Arteriovenous Malformation Presenting Research Articles

Sonographic Features of Uterine Arteriovenous Malformation: A Case Series.

Uterine arteriovenous malformation (AVM) is very rare but potentially life-threatening. Early and accurate diagnosis is the cornerstone of its management. The objective of this study is to encourage sonographers to become familiar with a variety of grayscale sonographic features, facilitating rapid recognition of the patterns and prompting them to apply color flow Doppler for a diagnosis of uterine AVM and possible further investigations or interventions. We present six cases of uterine AVM presenting with abnormal uterine bleeding at varying degrees of severity, from abnormal menstruation to life-threatening bleeding following curettage. All initially provided some clues of uterine AVM upon grayscale ultrasound, leading to the application of color Doppler flow to support a diagnosis, with confirmation using abdominal computer tomography angiography (CTA) in most cases, resulting in definitive treatment using uterine artery embolization or other interventions. Most importantly, this study provides various sonographic features of uterine AVM, such as appearances of small tubular structures, spongy patterns, a conceptive-product-like appearance, and spaghetti-like patterns. Hopefully, familiarity with these sonographic features can facilitate practitioners to make an early diagnosis, leading to proper further investigation and intervention, and to prevent serious complications from potentially being caused by this subtle but very serious disorder.

Arteriovenous malformation presenting as complex regional pain syndrome: illustrative case.

Complex regional pain syndrome (CRPS) is typically described as a peripheral nerve disorder in which exaggerated allodynia and hyperalgesia follow a minor injury. Some researchers propose a central mechanism, although current evidence is lacking. A 14-year-old female presented with classic CRPS symptoms of left upper-extremity weakness and hyperalgesia after a bout of sharp pain in her thumb while shoveling snow. A possible seizure prompted magnetic resonance imaging, revealing a right frontal Spetzler-Martin grade II arteriovenous malformation (AVM) adjacent to the primary motor cortex. Brodmann areas 1, 3a, and 3b, which are responsible for localizing and processing burning and painful sensations, were also involved. The patient underwent transarterial Onyx embolization in two sessions and microsurgical resection, after which her CRPS symptoms completely resolved. To our knowledge, this is the first reported case of a cerebral AVM presenting as CRPS, which supports a central mechanism. The authors propose that rapid growth of the AVM led to a vascular steal phenomenon of surrounding parenchyma, which disrupted the patient's normal motor function and nociceptive processing. Further validation in other series is needed.

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.

Functional Outcomes after Surgical Management of Peripheral AVM at a Tertiary Care Center

Abstract Introduction: Arteriovenous (AV) malformations (AVMs) are rare vascular developmental orders containing an arterial network draining directly into one or more veins without a capillary bed, accounting for 1.5% of total vascular anomalies. Clinical presentations include pulsating mass, pain, ulceration, bleeding, tissue necrosis, enlargement of draining vein, and venous hypertension and/or cardiac failure. Treatment modalities include surgical excision, coil embolization, and sclerotherapy. Surgical excision offers an opportunity for cure but is associated with a risk of injury to vital structures, causing permanent impairment. Due to the high-risk nature of the lesion, it is important to study the clinical presentation and management of peripheral AVM for better management of these patients. Objectives: The objective was to study the presentation, management, and postoperative outcomes in terms of recurrence and functional status of the limb after surgical management of AVMs. Patients and Methods: It was a retrospective study done for 5 years (2018–2023). Thirteen patients who underwent surgical excision of peripheral AVM were included. Recurrence and functional status were assessed by clinical evaluation during follow-up outpatient department visits. Conclusion: The mean age of presentation is 33 years, with male preponderance (70%). Seven patients had upper limb AVMs (four had forearm involvement, while the rest had elbow region involvement). Six patients had lower limb AVMs, out of which four patients had involvement of the thigh region, one had in the knee, and the other in the calf. All patients complained of difficulty in performing daily activities due to the lesion. Nine patients underwent direct surgical excision, and four patients underwent AVM embolization before surgery (three patients had coil embolization, and one had onyx embolization). The mean duration of stay in the hospital was 5 days. Three patients were advised body contour stockings at follow-up. Two patients had recurrence (one after 6 months of surgical excision and the other had prior coil embolization), and one patient (had preembolization) had continued difficulty in walking postprocedure. The rest of the patients did not develop recurrence/functional impairment. There was no postoperative mortality. Thus, surgical excision offers a chance at cure with durable, functional outcomes in patients with peripheral AVMs.

