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Objective: To evaluate the efficacy of sclerotherapy for congenital vascular malformation (CVM) using a combination of polidocanol foam and bleomycin liquid. Methods: A retrospective review of a prospectively collected data on patients who had sclerotherapy for CVM from May 2015 to July 2022 was performed. Results: A total of 210 patients with a mean age of 24.8 ± 2.0 years were included. Venous malformation (VM) was the most common type of CVM, accounting for 81.9% (172/210) of all patients. At 6 months follow-up, the overall clinical effective rate was 93.3% (196/210), and 50% (105/210) of patients were clinically cured. The clinical effective rates in VM, lymphatic, and arteriovenous malformation group were 94.2%, 100%, and 100%. Conclusion: Sclerotherapy using a combination of polidocanol foam and bleomycin liquid is an effective and safe treatment for venous and lymphatic malformations. It is a promising treatment option with satisfactory clinical outcome in arteriovenous malformations. [ABSTRACT FROM AUTHOR]

Klippel–Trenaunay syndrome is an uncommon, infrequent, congenital disorder characterized by a triad of capillary malformation, varicosities, and tissue and bone hypertrophy. The presence of two of these three signs is enough to obtain the diagnosis. Capillary malformations are usually present at birth, whereas venous varicosities and limb hypertrophy become more evident later. The syndrome has usually a benign course, but serious complications involving various organs, such as gastrointestinal and genitourinary organs, as well as the central nervous system, may be observed. Recently, Klippel–Trenaunay syndrome has been included in the group of PIK3CA-related overgrowth spectrum (PROS) disorders. In terms of this disorder, new results in etiopathogenesis and in modalities of treatment have been advanced. We report here a review of the recent genetic findings, the main clinical characteristics and related severe complications, differential diagnoses with a similar disorder, and the management of patients with this complex and uncommon syndrome. [ABSTRACT FROM AUTHOR]

The persistent left superior vena cava (PLSVC) is a common venous abnormality. However, malformation of the bilateral inferior venae cava (IVC) is extremely rare, with an incidence rate of.3%. IVC malformation is associated most frequently with heart defects and isomerism and often has a poor prognosis. We presented a case of vascular malformations in the fetus of bilateral caval veins with the interruption of the left‐sided venous return with hemiazygos continuation in presence of a right‐sided inferior caval vein. Also noted were the PLSVC and a dilated right heart with a widened pulmonary trunk. In this case, there were no heart defects or chromosomal abnormalities, and the newborn postpartum was in a good condition. [ABSTRACT FROM AUTHOR]

Background: The occurrence of Isolated left subclavian artery (ILSA) is relatively rare, ILSA is caused by the persistence of the dorsal segment of the sixth left arch, with regression of the fourth arch artery and interruption of the left dorsal aorta at the distal end of the seventh intersegmental artery on the left side during embryonic development. The left subclavian artery is connected to the pulmonary artery through an arterial duct, which can be closed or unobstructed. This abnormality can lead to congenital subclavian steal syndrome and vertebrobasilar artery insufficiency. Case presentation: We reported three fetuses with ILSA and intracardiac malformation. Among them, one case was suspected to be diagnosed with ILSA by echocardiography, while the other two cases were not diagnosed, but were accidentally discovered during autopsy. We have also conducted a literature review of its prenatal screening, diagnosis, management, and outcomes. Our three cases were tested by WES‐Trio (whole exome sequencing). Worldwide, the ILSA cases reported in English literature have not been detected by WES. And likely pathogenic results were found in our two cases. Although it could not explain the intracardiac malformation we found, it will help to explore the etiology in the future. Conclusions: Prenatal echocardiography detection and diagnosis of ILSA is a new challenge, which has different effects on the prognosis of the fetus. When finding intracardiac malformation with right aortic arch, we need to perform an unconventional view of ultrasound scanning and combine with CDFI to find the origin of the left subclavian artery. Although we cannot find the cause of the disease temporarily, but our genetic results can help prenatal genetic counseling. [ABSTRACT FROM AUTHOR]

We aimed to examine the impact of maternal hypothyroidism on placental pathology and perinatal outcomes in singleton live births resulting from IVF, using medical records of IVF births between 2009 and 2017 at a tertiary hospital. The primary outcomes included anatomical, inflammation, vascular malperfusion, and villous maturation placental features. Secondary outcomes included foetal, maternal, perinatal, and delivery complications. There were 1,057 live births, of which 103 (9.7%) and 954 (90.3%) were in the study and control groups, respectively. Patients in the study group were more likely to have diabetes mellitus, polycystic ovarian syndrome, gestational diabetes mellitus, and non-reassuring foetal heart rate (NRFHR) tracing during delivery. After adjustment for potential confounding factors, hypothyroidism was significantly associated with the bilobed placenta (aOR 4.1; 95% CI 1.2–14.3), retroplacental haematoma (aOR 2.4; 95% CI 1.2–4.9), decidual arteriopathy (aOR 2.0; 95% CI 1.2–4.1) and subchorionic thrombi (aOR 2.4; 95% CI 1.3–5.0). Additionally, there was a statistically significant relationship with NRFHR tracing. The incidence of acute chorioamnionitis and severe foetal inflammatory response was higher in the study group. In conclusion, the placental histopathology patterns of singleton IVF live births show that maternal hypothyroidism has a significant impact on adverse perinatal outcomes. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Although the ductal anomalous origin of the pulmonary artery (DOPA) constitutes a rare heart anomaly, this malformation has a high mortality rate due to the rapid development of pulmonary hypertension(PTH) and right heart failure. Case Presentation: We report a case of DOPA, in which ductus arteriosus originated from the left pulmonary artery. This article summarizes the embryogenesis, clinical manifestations, complications and prognosis, diagnosis and experience, and treatment strategies of DOPA. The most fundamental sonographic finding was the lack of confluence at the bifurcation of the main pulmonary artery. Scanning upper mediastinum views is essential for the diagnosis. In addition, three‐dimensional echocardiography with high‐definition flow imaging and spatio‐temporal image correlation technique facilitates the identification of the anomalous origin of the pulmonary artery. It should be considered a complementary modality in fetal cardiac examinations. Although rare, DOPA can be diagnosed prenatally, usually at the three‐vessel view (3VV). The early diagnosis of DOPA thus can prevent the potentially devastating effects of PHT and right heart failure. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to investigate the feasibility and effectiveness of 3D printing personalized guide plate in the management of recurrent intramuscular venous malformations (IVM). Methods: Fifteen patients with recurrent IVM were retrospectively assessed. 3D-slicer software was used to extract and reconstruct the imaging data from CT and/or MRI to highlight the morphology, size, and puncture depth of the lesion. With the guidance of personalized plate, complete excision of the IVM was adopted along the pre-marked (methylene blue, MB) margin. Results: Personalized guide plate matched involved extremity well, and MB-puncture approach was consistent with preoperative design. All IVMs were removed radically in one single session. Complete pain relief was obtained in all cases postoperatively. Conclusion: The application of 3D printing guide plate can be safe, effective, and reliable to confirming the precise margin of IVM, renders a promising technique with a high practical value in resection of recurrent lesion. [ABSTRACT FROM AUTHOR]

