摘要目的：观察慢性进行性眼外肌麻痹(CPEO)和 Kearns-Sayre 综合征(KSS)患者视网膜神经纤维层(RNFL)厚度,分析其影响因素。方法经肌肉病理活检确诊的 CPEO 患者22例纳入研究。其中,男性7例,女性15例。平均年龄(29.09±13.40)岁；平均起病年龄(16.4±10.7岁)。平均病程(11.30±7.30)年。22例患者中,CPEO 患者18例(CPEO 组)；KSS 患者4例(KSS 组)。患者均行频域光相干断层扫描(SD-OCT)检查。选取左眼为受检眼,以视盘中心为圆心,直径为3.45 mm 的环形扫描。扫描分为上方颞侧(ST)、颞上方(TU)、颞下方(TL)、下方颞侧(IT)、下方鼻侧(IN)、鼻下方(NL)、鼻上方(NU)、上方鼻侧(SN)。Pearson 相关分析法分析 RNFL 厚度与患者起病时间、病程的相关性。结果22例患者视盘旁全周平均 RNFL 厚度为(101.5±14.4)μm。ST、TU、TL、IT、IN、NL、NU、SN 平均 RNFL 厚度分别为(136.3±24.1)、(85.4±25.7)、(68.2±11.7)、(128.2±28.7)、(127.3±29.5)、(66.7±16.8)、(70.1±17.6)、(122.9±20.2)μm。KSS 组视盘旁全周及各象限平均 RNFL 厚度均较 CPEO 组低,但差异无统计学意义(P >0.05)。相关性分析结果显示,RNFL 厚度下降与起病年龄无相关性(r =-0.306,P =0.11)；与病程呈负相关(r =-0.518,P =0.03),尤其是鼻侧4个象限 RNFL 厚度与病程呈负相关(IN：r =-0.555,P =0.01；NL：r=-0.630,P =0.00；NU：r =-0.559,P =0.01,SN：r =-0.557,P =0.01)。结论CPEO、KSS 患者 RNFL 厚度差异无统计学意义；RNFL 厚度特别是鼻侧 RNFL 厚度与病程呈负相关。

abstractsObjective To determine the retinal nerve fiber layer thickness (RNFLT)of chronic progressive external ophthalmoplegia (CPEO)and kearns-sayre syndrome (KSS)patients using spectral-domain optical coherence tomography (SD-OCT)and to analyze the potential influence factors for RNFLT of these patients.Methods 18 CPEO and 4 KSS (CPEO with retinitis pigmentosa and cardiac block)patients, all were muscle biopsy confirmed,were included in this study.There were 7 males and 1 5 females,the average age was (29.09 ± 13.40)years,the average onset age was (1 6.4 ± 10.7 )years and the average disease duration was (1 1.30 ± 7.30)years.All the patients underwent SD-OCT examination for the left eye,the peripapillary RNFL thickness was measured using the Spectralis 3.45 mm circle scan protocol.8 quadrants were scanned including superior temporal (ST),temporal upper (TU),temporal lower (TL), inferior temporal (IT),inferior nasal (IN),nasal lower (NL),nasal upper (NU)and superior nasal (SN). The relationship between RNFLT and onset time,disease duration was analyzed by Pearson correlation analysis.Results The average RNFLT of ST,TU,TL,IT,IN,NL,NU,SN in the 22 patients were (136.3±24.1),(85.4±25.7),(68.2±1 1.7),(128.2±28.7),(127.3±29.5),(66.7±1 6.8),(70.1± 1 7.6)μm,respectively.The circumferential average RNFLT was (101.5 ± 14.4 )μm.There was no significant difference between the KSS group and CPEO group (P > 0.05 ). The decrease of the circumferential RNFLT had no relationship with the onset age (r = - 0.306,P = 0.1 1 ),but a negative relationship with the disease course (r=-0.5 18,P =0.03).There were negative relationships between the disease course and RNFLT of 4 nasal quadrants (IN:r=-0.555,P =0.01,NL:r=-0.630,P =0.00 NU:r =-0.559,P =0.01,SN:r =-0.557,P =0.01).Conclusion There is no difference in RNFLT of patients with CPEO and KSS.There is a negative relation between RNFLT (especially RNFLT of 4 nasal quadrants)and disease course.