小切口基质内透镜取出术及飞秒激光LASIK治疗高度近视的临床对比研究

目的　对比研究小切口基质内透镜取出术（ｓｍａｌｌｉｎｃｉｓｉｏｎｌｅｎｔｉｃｕｌｅｅｘｔｒａｃｔｉｏｎ,ＳＭＩＬＥ）及飞秒激光ＬＡＳＩＫ（ｆｅｍｔｏｓｅｃｏｎｄｌａｓｅｒｉｎｓｉｔｕｋｅｒａｔｏｍｉｌｅｕｓｉｓ,ＦＳ-ＬＡＳＩＫ）治疗高度近视患者的手术疗效。方法　４０例８０眼高度近视患者（等效球镜度数为－７．００～－１０．５０Ｄ）根据手术方式不同分为２组,其中ＳＭＩＬＥ组２０例４０眼行ＳＭＩＬＥ,ＦＳ-ＬＡＳＩＫ组２０例４０眼行ＶｉｓｕＭａｘＦＳ-ＬＡＳＩＫ,观察时间点为术后１ｄ、１周、１个月、３个月、６个月。分别观察两组术后裸眼视力、等效球镜度数、最佳矫正视力、角膜地形图形态等。结果　安全性:术后３个月ＳＭＩＬＥ组和ＦＳ-ＬＡＳＩＫ组最佳矫正视力达到术前最佳矫正视力的比例分别为９７．５％（３９／４０）、９５．０％（３８／４０）（Ｐ＞０．０５）,术后最佳矫正视力／术前最佳矫正视力分别为１．１５±０．１２、１．０９±０．１４（Ｐ＞０．０５）。有效性:术后３个月ＳＭＩＬＥ组和ＦＳ-ＬＡＳＩＫ组裸眼视力达到术前最佳矫正视力的比例分别为９５．０％（３８／４０）、８０．０％（３２／４０）（Ｐ＜０．０５）,术后裸眼视力／术前最佳矫正视力分别为１．１１±０．１３、０．９８±０．１４（Ｐ＜０．０５）。可预测性（精确性）:术后３个月ＳＭＩＬＥ组和ＦＳ-ＬＡＳＩＫ组等效球镜在±０．５０Ｄ的比例分别为９５．０％（３８／４０）、７０．０％（２８／４０）（Ｐ＜０．０５）;等效球镜在±０．７５Ｄ的比例分别为９７．５％（３９／４０）、８２．５％（３３／４０）（Ｐ＜０．０５）。稳定性:与术后１周相比,术后１个月、３个月及６个月各时间点ＳＭＩＬＥ组和ＦＳ-ＬＡＳＩＫ组等效球镜度数的差异均无统计学意义（Ｐ＞０．０５）。角膜地形图形态:ＳＭＩＬＥ组和ＦＳ-ＬＡＳＩＫ组角膜地形图都以平滑型最多,分别占８２．５％（３３／４０）、６０．０％（２４／４０）,两组比较差异有统计学意义（Ｐ＜０．０５）。结论　ＳＭＩＬＥ及ＦＳ-ＬＡＳＩＫ治疗高度近视均安全、稳定,而ＳＭＩＬＥ有效性、可预测性、精确性、术后角膜形态均稍好于ＦＳ-ＬＡＳＩＫ,矫正高度近视更值得临床应用。     

多发性硬化及其视觉诱发电位的临床表现

本文报导了１１例多发性硬化（ＭＳ）和它们的视觉诱发电位（ＶＥＰ）。在２２眼中２１眼为ＭＳ视神经炎，１眼视功能正常。２１眼ＭＳ视神经炎的ＶＥＰ全部异常，另１眼ＶＥＰ正常。ＭＳ视神经炎的ＶＥＰ检查结果是：１．ＰＶＥＰ６眼不能记录到波形，１２眼的Ｐ１００成分峰时延迟（１２４．６±９．７ｍｓ，Ｐ＜０．００１）、振幅降低（７．６±２．８μｖ，Ｐ＜０．００１）；２．ＦＶＥＰＰ１的峰时全部延迟（８６．５±８．９ｍｓ，Ｐ＜０．０５），振幅降低（１０．４±６．５μｖ，Ｐ＜０．０５），具有统计学的差异。     