Biomedical applications of electroporation are expanding out of the field of oncology into vaccination, treatment of arrhythmias and now in the treatment of vascular malformations. Bleomycin is a widely used sclerosing agent in the treatment of various vascular malformations. The application of electric pulses in addition to bleomycin enhances the effectiveness of the drug, as demonstrated by electrochemotherapy, which utilizes bleomycin in the treatment of tumors. The same principle is used in bleomycin electrosclerotherapy (BEST). The approach seems to be effective in the treatment of low-flow (venous and lymphatic) and, potentially, even high-flow (arteriovenous) malformations. Although there are only a few published reports to date, the surgical community is interested, and an increasing number of centers are applying BEST in the treatment of vascular malformations. Within the International Network for Sharing Practices on Electrochemotherapy (InspECT) consortium, a dedicated working group has been constituted to develop standard operating procedures for BEST and foster clinical trials. By treatment standardization and successful completion of clinical trials demonstrating the effectiveness and safety of the approach, higher quality data and better clinical outcomes may be achieved. [ABSTRACT FROM AUTHOR]

Introduction: Anomalous cardiac veins are not rare and pulmonary vein (PV) isolation for atrial fibrillation (AF) treatment should include these veins. Pulsed‐field ablation (PFA) is a novel technology for AF ablation with excellent efficacy and safety profile. In this case series, we describe our first experience of isolation of anomalous cardiac veins using PFA in patients with AF. Methods: We report a series of patients with congenital anomalies of the cardiac veins and AF, treated with PFA. All patients underwent cardiac computed tomography for procedural planning. Results: We included five patients (four males). Anomalous cardiac veins included a connection of a left common ostium to the coronary sinus, a partial and complete drainage of the right superior PV into the superior vena cava (SVC) with and without additional atrial septal defect, a persistent left SVC and an anomalous posterior PV. All anomalous PVs were isolated using PFA. No phrenic nerve palsy or other complications occurred. PFA of an abnormal right superior PV draining into the distal SVC was possible without affecting the sinus node. After a median of 4 months, four patients were free of recurrence. One patient had recurrent AF and perimitral reentry tachycardia, probably facilitated by PFA in the mitral isthmus region during isolation of an anomalous connection of the left common ostium to the coronary sinus. Conclusions: Using systematic preprocedural imaging and three‐dimensional‐electroanatomic mapping, the currently available PFA system seems well suited, efficient, and versatile for the treatment of AF in patients with anomalous cardiac veins. [ABSTRACT FROM AUTHOR]

Context: Congenital vascular anomalies are classically subdivided into vascular tumors and vascular malformations. The role of propranolol in the regression of infantile hemangioma (IH), a vascular tumor, is well established. Aims: This study aimed to analyze the therapeutic efficacy and complications associated with oral propranolol and adjuvant therapy in the treatment of vascular anomalies. Settings and Design: A prospective interventional study was undertaken over 10 years duration extending from 2012 to 2022 at a tertiary care teaching institute. Materials and Methods: All children with cutaneous hemangiomas and lymphatic and venous malformations under 12 years of age, except those with contraindications for administration of propranolol, were included in the study. Results: Out of 382 patients, there were 159 males and 223 females (male: female = 1:1.4). The majority (53.66%) were between ≥3 months and 1 year. There were 481 lesions in 382 patients. There were 348 patients with IH, and 11 were congenital hemangiomas (CHs). There were 23 patients with vascular malformations; lymphatic malformation (n = 19) and venous malformation (n = 4) were present. The size of the lesions ranged from 5 mm to 20 cm; 50.73% were 2-5 cm in size. Ulceration (>5 mm) was the most common complication present in 20/382 (5.24%) patients. Complications related to oral propranolol were seen in 23 (6.02%) patients. Drugs were given for a mean period of 10 months (range from 5 months to 2 years). At the end of the study, 282 (81.03%) out of 348 patients with IH showed an excellent response; 4 (36.36%) patients in the case of CH (n = 11) and 5 (21.74%) patients with vascular malformation (n = 23) showed excellent response. Conclusion: The study validates the use of propranolol hydrochloride as the first-line agent for the treatment of IHs and congenital hemangiomas. It may have an additive role in lymphatic malformations, and venous malformations, as a part of a multimodality treatment approach for vascular malformations. [ABSTRACT FROM AUTHOR]

