Multiple Arteriovenous Malformations Research Articles

Pulmonary arteriovenous malformations in Rendu-Osler-Weber syndrome.

Rendu-Osler-Weber syndrome, also known as hereditary hemorrhagic telangiectasia, is an autosomal dominant hereditary disorder. It is characterized by presence of multiple arteriovenous malformations (AVMs) and telangiectasias. This article reports two cases of patients with Rendu-Osler-Weber syndrome who had pulmonary AVMs and underwent successful endovascular treatment. A brief review of the literature shows that up to 50% of patients with the syndrome have pulmonary AVMs and there is usually a positive family history in these patients. These pulmonary AVMs are multiple in 30% of cases and are associated with the most severe disease complications. Most patients are asymptomatic, even in the presence of AVMs with right-left shunts. When these shunts exceed 25% of the total blood volume, dyspnea, cyanosis, digital clubbing, and extracardiac murmurs may occur. Endovascular treatment is safe and offers control of complications from hereditary hemorrhagic telangiectasia and is currently the treatment of choice for these lesions.

Abstract 285: Intraventricular And Subarachnoid Hemorrhage Secondary To Arteriovenous Malformation, A Diagnostic Neurologic Manifestation Of Osler‐Weber‐Rendu Disease

Introduction Osler‐Weber‐Rendu disease, also known as hereditary hemorrhagic telangiectasia (HTT), is a developmental vascular disease characterized by multiple arteriovenous malformations (AVMs) due to genetic defects in endothelial angiogenesis pathways. HHT is a clinical diagnosis based on the fulfillment of the Curacao Diagnostic Criteria, including involvement of internal organs with AVMs, significant recurrent nosebleeds, telangiectasias, and family history of HHT. HHT has a prevalence of 1.5 to 2 people/10,000, although this is considered a falsely low representation due to missed diagnosis and variable penetrance. Autosomal dominantly inherited heterozygous mutations disrupt the TGF‐beta‐mediated pathways causing aberrant blood vessel development with fragility and AVM formation. There are two forms of HHT: (HHT1) has a higher incidence in women and typically involves pulmonary and cerebral arteriovenous malformations (AVM). Mutations in the endoglin gene are thought to be responsible for HTT1 and comprise approximately 61% of the cases. HTT2 is associated with a higher rate of liver AVMs, involves a mutation in Activin A Receptor‐Like Type 1 (ACVRL1), and is responsible for approximately 37% of cases. Other genetic mutations include SMAD4 and GDF2, which are transmitter and binding proteins and occur about 2% of the time. Methods Case report and review of literature Results Case:A 52‐year‐old woman with a large frontoparietal arteriovenous malformation originally found at the age of 17, with a history of frequent epistaxis, mucocutaneous telangiectasias and two children with epistaxis and telangiectasias. Her diagnosis of HHT was made at her third hjospitalization due to a non‐traumatic subarachnoid hemorrhage. A Chest CT angiography and a CT angiography of the intra and extracranial vasculature demonstrated an extensive right holohemispheric cerebral AVM, an unruptured 8‐mm basilar artery aneurysm, and multiple enlarged pulmonary arteries with narrow inlet and outlets concerning for pulmonary AVMs. A subsequent digital subtraction angiogram (DSA) of the brain confirmed the extensive holohemispheric AVM, unruptured basilar artery aneurism with subarachnoid hemorrhage originating from the AVM. On examination she had multiple lingual, and hard/soft palate telangiectasias and an extensive anterior pedal telangiectasia. Given the heavy cerebral AVM burden with multiple cerebral artery feeders, the AVM angiosclerosis and basilar artery aneurysm embolization were deferred. The patient proceeded under medical management alone with a plan for personal and familial genetic testing. Conclusion HHT is a rare cause of cerebral AVMs at high risk of spontaneous bleeding. Managing these AVMs can be a challenge, given the extent and location of these. However, diagnosis of HHT can inform prognosis and guide medical management due to improved prognosis for HHT‐related AVMs and aneurysms compared to non‐HHT AVMs.

