眼科 ›› 2025, Vol. 34 ›› Issue (6): 447-455.doi: 10.1328 1/i.cnki.issn.1004-4469.2025.06.006

• 论著 • 上一篇    下一篇

视锥细胞营养不良和视锥-视杆细胞营养不良不同病程阶段黄斑区外层视网膜厚度变化的观察

王聪 刘克高 彭晓燕 白凤阁 陈长喜 章征   

  1. 首都医科大学附属北京同仁医院 北京同仁眼科中心 眼科学与视觉科学北京市重点实验室 北京市眼科研究所,北京 100730
  • 收稿日期:2025-07-14 出版日期:2025-11-25 发布日期:2025-11-25
  • 通讯作者: 刘克高,Email: liukegao@aliyun.com

Changes of the macular fovea thickness in outer retina in patients with cone dystrophy and cone-rod dystrophy at different stages

Wang Cong, Liu Kegao, Peng Xiaoyan, Bai Fengge, Chen Changxi, Zhang Zheng   

  1. Beijing Tongren Eye Center, Beijing Institute of Ophthalmology, Beijing Tongren Hospital, Capital Medical University; Beijing Key Laboratory of Ophthalmology and Visual Science, Beijing 100730, China
  • Received:2025-07-14 Online:2025-11-25 Published:2025-11-25
  • Contact: Liu Kegao, Email: liukegao@aliyun.com

摘要: 目的  观察视锥细胞营养不良(cone dystrophy,CD)和视锥-视杆细胞营养不良(cone-rod dystrophy,CRD)患者不同病程阶段黄斑区外层视网膜各层厚度的变化。设计  回顾性病例系列。研究对象  临床确诊的CD患者14例(28眼)、CRD患者21例(42眼)及正常对照者19名(38眼)。方法  采用OCT对所有患者和正常对照者黄斑中心凹视网膜进行分层观察和测量,按OCT上外层视网膜受损的层次将患眼的受损程度分成4级,即G1~G4级。观察不同病程阶段CD和CRD患者视网膜各层的变化,包括黄斑中心凹厚度(CMT)及外层视网膜(OR)、外核层(ONL)、视锥细胞内节的肌样部分(MZ)、椭圆体区(EZ)、视锥细胞外节(OS)、交错区(IZ)、RPE/Bruch膜复合体(RBC)的厚度,并与正常对照者进行比较。主要指标  G1~G4级视网膜CMT、OR、ONL、MZ、EZ、OS、IZ、RBC的厚度。结果  G1级患眼的EZ(12.76±1.15)μm和IZ(16.80±1.38)μm均较对照组的EZ(13.80±0.99)μm和IZ(19.41±1.60)μm薄(P均<0.001),CMT、OR、ONL、MZ、OS和RBC均与对照组无显著差异(P均>0.05)。G2级患眼的OR(96.72±5.82)μm、EZ(12.37±0.61)μm、IZ(15.44±2.21)μm和RBC(15.97±1.42)μm均较对照组的OR(105.62±3.56)μm、EZ、IZ和RBC(17.68±1.39)μm薄(P均<0.001),CMT、ONL、MZ和OS均与对照组无显著差异(P均>0.05)。G3级患眼的CMT(191.26±25.51)μm、OR(80.61±8.40)μm和MZ(22.81±2.34)μm均较对照组的CMT(210.46±13.07)μm、OR和MZ(27.57±2.60)μm薄(P均<0.001),EZ、IZ、OS和RBC均破坏严重而无法分层测量。G4级患眼的CMT(112.56±41.11)μm、OR(56.48±22.88)μm和ONL(56.08±21.54)μm均较对照组的CMT、OR和ONL薄(P均<0.001)。MZ、EZ、OS、IZ和RBC均无法测量。CD患者(14例28眼)均为G1级;CRD患者(21例42眼)在G2~4级。结论  分层测量黄斑区外层视网膜的厚度能够为不同病程阶段的损害程度提供分级依据。早期的CD患者可以出现EZ和IZ的变薄。

关键词: 相干光断层扫描图像, 视网膜电图, 视网膜黄斑中心凹厚度, 视锥细胞营养不良, 视锥-视杆细胞营养不良

Abstract:  Objective  To observe changes of the macular fovea thickness in outer retina layer by layer in patients with cone dystrophy (CD) and cone-rod dystrophy (CRD) at different stages. Design  Retrospective case series. Participants 14 cases (28 eyes) CD, 21 cases (42 eyes) CRD diagnosed clinically and 19 cases (38 eyes) normal control. Methods  Extent of the outer retina damage of all eyes was categorized into 4 grades (G1~G4) based on the extent of damaged layers in the outer retina by optical coherence tomography (OCT). OCT was adopted to measure the macular fovea thickness layer by layer. Layered changes of the macular fovea thickness were observed and compared with normal control in patients with CD and CRD at different stages, included central macular thickness (CMT), outer retina (OR), outer nuclear layer (ONL), myoid zone (MZ), ellipsoid zone (EZ), outer segment (OS), interdigitation zone (IZ), and RPE/Bruch membrane complex (RBC). Main Outcome Measures  The thickness of CMT, OR, ONL, MZ, EZ, OS, IZ and RBC in G1~G4. Results  The EZ (12.76±1.15) μm and IZ (16.80±1.38) μm of G1 eyes were thinner than EZ (13.80±0.99) μm and IZ (19.41±1.60) μm of the control group (all P<0.001). There was no significant difference between CMT, OR, ONL, MZ, OS, RBC and those of the control group (all P>0.05). The OR (96.72±5.82) μm, EZ (12.37±0.61) μm, IZ (15.44±2.21) μm and RBC (15.97±1.42) μm of G2 eyes were thinner than OR (105.62±3.56) μm, EZ, IZ and RBC (17.68±1.39) μm of the control group (all P<0.001). There was no significant difference between CMT, ONL, MZ, OS and those of the control group (all P>0.05). The CMT (191.26±25.51) μm, OR (80.61±8.40) μm and MZ (22.81±2.34) μm of G3 eyes were thinner than CMT (210.46±13.07) μm, OR and MZ (27.57±2.60) μm of the control group (all P<0.001). The EZ, IZ, OS and RBC were severely damaged and could not be measured layer by layer. The CMT (112.56±41.11) μm, OR (56.48±22.88) μm and ONL (56.08±21.54) μm of G4 were thinner than those of the control group (all P<0.001). MZ, EZ, OS, IZ and RBC could not be measured layer by layer. CD patients (14 cases, 28 eyes) were G1, CRD patients (21 cases, 42 eyes) were G2~4. Conclusion Layered measurement of the macular fovea thickness in outer retina can provide a basis for grading the extent of damage at different stages. thinned EZ and IZ may occur in early CD. 

Key words: Optical coherence tomography, Electroretinogram, Central macular thickness, Cone dystrophy, Cone-rod dystrophy