Low-flow Vascular Malformations Research Articles

Combination of lauromacrogol and bleomycin in the treatment of low-flow vascular malformations: a web-based meta-analysis.

Combination of lauromacrogol and bleomycin in the treatment of low-flow vascular malformations: a web-based meta-analysis.

Magnetic resonance - guided treatment of low-flow vascular malformations and the technologies to potentiate adoption.

Vascular malformations are congenital, non-neoplastic lesions that arise secondary to defects in angiogenesis. Vascular malformations are divided into high-flow (arteriovenous malformation) and low-flow (venous malformations and lymphatic malformations). Magnetic resonance imaging (MRI) is the standard for pre-and post-intervention assessments, while ultrasound (US), X-ray fluoroscopy and computed tomography (CT) are used for intra-procedural guidance. Sclerotherapy, an image-guided therapy that involves the injection of a sclerosant directly into the malformation, is typically the first-line therapy for treating low-flow vascular malformations. Sclerotherapy induces endothelial damage and necrosis/fibrosis with eventual involution of the malformation. Image-guided thermal therapies involve freezing or heating target tissue to induce cell death and necrosis. MRI is an alternative for intra-procedural guidance and monitoring during the treatment of vascular malformations. MR can provide dynamic, multiplanar imaging that delineates surrounding critical structures such as nerves and vasculature. Multiple studies have demonstrated that MR-guided treatment of vascular malformations is safe and effective. This review will detail (1) the use of MR for the classification and diagnosis of vascular malformations, (2) the current literature surrounding MR-guided treatment of vascular malformations, (3) a series of cases of MR-guided sclerotherapy and thermal ablation for the treatment of vascular malformations, and (4) a discussion of technologies that may potentiate interventional MRI adoption including high intensity focused ultrasound and guided laser ablation.

Deep learning-based differentiation of peripheral high-flow and low-flow vascular malformations in T2-weighted short tau inversion recovery MRI.

Differentiation of high-flow from low-flow vascular malformations (VMs) is crucial for therapeutic management of this orphan disease. A convolutional neural network (CNN) was evaluated for differentiation of peripheral vascular malformations (VMs) on T2-weighted short tau inversion recovery (STIR) MRI. 527 MRIs (386 low-flow and 141 high-flow VMs) were randomly divided into training, validation and test set for this single-center study. 1) Results of the CNN's diagnostic performance were compared with that of two expert and four junior radiologists. 2) The influence of CNN's prediction on the radiologists' performance and diagnostic certainty was evaluated. 3) Junior radiologists' performance after self-training was compared with that of the CNN. Compared with the expert radiologists the CNN achieved similar accuracy (92% vs. 97%, p = 0.11), sensitivity (80% vs. 93%, p = 0.16) and specificity (97% vs. 100%, p = 0.50). In comparison to the junior radiologists, the CNN had a higher specificity and accuracy (97% vs. 80%, p < 0.001; 92% vs. 77%, p < 0.001). CNN assistance had no significant influence on their diagnostic performance and certainty. After self-training, the junior radiologists' specificity and accuracy improved and were comparable to that of the CNN. Diagnostic performance of the CNN for differentiating high-flow from low-flow VM was comparable to that of expert radiologists. CNN did not significantly improve the simulated daily practice of junior radiologists, self-training was more effective.

Intralesional injection of bleomycin in the management of low flow vascular malformations: Results and factors affecting the outcome

ObjectiveEthanol has been a commonly used sclerosant for low-flow vascular malformations, but it carries a high risk of complications. Bleomycin has been recently introduced as a potentially effective treatment. The aim of this study was to evaluate the safety and efficacy of bleomycin intralesional injection for the treatment of low-flow vascular malformations and determine the different factors affecting the outcome. Patients and methodsA total of fifty patients with low-flow vascular malformations were enrolled in the study between April 2020 and March 2022. All patients underwent preoperative duplex ultrasound and magnetic resonance angiography. The procedure was performed under ultrasound and fluoroscopic guidance. All patients were assessed for the objective improvement, ultrasound assessment, and patient-reported outcome. ResultsThe overall rate of objective improvement was 79.53% (78.05% in venous and 87.5% in lymphatic malformations), whereas 81.25% of the patients showed a degree of size reduction or complete obliteration on postoperative ultrasound. The patient-reported outcome analysis showed a statistically significant improvement in the mean score for the pain, overall symptoms, and self-confidence. On regression analysis, the only factor associated with poor objective outcome was diffuse lesions (ill-defined or extending in more than one body region or one compartment). No major complications were recorded. ConclusionsBleomycin intralesional injection is a safe and effective treatment for low-flow vascular malformations.