Multiple Pulmonary Arteriovenous Malformations in a Young Patient Presenting with Stroke

Multiple Pulmonary Arteriovenous Malformations in a Young Patient Presenting with Stroke

Anesthetic management of a patient with Osler-Weber-Rendu syndrome with multiple pulmonary arteriovenous malformations and pheochromocytoma for femoral artificial bone replacement: a case report

BackgroundOsler-Weber-Rendu syndrome is characterized by mucocutaneous telangiectasia and arteriovenous malformations in organs. Anesthesia for patients with Osler-Weber-Rendu syndrome is challenging due to complications and physiological changes.Case presentationThe case was a 49-year-old female with Osler-Weber-Rendu syndrome, multiple pulmonary arteriovenous malformations and pheochromocytoma who presented for femoral bone head fracture with metastatic adenocarcinoma. The patient was scheduled to undergo bone tumor resection and artificial bone replacement, being positioned laterally with a planned operation duration of 5 h. Anesthesia was managed with spinal and epidural anesthesia, combined with sedation by sevoflurane using a supraglottic airway (SGA) device under spontaneous breathing. Her intraoperative and postoperative courses were uneventful.ConclusionNeuraxial anesthesia combined with general anesthesia using an SGA device to maintain spontaneous ventilation in order to minimize the risk of rupture of pulmonary arteriovenous malformations could be an option.

External validation of the R2eD AVM scoring system to assess rupture risk in pediatric AVM patients.

The assessment of hemorrhagic risk is of central importance in the management of pediatric brain arteriovenous malformations (AVMs). A recently published scoring system, the R2eD AVM (race, exclusive deep location or not, AVM size < 3 or ≥ 3, venous drainage exclusively deep or not, and monoarterial feeding or >1 arterial feeders) considers patient race and AVM location, size, venous drainage, and arterial supply and was successfully externally validated for use in adults to predict the risk of AVM hemorrhagic presentation. In this study the authors sought to externally validate the R2eD AVM score for prediction of hemorrhagic risk in the pediatric AVM population. A pediatric database at Boston Children's Hospital was retrospectively reviewed for all patients with a diagnosis of intracranial AVM. Exclusion criteria included age > 21 years, multiple AVMs, and incomplete clinical/radiographic data. Demographic data and R2eD AVM score calculations were collected for all patients. Univariate binary logistic regression and multivariate stepwise backward elimination models were used to determine factors associated with hemorrhagic presentation. A total of 212 AVMs were identified in 212 patients with vascular anomalies from 1995 to 2021, and 144 patients met the inclusion criteria (74 [51.4%] male, 111 [77.1%] White), of whom 87 (60.4%) patients presented with rupture and 122 (84.7%) underwent resection. The mean age at surgery was 12 years (range 3 months to 20 years). The R2eD AVM scoring system was applied to each patient. The score components of size < 3 cm and exclusive deep venous drainage were significantly associated with rupture risk (p < 0.05). The complete model for the R2eD AVM score had an area under the curve of 0.671 (95% CI 0.586-0.757). This study demonstrated poor external validity of the R2eD AVM score in predicting pediatric AVM rupture risk. The results suggest that future studies are warranted to determine a better scoring method to capture pediatric rupture risk, given the significant differences in clinical presentation in pediatric compared with adult AVM patients.

A Rare Case: Multiple Mandibular Arteriovenous Malformation with High Output: May a Tooth Extraction Alone Turn to a Catastrophic Condition?

A Rare Case: Multiple Mandibular Arteriovenous Malformation with High Output: May a Tooth Extraction Alone Turn to a Catastrophic Condition?

P184: A case of multiple hemangiomas and arteriovenous malformations in a patient with known Greig cephalopolysyndactyly syndrome

P184: A case of multiple hemangiomas and arteriovenous malformations in a patient with known Greig cephalopolysyndactyly syndrome

Образование в хиазмально-селлярной области у пациента с фиброзной дисплазией в практике оториноларинголога (клиническое наблюдение)

