Search Results (54)

Background: Mixed connective tissue disease (MCTD) is a rare systemic autoimmune disease which presents with clinical features that overlap with at least two connective tissue disorders, including systemic lupus erythematosus (SLE), systemic sclerosis (SSc), polymyositis (PM), dermatomyositis (DM), and rheumatoid arthritis (RA). It is characterized by the presence of anti-ribonucleoprotein (anti-U1RNP) antibodies. The mechanism of the vasculopathy associated with MCTD remains largely unknown. Optical coherence tomography angiography (OCTA) is a non-invasive imaging method of the microvasculature of the retina and choroid, providing the assessment of retinal perfusion. Objectives: The aim of the study was to evaluate the optical coherence tomography angiography (OCTA) parameters in patients with mixed connective tissue disease compared to healthy individuals. Methods: In this study, we compared the following parameters between patients with MCTD and healthy subjects: foveal avascular zone (FAZ), FAZ perimeter (PERIM), flow density (FD), choriocapillaris flow area (CCFA), outer retina flow area (ORFA), and foveal and parafoveal mean superficial and deep vessel density. Results: Parafoveal mean superficial vessel density and parafoveal mean deep vessel density were significantly lower in the MCTD group than in controls. The FAZ, FAZ PERIM, and FD values in the patients with MCTD were lower than in the control group and statistically significant for all parameters. Conclusions: The present study’s findings suggest the presence of ocular vascular abnormalities in patients suffering from MCTD. These abnormalities are characterized by decreased retinal vessel density and lower choriocapillaris flow. The results of the study demonstrate the significant role of OCTA in the diagnosis and monitoring of microvascular changes in patients with MCTD. Full article

Background and Clinical Significance: Fever of undetermined origin (FUO is a diagnostic challenge. It is essential to exclude infections, paraneoplastic syndromes, and large-vessel vasculitis (LVV). Case presentation: We describe a 59-year-old female with FUO and no apparent signs of infection. Laboratory results were consistent with inflammation (ESR 83, CRP 203 (ref. value (RV) < 5 mg/dl), ferritin 311 (RV < 120 µg/L), microcytic anemia, thrombocytosis. With administration of both ceftriaxone and levofloxacin, a decrease in CRP was monitored (51 mg/L). HRCT of the chest, abdomen, and pelvis revealed a saccular aneurysm of the descending thoracic aorta and an ectatic right common iliac artery. Due to suspicion of LVV, CT angiography was performed to exclude inflammatory changes in the blood vessels. Diffuse atherosclerosis with a 30 mm penetrating thoracic aortic ulcer (PAU) was found. PET-CT and leukocyte scintigraphy were used to rule out vasculitis and infection. The patient was successfully treated with Thoracic Endovascular Aortic Repair (TEVAR). Conclusions: As sophisticated imaging techniques become more widely used, more PAUs are being detected as incidental abnormalities in individuals without acute aortic syndrome. With adequate management, many people with PAU can live a stable and healthy life without experiencing significant consequences. Full article

The potential concurrence of uterine leiomyoma and adenomyosis has been mentioned in several studies to date, but as co-existing entities, not as a sequence of pathologic events. This is the case of a young 31-year-old nulliparous patient presenting with unspecific pain and abnormal uterine bleeding (AUB) pattern symptoms, which was clinically diagnosed with FIGO 0 fibroid “in status nascendi”. Following removal, the tumor turned out to be a STUMP at the histopathologic workup. After 9 months postoperatively and two unremarkable follow-ups, the patient presented again for pelvic pain and AUB, when ultrasound revealed a heterogeneous endo-uterine tumor of 5 cm, rich in large vessels, with rapid growth at serial ultrasound. MRI established the diagnosis of adenomyosis. In the context of a prior STUMP, nulliparity and rapidly enlarging uterine mass, despite conservative management counseling in a multidisciplinary team, the patient preference was towards radical surgery to prevent any future reproductive organ-related distress, and she opted for total hysterectomy with bilateral adnexectomy. The co-existence of fibroid and adenomyosis has been signaled by several authors, but this is the first report of such a sequence of events (STUMP to large adenomyoma) with swift development, to the best of our knowledge. Full article