Objective: To retrospectively correlate imaging findings post-sclerotherapy of low-flow vascular malformations with clinical outcome. Materials and Methods: We retrospectively evaluated 81 pediatric patients who had sclerotherapy in our department over a 14-year period. Patients with a diagnosis of low-flow vascular malformation, pre and post-treatment ultrasound (US) and clinical follow-up evaluation were included in the study. Exclusion criteria were coexisting high-flow vascular malformations, history of additional surgical or medical treatment to their malformation and large infiltrative lesions difficult to measure on US. Pre and post-treatment sonographic volumes of the malformation were assessed. Changes in volume were categorized into 6- increased volume, stable and volume decrease of 1-25%/26-50%/51-75%/75-100%. Clinical outcomes were categorized into 4 – worse, no change, improved and symptom free. In cases where pre-treatment MRI was available, the estimated malformation volumes in both modalities were correlated using Spearman's rank correlation. The change in sonographic volume was correlated with clinical outcome using Spearman's rank correlation. P -values <.05 were considered significant. Results: Twenty-nine patients were included in the study; 13 with venous malformation (VM), and 16 with lymphatic malformation (LM). Nineteen patients had both pre-treatment US and MRI, showing correlation in volume between the 2 modalities (P <.001). Post-treatment change in volume correlated with clinical outcome for combined venous and LMs (rho =.44, P =.02). No correlation was found when venous (rho =.48, P =.09) and lymphatic (rho =.33, P =.21) malformations were considered separately. Conclusion: Ultrasound can potentially be used as an objective tool in evaluating sclerotherapy treatment response of low-flow vascular malformations in the pediatric population. [ABSTRACT FROM AUTHOR]

Purpose: Here, we report our experience treating a patient with Maffucci syndrome and evaluate the outcomes resulting from surgical management combined with sclerotherapy in the treatment of head and neck venous malformations (VMs). A 19-years-old woman with multiple enchondromas and heterauxesis complained of masses in the oral cavity that had gradually increased in size and eventually affected her daily life. A tracheotomy was performed followed by traditional sclerotherapy to treat the oropharyngeal VMs. Next, we surgically excised the VMs of the oral cavity and maxillofacial skin. Results: Magnetic resonance imaging indicated that the oral VMs were nearly eradicated and the oropharyngeal VMs had stabilized. The patient's appearance and normal maxillofacial region function were restored. Conclusion: In summary, local resection combined with sclerotherapy facilitated timely and efficient VMs removal from the head and neck region of a patient with Maffucci syndrome. [ABSTRACT FROM AUTHOR]

Objective. The use of sirolimus in vascular anomalies is a special indication not authorized in its data sheet. The objective of this study was to increase the evidence of oral or topical use of sirolimus for this indication in the pediatric population. Materials and methods. An observational, retrospective study of patients under 18 years of age treated with oral or topical sirolimus for vascular anomalies was carried out. Diagnosis and location of lesions, administration route and dosage of sirolimus, blood levels of sirolimus in patients who received oral treatment, treatment duration, response, and toxicity were collected. Results. 18 patients - 7 with oral treatment and 11 with topical treatment - were included. With oral sirolimus, the overall response rate was 85.7%. Sirolimus was discontinued in 2 cases - as a result of full resolution and progression. 57.1% of patients had adverse effects, most of which were mild. Dyslipidemia was the most frequent adverse effect. Blood levels were monitored in all patients for dose adjustment purposes. With topical treatment, the overall response rate was 72.7%. Sirolimus was discontinued in 3 cases -due to progression in 2 cases and to stability in 1. 27.3% of patients had adverse effects, with itching standing out as the most frequent one. Conclusions. The favorable results of sirolimus treatment in our patients seem to confirm its effectiveness and safety in vascular anomalies, which make it stand as a therapeutic option in pediatric patients. However, further research is required to establish the optimal treatment regimen, treatment duration, and potential long-term adverse effects. [ABSTRACT FROM AUTHOR]

Pediatric interventional neuroradiology (PINR) is a relatively new field of diagnostic and therapeutic care in the pediatric population that has seen considerable advances in recent decades. However, it is still lagging behind adult interventional neuroradiology due to a variety of reasons, including the lack of evidence validating pediatric-specific procedures, the relative absence of pediatric-specific equipment, and the challenges in establishing and maintaining PINR competencies in a relatively small number of cases. Despite these challenges, the number and variety of PINR procedures are expanding for a variety of indications, including unique pediatric conditions, and are associated with reduced morbidity and psychological stigma. Continued technological advances, such as improved catheter and microwire designs and novel embolic agents, are also contributing to the growth of the field. This review aims to increase awareness of PINR and provide an overview of the current evidence base for minimally invasive neurological interventions in children. Important considerations, such as sedation, contrast agent use, and radiation protection, will also be discussed, taking into account the distinct characteristics of the pediatric population. The review highlights the usefulness and benefits of PINR and emphasizes the need for ongoing research and development to further advance this field. [ABSTRACT FROM AUTHOR]

Background: This study describes the management protocol for capillary-venous malformations in pediatric patients and reports the epidemiology of diagnosed and treated cases at the Unit of Odontostomatology of the Aldo Moro University of Bari from 2014 to 2022. Methods: The authors classified the intraoral and perioral capillary-venous malformations by superficial diameter (<1 cm, 1–3 cm, >3 cm) and ultrasonographical depth extension (≤5 mm, >5 mm). All patients underwent pulsed-mode diode laser transmucosal photocoagulation (8–12 W/cm2); those with malformations that were wide (>3 cm) and deep (>5 mm) received intralesional photocoagulation, too (13 W/cm2). The children received general anesthesia based on their compliance and lesions' extension. The follow-up lasted six months. Results: A total of 22 females and 14 males (age range 4–18 years) presented 63 capillary-venous malformations. Five patients with Sturge–Weber syndrome, seven with hereditary hemorrhagic telangiectasia, and five with angiomatosis showed multiple malformations. The authors found no intraoperative or postoperative complications. Seventeen patients with lesions >1 cm and >5 mm deep required multiple laser sessions to heal. Conclusion: The results of the current study support diode laser photocoagulation as the gold standard for the treatment of intraoral and perioral capillary-venous malformations in pediatric patients. [ABSTRACT FROM AUTHOR]

