Retinal vessel tortuosity as a prognostic marker for disease severity in Fabry disease

Purpose The aim of this case control study was to evaluate the prognostic value of automatically quantified retinal vessel tortuosity from fundus images and vessel density from OCT-A in Fabry disease and to evaluate the correlation of these with systemic disease parameters. Methods Automatically quantified perimacular retinal vessel tortuosity (MONA REVA software), acquired by fundus imaging, and perifoveal retinal vessel density, acquired by optic coherence tomography angiography (OCT-A) were compared between 26 FD patients and 26 controls. Gender and FD phenotype were analyzed to the obtained retinovascular data and correlated to the Mainz severity score index (MSSI) and plasma lyso-Gb3. Results Automatically quantified retinal vessel tortuosity indices of FD patients were significantly lower, reflecting an increased vessel tortuosity, compared to controls (p = 0.008). Males with a classical phenotype showed significantly lower retinal vessel tortuosity indices compared to males with an oligosymptomatic phenotype and females with a classical or oligosymptomatic phenotype (p < 0.001). The retinal vessel tortuosity index correlated significantly with systemic disease severity parameters [global MSSI (r = − 0.5; p < 0.01), cardiovascular MSSI (r = − 0.5; p < 0.01), lyso-Gb3 (r = − 0.6; p < 0.01)]. Conclusion We advocate fundus imaging based automatically quantified retinal vessel tortuosity index over OCT-A imaging as it is a quick, non-invasive, easily assessable, objective and reproducible marker.

A feasible alternative to transfemoral artery access for endovascular revascularization in acute ischemic stroke in cases with difficult vessel anatomy

Endovascular treatment in acute ischemic stroke is usually performed via a transfemoral approach. Catheterization can be problematic in cases with difficult anatomy: unfavorable arch type, vessel tortuosity and ostial stenosis, aorta coarc‑ tation, iliac artery occlusion. The aim of this review is to describe the place of another arterial approach in the manage‑ ment in acute ischemic stroke: direct common carotid

Hirschsprung’s Disease in a Twin Neonate With One Suffering From Multiple Skip Segments

This is a new case, affecting a twin that both with Hirschsprung’s disease, one was suffering from long-segment Hirschsprung’s disease with skip segmented. Our surgeon suspected the absence of abnormal vessel tortuosity in the transitional zone; thus, the appendix was sent for permanent pathology. There were ganglion cells in the colostomy site but no ganglion was found in the appendix. Complete biopsies from different parts of the intestine demonstrated positive ganglion cells in the transverse colon, ascending colon, and the distal ileum, jejunum, and duodenum, and negative ganglion cells in the rectum, sigmoid, descending colon, appendix, and 5 cm far from the ileocecal valve were observed. Therefore, the Kimura procedure was done. The most important key in such cases is the surgeons’ observation during operation.

Characterization of Lenticulostriate Arteries and Its Associations With Vascular Risk Factors in Community-Dwelling Elderly

Lenticulostriate arteries (LSAs) supply blood to important subcortical areas and are, therefore, essential for maintaining the optimal functioning of the brain’s most metabolically active nuclei. Past studies have demonstrated the potential for quantifying the morphology of LSAs as biomarkers of vascular fragility or underlying arteriopathies. Thus, the current study aims to evaluate the morphological features of LSAs, their potential value in cerebrovascular risk stratification, and their concordance with other vascular risk factors in community-dwelling elderly people. A total of 125 community-dwelling elderly subjects who underwent a brain MRI scan were selected from our prospectively collected imaging database. The morphological measures of LSAs were calculated on the vascular skeletons obtained by manual tracing, and the number of LSAs was counted. Additionally, imaging biomarkers of small vessel disease were evaluated, and the diameters of major cerebral arteries were measured. The effects of vascular risk factors on LSA morphometry, as well as the relationship between LSA measures and other imaging biomarkers, were investigated. We found that smokers had shorter (p = 0.04) and straighter LSAs (p &lt; 0.01) compared to nonsmokers, and the presence of hypertension is associated with less tortuous LSAs (p = 0.03) in community-dwelling elderly. Moreover, the middle cerebral artery diameter was positively correlated with LSA count (r = 0.278, p = 0.025) and vessel tortuosity (r = 0.257, p = 0.04). The posterior cerebral artery diameter was positively correlated with vessel tortuosity and vessel length. Considering the scarcity of noninvasive methods for measuring small artery abnormalities in the brain, the LSA morphological measures may provide valuable information to better understand cerebral small vessel degeneration during aging.

