Large Arteriovenous Malformations Research Articles

Early Infantile Diagnosis of Hereditary Hemorrhagic Telangiectasia Complicated by Child Abuse

We report a rare case of hereditary hemorrhagic telangiectasia (HHT) in a 4-month-old female with findings of child abuse. She presented with poor feeding, vomiting, and irritability after a short fall from the bed. Initial evaluation found subdural hematomas (SDH), persistent hypoxia, failure to thrive (FTT), a frenulum tear, facial lacerations, and bruising. The patient was admitted and an extensive work-up led to the diagnosis of brain and pulmonary arteriovenous malformations (AVMs) and finally the diagnosis of HHT. The SDH, cutaneous injuries, and oral injury were highly suspicious for child abuse and were reported to Child Protective Services (CPS) and law enforcement for investigation simultaneous to the medical work-up. Her hospital course was complicated by progressive hypoxemia with radiographic evidence of several large pulmonary AVMs for which she underwent successful embolization. Her head injury was indeterminate for physical abuse in the setting of a medical condition pre-disposing to intracranial hemorrhage. A few weeks later, she was re-admitted with repeat abusive injuries in the form of femur fractures. This case demonstrates the unique diagnostic dilemma when two diagnoses are occurring simultaneously – HHT and child abuse – and showcases the importance of a detailed family history, genetic testing, strong multidisciplinary collaboration with a holistic approach and medically informed CPS systems to ensure accurate diagnoses and safe disposition.

Artículo traducido] Tratamiento comparativo de lesiones mucocutáneas en pacientes con telangiectasia hemorrágica hereditaria con láser dual secuencial de colorante pulsado y neodimio: itrio-aluminio-granate versus neodimio: itrio-aluminio-granate solo: un estudio controlado aleatorizado doble ciego con evaluación de la calidad de vida

BackgroundHereditary haemorrhagic telangiectasia (HHT) is characterized by the presence of telangiectases and larger arteriovenous malformations in different organs. Mucocutaneous telangiectases can bleed and become an aesthetic concern, impairing quality of life (QoL). However, the best treatment approach has not been defined yet. ObjectiveTo evaluate the efficacy and safety of dual wavelength sequential 595/1064nm laser (DWSL) compared to 1064nm laser (Nd:YAG) alone. Secondarily, to evaluate QoL impairment in HHT patients, and its improvement with laser therapy. MethodsA comparative randomized split-body double-blinded prospective study (DWSL vs Nd:YAG). Demographic, clinical and treatment characteristics were recorded. The severity and degree of improvement were evaluated by three blinded examiners who scored pre-treatment and post-treatment pictures on a 5-point scale. Patients fulfilled Skindex-29 and FACE-Q® tests and assessed procedure-associated pain and patient satisfaction. Results111 treatment areas (55 treated with DWSL and 56 with Nd:YAG) from 26 patients were analyzed. The median number of laser sessions was 2 (interquartile range [IQR] 2–4; mean 2.90 vs 2.88, respectively). The median improvement score, irrespective of location, was significantly higher for Nd:YAG compared to DWSL: 3 (IQR 2–3; mean 2.61) vs 2 (IQR 2–3; mean 2.32), p=0.031. Both FACE-Q index and Skindex-29 test results improved significantly (p<0.001), and 92.4% patients reported a high degree of satisfaction (≥8). No severe adverse events were reported. ConclusionsDWSL and Nd:YAG laser are convenient, safe and effective treatment options for mucocutaneous telangiectases in HHT patients. However, Nd:YAG delivered better results with better tolerability. QoL was significantly improved by both treatments.

Hypofractionated stereotactic radiotherapy in Spetzler Martin grades 4 and 5 arteriovenous malformations in the pediatric population: Is it a viable alternative?

