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Pulmonary arteriovenous malformations (PAVMs) universally develop in patients with single ventricle congenital heart disease. Single ventricle PAVMs have been recognized for over 50 years but remain poorly understood. To improve our understanding, we developed a surgical rat model of Glenn circulation and characterized PAVM physiology over multiple time points. We performed a left thoracotomy and end-to-end anastomosis of the left superior vena cava to the left pulmonary artery (unilateral Glenn), or sham surgical control. To assess PAVM physiology, we quantified intrapulmonary shunting using two independent methods (bubble echocardiography and fluorescent microsphere injection). Additionally, we performed arterial blood gas measurements to assess oxygenation and plethysmography to assess ventilation. We identified pathologic intrapulmonary shunting by bubble echocardiography as early as 2 weeks post-Glenn, and shunting continued at 2- and 6-months post-Glenn. Shunting also progressed over time, demonstrated by increased shunting of 10 μm microspheres at 6 months. Shunting was accompanied by mildly decreased oxygenation but no differences in ventilation. Our surgical animal model of unilateral Glenn circulation recreates the clinical condition of single ventricle PAVMs with early and progressive intrapulmonary shunting. This model is poised to characterize single ventricle PAVM pathophysiology and lead to mechanistic and therapeutic discovery., (© 2024 The Author(s). Physiological Reports published by Wiley Periodicals LLC on behalf of The Physiological Society and the American Physiological Society.)

Arteriovenous Malformations are complex and challenging entities, and their treatment is often tailored on the patient. Interstitial sclerosing treatment with bleomycin is promising especially in cases where the aesthetical and/or functional burden of surgical intervention would be impactful as in the S3 AVM according to the SECg classification. We treated 15 patients presenting small (<10cm3) S3 arterio-venous malformations with 3 + 3 sessions of 15.000 IU of interstitial bleomycin. 9 patients presented moderate to good response to the first 3 sessions and thus surgical re-shaping was performed between the two cycles. US examination was done pre-treatment, before each session and every 3 months after the end of the protocol to assess the arterio-venous malformations. Fifteen of the seventeen patients concluded the protocol. 9 patients underwent surgical re-shaping while 6 skipped it because of their excellent response to the first 3 bleomycin injections. Major improvement in terms of appearance and symptoms (pulsations, ulcerations, bleeding or pain) were always achieved. There was a 20% of minor complications. The presented data are encouraging and seem to suggest that this may be a promising protocol to extend the use of bleomycin in arterio-venous malformations and reduce the impact of surgery., (Copyright © 2024 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Background: Previous reports have demonstrated the safety and efficacy of performing early Kawashima procedure (KP). Despite this, more recent studies have shown that the average age at the time of operation remains greater than one year of age. We report our experience with performing KP at an earlier age than previously reported., Methods: A retrospective review was completed of patients undergoing KP at a single center (January 2000 to June 2020). Clinical outcomes were examined., Results: Initial palliation was performed in 11 out of 12 patients. Age and weight at time of KP were 8.25 months (6.4-9.7) and 7.7 kg (6.5-8.6). Intensive care unit stay was 3.16 days (1-12), overall length of stay was 9.5 days (3-22). There was one unplanned reoperation, and no mortality in the cohort. Discharge oxygen saturation was 88% (80%-98%). Seven patients developed pulmonary arteriovenous malformations (PAVMs) with six proceeding to hepatic vein incorporation (HVI). Interval time to development of PAVMs was 42.3 months (16-121). Four of the 12 patients were left with antegrade pulmonary blood flow (PBF) and three (75%) remain without PAVMs. Median follow-up was 10 years (1.5-22) with 11 of 12 (91.67%) survival for the cohort., Conclusions: The KP can be done at a younger age than previously reported with adequate early and late results. Most patients will go on to develop PAVMs and require HVI but leaving patients with some antegrade PBF is likely protective but will need further investigation to show definitive benefit., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Both arteriovenous malformation (AVM) and trisomy 21 are rare diseases. Studies have shown that individuals with trisomy 21 may have potential vascular malformations, with the main site of onset being the portal system. This case involved an adult patient with trisomy 21 who presented with pelvic iliac arteriovenous malformations. The patient underwent interventional treatment, and the prognosis was favorable. It is important to enhance the screening for vascular malformations in patients with trisomy 21 syndrome. At the same time, interventional treatment for AVMs is effective and increasingly important for managing AVMs., (© 2024. The Author(s).)

