Klippel-Trenaunay Syndrome.

Klippel-Trenaunay syndrome or KTS is a complex vascular syndrome associated with overgrowth occurring as a result of somatic mutations in the PIK3CA gene. Patients are diagnosed on the basis of physical findings, sometimes with supportive imaging, of commonly a segmental anomaly with a cutaneous port-wine stain, lymphatic and venous malformations and overgrowth. The severity of the component vascular malformations and the degree of overgrowth varies from patient to patient which demands care given by a multi-professional team with regular follow-up in a specialist clinic. Some patients may present with acute life-threatening problems, often as a result of veno-thromboembolic events (VTEs) especially following surgical and invasive radiological procedures. Awareness of such problems is vital and prophylactic measures to reduce such risks are paramount. The interventional radiologist is vital to the care team as he/she can undertake procedures including endovascular closure of significant venous anomalies which predispose to such VTEs. Although these procedures can be lengthy and complex, they can now provide a minimally invasive means to reduce the risk from life-threatening and sometimes fatal VTEs. The results however from such interventions will require long-term studies which to date are unavailable.

Epidemiological Features of Nontraumatic Spontaneous Subarachnoid Hemorrhage in China: A Nationwide Hospital-based Multicenter Study.

BACKGROUND: Nontraumatic spontaneous subarachnoid hemorrhage (SAH) is associated with a high mortality. This study was conducted to investigate the epidemiological features of nontraumatic spontaneous SAH in China. METHODS: From January 2006 to December 2008, the clinical data of patients with nontraumatic SAH from 32 major neurosurgical centers of China were evaluated. Emergent digital subtraction angiography (DSA) was performed for the diagnosis of SAH sources in the acute stage of SAH (≤3 days). The results and complications of emergent DSA were analyzed. Repeated DSA or computed tomography angiography (CTA) was suggested 2 weeks later if initial angiographic result was negative. RESULTS: A total of 2562 patients were enrolled, including 81.4% of aneurysmal SAH and 18.6% of nonaneurysmal SAH. The total complication rate of emergent DSA was 3.9% without any mortality. Among the patients with aneurysmal SAH, 321 cases (15.4%) had multiple aneurysms, and a total of 2435 aneurysms were detected. The aneurysms mostly originated from the anterior communicating artery (30.1%), posterior communicating artery (28.7%), and middle cerebral artery (15.9%). Among the nonaneurysmal SAH cases, 76.5% (n = 365) had negative initial DSA, including 62 cases with peri-mesencephalic nonaneurysmal SAH (PNSAH). Repeated DSA or CTA was performed in 252 patients with negative initial DSA, including 45 PNSAH cases. Among them, the repeated angiographic results remained negative in 45 PNSAH cases, but 28 (13.5%) intracranial aneurysms were detected in the remaining 207 cases. In addition, brain arteriovenous malformation (AVM, 7.5%), Moyamoya disease (7.3%), stenosis or sclerosis of the cerebral artery (2.7%), and dural arteriovenous fistula or carotid cavernous fistula (2.3%) were the major causes of nonaneurysmal SAH. CONCLUSIONS: DSA can be performed safely for pathological diagnosis in the acute stage of SAH. Ruptured intracranial aneurysms, AVM, and Moyamoya disease are the major causes of SAH detected by emergent DSA in China.

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.

First 1,000 Cases of Gamma Knife Surgery for Various Intracranial Disorders in LSU Health-Shreveport: Radiological and Clinical Outcome.

