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Follow up Study on Visual Prognosis and Complications of Ectopia Lentis Lens

Recruiting
Conditions
Ectopia Lentis
Dislocation, Lens
Registration Number
NCT05654025
Lead Sponsor
Zhongshan Ophthalmic Center, Sun Yat-sen University
Brief Summary

This is a single-centre, observational cohort study with the following objectives: Firstly, the investigators aim to evaluate the vision-related quality of life and mental health status in congenital ectopia lentis patients. Secondly, the investigators target to investigate the correlations between genotype and clinical phenotype in patients of congenital ectopia lentis. Thirdly, to explore the metabolic profiles in the aqueous humor (AH) of patients with congenital ectopia lentis. Lastly, this study aims to explore the longitudinal changes of ocular biological parameters and complications in patients with postoperative congenital ectopia lens in the Chinese population.

Detailed Description

Congenital ectopia lens is a rare ophthalmic disease that seriously harms the physical and mental health of patients. Surgery is the only definite treatment at present. However, the changes in vision-related quality of life, mental health status, ocular biological parameters postoperative congenital dislocation of lens have not been systematically studied in the Chinese population.

In this trial, the investigators aim to evaluate the vision-related quality of life and mental health status in congenital ectopia lentis patients, investigate the correlations between genotype and clinical phenotype in patients of congenital ectopia lentis, explore the metabolic profiles in the aqueous humor (AH) of patients with congenital ectopia lentis, and explore the longitudinal changes of ocular biological parameters and complications in patients with postoperative congenital ectopia lens in the Chinese population.

Recruitment & Eligibility

Status
RECRUITING
Sex
All
Target Recruitment
604
Inclusion Criteria
  • Diagnosed with congenital lens dislocation and age ≥ 3 years old.
  • Agree to participate in this study with written informed consent from patients or legal guardians.
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Exclusion Criteria
  • History of ophthalmic trauma or other ophthalmic surgeries.
  • Combined with other ophthalmic diseases such as primary glaucoma, uveitis and corneal disease.
  • Patients who could not cooperate in the examinations.
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Study & Design

Study Type
OBSERVATIONAL
Study Design
Not specified
Primary Outcome Measures
NameTimeMethod
Change of best corrected visual acuityNon-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation

Best corrected visual acuity (BCVA) is evaluated with an ETDRS chart at each visit.

Incidence of complicationsNon-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation.

Estimate the incidence of complications for both non-surgical group and surgical group.

Secondary Outcome Measures
NameTimeMethod
Central cornea endothelial cell lossPreoperation, and 3 months, and 1, 2, 3 years postoperation

Central cornea endothelial cell loss will be calculated with endokeratoscope in surgical group.

Change of axial lengthNon-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation.

Axial length will be evaluated with ZEISS IOLMaster 700 at each visit.

Anterior chamber angle.Non-surgical group is assessed at first visit, and 1, 2, 3 years after enrollment. Surgical group will be assessed preoperation, 3 months, and 1, 2, 3 years postoperation.

Anterior chamber angle will be assessed using Tomey Casia 2 anterior segment optical coherence tomography (OCT).

Tilt and eccentricity of intraocular lens3 months, and 1, 2, 3 years postoperation

Tilt and eccentricity of intraocular lens was evaluated with Pentacam in surgical group.

Aortic root diameterNon-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation

Aortic root diameter will be measured using echocardiography.

Vision-related quality of lifeNon-surgical group is evaluated at first visit and 3 years after enrollment. Surgical group is evaluated at preoperation and 3 years postoperation.

Vision-related quality of life will be assessed with the Pediatric Eye Questionnaire (PedEyeQ).

High order aberrationsNon-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation

High order aberrations will be assessed with Nidek OPD-Scan III at each visit.

The state of zonulesNon-surgical group is assessed at first visit, and 1, 2, 3 years after enrollment. Surgical group will be assessed preoperation.

The state of zonules will be assessed using ultrasound biomicroscopy.

Body mass index (BMI)Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation.

Body mass index (BMI) is a person's weight in kilograms divided by the square of height in meters. The normal range for the Chinese population is 18.5 to 23.9. A high BMI can indicate high body fatness.

Metacarpophalangeal joint lengthNon-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation.

Metacarpophalangeal joint length will be measured using palmar radiograph.

Intraocular pressureNon-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation, 3 months, and 1, 2, 3 years postoperation.

Intraocular pressure with non-contact-tonometer at each visit.

Aortic Root (Sinuses of Valsalva) Z-score, adjusted by body-surface-area (Z-score)Non-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation

Z-Score will be calculated using the Marfan foundation's calculator (https://marfan.org/dx/zscore-children/). Normal Z-score range from -2 to 2. A dilated aortic root is defined as a Z-score ≥2.0. A larger Z-score is associated with an increased risk of aortic complications such as dissection, rupture, and valvular regurgitation.

Incidence of valvular heart diseaseNon-surgical group is evaluated at first visit, and 1, 2, 3 years after enrollment. Surgical group is evaluated at preoperation and 1, 2, 3 years postoperation

The incidence of valvular heart disease (VHD) is defined as the ratio of patients diagnosed with VHD to the total number of patients enrolled.

Choriocapillaris flow deficitsNon-surgical group is evaluated at first visit and 3 years after enrollment. Surgical group is evaluated at preoperation and 3 years postoperation.

Choriocapillaris flow deficits will be measured with optical coherence tomography angiography (OCTA) images.

Genetic mutation state of patientsPreoperation

Draw blood to detect the exact gene mutations in patients via whole-exome sequencing.

Choroidal thicknessNon-surgical group is evaluated at first visit and 3 years after enrollment. Surgical group is evaluated at preoperation and 3 years postoperation.

Choroidal thickness will be measured with optical coherence tomography (OCT) images.

Trial Locations

Locations (1)

Guangdong Provincial Clinical Research Center for Ocular Diseases, State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangdong Provincial Key Laboratory of Ophthalmology and Visual Science

🇨🇳

Guangzhou, Guangdong, China

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