先天性眼球震颤的视功能临床研究

对７８例不合并斜视和屈光不正的先天性眼球震颤（ｃｏｎｇｅｎｉｔａｌｎｙｓｔａｇｍｕｓ，ＣＮ）患者进行了视力、立体视和眼震图检查，结果表明：（１）ＣＮ患者近视力优于远视力，双眼同时注视视力优于单眼视力。（２）ＣＮ患者的双眼视觉显然已经得到发育，不但具有双眼单视功能，而且具有一定程度的立体视觉。（３）ＣＮ患者的立体视锐度较差与视力不良有关，与震颤参数无关。（４）立体视觉的检查受到多种因素的影响，各种检查方法间无本质差别。（５）眼震对视力的影响是多因素的。     

先天性眼球震颤的立体视觉

采用随机点立体视觉检查图测定了５７例先天性眼球震颤患者的立体视觉功能，结果显示：绝大多数钟摆型及半数以上跳动型眼震可造成立体视觉的严重损害；立体视觉随视力，特别是近视力的优劣而呈正比例变化。     

先天性眼球震颤的立体视觉

采用随机点立体视觉检查图测定了５７例先天性眼球震颤患者的立体视觉功能，结果显示：绝大多数钟摆型及半数以上跳型及半数以上跳动眼震可造成立体视觉的严重损害；立体视觉随视力，特别是近视力的优劣而呈正比例变化。     

先天性特发性眼球震颤手术治疗后视功能的恢复

背景先天性特发性眼球震颤的手术方式已日渐成熟，但其术后视功能的恢复仍然值得关注。目的探讨先天性特发性眼球震颤的手术治疗方法及其对视功能的影响。方法按照患者的头位扭转角大小和斜视度数的不同将60例先天性特发性眼球震颤患者分为4组，Ⅰ组：头位扭转角〈15°，水平斜视度≤15°，行本体感受器切除术；Ⅱ组：头位扭转角〈15°，水平斜视度〉15°，行本体感受器切除术联合斜视矫正术；Ⅲ组：头位扭转角≥15°，水平斜视度≤15°，行中间带移位术（部分同时施行本体感受器切除术）；Ⅳ组：患者头位扭转角≥15°，水平斜视度〉15°，行中间带移位联合斜视矫正术（部分同时施行本体感受器切除术），采用自身对照法对患者手术前后单眼及双眼远近最佳矫正视力、头位扭转角、斜视度、眼球运动及立体视觉的变化进行比较。结果与术前相比，术后各组患者第一眼位单眼及双眼远视力均明显提高，差异均有统计学意义（单眼：t=6．00、8．94、11．76、16．41，P=0．00；双眼：t=4．81，P=0．0l；t=6．27、4．25、4．18，P：0．00）。术后Ⅰ组和Ⅱ组患者单眼及双眼近视力与术前比较差异无统计学意义（单眼：t=1．63，P=0．18；t=1．17，P=0．26；双眼：t=2．45，P=0．07；t=2．09，P=0．06），而Ⅲ组和Ⅳ组患者术后单眼及双眼近视力均明显提高（单眼：t=2．91，P=0．01；t=5．32，P=0．00；双眼：t=3．36、4．30，P=0．00）。Ⅲ组和Ⅳ组中，术前有代偿头位的41例中，术后39例代偿头位消失，2例明显改善，头位扭转角手术前后比较差异有统计学意义（t=29．90、16．91，P=0．00）。术前26例合并斜视的患者中术后24例患者眼位正位，2例患者斜视欠矫，Ⅱ组、Ⅳ组斜视度明显降低，手术前后比较差异均有统计学意义（t=29．15、36．79，P=0．00）。所有患者术后眼球运动无受限。术前12岁以下的患儿共23例，立体视觉平均为256．52″±66．23″，术后1年其立体视觉平均为120．87″±45．31″，手术前后比较差异有统计学意义（t=12．06，P=0．00）。结论中间带移位术、本体感受器切除术及斜视矫正术可以改善先天性特发性眼球震颤患者的视功能、矫正头位及斜视。     

先天性特发性眼球震颤Parks法手术前后的立体视觉

目的:观察先天性特发性眼球震颤儿童Parks中间带移位术前后的立体视觉。方法:利用同视机图片和立体图本,检查12例先天性眼球震颤儿童术前和术后6mo正前方远、近距离立体视觉。结果:所有患者术后正前方眼球基本不震颤或明显减轻,12例患儿中7例代偿头位消失,获得立体视觉,5例术后代偿头位改善,其中3例建立同时视觉,但未建立立体视觉。结论:Parks法中间带移位术治疗先天性特发性眼球震颤,不仅能消除和减轻代偿头位与眼球震颤,提高视力,代偿头位消失的患者还能建立远、近立体视觉,提高视觉质量。     

