Search

Purpose: Morning glory disc anomaly (MGDA) is a rare congenital ophthalmologic disorder. Historically it has been diagnosed fundoscopically, with little in the literature regarding its imaging findings. The purpose of this study is to further characterize the orbital and associated intracranial magnetic resonance imaging (MRI) findings of MGDA in our tertiary pediatric center. Methods: A retrospective review was performed of fundoscopically-diagnosed cases of MGDA, that had been referred for MRI. All MRI studies were scrutinized for orbital and other intracranial abnormalities known to occur in association with MGDA. Results: 18 of 19 cases of MGDA showed three characteristic MRI findings: funnel-shaped morphology of the posterior optic disc, abnormal soft tissue associated with the retrobulbar optic nerve, and effacement of adjacent subarachnoid spaces. The ipsilateral (intraorbital) optic nerve was larger in one patient and smaller in six. The ipsilateral optic chiasm was larger in two patients and smaller in one. Conclusion: This study represents a comprehensive radiological-led investigation into MGDA. It describes the most frequently-encountered MRI findings in MGDA and emphasizes the importance of MRI in this cohort, i.e., in distinguishing MGDA from other posterior globe abnormalities, in assessing the visual pathway, and in screening for associated intracranial abnormalities – skull base/cerebral, vascular, and facial. It hypothesizes neurocristopathy as an underlying cause of MGDA and its associations. Caliber abnormalities of the ipsilateral optic nerve and chiasm are a frequent finding in MGDA. Optic pathway enlargement should not be labeled "glioma". (239/250). [ABSTRACT FROM AUTHOR]

For a safe sclerotherapy session to be completed in the orbital low-flow malformation (namely lymphovenous malformation or venolymphatic malformation), accurate identification of the target lesion for the drug injection is crucial. Regarding the dependability and viability of the injection approach, the authors have discussed their experiences with image-guided percutaneous sclerotherapy on a few patients. [ABSTRACT FROM AUTHOR]

Background Intracranial cavernous malformations (CMs), commonly known as cavernomas or cavernous angiomas, are low-flow, well-circumscribed vascular lesions composed of sinusoidal spaces lined by a single layer of endothelium and separated by a collagenous matrix without elastin, smooth muscle, or other vascular wall elements. A diameter greater than 3 cm for a CM is unlikely. These lesions may have atypical appearances on magnetic resonance imaging (MRI). MRI with advanced techniques such as a susceptibility-weighted image or T2-gradient echo, a diffusion-weighted image and corresponding apparent diffusion coefficient map, and diffusion tensor tractography have revolutionized the diagnostic approach to these lesions. Materials and Method The present study reviews the etiopathogenesis, clinical manifestations, MRI strategy, and MRI appearances of the CMs, with a few examples of the giant CMs from our archive. Results Intracranial giant CMs may have unexpected locations, sizes, numbers, and varied imaging appearances due to repeated hemorrhages, unusual enhancement patterns, intense perifocal edema, and unusual associations, making the differential diagnosis difficult. Conclusion Familiarity with the MRI appearances of the giant intracranial CMs and the differential diagnosis improves diagnostic accuracy and patient management. [ABSTRACT FROM AUTHOR]

Background Patients with vein of Galen malformations (VOGMs) can develop fine angiogenic networks with fistulous connections to the precursor of the vein of Galen. In these cases, transarterial embolization (TAE) with liquid embolic agents (LEAs) is challenging due to reflux in the pedicle leading to the network, causing poor penetration. Transvenous approaches carry a risk of hemorrhage from pathologic vasculature. Dual-lumen balloon microcatheters like the Scepter Mini (Microvention, Aliso Viejo, CA) improve distal pedicle access, preventing reflux. Objective Here, we report on the use of the Scepter Mini for TAE of angiogenic VOGM. Methods A single-institution retrospective chart review identified all VOGMs treated with Scepter Mini microcatheters. Clinical data, angioarchitecture, and technical parameters were reviewed. Results 17 Scepter Mini catheters were used in 12 embolization procedures of 7 patients with VOGM at a median age of 2.1 years. Patients presented with hydrocephalus (100%) and gross motor and speech delays (57.1%). Networks developed extra-axially into the subependymal zone fed by posterior choroidal, posterior cerebral, and thalamoperforator arteries. Posterior choroidal branches (n=7/17, 41.2%) were most frequently catheterized to achieve distal access to the network. Embolization with Onyx-18 and significant network penetration occurred in 17/17 uses. Near tip entrapment with LEA cast displacement occurred in 1/17 uses. Another patient experienced postprocedural intraventricular hemorrhage requiring a third ventriculostomy without permanent neurologic deficit. Conclusion The Scepter Mini provided excellent distal access with penetration to the fistula and extra-axial network reduction with few complications. The Scepter Mini provides a means for successful treatment of technically challenging angiogenic VOGM. [ABSTRACT FROM AUTHOR]