To analyze the outcomes of hypofractionated stereotactic radiotherapy (HFSRT) for Spetzler Martin grades 4 and 5 arteriovenous malformations (AVMs) in a pediatric population. Fourteen patients with Spetzler Martin (SM) grades IV and V large AVMs who underwent HFSRT between January 2013 and July 2019 were retrospectively reviewed. All patients received HFSRT at a dose of 30-36Gy in 5 to 6 fractions. They were followed up annually with clinical and imaging assessments to evaluate obliteration rates. The median age at presentation was 15years (range 8-21years). Ten (71%) were SM grade 4 AVMs and the rest were SM grade 5 AVMs. The majority presented with headache (8 [57%]), and 3 (21%) presented with bleeding. The median nidus volume was 39.4cc (IQR, 31.4-52.4). Two (14%) patients had infratentorial AVMs. All of them had deep venous drainage. The median clinical follow-up duration was 75months (range 31-107months). There was complete obliteration of the nidus in 3 (21%) patients with a median time to obliteration of 39months. HFSRT resulted in a reduction of the AVM volume to 12cc or less in nearly 70% of patients. None of the patients experienced re-bleeding. 79% reported an improvement in their symptoms. HFSRT is a highly effective treatment for high-grade AVMs in children, which can result in either complete elimination or significant reduction of the nidus volume or make it suitable for additional treatment, such as single-session stereotactic radiosurgery (SRS).

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.

Comparative Treatment of Mucocutaneous Lesions in Hereditary Haemorrhagic Telangiectasia Patients With Dual Sequential Pulsed Dye Laser and Neodymium: Yttrium-Aluminium-Garnet Versus Neodymium: Yttrium-Aluminium-Garnet Laser Alone: A Double-Blind Randomized Controlled Study With Quality-of-Life Evaluation

BackgroundHereditary haemorrhagic telangiectasia (HHT) is characterized by the presence of telangiectases and larger arteriovenous malformations in different organs. Mucocutaneous telangiectases can bleed and become an aesthetic concern, impairing quality of life (QoL). However, the best treatment approach has not been defined yet. ObjectiveTo evaluate the efficacy and safety of dual wavelength sequential 595/1064nm laser (DWSL) compared to 1064nm laser (Nd:YAG) alone. Secondarily, to evaluate QoL impairment in HHT patients, and its improvement with laser therapy. MethodsA comparative randomized split-body double-blinded prospective study (DWSL vs Nd:YAG). Demographic, clinical and treatment characteristics were recorded. The severity and degree of improvement were evaluated by three blinded examiners who scored pre-treatment and post-treatment pictures on a 5-point scale. Patients fulfilled Skindex-29 and FACE-Q® tests and assessed procedure-associated pain and patient satisfaction. Results111 treatment areas (55 treated with DWSL and 56 with Nd:YAG) from 26 patients were analyzed. The median number of laser sessions was 2 (interquartile range [IQR] 2–4; mean 2.90 vs 2.88, respectively). The median improvement score, irrespective of location, was significantly higher for Nd:YAG compared to DWSL: 3 (IQR 2–3; mean 2.61) vs 2 (IQR 2–3; mean 2.32), p=0.031. Both FACE-Q index and Skindex-29 test results improved significantly (p<0.001), and 92.4% patients reported a high degree of satisfaction (≥8). No severe adverse events were reported. ConclusionsDWSL and Nd:YAG laser are convenient, safe and effective treatment options for mucocutaneous telangiectases in HHT patients. However, Nd:YAG delivered better results with better tolerability. QoL was significantly improved by both treatments.

A Success Story of Cerebral Arteriovenous Malformation (AVM) Treated with Stereotactic Radiosurgery (SRS) in Bangladesh