改良飞秒激光和小切口飞秒激光基质内透镜取出术治疗高度近视术后泪膜稳定性研究

目的　对比分析改良飞秒激光基质内透镜取出术（ｒｅｆｏｒｍｉｎｇｆｅｍｔｏｓｅｃｏｎｄｌｅｎｔｉｃｕｌｅｅｘｔｒａｃｔｉｏｎ,Ｒ-ＦＬＥｘ）与小切口飞秒激光基质内透镜取出术（ｓｍａｌｌｉｎｃｉｓｉｏｎｌｅｎｔｉｃｕｌｅｅｘｔｒａｃｔｉｏｎ,ＳＭＩＬＥ）治疗高度近视术后泪膜稳定性的变化。方法　回顾性分析行全飞秒近视矫正手术治疗的高度近视患者４０例８０眼,其中ＳＭＩＬＥ组２０例４０眼,Ｒ-ＦＬＥｘ组２０例４０眼。观察术后１个月、３个月、６个月、１ａ两组患者的干眼症状,包括干涩感、烧灼感、异物感,并依次行基础泪液分泌试验（ＳｃｈｉｒｍｅｒＩｔｅｓｔ,ＳＩｔ）、泪膜破裂时间（ｂｒｅａｋｕｐｔｉｍｅｏｆｔｅａｒｆｉｌｍ,ＢＵＴ）、角膜荧光素染色检查（ｃｏｒｎｅａｌｆｌｕｏｒｅｓｃｅｉｎｓｔａｉｎｉｎｇ,ＦＬ）,并对其泪膜稳定性的变化进行对比分析。结果　干眼症状问卷调查评分:术后１个月ＳＭＩＬＥ组和Ｒ-ＦＬＥｘ组分别为０．３５±０．４８、０．９０±０．６７,差异有统计学意义（Ｐ＜０．０５）;术后３个月、６个月、１ａＳＭＩＬＥ组和Ｒ-ＦＬＥｘ组分别为０．１８±０．３８、０．０８±０．２７、０．０５±０．２２,０．２８±０．４５、０．１０±０．３０、０．１０±０．３０,差异均无统计学意义（均为Ｐ＞０．０５）。两组ＦＬ检查评分:术后１个月ＳＭＩＬＥ组和Ｒ-ＦＬＥｘ组分别为０．８８±０．６９、１．３０±０．６９,差异有统计学意义（Ｐ＜０．０５）;术后３个月、６个月、１ａＳＭＩＬＥ组和Ｒ-ＦＬＥｘ组分别为０．１８±０．３８、０．０８±０．２７、０．０３±０．１６,０．２８±０．４５、０．０５±０．２２、０．０５±０．２２,差异均无统计学意义（均为Ｐ＞０．０５）。ＳＩｔ试验结果:术后１个月、３个月ＳＭＩＬＥ组和Ｒ-ＦＬＥｘ组分别为（８．２０±０．７６）ｍｍ、（８．９５±０．６０）ｍｍ,（６．６５±０．６６）ｍｍ、（８．３０±０．６１）ｍｍ,差异均有统计学意义（均为Ｐ＜０．０５）;术后６个月、１ａＳＭＩＬＥ组和Ｒ-ＦＬＥｘ组分别为（９．６５±０．５８）ｍｍ、（１０．７０±０．９９）ｍｍ,（９．６３±０．８４）ｍｍ、（１０．７０±１．１２）ｍｍ,差异均无统计学意义（均为Ｐ＞０．０５）。两组ＢＵＴ检查结果:术后１个月、３个月ＳＭＩＬＥ组和Ｒ-ＦＬＥｘ组分别为（７．７５±０．９８）ｓ、（８．９０±０．９３）ｓ,（６．２５±０．８９）ｓ、（８．５０±０．７８）ｓ,差异均有统计学意义（均为Ｐ＜０．０５）;术后６个月、１ａＳＭＩＬＥ组和Ｒ-ＦＬＥｘ组分别为（９．６５±０．５８）ｓ、（１０．７０±０．９９）ｓ,（９．５３±０．７８）ｓ、（１０．３３±１．１９）ｓ,差异均无统计学意义（均为Ｐ＞０．０５）。结论　ＳＭＩＬＥ较Ｒ-ＦＬＥｘ术后早期泪膜稳定性好、患者舒适度高、干眼症状轻,至术后１ａ,泪膜稳定性基本恢复到术前水平。     

