Skin and Connective Tissue Diseases

All Conditions

Heart and Blood Diseases

Neoplasms

Diseases and Abnormalities at or Before Birth

Rosacea

Telangiectasis

Hemangioma

Hemangioma, Capillary

Port-Wine Stain

Skin Diseases

Vascular Diseases

Congenital Abnormalities

Skin Abnormalities

Neoplasms, Vascular Tissue

Neoplasms by Histologic Type

For the experimental group, the laser surgeon will be exposed to the OCT measurements and will select laser settings and determine treatment parameters based on the measurements.

For the control group, the laser surgeon will not be exposed to the OCT measurements and will select laser settings and determine treatment parameters based on standard of care, intuition, and experience.

Skin imaging to determine vascular characteristics such as plexus depth, vessel diameter, and vessel density.

Optical Coherence Tomography

Kristen M Kelly, MD

The purpose of this study is to obtain information (such as lesion depth, depth of the most superficial part of the lesion, and the size and density of blood vessels) with the assistance of an imaging device, and use this information to assist in selection of laser settings for the treatment of skin conditions. The imaging modality is called Optical Coherence Tomography (OCT). Multiple laser modalities will be used, including intense pulsed light lasers (BroadBand Light, Profractional Sciton), pulsed dye lasers (Vbeam Perfecta, Candela), long-pulse 755nm lasers (GentleLASE, Candela), Sciton long-pulse 1064nm lasers, and non-ablative and ablative fractional resurfacing lasers (Profractional, Sciton). All of the lasers noted above are the only ones that will be used in this study. These lasers have 510k clearance and are being used as per their approved indications in this study. The choice of laser type is based on the skin lesion and is recommended by the physician, and the subjects who are going to enroll in this study will already be planned to undergo laser treatment as a standard of care for their condition. This is a pilot study that will explore the utility of skin imaging in guiding the laser treatment of skin lesions.

The purpose of this research is to assist laser treatment of skin conditions by imaging the skin to obtain information about lesional skin. This is a pilot study that will explore the utility of skin imaging in guiding the laser treatment of skin lesions. This study will utilize Optical Coherence Tomography (OCT). Data acquisition of the skin will guide laser treatment by providing information on skin lesion characteristics.

There have been shown to be many applications for OCT. This imaging modality has been used to examine normal skin, burn scars, hemangiomas, nevus flammeus, fibrosarcomas, rosacea, and telangiectasias. Skin conditions to be studied include vascular lesions, scars, and inflammatory conditions. Normal and lesional skin will be assessed.

Currently, laser settings are selected without the assistance of imaging. Imaging with OCT provides more information about the characteristics of the skin lesion (such as lesion depth, depth of the most superficial part of the lesion, and the size and density of blood vessels), which could allow for more informed selection of laser settings to treat individual skin lesions. OCT has been used to examine laser treatments as well, but the proposed protocol would include the use of OCT to examine the stated skin lesions above before and after laser treatment in order to compare to historical/prospective controls that did not undergo OCT imaging. The goal of this study is to optimize laser treatment with the assistance of OCT and guide future laser treatments.

OCT is an imaging modality that uses light to image turbid media such as living tissues, and has been successfully used to generate high resolution (\~10 micron) cross-sectional images of tissue microstructure in the human retina, skin, gastrointestinal tract, and genitourinary tract. OCT systems are now commercially available for ophthalmic and dermatologic use, and there are several clinical reports on the use of OCT in the vascular system and aero digestive tract, Ophthalmology, Pleural disorder, Neural tissue, Aneurysm healing, and Oral Pathology. This device focuses low power non-laser broad band infrared light onto tissue and does not involve input of significant amounts of energy into the subject; no temperature rise occurs. Because the wavelength of light used for imaging does not have adverse tissue effects, there is no risk.

Imaging with OCT provides information about the characteristics of the skin lesion (such as lesion depth, depth of the most superficial part of the lesion, and the size and density of blood vessels). OCT poses no known risks to the patients.