Machine Learning Models for Brain Arteriovenous Malformations Presenting with Hemorrhage Based on Clinical and Angioarchitectural Characteristics

This study aims to develop the best diagnostic model for brain arteriovenous malformations (bAVMs) rupture by using machine learning (ML) algorithms. We retrospectively included 353 adult patients with ruptured and unruptured bAVMs. The clinical and radiological data on patients werecollected. The significant variables were selected using univariable logistic regression. We constructed and compared the predictive models using five supervised ML algorithms, multivariable logistic regression, and R2eDAVM scoring system. A complementary systematic review and meta-analysis of studies was aggregated to explore the potential predictors for bAVMs rupture. We found that a small nidus size of <3cm, deep and infratentorial location, longer filling time, and deep and single venous drainage were associated with a higher risk of bAVMs rupture. The multilayer perceptronmodel showed the best performance with an area under the curvevalue of 0.736 (95% CI 0.67-0.801) and 0.713 (95% CI 0.647-0.779) in the training and test dataset, respectively. In our pooled analysis, we also found that the male sex, a single feeding artery, hypertension, non-White race, low Spetzler-Martin grade, and coexisting aneurysms are risk factors for bAVMs rupture. This study further demonstrated the clinical and angioarchitectural characteristics in predicting bAVMs hemorrhage.

A Haunting Tremor: A Rare Presentation of an Unruptured Arteriovenous Malformation and Review of Related Literature

A Haunting Tremor: A Rare Presentation of an Unruptured Arteriovenous Malformation and Review of Related Literature

Double anterograde/retrograde approach for embolization of pulmonary arteriovenous malformation presenting with acute respiratory failure.

A 54-year-old male with multiple cardiovascular risk factors and past medical history of long-standing dyspnea was admitted for acute respiratory failure. Physical examination revealed near-normal blood pressure, sinus tachycardia, tachypnea, and reduced oxygen saturation (90%) with mild distal-limb cyanosis. On admission ECG, chest X-ray, transthoracic echocardiography, and laboratory tests were performed to exclude new-onset heart failure and/or an acute coronary syndrome. Then, he underwent pulmonary CT angiography seeking for pulmonary embolism that showed a voluminous pulmonary arterio-venous malformation (PAVM) with the feeding artery coming from the right pulmonary artery and venous drainage confluent into the right pulmonary vein (Panel 1A and C). A thrombophilic screening showed a heterozygous mutation for the methylenetetrahydrofolate reductase-1 gene and reduced serum-free protein S levels. The invasive left–right heart catheterization revealed mild-to-moderate pulmonary hypertension and moderate arterial desaturation (O2 saturation, 91%). Considering the large size of both the feeding artery and vein drainage (10 mm) and in order to reduce postprocedural embolization risk and recanalization rate,1,2 a contemporary percutaneous embolization of both PAVM branches (Panel 1C and D; Supplementary material online, Videos S1–3) with two Amplatzer Vascular Plug (AVP) II® 20 mm (Abbott, Plymouth, MN, USA) was planned using a double access technique, transfemoral trans-septal (retrograde) and transfemoral transpulmonary (anterograde) with a post-procedural improvement of arterial saturation (up to 99%). The patient was discharged with a marked clinical improvement (NYHA Class I) and complete exclusion of the PAVM at six months follow-up (Panel 1E and F).

A comparison of incidental and symptomatic unruptured brain arteriovenous malformations in children.

Patients with unruptured brain arteriovenous malformations (AVMs) may present with headaches, seizures, and/or neurological deficits. A smaller number of cases may be discovered incidentally. These lesions remain incompletely understood due to their sparse reporting. Herein, the authors describe the largest series to date comparing the presentation, angioarchitecture, and management of incidental versus symptomatic unruptured AVMs in children. The authors performed a retrospective analysis of patients who presented with brain AVMs from 1998 to 2022 at the University of California, San Francisco. Inclusion criteria were age ≤ 18 years at the time of presentation and an angiographically proven unruptured AVM that had been diagnosed postnatally. Of 76 children with unruptured AVMs, 66 (86.8%) presented with headaches, seizures, and/or neurological deficit. Ten AVMs (13.1%) were incidentally discovered through unrelated disease workup (50%), cranial trauma (40%), or research study participation (10%). Compared with patients with symptomatic unruptured AVMs, patients with incidental unruptured AVMs had a smaller mean ± SD maximum nidus diameter (2.82 ± 1.1 vs 3.98 ± 1.52 cm, p = 0.025) and fewer had deep venous drainage (20% of patients vs 61%, p = 0.036). They also presented at an earlier age (10 ± 5.2 vs 13.5 ± 4 years, p = 0.043) and with longer duration to first treatment (541 ± 922 vs 196 ± 448 days, p = 0.005). During the observation period, 1 patient developed recurring headaches and demonstrated AVM nidus growth. Four AVMs greater than 3 cm in size or in a deep location were treated with radiosurgery. Six other AVMs were treated with resection, with 2 receiving preoperative embolization. Eight AVMs (80%) were obliterated on last follow-up. Postprocedural complications included 2 transient neurological deficits after resection and 1 case of delayed seizure development after radiosurgery. The mean follow-up period was 5.7 ± 5.7 years without any hemorrhage episodes. A substantial proportion of pediatric patients with unruptured AVMs are discovered incidentally. With earlier presentation and more elementary angioarchitecture than symptomatic unruptured AVMs, these incidental lesions provide a snapshot into the natural history of AVM before symptom development or rupture.