0～6岁儿童眼病的社区筛查

0～6岁是儿童视觉功能发育的关键时期,此期内许多眼部疾病不仅可导致儿童视力发育异常,甚至可造成视力残疾(低视力和盲).儿童视残的眼疾中先天性遗传性眼病占47.95％,屈光不正、弱视占17.95％,角膜病占10.26％,视神经疾病占6.92％,先天性白内障占3.08％,视网膜病变、青光眼、眼外伤占10.26％,其他占3.58％.一些眼病有明显的外观异常,如先天性眼球震颤,能够被家长发现,或有明确的病史,如眼外伤,使得家长能够及时带孩子就诊看病.但许多眼病的早期眼的外观无明显异常,不容易被家长发现,如早产儿视网膜病变、视网膜母细胞瘤、先天性白内障、先天性青光眼、斜视和弱视等眼病,当出现明显的眼部及视力异常时,可能已经错过了最佳的矫治时间,造成视觉功能发育异常,或形成视力残疾,甚至危及生命.     

家族性先天性眼球震颤患者眼底病变特点的临床观察

目的：研究家族性先天性眼球震颤患者的眼底病变特点。

方法：对我院就诊的家族性先天性眼球震颤3个家系患者20例40眼进行眼位、屈光状态、眼前节、眼底彩色照相、光学相干断层扫描(OCT)、视网膜电生理(ERG)和视觉诱发电位(VEP)等检查。

结果：家系A共8例患者,均为水平型眼球震颤,其中斜视6例(伴代偿头位2例),核性白内障2例,后极性白内障2例,早产儿视网膜病变1例,高度屈光不正2例。家系B共6例患者,均为水平型眼球震颤,其中白化病3例(伴黄斑发育不良2例),高度屈光不正2例,全色盲1例,核性白内障1例。家系C共6例患者(5例水平型眼球震颤,1例旋转型眼球震颤),其中Leber先天性黑矇2例,家族性渗出性视网膜病变1例,后极性白内障2例,虹膜萎缩1例,斜视2例。

结论：家族性先天性眼球震颤患者要进行全面的眼部检查,尽可能找出病因,改善患者视功能。     

先天性冲动型眼球震颤手术前后的近立体视觉

目的探讨先天性冲动型眼球震颤患者采用Parks法手术治疗前后的近立体视锐度变化。方法用颜少明等研著的《立体视觉检查图》(下文简称颜氏图)测定15例先天性冲动型眼球震颤患者术前第一眼位、静止眼位和术后第一眼位近立体视锐度。结果第一眼位双眼最佳矫正视力和近立体视锐度在术后较术前提高,差异有显著性。结论先天性冲动型眼球震颤患者经Parks修正法手术治疗后,症状改善,视力提高,近立体视觉也得以相应提高。     

先天性视网膜皱襞

患者女性,7岁.因自幼视力低下伴眼球震颤、斜视,于2009年8月6日来河北省沧州市中心医院眼科就诊.其母亲患双眼先天性白内障、眼球震颤、斜视,左眼视网膜脱离.     

先天性特发性水平眼球震颤伴斜视21例手术疗效观察

目的 观察分析先天性特发性水平眼球震颤的手术治疗效果.方法 收集自2009年1月至2011年1月先天性冲动型眼球震颤伴斜视病例21例,既往无眼外伤史和眼部手术史,男15例,女6例,年龄8～25岁.手术方法采用Parks法联合斜视矫正术.结果 21例患者术后原在位双眼同时视视力均提高2行或2行以上;18例患者头位面转角完全矫正,3例存在15°以内的面转角,其中2例欠矫,1例过矫.19例眼位正位,1例欠矫,1例过矫.结论 对于伴有头位和斜视先天性水平性冲动型眼球震颤患者,手术治疗是首选,可以有效地提高视力,改善头位,同时矫正斜视.     