The vascular anatomy of the stomach is both complex and highly variable, with direct implications for oncologic, bariatric, esophageal, and interventional procedures. This comprehensive review combines anatomical, radiological, and surgical evidence on arterial and venous variations in the stomach. The left gastric artery, traditionally the first branch of the coeliac trunk, often shows variants such as a direct aortic origin or association with an abnormal left hepatic artery. The right gastric artery most frequently arises from the proper hepatic artery, but its origin can vary significantly. The gastroepiploic arteries exhibit diversity in their origin, size, and connection patterns, with occasional duplication or absence. Additional vessels, including the posterior gastric artery and the short gastric arteries, also contribute to variations in arterial supply. Venous drainage largely follows the arterial pattern. The left and right gastric veins and the gastroepiploic venous arcade are major routes, while variants of the left gastric vein and the gastrocolic trunk (Henle’s trunk) contribute to complexity through different convergence patterns. These vascular variations have significant clinical implications, as they impact the safety of D2 lymphadenectomy, the risk of ischemic complications during laparoscopic sleeve gastrectomy, the success of gastric conduit formation in esophagectomy, and the effectiveness of transarterial embolization for upper gastrointestinal bleeding. Preoperative vascular mapping with multidetector computed tomography angiography and 3D reconstruction reliably defines individual anatomy, allowing for customized surgical planning and reducing operative risks. Recognizing both common and rare gastric vascular variants is essential for safe and effective surgical and endovascular management of gastric disease. Full article

Giant cell arteritis (GCA) and Takayasu arteritis (TAK) are forms of primary large vessel vasculitis (LVV) affecting the aorta and its major branches. Timely diagnosis and accurate monitoring are essential to prevent irreversible damage. Current assessment strategies rely heavily on symptoms, physical examination, and inflammatory markers, which lack sensitivity and specificity, particularly in patients treated with IL-6 inhibitors. This narrative review provides a comprehensive overview of the role of imaging in monitoring LVV. Ultrasound, magnetic resonance imaging, and positron emission tomography better reflect disease activity and treatment response compared to conventional clinical and laboratory measures. Notably, emerging imaging-based tools such as the OMERACT GCA Ultrasound Score, the Takayasu Ultrasound Index, and the TAK Integrated Disease Activity Index (TAIDAI) are promising treat-to-target instruments. While computed tomography is primarily used to assess structural damage, conventional angiography now plays a more limited role, mainly reserved for procedural planning and haemodynamic evaluation. A key challenge remains: interpreting persistent vascular abnormalities, which may indicate active disease, vascular remodelling, or irreversible damage. Standardisation of imaging protocols and interpretation is needed, alongside further research on the prognostic value of imaging for relapse risk. This review supports a multimodal, patient-tailored approach in which imaging is central to the long-term management of LVV. Full article

Background/Objectives: Henoch–Schönlein purpura (HSP), or IgA vasculitis, is the most common small-vessel vasculitis in children, yet Indian cohort data remain limited. We aimed to describe the clinical profile, renal involvement, treatment patterns, relapse, and outcomes of pediatric HSP at a tertiary centre in South India. Methods: We conducted a retrospective review of children <18 years diagnosed with HSP (January 2013–October 2018) using EULAR/PRINTO/PRES criteria. Demographics, clinical features, laboratory parameters, treatments, and outcomes were abstracted from records and analyzed in SPSS (descriptive statistics; Chi-square/Fisher’s exact and t/non-parametric tests as appropriate). Subgroup comparisons included renal vs. non-renal disease and age <6 vs. ≥6 years. An exploratory analysis examined predictors of nephritis. Results: Of 43 children identified, 2 were excluded (misclassified as systemic lupus erythematosus); 41 were analyzed. Mean age was 8.5 years (range 3–17), male: female 1.4:1. A preceding febrile illness or upper respiratory tract infection was noted in 41.4% and 17%, respectively. Palpable purpura was universal; joint involvement 73.1%, abdominal pain 61.0%, vomiting 41.5%. Renal involvement 17% occurred only in children ≥6 years; exploratory testing supported a strong age-linked signal for nephritis. Laboratory abnormalities included anemia (48.7%), thrombocytosis (19.5%), and elevated ESR (51.2%). Skin biopsy (n = 29) showed IgA and complement deposition; renal biopsy (n = 2) showed ISKDC grades II–III. Treatments included NSAIDs 71.6%, corticosteroids 31.7%, and dapsone 24.4% (used for severe systemic/persistent cutaneous disease). Rash relapse 7.3% clustered with joint plus abdominal symptoms and was not observed among children with nephritis. At a mean 18.9-month follow-up, one child required long-term antihypertensives; no child progressed to end-stage renal disease. Conclusions: Pediatric HSP in this South-Indian cohort followed a largely self-limited course with favourable renal outcomes. Age ≥6 years flagged higher renal risk, supporting age-targeted urine and blood-pressure surveillance, while relapse appeared to follow a non-renal trajectory (joint/abdominal clustering). Steroid and dapsone use reflected clinical severity rather than relapse risk. Findings align with Indian series and suggest lower renal morbidity than some East-Asian reports, adding region-specific evidence to guide monitoring and counselling. Full article