OBJECTIVE: Gamma knife radiosurgery (GKRS) has emerged as an important therapeutic alternative for different intracranial lesions. We have reviewed our institution's first 1,000 cases of radiosurgeries. MATERIALS AND METHODS: We performed a retrospective review (2000-2013) of 1,017 radiosurgeries in 911 patients with various intracranial lesions including vestibular schwannoma (82), meningioma (136), metastatic brain tumors (298), astrocytoma (49), pituitary adenoma (92), arteriovenous malformation (85) and trigeminal neuralgia (169). RESULTS: GKRS in different intracranial lesions showed significant variations in outcome and complications. Overall, the local tumor growth control for benign and malignant tumors was 89 percent and 70 percent respectively. The rate of obliteration of arteriovenous malformation nidus was 79 percent. The complete and partial relief of pain in the patients with trigeminal neuralgia was 55.6 percent and 22.4 percent respectively. CONCLUSION: At recent follow-up, GKRS showed good control of different tumor growth, obliteration of AVM nidus and remission of trigeminal neuralgia pain, good overall and progression free survival rate, possible preservation of neurological functions, lesser number of complications, and improvement of quality of life. Therefore, GKRS is an important treatment option for patients with different benign intracranial tumors, AVM and trigeminal neuralgia. However, GKRS is not effective for recurrent malignant tumors in the brain.

Factors predictive of obliteration after arteriovenous malformation radiosurgery.

OBJECTIVE: To investigate predictive factors of complete obliteration following treatment with linac-based stereotactic radiosurgery for intracerebral arteriovenous malformations. METHODS: Archived plans for 48 patients treated at the British Columbia Cancer Agency and who underwent post-treatment digital subtraction angiography to assess obliteration were studied. Actuarial estimates of obliteration were calculated using the Kaplan-Meier method. Univariate and multivariate Cox proportional hazards models were used for analysis of incidence of obliteration. Log-rank test was used to search for parameters associated with obliteration. RESULTS: Complete nidus obliteration was achieved in 38/48 patients (79.2%). Actuarial rate of obliteration was 75.9% at 4 years (95% confidence interval 63.1%-88.6%). On univariate analysis, prescribed dose to the margin (p=0.002) and dose to isocentre (p=0.022) showed statistical significance. No parameters were significant in a multivariate model. According to the log-rank test, prescribed dose to the margin of >20 Gy (p=0.004) and dose to the isocentre of >25 Gy (p=0.004) were associated with obliteration. CONCLUSION: Reported series in the literature suggest a number of different factors are predictive of complete obliteration of arteriovenous malformations following radiosurgery. However, differing definitions of volume and complete obliteration makes direct comparison between series difficult. This study demonstrates that complete obliteration of the nidus following linear accelerator-based stereotactic radiosurgery for arteriovenous malformations appears to be most closely related to the prescribed marginal dose. In particular, a marginal dose of >20 Gy is strongly associated with obtaining complete obliteration of the nidus.

Natural history and outcome of hepatic vascular malformations in a large cohort of patients with hereditary hemorrhagic teleangiectasia.

BACKGROUND: Hereditary hemorrhagic telangiectasia is a genetic disease characterized by teleangiectasias involving virtually every organ. There are limited data in the literature regarding the natural history of liver vascular malformations in hemorrhagic telangiectasia and their associated morbidity and mortality. AIM: This prospective cohort study sought to assess the outcome of liver involvement in hereditary hemorrhagic telangiectasia patients. METHODS: We analyzed 16 years of surveillance data from a tertiary hereditary hemorrhagic telangiectasia referral center in Italy. We considered for inclusion in this study 502 consecutive Italian patients at risk of hereditary hemorrhagic telangiectasia who presented at the hereditary hemorrhagic telangiectasia referral center and underwent a multidisciplinary screening protocol for the diagnosis of hereditary hemorrhagic telangiectasia. Of the 502 individuals assessed in the center, 154 had hepatic vascular malformations and were the subject of the study; 198 patients with hereditary hemorrhagic telangiectasia and without hepatic vascular malformations were the controls. Additionally, we report the response to treatment of patients with complicated hepatic vascular malformations. RESULTS: The 154 patients were included and followed for a median period of 44 months (range 12-181); of these, eight (5.2%) died from VM-related complications and 39 (25.3%) experienced complications. The average incidence rates of death and complications were 1.1 and 3.6 per 100 person-years, respectively. The median overall survival and event-free survival after diagnosis were 175 and 90 months, respectively. The rate of complete response to therapy was 63%. CONCLUSIONS: This study shows that substantial morbidity and mortality are associated with liver vascular malformations in hereditary hemorrhagic telangiectasia patients.