The effects of anomalous vasculature impeding optimal exposure to an anterior lumbar interbody fusion approach are limited in literature. We present five individual, unique cases of vascular anomalies in patients undergoing two-stage anterior-posterior lumbar interbody fusion. Cases 1, 2, 4, and 5 have yet to be described in literature in context of anterior lumbar interbody fusions. Case 3 presents anomalous vasculature that has only been described in two other case reports. Case 1 presents the right internal iliac vein originating from the left common iliac vein which was transected for L4-L5 vertebral disc exposure. Case 2 presents the left internal iliac vein originating from the right common iliac vein which required an oblique approach. Case 3 presents a duplicated inferior vena cava that was taken into account but did not interfere with the anterior retroperitoneal approach. Case 4 presents large osteophytes adhering to the left common iliac vein which limited safe dissection and mobilization. Case 5 presents the left internal iliac vein with a high takeoff spanning across the L5-S1 vertebral disc space and requiring transection. This case series highlights the need for preoperative imaging and a working detailed knowledge of anatomy to avoid damaging vasculature that can potentially lead to fatal consequences. The information given in this case series should inform both spine and vascular surgeons on proper preoperative planning. To maximize operative efficiency and safety, spine surgeons and vascular surgeons should collaborate to minimize surgical complications. [ABSTRACT FROM AUTHOR]

Congenital vascular malformations (CVMs) are the result of an aberrant development during embryogenesis. Although these lesions are present at birth, they are not always visible yet. Once symptomatic, patients suffer from pain, bleeding, ulcers, infections or lymphatic leakage, depending on the subtype of vessels involved. Treatment includes conservative management, surgery, sclerotherapy, embolization and pharmacological therapy. The clinical presentation varies widely and treatment can be challenging due to the rarity of the disease and potential difficulties of treatment. This review gives an overview of the historical developments in diagnosis and classification and exposes the key elements of innovations in the past decades on the identification of genetic mutations and personalized treatment. These advances in the field and a multidisciplinary approach are highly valuable in the optimization of clinical care aimed at both curing or stabilizing the CVM and pursuing physical and psychosocial wellbeing. [ABSTRACT FROM AUTHOR]

Introduction: Persistent left superior vena cava (PLSVC) is one of the major sources of triggers and drivers of atrial fibrillation (AF). There has been no established PLSVC ablation procedure to eliminate the arrhythmogenicity along the entire length of PLSVC. Methods and Results: A 70‐year‐old woman with a history of two previous catheter ablations for AF, mitral valvuloplasty, and an unroofed coronary sinus‐type atrial septal defect closure underwent the redo AF ablations. The AF trigger and driver were identified within the patient's enlarged PLSVC. The AF was treated by complete PLSVC free wall isolation. Conclusion: Complete PLSVC free wall isolation may be an effective ablation method to eliminate the arrhythmogenicity along the entire length of the PLSVC. [ABSTRACT FROM AUTHOR]

Introduction: Management of transvenous leads in patients with congenital heart disease (CHD) can be complicated by venous obstructions and residual shunts. We present our experience performing concurrent lead extraction and dilation/stenting of venous pathways, including patients with complete venous obstruction. Methods: All cases of concurrent lead extraction and recanalization of vena cavae/baffles between 2017 and 2021 at Boston Children's Hospital were retrospectively included and reviewed for safety and efficacy. Results: Eight patients, 4 female, median 38.5 years of age (range 16.7−49 years) and 81.6 kg weight (range 41.3−97.8 kg) at time of procedure were included. All patients had CHD, a majority (n = 7) having transposition of the great arteries palliated via atrial switch. All leads were removed in their entirety, with most patients having two leads extracted (n = 7). Median lead dwell time was 13.8 years (range 3.6−35.3 years). Three patients had complete obstructions, three required stenting of their innominate veins and three required recanalization of their femoral vessels. Median procedure time was 9.8 h (range 5.4−12.8 h). Complications included blood transfusion (n = 2), arrhythmia (n = 3), pleural effusion (n = 1), and pressure ulcer (n = 1). There were no cardiac perforations, venous tears, or deaths. Conclusion: Lead extraction along with dilation and stenting of venous anomalies, though long in duration, proved effective with minimal complications. This combined procedure can safely and effectively resolve complete obstructions secondary to transvenous leads. [ABSTRACT FROM AUTHOR]