Intraosseous occurrence of a spinal AVM is anecdotal, with only four such cases reported previously. This is the first report of a spinal intraosseous AVM in the cervical vertebrae. A 44-year-old male patient presented with a 2-month history of progressive quadriparesis and bladder dysfunction. Magnetic resonance imaging showed multiple flow voids within the C4 and C5 vertebral bodies, and an extradural component causing cord compression. CT showed extensive bony destruction at both levels. The diagnosis of an intraosseous AVM was confirmed with spinal angiography. The AVM was noted to be fed by branches from the ascending cervical arteries and the vertebral artery. The nidus was draining into the vertebral venous plexus and thence into the jugular vein through the marginal sinus. The patient underwent partial embolization of the AVM. Surgical resection was attempted but found to be unfeasible due to torrential bleeding. A 360-degree stabilization along with decompressive laminectomies was performed, resulting in clinical improvement and disease stabilization at one year follow-up. The case and its management dilemmas are discussed in light of a brief literature review., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Austria, part of Springer Nature.)

A 42-year-old man was referred to our hospital because of anemia. The patient underwent gastroscopy and colonoscopy, but no bleeding site was detected. Abdominal contrast-enhanced computed tomography (CT) showed vascular dilatation along the wall of the small intestine. Small bowel capsule endoscopy and antegrade double-balloon endoscopy (DBE) were performed, and the patient was diagnosed with a small intestinal arteriovenous malformation (AVM). The AVM was clipped using DBE. After clipping, abdominal contrast-enhanced CT and small bowel angiography revealed the disappearance of the AVM. DBE may be a viable therapeutic option, helping avoid surgery and its associated risks.

Introduction: Brain arteriovenous malformations (bAVMs) present complex challenges in neurosurgery, requiring precise pre-surgical planning. In this context, 3D printing technology has emerged as a promising tool to aid in understanding bAVM morphology and enhance surgical outcomes, particularly in pediatric patients. This study aims to assess the feasibility and effectiveness of using 3D AVM models in pediatric bAVM surgery., Methodology: The study was conducted at Great Ormond Street Hospital, and cases were selected sequentially between October 2021 and February 2023. Eight pediatric bAVM cases with 3D models were compared to eight cases treated before the introduction of 3D printing models. The 3D modelling fidelity and clinical outcomes were assessed and compared between the two cohorts., Results: The study demonstrated excellent fidelity between 3D models and actual operative anatomy, with a median difference of only 0.31 mm. There was no statistically significant difference in angiographic cure rates or complications between the 3D model group and the non-3D model group. Surgical time showed a non-significant increase in cases involving 3D models. Furthermore, the 3D model cohort included higher-grade bAVMs, indicating increased surgical confidence., Conclusion: This study demonstrates the feasibility and efficacy of utilizing 3D AVM models in pediatric bAVM surgery. The high fidelity between the models and actual operative anatomy suggests that 3D modelling can enhance pre-surgical planning and intraoperative guidance without significantly increasing surgical times or complications. Further research with larger cohorts is warranted to confirm and refine the application of 3D modelling in clinical practice., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Treatment for large (> 10 mL) arteriovenous malformations (AVMs) remains highly challenging. This study evaluated long-term effect of time-staged gamma knife radiosurgery (GKS) for large AVMs., Methods: For patients with large AVMs treated by time-staged GKS over 10 years, time-staged GKS was repeated every three years targeting the entire nidus if total obliteration was not achieved. Obliteration rate and post-GKS complications were assessed based on 10 mL volume interval of AVMs. Prognostic factors for these outcomes were evaluated using Cox regression analysis., Results: Ninety-six patients were analyzed. For AVMs in the 10-20 mL subgroup, a dose ≥ 13.5Gy yielded higher obliteration rate in the first GKS. In the 20-30 mL subgroup, a second GKS significantly boosted obliteration. AVMs > 30 mL did not achieve any obliteration with the first GKS. Among 35 (36.4%) cases lost to follow-up, 7 (7.2%) were lost due to GKS complications. Kaplan-Meier analysis showed that each subgroup needed different time for achieving 50% favorable obliteration outcome rate: 3.5, 6.5, and 8.2 years for 10-20 mL, 20-30 mL, and > 30 mL subgroup, respectively. Total obliteration rate calculated by intention-to-treat method: 73%, 51.7%, 35.7%, respectively, 61.5% overall. Post-GKS hemorrhage and chronic encapsulated expanding hematoma (CEEH) occurred in 13.5% and 8.3% of cases, respectively. Two patients died. Dose and volume were significant prognostic factors for obliteration. Initial AVM volume was a significant prognostic factor of post-GKS hemorrhage and CEEH., Conclusion: Time-staged GKS for large AVMs less than 30 mL has highly favorable long-term outcome and a tolerable complication rate., Competing Interests: The authors have no potential conflicts of interest to disclose., (© 2024 The Korean Academy of Medical Sciences.)