As crucial transportation hubs and economic nodes, the underwater security and infrastructure maintenance of harbors are of paramount importance. Harbors are characterized by high vessel traffic and complex underwater environments, where traditional underwater inspection methods, such as diver operations, face challenges of low efficiency, high risk, and limited operational range. This paper introduces a collaborative survey and disposal system that integrates a deformable unmanned surface vehicle (USV) with a lightweight remotely operated vehicle (ROV). The USV is equipped with a side-scan sonar (SSS) and a multibeam echo sounder (MBES), enabling rapid, large-area searches and seabed topographic mapping. The ROV, equipped with an optical camera system, forward-looking sonar (FLS), and a manipulator, is tasked with conducting close-range, detailed observations to confirm and dispose of abnormal objects identified by the USV. Field trials were conducted at an island harbor in the South China Sea, where simulated underwater objects, including an iron drum, a plastic drum, and a rubber tire, were deployed. The results demonstrate that the USV-ROV collaborative system effectively meets the demands for underwater environmental measurement, object localization, identification, and disposal in complex harbor environments. The USV acquired high-resolution (0.5 m × 0.5 m) three-dimensional topographic data of the harbor, effectively revealing its topographical features. The SSS accurately localized and preliminarily identified all deployed simulated objects, revealing their acoustic characteristics. Repeated surveys revealed a maximum positioning deviation of 2.2 m. The lightweight ROV confirmed the status and location of the simulated objects using an optical camera and an underwater positioning system, with a maximum deviation of 3.2 m when compared to the SSS locations. The study highlights the limitations of using either vehicle alone. The USV survey could not precisely confirm the attributes of the objects, whereas a full-area search of 0.36 km² by the ROV alone would take approximately 20 h. In contrast, the USV-ROV collaborative model reduced the total time to detect all objects to 9 h, improving efficiency by 55%. This research offers an efficient, reliable, and economical practical solution for applications such as underwater security, topographic mapping, infrastructure inspection, and channel dredging in harbor environments. Full article

Sepsis causes profound microcirculatory dysfunction, where heterogeneous capillary perfusion and abnormal vasomotion contribute to tissue hypoxia and organ failure. Sublingual videomicroscopy is established in large animals and humans to monitor these alterations, but this approach has not been applied in murine models. We developed a method to assess sublingual perfusion and vasomotion in mice using sidestream dark field (SDF) videomicroscopy. Male C57BL/6 mice were anesthetized, and sublingual vessels were imaged for 90 min. Perfusion and vasomotion parameters were assessed, and a Fast Fourier Transform was performed on vasomotion data to characterize the frequency components of rhythmic microvessel diameter changes. Another group of animals was administered lipopolysaccharide (LPS) intraperitoneally as a model of systemic inflammation in sepsis. LPS-treated mice exhibited a significant decline in the proportion of perfused vessels at 90 min and in the microvascular flow index. Very low-frequency vasomotion (0.005–0.15 Hz) declined in controls but was preserved during endotoxemia, suggesting an active microvascular tone response to inflammatory stress. This study establishes the first murine protocol for sublingual SDF imaging, allowing early detection of perfusion deficits and vasomotor changes in experimental sepsis. The minimal-invasive approach offers a translational platform for mechanistic and therapeutic studies in sepsis. Full article

Background/Objectives: The rapid development of artificial intelligence is transforming the face of medicine. Due to the large number of imaging studies (pre-, intra-, and postoperative) combined with histopathological and molecular findings, its impact may be particularly significant in neurosurgery. We aimed to perform a scoping review of recent applications of deep learning in MRI-based diagnostics of brain tumors relevant to neurosurgical practice. Methods: We conducted a systematic search of scientific articles available in the PubMed database. The search was performed on 22 April 2024, using the following query: ((MRI) AND (brain tumor)) AND (deep learning). We included original studies that applied deep-learning methods to brain tumor diagnostics using MRI, with potential relevance to neuroradiology or neurosurgery. A total of 893 records were retrieved, and after title/abstract screening and full-text assessment by two independent reviewers, 229 studies met the inclusion criteria. The study was not registered and received no external funding. Results: Most included articles were published after 1 January 2022. The studies primarily focused on developing models to differentiate between specific CNS tumors. With improved radiological analysis, deep-learning technologies can support surgical planning through enhanced visualization of cerebral vessels, white matter tracts, and functional brain areas. Over half of the papers (52%) focused on gliomas, particularly their detection, grading, and molecular characterization. Conclusions: Recent advancements in artificial intelligence methods have enabled differentiation between normal and abnormal CNS imaging, identification of various pathological entities, and, in some cases, precise tumor classification and molecular profiling. These tools show promise in supporting both diagnosis and treatment planning in neurosurgery. Full article