Purpose: To share a case of cerebral Arteriovenous Malformation (AVM) patient, treated with Stereotactic Radiosurgery (SRS) at Evercare Hospital Dhaka, treatment process and successful outcome. Background: Arteriovenous malformation is an anomaly, mostly congenital due to a misfit in vasculogenic, where arteries and veins come to a direct connection. Treatment options include observation, embolization, microsurgery, Stereotactic Radiosurgery (SRS) either alone or in combination. This report describes our experience in treating a patient of AVM by SRS. Stereotactic Radiosurgery is an established modality to achieve desired nidus obliteration, excellent compliance being noninvasive, and reduced treatment-related morbidity. This case report also reviewed the literature in detail. After a thorough literature search, to the best of our knowledge, this shall be the first published case report of AVM successfully treated with SRS in Bangladesh. Case Presentation: In March 2019, 25-years old male presented in Radiation Oncology OPD as a diagnosed case of AVM, having complaints of headache for two years. Headache was occasional and often associated with vertigo and dizziness. He had no neurologic deficit. A radiological assessment revealed a large (4.6x4.4cm) AVM in the left parieto-occipital lobe showing serpiginous enhancement with IV contrast. After routine workup, he underwent stereotactic Radiosurgery in March’2019. A dose of 23Gy was delivered to the malformation, including all nidus. Acute radiation toxicity was noted as transient hair loss only over the irradiated area and no other side effect. Radiological Assessment was done at six months, 1year, and 2.5 years. After having a stable size at six months, complete nidus obliteration was achieved at one year. His last visit to our OPD was in July 2021, with radiologically complete nidus obliteration without any significant clinical symptoms. Conclusions: Linear Accelerator-based SRS is an excellent treatment option for AVM, encouraging clinical outcomes for carefully selected patients. Bang. J Neurosurgery 2022; 12(1): 53-64

Amputation and Immediate Total Ear Reconstruction for a Large Arteriovenous Malformation

Amputation and Immediate Total Ear Reconstruction for a Large Arteriovenous Malformation

Quantitative Analysis of Parenchymal Effects and Flow of Large Arteriovenous Malformations Managed With Stereotactic Radiosurgery.

Stereotactic radiosurgery (SRS) of larger arteriovenous malformations (AVM) is associated with an elevated incidence of adverse radiation effects (ARE). To date, volume-response and dose-response models have been used to predict such effects. To understand radiological outcomes and their hemodynamic effects on the regional brain. A retrospective analysis was conducted at our institution using a prospective registry of patients managed between 2014 and 2020. We included patients with AVM with a nidus larger than 5 cc who received either single-session or volume-staged Gamma Knife radiosurgery. AVM volume changes, volumes of parenchymal response, and obliteration were analyzed and correlated with transit times and diameters of feeding arteries and draining veins. Sixteen patients underwent single-session SRS, and 9 patients underwent volume-staged SRS. The average AVM volume was 12.6 cc (5.5-23). The AVM locations were predominantly lobar (80%) and 17 (68%) were in critical locations. The mean margin dose was 17.2 Gy (15-21), and the median V12Gy was 25.5 cc. Fourteen (56%) AVMs had a transit time shorter than 1 second. The median vein-artery ratio (sum diameter of the veins/sum diameter of feeding arteries) was 1.63 (range, 0.60-4.19). Asymptomatic parenchymal effects were detected in 13 (52%) patients and were symptomatic in 4 (16%) patients. The median time to ARE was 12 months (95% CI 7.6-16.4). On univariate analysis, significant predictors of ARE were lower vein-artery ratio ( P = .024), longer transit time ( P = .05), higher mean dose ( P = .028), and higher D95 ( P = .036). Transit times and vessel diameters are valuable predictors of the subsequent parenchymal response after SRS. A more quantitative understanding of blood flow is critical for predicting the effects on the regional brain after AVM radiosurgery.