Background: At present, there is no golden standard for treatment of extracranial arteriovenous malformations (AVMs) and recurrence remains a major challenge with limited available evidence on the associated factors. This study aimed to evaluate the effectiveness of surgical treatment options, in terms of size reduction, symptoms, and early recurrence in patients treated surgically for AVMs., Methods: A retrospective cohort study was conducted to evaluate patients with AVMs following surgical treatment in 2 centers from 2005 to 2020. Posttreatment lesion size and symptoms, as well as recurrence, were assessed. Multiple regression analysis was performed to identify factors associated with recurrence., Results: Forty-four surgical treatment cases in 31 patients were assessed with a mean follow-up duration period of 67.9 ± 39.5 months. Treatment included total resection in 26 cases (59.1%) and partial resection 18 (40.9%), with free flap coverage used in 19 cases (43.2%). No acute exacerbation following treatment was observed in our cohort. Total resection significantly reduced posttreatment lesion size ( P < 0.001), symptoms ( P < 0.001), and recurrence (20.0%, P = 0.03). The recurrence rate was significantly higher after partial resection (73.7%, P = 0.03). Total resection was identified as an associated factor for significantly reduced AVM recurrence (odds ratio: 0.12; 95% confidence interval: 0.03, 0.52). However, the use of free flaps did not significantly reduce recurrence, post treatment size or improve AVM symptoms., Conclusions: Total resection is the optimal treatment for AVMs. Free flaps are useful in covering large defects but the regulative effect of free flap remains controversial., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Vascular malformations in the extremities are a common site of occurrence; arteriovenous malformations (AVMs) are the least frequent of all vascular malformations, estimated at 5% to 20%. The first step in management is to perform a thorough clinical examination. Symptoms are assessed, and staging is performed using the Schobinger classification. Next, ultrasonography and contrast-enhanced computed tomography are used to confirm the diagnosis of AVM and to confirm the extent of the malformation. Surgery is the first-line treatment and reconstruction is performed. In cases where surgery is not feasible, embolization and sclerotherapy may be used to alleviate symptoms., Competing Interests: Disclosures Conflict of interests: None. Statement of financial disclosure: None of the authors have a financial interest in the discussed article. No funding was received for this article., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: To evaluate the efficacy of the combined approach of preoperative endovascular embolization (EE) and surgical excision (SE) for scalp arteriovenous malformation (AVM) and present an illustrative case report., Methods: A systematic review was conducted using online databases (PubMed/Medline, Cochrane, and Embase) on February 15, 2023. The inclusion criteria were any type of study of patients with scalp AVMs who were diagnosed and confirmed through angiography and treated with combined preoperative EE and SE. All the articles that met the inclusion criteria were included in this study., Results: A total of 49 articles (91 patients) were included. The patients' age ranged from 10 days to 70 years at the time of presentation. The most common symptoms were a pulsatile mass in 51 patients (56.04%), progressively growing mass in 31 patients (34.06%), and bruits and/or thrills in 22 patients (24.17%). Complications of preoperative EE and SE were observed in only 5 patients; 3 patients (3.29%) had harvested skin graft marginal necrosis, 1 patient (1.09%) had skin necrosis, and 1 patient (1.09%) had a wound infection. Only 2 patients (2.19%) reported a recurrent or residual mass during a median follow-up period of 12 months., Conclusions: The management of scalp AVMs can be challenging; therefore, focused, and accurate identification of the complexity of the vascular anatomy is required. The combined method of preoperative EE and SE showed satisfactory outcomes with low rates of complications and recurrence; thus, we recommend this approach for the management of scalp AVMs., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Arteriovenous malformations of the head and neck are rare and inborn errors of vascular morphogenesis. They pose therapeutic challenges owing to their bleeding tendency and a high chance of recurrence. Treatment modalities include digital subtraction angiography with embolisation as a primary treatment or can be done preoperatively followed by surgical excision. We present a case of recurrent arteriovenous malformation of the postauricular region in an adolescent male who was taken up for upfront surgical excision with the help of a tumescent solution, thereby eliminating the need for embolisation and drastically reduced intraoperative blood loss., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

A previously healthy adult male presented with a slowly enlarging orbital mass associated with 5 mm of non-pulsatile proptosis. On imaging, a soft tissue lesion with avid contrast enhancement and associated bony hyperostosis was noted. The lesion and hyperostotic bone were surgically debulked, and significant arterial bleeding was noted intraoperatively consistent with an arteriovenous malformation. Histopathologic analysis revealed a vascular malformation with enhanced microvasculature infiltrating the periosteum. While vascular lesions elsewhere in the body can be associated with skeletal changes, bony hyperostosis is a rare feature of orbital vascular malformations.