Baseline Quality of Life in Pediatric Patients With Low-flow Vascular Malformations.

The aim of this study was to quantify and describe baseline patient and parent-proxy health-related quality of life scores in patients with low-flow vascular malformations at a single, tertiary-care vascular anomalies clinic. This is a retrospective study of data collected on patients with low-flow vascular malformations between the ages of 2 to 25 who were seen at a single, tertiary-care center vascular anomalies clinic. A total of 266 patients are included in this study. Patients with lymphatic malformations report decreased quality of life scores as compared with venous malformations in the emotional, psychological, school, and social domains. Patients with lower extremity malformation report decreased quality of life scores as compared with head/neck, trunk, upper extremity, and multifocal malformations; most notably in the physical domain. Treatment of low-flow vascular malformations should aim to improve patient quality of life. The use of standardized health-related quality of life measures in this study quantifies baseline quality of life scores among patients with low-flow vascular malformations.

Influence of genetic variants on the pharmacokinetics and pharmacodynamics of sirolimus: a systematic review.

Sirolimus is an antiproliferative and immunosuppressive compound inhibiting the mTOR pathway, which is often activated in congenital low-flow vascular malformations. Studies have demonstrated the efficacy of sirolimus for this disease. Studies in kidney transplant patients suggest that genetic variants can influence these pharmacokinetic parameters. Therefore, a systematic literature search was performed to gain insight into pharmacogenetic studies with sirolimus. Most studies investigatedCYP3A4 and CYP3A5, with inconsistent results. No pharmacogenetic studies focusing on sirolimus have been performed for low-flow vascular malformations. We analyzed two common variants of CYP3A4 and CYP3A5 (CYP3A4*22 and CYP3A5*3, respectively) in patients (n=59) with congenital low-flow vascular malformations treated with sirolimus. No association with treatment outcome was identified in this small cohort of patients.

Cerebral cavernomatosis and epilepsy: treatment and serial imaging follow-up

Cerebral malformations, known as cavernomas, are low flow vascular malformations that have a multilobulated appearance. A large percent of cavernous malformations have seizures as initial symptoms. We report a case of a 48 years old patient presented in the neurological department for seizure work-up. We performed serial brain imaging to follow the evolution of bleeding cavernomas and electroencephalography to find epileptogenic cavernomas. Conservative management was decided and the evolution under antiepileptic drugs was good, but with some compliance issues further discussed.

Surgery for Port-Wine Stains: A Systematic Review.

Port-wine stains (PWS) are congenital low-flow vascular malformations of the skin. PWS tend to become thicker and darker with time. Laser therapy is the gold standard and the first-line therapy for treating PWS. However, some resistant PWS, or PWS that have tissue hypertrophy, do not respond to this therapy. Our aim is to evaluate the role of surgery in the treatment of PWS birthmarks. A literature search was performed in PubMed, Scopus, Web of Science (WOS) and Google Scholar for all papers dealing with surgery for port-wine stains, from January 2010 to December 2020 using the search strings: (capillary vascular malformation OR port-wine stains OR Sturge Weber Syndrome OR sws OR pws) AND (surgical OR surgery). Ten articles were identified and used for analysis. They were almost all case series with a short follow up period and lacked an objective-systematic score of evaluation. Delay in treatment of port wine stains may result in soft tissue and bone hypertrophy or nodules with disfiguring or destructive characteristics. The correction of PWS-related facial asymmetry often requires bone surgery followed by soft tissue corrections to achieve a more harmonious, predictable result.

Abstract No. 577 Mixed-Methods Assessment of the Patients-Reported Outcome Measure for Vascular Malformations Questionnaire (PROVAM) in Patients with Low-Flow Vascular Malformations: A Prospective Cohort Study

Abstract No. 577 Mixed-Methods Assessment of the Patients-Reported Outcome Measure for Vascular Malformations Questionnaire (PROVAM) in Patients with Low-Flow Vascular Malformations: A Prospective Cohort Study

Abstract No. 200 Comparison Between Patients-Reported Outcome Measure for Vascular Malformations Questionnaire (PROVAM) and Outcome Measures for VAscular Malformations (OVAMA) Responses in Patients with Low-Flow Vascular Malformations

Abstract No. 200 Comparison Between Patients-Reported Outcome Measure for Vascular Malformations Questionnaire (PROVAM) and Outcome Measures for VAscular Malformations (OVAMA) Responses in Patients with Low-Flow Vascular Malformations

Subcutaneous Granuloma Annulare vs. Subcutaneous Vascular Malformations in Children: A Diagnostic Challenge.