LASIK术后白内障患者应用不同公式计算的人工晶状体植入后效果分析

目的　评估ＬＡＳＩＫ术后白内障患者应用ＳＲＫ-Ｔ公式、ＨＯＦＦＥＲ-Ｑ修正公式和Ｈａｉｇｉｓ-Ｌ修正公式进行白内障超声乳化＋人工晶状体（ｉｎｔｒａｏｃｕｌａｒｌｅｎｓ,ＩＯＬ）植入术后的临床效果。方法　收集从２０１１年１月至２０１５年２月行白内障超声乳化＋ＩＯＬ植入术且既往有ＬＡＳＩＫ术史的患者１３例（２１眼）。根据不同ＩＯＬ计算公式分为３组,分别为ＳＲＫ-Ｔ公式（６眼）、ＨＯＦＦＥＲ-Ｑ修正公式（５眼）和Ｈａｉｇｉｓ-Ｌ修正公式（１０眼）。所有患者在白内障术前术后均进行常规检查,均由同一手术医师进行透明角膜切口超声乳化＋ＩＯＬ植入术。术后随访６个月,使用配对ｔ检验比较手术前后裸眼视力、最佳矫正视力、等效球镜、眼压、角膜内皮细胞密度的变化,对不同公式计算的术后等效球镜和术后等效球镜与目标屈光度之间的差值进行方差分析。结果　术后等效球镜和术后等效球镜与目标屈光度之间的差值,在±０．２５Ｄ内分别为５眼（２３．８１％）和１眼（４．７６％）,在±０．５Ｄ内均为６眼（２８．５７％）,在±１．０Ｄ分别为１０眼（４７．６２％）、１１眼（５２．３８％）。与术前相比,术后６个月时裸眼视力、最佳矫正视力明显提高（均为Ｐ＜０．００１）,等效球镜偏差度数显著减小（Ｐ＜０．００１）。应用ＳＲＫ-Ｔ公式、ＨＯＦＦＥＲ-Ｑ修正公式和Ｈａｉｇｉｓ-Ｌ修正公式后,术后等效球镜误差在±１．０Ｄ内的分别为６７％、８０％、３０％;而术后等效球镜与目标屈光度之间的差值,误差在±１．０Ｄ内的分别为５０％、６０％、５０％。术后等效球镜和术后等效球镜与目标屈光度之间差值采用Ｈａｉｇｉｓ-Ｌ修正公式和ＨＯＦＦＥＲ-Ｑ修正公式组间差异有统计学意义（Ｐ＜０．００１、Ｐ＝０．００１）、Ｈａｉｇｉｓ-Ｌ修正公式和ＳＲＫ-Ｔ公式组间差异有统计学意义（Ｐ＜０．００１、Ｐ＜０．００１）。结论　ＬＡＳＩＫ术后白内障患者在进行白内障超声乳化＋ＩＯＬ植入术后,ＨＯＦＦＥＲ-Ｑ修正公式计算的ＩＯＬ度数误差相对较小。     

原发性眼眶内脑膜瘤的临床及病理,免疫组化观察

目的 探讨眼眶内原发性脑膜瘤的临床、病理及免疫组化特点。方法 回顾性分析１５例眶内脑膜瘤的临床资料，复习症理切片及免疫组化结果。结果 患者男６例、女９例，４￣５２岁（平均３３．３岁）。主要临床表现为眼球突出、视力严重受损及视神经萎缩。大体病理形态与颅内脑膜瘤相同。２例侵犯眼眶骨质，但核分裂相少。免疫组化阳性率为：ＶＩＭ１００％，ＥＭＡ６０％，ＣＫ４６．６７％，Ｓ１００４０％，ＮＳＥ６０％，ＧＦＡＰ     

非诺贝特治疗糖尿病视网膜病变合并肾病的临床研究

目的　观察非诺贝特治疗糖尿病视网膜病变合并肾病的临床疗效。方法　２８例（５６眼）２型糖尿病患者并发糖尿病视网膜病及肾病在控制血糖基础上随机分为Ａ、Ｂ两组。Ａ组（对照组,１４例２８眼）口服安慰剂ＶｉｔＣ片０．１ｇ;Ｂ组（试验组,１４例２８眼）口服非诺贝特片０．２ｇ;均为每天３次,饭前０．５ｈ口服,连续用４２ｄ,观察两组治疗前后视力、眼底的变化、血压、肾功能、２４ｈ尿蛋白、尿转铁蛋白、血浆基质金属蛋白酶２（ｍｅｔａｌｌｏｐｒｏｔｅｉｎａｓｅ２,ＭＭＰ２）和组织型基质金属蛋白酶抑制剂１（ｔｉｓ-ｓｕｅｉｎｌａｉｂｉｔｏｒｏｆｍｅｔａｌｌｏｐｒｏｔｅｉｎａｓｅｓ１,ＴＩＭＰ-１）水平。结果　治疗前两组患者一般情况比较差异均无统计学意义（均为Ｐ＞０. ０５）。治疗４２ｄ后,视力提高３２眼,其中Ａ组６眼（占１８．７％）,Ｂ组２６眼（占８１．３％）,两组视力较治疗前改善的眼数比较差异有统计学意义（χ２＝１２．６１９,Ｐ＜０．０５）;Ａ组患者较治疗前各项指标差异均无统计学意义（ｔ２４ｈ尿蛋白＝１．２５４、ｔＣｒ＝１．３０２、ｔＢＵＮ ＝０．５３９、ｔ尿β２微球蛋白＝０．９２６、ｔＦＡ ＝１．０２６、ｔＦｉｂ＝０．９５４、ｔＥＴ-１＝１．１２４、ｔＭＭＰ２／ＴＩＭＰ１＝０．９８２,均为Ｐ＞０．０５）;Ｂ组２４ｈ尿蛋白、肾功能（Ｃｒ、ＢＵＮ、尿β２微球蛋白）、ＦＡ、Ｆｉｂ、ＥＴ-１和ＭＭＰ２／ＴＩＭＰ１水平均比治疗前明显降低（ｔ２４ｈ尿蛋白＝６．７３９、ｔＣｒ＝８．３７８、ｔＢＵＮ ＝６．２６４、ｔ尿β２ＭＧ ＝５. ５４２、ｔＦＡ ＝７．０９２、ｔＦｉｂ＝５．４２８、ｔＥＴ-１＝６．５５４、ｔＭＭＰ２／ＴＩＭＰ１＝８．９２２,均为Ｐ＜０．０５）。治疗后两组患者各项指标比较差异均有统计学意义水平（ｔ２４ｈ尿蛋白＝４．４３２、ｔ尿β２微球蛋白＝５．４２８、ｔＦＡ ＝５．６１６、ｔＣｒ＝８．８２１、ｔＢＵＮ ＝６．４８２、ｔＦｉｂ＝５．９０４、ｔＥＴ-１＝９．１６２、ｔＭＭＰ２／ＴＩＭＰ１＝５．３４２,均为Ｐ＜０．０５）。结论　非诺贝特治疗临床期糖尿病视网膜病变及肾病,可提高患者视力,能更有效改善视网膜微循环和肾血流动力学,保护眼底和肾功能。     