BACKGROUND An arteriovenous malformation (AVM) is an abnormal connection between an artery and a vein, bypassing the capillary network. An AVM of the superficial temporal artery (STA) can occur after trauma, iatrogenic injury, infection, or spontaneously. Spontaneous, or iatrogenic, presentations of STA AVM are thought to be rare, with very few reported cases. Symptoms include local pain, headache, tinnitus, or paresthesia, in addition to a palpable mass associated with thrill on palpation. Options for diagnosis include intra-arterial angiography, doppler ultrasound, magnetic resonance angiography (MRA), and computed tomography angiography (CTA). Current management options include surgical excision, ligation, and embolization; however, it is unknown which treatment is superior in terms of recurrence and which carries a lower risk of complications. CASE REPORT We present a case of a spontaneous STA AVM in a 76-year-old woman with past medical history significant for seasonal allergies and hyperlipidemia, who presented with pulsatile tinnitus and a palpable, tender mass located to the left temporal area. The mass had been present for several years, with gradual increase in size two to three years prior to presentation. She denied any history of trauma or procedure prior to presentation of the pulsatile mass. She underwent open excision with complete resolution of symptoms and no recurrence at 11-month follow-up. CONCLUSIONS AVM of the STA is a condition that can occur secondary to trauma, infection, iatrogenic injury, or spontaneously. Spontaneous, or iatrogenic, presentations of STA AVM are thought to be rare, with very few cases documented in the literature. Surgical treatment remains the standard of management, with options including surgical excision, ligation, or embolization.

Objective: Conus medullaris arteriovenous malformation (AVM) is rare and challenging to treat. To better define the presentation, prognosis, and optimal treatment of these lesions, the authors present their treatment experiences for conus medullaris AVM., Methods: Eleven patients with AVM of the conus medullaris were identified between March 2013 and December 2021. Among these patients, 7 who underwent microsurgical treatment were included. Patient data, including age, sex, symptoms at presentation, neurological status, radiological findings, nidus depth (mainly pial lesion vs intramedullary lesion), type of treatment, and recurrence at follow-up, were collected. Postoperative angiography was performed in all patients. Spinal cord function was evaluated using the Frankel grade at the time of admission and 1 year after surgery., Results: All 7 patients presenting with myeloradiculopathy were treated surgically. Four patients (57.1%) underwent endovascular embolization, followed by resection. The other 3 patients underwent microsurgery only. Complete occlusion was confirmed with postoperative angiography in all patients. Of the 3 patients who were nonambulatory before surgery (Frankel grade C), 2 were able to walk after surgery (Frankel grade D) and 1 remained nonambulatory (Frankel grade C) at 1-year follow-up., Conclusions: Based on the authors' clinical experiences, the results of multimodal treatment for conus medullaris AVM are good, with microsurgical treatment playing an important role. The microsurgical strategy can differ depending on the location of the nidus, and when possible, good results can be expected through microsurgical resection.