Motor and sensory characteristics of infantile nystagmus

BACKGROUND/AIMS: Past studies have explored some of the associations between particular motor and sensory characteristics and specific categories of non-neurological infantile nystagmus. The purpose of this case study is to extend this body of work significantly by describing the trends and associations found in a database of 224 subjects who have undergone extensive clinical and psychophysical evaluations. METHODS: The records of 224 subjects with infantile nystagmus were examined, where 62% were idiopaths, 28% albinos, and 10% exhibited ocular anomalies. Recorded variables included age, mode of inheritance, birth history, nystagmus presentation, direction of the nystagmus, waveform types, spatial and temporal null zones, head postures and nodding, convergence, foveation, ocular alignment, refractive error, visual acuity, stereoacuity, and oscillopsia. RESULTS: The age distribution of the 224 patients was between 1 month and 71 years, with the mean age and mode being 23 (SD 16) years and 16-20 years respectively. By far the most common pattern of inheritance was found to be autosomal dominant (n = 40), with the nystagmus being observed by the age of 6 months in 87% of the sample (n = 128). 139 (62%) of the 224 subjects were classified as idiopaths, 63 (28%) as albinos, and 22 (10%) exhibited ocular anomalies. Conjugate uniplanar horizontal oscillations were found in 174 (77.7%) of the sample. 32 (14.3%) had a torsional component to their nystagmus. 182 (81.2%) were classed as congenital nystagmus (CN), 32 (14.3%) as manifest latent nystagmus (MLN), and 10 (4.5%) as a CN/MLN hybrid. Neither CN nor MLN waveforms were related to any of the three subject groups (idiopaths, albinos, and ocular anomalies) MLN was found in idiopaths and albinos, but most frequently in the ocular anomaly group. The most common oscillation was a horizontal jerk with extended foveation (n = 49; 27%). The amplitudes and frequencies of the nystagmus ranged between 0.3-15.7 degrees and 0.5-8 Hz, respectively. Periodic alternating nystagmus is commonly found in albinos. Albino subjects did not show a statistically significantly higher nystagmus intensity when compared with the idiopaths (p>0.01). 105 of 143 subjects (73%) had spatial nulls within plus or minus 10 degrees of the primary position although 98 subjects (69%) employed a compensatory head posture. Subjects with spatial null zones at or beyond plus or minus 20 degrees always adopted constant head postures. Head nodding was found in 38 subjects (27% of the sample). Horizontal tropias were very common (133 out of 213; 62.4%) and all but one of the 32 subjects with MLN exhibited a squint. Adult visual acuity is strongly related to the duration and accuracy of the foveation period. Visual acuity and stereoacuity were significantly better (p<0.01) in the idiopaths compared to the albino and ocular anomaly groups. 66 subjects out of a sample of 168 (39%) indicated that they had experienced oscillopsia at some time. CONCLUSIONS: There are strong ocular motor and sensory patterns and associations that can help define an infantile nystagmus. These include the nystagmus being bilateral, conjugate, horizontal uniplanar, and having an accelerating slow phase (that is, CN). Decelerating slow phases (that is, MLN) are frequently associated with strabismus and early form deprivation. Waveform shape (CN or MLN) is not pathognomonic of any of the three subject groups (idiopaths, albinos, or ocular anomalies). There is no one single stand alone ocular motor characteristic that can differentiate a benign form of infantile nystagmus (CN, MLN) from a neurological one. Rather, the clinician must consider a host of clinical features.     

手术治疗先天性眼球震颤合并斜视

目的 探讨先天性眼球震颤合并斜视的手术方法和效果.方法 回顾性分析先天性眼球震颤合并斜视手术24例.手术的设计根据中间带位置、代偿头位、斜视角度和主导眼的关系进行综合考虑,代偿头位矫正在主导眼上施行,斜视矫正在非主导眼上施行.结果 手术后随访3 ～12个月,平均（6.38±2.18）月.20例（83.33％）术后代偿头位消失,3例（12.50％）代偿头位明显改善,残留原方向15°以内头位扭转角,1例（4.17％）过矫.18例（75.00％）术后眼位达到正位,5例（20.83％）达到轻度欠矫或过矫,1例（4.17％）明显欠矫.所有病例原在位眼震减轻或消失,原在位双眼同时视视力均较术前提高2行或2行以上.术前术后双眼同时视视力及头位扭转角变化差异均有统计学意义（z=-5.83,-3.37,P ＜0.01）.结论 合并有斜视的先天性眼球震颤,手术既要矫正斜视又要解决代偿头位,合理的手术设计可以取得良好的治疗效果.     