Thrombosis in hereditary hemorrhagic telangiectasia

Thrombosis in hereditary hemorrhagic telangiectasia

Gamma Knife Radiosurgery for Arteriovenous Malformations Presenting as Secondary Trigeminal Neuralgia: A Case Series.

Gamma Knife Radiosurgery for Arteriovenous Malformations Presenting as Secondary Trigeminal Neuralgia: A Case Series.

An arteriovenous malformation presenting with proptosis – a case not to be missed

No abstract available

Extracranial arterio-venous malformation presenting as a scalp swelling

Extracranial arterio-venous malformation presenting as a scalp swelling

Telangiectatic pulmonary arteriovenous malformation presenting as a suspected malignant part-solid nodule.

Telangiectatic pulmonary arteriovenous malformation presenting as a suspected malignant part-solid nodule.

Intestinal arteriovenous malformations: a diagnostic and therapeutic challenge

Arteriovenous malformations (AVM) of the intestine are rare causes of gastrointestinal bleeding. Diagnosis depends upon accurate interpretation of imaging features and histopathology to differentiate them from other vascular malformations. Presentation of colonic AVM with mucous diarrhea and pain is rare and poses diagnostic challenges. Usually, patients presenting with bleed need surgical intervention. The current report discussed one patient with chronic liver disease, extrahepatic portal vein thrombosis with small bowel AVM and two patients with colonic AVM, highlighting management strategies adopted.

Quantitative evaluation of the hemodynamic differences between ruptured and unruptured cerebral arteriovenous malformations using angiographic parametric imaging-derived radiomics features.

Imaging features of cerebral arteriovenous malformations (AVMs) are mainly interpreted according to demographic and qualitative anatomical characteristics. This study aimed to use angiographic parametric imaging (API)-derived radiomics features to explore whether these features extracted from digital subtraction angiography (DSA) were associated with the hemorrhagic presentation of AVMs. Patients with AVM were retrospectively evaluated. Among them, 80% were randomly assigned to a training dataset, and the remaining 20% were assigned to an independent test dataset. Radiomics features were extracted from DSA by API. Then, informative features were selected from radiomics features and clinical features using the Least Absolute Shrinkage and Selection Operator (LASSO) algorithm. A model was constructed based on the selected features to classify the dichotomous hemorrhagic presentation in the training dataset. The model performance was evaluated in the test dataset with confusion matrix-related metrics. A total of 529 consecutive patients with AVMs between July 2011 and December 2020 were included in this study. After being selected by the LASSO algorithm and analyzed by multivariable logistic regression, three clinical features, namely, age (p = 0.01), nidus size (p < 0.001), and venous drainage patterns (p < 0.001), and four radiomics features were used to construct a model in the training dataset. On the independent test dataset, the model demonstrated a sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of 0.852, 0.844, 0.881, 0.809, and 0.849, respectively. The radiomics features extracted from DSA by API could be potential indicators for the hemorrhagic presentation of AVMs.

Pseudo-residual nidus after arteriovenous malformation surgery: illustrative case

BACKGROUNDIntraoperative indocyanine green video angiography (ICG-VA) is useful for determining the extent of lesion removal during cerebral arteriovenous malformation (AVM) surgery. The authors described a case of surgical removal of an AVM presenting with early venous filling mimicking a residual nidus on intraoperative ICG-VA.OBSERVATIONSA 7-year-old girl experienced a sudden disturbance of consciousness. Computed tomography revealed right frontal intracerebral hemorrhage. Digital subtraction angiography showed a Spetzler-Martin grade 1 AVM in the right frontal lobe. The patient received surgical removal of the AVM after endovascular embolization. After removal of the nidus, the first intraoperative ICG-VA revealed early venous filling of the cortex around the excision cavity. Additional resection of the cortex around this area was performed. Histopathological examination of the lesion revealed a dilated normal vascular structure without an AVM.LESSONSEarly venous filling in the surrounding brain tissue after AVM removal does not necessarily indicate a residual nidus. The need for additional resection of the lesion depends on the eloquence of the area.