There continuous to be widespread misuse of nomenclature used to described vascular anomalies, This is even more pronounced in the case of intra-osseous lesions. Bone involvement is more common with vascular malformations and extremely rare in haemangiomas. An accurate diagnosis is mandatory for tailored management and often based on a thorough history, clinical examination, and cross-sectional imaging. Surgery remains the main stay for the management of symptomatic venous malformations. Embolisation with or without surgery is the main stay for arteriovenous malformations. Virtual surgical planning, with surgical guides and patient specific implants help achieve predictably excellent results., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Background: The prognosis for spinal artery aneurysms associated with spinal cord arteriovenous malformations (AVMs) is poor because of the high rupture rate of aneurysms. However, endovascular treatment remains technically difficult because the catheter system must be constructed via the small-caliber anterior spinal artery (ASA) or posterior spinal artery (PSA), which feeds functionally eloquent spinal cord. A 2.6F Carnelian HF-S microcatheter (Tokai Medical Products, Aichi, Japan) has been specifically designed to assist a 1.6F Carnelian MARVEL S microcatheter (Tokai Medical Products) as a small-profile 'platform catheter' close to the target lesion. Here we present a prenidal ASA aneurysm treated using a 2.6F Carnelian HF-S microcatheter as an intraspinal canal platform catheter and review related literature., Case Presentation: A 50-year-old man presented with a subarachnoid haemorrhage due to cervical spinal cord AVM. Diagnostic vertebral angiography revealed the AVM supplied by the PSA originated from the right C2 segmental artery and ASA arising from the right V4 segment. Superselective angiography for each feeder was achieved through a 2.6F Carnelian HF-S microcatheter, and a prenidal ASA aneurysm was diagnosed, which was clinically consistent with haemorrhagic origin. A 1.6F Carnelian MARVEL S microcatheter was cannulated into the aneurysm through the 2.6F Carnelian HF-S microcatheter positioned at the ASA. The aneurysm coiling was successfully performed without system instability or periprocedural complications., Conclusions: Only a few cases have described endovascular treatment for spinal artery aneurysms. To date, no reports have been published regarding the use of an intraspinal canal platform catheter to treat spinal artery aneurysms. A 2.6F Carnelian HF-S microcatheter served as a useful intraspinal canal platform catheter for coil embolization of the ASA aneurysm. This system can provide excellent accessibility and controllability for endovascular treatment of spinal artery lesions.

Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Objective: To present a case of concurrent uterine arteriovenous malformation (AVM) and isthmocele, treated with ethylene vinyl alcohol copolymer (EVAC) embolization of the AVM followed by robotic isthmocele repair., Design: A stepwise video demonstration with narration., Setting: A tertiary care academic hospital. Patient is a 37-year-old with one previous cesarean section who presented with persistent heavy vaginal bleeding after a dilation and evacuation procedure. Imaging showed evidence of an isthmocele and an iatrogenic uterine AVM secondary to the dilation and evacuation procedure. Both entities are morbid conditions associated with significant operative blood loss. Embolization of the acquired AVM was first performed to stabilize bleeding. In addition, owing to the extensive uterine defect and history of infertility, surgical repair of the isthmocele was recommended., Interventions: A multidisciplinary approach combining interventional radiology and gynecologic surgery expertise, implementing several strategies to minimize blood loss: 1. Image-guided uterine AVM embolization with EVAC [1] 2. Hysteroscopic identification of isthmocele and residual EVAC in the cavity, with fluorescence transillumination to clearly delineate isthmocele borders 3. Robot-assisted laparoscopic approach for bladder flap creation, as well as retroperitoneal space dissection to skeletonize uterine arteries 4. Transient occlusion of uterine arteries using vascular clamps to minimize operative blood loss given the isthmocele size and its proximity to the left uterine artery 5. Resection of the isthmocele and removal of residual intracavitary EVAC 6. Multilayer, bidirectional hysterotomy closure and vascular clamp removal to restore uterine blood supply CONCLUSIONS: Successful multidisciplinary treatment of concurrent uterine AVM and isthmocele. Cesarean delivery at 36 to 37 weeks' gestational age was recommended for future deliveries., (Copyright © 2023 AAGL. Published by Elsevier Inc. All rights reserved.)