147. MAP2K1 Mutant Murine Model Recapitulates Human Cutaneous Arteriovenous Malformation

PURPOSE: Arteriovenous malformation (AVM) is a congenital vascular anomaly caused by somatic activating mutations in MAP2K1 in endothelial cells (ECs). AVMs cause deformity, bleeding, ulceration, pain, and death. We recently generated a conditional Map2k1-K57N mouse model by inserting a Map2k1-K57N cDNA-IRES (independent ribosomal entry side)-GFP cassette in the mouse ROSA locus under control of the CAG promoter. Mice develop vascular malformations in the brain, ear, and intestines. However, brain lesions cause lethality between 23-71 days after birth and the animals do not develop large AVM lesions in the skin. The purpose of this study was to extend the lifespan of mutant animals and study whether they developed significant cutaneous AVMs over time. METHODS: We inserted a LoxP flanked gene trap (GT) downstream of the promotor to prevent expression of the mutant allele (R26GT-Map2k1-GFP). Using the tamoxifen inducible EC specific Tg-Cdh5CreER transgene, we activated Map2k1-K57N expression in ECs at P1. In an effort to increase the lifespan of the mice, we reduced the amount of tamoxifen to 1/10th of our previous dose (5 ul of 1 ug/ul 4-OH-tamoxifen in acetone) applied topically to the left ear. RESULTS: We obtained 1 Tg-Cdh5Cre+/-;R26GT-Map2k1-GFP/+ mouse that survived 4 months after birth. The mouse exhibited 2 large cutaneous AVMs; 1 involved the orbital area and 1 affected the neck. The mouse also exhibited intestinal lesions but not brain malformations. The integument lesions shared a similar phenotype to human AVM. CONCLUSION: Decreasing the tamoxifen dose of Tg-Cdh5Cre+/-;R26GT-Map2k1-GFP/+ mice extends their lifespan. These mice develop large AVMs of the integument suggesting the lesions behave similarly to human lesions, progressing from Stage 1 to Stage 2 as mice age. This successful murine model can be used to study the pathophysiology of AVM as well as to test novel pharmacotherapy against the lesion.

C32 CONSTRICTIVE PERICARDITIS WITH PLATYPNEA ORTHODEOXIA

Abstract A 56–year–old woman is admitted to our centre for biventricular heart failure. In anamnesis she reports a submandibular phlegmon complicated by pleuropericarditis at the age of 13 years requiring repeated pericardiocentesis. In the last three years she has been hospitalised twice for ascites and discharged with a diagnosis of idiopathic liver cirrhosis. On admission she presented with peripheral oedema, ascites and hippocratic fingers; oxygen saturation is 80% in room air. On blood tests creatinine, transaminases, albumin, coagulation and NT–proBNP are normal, bilirubin is elevated. An echocardiography is performed showing a calcific spicola impinging the free wall of the right ventricle producing endocavitary obstruction; diffusely thickened pericardium, septal bounce, annulus reversus and expiratory reflux in the suprahepatic veins. Biventricular contractile function is normal and no significant valvulopathy is present. CT scan confirms the presence of diffuse pericardial calcifications, small liver and irregular profile with large ascitic effusion. Decongestive therapy is started with weight loss of 22 kg and regression of oedema but severe hypoxia persists with evidence of platypnea–orthodeoxia syndrome. An echocardiography is repeated showing passage of microbubbles in the left atrium from the sixth beat onwards, compatible with intrapulmonary shunt. A large arteriovenous malformation (AVM) of the right lower pulmonary branch is found at CT angiography, which is also revealed by targeted invasive angiography. Cardiac catheterisation and cardiac MRI confirmed the diagnosis of constrictive pericarditis. Percutaneous closure of the AVM with complete resolution of the hypoxia and subsequent pericardiectomy is performed. The patient is discharged on day XXVII with normal saturation on room air, in good haemodynamic compensation and with normal liver stasis parameters. In conclusion, constrictive pericarditis is a rare cause of heart failure and to our knowledge this is the second case described reporting a pulmonary AVM secondary to constrictive pericarditis.