Cerebral tumors at their onset are asymptomatic or manifest in minor nonspecific signs. Nosebleed is considered one of the most common types of ENT emergency, a manifestation of brain tumors and postoperative complications. The article presents a case of a 35-year-old male patient who visited an otorhinolaryngologist with complaints of nosebleeds. Anamnesis showed that the patient had previously undergone transsphenoidal tumor resection due to the tumor in the chiasmo-sellar region detected by computed tomography (CT). Tumor nature at that moment could not be established due to intraoperative hemorrhage. CT angiography was performed to clarify the nature of hemorrhage, which revealed multiple brain arteriovenous malformations. CT also revealed a pattern of the patient: the diffuse bone lesion of the skull and facial skeleton, characteristic of fibrous dysplasia, which was first diagnosed at the age of 12. The article discusses further tactics of patient management with recurrent nosebleeds and breathing difficulties, taking into account a multidisciplinary approach. KEYWORDS: arteriovenous malformations, nasal septal perforation, tumor of the chiasmo-cellular region, ethmoidal air cells, fibrous dysplasia, biopsy. FOR CITATION: Khapaeva M.M., Zhuboev R.T., Khakuasheva I.A. Tumor in the chiasmo-sellar region of a patient with fibrotic dysplasia: practice of an otorhinolaryngologist (case report). Russian Medical Inquiry. 2023;7(8). (in Russ.). DOI: 10.32364/2587-6821-2023- 7-8-10.

“Rendezvous with Osler Weber Rendu” - Closure of Multiple Pulmonary Arteriovenous Malformations with Amplatzer Vascular Plug II in Hereditary Hemorrhagic Telangiectasia

“Rendezvous with Osler Weber Rendu” - Closure of Multiple Pulmonary Arteriovenous Malformations with Amplatzer Vascular Plug II in Hereditary Hemorrhagic Telangiectasia

Prenatal diagnosis of multiple intracranial arteriovenous malformations.

Ultrasound in Obstetrics & GynecologyAccepted Articles Picture of the Month Prenatal diagnosis of multiple intracranial arteriovenous malformations V. Krishnan, Corresponding Author V. Krishnan drvivekkrishnan@gmail.com orcid.org/0000-0003-0767-0982 Fetal Medicine & Perinatology, Amrita Centre of Excellence in Fetal Care, AMRITA Institute of Medical Sciences, Kochi, IndiaCorrespondence to:Dr V. Krishnan MS, DGO, DNB, PDF (Fetal Med.), MNAMS. Clinical Associate Professor & Head, Division of Fetal Medicine & Perinatology, Amrita Institute of Medical Sciences, Ponekkara PO Kochi, Kerala, India Pin: 682 041, Email: drvivekkrishnan@gmail.comSearch for more papers by this authorR. Elayedatt, R. Elayedatt orcid.org/0000-0002-5985-4890 Fetal Medicine & Perinatology, Amrita Centre of Excellence in Fetal Care, AMRITA Institute of Medical Sciences, Kochi, IndiaSearch for more papers by this authorM. Hausman-Kedem, M. Hausman-Kedem orcid.org/0000-0001-5315-3738 Pediatric Neurology Institute, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, IsraelSearch for more papers by this authorK. K. Haratz, K. K. Haratz orcid.org/0000-0001-9868-3548 Senior Consultant, Ultrasound in ObGyn Unit - Lis Maternity Center - Tel Aviv Medical CenterSearch for more papers by this author V. Krishnan, Corresponding Author V. Krishnan drvivekkrishnan@gmail.com orcid.org/0000-0003-0767-0982 Fetal Medicine & Perinatology, Amrita Centre of Excellence in Fetal Care, AMRITA Institute of Medical Sciences, Kochi, IndiaCorrespondence to:Dr V. Krishnan MS, DGO, DNB, PDF (Fetal Med.), MNAMS. Clinical Associate Professor & Head, Division of Fetal Medicine & Perinatology, Amrita Institute of Medical Sciences, Ponekkara PO Kochi, Kerala, India Pin: 682 041, Email: drvivekkrishnan@gmail.comSearch for more papers by this authorR. Elayedatt, R. Elayedatt orcid.org/0000-0002-5985-4890 Fetal Medicine & Perinatology, Amrita Centre of Excellence in Fetal Care, AMRITA Institute of Medical Sciences, Kochi, IndiaSearch for more papers by this authorM. Hausman-Kedem, M. Hausman-Kedem orcid.org/0000-0001-5315-3738 Pediatric Neurology Institute, Tel Aviv Sourasky Medical Center, Sackler Faculty of Medicine, Tel Aviv University, IsraelSearch for more papers by this authorK. K. Haratz, K. K. Haratz orcid.org/0000-0001-9868-3548 Senior Consultant, Ultrasound in ObGyn Unit - Lis Maternity Center - Tel Aviv Medical CenterSearch for more papers by this author First published: 02 February 2022 https://doi.org/10.1002/uog.24871 This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process, which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1002/uog.24871. AboutPDF ToolsExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Accepted ArticlesAccepted, unedited articles published online and citable. The final edited and typeset version of record will appear in the future. RelatedInformation