There are various subcutaneous lesions in children and often there is difficulty in obtaining an accurate diagnosis by non-invasive diagnostic procedures. Subcutaneous granuloma annulare (SGA) is a rare granulomatous disease that, even after imaging, is often mistaken for a low-flow subcutaneous vascular malformation (SVM). This study aimed to accurately identify clinical and imaging clues to distinguish SGA from low-flow SVM. We retrospectively analyzed complete hospital records of all children with a confirmed diagnosis of SGA and low-flow SVM who underwent MR imaging at our institution from January 2001 to December 2020. Their disease history, clinical and imaging findings, management, and outcome were evaluated. Among 57 patients with granuloma annulare, we identified 12 patients (nine girls) with a confirmed SGA diagnosis who underwent a preoperative MRI. Their median age was 3.25 years (range 2-5 years). Of 455 patients diagnosed with vascular malformations, 90 had malformations limited to the subcutaneous area. Among them only 47 patients with low-flow SVM were included in the study and further analyzed. Our SGA cohort had a female predilection (75%) and a short history of lump appearance of 1.5 months. SGA lesions were immobile and firm. Before MRI, patients underwent initial evaluation by ultrasound (100%) and X-ray (50%). Surgical tissue sampling was performed in all SGA patients to establish a diagnosis. All 47 patients with low-flow SVM were diagnosed correctly by MRI. A total of 45 patients (96%) underwent surgical resection of the SVM. A careful retrospective review of imaging findings of patients with SGA and SVM showed that SGA present as homogenous lesions in the shape of an epifascial cap with a typical broad fascial base extending towards the subdermal tissue in the middle of the lesion. In contrast, SVMs always present with variable-sized multicystic or tubular areas. Our study shows clear clinical and imaging differences between low-flow SVMs and SGA. SGA presents characteristically in the shape of a homogenous "epifascial cap," which distinguishes these lesions from multicystic heterogenous SVMs.

Evolving indications for pediatric neurointerventional radiology: A single institutional 25-year experience in infants less than one year of age and a brief historical review.

Pediatric neurointerventional radiology is an evolving subspecialty with growing indications and technological advancement such as miniaturization of devices and decreased radiation dose. The ability to perform these procedures is continuously balanced with necessity given the inherently higher risks of radiation and cerebrovascular injury in infants. The purpose of this study is to review our institution's neurointerventional experience in infants less than one year of age to elucidate trends in this patient population. We retrospectively identified 132 patients from a neurointerventional database spanning 25 years (1997-2022) who underwent 226 procedures. Treatment type, indication, and location as well as patient demographics were extracted from the medical record. Neurointerventional procedures were performed as early as day of life 0 in a patient with an arteriovenous shunting malformation. Average age of intervention in the first year of life is 5.9 months. Thirty-eight of 226 procedures were completed in neonates. Intra-arterial chemotherapy (IAC) for the treatment of retinoblastoma comprised 36% of neurointerventional procedures completed in infants less than one year of age followed by low flow vascular malformations (21.2%), vein of Galen malformations (11.5%), and dural arteriovenous fistulas (AVF) (9.3%). Less frequent indications include non-Galenic pial AVF (4.4%) and tumor embolization (3.0%). The total number of interventions has increased secondary to the onset of retinoblastoma treatment in 2010 at our institution. The introduction of IAC for the treatment of retinoblastoma in the last decade is the primary driver for the increased trend in neurointerventional procedures completed in infants from 1997 to 2022.