A complex approach to the treatment of arteriovenous IV-V degree malformations according to Spetzler‒Martin scale. Clinical case

Four therapeutic approaches have been developed for the treatment of arteriovenous malformations (AVMs): microsurgery, radiosurgery, embolization, and conservative treatment. The lack of consensus regarding the choice of treatment method and the different specializations of the doctor who are onvolved to the patient with AVM often determine the wrong treatment strategy. We performed a prospective analysis of the results of AVM of the IV degree according to Spetzler‒Martin scale, 4–5 points on the Buffalo scale complex treatment, based on clinical and tomographic data after the use of partial endovascular embolization and radical microsurgical removal of the AVM node assisted by cell saving technology. According to cerebral angiography 4 months follow-up there is no visible AVM vessels, the malformation was completely resected.Treatment of large and giant AVMs (IV and V degrees according to the Spetzler‒Martin scale) requires tailored surgical treatment approache using endovascular, microsurgical and radiosurgical techniques, but in many cases without achieving a radical result. The use of cell saver technology is necessary to reduce the risks of intraoperative complications associated with blood loss during microsurgical intervention, and enables radical removal of the AVM and recovery of the patient. The role of cell saver technology is crucial in vascular microsurgical interventions, which are often accompanied by a significant volume of blood loss, ensuring rapid autologous hemotransfusion and restoration of circulating blood volume. Endovascular embolization is a necessary step to reduce the risks of intraoperative complications during microsurgical intervention, which, together with the use of cell saver technology, makes it possible to achieve radical AVM removal and patient recovery.

Volume Fractionation Stereotactic Radiosurgery for Large Volume Intracranial Arteriovenous Malformations.

Arteriovenous malformations (AVMs) are common intracranial vascular anomalies. Common treatment modalities used to manage AVMs are surgical excision, embolization, and stereotactic radiosurgery (SRS). Large AVMs are defined as AVMs larger than 10 cm3 and pose a therapeutic challenge with high rates of treatment-related morbidity and mortality. Single-stage SRS is a good option for small AVMs but carries high risks of radiation-induced complications in large AVMs. Volume-staged SRS (VS-SRS) is a newer strategy used in large AVMs that allows one to deliver an optimal radiation dose to the AVMs while reducing the risk of radiation damage to the normal brain. It involves the division of AVM into multiple small sectors which are irradiated at different time intervals with high radiation doses. Good obliteration rates with less risk of radiation-induced complications have been described in the literature with VS-SRS.

Hypofractionated Stereotactic Radiotherapy with CyberKnife for Large Arteriovenous Malformations and Arteriovenous Malformations Located in Eloquent Areas.

Literature has yet to establish an appropriate treatment strategy for large arteriovenous malformations (AVMs) and AVMs located in eloquent areas. In this study, the treatment outcomes of hypofractionated stereotactic radiotherapy (HSRT) with CyberKnife (CK) for large AVMs and AVMs in eloquent areas were evaluated. This study retrospectively evaluated 38 consecutive patients with AVMs treated with HSRT in the Japanese Red Cross Medical Center between August 2010 and July 2015. Obliteration rates and hemorrhage rates at 3- and 5-years of follow-up were calculated. Factors for hemorrhage and obliteration were analyzed with logistic regression analysis. Fourteen (36.8%) patients had a history of hemorrhage. Twenty (52.6%) AVMs were larger than 10 mL, and 34 (89.5%) AVMs were located in eloquent areas. The majority of the AVMs (84.2%) were classified into high grades (grades 3, 4, and 5) using the Spetzler-Martin grading scale. The median modified radiosurgery-based AVM score was 2.05, and the median Virginia Radiosurgery AVM Score was 3. The mean marginal dose was 24.5 ± 2.5 Gy. Twenty-three and 15 patients received three- and five-fraction stereotactic radiotherapy, respectively. At 3 and 5 years posttreatment, two (2.0%/year) and six (6.7%/year) patients had hemorrhage with obliteration rates of 15.2% and 16.7%, respectively. AVM localization in eloquent areas was a risk factor for obliteration failure. This study revealed that HSRT with CK for large AVMs and AVMs located in eloquent areas contributed to hemorrhage risk reduction and obliteration, at least in the early stages.

Risk for Hemorrhage the First 2 Years After Gamma Knife Surgery for Arteriovenous Malformations: An Update.