How Cerebral Vessel Tortuosity Affects Development and Recurrence of Aneurysm: Outer Curvature versus Bifurcation Type

Background and Purpose Previous studies have assessed the relationship between cerebral vessel tortuosity and intracranial aneurysm (IA) based on two-dimensional brain image analysis. We evaluated the relationship between cerebral vessel tortuosity and IA according to the hemodynamic location using three-dimensional (3D) analysis and studied the effect of tortuosity on the recurrence of treated IA.Methods We collected clinical and imaging data from patients with IA and disease-free controls. IAs were categorized into outer curvature and bifurcation types. Computerized analysis of the images provided information on the length of the arterial segment and tortuosity of the cerebral arteries in 3D space.Results Data from 95 patients with IA and 95 controls were analyzed. Regarding parent vessel tortuosity index (TI; <i>P</i><0.01), average TI (<i>P</i><0.01), basilar artery (BA; <i>P</i>=0.02), left posterior cerebral artery (<i>P</i>=0.03), both vertebral arteries (VAs; <i>P</i><0.01), and right internal carotid artery (<i>P</i><0.01), there was a significant difference only in the outer curvature type compared with the control group. The outer curvature type was analyzed, and the occurrence of an IA was associated with increased TI of the parent vessel, average, BA, right middle cerebral artery, and both VAs in the logistic regression analysis. However, in all aneurysm cases, recanalization of the treated aneurysm was inversely associated with increased TI of the parent vessels.Conclusions TIs of intracranial arteries are associated with the occurrence of IA, especially in the outer curvature type. IAs with a high TI in the parent vessel showed good outcomes with endovascular treatment.

Vascular Patterning as Integrative Readout of Complex Molecular and Physiological Signaling by VESsel GENeration Analysis

The molecular signaling cascades that regulate angiogenesis and microvascular remodeling are fundamental to normal development, healthy physiology, and pathologies such as inflammation and cancer. Yet quantifying such complex, fractally branching vascular patterns remains difficult. We review application of NASA’s globally available, freely downloadable VESsel GENeration (VESGEN) Analysis software to numerous examples of 2D vascular trees, networks, and tree-network composites. Upon input of a binary vascular image, automated output includes informative vascular maps and quantification of parameters such as tortuosity, fractal dimension, vessel diameter, area, length, number, and branch point. Previous research has demonstrated that cytokines and therapeutics such as vascular endothelial growth factor, basic fibroblast growth factor (fibroblast growth factor-2), transforming growth factor-beta-1, and steroid triamcinolone acetonide specify unique “fingerprint” or “biomarker” vascular patterns that integrate dominant signaling with physiological response. In vivo experimental examples described here include vascular response to keratinocyte growth factor, a novel vessel tortuosity factor; angiogenic inhibition in humanized tumor xenografts by the anti-angiogenesis drug leronlimab; intestinal vascular inflammation with probiotic protection by <i>Saccharomyces boulardii</i>, and a workflow programming of vascular architecture for 3D bioprinting of regenerative tissues from 2D images. Microvascular remodeling in the human retina is described for astronaut risks in microgravity, vessel tortuosity in diabetic retinopathy, and venous occlusive disease.

Crossing the Carotid Siphon: Techniques to Facilitate Distal Access in Tortuous Anatomy: 2-Dimensional Operative Video

As capabilities for endovascular treatment of intracranial vascular pathologies continue to expand, the need for access to the distal internal carotid artery with rigid support catheter systems continues to increase. One of the dominant factors limiting this access is patient anatomy, specifically vessel tortuosity. Increased tortuosity of the carotid siphon is a frequently encountered anatomic variant and may complicate endovascular procedures in adults and children.1,2 Failed attempts to navigate the carotid siphon with a distal access catheter carry a risk of vessel injury and treatment failure. For this reason, techniques that aid in supporting safe advancement of a distal access catheter across a tortuous carotid siphon are essential.3,4 In this video, we demonstrate 2 ways in which this may be accomplished. The first technique uses a larger diameter microcatheter, such as the AXS Offset catheter (Stryker, Kalamazoo, Michigan), to increase support for the distal access catheter, while the second uses a buddy wire technique to accomplish this increased support. Both of these techniques can help increase the safety of navigating a tortuous carotid siphon and increase the likelihood of successful treatment. The procedures shown were performed with the informed consent of the patients.