A multitude of studies have been conducted that validate the utility of OCT in imaging many types of skin lesions. Vascular lesions formulate a good portion of skin diseases studied by OCT, which includes rosacea, port-wine stain, hemangiomas, fibrosarcomas, cherry angiomas, and telangiectasias. Other studies have examined imaging of burn scars, the micro-circulation of the skin, and vascular changes with topical medical application. In all of these studies, OCT imaging aided treatment as well as provided a method to assess treatment outcome.

For port-wine stains, OCT has provided information such as vessel diameter and depth, which was discovered to be quite variable in port-wine stains, indicating that tailored laser treatments are likely to improve result. Byers et al. noted that OCT was a robust and non-invasive method for observing longitudinal dynamics of the subcutaneous microcirculation of tumors. Telangiectasias are a prominent feature of rosacea, and OCT has elucidated information about their treatment with intense-pulsed light to simply examine the effect of the treatment on the targeted lesions.

A prospective study on the impact of Optical Coherence Tomography in laser treatment of vascular skin conditions.

For the control group, the laser surgeon would be unaware of OCT measurements of patients in the control group and would need to rely on his/her experience and intuition to determine the laser settings to treat the patient. For the experimental group, the laser surgeon will be exposed to the OCT measurements.

Skin Imaging to Inform Laser Treatments

All co-researchers and research personnel only on this IRB protocol will have access to IPD.

Any adverse effects of treatment such as scarring, blistering, bruising, and scabbing.

Primary outcome variable includes assessment of change in photographed lesion. Two board-certified dermatologists will grade the degree of change of the photographed lesion in quartiles (no change or darkening=0-25, mild lightening=26-50, good lightening=51-75, excellent lightening=76-95, complete resolution=96-100).

University of California, Irvine

Professor of Dermatology

Number of subjects at risk for adverse events is 55. 55 experimental group subjects were consented to this study. However, due to the global pandemic, subjects could not come for study visits, which significantly precluded the study team from following up with the subjects for several months. Therefore, the study was terminated.

For the experimental group, the laser surgeon will be exposed to the OCT measurements and will select laser settings and determine treatment parameters based on the measurements.

Optical Coherence Tomography: Skin imaging to determine vascular characteristics such as plexus depth, vessel diameter, and vessel density.

Prospective Study Group

For the control group, the laser surgeon will not be exposed to the OCT measurements and will select laser settings and determine treatment parameters based on standard of care, intuition, and experience.

Optical Coherence Tomography: Skin imaging to determine vascular characteristics such as plexus depth, vessel diameter, and vessel density.

Prospective Control Group

Total

Age, Categorical

Age, Continuous

Sex: Female, Male

Ethnicity (NIH/OMB)

Race (NIH/OMB)

United States

Region of Enrollment

55 experimental group subjects were consented to this study. However, due to the global pandemic, subjects could not come for study visits, which significantly precluded the study team from following up with the subjects for several months. Therefore, the study was terminated. The preoperative photographs could not be compared to the postoperative photographs since the subjects could not be followed.

Kristen Kelly, MD

55 experimental group subjects were consented to this study. However, due to the global pandemic, subjects could not come for study visits, which significantly precluded the study team from following up with the subjects for several months. Therefore, the study was terminated. Although we have preoperative photographs, we did not have postoperative photographs of the subjects because we were not able to see them in followup.

Assessment of Change in Photographed Lesion

55 experimental group subjects were consented to this study. However, due to the global pandemic, subjects could not come for study visits, which significantly precluded the study team from following up with the subjects for several months. Therefore, the study was terminated.

Number of Participants With Scarring, Blistering, Bruising, and Scabbing

Overall Study

Symptoms and General Pathology

Digestive System Diseases

Respiratory Tract (Lung and Bronchial) Diseases

Ear, Nose, and Throat Diseases

Nervous System Diseases

Blood and Lymph Conditions

Rare Diseases

Hemorrhage

Gastrointestinal Hemorrhage

Vascular Malformations

Epistaxis

Intracranial Arteriovenous Malformations

Arteriovenous Malformations

Telangiectasia, Hereditary Hemorrhagic

Gastrointestinal Diseases

Cardiovascular Abnormalities

Nose Diseases

Respiratory Tract Diseases

Otorhinolaryngologic Diseases

Signs and Symptoms, Respiratory

Hemostatic Disorders

Blood Coagulation Disorders

Hemorrhagic Disorders

Hematologic Diseases

Cerebrovascular Disorders

Brain Diseases

Central Nervous System Diseases

Central Nervous System Vascular Malformations

Nervous System Malformations

Intracranial Arterial Diseases

Intracranial Arteriovenous Malformation

Hereditary Hemorrhagic Telangiectasia

Those diagnosed with HHT based on the Curacao diagnostic criteria or genetic testing