Background: Cutaneous erythema is one of the most common signs of arteriovenous malformations (AVMs) in the head and neck region, influencing aesthetic appearance. Surgical resection of AVMs may lead to cicatrization of the skin or aggravation of the lesion. Laser treatment, although effective in improving superficial vascular lesions, cannot prevent deep AVMs from further development., Objective: The authors propose an absolute ethanol embolization therapy that can effectively and safely eradicate the nidus with a favorable aesthetic outcome., Methods: The authors conducted a retrospective observational study of 14 AVM patients with distinct cutaneous erythema in the head and neck region undergoing embolotherapy in a single primary care center. Symptoms before and after treatment, complications, and degree of devascularization were recorded and assessed. Changes in cutaneous redness were evaluated using a previously reported quantitative measurement., Results: Complete symptomatic relief was observed in 5 patients, and major improvement was observed in 9 patients. The mean Δ a * value of the color change had a significant reduction of 6.50 ± 4.04, p < .001, indicating a remarkable remission of cutaneous erythema., Conclusion: Ethanol embolization is an effective and safe treatment for head and neck AVMs with excellent aesthetic outcomes and might become a potential treatment method for other superficial vascular anomalies., (Copyright © 2023 by the American Society for Dermatologic Surgery, Inc. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Background: Chronic encapsulated intracerebral hematoma (CEICH) is a rare type of intracerebral hematoma (ICH) with peculiar radiological features and presenting as subtle slow-growing lesion exerting mass effect. We performed a systematic review of the literature focused on diagnosis and management of patients affected by CEICH., Material and Methods: A literature search according to the PRISMA statement was conducted using PubMed and Scopus databases and pertinent Mesh terms. All papers that reported intraventricular CEICH, or CEICH cases treated conservatively or by CT-guided needle aspiration were not included in this study. A total of 40 papers were included in this review, with 58 patients (38 males and 20 females) and a mean age of 41.44 ± 20.05 years (range 1-80)., Results: Neurological symptoms of onset include those related to an increase in intracranial pressure (ICP) in 28/58 cases (48.2%), seizures in 17/58 cases (29.3%), motor deficits in 14/58 cases (24.1%). The most frequent localization is atypical in 45/58 cases (77.6%). Surgical approach is not specified in 21/58 cases (36.2%), craniotomy was performed in 31/58 cases (53.4%), craniectomy in 5/58 cases (8.6%) and only in one case (1.7%) an endoscopic approach was performed. CEICH are usually located in an atypical site., Conclusions: There is not an association with anticoagulants and antiplatelets intake. Arteriovenous malformation is the most frequent cause. Surgery is suggested, and craniotomy is the most used approach even if further investigation should be directed to analyze the efficacy of endoscopic approach of these lesions, which may show favorable outcome.

Background: Uterine arteriovenous malformation (UAVM) is a relatively rare but potentially life-threatening situations abnormal vascular connections between the uterine arterial and venous systems. Lack of recognized guidelines and clinic experience, there is a lot of clinic problems about diagnosis and treatment. By analyzing the clinical data of patients with pregnancy-related UAVM, we aim to confirm the safety of direct surgeries and the benefit of pretreatment (uterine artery embolization or medical therapy) before surgery, and to explore more optimal therapies for patients with pregnancy-related UAVM., Methods: A total of 106 patients in Qilu Hospital of Shandong University from January 2011 to December 2021 diagnosed of pregnancy-related UAVM were involved in this study. Depending on whether preoperative intervention was performed, the patients were divided into direct surgery group and pretreatment group (uterine artery embolization or medical management). Clinical characteristics, operative related factors and prognosis were analyzed., Results: The most common symptom of pregnancy-related UAVM was vaginal bleeding (82.5%), which could also be accompanied by abdominal pain. Pretreatments (uterine artery embolization or medical therapy) had no obvious benefit to the subsequent surgeries, but increased the hospital stay and hospital cost. Direct surgery group had satisfactory success rate and prognosis compared to pretreatment group., Conclusion: For pregnancy-related UAVM, direct surgery has good effects and high safety with shorter hospital stays and less hospital cost. What is more, without uterine artery embolization and other medical therapy, patients could remain better fertility in future., (© 2023. BioMed Central Ltd., part of Springer Nature.)

A significant contributing factor to the progression of late cyanosis in individuals undergoing Kawashima operation is pulmonary arteriovenous malformations. Following the Fontan procedure, arteriovenous malformations may regress. However, in cases with extensive malformations causing severe cyanosis, lobectomy can also be a possible treatment approach. Thereby, we present our two-step treatment strategy in a late Fontan completion complicated by arteriovenous malformations in a Kawashima patient.

Background: Although case reports of video-assisted thoracic surgery (VATS) for pulmonary arteriovenous malformation (PAVM) have been published, studies analyzing more than 10 cases were limited. A retrospective single-arm cohort study was performed to investigate the efficacy of VATS in 23 consecutive patients with idiopathic peripherally located simple type PAVM., Methods: VATS was performed for wedge resection of 24 PAVMs in 23 patients, which included 4 males and 19 females with an age range of 25 to 80 years (mean: 59.6 ± 13.0). Two patients underwent simultaneous resection of lung carcinoma, one by wedge resection and another by lobectomy. Each medical record was analyzed according to the resected specimen, bleeding volume, postsurgical hospital stay length, duration of chest tube placement, and VATS time. The distance between pleural surface/fissure and PAVM was measured on CT, and the influence of this distance on identification of PAVM was investigated., Results: In all 23 patients, VATS was successfully performed, and the venous sac was included in each resected specimen. Bleeding volume was less than 10mL in all but one with 1900 mL bleeding volume due to simultaneous lobectomy for carcinoma, not wedge resection of PAVM. Postsurgical hospital stay length, duration of chest tube placement, and VATS time were 5.0 ± 1.4 days, 2.7 ± 0.7 days, and 49.3 ± 39.9 min, respectively. In 21 PAVMs with a distance of 1 mm or less, purple vessel or pleural bulge of PAVM was identified soon after insertion of a thoracoscope. In the remaining 3 PAVMs with a distance of 2.5 mm or more, additional efforts were needed for identification., Conclusion: VATS was found to be a safe and effective to treatment for idiopathic peripherally located simple type PAVM. When the distance between pleural surface/fissure and PAVM was 2.5 mm or more, a plan and strategy for identification of PAVM should be prepared before VATS., (© 2023. The Author(s).)