Knowledge about the natural course of brain arteriovenous malformations (AVMs) have increased during the past 20 years, as has the number of AVMs treated, especially larger ones. It is thus timely to again analyze the risk for hemorrhage after Gamma Knife Surgery (GKS). To confirm or contradict conclusions drawn 20 years ago regarding factors that affect the risk for post-GKS hemorrhage. The outcome after GKS was studied in 5037 AVM patients followed for up to 2 years. The relation between post-treatment hemorrhage rate and a number of patient, AVM, and treatment parameters was analyzed. The results were also compared with the results from our earlier study. The annual post-treatment hemorrhage rate was 2.4% the first 2 years after GKS. Large size, low treatment dose, and old age were independent risk factors for AVM hemorrhage. After having compensated for the factors above, peripheral AVM location and female sex, at least during their child bearing ages, were factors associated with a lower post-GKS hemorrhage rate. Large AVMs (>5 cm 3 ) treated with low doses (≤16 Gy) had higher and small AVMs treated with high doses a lower risk for hemorrhage as compared with untreated AVMs. This was detectable within the first 6 months after GKS. No difference in hemorrhage rate could be detected for the other AVMs. Based on our findings, it is advisable to prescribe >16 Gy to larger AVMs, assuming that the risk for radiation-induced complications can be kept at an acceptable level.

S3041 Hepatic Artery Dissection – A Case Report

Introduction: Visceral artery aneurysms are rare pathologies with reported incidence rates between 0.01% to 0.2%; even rarer are hepatic artery dissections (HAD). Most aneurysms rupture or are incidentally found on autopsy, but imaging advancements has enabled earlier identification of aneurysms; in this case, a HAD. Case Description/Methods: A 77-year-old female with medical history pertinent for sickle cell trait, acquired factor VIII inhibitor coagulopathy, history of type 2 thoracic aortic dissection, and hypertension presented to the emergency room with a week of mild fatigue, postprandial epigastric burning pain, and intermittent stabbing right upper quadrant pain. Right upper quadrant ultrasound demonstrated a hypervascularized right hepatic lobe mass with large tortuous internal vessels. CT abdomen & pelvis without contrast showed a large hypodensity in the right liver with curvilinear density. MRI demonstrated large arteriovenous malformation (AVM) within the right hepatic lobe and focal hepatic parenchymal hemorrhage centered within AVM nidus. Gastroenterology was consulted and CT angiogram findings were suspicious for common HAD, proper hepatic & intrahepatic pseudoaneurysms, and intrahepatic rupture. Interventional radiology was consulted. Hepatic angiogram showed a large defect in the proximal hepatic artery with brisk opacification, thought to represent a pseudoaneurysm. This prompted framing coil placement across the large defect and coils extending from the proper hepatic artery to the common hepatic artery with significant reduction in forward flow & pseudoaneurysm opacification. Per hepatobiliary & vascular surgery, no need for additional surgical intervention. Postoperative course was complicated by fever & leukocytosis, worsening kidney injury, and anemia; attributed to the procedure and resolved with supportive care. Patient discharged on postoperative day 8 with outpatient follow-up. (Figure) Discussion: This case highlights the importance of imaging for earlier detection of hepatic artery aneurysms (HAA) in minimizing risk of catastrophic hemorrhage. HAA are rare but clinically important given they are associated with high incidence of rupture and mortality up to 20%. Although surgery is preferred for extrahepatic findings, endovascular interventions are favored for intrahepatic findings depending on size and presence of pseudoaneurysms, such as this case. They have led to more favorable short & longer-term outcomes and lower mortality & morbidity rates, particularly in poor surgical candidates.Figure 1.: Hepatic angiography and embolization was performed. A large defect with brisk opacification of the proper and common hepatic arteries were seen (A), prompting deploy of a framing coil. (B) Embolization was then performed on the hepatic artery with three coils (5mm, 6mm, & 8mm). Coils were examined from proper hepatic artery to the common hepatic artery. (C) Following placement of the coils, significant reduction in flow and opacification of the pseudoaneurysm was seen.