Hereditary Haemorrhagic Telangiectasia of Nasal Mucosa- A Case Report

Hereditary Haemorrhagic Telangiectasia (HHT) is also known as Osler-Weber-Rendu syndrome is a development disorder of blood vessels. It is characterised by multiple telangiectases and Arteriovenous Malformations (AVMS) that cause direct connections between arteries and veins bypassing intermediate capillaries. The most prominent sites for telangiectasia are the lips, tongue, face, mucosa of the nose, oral cavity, and gastrointestinal tract. Telangiectases can rupture and bleed easily due to their thin walls, narrow tortuous courses, and proximity to the skin's surface or a mucous membrane. Depending on the organ affected, these symptoms include repeated persistent epistaxis, skin lesions, haemoptysis, gastrointestinal haemorrhage, and stroke. Complications of HHT include bleeding, anaemia, followed by stroke, pulmonary AVM, Transient Ischaemic Attack (TIA), hypovolemic shock due to severe intractable bleed, eventually leading to high output failure. Present case is of a 45-year-old female who presented with complaints of recurrent epistaxis. Diagnostic nasal endoscopy showed multiple telengiectasic spots which were cauterised with trichloroacetic acid. However recurrent episodes of bleed were encountered.

Feasibility of Single-Shot Whole Thoracic Time-Resolved MR Angiography to Evaluate Patients with Multiple Pulmonary Arteriovenous Malformations.

ObjectiveTo evaluate the feasibility of single-shot whole thoracic time-resolved MR angiography (TR-MRA) to identify the feeding arteries of pulmonary arteriovenous malformations (PAVMs) and reperfusion of the lesion after embolization in patients with multiple PAVMs.Materials and MethodsNine patients (8 females and 1 male; age range, 23–65 years) with a total of 62 PAVMs who underwent percutaneous embolization for multiple PAVMs and were subsequently followed up using TR-MRA and CT obtained within 6 months from each other were retrospectively reviewed. All imaging analyses were performed by two independent readers blinded to clinical information. The visibility of the feeding arteries on maximum intensity projection (MIP) reconstruction and multiplanar reconstruction (MPR) TR-MRA images was evaluated by comparing them to CT as a reference. The accuracy of TR-MRA for diagnosing reperfusion of the PAVM after embolization was assessed in a subgroup with angiographic confirmation. The reliability between the readers in interpreting the TR-MRA results was analyzed using kappa (κ) statistics.ResultsFeeding arteries were visible on the original MIP images of TR-MRA in 82.3% (51/62) and 85.5% (53/62) of readers 1 and 2, respectively. Using the MPR, the rates increased to 93.5% (58/62) and 95.2% (59/62), respectively (κ = 0.760 and 0.792, respectively). Factors for invisibility were the course of feeding arteries in the anteroposterior plane, proximity to large enhancing vessels, adjacency to the chest wall, pulsation of the heart, and small feeding arteries. Thirty-seven PAVMs in five patients had angiographic confirmation of reperfusion status after embolization (32 occlusions and 5 reperfusions). TR-MRA showed 100% (5/5) sensitivity and 100% (32/32, including three cases in which the feeding arteries were not visible on TR-MRA) specificity for both readers.ConclusionSingle-shot whole thoracic TR-MRA with MPR showed good visibility of the feeding arteries of PAVMs and high accuracy in diagnosing reperfusion after embolization. Single-shot whole thoracic TR-MRA may be a feasible method for the follow-up of patients with multiple PAVMs.

Shoulder Pain Associated with Multiple Arteriovenous Malformations Successfully Treated with Embolization

Shoulder Pain Associated with Multiple Arteriovenous Malformations Successfully Treated with Embolization

Neurovascular Manifestations in Pediatric Patients With Hereditary Haemorrhagic Telangiectasia