Objective: Small extracellular vesicle (sEV)‐mediated intercellular communication is increasingly the key for the understanding of venous malformations (VMs). This study aims to clarify the detailed changes of sEVs in VMs. Subjects and Methods: Fifteen VM patients without treatment history and twelve healthy donors were enrolled in the study. sEVs were isolated from both fresh lesions and cell supernatant, and were examined by western blotting, nanoparticle tracking analysis and transmission electron microscopy. Western blot analysis, immunohistochemistry and immunofluorescence were adopted to screening candidate regulator of sEV size. Specific inhibitors and siRNA were employed to validate the role of dysregulated p‐AKT/vacuolar protein sorting‐associated protein 4B (VPS4B) signaling on the size of sEVs in endothelial cells. Results: The size of sEVs derived from both VM lesion tissues and cell model was significantly increased. VPS4B, whose expression level was mostly significantly downregulated in VM endothelial cells, was responsible for the size change of sEVs. Targeting abnormal AKT activation corrected the size change of sEVs by recovering the expression level of VPS4B. Conclusion: Downregulated VPS4B in endothelial cells, resulted from abnormally activated AKT signaling, contributed to the increased size of sEVs in VMs. [ABSTRACT FROM AUTHOR]

Objective: The International Union of Phlebology recommends measuring at least D-dimer and fibrinogen levels in the diagnosis of extensive extra-truncular venous malformations, with a surface area of 10 cm2 or those which are deep, as well as prior to any interventional procedure. The aim of the study was to characterise venous malformations associated with a possible vascular complication. Method: This study was an observational and multicentre study. The objective was to explore the presence of a possible coagulation disorder among patients with venous malformation. The primary endpoint was to characterise venous malformations with increased D-dimer levels. Results: The majority of the 72 venous malformations were located in the trunk region, mostly in intramuscular or subcutaneous locations. There were 72 venous malformations with increased D-dimer levels including 3 with biological disseminated intravascular coagulation (elevated D-dimer and fibrinogen <1 g/L). The anticoagulant treatments administered were very heterogeneous in class and dosage, and at the end of the treatment, 17 elevated D-dimers were persistent, 9 venous malformations remained painful and 27 showed thrombotic regression. Conclusion: Venous vascular malformations are probably underestimated and should probably be explored more systematically in terms of coagulation disorder regardless of size or symptomatology. The therapeutic recommendations to treat localised intravascular coagulation with low-molecular weight are not widely applied. Studies are needed, in particular to assess the role of oral anticoagulants in the management of coagulation disorder among patients with venous malformation. [ABSTRACT FROM AUTHOR]

Megalencephaly is a developmental disorder due to an abnormal neuronal proliferation and migration during intrauterine or postnatal brain development that leads to cerebral overgrowth and neurological dysfunction. This cerebral overgrowth may affect the whole encephalon or only a region; when it involves one hemisphere it is referred to as hemimegalencephaly. Megalencephaly presents with a head circumference measurement of 2 standard deviations above the average measure for age. This group of disorders is clinically characterized by early onset and refractory to therapy epilepsy, neurodevelopmental disorders, behavioral problems, and autism spectrum disorder. Syndromic forms of megalencephaly should be considered when associated with other congenital abnormalities. Megalencephaly in fact could be associated with segmental overgrowth and cutaneous/vascular abnormalities (i.e., Proteus syndrome, CLOVES [congenital lipomatous overgrowth, vascular malformations, epidermal naevi, scoliosis, and/ or skeletal abnormalities] syndrome, Klippel-Trenaunay syndrome, megalencephaly-capillary malformation-polymicrogyria syndrome , megalencephaly-postaxial polydactyly-polymicrogyria-hydrocephalus syndrome, etc.) or generalized overgrowth (i.e., Weaver or Beckwith-Wiedemann syndrome) as well as with nanism in achondroplasia where megalencephaly is associated with disproportionate short stature, primary skeletal dysplasia, characteristic facies (prominent forehead, flat nasal bridge), narrow chest, and normal intelligence. It is possible to identify three main groups of disorders associated with megalencephaly: idiopathic or benign, metabolic, and anatomic. The idiopathic (benign) form indicates an abnormal increased head circumference in absence of neurological impairment, such as in benign familial megalencephaly. In metabolic megalencephaly (such as in organic acid disorders, metabolic leukoencephalopathies, or lysosomal diseases) there is an increase of different constituents that increase the size of the brain, whereas in the anatomical form there are underlying genetic causes. Neuroimaging is crucial for diagnosis, as it can reveal a generalized brain growth or a segmental one and possible specific frameworks associated. In all these conditions it is necessary to identify possible microdeletion-microduplication by chromosomal arrays. [ABSTRACT FROM AUTHOR]