Objective: The study aimed to evaluate the early follow-up quality of life (QoL), pain and mental health of patients with congenital vascular malformation (CVM) from a variety of treatment options. Methods: All patients with CVM who received care and had follow-up between February 1st 2018 and January 31st 2020 were included. The health-related QoL, pain, and mental health were assessed with RAND Health Care 36-Item Short Form Survey (SF-36), visual analogue score for pain (VAS-P) and Hospital Anxiety and Depression Scale (HADS). Paired t-test was used for all analyses. p <.05 were considered significant. Results: In total, 110 patients with a mean age of 36.9 years were included in this study. In all patients following care, significant improvement was found in the bodily pain domain of SF-36 and VAS-P (both p =.01). This was largely driven by high-flow vascular malformation patients who responded better to embolo-sclerotherapy, which revealed significant improvement in the bodily pain domain of SF-36 (p =.002) and VAS-P (p =.02). Patients who received supportive treatment only reported significant improvement in mental health (p =.004) and social functioning (p =.03) domains of SF-36. Meanwhile, patients treated with embolo-sclerotherapy reported significant improvement only in VAS-P (p =.02). Conclusions: This study concluded that the effects of care on early follow-up QoL, pain and mental health of patients with CVM were heterogenous. Future research should therefore, include larger sample size and longer term follow-up to understand the various factors that affect the QoL and mental health of these patients, as well as the holistic approaches to manage them. [ABSTRACT FROM AUTHOR]

Axillary lymphangiomas are congenital vascular malformations seen rarely in the adult age spectrum. We report a case where a rapidly growing, large, axillary lymphangioma in a 45-year-old man was managed successfully with surgical resection. While there is an expanding role of sclerotherapeutic techniques in the management of lymphangiomas, we feel that complete surgical resection in eligible cases offers the best outcome for the patient. [ABSTRACT FROM AUTHOR]

The article focuses on describing the case of a 21-year-old male with linear verrucous venous malformation (VVM) in his left lower extremity, emphasizing its clinical, dermoscopic, and pathological features. Topics include the clinical presentation and progression of VVM, differential diagnoses, and treatment options like deep excision and laser therapy for managing this rare congenital vascular anomaly.

Objective: To analyse the image characteristics of fibro-adipose vascular anomaly (FAVA) and the value of ultrasound and magnetic resonance imaging (MRI) for its diagnosis. Methods: The clinical and imaging data characteristics of 10 patients with FAVA admitted to our hospital between January 2018 and December 2020 who underwent ultrasound and MRI diagnosis were retrospectively analysed. Results: A total of 10 patients (six males and four females) with pathologically confirmed FAVA, aged from 3 to 27 years (median: 13 years), underwent ultrasound and MRI; of these, two patients underwent MRI-enhanced examinations. All lesions involved mainly muscle, with a few disruptions and involvements of subcutaneous fat. Five cases were located subcutaneously near the fascia; one case was adjacent to the periosteum. Ultrasound showed fascial tail in seven cases, and MRI showed fascial tail in six cases. Both ultrasound and MRI showed the lesions to be oval-shaped masses with blurred borders and a mean maximum diameter of 99.8 ± 48.7 mm. Ultrasound showed a mass with a mixture of high and low echoic areas. Magnetic resonance imaging imaging showed a heterogeneous, mixed-signal intensity on T1-weighted images, probably influenced by the fibrous and fatty components of the lesion. T2-weighted images showed a mixed-high signal. Enhanced computed tomography scans showed significant heterogeneous enhancement of the lesions. Conclusion: Both ultrasound and MRI showed that fascial tail sign is an important imaging feature for FAVA disease; this provides a reliable basis for its diagnosis and can be used to distinguish it from venous malformations, which have no fascial tail signs. Therefore, fascial tail signs can be used as imaging features and require special attention in the diagnosis of FAVA disease. [ABSTRACT FROM AUTHOR]

Introduction: Persistent left superior vena cava (PLSVC) is accompanied by enlarged coronary sinus (CS) and deformation of the triangle of Koch. This makes anatomical evaluation of the atrioventricular (AV) nodal pathways difficult. Methods: We attempted cryoablation of retrograde fast pathway located in the enlarged CS roof of PLSVC for slow‐fast AV nodal reentrant tachycardia (AVNRT) induced by inadvertent antegrade fast pathway elimination during ablation of left atrial tachycardia. Results: Slow‐fast AVNRT was successfully eliminated without AV block progression. Conclusions: This is the first case of successful retrograde fast pathway ablation of the CS ostial roof for slow‐fast AVNRT with PLSVC. [ABSTRACT FROM AUTHOR]