BackgroundHereditary hemorrhagic telangiectasia (HHT) is a multiorgan vascular dysplasia with limited data regarding its neurovascular manifestations and genotype-phenotype correlation in children. The objective of this study was to describe the neurovascular findings in a large cohort of children with HHT and correlate between phenotype and genotype. MethodsThis retrospective study was conducted on 221 children (<18 years) with a definite or possible diagnosis of HHT based on Curacao criteria, or with positive genetics for the mutated genes of ENG, ACVRL-1, and SMAD-4, who also underwent brain MRI and/or conventional angiography. Demographic and clinical information, imaging findings, and follow up information were gathered. ResultsTwo hundred twenty-one children with HHT (70.6% genetically confirmed, and 99.5% positive family history) were included, with a median age of 7 years (interquartile range: 3 to 11 years) and 58.8% male predominance. Neurovascular lesions were found in 64 of 221 (28.9%), with 3.1% prevalence of intracranial hemorrhage. The most commonly observed vascular malformations were developmental venous anomalies (48.5%) and brain arteriovenous malformations (AVMs) (31.2%), followed by capillary malformations (14.1%). Multiple AVMs were seen in 10.0% of the cohort. We found no instances of de novo AVM (1281.8 patient-years).A significantly higher proportion of patients with ENG mutations (19.7%) had brain AVM than those with ACVRL-1 (4.9%) and SMAD-4 (0%) mutations (P < 0.01). There was no significant difference in the hemorrhagic risk of shunting lesions associated with ENG (35.3%) or ACVRL-1 (33.3%) positivity (P = 0.9). ConclusionsWe describe the neurovascular imaging and genetic findings from a large pediatric cohort of HHT, to enhance clinical awareness and guide management of patients with HHT.

Successful treatment of bilateral multiple pulmonary arteriovenous malformations in a patient with brain abscess and severe hypoxemia using a combination of transcatheter embolotherapy and surgical resection: a case report

BackgroundA pulmonary arteriovenous malformation is an abnormal dilated blood vessel that makes direct communication between a pulmonary artery and pulmonary vein and can be associated with hypoxemia or neurological complications, including brain abscess and cerebral infarction. Treatment of pulmonary arteriovenous malformation includes surgical resection and transcatheter embolotherapy, however the adaptation of therapies should be considered when a patient is in bad condition.Case presentationA 51-year-old man was admitted after developing fever, consciousness disorder, and hypoxemia. Magnetic resonance imaging of the brain showed a brain abscess. Bilateral pulmonary arteriovenous malformations were found by contrast computed tomography. Because of a family history of pulmonary arteriovenous malformation, a history of epistaxis, and the existence of oral mucosa telangiectasia, he was diagnosed with hereditary hemorrhagic telangiectasia and brain abscess caused by intrapulmonary right-to-left shunt. The brain abscess improved with antibiotic treatment; however, the administration of oxygen did not ameliorate his hypoxemia. His hypoxemia was exacerbated by positive pressure ventilation. Considering his systemic and respiratory condition, we considered surgery to involve a high degree of risk. After controlling his brain abscess and pneumonia, transcatheter embolotherapy was performed. This improved his systemic condition, enabling surgical treatment.ConclusionsThis middle-aged patient suffering from brain abscess and severe hypoxemia with multiple pulmonary arteriovenous malformations was successfully treated by a combination of transcatheter embolotherapy and surgery. The adaptation and combination of therapies, as well as the sequence of treatments, should be considered depending on the patient status and lesions.

Multiple Site Peripheral Arteriovenous Malformations A Definitive Diagnosis Using Duplex Ultrasound CME credit March 2021

Multiple Site Peripheral Arteriovenous Malformations A Definitive Diagnosis Using Duplex Ultrasound CME credit March 2021

Hereditary Hemorrhagic Telangiectasia or Osler-Weber -Rendu Syndrome: Management of Epistaxis in 4 Cases

Hereditary Hemorrhagic Telangiectasia, also known as Osler-Weber-Rendu Syndrome is an autosomal dominant disorder causing systemic abnormalities of the vascular structure. There are multiple arteriovenous malformations present in the skin and mucosal surface of the nail beds, nose, gastrointestinal tract, lungs and brain. Epistaxis is the common presentation symptom, which may require multiple hospital admissions and blood transfusions. It is extremely rare disease in our population. We report 4 cases of HHT who presented to us with moderate to severe epistaxis and how we managed these patients.