Vascular anomalies are localized congenital morphogenetic defects in the blood vessels. They represent a broad spectrum of disorders, ranging from a simple "birthmark" to life-threatening entities. Most (60%) occur in the head and neck region, and each anomaly has a different etiology and clinical behavior; therefore, an accurate diagnosis is crucial for appropriate evaluation and management. This study aims to briefly elaborate on the vascular anomalies, their etiology, and clinical features and assess the role of sclerosing agents in treating venous malformations. The International Society for the Study of Vascular Anomalies (ISSVA) classifies vascular anomalies into vascular tumors and malformations, with subdivisions of each. This classification is accepted worldwide and has become the official system for classifying vascular anomalies. Hemangiomas are the most common type of vascular tumors, while venous malformations are the most common type of vascular malformations. Venous malformations can be treated conservatively without surgical disfigurement. Sclerotherapy is the first-line treatment for venous malformations, as it is an easy-to-perform procedure with minimal equipment needed. [ABSTRACT FROM AUTHOR]

Congenital extrahepatic portosystemic shunts (CEPS) are rare anomalies connecting the portal system to the inferior vena cava. This report discusses a 10-year-old boy with Type II c CEPS, presenting cyanosis and dyspnea. Surgical ligation resulted in significant improvement in symptoms. Early identification and intervention are crucial, necessitating a protocolized approach. [ABSTRACT FROM AUTHOR]

Vascular anomalies are a diverse group of abnormal blood vessel developments that can occur at birth or shortly afterward. Embolization and sclerotherapy have been utilized as a treatment option for these malformations but may cause moderate-to-severe pain. This study aims to evaluate the utilization of peripheral nerve blocks in opioid consumption, pain scores, and length of stay. A retrospective chart review was conducted at the UPMC Children's Hospital of Pittsburgh for all patients who underwent embolization and sclerotherapy between 2011 and 2020. Patient data were collected to compare opioid consumption, pain scores, and length of stay. In total, 854 procedures were performed on 347 patients. The morphine milligram equivalent per kilogram mean difference between groups was 0.9 (0.86, 0.95) with a p-value of <0.001. The pain score mean ratio was −1.17 (−2.2, −0.1) with a p-value of 0.027. The length of stay had an incident rate ratio of 0.94 (0.4, 2) and a p-value of 0.875. By decreasing opioid consumption and postoperative pain scores, peripheral nerve blocks may have utility in patients undergoing embolization and sclerotherapy while not clinically increasing the length of stay for patients. Their use should be individualized and carefully discussed with the interventional radiologist. [ABSTRACT FROM AUTHOR]

Purpose: To highlight median arcuate ligament syndrome as a potential cause for celiac artery stenosis and pancreaticoduodenal artery aneurysm, and describe treatment options in this setting. Case Report: A 63-year-old male presented with a pancreaticoduodenal artery aneurysm and concomitant celiac artery stenosis that was treated with celiac artery stenting and aneurysm coiling. He subsequently developed stent fracture and celiac artery occlusion secondary to previously unrecognized median arcuate ligament syndrome causing reperfusion of the aneurysm. This was treated with open median arcuate ligament release and aorta to common hepatic artery bypass with good clinical result and stable 20-month surveillance imaging. Conclusion: It is critical to recognize median arcuate ligament syndrome as a cause of celiac artery stenosis in the setting of pancreaticoduodenal artery aneurysm given the high risk of failure of endovascular stenting. Open aorto-hepatic artery bypass and endovascular aneurysm coiling should be the preferred approach in these patients. [ABSTRACT FROM AUTHOR]

Purpose: The use of magnetic resonance angiography (MRA) for assessing CNS fetal vasculature has been limited. The aim of this study was to determine the feasibility and added value of 2D time-of-flight (TOF) MRA of the fetal brain vasculature with a 1.5 T scanner. Methods: We conducted a prospective study (September 2018 to October 2022) by consecutively selecting pregnant women (≥ 18 years) with clinical indication to fetal brain MRI. On a 1.5 T scanner, a 2D TOF MRA acquisition was obtained at the end of the clinical protocol. Two neuroradiologists independently reviewed all MRIs; a qualitative scale of motion artifacts was applied to MRA images; represented vessels in MRA and T2 images were registered. Results: Thirty-five fetal brain MRIs. Mean maternal age: 32 years; mean fetal gestational age (GA): 31 weeks. Artifacts were found in 74% of MRA. The number of MRAs performed without artifacts increased with GA. On MRA, the identification of the majority of vessels increased with GA; statistical significance was reached in the identification of torcular Herophili (p = 0.026), vein of Galen (p < 0.001), internal cerebral veins (p = 0.002), basilar artery (p = 0.027), vertebral arteries (p = 0.025), and middle cerebral arteries (p = 0.044). Significantly, MRA depicted the sigmoid sinuses and internal jugular veins more frequently. Vascular pathology was found in 3/35 fetal brain MRIs. Conclusion: Although artifacts were found in 74% of cases, MRA acquisitions were informative and of sufficient diagnostic quality in most studies. This technique may represent a valuable complimentary tool in CNS prenatal vascular studies. [ABSTRACT FROM AUTHOR]