Melissa A Dickey, MSN

Nolie Krock, MSc

Sayeh Jafari

Victoria Rueda

Justin McWilliams, MD

Lucas Cusumano, MD

Lindsay Park, MS

Steven Hetts, MD

Miles Conrad, MD

Johan Allingmon

Peter Hountras, MD

Katharine Henderson

Jeffrey Pollak, MD

Melissa James, RN

James Gossage, MD

Madeleine Macy, BS

Carolena Othman, MS

Josanna Rodriguez-Lopez, MD

Greg Schwichtenberg

Sue Ann Donlinger

Vivek Iyer, MD

Melissa Beasley, AA

Kristine Kempf

Murali Chakinala, MD

Kelsey Weddig, RN, BSN

Charles Murphy, MD

Karen Smith, RN

Kristi Kirkland

Raj Kasthuri, MBBS

Lori Russell, RN

Eleanor Lottsfeldt, BA

Mark Chesnutt, MD

Claire Kaufman, MD

Ala Streater, BS

Isaac Elysee, MS

Theodore Drivas, MD

Mhd Wisam Alnablsi, MD

John Battaile, MD

An Lu, MD

Cassidy Sion, RN, BSN

Kevin Whitehead, MD

The Comprehensive HHT Outcomes Registry of the United States (CHORUS) is an observational registry of patients diagnosed with Hereditary Hemorrhagic Telangiectasia (HHT). The purpose of this study is to better understand HHT, the symptoms and complications it causes, and the impact the disease has on people's lives. The investigators will collect long-term information about the participant, allowing us to understand how the disease changes over time, and what factors can influence those changes. Ultimately, this should help improve treatments for the disease.

Another important goal of the study is to provide a way to contact people to participate in future clinical trials and other research. The registry will be a centralized resource for recruitment for clinical trials. People in the registry will not be obligated to join any of these additional studies, but if interested, can agree to be contacted if they may be eligible for a study.

Participants will:

* Be asked to provide permission to collect information from their medical records, including things like demographic information, diagnosis information, family history, test results, treatment information, symptoms, complications, lifestyle and other relevant medical information.
* Be asked study-related questions by phone or at a clinic visit.
* Be asked study-related questions every year after enrollment for up to 10 years or until the study ends. A member of the study team will communicate with participants by phone or at clinic visits to collect information regarding any changes to their health over the previous year/s including new test results, treatment information, symptoms, and complications from HHT.

The Comprehensive HHT Outcomes Registry of the United States (CHORUS) is a research initiative led by the HHT Foundation International, Inc. ("Cure HHT"). The study focuses on Hereditary Hemorrhagic Telangiectasia (HHT), a rare genetic disorder characterized by the development of abnormal blood vessels in various organs of the body, including the brain, spine, lungs, liver, GI tract, skin, nasal mucosa, and oral cavity. The prevalence of HHT is estimated to be 1 in 5000, affecting children and adults. These abnormal blood vessels can lead to acute and chronic bleeding, stroke, heart failure, and death. Treatments are currently predominately limited to managing complications, while approximately 90% of adults have ongoing symptoms, despite the best surgical and medical therapies. With recent drug developments related to angiogenesis, there is hope for effective novel therapies.

A natural history registry for HHT has significant implications for improving the understanding and management of this rare genetic disorder. The purpose of this study is to better understand HHT, the symptoms and complications it causes ("outcomes"), and to understand how the disease impacts people's lives. The investigators hypothesize that a natural history registry for HHT will improve our understanding of the disease, lead to better management of patients, and ultimately, contribute to developing novel therapies to treat this disease.

