ABSTRACT
Background Congenital nystagmus is a common disease threatening visual function.The clinical description of congenital nystagmus is still not enough now. Objective The aim of this study was to report the motor and sensory characteristics of congenital nystagmus. Methods A retrospective clinical case analysis was designed.The clinical data of 376 patients with congenital nystagmus were collected.These patients were diagnosed in Henan Eye Institute from January,2005 to August,2011.The clinical characteristics of patients,including age,pedigree analysis,nystagmus classification,ocular alignment,visual acuity,refractive error,stereoacuity and oscillopia,was summarized.Informed consent from all patients and custodian were obtained. Results The age distribution of most patients was ＞5-10 years and ＞ 15-20 years,with the percentage 24.73％ and 24.20％,respectively.Of 376 patients,sensory defect nystagmus was 172 cases,and that of motor sensory defect was 204 cases.The most common inheritance pattern was autosomal dominant.Conjugate uniplanar horizontal oscillations were found in 73.94％patients,and strabismus was found in 66.36％ in this study.Sensory defected subjects did not show a statistically significantly higher strabismus incidence when compared with the idiopaths (x2 =3.048,P =0.081 ).The binocular distance vision in all the subjects was declined in comparison with normal eyes,the best corrected vision was 0.27 ±0.11 and 0.50± 0.13 in sensory defected nystagmus and idiopaths nystagmus respectively,showing a significant difference( t =16.495,P =0.000 ).The incidence of refractive error in this study was 77.62％.Incidence of astigmatism(75.17％ ) was higher than that of myopia and hyperopia ( 39.03％ and 36.12％ ).No significant difference was seen in refractive error incidence between the sensory defected and the idiopaths (x2 =1.337,P =0.248).Patients of 65.18％ displayed non-stereoacuity or very gross acuity levels (3000) in this study,and the patients with idiopathic nystagmus showed better stereoacuity level in comparison with sensory defected ones (x2 =7.058,P =0.008 ).Five of 313 patients existed experienced oscilopla under binocular viewing conditions.Conclusions Autosomal dominant inheritance is the most common pattern of congenital nystagmus.Conjugate uniplanar horizontal oscillation is thought to be a common type of nystagmus.Incidence of strabismus is higher,and visual acuity and stereoaeuity are worse in congenital nystagmus than in common population.Oscillopia is seldom found in congenital nystagmus.
ABSTRACT
Background The surgery for congenital idiopathic nystagmus has make great progression recent year,but the influence of surgery on visual function is closely concerned.Objective This work was to study the surgeries for congenital idiopathic nystagmus with or without strabismus and explore the effect of horizontal rectus muscle surgery on the visual function and ocular movement in congenital idiopathic nystagmus.Methods This was a case-observational study.Sixty cases with idiopathic congenital nystagmus were enrolled assigned to four groups as follows:The patients of group Ⅰ appeared the head postures of less than 15 ° and heterotropia less than 15△underwent extraocular proprioceptiou resection,and those of group Ⅱ presented with the head postures of less than 20° and heterotropia more than 15△ underwent extraocular proprioception resection and stabismus surgery.In the group Ⅲ,the patients had the head postures of more than 15° and heterotropia less than 15△ underwent Park's surgery and part of them underwent extraocular proprioception resection) and the cases in group Ⅳ were selected with the head postures of more than15° and heterotropia more than 15△ underwent Park' s surgery and strabismus surgery ( part of them underwent extraocular proprioception resection).Baseline and follow-up evaluations included the best distance and near corrected visual acuity,head posture,ocular movement and Titmus stereotest.The examination results were compared between before and after operation.Results One year after operation,monocular and binocular distance visual acuity improved significantly in all four groups ( monocular:t =6.00,8.94,11.76,16.41,P =0.00 ; binocular:t=4.81,P=0.01 ; t =6.27,4.25,4.18,P =0.00 ),but monocular and binocular near visual acuity improved significantly only in groups Ⅲ and Ⅳ after operation compared with before operation ( monocular:t=2.91,P=0.01 ; t =5.32,P =0.00 ; binocular:t =3.36,t =4.30,P =0.00 ).The compensatory head posture from 39 subjects was disappeared after operation,showing an evident difference between before and after surgery ( group Ⅲ:t =29.90,P=0.00;group Ⅳ:t=16.91,P=0.00).Strabismus were corrected rightly for 24 patients and undercorrection for 2 patients.( group Ⅱ:t =29.15,P =0.00 ; group Ⅳ:t =36.79,P =0.00 ).The binocular visual function of 23 cases with the age below 12 years were improved throughout the follow up duration ( t =12.06,P =0.00).Conclusions Park' s surgery,extraocular proprioception resection and strabismus corrective operation can improve the visual function and head posture in congenital idiopathic nystagmus patients.