中西医结合治疗急性球后视神经炎的临床观察

中药丹枝逍遥散合桃红生四物汤加减，配合静脉滴注脉络宁治疗急性球后视神经炎１２例１３眼。以视力、眼底检查、结合图形视觉诱发电位（Ｐ－ＶＥＰ）、对比敏感度（ＣＳＦ）、眼底荧光血管造影（ＦＦＡ）、视野等检查。结果作为综合分析评价疗效的标准。结果治愈５例５眼，占３８．４６％；显效４例５眼，占３８．４６％；有效２例２眼，占１５．３８％；无效１例１眼，占７．６９％；总有效率９２．３１％。治疗后有１０眼视力≥１．０，Ｐ－ＶＥＰＣＳＦ，视野均有不同程度的改善。     

鼠神经生长因子联合高压氧治疗视神经萎缩的疗效

目的　探讨鼠神经生长因子（ｎｅｒｖｅｇｒｏｗｔｈｆａｃｔｏｒ,ＮＧＦ）联合高压氧治疗视神经萎缩的疗效。方法　将２０１２年６月至２０１５年５月我院收治的视神经萎缩患者７６例（８８眼）,随机分成对照组和观察组,对照组３６例（４２眼）,采用传统治疗加ＮＧＦ肌肉注射;观察组４０例（４６眼）,在传统治疗加ＮＧＦ肌肉注射基础上联合使用高压氧治疗,比较分析２组疗效、治疗前后视力、视野改变及荧光充盈迟缓时间、视觉诱发电位（ＶＥＰ）Ｐ１００潜伏期等。结果　对照组治疗后总有效率７６．２％,观察组总有效率９３．５％,对照组疗效明显低于观察组,差异有统计学意义（Ｚ＝－２．０１０,Ｐ＝０．０４）,总有效率对比差异亦有统计学意义（χ２＝５．２１,Ｐ＝０．０２）。病程≤２周的患者中,观察组疗效高于对照组,差异有统计学意义（χ２＝４．８３６,Ｐ＝０．０２８）,但病程＞２周的总有效率两组比较差异则无统计学意义（Ｐ＞０．０５）。对照组与观察组不同病因的临床疗效比较,差异均无统计学意义（均为Ｐ＞０．０５）。两组视力、视野、眼底荧光充盈迟缓时间、ＶＥＰＰ１００潜伏期各项指标治疗前后比较差异均有统计学意义（均为Ｐ＜０．０１）,提示对照组和观察组治疗均有效。比较对照组与观察组在治疗前的视力、视野、眼底荧光充盈迟缓时间、ＶＥＰＰ１００潜伏期各指标差异均无统计学意义（均为Ｐ＞０．０５）,具有可比性;但在治疗后上述各项指标差异均有统计学意义（均为Ｐ＜０．０５）。结论　ＮＧＦ联合高压氧治疗视神经萎缩疗效确切,可促进视功能的恢复,值得临床上推广与应用。     