Terson's syndrome as a prognostic factor for mortality of spontaneous subarachnoid haemorrhage.

PURPOSE: To evaluate the prognosis of mortality in patients with spontaneous subarachnoid haemorrhage associated with Terson's syndrome. METHODS: A prospective, consecutive case series study was conducted in patients admitted to the emergency room with a diagnosis of acute subarachnoid haemorrhage. After a complete neurological examination, funduscopic examination using binocular indirect ophthalmoscopy under mydriasis was performed upon admission and at days 3, 7, 30 and 60 after the onset. In all cases, the diagnosis of intracranial bleeding was made by computerized tomography, and the clinical condition was graded according to the Hunt & Hess and Glasgow coma scales. RESULTS: Forty-seven patients with the diagnosis of subarachnoid haemorrhage were enrolled. Forty-four cases were associated with a ruptured aneurysm and three cases with arterio-venous malformation. Fourteen patients (29%) were diagnosed with Terson's syndrome. Seven patients (50%) with Terson's syndrome died, whereas death occurred in three patients (9%) without Terson's syndrome (p = 0.002). Ocular findings in Terson's syndrome were preretinal, intraretinal, sub-retinal and vitreous haemorrhage. Associated ocular findings included third-nerve palsy, papilloedema and subconjunctival haemorrhage. CONCLUSION: The presence of Terson's syndrome was associated with an increased mortality rate (50% versus 9%; p < 0.01). Therefore, patients with the diagnosis of intracranial haemorrhage should be submitted to a funduscopic examination, because the presence of intraocular haemorrhage is an important life-threatening prognostic factor.

Stereotactic radiotherapy and radiosurgery in pediatric patients: analysis of indications and outcome.

INTRODUCTION: We describe indications, outcomes, and risk profiles of fractionated stereotactic radiotherapy (SRT) and single fraction "radiosurgery" (SRS) in pediatric patients compared to the adult population and evaluate the causal role of SRS and SRT in inducing new neurological complications. MATERIALS AND METHODS: Six children with AVMs and 12 children with neoplastic diseases were prospectively followed for >2 years after SRT/S. The survival, control of pathology, and specified neurological complications were analyzed. In tumor patients, the median overall survival time was 45 months (range 5-103) and the median progression free survival time was 35 months (range 5-98). RESULTS: Control or regression of the tumor was obtained in 83% of patients with neoplastic disease. Three patients with malignant tumors died from disease progression. In AVMs the median time follow up was 52 months (range 27-100). All AVMs were obliterated. New neurological deficits occurred in 67%. SRT/S was considered the direct cause in 25%. All the neurological deficiencies related to SRT/S were focal and related to the irradiated areas. In tumor patients, midline lesions, malignant diagnosis, and additional treatment with surgery, chemotherapy, and craniospinal irradiation seemed to increase the risk of new deficits after SRT/S. In AVM patients, a high Spetzler-Martin grade seemed to carry a higher complication risk. CONCLUSION: The risk of uncontrolled tumor disease or the risk of hemorrhage of non-obliterated AVM must be balanced against the overall risks and benefits of SRT/S. Following SRT/S, the risk of worsening pre-existing deficits is relatively high. The risk of inducing new long-term deficits is relatively low.

Long-term outcome of patients with hereditary hemorrhagic telangiectasia and severe hepatic involvement after orthotopic liver transplantation: a single-center study.