Background: Venous malformations (VMs) are the most common vascular malformations, which can be classified as focal, multifocal, or diffuse types. But the risk of focal venous malformations with muscle involvement is not well defined. Methods: This is a single-center, retrospective review of patients treated for focal VMs between February 2021 and February 2022. Results: We assessed 26 patients focal VMs with 47 lesions; 18 (69%) were unifocal, 3 (12%) were dual-focal, and 5 (19%) were multifocal type VMs, and 29 (62%) were intramuscular VMs. The lower limbs intramuscular VMs had a significantly elevated risk of focal VMs (relative risk [RR],1.7; 95% confidence interval [CI], 1.148–2.394). Conclusion: Intramuscular involvement of the body should be considered in focal VMs. The lower limbs intramuscular VMs had a significantly elevated risk of focal VMs. [ABSTRACT FROM AUTHOR]

Background Most neonates with persistent left superior vena cava (PLSVC) have no clinical symptoms or hemodynamic changes, and this anomaly is only found during cardiac catheterization, pacemaker implantation, or central venous catheterization. Electrocardiogram (ECG) localization is helpful for the application of the peripherally inserted central catheter (PICC) technique in neonates with PLSVC. Objective To explore the characteristic waveforms of the P wave when a PICC under ECG localization is applied in neonates with PLSVC. Study Design The observation and management strategies for the P wave changes during catheter insertion (CI) of two neonates with PLSVC admitted to our institution between January and July 2020, who underwent PICC line insertion, were summarized. Results The characteristic P wave changes in two children with a PICC line inserted via the PLSVC were observed. When a wide inverted P wave appeared on ECG, the catheter was immediately withdrawn by 0.5 cm, a bidirectional P wave gradually appeared and then disappeared. After that, the catheter was further withdrawn by 0.5 cm. After catheterization, the optimal position of the PICC was confirmed by X-ray photography and bedside B-ultrasound. The PICC line was removed as scheduled after indwelling for 18 and 29 days, respectively, in the two cases, and no PICC-related complications occurred during indwelling. Conclusion The characteristic P wave changes on ECG during CI provide important clinical reference values for the application of the PICC technique under ECG localization in neonates with PLSVC. Key Points Electrocardiogram localization. Peripherally inserted central catheter. Persistent left superior vena cava. [ABSTRACT FROM AUTHOR]

Congenital anomalies of inferior vena cava are increasingly being recognized with the technical advancements and increased utilization of cross-sectional imaging techniques. Duplication of inferior vena cava classically involves duplication of the infrarenal segment, where both inferior vena cava ascend on either side of the abdominal aorta until they form a confluence at the level of the renal veins. It has been extensively described in literature with few reports of more complex variation in the form of duplicated infrarenal inferior vena cava with azygos or hemiazygos continuation. This article describes extremely rare complete duplication of inferior vena cava involving both suprarenal and infrarenal segments. Moreover, the complete duplication of inferior vena cava is seen in association with concomitant double superior vena cava, in a patient with visceroatrial situs solitus and associated congenital heart disease, which to the best of our knowledge, has not been reported so far in literature. This study also highlights the utility of multidetector computed tomography in accurate identification of such anomalies. [ABSTRACT FROM AUTHOR]

According to the International Society for the Study of Vascular Anomalies (ISSVA) classification, vascular anomalies include a diverse range of pathologies, classified as either vascular tumors or vascular malformations. This classification, last revised in 2018, aims to explain the biological basis of vascular lesions and help clinicians to manage the anomalies. In vascular tumors, there are proliferative changes of endothelial cells, while vascular malformations primarily consist of structural vascular abnormalities. Infantile hemangioma is the most common soft-tissue vascular tumor. Vascular malformations are an extensive group of malformations of the arterial, venous, and lymphatic systems, either in isolation or in combination. Radiological evaluation plays a key part in the management of pediatric patients with these entities. The understanding of sonography and magnetic resonance imaging findings entails its correlation with clinical findings at the time of scanning. [ABSTRACT FROM AUTHOR]

