriovenous Malformation in the Jejunum Mimicking a Gastrointestinal Stromal Tumor / 대한소화기학회지

A 51-year-old man visited the tertiary-care hospital with a 2-week history of dizziness and dyspnea on exertion. The initial hemoglobin level was 5.8 g/dL, without any history of hematochezia or melena. The esophagogastroduodenoscopy (EGD) was normal. During colonoscopic preparation, the patient experienced hematochezia and became hypotensive. On angiography, no extravasation of contrast media was observed. A CT scan with angiography showed a small high-density area in the jejunal lumen, suggesting extravasation of the contrast media. Capsule endoscopy was performed, and oozing bleeding was suspected in the proximal to mid jejunum. The patient was referred to our hospital. Repeated EGD and CT enterography did not reveal any significant bleeding. An antegrade double balloon endoscopy was performed, and an approximately 2-cm-sized submucosal tumor with ulceration and a non-bleeding exposed vessel was observed in the mid jejunum. The presumed diagnosis was jejunal gastrointestinal stromal tumor. The mass was surgically resected, and the final histopathological diagnosis was arteriovenous malformation.

Malformação arteriovenosa gástrica: tratamento por embolização / Gastric arteriovenous malformation: treatment by embolization

Sangramento digestivo é causa comum de internação nos serviços de emergência. Hemorragias originadas de malformações arteriovenosas no estômago foram raramente descritas na literatura. O tratamento por embolização oferece boa chance de controle do sangramento. Descrevemos caso de paciente com hematêmese e melena recorrentes, cuja angiografia identificou malformação arteriovenosa na grande curvatura do estômago. Foi realizado tratamento da hemorragia por meio de embolização arterial.

Gastrointestinal bleeding is a common cause of admission to emergency services. Hemorrhages originated from arteriovenous malformations in the stomach have been rarely described in the literature. Embolization offers a good chance for the control of bleeding. We describe the case of a patient with recurrent hematemesis and melena whose angiography identified arteriovenous malformations in the greater curvature of the stomach. The bleeding was treated by arterial embolization.

Hemangioma racemoso da retina: um caso clínico / Retinal racemose hemangioma: case report

O Hemangioma racemoso da retina é uma malformação congênita caracterizada por comunicações arteriovenovas (AV) retinianas. Em cerca de 30 por cento dos casos pode haver manifestações a nível do sistema nervoso central (SNC), denominando-se então por Síndrome de Wyburn-Mason. Os autores apresentam um caso clínico de um doente do sexo masculino de 39 anos que recorre ao serviço de Urgência devido a diminuição da acuidade visual do olho esquerdo (OE) com cerca de seis dias de evolução.Apresenta melhor acuidade visual corrigida olho direito (OD): 4/10 e olho esquerdo (OE): 2/10.À fundoscopia apresenta disco óptico com limites indefinidos, alguma palidez, dilatação e tortuosidade, AV marcada bilateralmente. O hemangioma racemoso da retina é um diagnóstico clínico raro, efectuado por meio do exame oftalmológico. É importante verificar o envolvimento do SNC por meio da realização de angiorressonância magnética nuclear. Atualmente não existe nenhum tratamento preconizado, sendo importante a realização de observações periódicas para averiguação de eventuais complicações.

Racemose Haemangioma (RH) is a congenital anomaly, characterized by retinal arteriovenous(AV) malformation. About 30 percent of patients have signs of central nervous system (CNS) involvement, which has been called the Wyburn-Mason syndrome. The authors present a 39-year-old male patient, who was admitted at the emergency room with decreased visual acuity of his left eye, for the last 6 days. Ophthalmic examination was performed and showed a visual acuity of 4/10 in his right eye and of 2/10 in the left eye. Fundoscopy examination revealed pallor of both optic discs, with blurred limits and a characteristic dilation and AV connections. RH of retina is a rare entity which can be diagnosed by ophthalmic examination. Once this diagnosis is established, involvement of CNS has to be rulled out performing an angioMRI. Currently, no specific treatment has been defined, except a periodic follow-up to prevent complications.

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.

Malformaciones arteriovenosas pulmonares: aproximación a una patología poco frecuente a partir de tres casos clínicos / Pulmonary arteriovenous malformations an approach to a rare condition based on three clinical cases

Las malformaciones arteriovenosas pulmonares (MAVP), son verdaderas fístulas vasculares de alto flujo y baja presión carentes de filtro capilar pulmonar. Consisten en la unión de una arteria a una vena mediante unsaco aneurismático. Se trata de una patología muy poco frecuente y con una alta asociación con la enfermedad telangiectasica hereditaria. A continuación se realiza un análisis de tres casos con diferentes formas clínicas de presentación.Se destaca la gran importancia de la angio tomografía como métodode diagnóstico así como la alta eficacia de la arteriografía con embolizacion para su resolución.