Vascular anomalies grouped into vascular tumors (hemangioma) (HI) and vascular malformation (VM) are benign vascular lesions that are difficult to distinguish from one another clinically and often confused with each other at histopathology. This confusing terminology leads to improper diagnosis, illogical treatment, and misdirected research. This study aimed to study GLUT 1, S-100, and nerve bundle to differentiate hemangioma and vascular malformation. Thirty two cases of vascular lesions (26 vascular malformations and 6 hemangiomas) were taken into the study. For histological evaluation and immunohistochemistry (IHC), samples of vascular lesions were collected in formalin. All the hematoxylin and eosin-stained slides were evaluated under light microscope for histology and nerve bundles. Immunohistochemical staining was performed by streptavidin–biotin method for GLUT 1 and S-100. GLUT 1 was positive in all 6 cases of hemangiomas (100%) and only in 1 case of vascular malformation. Nerve bundle was present in 24 cases of vascular malformation (92.3%) out of 26 cases but not in any cases of hemangioma and S-100 was found positive in all vascular malformation cases (100%) but not in hemangioma. So GLUT 1 expression, S-100, and presence of nerve bundle in vascular lesions can help to differentiate hemangioma and vascular malformation. [ABSTRACT FROM AUTHOR]

Purpose: This study evaluated the results of polidocanol sclerotherapy in the treatment of venous malformations (VM) including patient satisfaction, perceived improvement, and predictors of satisfaction. Material and Method: Patients with VM that underwent polidocanol foam sclerotherapy between June 2013 and July 2021 in a single center were retrospectively evaluated. Patient demographics, VM, and treatment characteristics were analyzed. Patient-reported outcomes and satisfaction were analyzed with a questionnaire. Results: This study included 232 (136, 58.6%, female) patients. The mean age was 24.49 ± 12.45 years (range 3–72). The clinical response rate was 82.3%. The rate of satisfaction was 82.3%, and 116 (50%) patients were significantly satisfied. There were no major complications. Clinical response and VM margin were related to satisfaction (p < 0.01, p = 0.012, respectively). Clinical response to pretreatment swelling was related to significant satisfaction (p = 0.02). Conclusion: Polidocanol sclerotherapy was safe and effective in VM treatment with high satisfaction and low complication rates. [ABSTRACT FROM AUTHOR]

Objective: Pulsatile tinnitus can be a symptom of vascular pathology. However, many of these pathologies present as incidental findings on scanning for unrelated symptoms. This study investigated whether the pathologies attributed to pulsatile tinnitus could instead be considered incidental findings. Methods: This retrospective study evaluated imaging results of 272 pulsatile tinnitus cases for clinically relevant pathologies, and examined correlations between the site of symptoms and the imaging findings. Results: Of 272 patients, 238 (88 per cent) had normal scans, 17 (6 per cent) had clinically insignificant incidental findings, and 18 (7 per cent) had findings requiring further investigation or intervention; regarding these latter 18 patients, findings for 8 patients (42 per cent) did not correlate with the symptomatic side. The rates of intracranial aneurysm and arteriovenous malformation in the pulsatile tinnitus group were comparable to those in normal populations. Conclusion: The comparable rates of vascular abnormalities within the symptomatic pulsatile tinnitus group, plus clinically relevant findings contralateral to symptoms, suggest that vascular pathologies could be incidental findings rather than causes of pulsatile tinnitus. Evaluation is recommended of the effectiveness of the new National Institute for Health and Care Excellence guidelines for pulsatile tinnitus investigation. [ABSTRACT FROM AUTHOR]

Objectives. 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. Methods. 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. Results. 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. Conclusions. 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. [ABSTRACT FROM AUTHOR]

Abernethy malformation, also known as congenital extrahepatic shunt, is a rare anomaly, which is characterized by partial or complete diversion of the portal blood into the systemic venous circulation. The clinical manifestations of Abernethy malformation during childhood include neonatal cholestasis, failure to thrive, mental retardation, and other congenital defects. We report a case of Abernethy malformation Type II in a 9‐year‐old boy, whose left ventricle was slightly enlarged because of several major aortopulmonary collateral arteries (MAPCAs) but laboratory examinations were normal 5 years earlier. The characteristics of congenital heart disease in patients with Abernethy malformation are discussed. We propose that physicians should be aware of the possibility of Abernethy malformation in children with enlargement of the left ventricular due to systemic‐pulmonary collateral circulation. [ABSTRACT FROM AUTHOR]

The hemodynamics of anomalous origin of the pulmonary artery (PA) from the aorta is challenging. Different sources of blood supply to the lungs lead to a unique state of differential flow, pressure, and pulmonary vascular resistance in each lung. The decision for surgical reimplantation of the anomalous PA during infancy is easy. The assessment of operability beyond infancy, however, is perplexing. In this report, we describe stepwise multimodal hemodynamic evaluation and successful surgical management in a 15-year-old boy with an isolated anomalous origin of the right PA from the aorta. We also report 5-year hemodynamic data confirming sustained benefit over the long term, thus providing much-needed clinical validation of often cited Poiseuille's and Ohm's laws. [ABSTRACT FROM AUTHOR]

Introduction: Venous varices are vascular lesions caused by vasodilatation. These oral cavity lesions are usually seen as papules or dark blue nodules on the tongue's ventral surface buccal and retro-commissural mucosae. They are generally asymptomatic but may cause problems such as bleeding on trauma or lack of esthetics. Although many treatment modalities exist for these lesions, the laser is a novel and less invasive method. It secures patient satisfaction more than other methods due to the lack of bleeding and pain. Unlike previous laser therapy methods used for cutting and excision of small vascular lesions, in this lesson, the coagulation properties of laser are employed to eliminate the lesion. Case Presentation: To illustrate, a 70-year-old female person was admitted to the dental clinic of the Semnan University of Medical Sciences, Iran, for a dental examination. She has no systemic problems. During the extra-oral examination, the lesion was visualized on the right side of her lower lip, and the initial diagnosis of a venous lake was made. The lesion was asymptomatic and had been present for several years. Likewise, after obtaining informed consent, treatment with a 940-diode laser was performed. Immediately after the irradiation, the lesion disappeared, and after four weeks, the lip's texture was completely normal. Conclusions: Exploiting the coagulation properties of the laser to eliminate venous lakes is advantageous over other methods because of the lack of bleeding or pain. Furthermore, this method has a shorter healing time. [ABSTRACT FROM AUTHOR]