Responsiveness of the Patient-Reported Outcome Measure for Vascular Malformation Questionnaire in Patients with Low-Flow Vascular Malformations

PurposeTo assess the responsiveness, defined as the ability to detect change in a patient’s health or function, of the Patient-Reported Outcome Measure for Vascular Malformation (PROVAM) questionnaire in a cohort of patients with low-flow vascular malformations (VMs). Materials and MethodsPROVAM was previously developed to assess symptoms, functional limitations, and social/emotional effects experienced by patients with VMs. This is a prospective cohort study of 56 patients with venous and lymphatic VMs who completed at least 2 PROVAM questionnaires, of whom 43 had undergone treatment with sclerotherapy in the interim between questionnaires. External responsiveness was assessed using a receiver operating characteristic (ROC) curve to ascertain whether a change in the total PROVAM score predicts whether patients reported symptom improvement and by correlating the change in the total PROVAM score and change in symptoms reported during clinic visit. Internal responsiveness was evaluated using Wilcoxon signed rank test, Cohen d effect size (ESp), and standard response mean difference (SRM). ResultsThe total PROVAM score demonstrated excellent discrimination for symptom improvement with an area under the ROC curve of 0.856. There was a statistically significant, moderate positive correlation between the change in the total PROVAM score and the change in patient symptoms as determined from clinical visits (Spearman correlation coefficient [rs] = 0.67, P < .001). The total PROVAM score and all subdomain scores improved significantly after treatment (all P < .05). ESp and SRM were 0.80 and 0.83, respectively. ConclusionsPROVAM is responsive to improvement after treatment and may be useful to assess health-related quality of life in patients treated for VMs.

Head and Neck Low Flow Vascular Lesions Treated with Corseting Technique

Head and neck vascular lesions management has been a great challenge to the surgeons due to catastrophic bleeding that obscures the visibility during the surgery. A proposed surgical technique called CORSET SUTURING for the treatment of non cutaneous low-flow vascular malformations in the head and neck region minimizes the blood loss and facial disfigurement. Corset sutures are placed to strangulate the lesion and restore the facial symmetry with the least amount of comorbidities. The aim of the present study is to describe the clinical characteristics of patients treated by corset suturing technique, material used and surgical morbidity, in order to contribute to a better understanding of this technique. Medical records and images of 15 patients treated by the same corset suturing protocol were retrieved and analysed. The indications, advantages and disadvantages, technique and complications observed are discussed. There was a significant reduction in the bulkiness of tumour mass noted and also a return of the regional facial outline was evident. In conclusion, corset suturing has an important role in management of diffuse low-flow vascular malformations of the head and neck as it is found to be a simple, cost-effective, less scarring and acceptable method, hence can be considered as an alternative to the other expensive methods such as embolization, with least surgical comorbidities.

Approach to the Management of Vascular Malformations of Head and Neck and Its Challenges: An Institutional Experience

Abstract Background: Vascular malformations (VMs) in the region of the head and neck are a complex group of lesions that present with serious challenges in diagnosis and management. We sought to study the efficacy and safety of a therapeutic algorithm emphasizing multimodal management in the treatment of head and neck VMs. Methodology: A retrospective study was conducted reviewing all the patient information from the database who were treated for VM of the head and neck at the institution. Patients who presented during the period between January 2012 and December 2022 were included in the study. Conservative care, sclerotherapy, embolization, surgical excision, or a combination of these techniques were among the management options based on the clinical profile of the patient as well as the characteristics of the lesion. Results: 91 VMs in the region of the head and neck included 45 (49%) males and 46 (51%) females, with an age range of between 4 years and 71 years (mean 23.9 ± 14.06 years). There were 53 low-flow vascular malformations (LFVMs), of which 6 (11.9%) were managed conservatively, 26 (49.05%) were treated with sclerotherapy, 20 (37.7%) underwent excision, and 1 (1.8%) was treated with combination therapy. Of the 38 high-flow vascular malformations (HFVMs), 12 (31.5%) were treated with arterial embolization, 13 (34.2%) were primarily resected, 2 (5.2%) with excision and reconstruction, 5 (13.1%) underwent embolization and excision, and 1 (2.6%) underwent emergency external carotid artery ligation. In the LFVM group, response to sclerotherapy led to improvements in 24 (92%) patients, while surgical resection led to improvements in 20 (100%) individuals. In the HFVM group, embolization therapy improved 9 (75%), combination therapy improved 5 (100%), and surgical resection improved 15 (100%) of the patients. Conclusions: In our experience, the implementation of a multimodal management algorithm has had favorable outcomes with an acceptable complication rate in head and neck VMs.