Interventional Treatment Options in Children with Extracranial Vascular Malformations.

Extracranial vascular malformations vary greatly and belong to the complex field of orphan diseases and can involve all segments of the vascular tree: arteries, capillaries, and veins, and similarly the lymphatic system. The classification according to the International Society for the Study of Vascular Anomalies (ISSVA) represents an important guidance for selecting appropriate therapy. Although many of the principles of endovascular treatment, including image-guided sclerotherapy and embolization, are similar in adult and pediatric practice, there are some distinct differences regarding the treatment of vascular malformations of children. Thus, it is crucial to involve longer-term plan about managing these chronic diseases and their impact on a growing child. This review provides a detailed overview over the clinical presentation of venous, lymphatic, and arteriovenous malformations in children and emphasizes the specifics of their interventional treatment options, including distinct pediatric dose limitations and procedure-related side effects.

Quantified flow and angioarchitecture show similar associations with hemorrhagic presentation of brain arteriovenous malformations

IntroductionThe purpose of our study was to elucidate the impact of brain arteriovenous malformation (BAVM) flow and wall shear stress (WSS) on angioarchitecture and to evaluate their association with hemorrhagic presentations. Materials and methodsForty-one patients with BAVMs were evaluated by phase-contrast MR angiography. Volume flow rate and WSS were quantified. Angioarchitectural features such as location, angiogenesis, venous stenosis, venous ectasia, venous phlebitis, venous rerouting, exclusive deep vein and venous sac were evaluated by two neuroradiologists. The correlation between BAVM flow and size was evaluated with Spearman correlation coefficients. Differences of size, flow, and WSS between the hemorrhagic and non-hemorrhagic groups, the seizure and non-seizure groups, and between the different groups based on angioarchitecture were evaluated with Mann-Whitney U tests. Accuracy in predicting hemorrhage was evaluated with receiver operating characteristic curves. ResultBAVM flow was highly correlated with volume (ρ = 0.77). Higher flow was more commonly associated with angiogenesis, venous ectasia, venous rerouting, and venous phlebitis. Flow and angioarchitecture showed similar efficacy in differentiating hemorrhagic from non-hemorrhagic BAVMs. WSS did not demonstrate differences across any clinical groups. ConclusionFlow quantification and angioarchitecture analysis of BAVMs showed similar efficacy as evaluated by associations with hemorrhagic presentation. High flow affects both arterial and venous angioarchitecture, reflecting the nature of low vascular resistance in BAVMs.

Frontal Arteriovenous Malformation Presenting as Painful Unilateral Conjunctiva Injection.

Case PresentationArteriovenous malformations (AVM) have a variety of clinically significant manifestations. This report details a patient who presented with unilateral conjunctiva injection, which was found to be due to an atypical manifestation of an AVM with a large draining vein mimicking carotid cavernous fistula.DiscussionWhile imaging for patients presenting with eye pain and unilateral conjunctiva injection is not always warranted, emergency physicians should keep their differential diagnosis broad and pursue additional workup when warning signs of more sinister pathology present.

Correlation between angioarchitectural characteristics of brain arteriovenous malformations and clinical presentation of 183 patients.

The correlation between angioarchitecture and clinical presentation of brain arteriovenous malformation (bAVM) remains a subject of debate. The main purpose of the present study was to assess the correlation between angioarchitectural characteristics of bAVM and clinical presentation. A retrospective review of all consecutive patients presenting a bAVM who underwent a cerebral angiography at Beneficencia Portuguesa Hospital in São Paulo between January 2006 and October 2016 was carried out. Patients were divided in five groups: group 1 - hemorrhage; group 2 - seizure; group 3 - headache; group 4 - progressive neurological deficits (PND); group 5 - incidental). A total of 183 patients were included, with group 1 comprising 56 cases, group 2 49 cases, group 3 41 cases, group 4 28 cases, and group 5 9 cases. Regarding hemorrhage presentation, a statistical correlation was observed with female gender (P < 0.02), Spetzler-Martin 3B (P < .0015), and lesions with low flow (P < 0.04). A positive association was found between group 2 and age less than 36 years (P < 0.001), male sex (P < 0.018), presence of superficial lesions not classified as SM 3B (P < 0.002), presence of venous ectasia (p <0.03), and arterial steal phenomenon (P < 0.03). Group 4 was associated with older age (P < 0.01). Angioarchitectural characteristics can be correlated with some clinical presentations as well as with some clinical data, making it possible to create predictive models to differentiate clinical presentations.