2.0mm小切口飞秒激光角膜基质透镜取出术与飞秒激光辅助的LASIK矫正近视的疗效比较

目的　比较２．０ｍｍ微切口飞秒激光角膜基质透镜取出术（２．０ｍｍｓｍａｌｌｉｎｃｉｓｉｏｎｌｅｎｔｉｃｕｌｅｅｘｔｒａｃｔｉｏｎ,２．０ｍｍＳＭＩＬＥ）与飞秒激光辅助的ＬＡＳＩＫ（ｌａｓｅｒｉｎｓｉｔｕｋｅｒａｔｏｍｉｌｅｕｓｉｓｗｉｔｈｆｅｍｔｏｓｅｃｏｎｄｌａｓｅｒ,ＦＳ-ＬＡＳＩＫ）矫正近视的疗效。方法　研究纳入行２．０ｍｍＳＭＩＬＥ的近视患者４８例（９６眼）,同期行ＦＳ-ＬＡＳＫ者５０例（１００眼）,记录并比较两组术前及术后１ｄ、１周、１个月、３个月及６个月的裸眼视力（ｕｎｃｏｒｒｅｃｔｅｄｖｉｓｕａｌａｃｕｉｔｙ,ＵＣＶＡ）、最佳矫正视力（ｂｅｓｔｃｏｒｒｅｃｔｅｄｖｉｓｕａｌａｃｕｉｔｙ,ＢＣＶＡ）、屈光度、生活质量量表（ｑｕａｌｉｔｙｏｆｌｉｆｅ,ＱＯＬ）得分及手术满意度量表评分。结果　在术后１周以后ＳＭＩＬＥ组视力高于ＦＳ-ＬＡＳＩＫ组,且状态较ＦＳ-ＬＡＳＩＫ组更为稳定。术后１个月、３个月、６个月ＳＭＩＬＥ组ＵＣＶＡ≥术前ＢＣＶＡ的比例高于ＦＳ-ＬＡＳＩＫ组,差异均有统计学意义（均为Ｐ＜０．０５）。术后早期（术后１周）ＳＭＩＬＥ组存在１例过矫,而ＦＳ-ＬＡＳＩＫ组存在１例欠矫,但在术后１个月、３个月、６个月两组手术患者均在±１．００Ｄ范围内。术后平均角膜前表面形态变异指数及垂直不对称指数在各时间点均为ＦＳ-ＬＡＳＩＫ组大于ＳＭＩＬＥ组,差异均有统计学意义（均为Ｐ＜０．０５）。两组术前及术后６个月ＱＯＬ得分组间比较,差异均无统计学意义（均为Ｐ＞０．０５）,术后６个月ＱＯＬ得分均较术前有所增加,且差异均有统计学意义（ＳＭＩＬＥ:ｔ＝－１３．８５,Ｐ＝０．００;ＦＳ-ＬＡＳＩＫ:ｔ＝－１３．２１,Ｐ＝０．００）,而两组术后６个月ＱＯＬ得分比较,差异无统计学意义（Ｐ＞０．０５）。两组均有较高的再次手术选择率及手术推荐率,并且未发生严重的并发症。结论　２．０ｍｍＳＭＩＬＥ与ＦＳ-ＬＡＳＩＫ均具有良好的有效性、稳定性以及可预测性,术后都可获得良好的视力及良好的生活质量,前者更好地保持了角膜前表面的形态。     

血清烯醇化酶定量检测对视网膜母细胞瘤诊断和监测的应用价值

目的探讨血清烯醇化酶,尤其是神经元特异性烯醇化酶（ｎｅｕｒｏｎ－ｓｐｅｃｉｆｉｃｅｎｏｌａｓｅ,ＮＳＥ）含量测定对视网膜母细胞瘤（ｒｅｔｉｎｏｂｌａｓｔｏｍａ,Ｒｂ）诊断和病情监测的价值。方法应用醋酸纤维薄膜快速电泳＋荧光分光光度法,对３５例经病理检查确诊的Ｒｂ患者,分别于眼球摘除时,术后３、６、１２和≥１８个月后及３１例对照组进行血清烯醇化酶总量和ＮＳＥ含量定量检测,观察比较血清烯醇化酶总量和ＮＳＥ含量的变化。结果Ｒｂ患者血清烯醇化酶总量为２０７．０±６５．９Ｕ／Ｌ,ＮＳＥ含量为９８．４±４０．３Ｕ／Ｌ,与对照组血清测定值（分别为１４０．７±４６．０Ｕ／Ｌ和４１．０±１０．９Ｕ／Ｌ）比较明显升高,两组间差异有显著性（Ｐ＜０．００１）。８２．６％Ｒｂ患者血清ＮＳＥ含量明显高于对照组上限水平。Ｒｂ患儿术后６、１２和≥１８个月血清烯醇化酶总量和ＮＳＥ含量逐渐下降（ＮＳＥ含量下降较缓慢）,与治疗前的测定值相比差异有显著性;至≥１８个月后血清ＮＳＥ含量和烯醇化酶总量降至对照组测定值水平。结论血清烯醇化酶尤其ＮＳＥ含量测定对Ｒｂ患者有一定的辅助诊断价值。     

Magnetic resonance imaging for tumor restaging after chemotherapy in retinoblastoma with optic nerve invasion

Purpose: Extraocular retinoblastoma with optic nerve invasion is treated by a multimodal protocol consisting of neoadjuvant chemotherapy, enucleation, and adjuvant therapy. This study was conducted to evaluate the performance of magnetic resonance imaging (MRI) used for tumor restaging in these children after systemic chemotherapy administration.

Methods: Contrast-enhanced MRI scan of orbits and brain was performed at diagnosis and patients were treated with neoadjuvant chemotherapy. After chemotherapy, MRI scan was repeated for tumor restaging and residual post-laminar thickening and/or enhancement of the affected optic nerve, if any, was recorded. MRI findings were correlated with histopathology in enucleated specimens. The main outcome measures were specificity, sensitivity, and accuracy of MRI in predicting post-laminar invasion after neoadjuvant chemotherapy.