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Thoracic vascular malformations are a set of complex congenital abnormalities that result in major alterations of the patient's anatomy. It can be discovered incidentally or the anomalies may generate heart failure with a right-to-left shunt, dysphagia, dyspnea, stridor, cough, recurrent bronchopulmonary infections, hypoxemia, paradoxical emboli, and occasionally apneas and massive hemothorax. The knowledge of the embryology, and the normal development of the vascular structures of the thorax, allows to understand these abnormalities—and thus helps in making an accurate diagnosis—with its possible complications, symptoms, and treatments for the patient. The following is a review of the most common thoracic vascular malformations and their imaging findings. [ABSTRACT FROM AUTHOR]

Congenital hepatopulmonary fusion (HPF) is a rare anomaly characterized by a fusion between the liver and lung parenchyma. HPF cases have been scarcely reported in the literature. An extensive search of publications was performed in the PubMed and Google Scholar databases without a time limit. In total, 34 clinical case reports were found in the literature, and a study by the Congenital Diaphragmatic Hernia (CDH) Study Group reported data on 10 patients with HPF. Of these 44 infants, 20 were male, 20 were female, and four were reported without gender specification. Nineteen (43.2%) patients required intubation on the first day of life. Six (13.6%) patients had their clinical presentation during the first year of life, and four (9%) clinically presented with HPF between 2.5 and 11 years of age. In these patients, cough, asthma-like symptoms, dyspnea, hemoptysis, right-side chest pain, respiratory infections, and pneumonia were the relevant clinical signs. Right-lung vascular anomalies were present in 18 (40.9%) patients. A complete liver and lung separation was successful in 17 (38.6%) patients. The overall survival was 56.8%. Congenital HPF has no gender predominance. In most cases, it behaves similar to a right CDH in need of resuscitation and intubation after birth. The majority of the cases are discovered during the surgical procedure for CDH. The best surgical approach has not been established and depends on the degree of fusion and vascular anomalies. An advanced imaging assessment is necessary before a surgical approach is attempted. The prognosis is ominous. [ABSTRACT FROM AUTHOR]

Objective: To report the clinical effectiveness and the safety of cryoablation in first or second-line therapy in symptomatic soft tissues vascular malformation, a mini-invasive therapeutic alternative to sclerotherapy or surgery.Materials and Methods: This retrospective and monocentric study included patients with symptomatic low-flow vascular malformation. The interventions were carried out under computed tomography (CT) scan, Cone-beam CT (CBCT) and/or USA guidance. Clinical response was evaluated by collecting the Numerical Rating Scale (NRS) for symptoms before and after the ablation. Safety was assessed based on criteria proposed by the Cardiovascular and Interventional Radiological Society of Europe. Imaging response was evaluated with post-ablation Magnetic Resonance Imaging.Results: Twenty-one patients were included. Cryoablation was the first operative treatment for 12 patients (12/21, 57%). The remaining patients had already undergone surgery (2/21, 9%) or one or more sclerotherapy procedures (7/21, 33%).Symptoms assessed by Numerical Rating Scale dropped from a median of 7 [IQR 6-8] before the procedure to a median of 1 [IQR 0-2] after cryoablation (p < 0.001). Half of the patients declared a full effectiveness of cryoablation on their symptoms (11/21). No major complications and four minor adverse events (two skin lesions, two patients with neuropathic pain) were reported (19%). Lesional volume significantly decreased after cryoablation (median from 3.7 cm3 [1-10.4] to 0.25 cm3 [0-2.0], p < 0.001).Conclusion: Cryoablation seems to be a safe and effective first- or second-line therapy for soft tissue vascular malformations. [ABSTRACT FROM AUTHOR]

Abernethy malformation is a very rare congenital vascular malformation with complete or partial shunting of portal flow to the systemic circulation which manifests with symptoms of recurrent respiratory tract infections and failure to thrive. We present here a case of a 12-year-old boy diagnosed as a case of Abernethy malformation. [ABSTRACT FROM AUTHOR]

The article informs about selected phlebological research topics, including studies on combined foam sclerotherapy and endovenous laser ablation for recurrent varicose veins, epigenetic changes in venous cells under shear stress, comparison of phlebectomy techniques, genetic factors in pulmonary thromboembolism susceptibility, sirolimus treatment for pediatric vascular anomalies, fluoroscopy-guided foam sclerotherapy, and the effects of polidocanol microfoam on venous leg ulcer healing.

Objectives: Congenital vascular lesions commonly present in the head and neck, and most are managed conservatively. Location and rapid growth, however, may necessitate surgical intervention. Endoscopic endonasal surgery (EES) in the pediatric population has emerged as a viable option in treating sinonasal and skull base lesions. Utilizing these techniques in newborns carries unique challenges. The objective of this report is to describe the successful use of direct intralesional embolization followed by endoscopic endonasal resection of a venous malformation in a postnatal patient. Methods: We reviewed the case reported and reviewed the pertinent literature. Results: A 6-week-old infant was found to have a large right-sided sinonasal lesion confirmed as a venous malformation. Rapid growth, impending orbital compromise, and potential long-term craniofacial abnormalities demanded the need for urgent surgical intervention. Risk of bleeding was mitigated with direct intralesional embolization. Immediately afterward, the patient underwent endoscopic endonasal resection of the lesion. EES in the very young presents multiple challenges both anatomically and behaviorally. A multidisciplinary approach lead to a successful outcome. Conclusion: We report a case of a 6-week-old infant, the youngest reported patient to the authors' knowledge, who successfully underwent direct intralesional embolization followed by endoscopic endonasal resection of a sinonasal vascular malformation. This report highlights the challenges of this technique in the very young and demonstrates it as a viable treatment strategy for sinonasal vascular anomalies in this population. [ABSTRACT FROM AUTHOR]