Malformações pulmonares congênitas / Congenital lung malformations

As malformações congênitas do pulmão são raras e variam muito na sua forma de apresentação clínica e gravidade, dependendo principalmente do grau de envolvimento pulmonar e de sua localização na cavidade torácica. Elas podem se manifestar em qualquer idade e podem ser fonte de importante morbidade e mortalidade em lactentes e crianças. Os indivíduos com malformações congênitas do pulmão podem apresentar sintomas respiratórios ao nascimento, enquanto outros podem permanecer assintomáticos por longos períodos. Atualmente, com o uso rotineiro da ultrassonografia pré-natal, vem ocorrendo um aumento no diagnóstico mais precoce dessas malformações. A manifestação clínica dessas malformações varia desde uma disfunção respiratória pós-natal imediata a um achado acidental na radiografia de tórax. O diagnóstico precoce e o tratamento imediato oferecem a possibilidade de um desenvolvimento pulmonar absolutamente normal. Quando assintomáticos, a conduta para o tratamento dos pacientes com malformações pulmonares ainda é controversa, uma vez que o prognóstico dessas afecções é imprevisível. O manejo dessas lesões depende do tipo de malformação e de sintomas. Devido ao risco de complicação, a maioria dos autores sugere a ressecção da lesão no momento em que essa é identificada. A lobectomia é o procedimento de escolha, fornecendo excelentes resultados a longo prazo. Este artigo descreve as principais malformações pulmonares congênitas, seu diagnóstico e controvérsias quanto o tratamento.

Congenital lung malformations are rare and vary widely in their clinical presentation and severity, depending mostly on the degree of lung involvement and their location in the thoracic cavity. They can manifest at any age and can be the source of significant morbidity and mortality in infants and children. Individuals with congenital lung malformations can present with respiratory symptoms at birth or can remain asymptomatic for long periods. Recently, there has been an increase in the early diagnosis of these malformations, a change that is attributable to the routine use of prenatal ultrasound. The clinical manifestation of these malformations varies from respiratory distress in the immediate postnatal period to an incidental finding on chest X-rays. Early diagnosis and prompt treatment offer the possibility of absolutely normal lung development. The treatment of asymptomatic patients with lung malformations is controversial, because the prognosis of these diseases is unpredictable. The management of these lesions depends on the type of malformation and symptoms. Because of the risk of complications, most authors recommend resection of the lesion at the time of diagnosis. Lobectomy is the procedure of choice and yields excellent long-term results. This article describes the principal congenital lung malformations, their diagnosis, and the controversies regarding treatment.

Gestação em pacientes com síndrome de Klippel-Trenaunay: relatos de dois casos / Pregnancy in patients with Klippel-Trenaunay syndrome: two case reports

Contexto: A síndrome de Klippel-Trenaunay é uma condição congênita rara caracterizada por uma tríade de anormalidades:malformações capilares, venosas e hipertrofia óssea e de tecidos moles. A gestação em pacientes portadoras dessa síndrome é historicamente desencorajada devido ao possível alto risco obstétrico pela exacerbação da síndrome, havendo apenas 21 casos descritos.Relato de caso: O presente relato apresenta a evolução obstétrica de duas gestantes portadoras de síndrome de Klippel-Trenaunay acompanhadas em São Paulo, Brasil, descrevendo as intercorrências obstétricas, o manejo dessas pacientes e sua evolução. As complicações obstétricas observadas foram: piora da assimetria corporal, sangramento dos hemangiomas cutâneos, fenômenos tromboembólicos, edema importante de membros inferiores. Não foramobservadas complicações fetais. Discussão: As complicações maternas são frequentes e podem estar associadas ao tempo de evolução da síndrome de Klippel -Trenaunay e, principalmente, à gravidade da doença. Embora possa haverexacerbação das manifestações prévias da síndrome, com hemorragia, coagulação intravascular disseminada, eventostromboembólicos e dor, em geral, observam-se complicações menores, cujo manejo conservador permite boa evolução obstétrica, exceção feita a um caso de malformação congênita.Conclusão: Não há evidências com base na literatura preexistente para desencorajar a gestação nessas pacientes. O entendimento da síndrome e cuidado multidisciplinar pré-natal, intra e pós-parto mostram-se fundamentais para o bom desfecho da gestação.

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.