Results: A total of 46 eyes (46 patients) were studied. Optic nerve thickening on MRI had a sensitivity, specificity, and accuracy of 100% (95% Confidence Interval (CI): 64.6–100%), 76.9% (95% CI: 61.7–87.4%), and 80.4% (95% CI: 66.8–89.4%), respectively. Optic nerve enhancement had a sensitivity, specificity, and accuracy of 85.7% (95% CI: 48.7–97.4%), 79.5 % (95% CI: 64.5–89.2%), and 80.4% (95% CI: 66.8–89.4%), respectively. Combined thickening and enhancement of the optic nerve had a sensitivity, specificity, and accuracy of 100% (95% CI: 60.9–100%), 82.4% (95% CI: 66.5–91.7%), and 85% (95% CI: 70.9–92.9%), respectively.

Conclusion: MRI is a valuable tool for restaging of retinoblastoma and predicting residual optic nerve disease after neoadjuvant chemotherapy. Combined thickening and enhancement on MRI appeared to be a more reliable indicator of post-laminar invasion as compared to thickening or enhancement alone.     

Neoadjuvant chemotherapy for extensive unilateral retinoblastoma

AIM: The role of neoadjuvant chemotherapy was studied when first line enucleation cannot be safely performed in unilateral extensive retinoblastoma (major buphthalmia or radiologically detectable optic nerve involvement). METHODS: Six patients, referred for unilateral retinoblastoma, presented with major buphthalmia (two) or optic nerve invasion (four): they were treated by neoadjuvant chemotherapy using etoposide and carboplatin. RESULTS: Good tumour response was observed in the two patients with buphthalmia and in three of four cases with optic nerve involvement. Meningeal progressive disease was observed in the last patient. The five patients without disease progression were then operated on: anterior enucleation in the patients with buphthalmia and enucleation via a double neurosurgical and ophthalmological approach with prechiasmatic optic nerve section in the other three cases. Postoperative chemotherapy was performed in these five patients. Local radiotherapy to the chiasmatic region and posterior part of the optic canal was necessary in only one patient. The non-operated patient died with disease progression 6 months after the diagnosis. The other five patients are alive with a follow up of 12, 15, 21, 36, and 40 months after stopping treatment. CONCLUSION: Neoadjuvant chemotherapy can be useful in extensive unilateral retinoblastoma with buphthalmia and/or radiological optic nerve invasion at diagnosis.     

Tumour angiogenesis as a prognostic factor for disease dissemination in retinoblastoma

AIM: To evaluate tumour angiogenesis as a predictor of prognosis in retinoblastoma. METHODS: This was a retrospective, non-randomised comparative clinicopathological study. The histopathology from 24 cases of Reese-Ellsworth (RE) group V unilateral retinoblastoma treated by enucleation alone was reviewed. Group I consisted of five patients (four RE group Vb and one group Va) who developed disseminated disease at a mean of 10.4 months after enucleation. The remaining 19 patients constitute group II (18 RE group Vb and 1 group Va), none of whom had developed metastatic disease with a mean follow up of 54 months. None of the 24 patients had evidence of extraocular disease at enucleation. The surgical specimens from patients with unilateral retinoblastoma treated by enucleation at Hospital do Cancer AC Camargo between January 1992 and December 1995 were identified, reviewed and the clinical data recorded. Two subsequent histological sections were prepared. One stained with haematoxylin and eosin for assessment of choroidal and optic nerve invasion, and the other for immunoreaction with an endothelium specific marker (antibody anti-CD 34). The main outcome measures were choroidal and/or optic nerve invasion and quantification of the tumour's relative vascular area (TRVA) obtained by Chalkley counting. RESULTS: Choroidal invasion was present in three eyes of group I (all massive) and six eyes of group II (two focal and four massive). Optic nerve invasion was found in two eyes of group I (all post-laminar) and four eyes of group II (three prelaminar and one post-laminar). There was no statistical difference regarding choroidal or optic nerve between the two groups. The TRVA was the only independent variable found to predict disease dissemination (p = 0.008 by Cox analysis). A TRVA equal to or greater than 3.9% had 100% sensitivity and 79% specificity in predicting disease dissemination. CONCLUSIONS: Quantification of angiogenesis, through measurement of the TRVA, can help to identify patients with retinoblastoma at high risk for disease dissemination after enucleation.     