【In Press】 Clinical features of strabismus and nystagmus in bilateral congenital cataracts

AIM: To evaluate the prevalence, clinical features, and the factors affecting onset of strabismus and nystagmus in patients with bilateral congenital cataracts. METHODS: This study evaluated 116 eyes of 58 patients who underwent lens removal for the treatment of bilateral congenital cataracts between January 1999 and January 2011. The presence and type of strabismus and nystagmus were determined before and after surgery. Type of strabismus and final visual acuity were compared in patients with and without nystagmus. Patients were divided into three groups (orthotropia/orthotropia, orthotropia/strabismus, and strabismus/strabismus) according to their preoperative and postoperative ocular alignment. Age at cataract surgery and associations of nystagmus and primary intraocular lens (IOL) implantation with strabismus were analyzed. RESULTS: Six patients (10.3%) had strabismus preoperatively and an additional 11 (19.0%) developed postoperative strabismus. Exotropia was more common than esotropia both preoperatively and postoperatively. Eighteen patients (31.0%) had postoperative nystagmus, with sensory nystagmus being the most common type. Of the 18 patients with nystagmus, 10 had strabismus, with exotropia being more common than esotropia. Postoperative visual acuity was poor in patients with nystagmus. Age at cataract surgery and rate of primary IOL implantation were significantly lower, and postoperative nystagmus was more common, in the orthotropia/strabismus group than in the other two groups. CONCLUSION: Exotropia and sensory nystagmus are common in patients with bilateral congenital cataracts. Age at cataract surgery and rate of IOL implantation were lower and nystagmus more common in patients with postoperative onset of strabismus. Nystagmus was associated with poor visual prognosis.     

Clinical features of strabismus and nystagmus in bilateral congenital cataracts

AIM: To evaluate the prevalence, clinical features, and the factors affecting onset of strabismus and nystagmus in patients with bilateral congenital cataracts. METHODS: This study evaluated 116 eyes of 58 patients who underwent lens removal for the treatment of bilateral congenital cataracts between January 1999 and January 2011. The presence and type of strabismus and nystagmus were determined before and after surgery. Type of strabismus and final visual acuity were compared in patients with and without nystagmus. Patients were divided into three groups (orthotropia/orthotropia, orthotropia/strabismus, and strabismus/strabismus) according to their preoperative and postoperative ocular alignment. Age at cataract surgery and associations of nystagmus and primary intraocular lens (IOL) implantation with strabismus were analyzed. RESULTS: Six patients (10.3%) had strabismus preoperatively and an additional 11 (19.0%) developed postoperative strabismus. Exotropia was more common than esotropia both preoperatively and postoperatively. Eighteen patients (31.0%) had postoperative nystagmus, with sensory nystagmus being the most common type. Of the 18 patients with nystagmus, 10 had strabismus, with exotropia being more common than esotropia. Postoperative visual acuity was poor in patients with nystagmus. Age at cataract surgery and rate of primary IOL implantation were significantly lower, and postoperative nystagmus was more common, in the orthotropia/strabismus group than in the other two groups. CONCLUSION: Exotropia and sensory nystagmus are common in patients with bilateral congenital cataracts. Age at cataract surgery and rate of IOL implantation are lower and nystagmus more common in patients with postoperative onset of strabismus. Nystagmus is associated with poor visual prognosis.     

The effect of bilateral horizontal rectus recession on visual acuity, ocular deviation or head posture in patients with nystagmus.

PURPOSE: This work was undertaken to evaluate the effect of bilateral horizontal rectus recession on visual acuity, ocular deviation and head posture in patients with nystagmus. METHODS: Twenty patients underwent recession of 4 horizontal rectus muscles to dampen the nystagmus. At least 2 muscles were recessed posterior to the equator. RESULTS: Monocular and binocular visual acuity improved from 1 to 3 Snellen lines after the procedure in 13 patients (76.5%). Visual changes in log MAR notations were statistically significant with Wilcoxon analysis in each and both eyes. Preoperative binocular visual acuity was 0.73 +/- 0.26 log MAR that reached to 0.62 +/- 0.32 log MAR after surgery (P = 0.02). The greatest improvement was observed in patients with congenital motor nystagmus. Ten patients had horizontal strabismus in addition to nystagmus before the surgery. One-millimeter additional recession of both medial rectus muscles caused an average reduction of 6 PD in esotropia. Corresponding figures for similar additional recessions of the lateral rectus muscles was 11 PD for correction of exotropia. Abnormal head posture decreased in all the cases and improved completely in most of them. CONCLUSIONS: Large horizontal rectus recession can improve visual acuity and decrease nystagmus in sensory and motor types. By revision in surgical planning, strabismus and abnormal head posture can also be corrected.     

Early binocular visual experience may improve binocular sensory outcomes in children after surgery for congenital unilateral cataract.

