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Abstract
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder that causes vascular malformations throughout the body. The most prevalent and accessible of these lesions are found throughout the skin and mucosa, and often rupture causing bleeding and anemia. A recent increase in potential HHT treatments have created a demand for quantitative metrics that can objectively measure the efficacy of new and developing treatments. We employ optical coherence tomography (OCT)—a high resolution, non-invasive imaging modality in a novel pipeline to image and quantitatively characterize dermal HHT lesion behavior over time or throughout the course of treatment. This study is aimed at detecting detailed morphological changes of dermal HHT lesions to understand the underlying dynamic processes of the disease. We present refined metrics tailored for HHT, developed from a pilot study using 3 HHT patients and 6 lesions over the course of multiple imaging dates, totalling to 26 lesion images. Preliminary results from these lesions are presented in this paper alongside representative OCT images. This study provides a new objective method to analyse and understand HHT lesions using a minimally invasive, accessible, cost-effective, and efficient imaging modality with quantitative metrics describing morphology and blood flow.
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1 Ryerson University, Deparment of Electrical, Computer, and Biomedical Engineering, Toronto, Canada (GRID:grid.68312.3e) (ISNI:0000 0004 1936 9422)
2 Ryerson University, Deparment of Electrical, Computer, and Biomedical Engineering, Toronto, Canada (GRID:grid.68312.3e) (ISNI:0000 0004 1936 9422); Nanjing University of Science and Technology, Department of Optical Engineering, Nanjing, China (GRID:grid.410579.e) (ISNI:0000 0000 9116 9901)
3 Sunnybrook Research Institute, Physical Sciences Platform, Hurvitz Brain Sciences Research Program, Toronto, Canada (GRID:grid.17063.33) (ISNI:0000 0001 2157 2938)
4 University of Toronto, Toronto HHT Centre, Division of Respirology, Department of Medicine, St. Michael’s Hospital, Toronto, Canada (GRID:grid.17063.33) (ISNI:0000 0001 2157 2938)
5 University of Toronto, Division of Dermatology, Toronto, Canada (GRID:grid.17063.33) (ISNI:0000 0001 2157 2938)
6 University of California, San Francisco, Department of Neurological Surgery, San Francisco, USA (GRID:grid.266102.1) (ISNI:0000 0001 2297 6811)
7 St. Michael’s Hospital, Li Ka Shing Knowledge Institute, Toronto, Canada (GRID:grid.415502.7)
8 Ryerson University, Deparment of Electrical, Computer, and Biomedical Engineering, Toronto, Canada (GRID:grid.68312.3e) (ISNI:0000 0004 1936 9422); Nanjing University of Science and Technology, Department of Optical Engineering, Nanjing, China (GRID:grid.410579.e) (ISNI:0000 0000 9116 9901); University of Toronto, Department of Surgery, Division of Neurosurgery, Sunnybrook Health Sciences Centre, Toronto, Canada (GRID:grid.17063.33) (ISNI:0000 0001 2157 2938)
9 Washington University School of Medicine, St. Louis, USA (GRID:grid.4367.6) (ISNI:0000 0001 2355 7002)
10 HHT Foundation, Monkton, USA (GRID:grid.4367.6)
11 Augusta University, Division of Pulmonary, Critical Care, and Sleep Medicine, Augusta, USA (GRID:grid.410427.4) (ISNI:0000 0001 2284 9329)
12 Yale University School of Medicine, Yale HHT Center, New Haven, USA (GRID:grid.47100.32) (ISNI:0000000419368710)
13 Mayo Clinic, Division of Pulmonary and Critical Care Medicine, Rochester, USA (GRID:grid.66875.3a) (ISNI:0000 0004 0459 167X)
14 University of North Carolina at Chapel Hill, Division of Hematology and Oncology, Department of Medicine, Chapel Hill, USA (GRID:grid.10698.36) (ISNI:0000000122483208)
15 University of Toronto, Division of Neuroradiology, Department of Medical Imaging, Toronto Western Hospital, Toronto, Canada (GRID:grid.17063.33) (ISNI:0000 0001 2157 2938)
16 Barrow Neurological Institute, Department of Neurosurgery, Phoenix, USA (GRID:grid.427785.b) (ISNI:0000 0001 0664 3531)
17 Johns Hopkins University School of Medicine, Department of Neurosurgery, Baltimore, USA (GRID:grid.21107.35) (ISNI:0000 0001 2171 9311)
18 St. Antonius Hospital, Department of Pulmonology, Nieuwegein, The Netherlands (GRID:grid.415960.f) (ISNI:0000 0004 0622 1269)
19 Duke University, Molecular Genetics and Microbiology, Durham, USA (GRID:grid.26009.3d) (ISNI:0000 0004 1936 7961)
20 David Geffen School of Medicine at UCLA, Department of Radiology, Los Angeles, USA (GRID:grid.19006.3e) (ISNI:0000 0000 9632 6718)
21 University of Utah, Department of Radiology, Salt Lake City, USA (GRID:grid.223827.e) (ISNI:0000 0001 2193 0096)
22 Yale University School of Medicine, Radiology and Biomedical Imaging, New Haven, USA (GRID:grid.47100.32) (ISNI:0000000419368710)
23 Hospital for Sick Children, Division of Respiratory Medicine, Department of Pediatrics, Toronto, Canada (GRID:grid.42327.30) (ISNI:0000 0004 0473 9646)
24 Mayo Clinic, Pulmonology and Critical Care Medicine, Scottsdale, USA (GRID:grid.417468.8) (ISNI:0000 0000 8875 6339)
25 University of Alberta, Department of Medicine, Edmonton, Canada (GRID:grid.17089.37) (ISNI:0000 0001 2190 316X)
26 Washington University School of Medicine, St. Louis Children’s Hospital, Edward Mallinkrodt Department of Pediatrics, St. Louis, USA (GRID:grid.416775.6) (ISNI:0000 0000 9953 7617)
27 St. Paul’s Hospital, Respirologist, Pacific Lung Health Centre, Vancouver, Canada (GRID:grid.416553.0) (ISNI:0000 0000 8589 2327)