Dislocation, Lens, Ectopia Lentis
Conditions
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.
Interventions
None listed
Sponsors
Zhongshan Ophthalmic Center, Sun Yat-sen University
Study design
Observational model
COHORT
Time perspective
OTHER
Eligibility
Sex/Gender
ALL
Age
3 Years to No maximum
Healthy volunteers
Yes
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.
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.
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Change of best corrected visual acuity | Non-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 complications | Non-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
| Measure | Time frame | Description |
|---|---|---|
| Central cornea endothelial cell loss | Preoperation, and 3 months, and 1, 2, 3 years postoperation | Central cornea endothelial cell loss will be calculated with endokeratoscope in surgical group. |
| The state of zonules | Non-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. |
| 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 lens | 3 months, and 1, 2, 3 years postoperation | Tilt and eccentricity of intraocular lens was evaluated with Pentacam in surgical group. |
| Intraocular pressure | Non-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 diameter | 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 | Aortic root diameter will be measured using echocardiography. |
| 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. |
| Change of axial length | Non-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. |
| 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 length | 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. | Metacarpophalangeal joint length will be measured using palmar radiograph. |
| Choroidal thickness | Non-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. |
| Choriocapillaris flow deficits | Non-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 patients | Preoperation | Draw blood to detect the exact gene mutations in patients via whole-exome sequencing. |
| Vision-related quality of life | Non-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). |
| Incidence of valvular heart disease | 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 | The incidence of valvular heart disease (VHD) is defined as the ratio of patients diagnosed with VHD to the total number of patients enrolled. |
| High order aberrations | Non-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. |
Countries
China
Contacts
Primary ContactDangying Zheng, M.D
Outcome results
None listed