Hepatic involvement occurs in up to 74% of patients with hereditary hemorrhagic telangiectasia (HHT) and is characterized by a spectrum of arteriovenous malformations. Three different types of intrahepatic shunting may be present: hepatic artery to hepatic veins, hepatic artery to portal vein, and portal vein to hepatic vein. Hepatic involvement in HHT may lead to biliary ischemia, portal hypertension, or high-output cardiac failure (HOCF). Orthotopic liver transplantation (OLT) has been proposed as the only definitive curative treatment. The aim of this study was to evaluate the long-term outcome of patients with hepatic involvement due to HHT after OLT with respect to mortality, cardiac and hepatic status, epistaxis, and quality of life. Patients with HHT and severe hepatic vascular malformations who underwent OLT in the Lyon Liver Transplant Unit (LLTU) from 1993 to 2007 were followed at the LLTU and the French Reference Center for HHT. Quality of life was evaluated with the Short Form 36 questionnaire. There were 13 patients who fulfilled the entry criteria of the study (12 women and 1 man). The mean age at the time of OLT was 51.8 years (range = 33-65 years). Indications for OLT were cardiac failure (n = 9), biliary necrosis (n = 2), both cardiac failure and biliary necrosis (n = 1), and hemobilia (n = 1). The mean duration of follow-up was 109 months (range = 1-200 months). Twelve patients (92.3%) are still alive. For the 9 patients with HOCF, the mean cardiac index decreased from 5.4 L/minute/m(2) before OLT to 3.0 L/minute/m(2) after OLT. No severe hepatic complications were observed after OLT. Nine of the surviving patients (75%) experienced dramatic improvements in epistaxis and quality of life, including an ability to undertake more physical activity. In conclusion, OLT is an important therapeutic option for patients with HHT who have severe hepatic involvement. In the reported cohort, the mortality after OLT for this indication was low.

Natural history of arteriovenous malformations: presentation, risk of hemorrhage and mortality.

Brain arteriovenous malformations (AVMs) are highly complex vascular lesions, which may become symptomatic by causing hemorrhagic stroke, epilepsy, chronic headache and/or focal neurological deficits. An increasing number of AVMs are also found incidentally in MRI and CT scans performed for other reasons. It is mandatory to understand the natural history and prognosis of these malformations when evaluating the risks that may be associated with the treatment. In this review, we discuss the symptomatology, hemorrhage risk and mortality associated with their natural course.

Outcome after spontaneous and arteriovenous malformation-related intracerebral haemorrhage: population-based studies.

Spontaneous (non-traumatic) intracerebral haemorrhage (ICH) has a high case-fatality and leaves many survivors disabled. Clinical characteristics and outcome seem to vary according to the cause of ICH, but population-based comparisons are scarce. We studied two prospective, population-based cohorts to determine differences in outcome [case-fatality and modified Rankin Scale (mRS)] after incident ICH due to brain arteriovenous malformations (AVM) [Scottish Intracranial Vascular Malformation Study (SIVMS), n = 90] and spontaneous ICH [Oxford Vascular Study (OXVASC), n = 60]. Patients with AVM-ICH were younger, had lower pre-stroke and admission blood pressure (BP), higher admission Glasgow Coma Scale (GCS) and were more likely to have an ICH in a lobar location than patients with spontaneous ICH (sICH). Case fatality throughout 2-year follow-up was greater following sICH than AVM-ICH [34/56 (61%) versus 11/90 (12%) at 1 year, odds ratio (OR) 11 (95% Confidence Interval (CI) 5-25)], as was death or dependence (mRS >or= 3) [40/48 (83%) versus 26/65 (40%) at 1 year, OR 8 (3-19)]. Differences in outcome persisted following stratification by age and sensitivity analyses. In multivariable analyses of 1 year outcome, independent predictors of death were sICH (OR 21, 4-104) and increasing ICH volume (OR 1.03, 1.01-1.05), and independent predictors of death or dependence were sICH (OR 11, 2-62) and GCS on admission (OR 0.79, 0.67-0.93). Outcome after AVM-ICH is better than after sICH, independent of patient age and other known predictors of ICH outcome.