Single umbilical artery (SUA) is an uncommon yet clinically noticeable anomaly that has been suspected to be correlated with a wide range of pregnancy complications. We hereby present a 30-year-old pregnant woman who was diagnosed with SUA fetus in the 20th week of her pregnancy and was admitted to our center in the 33rd week. During the hospitalization, Doppler studies were performed to monitor fetal development. Later, fetoplacental insufficiency and brain-sparing effect were reported on Doppler ultrasound, indicating asymmetrical Intrauterine growth restriction (IUGR). SUA might be associated with concurrent fetal anomalies including cardiological, nephrological, gastrointestinal, and nervous disorders. Moreover, there is an increased risk of small for gestational age and IUGR compared with normal pregnancies. It is crucial to assess the umbilical cord anatomy during pregnancy to diagnose SUA at lower gestational ages and schedule a precise follow-up to prevent adverse outcomes. [ABSTRACT FROM AUTHOR]

A 50‐year‐old woman underwent catheter ablation for atrial fibrillation. Preoperative computed tomography revealed a left‐sided variant of the right top pulmonary vein (PV) and a persistent left superior vena cava. The right top PV was successfully isolated through a wide antral circumferential ablation line simultaneously with the right PVs. [ABSTRACT FROM AUTHOR]

Atraumatic splenic rupture is rare and not often considered in the differential diagnosis for patients with abdominal pain. This article describes a patient with atraumatic splenic rupture complicated by a congenital splenorenal anomalous shunt. The congenital anomaly increases patient risk and the degree of surgical difficulty, even if it is identified preoperatively. [ABSTRACT FROM AUTHOR]

Objectives: Single umbilical artery (SUA) is considered the most common abnormality of the umbilical artery. The objective of the study was to evaluate the perinatal prognosis of fetuses with SUA and to describe the associated malformations. The significance of the study is represented by examining whether our findings are in correlation with data already described. Methods: We performed a prospective cohort study on singleton pregnancies complicated with SUA. The study population was composed of women with singleton pregnancies who were examined at the Department of Obstetrics and Gynecology of the Târgu Mures County Emergency Clinical Hospital between 2012 and 2021. Results: The incidence of SUA in the study population was 0.48%. C-section was performed in 40 cases with SUA and in 5258 cases with no SUA (RR:1.56, P<0.05.) From the total number of 2249 premature deliveries, 23 newborns were diagnosed with SUA (RR:2.12, P<0.05.) From the total number of 869 deliveries with low birth weight (LBW) newborns, 13 were associated with SUA (RR: 3.12, P<0.05.) There were 206 pregnancies noted with antenatal fetal demise after 24 weeks of gestation, and only 2 of them were with SUA (RR:2.01, P>0.05.) Fetal and neonatal malformations were described in 290 cases, and 28 were associated with SUA (R:21.96, P<0.05.) In 57 of 85 cases (67.05%), we found iSUA, and 28 newborns (32.95%) had minor, major, or other associated pathologies. We found two cases of trisomy 18 and one case with trisomy 13 associated with SUA. Investigating the malformations associated with SUA, the most common were cardiac and great vessels malformations (12), followed by limb malformations (8), urogenital malformations (7), digestive tract malformations (7), central nervous system malformations (4), and in one case we found cleft palate. Conclusions: Perinatal prognosis regarding SUA is significantly poorer than in cases without this pathology. One-third of fetuses with SUA were associated with fetal anomalies. The most common pathologies associated with SUA were cardiovascular, limb, urogenital, and digestive system malformations. Our data are similar to those described in other studies; therefore, we conclude, we can implement the general recommendations in our region regarding counselling patients. [ABSTRACT FROM AUTHOR]

A persistent stapedial artery is a congenital vascular malformation that can cause tinnitus and/or conductive hearing loss. Although rare, this case highlights the importance of recognizing aberrant anatomy as a potential cause of patients' symptoms. It also demonstrates how to recognize and treat patients with a symptomatic persistent stapedial artery. [ABSTRACT FROM AUTHOR]