The investigators will collaborate with multiple HHT Centers of Excellence across the U.S. to establish a comprehensive registry of HHT patients. The study aims to enroll approximately 10,000 HHT patients over a 10-year period. Longitudinal data will be collected both retrospectively and prospectively, with a focus on increasing the understanding of this rare disease, accelerating the development of new diagnostic and treatment options, and working collaboratively with clinicians who care for individuals with HHT to identify and address gaps in the system of care, especially those from underserved populations.

The study aims to serve as a centralized resource for future clinical trials and research in HHT. Data security and confidentiality are prioritized, and participants have the option to withdraw from the study at any time. The study is funded by the U.S. Department of Health Resources and Service Administration (HRSA) through a grant awarded to Cure HHT.

Comprehensive HHT Outcomes Registry of the United States (CHORUS)

CHORUS: Comprehensive HHT (Hereditary Hemorrhagic Telangiectasia) Outcomes Registry of the United States

Utilizing the "HHT Baseline Assessment Scale," this measure focuses on recruiting a diverse cohort of HHT patients across North America. Comprehensive baseline clinical, demographic, and lifestyle data will be collected and entered into CHORUS. The data, aggregated as mean change from baseline using the scale, will serve as a foundational resource for future clinical translational studies, addressing the urgent need for natural history data in HHT.

Utilizing the "HHT Clinical Outcomes Scale," this measure aims to prospectively and longitudinally assess the clinical outcomes of HHT patients. Determinants such as demographic factors, environmental influences, lifestyle choices, comorbidities, medications, HHT-genotype, and organ vascular malformations (VMs) will be measured. The data, aggregated as mean change from baseline using the scale, will enhance understanding and accelerate the development of new diagnostic and treatment options for HHT.

Description: Utilizing the "HHT Clinical Outcomes Scale," this measure aims to prospectively and longitudinally assess severe complications in HHT. This measure focuses on measuring rates of severe complications in HHT. Determinants of these complications will be characterized, and the data will be aggregated as mean change from baseline using the index, providing valuable insights into the disease progression.

Description: Utilizing the "Epistaxis Severity Scale," this measure aims to characterize epistaxis in adults with HHT, considering aspects such as frequency, duration, intensity, and variability. Outcome determinants related to epistaxis will be elucidated, and the data will be aggregated as mean change from baseline using the scale to inform comprehensive patient care.

Utilizing the "Organ VMs Assessment Tool," this measure involves the prospective measurement of the development and growth of organ vascular malformations (VMs) in HHT patients over time. Outcome determinants related to these VMs will be investigated, and the data will be aggregated as mean change from baseline using the tool, contributing to a better understanding of disease progression.

Utilizing the "HHT Treatment Outcomes Scale," this measure involves the prospective measurement of treatment outcomes for HHT and its clinical manifestations. Treatment outcome determinants will be characterized, and the data will be aggregated as mean change from baseline using the scale to guide effective management strategies for patients with HHT.

Study title	NCT ID	Conditions	Interventions	Eligibility	Organization
Skin Imaging to Inform Laser Treatments	NCT04336163	Port-Wine Stain Rosacea Telangiectasia Angioma	Optical Coheren...	4 Years -	University of California, Irvine
Comprehensive HHT Outcomes Registry of the United States (CHORUS)	NCT06259292	Hereditary Hemo... Arteriovenous M... Telangiectasia Epistaxis GastroIntestina... Cerebral Arteri... Vascular Malfor...		-	Cure HHT
Comprehensive HHT Outcomes Registry of the United States (CHORUS)	NCT06259292	Hereditary Hemo... Arteriovenous M... Telangiectasia Epistaxis GastroIntestina... Cerebral Arteri... Vascular Malfor...		-	Cure HHT
Skin Imaging to Inform Laser Treatments	NCT04336163	Port-Wine Stain Rosacea Telangiectasia Angioma	Optical Coheren...	4 Years -	University of California, Irvine

Spots Global Cancer Trial Database for telangiectasia