不同眼组织病理侵犯对单眼视网膜母细胞瘤生存预后的影响

　　目的　观察单眼视网膜母细胞瘤（RB）患者患眼肿瘤组织不同眼组织病理侵犯对生存率及预后的影响。方法　分析77例单眼RB行I期眼球摘除手术的患者随访观察资料。所有患者治疗后随访1周~89个月,平均随访时间49个月。采用复诊、电话问询及信访方式,随访了解患者的生存情况。应用Kaplan-Meier方法,分析视神经侵犯、脉络膜侵犯、脉络膜侵犯有无合并视神经侵犯以及眼前节受侵犯等眼肿瘤组织不同眼组织病理侵犯与患者生存率的相互关系。结果　2年后生存率保持在88.31％。视神经切面断端侵犯组生存率为16.67％,与其他不同程度视神经侵犯组生存率比较,差异有统计学意义（χ²=19.51,18.42,18.42,14.39;P值均=0.000 0）;大部分脉络膜侵犯合并巩膜内、外侵犯组生存率为60.00％,与无脉络膜侵犯组生存率比较,差异有统计学意义（χ2=7.69,P=0.005 5）;大部分脉络膜侵犯合并不同程度视神经侵犯组生存率为75.00％,与无脉络膜侵犯的视神经侵犯组生存率比较,差异有统计学意义（χ²=4.25,P=0.039 3）;巩膜及巩膜外侵犯合并视神经侵犯组生存率为60.00%,与无脉络膜侵犯的视神经侵犯组生存率比较,差异有统计学意义（χ²=7.59,P=0.005 9）;眼前节受侵犯组与眼前节未受侵犯组生存率比较,差异无统计学意义（χ²=0.05,P=0.823 5）。结论 视神经切面断端侵犯是预后最差的病理因素,死亡风险最大;大部分脉络膜侵犯合并不同程度视神经侵犯者和巩膜侵犯者的预后相对较差;眼前节侵犯对预后无明显影响。      

Retinoblastoma patients with high risk ocular pathological features: who needs adjuvant therapy?

AIMS: To describe the outcome of patients with non-metastatic unilateral retinoblastoma with high risk histopathological features after primary enucleation, and to clarify the need and results of adjuvant therapy.Patients and methods: From 1980 to 2001 adjuvant therapy was recommended only to patients with scleral involvement, post-laminar optic nerve involvement (PLONI) with either a positive margin or associated choroidal involvement, or (before 1994) isolated PLONI. RESULTS: 108 of 224 patients had at least one high risk feature (choroidal, scleral, anterior chamber, and/or PLONI). Patients with isolated choroidal (n = 55) or anterior chamber (n = 2) invasion, and most with PLONI without other risk factors (n = 21) were not treated; three relapsed but are long term survivors after intensive therapy. Four with isolated PLONI received adjuvant chemotherapy and none relapsed. Three of 11 with PLONI and concomitant choroidal or scleral involvement who received adjuvant therapy relapsed, versus two of four not treated. Two of five with scleral disease relapsed. All 12 with cut end involvement received adjuvant treatment and none relapsed. In the total group, all four patients who relapsed after adjuvant therapy died. CONCLUSIONS: Relapsing patients can be rescued with intensive therapy. Those with isolated choroidal or PLONI have a good prognosis without adjuvant therapy. Patients with PLONI with a positive margin have a good prognosis if treated with combined therapy. Those with scleral involvement or PLONI with concomitant choroid disease may benefit from adjuvant therapy.     

Vitrectomy in eyes with unsuspected retinoblastoma

OBJECTIVE: To analyze patient management and prognosis after vitrectomy in eyes with unsuspected retinoblastoma. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Eleven consecutive patients who had undergone vitrectomy on an eye with unsuspected retinoblastoma. MAIN OUTCOME MEASURES: The two main outcome measures were ultimate patient management and the development of retinoblastoma metastasis. RESULTS: Of more than 900 consecutive patients with retinoblastoma managed on the Ocular Oncology Service at Wills Eye Hospital in Philadelphia, 11 (1%) had prior vitrectomy in an eye with viable tumor before referral to us for suspected retinoblastoma. The main preoperative diagnoses included vitreous hemorrhage in seven patients (64%), toxocariasis in two patients (18%), toxoplasmosis in one patient (9%), and endophthalmitis in one patient (9%). In no case was retinoblastoma suspected before vitrectomy. The mean patient age at vitrectomy was 6 years. Retinoblastoma was later suspected during vitrectomy in two patients (18%), on cytologic examination of the vitrectomy specimen in eight patients (73%), and after referral in one patient (9%). The mean interval between vitrectomy and referral to us was 23 days. On examination, the globe was classified as Reese-Ellsworth group Vb in all 11 patients (100%). Anterior chamber tumor cells were clinically visible in four eyes (36%), hyphema in two eyes (18%), and iris neovascularization in two eyes (18%). Retinoblastoma cells were visualized in the vitreous in seven eyes (64%) and not visualized in four eyes (36%) that had vitreous blood. Enucleation was necessary in all 11 patients (100%). Adjuvant treatment was delivered in 10 patients (91%), using orbital radiotherapy in nine patients (82%) and chemotherapy in nine patients (82%). Histopathologic evidence of retinoblastoma invasion was documented in the episclera (two eyes; 18%), anterior chamber (seven eyes; 64%), iris (five eyes; 45%), ciliary body (five eyes; 45%), choroid (three eyes; 27%), and optic nerve (four eyes; 36%; prelaminar, two eyes; postlaminar, two eyes). The vitrectomy ports, Tenon's fascia, cut end of the optic nerve, and orbit were free of tumor. Of the 10 patients who received prophylactic chemotherapy, radiotherapy, or both in addition to enucleation for prevention of retinoblastoma metastasis, none (0%) experienced metastasis or orbital recurrence during the mean follow-up of 7 years (range, 0.2-24 years) from the time of retinoblastoma diagnosis. However, one patient was referred to us after the development of metastatic retinoblastoma, and despite aggressive chemotherapy and radiotherapy after enucleation, died 24 months later. CONCLUSIONS: Retinoblastoma may present with atypical features such as vitreous hemorrhage or signs of vitreous inflammation, particularly in older children. Vitrectomy should be avoided in these cases until the possibility of underlying retinoblastoma is excluded. If vitrectomy is performed in an eye with unsuspected retinoblastoma, enucleation combined with adjuvant chemotherapy, radiotherapy, or both without delay is advised to prevent systemic tumor dissemination.     