Inhibiting PI3K and MAPK Pathways

Extracranial arteriovenous malformations (AVMs) are associated with significant morbidity and mortality and lack consistently effective non-surgical interventions. Genetic mutations have been associated with AVMs, leading to success in using targeted therapies to reduce comorbidities. We report a 19-year-old male with phosphatase and tensin homolog hamartoma tumor syndrome with a large AVM of the left leg, complicated by progressive dilated cardiomyopathy, requiring multiple trans-arterial and trans-venous embolizations. Despite management using systemic mammalian target of rapamycin inhibition by sirolimus and surgical interventions, this patient developed cardiac failure and chronic skin ulcers over the distal left leg, presumed to be due to stasis and tissue ischemia, leading to development of debilitating pain. Trametinib was added to target activation of mitogen-activated protein kinase pathway. His cardiac disease and AVM responded when trametinib and sirolimus were combined. After 2 months, his chronic skin ulcers healed completely. The patient can ambulate without the need for any pain medication, and his cardiac condition stabilized.

An unexpected cause of vaginal hemorrhage.

A 25-year-old woman with a remote history of dilation and curettage presented to the emergency department with a history of 10 days of vaginal bleeding and lower abdominal pain. The physical examination was significant for cervical passage of blood and blood clots, uterine tenderness, hypotension, and tachycardia. An ultrasound of the pelvis showed multiple anechoic, serpentine, and branching spaces in the myometrium along the uterine wall with markedly hypervascular flow (Figures 1, 2; Video S1). The patient required transfusion of 2 units of packed red blood cells for hypovolemic/hemorrhagic shock. Vaginal bleeding spontaneously ceased. Magnetic resonance imaging (MRI) of the pelvis without contrast showed a large arteriovenous malformation within the distal anterior endometrium/myometrium (Figure 3). Uterine arteriovenous malformation (AVM) is a rare condition of abnormal venous and arterial connections.1 However, with improved medical imaging (ultrasound, MRI), cases have been increasing in the medical literature.2 It most commonly is diagnosed around age 30 years in women who have had various obstetric interventions (acquired), including trauma, uterine surgery, or even normal vaginal delivery.1 It is also possible to have congenital AVMs.1 Women are often asymptomatic. However, AVMs can be life threatening if the patient has uterine hemorrhage causing vaginal bleeding. This patient had an uncomplicated hospital course and was discharged home in stable condition. She had an outpatient follow-up appointment to determine interventional embolization therapy versus hysterectomy to prevent another hemorrhagic event. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.

Hybrid surgery for coexistence of cerebral arteriovenous malformation and primitive trigeminal artery: A case report and literature review

The primitive trigeminal artery (PTA), an abnormal carotid-basilar anastomosis, forms the vascular anomaly connection between the internal carotid artery and vertebrobasilar system. Rarely, PTA can be complicated by several other cerebrovascular disease, including arteriovenous malformations (AVMs), intracranial aneurysms, moyamoya disease, and carotid-cavernous malformations. Herein, we reported a rare case of PTA combined with an AVM in a male patient. The patient was a 28-year-old male with epileptic seizures at the onset of symptoms. Magnetic resonance imaging showed abnormal signal foci and localized softening foci formation with gliosis in the right parietal temporal lobe. Furthermore, using a digital subtraction angiogram (DSA), it was found that an abnormal carotid-basilar anastomosis had developed through a PTA originating from the cavernous portion of the right internal carotid artery (ICA) and a large AVM on the surface of the right carotid artery. The lesion of AVM tightly developed and draining into superior sagittal sinus. A hybrid operating room was used for the surgery. The main feeding arteries of the AVM originating from three major arteries, including the right middle cerebral artery, the right anterior cerebral artery, and the right posterior cerebral artery, were clipped and subsequently, then the AVM was thoroughly removed. The intraoperative DSA showed that the AVM had been resected completely. Postoperative pathological examination of the resected specimen indicated the presence of an AVM. The patient recovered well after surgery and has been symptom-free for more than 3 months. In summary, the pathogenesis of the coexistence of PTA and AVM remains unknown. As highlighted in this case report, hybrid surgery can be used to remove AVMs and can improve the patients' prognosis. To our best knowledge, this is the first case in the literature of successful AVM treatment using hybrid surgery.