PURPOSE: To compare the effect of intensive and reduced occlusion therapy regimens on binocular sensory outcomes, visual acuity, and the prevalence of strabismus in children after surgery for congenital unilateral cataract. METHODS: Two nonrandomized groups of patients were studied prospectively: (1) an intensive occlusion group (n = 29) patched 80% of waking hours were followed for a median 6.9 years and (2) a reduced occlusion group (n = 8) patched 25% to 50% of waking hours were followed for a median 4.3 years. Six subjects in the intensive group and 4 in the reduced occlusion group had secondary intraocular lenses. Two subjects in the intensive group had epikeratophakia surgery. Binocular sensory function was assessed with random dot and contour stereoacuity tests and the Worth 4-dot test. The prevalence and age at onset of strabismus were determined from the patients' charts. RESULTS: A higher proportion of subjects in the reduced occlusion group (50%) had stereoacuity or fusion compared with the intensive occlusion group (14%), a borderline significant difference (P =.08). No significant difference (P =.55) was found in median visual acuity between the intensive (20/50) and the reduced occlusion (20/55) groups. The 90% prevalence of strabismus in the intensive occlusion group was slightly higher than the 63% prevalence in the reduced occlusion group, although this difference was not significant (P =.18). CONCLUSIONS: These results suggest that a reduced occlusion protocol may be associated with better binocular sensory outcomes and a reduced prevalence of strabismus without compromising good visual acuity in children treated for congenital unilateral cataract.     

儿童白内障摘除人工晶状体植入术后视功能的长期随访

目的　评价儿童白内障摘除后房型人工晶状体 (IOL)植入术后视力和双眼视功能的情况。方法　对年龄<13岁的儿童白内障患者 47例(68只眼 )行白内障摘除人工晶状体植入术,其中单眼外伤性白内障 12例,单眼先天性白内障 14例,双眼先天性白内障 21例。记录患者的术前情况,包括白内障类型、晶状体混浊程度、手术年龄及合并症情况,检查术后最佳矫正远视力 (BCDVA)、最佳矫正近视力 (BCNVA)及双眼视功能情况。平均随访时间 ( 41 3±12 3 )个月。结果　术后BCDVA≥0 5和BCNVA≥0 5均为 34只眼 ( 50 0% )。18例患者 ( 38 3% )术后获得良好双眼视功能。不同BCDVA术眼在手术年龄、晶状体混浊程度及合并症 (斜视和眼球震颤 )方面的差异有统计学意义(P<0 05 );术后获得不同双眼视功能者在手术年龄、合并症 (斜视和眼球震颤 )及双眼BCDVA方面的差异有统计学意义 (P<0 05)。结论　儿童白内障摘除后房型IOL植入术后患者可获得较好视力和一定双眼视功能,但手术效果与手术时机、晶状体混浊程度及合并症 (斜视和眼球震颤)等多种因素密切相关。     

先天性上斜肌麻痹患者28例术后双眼视功能变化

目的:探讨先天性上斜肌麻痹患者手术后双眼视功能变化。方法:先天性上斜肌麻痹患者28例手术前、后分别行斜视度和双眼视功能检查,统计分析其变化。结果:术后正位率89.3%。手术后有同时视、融合和立体视的患者分别增加9,13和13例,与术前相比差异具有显著性(P<0.05)。近立体视觉有明显改善。术前斜视度小、视力好且有融合功能的患者术后双眼视功能恢复好。结论:先天性上斜肌麻痹手术治疗在改善眼位的同时,双眼视功能部分得以重建。视功能恢复与视力、斜视度及有无融合功能有关。     

Effectiveness of retroequatorial recession surgery in congenital nystagmus

OBJECTIVE: The aim of this retrospective study was to evaluate the effectiveness of the retroequatorial recession technique in the surgical treatment of nystagmus and associated strabismus. METHOD: The effect of large recessions of four horizontal extraocular muscles was analyzed qualitatively in seven patients with congenital nystagmus. RESULTS: The ages of the patients ranged between 12 and 42 years (mean, 21.5 years). Of the seven patients, four had motor and three had sensory nystagmus. Visual acuity increased in five (71.4%) patients. Planned recovery of associated strabismus was attained in six patients (85.7%). In five patients who had abnormal head position, the head positions and nystagmus intensities were found to be decreased. No postoperative complication, such as diplopia, was encountered. CONCLUSION: The retroequatorial recession technique is preferable in patients who have congenital nystagmus, especially in the presence of horizontal squint associated with low visual acuity, because of its success in increasing visual acuity and decreasing the amplitude of nystagmus, its reversibility, making posssible new operations in the future, as well as the absence of any significant complications.