Background: Persistent left superior vena cava (PLSVC) is the most prevalent form of thoracic venous abnormality and can serve as a significant arrhythmogenic source in atrial fibrillation (AF). Methods and results: Among the 3950 patients who underwent radiofrequency ablation for AF between September 2014 to April 2020, 17 patients (mean age 59.4 ± 8.0 years, 64.7% male) with PLSVC were identified. Among them, nine patients (52.9%) had a prior history of pulmonary vein isolation (PVI) alone. Eight out of nine patients who experienced AF recurrence underwent PLSVC isolation with or without pulmonary vein (PV) reconnection. For the remaining eight patients (47.1%), PVI plus PLSVC isolation were performed during the index procedure. Ectopy originating from PLSVC was documented in 11 patients (64.7%) and successful PLSVC isolation was achieved in 16 patients (94.1%). After a median follow‐up of 28.3 months, freedom from AF/ atrial tachycardia (AT) was observed in 13 patients (76.5%). Conclusion: Empirical PLSVC isolation beyond PVI appears to be a feasible and safe strategy to prevent AF recurrence in patients with concomitant PLSVC. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 32-year-old man with a rare manifestation of port-wine stain (PWS), characterized by rapid hypertrophy and nodularity indicative of a combined capillary-lymphatic-arteriovenous malformation (CLAVM). It mentions that the patient, who had a purple macule since birth, developed additional nodules and discharges, leading to a diagnosis through imaging and biopsy.

Children who have lymphatic malformations frequently experience functional limitations and aesthetic abnormalities that have a significant impact on their quality of life and may pose a threat to their lives. Conventional treatments such as surgery or sclerotherapy are rarely curative, demonstrating the great need for new treatment modalities. Recently, oral or topical administration of sirolimus has successfully treated lymphatic malformations. We report the 6-month treatment outcome of a 7-year-old boy with lesions consistent with lymphangioma circumscriptum on the left side of the neck since birth who was successfully treated with topical sirolimus. [ABSTRACT FROM AUTHOR]

Hereditary hemorrhagic telangiectasia (HHT) is a rare genetic disorder characterized by angiodysplasia which may manifest with telangiectasia and arteriovenous malformations (AVMs) in the central nervous system, lung, liver, and gastrointestinal tract. Women suffering from HHT may have a normal pregnancy course but some pregnancies are at risk of fulminant complications related to hormonal and cardiovascular changes of pregnancy. Hence, it becomes very important to diagnose this condition promptly and monitor women with HHT carefully to reduce morbidity and mortality associated with the disease. We present two women diagnosed with HHT in pregnancy who presented to our institution with different clinical scenarios. Based on the clinical findings, family history and high index of suspicion, we were able to diagnose and manage them accordingly. [ABSTRACT FROM AUTHOR]

Background: Heyde's syndrome is known as a combination of gastrointestinal (GI) bleeding and aortic valve stenosis. However, there are no reports of an association between GI bleeding and bioprosthetic valve stenosis initially occurred after aortic valve replacement (AVR), even though there are several reports that GI bleeding due to native aortic valve stenosis disappeared after AVR, and GI bleeding recurred due to bioprosthetic valve stenosis or patient-prosthesis mismatch. Case presentation: An 80-year-old woman who was on hemodialysis for 13 years had undergone AVR with a bioprosthetic valve for aortic regurgitation 3 years prior. She was admitted with acute heart failure and anemia that required repeated blood transfusions. Capsule endoscopy revealed multiple active hemorrhages of the small intestine due to angiodysplasia. Echocardiography showed severe bioprosthetic valve stenosis in the aortic valve position as a result of structural valve deterioration (SVD). Because Heyde's syndrome was strongly suspected even though gel electrophoresis analysis of von Willebrand factor multimers, the gold standard examination for the definitive diagnosis of Heyde's syndrome, was not performed, a redo AVR with a new bioprosthetic valve was performed. After the second AVR, both the heart failure and anemia due to GI bleeding promptly improved. Conclusions: Bioprosthetic valve stenosis due to SVD can bring GI bleeding just as in native aortic valve stenosis. Redo AVR is a promising treatment if the combination of GI bleeding and valve stenosis in the aortic valve position appears even after AVR. [ABSTRACT FROM AUTHOR]

Median arcuate ligament syndrome (MALS) is an uncommon disorder characterized by postprandial abdominal pain, vomiting, anorexia, and weight loss. The symptoms are due to the MAL compressing the celiac artery and irritating the celiac plexus/ganglion. We report the case of one patient who presented with chronic abdominal pain, bloating, vomiting, and weight loss. Computed tomography angiography led to the diagnosis of MALS. The patient was treated surgically by laparoscopic division of the MLA and excision of the celiac plexus. He was discharged on day two without complications. On one-month follow-up, he was symptom-free, eating well, and had gained weight. He is on regular follow-up. [ABSTRACT FROM AUTHOR]

