Gräf Michael, Seliametova Leniie, Hausmann Anja
Fachbereich Medizin, Justus-Liebig-Universität Gießen, Gießen, Deutschland.
Standort Gießen, Universitätsklinikum Gießen und Marburg GmbH, Gießen, Deutschland.
Ophthalmologie. 2023 Jun;120(6):628-632. doi: 10.1007/s00347-022-01784-3. Epub 2022 Dec 5.
Large muscle recessions reduce anomalous head turn (AHT) in infantile nystagmus syndrome (INS). Their quantitative effect to reduce AHT in fusion maldevelopment nystagmus syndrome (FMNS) is unknown.
To evaluate the reduction of AHT by large medial rectus muscle recession in patients with FMNS.
We analyzed data of 12 patients with pure FMNS who received large medial rectus muscle recessions on the sound eye or only eye between 2014 and 2019. Medians and ranges (min-max) were: age at surgery 14 years (3-43 years); decimal BCVA sound eye/fellow eye 0.56 (0.4-1.0)/0.01 (0-0.4); AHT at 5 m/0.3 m fixation distance 35° (15-45°)/20° (0-45°); amount of recession 13 mm (11.5-15 mm); follow-up 13 (4-39) months.
At the final visit, AHT at 5 m/0.3 m was 10° (0-20°/0-15°). The individual reduction of AHT at 5 m amounted to 25° (8-35°) without any overcorrection. The available visual field of functionally monocular patients increased correspondingly. Slight overcorrection at 0.3 m occurred in 2 cases. Adduction of the sound eye was limited to 25e-40°). BCVA and anomalous head posture in vertical and frontal planes did not improve.
Large medial rectus muscle recession on the sound eye improves AHT in FMNS by a similar amount compared to INS and the available visual field of functionally monocular patients.
在婴儿型眼球震颤综合征(INS)中,大肌肉后退术可减少异常头位转动(AHT)。其对融合发育不良性眼球震颤综合征(FMNS)中减少AHT的定量效果尚不清楚。
评估FMNS患者行大的内直肌后退术对AHT的减少情况。
我们分析了2014年至2019年间12例单纯FMNS患者的数据,这些患者在健眼或仅有的一只眼睛上接受了大的内直肌后退术。中位数和范围(最小值 - 最大值)为:手术年龄14岁(3 - 43岁);十进制最佳矫正视力(BCVA)健眼/对侧眼0.56(0.4 - 1.0)/0.01(0 - 0.4);在5米/0.3米注视距离时的AHT为35°(15 - 45°)/20°(0 - 45°);后退量13毫米(11.5 - 15毫米);随访13(4 - 39)个月。
在最后一次随访时,5米/0.3米处的AHT为10°(0 - 20°/0 - 15°)。5米处AHT个体减少量达25°(8 - 35°),且无任何过矫情况。功能上为单眼患者的可用视野相应增加。2例在0.3米处出现轻微过矫。健眼内收受限至25° - 40°。BCVA以及垂直和额面的异常头位均未改善。
与INS相比,在FMNS中对健眼行大肌肉后退术改善AHT的程度相似,且功能上为单眼患者的可用视野也相似。