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...

[Intracranial aneurysm, arteriovenous malformation, and carotid artery stenosis : endovascular prophylactic therapy]. / Intrakranielle Aneurysmen, arteriovenöse Malformationen und Karotisstenosen : Aktuelle Ubersicht zur interventionellen Präventionstherapie.

The treatment of acute cerebrovascular disease plays a prominent role in clinical routine. However, prophylactic therapy of cerebrovascular disease including endovascular options is becoming more and more important. With particular regard to endovascular therapies, this summary deals with the natural course of cerebrovascular diseases and different treatment strategies for asymptomatic intracranial aneurysms, cerebral arteriovenous malformations, and extracranial carotid artery stenoses.

Estimates of maternal risks of pregnancy for women with hereditary haemorrhagic telangiectasia (Osler-Weber-Rendu syndrome): suggested approach for obstetric services.

OBJECTIVES: Hereditary haemorrhagic telangiectasia (HHT) affects 1 in 5-8000 individuals. Pregnancy outcomes are rarely reported. The major reason is that most women do not have their HHT diagnosed prior to pregnancy. Using a large well-characterised series, we studied all pregnancies known to have occurred in HHT-affected women, whether or not their diagnosis was known at the time of pregnancy. Our aim was to estimate rates and types of major complications of HHT in pregnancy, to guide management decisions. DESIGN: Cohort study, with prospective, retrospective and familial components. SETTING/POPULATION: Tertiary referral centre population. METHODS: All 262 pregnancies in the 111 women with HHT and pulmonary arteriovenous malformations (PAVMs) reviewed between 1999 and 2005 were studied. Eighty-two women (74%) did not have a diagnosis of HHT/PAVM at the time of pregnancy. 222 pregnancies in their 86 HHT-affected relatives were also studied. MAIN OUTCOME MEASURES: PAVM bleed, stroke and maternal death. RESULTS: Thirteen women experienced life-threatening events during pregnancy: 1.0% (95% CI 0.1-1.9) of pregnancies resulted in a major PAVM bleed; 1.2% (0.3-2.2%) in stroke (not all were HHT related); and 1.0% (0.13-1.9%) in maternal death. All deaths occurred in women previously considered well. In women experiencing a life-threatening event, prior awareness of HHT or PAVM diagnosis was associated with improved survival (P = 0.041, Fisher's exact test). CONCLUSIONS: Most HHT pregnancies proceed normally. Rare major complications, and improved survival outcome following prior recognition, means that pregnancy in a woman with HHT should be considered high risk. Recommendations for pregnancy management are provided.

Long-term follow-up after embolization of pulmonary arteriovenous malformations with detachable silicone balloons.

Long-term follow-up results after embolization of 13 pulmonary arteriovenous malformations in 10 patients by use of 14 detachable silicone balloons are given. Patients were followed for a mean of 99 months (range, 63-123 months) with chest x-rays and for a mean of 62 months (range, 3-101 months) with pulmonary angiography. Fifty-four percent of the balloons were deflated at latest radiographic chest film follow-up, but at pulmonary angiographic follow-up all embolized malformations were without flow irrespective of whether or not the balloons were visible. Detachable silicone balloons are not available anymore, but use of these balloons for embolization of pulmonary arteriovenous malformations has been shown to be a safe and precise method, with immediate occlusion of the feeding artery and with long-lasting occlusion, even though many balloons deflate with time, leaving a fibrotic scar replacing the pulmonary arteriovenous malformation. No case of recanalization has been discovered, and these results seem to justify a reduced number of controls of these balloon-embolized malformations.

[Embolization of localized pulmonary arteriovenous malformations in adults]. / Embolisation des malformations artérioveineuses pulmonaires localisées de l'adulte.