Surgical management of low flow vascular malformations

Surgical management of low flow vascular malformations

Vascular Malformations in Paediatric Age Group: Learning through Mistakes

Vascular malformations were known in all paediatric age groups including neonates to adolescence. These were known since very long time. Many such cases were missed earlier due to lack of diagnostic imaging techniques. Proper diagnosis of such lesions is very challenging. So, proper identification and early treatment is of utmost importance. Any misdiagnosis can lead to inappropriate management and outcome. With recent advances in imaging techniques and better interventional facilities, the rate of diagnosis has improved but still the rate of misdiagnosis remains high. Though the newer treatment modalities are available, but their availability is restricted to tertiary care multifacility hospitals. Therefore, even after proper diagnosis, the management remains inadequate. This series is about three different patients of vascular malformations, belonging to different paediatric age groups. The clinical presentations, diagnosis and management were varied but diagnosis was possible due to high level of clinical suspicion and modern imaging techniques. The first patient initially seemed to have a post-traumatic swelling or juvenile idiopathic arthritis, but was later diagnosed as peripheral vascular malformation. The second patient was suspected to have haemangioma, but final diagnosis based on radiographical findings revealed low flow vascular malformation of face. The third patient was diagnosed with vascular malformation of head, but was clinically thought to be a sebaceous cyst.

Acute cardiopulmonary decompensation following ethanol sclerotherapy under general anesthesia

Ethanol sclerotherapy is a therapeutic method commonly applied to address low-flow vascular malformations. However, numerous complications, including hemodynamic instability, may develop after ethanol injection. We present a case who experienced cardiopulmonary instability during intraoperative percutaneous ethanol injection under general anesthesia and spontaneously recovered 5 min after ethanol injection. The toxic effects of the treatment were associated with the injection volume and may be related to ethanol-induced pulmonary vasoconstriction and transient right ventricular dysfunction. Prompt and proper management, including providing supportive treatment, decreasing pulmonary vascular resistance with pulmonary vasodilators, and improving right ventricular function, should be carefully prepared to minimize the progression of cardiovascular collapse.

The Usefulness of MRI Dynamic Flow Sequences in Differentiating High- and Low-Flow Vascular Malformations.

Peripheral arteriovenous malformations (PVMs) can be classified into high-flow malformations (HFVMs) and low-flow malformations (LFVMs). Adequate distinguishment is crucial for therapeutic decision and can be done using dynamic contrast-enhanced MRI (DCE-MRI). The main aim of this retrospective study was to determine the diagnostic value of quantitative DCE-MRI ratios for differentiation between HFVM and LFVM, their optimal cut-off points, and predictive values. DCE-MRI time-resolved angiography with stochastic trajectory (TWIST) examinations of 90 patients with PVMs were included [28 HFVM (31%), 62 LFVM (69%)]. The measurements of artery-lesion time, maximum lesion enhancement, slope of the enhancement curve, and maximum percentage increase of signal intensity (SI) were obtained. The optimal cut-offs for HFVMs calculated using the Youden index were: for slope of enhancement curve < 8.7 s (sensitivity of 86%, specificity of 89%), artery-lesion time ≤ 5.6 s (sensitivity of 89%, specificity of 77%), time to maximum enhancement ≤ 30 s (sensitivity of 94%, specificity of 100%), and maximum percentage enhancement of the lesion > 662% (sensitivity of 68%, specificity of 69%). To summarize, DCE-MRI is very valuable for differentiation between HFVM and LFVM, especially if quantitative measurements are done.

Intravascular Endothelial Hyperplasia of the Foot

Intravascular endothelial hyperplasia is a benign soft tissue mass rarely reported in the foot. Advanced imaging and confirming a benign diagnosis are critical for any soft tissue mass. This paper identifies 2 patients that developed intravascular endothelial hyperplasia tumors which required surgical excision. A 17-year-old male patient presented to clinic complaining of a painful bump to the arch of his right foot which he related to an injury 9 months prior. Magnetic resonance imaging of the right foot revealed a mass within the plantar subcutaneous fat that was serpiginous in nature similar to adjacent branching vessels favoring a low-flow vascular malformation. A 38-year-old female with Multiple Sclerosis presented with complaints of persistent symptoms of pain to the 1st interspace, difficult ambulation and neuritis. Ultrasound and MRI observed solid, multilobulated mass, with internal vascular malformation, MRI describing intrinsic involvement along the abductor musculature and flexor tendons. Both lesions were surgically excised and sent for pathology. Pathology report indicated a diagnosis of intravascular papillary endothelial hyperplasia or Masson's tumor in both cases. Pathology diagnosis of intravascular papillary endothelial hyperplasia is generally good with wide resection leading to low recurrence rates. Both patients in the current study have progressed postoperatively with resolution of symptoms and without recurrence.