Safety and Tolerability of ZVS101e in Patients With Bietti 's Crystalline Dystrophy

Inclusion Criteria:

1. Willingness to adhere to protocol as evidenced by written informed consent;
2. Patients with clinical diagnosis of Bietti's crystalline dystrophy (BCD) (age ≥ 18years) (the age is based on the time of signing the informed consent form);
3. Genetic test confirmed to carry two pathogenic variants of CYP4V2 and carry nopathogenic mutations of other ophthalmic genetic diseases;
4. Agree to use reliable barrier contraception for 2 year after administration ofZVS101e;
5. The study eye must meet the following requirements: BCVA between 2.3 LogMAR and 0.5LogMAR (including 2.3 LogMAR and 0.5 LogMAR); No refractive medium turbidity thataffects fundus examination; Visible photoreceptor (outer nuclear) layer on a standardoptical coherence tomography (OCT) scan.

Exclusion Criteria:

1. Lack of sufficient viable retinal cell. Specifically, if indirect ophthalmoscopyreveals less than I disc area of retina which is not involved by complete retinaldegeneration, these eyes will be excluded. In addition, in eyes where OCT scans ofsufficient quality can be obtained, areas of retina with thickness measurements lessthan 100 μm, or absence of neural retina, will not be targeted for delivery ofAAV2-hCYP4V2;
2. Existing or pre-existing of choroidal neovascular (CNV) lesions that were secondary toBCD, or other eye conditions interfering with the surgery or the interpretation of theclinical endpoint, in the investigators' opinion;
3. The study eye has been treated with other drugs within 3 months that could affect theevaluation of the investigational drug (such as ranibizumab, bevacizumab, aflibercept,conbercept);
4. The study eye has been treated with the following intraocular procedures: retinaldetachment surgery, vitrectomy;
5. Pre-existing eye conditions that the investigator evaluates could interfere withocular evaluation, preclude surgery, interfere with interpretation of study endpointsor surgical complications (such as glaucoma, high refractive error, diabetesretinopathy or retinal vasculitis );
6. Currently taking or may require systemic medications that can cause ocular toxicity,such as psoralen, risedronate, or tamoxifen;
7. Patient with allergic constitution (such as those allergic to two or more drugs andfood);
8. Those with the following laboratory abnormalities which are clinically significant: Liver function: chronic liver disease, ALT increased >3 times the upper limit ofnormal; With uncontrolled hypertension, mean systolic blood pressure ≥ 160 mmHg ormean diastolic blood pressure ≥ 100 mmHg; With uncontrolled diabetes, HbA1c>10%;Patients with abnormal coagulation function (prothrombin time ≥ upper limit of normal (3 seconds' longer), activated partial thromboplastin time ≥ upper limit of normal (10seconds' longer)); Serum virology test: Active hepatitis B, hepatitis C virus antibody (HCV-Ab), human immunodeficiency virus antibody (HIV-Ab) or syphilis antibodypositive; Abnormality of tumor markers (alpha fetoprotein, carcinoembryonic antigen,CA125 carbohydrate antigen, CA153 carbohydrate antigen, CA199 carbohydrate antigen)
9. Having any past or present medical history that may affect the safety of the trial orthe in vivo process of the drug, especially the medical history of cardiovascular,hepatic, renal, endocrine, gastrointestinal, pulmonary, neurological, hematological,oncologic, immunological or metabolic disorders and others that are thought clinicallysignificant by the investigator, such as diabetes, severe cardiac failure (New YorkHeart Association Class III and IV);
10. Participation in any medicine or medical device clinical trials within 3 months priorto enrollment;
11. Neutralizing antibodies to rAAV> 1:1000 by immunologic test;
12. For females in pregnancy or lactation period;
13. Any other conditions which leads the investigator to determine the participant isunsuitable for this study.