中华眼底病杂志
中華眼底病雜誌
중화안저병잡지
CHINESE JOURNAL OF OCULAR FUNDUS DISEASES
2011年
4期
335-338
,共4页
曹绪胜%马凯%纪海霞%彭晓燕
曹緒勝%馬凱%紀海霞%彭曉燕
조서성%마개%기해하%팽효연
视网膜疾病/先天性%眼疾病,遗传性/诊断%荧光%荧光素血管造影术
視網膜疾病/先天性%眼疾病,遺傳性/診斷%熒光%熒光素血管造影術
시망막질병/선천성%안질병,유전성/진단%형광%형광소혈관조영술
Retinal diseases/congenital%Eye diseases,hereditary%Fluorescence%Fluorescein angiography
目的 观察儿童遗传性视网膜疾病的眼底自身荧光(FAF)特征.方法 回顾性分析22例临床资料完整、年龄5～14岁之问的遗传性视网膜疾病患儿的FAF检查结果.其中,Best卵黄样病变8例16只眼,Stargardt病3例6只眼,视锥细胞营养不良3例6只眼,原发性视网膜色素变性(RP)5例10只眼,X连锁青少年型视网膜劈裂症3例6只眼.仔细询问现病史及家族史,行视力、裂隙灯显微镜眼前节检查,间接眼底镜检查,彩色眼底像和FAF照相,其中部分患儿接受了荧光素眼底血管造影(FFA)、视网膜电流图、眼电图、光相干断层扫描检查.对上述患儿的FAF结果特征进行归纳总结,并与其眼底照相和/或FFA结果进行比较分析.结果 3例Stargardt病患儿的6只眼及3例视锥细胞营养不良患儿的6只眼FAF检查显示黄斑区可见对称性的圆形、近圆形弱荧光或荧光缺如区,2例视锥细胞营养不良患儿的4只眼及1例Stargardt病患儿的2只眼可见弱荧光或荧光缺如区外缘环以强荧光;Best卵黄样病变患儿黄斑区可见一个强度均匀或不均匀的强FAF病灶;RP患儿后极部视网膜FAF增强,黄斑区周围可见宽度不等的环形强荧光带,拱环区FAF正常;3例X连锁青少年型视网膜劈裂症患儿中5只眼中心凹部位FAF检查可见蜂窝或颗粒状强荧光.结论 Stargardt病及视锥细胞营养不良患儿黄斑区为近圆形弱荧光,部分病变区外缘环以强荧光;Best卵黄样病变患儿黄斑区为强度均匀或不均匀的强FAF病灶;RP患儿后极部视网膜FAF增强,拱环区FAF正常,黄斑区周围可见宽度不等的环形强荧光带;X连锁青少年型视网膜劈裂症患儿中心凹部位为蜂窝或颗粒状强荧光.
目的 觀察兒童遺傳性視網膜疾病的眼底自身熒光(FAF)特徵.方法 迴顧性分析22例臨床資料完整、年齡5～14歲之問的遺傳性視網膜疾病患兒的FAF檢查結果.其中,Best卵黃樣病變8例16隻眼,Stargardt病3例6隻眼,視錐細胞營養不良3例6隻眼,原髮性視網膜色素變性(RP)5例10隻眼,X連鎖青少年型視網膜劈裂癥3例6隻眼.仔細詢問現病史及傢族史,行視力、裂隙燈顯微鏡眼前節檢查,間接眼底鏡檢查,綵色眼底像和FAF照相,其中部分患兒接受瞭熒光素眼底血管造影(FFA)、視網膜電流圖、眼電圖、光相榦斷層掃描檢查.對上述患兒的FAF結果特徵進行歸納總結,併與其眼底照相和/或FFA結果進行比較分析.結果 3例Stargardt病患兒的6隻眼及3例視錐細胞營養不良患兒的6隻眼FAF檢查顯示黃斑區可見對稱性的圓形、近圓形弱熒光或熒光缺如區,2例視錐細胞營養不良患兒的4隻眼及1例Stargardt病患兒的2隻眼可見弱熒光或熒光缺如區外緣環以彊熒光;Best卵黃樣病變患兒黃斑區可見一箇彊度均勻或不均勻的彊FAF病竈;RP患兒後極部視網膜FAF增彊,黃斑區週圍可見寬度不等的環形彊熒光帶,拱環區FAF正常;3例X連鎖青少年型視網膜劈裂癥患兒中5隻眼中心凹部位FAF檢查可見蜂窩或顆粒狀彊熒光.結論 Stargardt病及視錐細胞營養不良患兒黃斑區為近圓形弱熒光,部分病變區外緣環以彊熒光;Best卵黃樣病變患兒黃斑區為彊度均勻或不均勻的彊FAF病竈;RP患兒後極部視網膜FAF增彊,拱環區FAF正常,黃斑區週圍可見寬度不等的環形彊熒光帶;X連鎖青少年型視網膜劈裂癥患兒中心凹部位為蜂窩或顆粒狀彊熒光.