Background/Objectives： Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a prevalent Mendelian disorder caused by mutations in the NOTCH3 gene, primarily impacting cerebral small blood vessels. This review aims to explore the involvement of large intracranial arteries in CADASIL, particularly focusing on the association with RNF213 polymorphisms, especially in Asian populations. Methods： A comprehensive literature review was conducted to gather data on the morphological features of both small and large intracranial arteries in CADASIL, examining clinical manifestations, imaging findings, and genetic associations. Results： The findings indicate that while CADASIL is predominantly characterized by small vessel disease, a significant number of patients also exhibit large artery involvement, particularly Asian populations where RNF213 polymorphisms may play a critical role. The review highlights the evidence of intracranial stenosis and the potential implications of traditional vascular risk factors, such as hypertension and diabetes mellitus, which are prevalent in these populations. Conclusions： The involvement of larger intracranial arteries in CADASIL underscores the complexity of the disease, suggesting that both genetic predispositions and environmental factors contribute to vascular abnormalities. Further research is needed to clarify these relationships and improve diagnostic and therapeutic strategies for CADASIL patients. Full article

Background and Clinical Significance: Kaposi sarcoma (KS) is a rare angio-proliferative mesenchymal tumor that predominantly affects the skin and mucous membranes but may involve lymph nodes and visceral organs. Clinically, it manifests as red-purple-brown papules, nodules, or plaques, either painless or painful, often with disfiguring potential. The diagnosis is traditionally based on clinical and histopathological evaluation, although non-invasive imaging techniques are increasingly used to support diagnosis and treatment monitoring. We report a case of HHV-8-negative Kaposi sarcoma evaluated with multiple non-invasive imaging modalities to highlight their diagnostic utility. Case Presentation: An 83-year-old man presented with multiple painful, violaceous papulo-nodular lesions, some ulcerated, on the lateral aspect of his left foot. Dermoscopy revealed the characteristic rainbow pattern. Dynamic Optical Coherence Tomography (D-OCT) allowed real-time visualization of microvascular abnormalities, identifying large serpentine and branching vessels with clearly delineated capsules. Line-field Optical Coherence Tomography (LC-OCT) showed irregular dermal collagen, vascular lacunae, and the presence of spindle cells and slit-like vessels. Histological analysis confirmed the diagnosis of Kaposi sarcoma, revealing a proliferation of spindle-shaped endothelial cells forming angulated vascular spaces, with red blood cell extravasation and a mixed inflammatory infiltrate. Conclusions: Non-invasive imaging tools, including dermoscopy, D-OCT, and LC-OCT, have emerged as valuable adjuncts in the diagnosis and monitoring of KS. These techniques enable in vivo assessment of vascular architecture and tissue morphology, enhancing clinical decision-making while reducing the need for immediate biopsy. Dermoscopy reveals polychromatic vascular features, such as the rainbow pattern, while D-OCT and LC-OCT provide high-resolution insights into vascular proliferation, tissue heterogeneity, and cellular morphology. Dermoscopy, dynamic OCT, and LC-OCT represent promising non-invasive diagnostic tools for the assessment of Kaposi sarcoma. These technologies provide detailed morphological and vascular information, enabling earlier diagnosis and more personalized management. While histopathology remains the gold standard, non-invasive imaging offers a valuable complementary approach for diagnosis and follow-up, particularly in complex or atypical presentations. Ongoing research and technological refinement are essential to improve accessibility and clinical applicability. Full article

