儿童进行性限制性斜视临床特征分析及手术疗效观察

Clinical characteristics and surgical outcomes in pediatric progressive restrictive strabismus

目的探讨儿童进行性限制性斜视的临床特征、影像学表现、组织病理学特点及手术效果。方法回顾性病例系列研究。收集2017年6月至2022年10月在天津市眼科医院就诊的进行性限制性斜视9例(9只眼)患儿的资料,比较患儿双眼眼球突出度及受累眼在第一眼位及水平内外转时睑裂高度的改变,总结患儿的临床特征并对行斜视矫正手术患儿术中情况、手术效果及术后组织病理学结果进行分析。采用Wilcoxon符号秩检验和Friedman双向秩方差分析进行统计学处理。结果9例患儿均为单眼发病,其中男性4例,女性5例;右眼3例,左眼6例;发病年龄为2~40个月。患儿双眼眼球突出度右眼为13.00(12.00,13.00)mm,左眼为12.00(12.00,13.50)mm,差异无统计学意义(Z=-1.00,P=0.317);受累眼睑裂高度内转为8.00(7.25,8.00)mm,第一眼位为7.50(7.00,8.00)mm,外转为8.00(7.75,8.00)mm,差异无统计学意义(χ^(2)=1.00,P=0.607)。所有患儿头颅CT或MRI检查均未见明显异常,血清学及免疫学检查亦未见明显异常,而眼眶CT或MRI检查均提示不同眼外肌肌腹增粗。9例患儿中6例行斜视矫正手术,手术患儿术前均行被动牵拉试验,受累眼向各个方向运动均受到不同程度限制。术后第一眼位正位,但眼球运动仍受限。对3例患儿术中眼外肌取材行组织病理学检查,1例提示主要为增生的胶原纤维;1例在弥漫型增生的胶原纤维之间散在束状排列的平滑肌纤维,1例表现为异常增生的横纹肌纤维,部分横纹肌纤维直径大小不一,配对盒(PAX)基因阳性的卫星细胞增多,以表达慢肌球蛋白的肌纤维为主。结论儿童进行性限制性斜视呈限制性改变,受累眼眼球突出度及睑裂高度无明显改变,影像学检查显示受累眼外肌肌腹增粗;被动牵拉试验发现受累眼向各个方向运动均受到不同程度限制,虽然斜视矫正手术可以改善眼位,但术后眼球运动仍存在受限因素;部分患儿组织病理学表现为骨骼肌纤维或胶原纤维异常增生。

Objective To investigate the clinical features,imaging manifestations,histopathological characteristics,and surgical outcomes in pediatric progressive restrictive strabismus.Methods A retrospective case series study was conducted,including data from 9 cases(9 eyes)of pediatric progressive restrictive strabismus treated at Tianjin Eye Hospital from June 2017 to October 2022.The study compared the degree of globe protrusion in both eyes,changes in eyelid fissure height during internal and external rotation in the primary gaze,summarized clinical characteristics,and analyzed intraoperative conditions,surgical outcomes and postoperative histopathological results of strabismus correction surgery.Statistical analysis was performed using Wilcoxon signed-rank test and Friedman two-way analysis of variance.Results All 9 cases involved unilateral onset,with 4 males and 5 females.Three cases affected the right eye,and six affected the left eye.Onset age ranged from 2 to 40 months.The degree of globe protrusion in the affected eyes was 13.00(12.00,13.00)mm for the right eye and 12.00(12.00,13.50)mm for the left eye,with no statistically significant difference(Z=-1.00,P=0.317).There were no significant changes in eyelid fissure height during internal rotation[8.00(7.25,8.00)mm],primary gaze[7.50(7.00,8.00)mm],and external rotation[8.00(7.75,8.00)mm]in the affected eyes(χ^(2)=1.00,P=0.607).No apparent abnormalities were observed in head CT or MRI scans,serum,or immunological tests.However,orbital CT or MRI scans indicated thickening of different extraocular muscle bellies.Six out of nine cases underwent strabismus correction surgery,and postoperative examination revealed restriction in eye movement despite achieving orthophoria in the primary gaze.Tissue pathology of three cases showed increased collagen fiber proliferation in one,scattered bundles of smooth muscle fibers amid diffuse collagen fiber proliferation in another,and abnormal proliferation of striated muscle fibers with varying diameters,increased paired box(PAX)7-positive satellite cells expressing slow muscle myosin in the third case.Conclusions Pediatric progressive restrictive strabismus presents with restrictive changes,without significant alterations in globe protrusion and eyelid fissure height.Imaging examinations reveal thickening of the extraocular muscle bellies in the affected eye.Although strabismus correction surgery improves eye position,postoperative eye movement remains restricted.Histopathological findings in some cases show abnormal proliferation of skeletal muscle fibers or collagen fibers.