목적 관찰인동유전성시망막질병적안저자신형광(FAF)특정.방법 회고성분석22례림상자료완정、년령5～14세지문적유전성시망막질병환인적FAF검사결과.기중,Best란황양병변8례16지안,Stargardt병3례6지안,시추세포영양불량3례6지안,원발성시망막색소변성(RP)5례10지안,X련쇄청소년형시망막벽렬증3례6지안.자세순문현병사급가족사,행시력、렬극등현미경안전절검사,간접안저경검사,채색안저상화FAF조상,기중부분환인접수료형광소안저혈관조영(FFA)、시망막전류도、안전도、광상간단층소묘검사.대상술환인적FAF결과특정진행귀납총결,병여기안저조상화/혹FFA결과진행비교분석.결과 3례Stargardt병환인적6지안급3례시추세포영양불량환인적6지안FAF검사현시황반구가견대칭성적원형、근원형약형광혹형광결여구,2례시추세포영양불량환인적4지안급1례Stargardt병환인적2지안가견약형광혹형광결여구외연배이강형광;Best란황양병변환인황반구가견일개강도균균혹불균균적강FAF병조;RP환인후겁부시망막FAF증강,황반구주위가견관도불등적배형강형광대,공배구FAF정상;3례X련쇄청소년형시망막벽렬증환인중5지안중심요부위FAF검사가견봉와혹과립상강형광.결론 Stargardt병급시추세포영양불량환인황반구위근원형약형광,부분병변구외연배이강형광;Best란황양병변환인황반구위강도균균혹불균균적강FAF병조;RP환인후겁부시망막FAF증강,공배구FAF정상,황반구주위가견관도불등적배형강형광대;X련쇄청소년형시망막벽렬증환인중심요부위위봉와혹과립상강형광.
Objective To observe the autofluorescence (AF) manifestation in children with hereditary retinal diseases. Methods The clinical data of 22 children (aged from 5 to 14 years) with hereditary retinal diseases were retrospectively analyzed. There were 8 children (16 eyes) with Best vitelliform macular dystrophy, 3 children (6 eyes) with Stargardt macular dystrophy, 3 children (6 eyes) with macular cone dystrophy, 5 children (10 eyes) with primary retinitis pigmentosa, and 3 children (6 eyes) with X-linked juvenile retinoschisis. The routine clinical examinations included present history, family history, visual acuity, silt-lamp microscopy, indirect ophthalmoscopy, color fundus photography and fundus autofluorescence angiography (FAF). Some patients received fundus fluorescein angiography (FFA),electroretinogram (ERG), electrooculogram (EOG), and ocular coherence tomography (OCT). The characteristics of AF in all the children were analyzed, and were compared with the images of color fundus and/or FFA. Results Symmetry round macular fluorescent weak or absent area was found in all Stargardt disease and cone dystrophy. Weak AF area with surrounded circular increased AF was found in 2 children (4 eyes) with cone dystrophy and 1 child (2 eyes) with Stargardt macular dystrophy. A central round area with regular or irregular intense AF was observed in Best vitelliform macular dystrophy. RP children showed increased AF out of the macular region. Cellular or granular strong AF was found in the fovea of 3 children (5 eyes) with X-linked juvenile retinoschisis. Conclusion The children with hereditary retinal diseases had special AF changes.