Background/Objectives: Orthostatic intolerance is prevalent in patients with myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS) and is caused by an abnormal reduction in cerebral blood flow (CBF). In healthy controls (HCs), CBF is regulated complexly, and cardiac output (CO) is an important determinant of CBF. A review in HC showed that a 30% reduction in CO results in a 10% reduction in CBF. In contrast, we showed in ME/CFS patients with a normal HR (HR) and blood pressure response during a tilt test that CO and CBF decreased to a similar extent. The relation between CO and CBF in ME/CFS patients with postural orthostatic tachycardia syndrome (POTS) is unknown. Therefore, the aim of this study is to assess the relation between CBF and CO, in ME/CFS patients with POTS. The methods used in this retrospective study analyze this relation in a large group of patients. We also analyzed the influence of clinical data. A total of 260 ME/CFS patients with POTS underwent tilt testing with measurements of HR, BP, CBF, CO, and end-tidal PCO₂. We measured CBF using extracranial Doppler flow velocity and vessel diameters obtained with a General Electric echo system, and suprasternal aortic flow velocities were measured using the same device. We recorded end-tidal PCO₂ using a Nonin Lifesense device. Results: End-tilt HR and the HR increase were significantly higher in the patients with a %CO reduction ≥ −15% than in the other group. End-tilt CO was higher and the %CO reduction was lower in patients with %CO reduction ≥ −15% than in the other group. CBF data (supine, end-tilt and the %CBF reduction) were not different between the two patient groups. The use of HR increases and %SV reductions were not as discriminative as the %CO reduction. Conclusions: In ME/CFS patients with POTS during tilt testing with measurements of both the CO and the CBF, two different patterns were observed: (1) appr. two-thirds of patients had an almost 1:1 relation between the %CBF reduction and the %CO reduction. (2) Appr. one-third of patients showed a limited reduction in CO together with a substantial increase in HR. In these patients, there was no relation between the CO and CBF reduction. These data suggest the presence of a hyperadrenergic response. Full article

Contrast-enhanced US (CEUS) is now widely used to observe the hemodynamics of the liver. The CEUS diagnosis mainly consists of evaluating hepatic artery and portal vein flow changes in liver diseases, but it has not been widely used for the diagnosis of hepatic venous (HV) abnormalities in the clinical setting. For this background, this review tried to reconsider this problem. In short, observing HV CEUS findings, especially HV transit time, serves to largely narrow the differential diagnosis and increase the diagnostic confidence of the CEUS. However, diagnosing HV CEUS diagnosis in a wide range of liver diseases requires understanding of vascular anatomy of the upper abdomen and vascular structure of each disease. Additionally, interpreting CEUS findings of HCC should be prudent, because its drainage vessels change according to the histological progression, from the HV to the portal vein. Thus, the most important way of making use of the CEUS information is interpreting it in conjunction with the clinical data. Full article

For decades, endovascular embolization (EE) has been a common technique for the treatment of several vascular abnormalities where the affected vessel is occluded using biocompatible embolic agents. In this work, we developed a NIPAAm-based temperature responsive, dual-crosslinking biocompatible and non-toxic injectable hydrogel system as a liquid embolic agent for EE. The swelling and mechanical properties of the hydrogel were tuned and optimized for its in vivo application. The in vivo study was carried out with nine swine models, including three animals for exploratory study and six animals for acute confirmatory study for the occlusion of surgically created aneurysm and rete mirabile. The polymer hydrogel was delivered into the vascular malformation sites using a catheter guided by angiography. After the injection, the liquid embolic agent was transformed into a solid implant in situ via cross-linking through chemical and thermal processes. During the exploratory study, it was observed that one of the three aneurysms and all the RMs were occluded. During the acute confirmatory study, all the aneurysms and the RMs of six animals were successfully occluded. Overall, our study presents the construction and characterization of a novel injectable hydrogel system capable of successfully occluding vascular malformation in large animals. In the future, after further modification and validation, this material may be used as a liquid embolic agent in clinical studies. Full article

Coronary artery fistulas (CAFs) are rare congenital anomalies, presenting in 0.05–0.9% of cases, characterized by an aberrant connection between a coronary artery and a cardiac chamber or great vessel. Clinical manifestations can include heart failure, myocardial ischemia due to coronary steal, arrhythmias, or infective endocarditis. We report a case of a 39-year-old man initially evaluated in 2016 for peripheral edema and suspected right ventricular (RV) abnormality. Earlier assessments indicated a left anterior descending (LAD) coronary artery–RV fistula, but initial catheterization was nondiagnostic. Transthoracic echocardiography (TTE) revealed a dilated left coronary artery (LCA) and an RV apex aneurysm, confirmed by CT and coronary angiography, showing a 14 mm LAD fistula with large aneurysmal sacs (45.6 × 37.3 mm). Cardiac MRI demonstrated a tortuous LAD fistula draining into RV aneurysmal sacs with preserved biventricular function. Surgical intervention was recommended, but the patient declined and was lost to follow-up until 2022, being asymptomatic. Re-evaluation showed progression in aneurysm size (47 × 45 mm and 16 × 18 mm) without ventricular functional change. Follow-up TTE in 2023 indicated stable findings. This case emphasizes the necessity of multimodal imaging (TTE, CT, MRI, angiography) for CAF diagnosis and management planning. Given the variability in CAF presentation and outcomes, individualized management—including surgical, percutaneous, or conservative strategies—is crucial. Persistent follow-up is essential for monitoring potential complications and guiding treatment, even in asymptomatic patients refusing intervention. Full article