With the advent of computed tomographic interrogation, it is increasingly frequent to find venous channels that provide direct connections between the pulmonary and systemic veins. These channels, before the introduction of three-dimensional techniques for clinical imaging, were usually found providing an "overflow" for the obstructed left atrium in settings such as hypoplastic left heart syndrome, or divided left atrium. Similar channels, however, had been described almost 100 years ago, with one accurately described as a jugulo-pulmonary vein. Nowadays, however, it is much more usual to find the channels described as levoatrial cardinal veins, even though it is recognized that they are not "levo," often not "atrial," and for sure not "cardinal." In this review, we assemble the evidence supporting the notion that they are better considered as pulmonary-to-systemic collateral channels. We emphasize their similarity, in terms of development, to the sinus venosus and coronary sinus defects. [ABSTRACT FROM AUTHOR]

This single-center, single-arm, prospective open-label trial is being conducted to evaluate the short-term efficacy and safety of percutaneous sclerotherapy with polidocanol foam for painful venous malformations. This study will include patients who were clinically diagnosed with venous malformation by using ultrasound and/or magnetic resonance imaging, and whose pain persisted even after treatment with medications. Written informed consent for sclerotherapy will be obtained from all patients. The institutional review board approved this prospective study protocol. The primary endpoint is pain relief at three months after sclerotherapy. Local pain related to venous malformation will be evaluated using a numeric rating scale. Patient recruitment commenced in December of 2020. Enrolment of 13 patients is planned over a 3-year recruitment period. Herein, we describe the details of the clinical trial protocol. [ABSTRACT FROM AUTHOR]

Introduction: Syndromes associated with vascular malformation and soft tissue overgrowth in the paediatric population present with multiple soft tissue swellings. Ultrasound is the initial investigation of choice for paediatric soft tissue swellings. Ultrasound evaluation can accurately assess the nature of vascular malformations and pattern of lipomatous hypertrophy in areas of soft tissue overgrowth to facilitate early diagnosis of such syndromes. Case Report: Here, we report a case of CLOVES (congenital lipomatous overgrowth (CLO), vascular malformations (V), epidermal nevi (E), and spinal/skeletal anomalies/scoliosis (S)) syndrome in a 6-year-old girl referred for evaluation of soft tissue swellings. CLOVES syndrome is a rare overgrowth syndrome in the paediatric population which presents with multiple soft tissue swellings. The ultrasound and clinical features of the syndrome have been illustrated to help radiologists accurately diagnose this rare syndrome based on detailed ultrasound and clinical evaluation. Discussion: Radiological features of CLOVES syndrome and differentiating ultrasound features of other such syndromes have been described in detail. A systematic stepwise approach to diagnosing complex syndromic associations of vascular malformations with lipomatous overgrowth has been proposed. Role of ultrasound in the management, Wilms tumour screening and follow-up of CLOVES syndrome have also been discussed. Conclusion: Ultrasound plays a crucial role in the early diagnosis and management of complex syndromes presenting with soft tissue swelling in the paediatric population. It also aids in the differentiation of such syndromes, tumour screening, guided sclerotherapy and follow-up of vascular lesions encountered in such syndromes. [ABSTRACT FROM AUTHOR]

Even though vascular anomalies of the hand are rare entities (7%), they are relevant regarding soft tissue mass differential diagnosis on the hand. 2 The majority of cases tend to be malformations, once denominated as deep soft tissue hemangiomas. 1,3 Due to evolving knowledge regarding vascular anomalies, the prognosis, guidelines regarding treatment and aftercare following therapy/surgery will be more accurate and detailed if the correct diagnosis is established. Retrospective data collection and analysis between 2008 and 2019, from the Orthopedic Department of Semmelweis University tumor registry. Comparison to the current literature (PubMed, Ovid). Study level of evidence III. Standardly distributed data with confidence level of 95%. N = 16, average age of 34,2. 63,5% of lesions were digital. Hemangiomas accounted for 67%. The accuracy of clinical vs. histological diagnosis was 77%. Recurrences 25%. Variable follow up period. Clinical examination, radiological evaluation, patient history and fine needle biopsy are often accurate guides. Nevertheless in our study, histological analysis of the surgical biopsy was the most effective method in establishing a definite diagnosis. Conservative and watchful waiting approach are the first line, and when complemented with appropriate imaging, should suffice until absolute or relative operative indications are present. [ABSTRACT FROM AUTHOR]

Venous malformations, the most common type of vascular malformation, are slow-flow lesions resulting from disorganized angiogenesis. The International Society for the Study of Vascular Anomalies (ISSVA) classification offers a categorization scheme for venous malformations based on their genetic landscapes and association with congenital overgrowth syndromes. Venous malformations present as congenital lesions and can have broad physiologic and psychosocial sequelae depending on their size, location, growth trajectory, and tissue involvement. Diagnostic evaluation is centered around clinical examination, imaging evaluation with ultrasound and time-resolved magnetic resonance imaging, and genetic testing for more complex malformations. Interventional radiology has emerged as first-line management of venous malformations through endovascular treatment with embolization, while surgery and targeted molecular therapies offer additional therapeutic options. In this review, an updated overview of the genetics and clinical presentation of venous malformations in conjunction with key aspects of diagnostic imaging and treatment are discussed. [ABSTRACT FROM AUTHOR]