Objective: Recently, the occlusion rate of transarterial embolization (TAE) for intracranial non-sinus-type dural arteriovenous fistulas (NSDAVFs) has improved after ONYX was introduced. Additionally, when TAE for NSDAVF is unsuccessful, transvenous embolization (TVE) has become available as an alternative treatment. We investigated the factor for the favorable occlusion rate of endovascular treatment for NSDAVF at our institutions. Methods: Two hundred and twenty-seven patients with intracranial dural arteriovenous fistulas (DAVFs) were treated at our institutions between September 2014 and October 2022. The patients diagnosed with NSDAVF in all DAVFs who underwent endovascular treatment were included. The clinical characteristics, angiographical outcomes, and clinical outcomes of patients who underwent endovascular treatment were evaluated. Results: Thirty-eight patients had intracranial NSDAVF (tentorial: 23 cases, parasagittal-convexity: 7, anterior cranial fossa: 6, middle cranial fossa: 2). Our participants' mean age was 64.8 ± 11.3 years, and 31 (81.6%) of them were males. Patients' symptoms were as follows: asymptomatic (24), hemorrhage (10), tinnitus (3), and trigeminal neuralgia (1). TAE and TVE were performed on 35 and 3 patients, respectively. The rate of immediate angiographical occlusion was 84.2% (32/38). The follow-up angiographical occlusion rate in 6 months was 88.5% (31/35). Complications occurred in three cases. There was no morbidity or mortality after 30 days. Conclusion: TAE using the combination of the new microcatheter and microguidewire and TVE in the case of difficult or failed TAE for NSDAVF could achieve high success rates and safety. [ABSTRACT FROM AUTHOR]

Background Anterior cranial fossa dural arteriovenous fistulas (ACF-dAVFs) are aggressive vascular lesions. The pattern of venous drainage is the most important determinant of symptoms. Due to the absence of a venous sinus in the anterior cranial fossa, most ACF-dAVFs have some degree of drainage through small cortical veins. We describe the natural history, angiographic presentation and outcomes of the largest cohort of ACF-dAVFs. Methods The CONDOR consortium includes data from 12 international centers. Patients included in the study were diagnosed with an arteriovenous fistula between 1990-2017. ACF-dAVFs were selected from a cohort of 1077 arteriovenous fistulas. The presentation, angioarchitecture and treatment outcomes of ACF-dAVF were extracted and analyzed. Results 60 ACF-dAVFs were included in the analysis. Most ACF-dAVFs were symptomatic (38/60, 63%). The most common symptomatic presentation was intracranial hemorrhage (22/38, 57%). Most ACF-dAVFs drained through cortical veins (85%, 51/60), which in most instances drained into the superior sagittal sinus (63%, 32/51). The presence of cortical venous drainage predicted symptomatic presentation (OR 9.4, CI 1.98 to 69.1, p=0.01). Microsurgery was the most effective modality of treatment. 56% (19/34) of symptomatic patients who were treated had complete resolution of symptoms. Improvement of symptoms was not observed in untreated symptomatic ACF-dAVFs. Conclusion Most ACF-dAVFs have a symptomatic presentation. Drainage through cortical veins is a key angiographic feature of ACF-dAVFs that accounts for their malignant course. Microsurgery is the most effective treatment. Due to the high risk of bleeding, closure of ACF-dAVFs is indicated regardless of presentation. [ABSTRACT FROM AUTHOR]

Uterine vascular anomalies (UVAs), while rare, can result in severe, life-threatening hemorrhage. An understanding of the presentation and management options for UVAs is important for interventional radiologists to appropriately evaluate and care for these patients. The authors propose a standardized terminology for UVAs to avoid confusion and conflating congenital from acquired vascular lesions, which have a different pathophysiology. Limited high-level evidence and no definitive guidelines for UVA management exist, although endovascular treatment with uterine artery embolization has generally become the first-line approach for symptomatic or persistent UVAs with high technical and clinical success rates. There is also no consensus on the optimal embolization technique; the authors propose an initial approach to first embolize the dominant uterine artery supplying the UVA with gelatin sponge, with the option to embolize the contralateral side at the time of initial embolization if there is persistent supply (avoiding bilateral empiric embolization). Repeat embolization is feasible and recommended in the setting of recurrence, and both clinical and imaging follow-up is important. Ultimately, a multidisciplinary approach with individualized patient management is needed, particularly in the face of a lack of consensus guidelines for the management of symptomatic UVAs. [ABSTRACT FROM AUTHOR]

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]

Dieulafoy's disease is a rare and important cause of recurrent upper gastrointestinal bleeding, which can cause fatal gastrointestinal bleeding. We reported a patient with Dieulafoy's disease in both of the stomach and duodenum for the first time. The patient achieved no effective hemostasis after two endoscopic treatments and two arterial angiography embolizations. Finally, successful hemostasis was achieved through surgery. There are many endoscopic treatment methods, but there is no consensus on the best treatment for hemorrhagic Dieulafoy's disease. Surgery is only a last resort after the failure of endoscopic treatment and angiography embolization. [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]

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]