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

Ectopia Lentis Clinical Trial

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT05654025
• Other study ID #: 2022KYPJ207
• Status: Recruiting
• Start date: December 5, 2022
• Est. completion date: December 2027

Study information

• Verified date: December 2022
• Source: Zhongshan Ophthalmic Center, Sun Yat-sen University
• Contact: Dangying Zheng, M.D
• Phone: 020-87330478
• Email: zhengdyy[@]163.com
• Is FDA regulated: No
• Study type: Observational

Clinical Trial 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.

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 information / eligibility

• Status: Recruiting
• Enrollment: 604
• Est. completion date: December 2027
• Est. primary completion date: December 2027
• Accepts healthy volunteers: Accepts Healthy Volunteers
• Gender: All
• Age group: 3 Years and older

Eligibility

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.

Related Conditions & MeSH terms

• Ectopia Lentis
• Lens Subluxation

Locations

Country	Name	City	State
China	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

Sponsors (1)

Lead Sponsor	Collaborator
Zhongshan Ophthalmic Center, Sun Yat-sen University

Country where clinical trial is conducted

China, 

References & Publications (12)

Chen H, Ng KY, Li S, Jin G, Cao Q, Lian Z, Luo X, Ding X, Zheng D. CHARACTERISTICS OF THE FOVEAL MICROVASCULATURE IN CHILDREN WITH MARFAN SYNDROME: An Optical Coherence Tomography Angiography Study. Retina. 2022 Jan 1;42(1):138-151. doi: 10.1097/IAE.00000 — View Citation

Chen T, Chen J, Jin G, Zhang M, Chen Z, Zheng D, Jiang Y. Clinical Ocular Diagnostic Model of Marfan Syndrome in Patients With Congenital Ectopia Lentis by Pentacam AXL System. Transl Vis Sci Technol. 2021 Jun 1;10(7):3. doi: 10.1167/tvst.10.7.3. — View Citation

Chen Z, Chen T, Zhang M, Chen J, Deng M, Zheng J, Lan LN, Jiang Y. Fibrillin-1 gene mutations in a Chinese cohort with congenital ectopia lentis: spectrum and genotype-phenotype analysis. Br J Ophthalmol. 2022 Dec;106(12):1655-1661. doi: 10.1136/bjophthal — View Citation

Fuchs J, Rosenberg T. Congenital ectopia lentis. A Danish national survey. Acta Ophthalmol Scand. 1998 Feb;76(1):20-6. doi: 10.1034/j.1600-0420.1998.760105.x. — View Citation

Guo D, Jin G, Zhou Y, Zhang X, Cao Q, Lian Z, Guo Y, Zheng D. Mutation spectrum and genotype-phenotype correlations in Chinese congenital ectopia lentis patients. Exp Eye Res. 2021 Jun;207:108570. doi: 10.1016/j.exer.2021.108570. Epub 2021 Apr 16. — View Citation

Jin GM, Fan M, Cao QZ, Lin JX, Zhang YC, Lin JQ, Wang YY, Young CA, Zheng DY. Trends and characteristics of congenital ectopia lentis in China. Int J Ophthalmol. 2018 Sep 18;11(9):1545-1549. doi: 10.18240/ijo.2018.09.19. eCollection 2018. — View Citation

Kaur K, Gurnani B. Ectopia Lentis. 2023 Jan 16. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. Available from http://www.ncbi.nlm.nih.gov/books/NBK578193/ — View Citation

Lin J, Gong N, Cao Q, Zhou Y, Cai Y, Jin G, Young CA, Yang J, Wang Y, Zheng D. What hinders congenital ectopia lentis patients' follow-up visits? A qualitative study. BMJ Open. 2020 Mar 12;10(3):e030434. doi: 10.1136/bmjopen-2019-030434. — View Citation

Liu L, Li X, Cao Q, Lian Z, Wu J, Jin G, Zheng D. A Modified Knotless Transscleral Intraocular Lens Fixation Technology for Congenital Ectopia Lentis. Ophthalmol Ther. 2023 Feb;12(1):99-110. doi: 10.1007/s40123-022-00586-9. Epub 2022 Oct 15. — View Citation