视网膜母细胞瘤预后相关病理因素分析

目的 研究视网膜母细胞瘤患者预后的相关病理因素,探讨评估患者预后的病理学指标.方法 对1995-2004年期间在中山大学中山眼科中心经眼球摘除并病理确诊的125例视网膜母细胞瘤单眼患者的临床病理资料和随访资料进行回顾性研究,应用Cox比例风险模型单因素和多因素分析影响预后的病理因素.结果 视网膜母细胞瘤患者总体5年生存率为89%,单因素预后分析显示视神经断端浸润(χ2=114.150,P=0.000)、巩膜浸润(χ2=35.080,P=0.000)、眶组织浸润(χ2=28.765,P=0.000)、虹膜浸润(χ2=18.242,P=0.000)、睫状体浸润(χ2=11.495,P=0.001)和角膜浸润(χ2=10.240,P=0.001)为影响预后的重要因素;多因素预后分析显示视神经筛板后浸润(P=0.045,Wald值4.013)和视神经断端浸润(P=0.000,Wald值37.559)为影响预后的重要因素.结论 视网膜母细胞瘤病理检查有广泛脉络膜浸润、巩膜浸润、眶组织浸润、视神经筛板后浸润和视神经断端浸润是影响患者预后的重要危险因素.     

Prognosis of retinoblastoma. Report of 50 cases

PURPOSE: The aim of this study was to analyze the factors influencing the prognosis of retinoblastoma. MATERIALS AND METHODS: In 50 children with retinoblastoma, 69 eyes were reviewed. All patients had a full ophthalmic examination, a B-scan ultrasound, a computerized tomography scan and a pediatric examination. We performed enucleation in 47 eyes (with a histopathological study), external beam irradiation in 16 eyes, curitherapy in 4 eyes, cryotherapy in 3 eyes, and adjuvant chemotherapy in 7 cases. RESULTS: The global survival rate was 87.5%. The main aggravating factors were: the size of the tumor and the extraretinal involvement with extension within the chroid, the sclera, and the optic nerve. CONCLUSION: The prognosis of retinoblastoma mainly depends on the extraretinal invasion.     

视网膜母细胞瘤眼球摘除术后放化疗对生存预后影响的研究

目的探讨视网膜母细胞瘤（Rb）眼球摘除术后辅助治疗对预后的影响研究。方法随访中山大学中山眼科中心1997年1月-2001年12月Rb 102例眼球摘除术或眶内容摘除术,术后进行辅助治疗的64例的生存情况,术后放疗、化疗对生存预后影响的研究。结果筛板后侵犯者25例,进行辅助治疗19例,21例存活,4例死亡;视神经断端侵犯者7例,均给以术后辅助治疗,1例存活,6例死亡;小部分脉络膜侵犯44例,35例术后给以辅助治疗,36例存活,8例死亡;大部分脉络膜侵犯6例,3例进行术后辅助治疗,3例存活,3例死亡;眼前段受侵犯16例,8例术后给以辅助治疗,10例存活,6例死亡;眶组织侵犯12例,辅助治疗11例,4例存活,8例死亡。结论视神经断端侵犯者预后很差,术后积极的辅助治疗生存率很低;眶组织侵犯者眶内容摘除术后放疗对生存预后有帮助;单纯眼前段侵犯者术后辅助治疗要审慎;脉络膜侵犯和视神经近端侵犯者的术后辅助治疗仍需进一步研究。     

Risk of metastasis in retinoblastoma]

580 patients with retinoblastoma were analysed retrospectively. Median follow up was 5.5 years. 41 patients developed metastases within 5 years. Clinical and histopathological risk factors were analysed by univariate tests with regard to the occurrence of metastases. Significant variables were then reevaluated using Cox proportional hazards method. Four factors correlated independently with the development of metastases: optic nerve invasion with involvement of the resection line, optic nerve invasion without involvement of the resection line, choroidal invasion and enucleation of an affected eye more than 120 days after initial diagnosis. The regression coefficient beta of the Cox-model was used for a score classification with groups of high, medium and low metastatic risk. The 5-year incidence of metastases was 68%, 43% and 4% respectively.