Background: Scalp arteriovenous malformation (AVM) is a rare congenital disease that may present with massive bleeding. To date, surgical excision remains the definitive management. However, the procedure could lead to intraoperative bleeding due to the tumor's high blood flow and complex vascularity., Case Report: A 49-year old Filipino male presented with a bleeding giant scalp AVM. Computed tomographic scan and duplex studies showed multiple feeding vessels with turbulent flow arising primarily from the right superficial temporal, right posterior auricular, and occipital vessels. Prior to surgery, the patient underwent transfusion due to preoperative hemoglobin of 6 g/dL. Proximal control of the right external carotid artery was performed through a supine position and left in place to reduce the majority of blood flow to the AVM. The patient was turned to a prone position for surgical planning to achieve maximal skin-sparing dissection prior to excision. First, ligation of bilateral superficial temporal and posterior auricular arteries was performed. Next, excision above the periosteum with segmental ligation of feeding vessels around the AVM was carried out. Reconstruction of the defect was done via scalp advancement flap and split-thickness skin grafting. Intraoperative blood loss was 1.6 L. On the sixth postoperative day, the patient was discharged with 100% graft take., Conclusion: Management of scalp AV malformation is challenging, and despite measures to decrease intraoperative bleeding, blood loss is still high. While preoperative embolization has been reported to decrease the risk of bleeding, this procedure is not currently available in our setting. Our case highlights the complexity of giant scalp AV malformation management in a limited-resource setting. Even in the absence of endovascular intervention, outright surgical excision of AVM can be performed, albeit with higher levels of blood loss.

Microvascular pulmonary arteriovenous malformations (pAVMs) can lead to profound hypoxemia. "Hepatic factor" is postulated to play a role in their development. Certain patients with congenital heart disease are at particular risk to develop pAVMs, including those with heterotaxy syndromes and complex Fontan palliation. Ideally, an underlying cause is identified and corrected, although pAVMs may persist despite those interventions. We report a patient with heterotaxy syndrome s/p Fontan who had pAVMs that persisted despite Fontan revision with equal hepatic flow to both lungs. We employed a novel method to produce a diabolo configuration of a large covered stent to restrict lung flow while maintaining the potential for future dilation.

Background: Intramedullary spinal arteriovenous malformation (ISAVM, glomus type) is a type of spinal cord arteriovenous malformation, which is a rare disease known often to have a complex vascular supply interfering with that of the spinal cord, and is in complex anatomical relations with cord structures and nerve roots. Though microsurgical and endovascular treatment has mainly been the standard options, in high-risk cases with these treatments, stereotactic radiotherapy (SRT) might be the option of choice., Methods: We retrospectively reviewed 10 consecutive patients with ISAVM who received SRT using CyberKnife at the Japanese Red Cross Medical Center (Tokyo, Japan) from January 2011 to March 2022., Results: No case in this series suffered from hemorrhage after applying SRT. One case experienced neurological impairment 10 years after SRT, which we attributed to venous congestion due to the remaining lesion. No case of radiation myelopathy was observed in this series. In one case, the nidus volume reduction and loss of flow voids were obvious, though improvement in the neurological outcome was not apparent. No radiological changes were observed in the other 9 patients., Conclusions: Even in lesions without radiological changes, no hemorrhagic events were observed for an average period of 4 years. SRT may be a feasible option in treating ISAVM, especially for lesions in which microsurgical resection and endovascular treatment are inapplicable. To ascertain the safety and efficacy of this approach, further studies with more patients and longer follow-up is required., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Objective: To propose and develop a histopathological criteria to help diagnose vascular malformations., Methods: All patients who underwent surgical resection and had a confirmed histopathological diagnosis of vascular malformations from 01 March 2018-26 February 2020 were included. A criteria based on 10 parameters was developed to help diagnose vascular malformations. Discrepancies between clinical and histopathological diagnosis were evaluated., Results: A total of 18 cases were identified. There was a discrepancy between the clinical diagnosis and the initially reported histopathological diagnosis in 16 cases (88.9%). This was reduced to 7 (38.9%) and 6 cases (33.3%) with first and second time revised histopathological analysis using proposed criteria., Conclusions: The discrepancy between clinical and histopathological diagnoses of vascular malformations has highlighted the requirement of an agreed criteria for histopathologists to help formulate their diagnosis. The proposed criteria may be used as a guide in addressing this and guide treatment and improve clinical practice.