Meng X, Cao X, Jia Y, Pan J, Du Y, Li X. Sutured Scleral Fixation of Posterior Chamber Intraocular Lens in Children under the Age of 9 with Congenital Ectopia Lentis. Ophthalmic Res. 2021;64(5):837-843. doi: 10.1159/000516324. Epub 2021 Jul 9. — View Citation

Minsel WR, Bommert H, Pieritz R. [Relationship between speech--formal psychotherapeutic signs and results of client-centered discussion psychotherapy]. Z Klin Psychol Psychother. 1972;20(4):303-10. No abstract available. German. — View Citation

Zhang Y, Jin G, Young CA, Cao Q, Lin J, Lin J, Wang Y, Zheng D. Analysis of Corneal Astigmatism before Surgery in Chinese Congenital Ectopia Lentis Patients. Curr Eye Res. 2018 Aug;43(8):972-976. doi: 10.1080/02713683.2018.1470248. Epub 2018 May 23. — View Citation

* Note: There are 12 references in all — Click here to view all references

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Change of best corrected visual acuity	Best corrected visual acuity (BCVA) is evaluated with an ETDRS chart at each visit.	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
Primary	Incidence of complications	Estimate the incidence of complications for both non-surgical group and surgical group.	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.
Secondary	Change of axial length	Axial length will be evaluated with ZEISS IOLMaster 700 at each visit.	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.
Secondary	High order aberrations	High order aberrations will be assessed with Nidek OPD-Scan III at each visit.	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
Secondary	Central cornea endothelial cell loss	Central cornea endothelial cell loss will be calculated with endokeratoscope in surgical group.	Preoperation, and 3 months, and 1, 2, 3 years postoperation
Secondary	The state of zonules	The state of zonules will be assessed using ultrasound biomicroscopy.	Non-surgical group is assessed at first visit, and 1, 2, 3 years after enrollment. Surgical group will be assessed preoperation.
Secondary	Anterior chamber angle.	Anterior chamber angle will be assessed using Tomey Casia 2 anterior segment optical coherence tomography (OCT).	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.
Secondary	Tilt and eccentricity of intraocular lens	Tilt and eccentricity of intraocular lens was evaluated with Pentacam in surgical group.	3 months, and 1, 2, 3 years postoperation
Secondary	Intraocular pressure	Intraocular pressure with non-contact-tonometer at each visit.	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.
Secondary	Aortic root diameter	Aortic root diameter will be measured using echocardiography.	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
Secondary	Aortic Root (Sinuses of Valsalva) Z-score, adjusted by body-surface-area (Z-score)	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.	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
Secondary	Incidence of valvular heart disease	The incidence of valvular heart disease (VHD) is defined as the ratio of patients diagnosed with VHD to the total number of patients enrolled.	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
Secondary	Body mass index (BMI)	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.	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.
Secondary	Metacarpophalangeal joint length	Metacarpophalangeal joint length will be measured using palmar radiograph.	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.
Secondary	Choroidal thickness	Choroidal thickness will be measured with optical coherence tomography (OCT) images.	Non-surgical group is evaluated at first visit and 3 years after enrollment. Surgical group is evaluated at preoperation and 3 years postoperation.
Secondary	Choriocapillaris flow deficits	Choriocapillaris flow deficits will be measured with optical coherence tomography angiography (OCTA) images.	Non-surgical group is evaluated at first visit and 3 years after enrollment. Surgical group is evaluated at preoperation and 3 years postoperation.
Secondary	Genetic mutation state of patients	Draw blood to detect the exact gene mutations in patients via whole-exome sequencing.	Preoperation
Secondary	Vision-related quality of life	Vision-related quality of life will be assessed with the Pediatric Eye Questionnaire (PedEyeQ).	Non-surgical group is evaluated at first visit and 3 years after enrollment. Surgical group is evaluated at preoperation and 3 years postoperation.