Purpose: Phleboliths are often observed within Venous malformations (VM) and frequently indicated as cause of morbidity. The aim of this study is to investigate independent risk factors for phleboliths in a pediatric population and to determine if its presence influences clinical management.Methods: We retrospectively review data from patients diagnosed with VM in a vascular anomalies center during a 5-year period. Associations between phleboliths and potential risk factors were assessed. A multivariable analysis, was performed to assess the influence of phleboliths in the need for surgery.Results: We included 88 patients with a mean age of 10 years. Phleboliths were found in 33.0%. In univariate analysis, there were no significant differences between the two groups regarding age or gender, location, dimension or depth of the VM, pain and laboratory parameters. Multivariable analysis could not detect any independent risk factor for phleboliths. In contrast, multivariable logistic analysis revealed that when phleboliths were present, the need for surgical extirpation was more likely (p = 0.031).Conclusions: This study showed that patients who have phleboliths within their VM seem to require surgery more frequently. This constitutes an entirely innovative thought that could raise awareness to a lower threshold for surgery in this group of patients. [ABSTRACT FROM AUTHOR]

Persistent left superior vena cava (LSVC) that drains into the left atrium (LA) via the left superior pulmonary vein (LSPV) is a rare systemic venous drainage anomaly. It can cause cyanosis and unexplained recurrent strokes. Undiagnosed, it can seriously disrupt the conduct of the cardiopulmonary bypass (CPB), causing sudden air lock and/or flooding of the operative field with venous blood. Its connection with the LSPV outside the pericardium makes its intraoperative diagnosis difficult. We report here the case of a 48-year-old man operated for mitral and aortic valve endocarditis, complicating a Laubry-Pezzi syndrome. The opening of the LA was followed immediately by the entrance of high volume of air bubbles into the superior vena cava cannula which resulted in sudden air lock of the venous outflow line. After multiple lowerings and cessations of pump flow, partial clamping of this cannula resulted in flooding of the LA with venous blood coming from the LSPV. The heart luxation did not allow us to find the LSVC in its usual intrapericardial location, between the LSPV and the left appendage. We had to widely open the left pleura to expose its completely extrapericardial path and its communication with the LSPV. The LSVC was temporally clamped during the remainder of the surgical procedure, then ligated at both ends. The patient underwent mitral valve repair, closure of the infundibular septal defect, aortic valve replacement and tricuspid annuloplasty. He was discharged 10 days later. [ABSTRACT FROM AUTHOR]

Background Femoral access is the primary route for neurointerventional procedures in children. However, endovascular treatment may not always be possible through a femoral approach, necessitating conversion to alternative access routes. Objective To review the feasibility and safety of direct carotid puncture (DCP) in infants undergoing neuroendovascular interventions. Methods We conducted a retrospective review of all infants who underwent DCP as the access route to treat neuroendovascular pathologies between January 2011 and January 2021. Patients' demographics, clinical presentation, imaging findings, and technical details were reviewed. Results Between January 2011 and January 2021, five infants aged between 28 and 150 days underwent DCP out of 1129 neuroendovascular interventions performed in our institution (0.4%). All five infants (100%) were diagnosed with intracranial fistulas and were found to have severe tortuosity of the cervical arteries. DCP was performed as the initial access route in 2/5 patients and as crossover after a failed femoral attempt in 3/5 patients. DCP was performed under ultrasound guidance in all patients. Closure was performed by manual compression, without complications. Ultrasound showed patent cervical vessels in all patients at 3 months' follow-up. Conclusion Direct carotid access is a feasible and safe alternative route to treat neuroendovascular pathologies in infants and can be considered in cases of inaccessible or failed transfemoral access or in cases with severe arterial tortuosity in infants with intracranial fistulas. [ABSTRACT FROM AUTHOR]

The scope of conditions managed by embolization, which was initially used for the treatment of hemorrhage and vascular malformations, is constantly expanding. Apart from oncologic indications, embolization is used to treat a wide range of benign pathology, including uterine fibroids and benign prostatic hyperplasia. While various particulate embolic agents are successfully used for benign embolization, there is growing evidence that unique properties of these may result in different outcomes. This article reviews available evidence comparing various particles used for uterine fibroid embolization and prostate artery embolization. In addition, we provide an overview of periprocedural pharmacology and protocols facilitating same-day discharge for these interventions. [ABSTRACT FROM AUTHOR]

Vascular anomalies are heterogeneous conditions that affect blood and/or lymphatic vessels. Affected children may experience pain, functional loss, infection, coagulopathies, and psychological challenges. Diagnosis and management often warrant an interdisciplinary approach. There are seven vascular anomalies clinics in Canada that offer interdisciplinary care. This practice point outlines a treatment approach for the most common paediatric vascular anomaly (hemangioma). It reviews indications for referral to a specialized clinic, with focus on complex vascular anomalies, specifically infantile hemangioma, which can pose complications. [ABSTRACT FROM AUTHOR]

Inferior vena cava (IVC) can be involved by a wide gamut of developmental anomalies owing to its complex embryogenesis. Developmental anomalies of the IVC are not infrequent, seen in approximately 8.7% of the general population. Although most of the anatomical variations are asymptomatic, identification of these variations is important before planning any vascular surgery or interventional procedure in relation to the IVC to avoid inadvertent complications. Conventional venography has largely been replaced by noninvasive cross-sectional imaging modalities for detecting IVC abnormalities. Ultrasonography, often used for initial evaluation, is highly operator-dependent and the infrarenal part of IVC is often obscured by bowel gases. While magnetic resonance imaging is devoid of radiation risks, its use is limited due to limited availability and the frequent need for sedation. Computed tomography (CT) venography plays a pivotal role in the detection of these anomalies as it has an excellent spatial resolution along with availability of multiple postprocessing tools such as multiplanar reconstruction with generation of maximum intensity projection and volume-rendered images. This pictorial review focuses on the embryogenesis of IVC, various developmental anomalies of the IVC and its tributaries, their appearance on CT venography and conceivable clinical relevance. [ABSTRACT FROM AUTHOR]