S3527 Hepatic and Gastrointestinal Complications of Chronic Schistosomiasis: A Case Report on the Importance of Treatment Confirmation

INTRODUCTION: Schistosomiasis affects millions worldwide and can have devastating hepatosplenic and intestinal consequences. Though it is well-known to providers in tropical and subtropical parts of the world, those in non-endemic areas are often unfamiliar with the disease and its complications. CASE DESCRIPTION/METHODS: A 76-year-old Filipino female with a history of schistosomiasis complicated by portal hypertension presented to a tertiary care center for shortness of breath. In the emergency department, she was found to be hypoxic with increased work of breathing. Chest x-ray demonstrated a recurrent hepatic hydrothorax that required emergent thoracentesis. On admission, she reported subacute on chronic abdominal pain previously attributed to symptomatic cholelithiasis. Physical exam was notable for tachycardia to the 120s and a mildly, diffusely tender abdomen. Relevant laboratory tests included: white blood cell count 11,200/mm3 without eosinophilia, AST 44 U/L, ALT 21 U/L, alkaline phosphatase 172 IU/L, total bilirubin 1.2 mg/dL, and direct bilirubin 0.4 mg/dL. Imaging studies revealed a chronic portal vein thrombus (PVT), a 20–30 cm loop of severely thickened distal ileum, multiple inferior mesenteric arteriovenous malformations (AVMs), calcifications in the sigmoid colon, and cholelithiasis. Review of prior records showed that when she was 48 years old, the patient had a liver biopsy with Schistosoma japonicum ova in the portal tracts. Despite this, she never received treatment for schistosomiasis. After diagnosis, she had seen multiple providers in various specialties (hepatology, surgery, primary care) at different institutions. It was always assumed that she had been treated. This patient’s initial management included praziquantel, inferior mesenteric AVM embolization, prophylactic enoxaparin for PVT, a percutaneous cholecystostomy tube for symptomatic cholelithiasis, and diuresis for hepatic hydrothorax. She was admitted twice more within a month of discharge due to further complications of her disease. Ultimately, she was discharged with home hospice care. DISCUSSION: This case demonstrates the importance of confirming schistosomiasis treatment in previously diagnosed patients. This patient had seen multiple specialists prior to a provider realizing she had never received treatment. Ultimately, the complications of chronic schistosomiasis became life-limiting but may have been prevented if treatment had occurred at the time of diagnosis.

Clinico-Radiologic Outcomes After Stereotactic Radiosurgery for Patients with Complex High-Risk Multiple Arteriovenous Malformations

The present study aims to define the outcome and risks of patients with multiple arteriovenous malformations (AVMs) treated by stereotactic radiosurgery (SRS). We retrospectively analyzed the records of 1232 patients with AVMs who underwent SRS at our center between 1987 and 2017. We identified 10 patients who had SRS for multiple AVMs (total of 25). Eight patients presented with intracranial hemorrhage before SRS. Four patients had hereditary hemorrhagic telangiectasia. A Spetzler-Martin grade I AVM was diagnosed in 11 AVMs, grade II in 7, grade III in 6, and grade IV in 1 AVM. The median maximum diameter was 12 mm, the median target volume was 1.1 cm3, and the median margin dose was 20 Gy. Twenty-four AVMs were treated with single-session SRS, and 1 AVM was treated with volume-staged SRS. The angiographic complete obliteration rate of each AVM was 18.2%, 58.0%, and 66.4% at 3, 5, and 7 years, respectively. The angiographic complete obliteration rate of all treated AVMs in each patient was 11.1%, 51.4%, and 51.4% at 3, 5, and 7 years, respectively. In multivariate analysis, higher marginal dose (≥18 Gy, P= 0.031) was significantly associated with complete obliteration of AVMs. After obliteration of all their AVMs was confirmed no patient bled. Patients with complex multiple AVMs often presented with a brain hemorrhage. Reduction in bleeding risk after SRS requires complete obliteration that is more likely if the initial AVM margin dose is ≥18 Gy for each AVM.

A CASE OF OSLER WEBER RENDU SYNDROME AND ITS ANAESTHETIC IMPLICATIONS

Osler-Weber-Rendu disease or hereditary haemorrhagic telangiectasia (HHT) is an autosomal dominant genetic disorder. First recognised in the 19th century, this rare often undiagnosed familial disorder with abnormal vascular structures, causes bleeding from the nose and gastrointestinal tract. This condition is characterised by lack of communicating capillaries connecting arteries and veins resulting in multiple arteriovenous malformations (AVMs) and telangiectasia. We describe a case report of anaesthesia management of a patient with Osler-Weber-Rendu disease.