Analysis of results determination of level of vascular endothelial growth factor, receptor for vascular endothelial growth factor and big endotelin-1 in structure of arteriovenous malformations

Objective ‒ to assess the diagnostic significance of angiogenesis factors based on the study of the level of angiogenesis factors (vascular endothelial growth factor (VEGF-A), specific receptor for vascular endothelial growth factor (VEGFR-1), endothelial inflammation factor (Big Endothelin-1)) in arterial and venous blood of patients with arteriovenous malformations (AVM) and healthy individuals without AVM. Materials and methods. In 2019–2021 a study according to the data of enzyme-linked immunosorbent assay the level of VEGF-A, VEGFR-1 and Big Endothelin-1 in the plasma of arterial and venous blood of 50 (19 (38.0 %) men and 31 (62.0 % women)) with AVM of different localization was carried out. The age of patients ranged from 3 to 51 years, the mean age ‒ (27.79 ± 2.19) years. The control group consisted of 35 healthy individuals (20 men and 15 women) without AVM. Patients with AVMs were divided into two groups: with small and medium-sized AVM in the stage of compensation (n=32) and with large and giant AVM in the stage of subcompensation or decompensation (n=18). Results. A significant (p &lt; 0.05) difference was proved between the concentration of VEGF-A, VEGFR-1, Big Endothelin-1 in the plasma of arterial and venous blood of patients with AVM. This fact proves that the processes of pathological angiogenesis occur precisely in the structure of AVM. The value of VEGF-A, VEGFR-1 and BE-1 in patients with small and medium AVM was higher than in healthy individuals. The VEGF-A level was significantly (p &lt; 0.05) in 2.4 times higher than in the control group, the VEGFR-1 level ‒ in 1.4 times, and the ET-1 level ‒ in 1.5 times. The value of VEGF-A, VEGFR-1 and BE-1 level in patients with large and giant AVM was significantly (p &lt; 0.05) higher than in healthy individuals: VEGF-A ‒ in 7.2 times, VEGFR-1 ‒ in 2.36 times, and BE-1 ‒ in 1.7 times. Conclusions. The data obtained make it possible to statistically reliably state that the processes of pathological angiogenesis occur directly in the structure of AVM. A statistically significant dependence of the level of VEGF-A, VEGFR-1 and BE-1 in the blood plasma of patients on the size of the AVM and the stage of its compensation was revealed.

Inadvertent accident during balloon-occluded glue injection for facial AVM: rescue procedure using intentional glue injection

BackgroundGlue embolization during balloon inflation is a novel technique with many advantages. However, the procedure’s major complication is the adhesion of the balloon catheter by glue. Several studies have reported strategies to prevent this. However, no reports have described a rescue method after accidental adhesion occurs.Case presentationA 26-year-old male was referred to the department of interventional radiology for sclerotherapy of an aggravating large facial arteriovenous malformation (AVM). We planned a transvenous approach to decrease the velocity of AVM and increase the efficacy of the sclerotherapy treatment. We performed glue embolization of a major draining vein during microballoon inflation. Upon injection of the glue, inadvertent glue reflux occurred, and the microballoon was stuck to the vessel wall. While removing the microballoon catheter, its shaft broke in the guiding catheter. We filled the inner lumen of the guiding catheter with glue and waited for polymerization to fixate the broken microballoon catheter inside the guiding catheter. Fortunately, the stuck microballoon was separated, and two broken pieces of microballoon catheter were removed through femoral vein short sheath.ConclusionIntentional glue casting in the outer catheter is very useful when removing anything that is inside the catheter or stuck due to the glue reflux. It can be applied to various similar emergency situations.