Background: Uterine arteriovenous malformation(AVM) refers to the abnormal direct traffic between uterine arteries and veins, which can be characterized by the imaging examination, showing increased uterine vascularity and arteriovenous shunting. However, similar imaging manifestations can also be seen in a variety of conditions including retained production of conception, gestational trophoblastic disease, placental polyp, and vascular neoplasm., Case Presentation: Here we present a case of a 42-year-old woman who was suspected of suffering uterine AVM indicated by Doppler sonography and magnetic resonance imaging but was finally diagnosed with a persistent ectopic pregnancy located on the right uterine corner by pathology after laparoscopy. She recovered well after surgery., Conclusion: Uterine AVM is a rare and serious condition. In general, it presents special radiological manifestations. However, when complicated with other diseases it can also be distorting. Standardized diagnosis and management are important., (© 2023. The Author(s).)

In the context in intraventricular haemorrhage (IVH), intrathecal thrombolytic agents administered in conjunction with extraventricular drainage have been demonstrated to clear larger volumes of blood and reduce mortality rates. However, patients with arteriovenous malformations (AVM) have been mostly excluded from clinical trials. We describe a patient with hydrocephalus secondary to a ruptured AVM who was treated via external ventriculostomy, which was subsequently converted to a ventriculoperitoneal shunt (VPS). Eight months later, the AVM re-ruptured, causing IVH and rendering the patient comatose. Taking into consideration the patient's poor outlook, a single dose of intraventricular tissue plasminogen activator (t-PA) was administered through the shunt reservoir. The shunt maintained its function and the patient's condition ultimately improved. This impressive case demonstrates the utility of t-PA administered through an existing VPS in the setting of IVH due to ruptured AVM, highlighting its lifesaving potential in the appropriate patient and overall decrease in the cost of care by mitigating the need for shunt revision.

Objectives: The objective of this study is to evaluate the user-defined optimization settings in the Fast Inverse Planning (FIP) optimizer in Leksell GammaPlan® and determine the parameters that result in the best stereotactic radiosurgery (SRS) plan quality for brain metastases, benign tumors, and arteriovenous malformations (AVMs)., Methods: Thirty patients with metastases and 30 with benign lesions-vestibular schwannoma, AVMs, pituitary adenoma, and meningioma-treated with SRS were evaluated. Each target was planned by varying the low dose (LD) and beam-on-time (BOT) penalties in increments of 0.1, from 0 to 1. The following plan quality metrics were recorded for each plan: Paddick conformity index (PCI), gradient index (GI), BOT, and maximum organ-at-risk (OAR) doses. A novel objective score matrix was calculated for each target using a linearly weighted combination of the aforementioned metrics. A histogram of optimal solutions containing the five best scores was extracted., Results: A total of 7260 plans were analyzed with 121 plans per patient for the range of LD/BOT penalties. The ranges of PCI, GI, and BOT across all metastatic lesions were 0.58-0.97, 2.1-3.8, and 8.8-238 min, respectively, and were 0.13-0.97, 2.1-3.8, and 8.8-238 min, respectively, for benign lesions. The objective score matrix showed unique optimal solutions for metastatic lesions and benign lesions. Additionally, the plan metrics of the optimal solutions were significantly improved compared to the clinical plans for metastatic lesions with equivalent metrics for all other cases., Conclusion: In this study, FIP optimizer was evaluated to determine the optimal solution space to maximize PCI and minimize GI, BOT and OAR doses simultaneously for single metastatic/benign/non-neoplastic targets. The optimal solution chart was determined using a novel objective score which provides novice and expert planners a roadmap to generate the most optimal plans efficiently using FIP., (© 2023 The Authors. Journal of Applied Clinical Medical Physics published by Wiley Periodicals, LLC on behalf of The American Association of Physicists in Medicine.)