OBJECTIVES: To report our experience using embolization in managing localized pulmonary arteriovenous malformations in adults. MATERIAL: and methods. All patients presenting with localized pulmonary arteriovenous malformations treated with embolization were included in the study. Clinical presentation (respiratory symptoms and previous history of paradoxical embolism) and the characteristics of pulmonary arteriovenous malformations (single or multiple, location, diameter of the afferent artery and simple or complex angioarchitecture) before embolization were analyzed. The details of the procedure, including the number of pulmonary arteriovenous malformations embolized, the number of coils used, and the type of intraoperative complications were recorded. Postembolization clinical and imaging follow-up were described. RESULTS: Forty-two patients (26 women, 16 men; mean age, 45 years), including 36 with hereditary hemorrhagic telangiectasia were treated with embolization. Twenty-two patients (53%) were dyspneic and 12 (29%) had a previous history of paradoxical embolism prior to embolization. Forty-seven procedures were carried out on a total of 99 pulmonary arteriovenous malformations (mean, 2.3 per patient), using 530 coils (12.6 per patient). The pulmonary arteriovenous malformations were located in the lower lobes in 60% of cases and a simple architecture was reported in 81% of cases. The average diameter of the afferent artery was 6mm. No preoperative complications were reported. After embolization, two patients (5%) presented with a paradoxical embolism and five patients out of 22 (23%) remained dyspneic. The rate of complete occlusion of treated arteriovenous malformations was 92% using computer tomography. CONCLUSION: Embolization is a highly effective and safe technique for treating pulmonary arteriovenous malformations. Improvement in dyspnea and prevention of paradoxical embolism can be expected. A high technical success rate can be obtained by experienced interventional radiologists.

Treatment for extracranial arteriovenous malformations of the head and neck.

CONCLUSIONS: For extracranial arteriovenous malformations of the head and neck (HNAVMs), in which the nidus was accessible via the percutaneous route, ethanol sclerotherapy was a feasible and safe first-line treatment, although successful outcomes were obtained for only about half of the subjects. For other HNAVMs, surgical excision with embolization may be the best choice of treatment. OBJECTIVE: To suggest a treatment protocol for patients with HNAVMs by comparing the treatment outcomes and complications of ethanol sclerotherapy with those of surgical excision combined with embolization. MATERIAL AND METHODS: Twenty patients who had been diagnosed with HNAVM and treated between 1995 and 2002 were retrospectively reviewed. Ethanol sclerotherapy, surgical excision and embolization were used as treatments, either alone or in various combinations. The treatment outcomes and complications with the different modalities were analyzed. RESULTS: Ethanol sclerotherapy was used for 12 cases, with a success rate of 50.0% and a permanent complication rate of 8.3%. Surgical excision combined with embolization was used for 13 patients. Although all patients achieved successful resolution of their HNAVM after surgical excision, 15.4% suffered from permanent complications. In total, 16/20 patients (80.0%) eventually achieved a > or = 75% reduction in the size of their lesions.

Pharmacological therapy of vascular malformations of the gastrointestinal tract.

Vascular malformation (AVM) in the gastrointestinal tract is an uncommon, but not rare, cause of bleeding and iron deficiency anemia, especially in an aging population. While endoscopic coagulative therapy is the method of choice for controlling bleeding, a substantial number of cases require additional therapy. Adjunctive or even primary phamacotherapy may be indicated in recurrent bleeding. However, there is little evidence-based proof of efficacy for any agent. The bulk of support is derived from anecdotal reports or case series. The present review compares the outcome of AVM after no intervention, coagulative therapy or focus on pharmacological agents. Most of the literature encompasses two common AVMs, angiodysplasia and hereditary hemorrhagic telangiectasia. Similarly, the bulk of information evaluates two therapies, hormones (estrogen and progesterone) and the somatostatin analogue octreotide. Of these, the former is the only therapy evaluated in randomized trials, and the results are conflicting without clear guidelines. The latter therapy has been reported only as case reports and case series without prospective trials. In addition, other anecdotally used medications are discussed.