目的研究先天性眼外肌纤维化（congenital fibrosis of the extraocular muscles，CFEOM）家系发病者眼外肌及眼运动神经的影像学特征，探讨先天性眼外肌纤维化的发病机制。方法收集2个CFEOM家系，家系一临床表现符合CFEOM1，家系二临...目的研究先天性眼外肌纤维化（congenital fibrosis of the extraocular muscles，CFEOM）家系发病者眼外肌及眼运动神经的影像学特征，探讨先天性眼外肌纤维化的发病机制。方法收集2个CFEOM家系，家系一临床表现符合CFEOM1，家系二临床表现符合CFEOM3。对两家系共17名患者（家系－3名患者，家系二14名患者，其中男性5名，女性12名，年龄范围8—62岁，平均年龄34．4岁，34只眼）进行高分辨率MRI扫描，随机抽取18个正常成人（男女各9人，年龄范围10—59岁，平均年龄35．1岁，36只眼）作为对照。扫描方法：眼眶部行3mm层厚、T1加权MRI扫描；颅内行1mm层厚、3DFIESTA扫描；比较2组眼外肌、眼运动神经、上睑提肌和视神经的变化。对数据进行正态性检验并用t检验对数据进行统计学分析。结果MRI扫描显示发病者外直肌（LR）、内直肌（MR）、上直肌（SR）、下直肌（IR）及上斜肌（10）体积较正常对照组小，尤以上直肌明显，差异有统计学意义（LR：t=－5．535，P=0．003；MR：t=－7．187，P=0．001；SR：t=－23．749，P=0．001；IR：t=－6．907，P=0．001；IO：t=－5．057，P=0．004）；眼运动神经（动眼神经、展神经）的颅内段截面积较正常对照组小，差异有统计学意义（t=－23．749，－11．721，P〈0．05）；视神经较细（t=2．292，P=0．025），且均向鼻上方移位；上睑提肌发育不良。结论由眼运动神经异常支配继发眼外肌组织被致密纤维组织代替所致，可能与动眼神经核和滑车神经核发育缺陷有关。CFEOM的病因为神经源性。展开更多
AIM: To investigate changes in fundus excyclotorsion after inferior oblique myectomy or myotomy. METHODS: The records of 21 patients undergoing strabismus surgery by a single surgeon between 2009 and 2012 were examine...AIM: To investigate changes in fundus excyclotorsion after inferior oblique myectomy or myotomy. METHODS: The records of 21 patients undergoing strabismus surgery by a single surgeon between 2009 and 2012 were examined. Only patients who had undergone an inferior oblique myectomy or myotomy,with or without horizontal rectus muscle surgery, were evaluated. Digital fundus photographs were obtained,and the angle formed by a horizontal line passing through the optic disc center and a reference line connecting the foveola and optic disc center was measured. Associated clinical factors examined include age at the time of surgery, presence or absence of a head tilt, degree of preoperative vertical deviation,torsional angle, inferior oblique muscle overaction/superior oblique muscle underaction, and surgery laterality. Whether the procedure was performed alone or in combination with a horizontal rectus muscle surgery was also examined. RESULTS: Mean preoperative torsional angle was12. 0 ± 6. 4 °, which decreased to 6. 9 ± 5. 7 ° after surgery(P 【0.001, paired t-test). Torsional angle also decreased from 15. 1 ± 7. 0 ° to 6. 2 ± 4. 3 ° in the myectomy group(P 【0.001, paired t-test) but there were no significant changes in the myotomy group(P =0.093, Wilcoxon signed rank test). Multivariable linear regression analysis showed that preoperative torsional angle, degree ofinferior oblique overaction, and age at surgery independently and significantly affected postoperative torsional angle.CONCLUSION: Mean torsional angle decreased after inferior oblique myectomy. Degree of preoperative torsional angle, inferior oblique overaction, and age at surgery influence postoperative torsional angle.展开更多
文摘目的研究先天性眼外肌纤维化（congenital fibrosis of the extraocular muscles，CFEOM）家系发病者眼外肌及眼运动神经的影像学特征，探讨先天性眼外肌纤维化的发病机制。方法收集2个CFEOM家系，家系一临床表现符合CFEOM1，家系二临床表现符合CFEOM3。对两家系共17名患者（家系－3名患者，家系二14名患者，其中男性5名，女性12名，年龄范围8—62岁，平均年龄34．4岁，34只眼）进行高分辨率MRI扫描，随机抽取18个正常成人（男女各9人，年龄范围10—59岁，平均年龄35．1岁，36只眼）作为对照。扫描方法：眼眶部行3mm层厚、T1加权MRI扫描；颅内行1mm层厚、3DFIESTA扫描；比较2组眼外肌、眼运动神经、上睑提肌和视神经的变化。对数据进行正态性检验并用t检验对数据进行统计学分析。结果MRI扫描显示发病者外直肌（LR）、内直肌（MR）、上直肌（SR）、下直肌（IR）及上斜肌（10）体积较正常对照组小，尤以上直肌明显，差异有统计学意义（LR：t=－5．535，P=0．003；MR：t=－7．187，P=0．001；SR：t=－23．749，P=0．001；IR：t=－6．907，P=0．001；IO：t=－5．057，P=0．004）；眼运动神经（动眼神经、展神经）的颅内段截面积较正常对照组小，差异有统计学意义（t=－23．749，－11．721，P〈0．05）；视神经较细（t=2．292，P=0．025），且均向鼻上方移位；上睑提肌发育不良。结论由眼运动神经异常支配继发眼外肌组织被致密纤维组织代替所致，可能与动眼神经核和滑车神经核发育缺陷有关。CFEOM的病因为神经源性。
基金Supported by Basic Science Research Program through the National Research Foundation of Korea (NRF) funded by the Ministry of Education, Science and Technology (No.2012R1A1A2004809)
文摘AIM: To investigate changes in fundus excyclotorsion after inferior oblique myectomy or myotomy. METHODS: The records of 21 patients undergoing strabismus surgery by a single surgeon between 2009 and 2012 were examined. Only patients who had undergone an inferior oblique myectomy or myotomy,with or without horizontal rectus muscle surgery, were evaluated. Digital fundus photographs were obtained,and the angle formed by a horizontal line passing through the optic disc center and a reference line connecting the foveola and optic disc center was measured. Associated clinical factors examined include age at the time of surgery, presence or absence of a head tilt, degree of preoperative vertical deviation,torsional angle, inferior oblique muscle overaction/superior oblique muscle underaction, and surgery laterality. Whether the procedure was performed alone or in combination with a horizontal rectus muscle surgery was also examined. RESULTS: Mean preoperative torsional angle was12. 0 ± 6. 4 °, which decreased to 6. 9 ± 5. 7 ° after surgery(P 【0.001, paired t-test). Torsional angle also decreased from 15. 1 ± 7. 0 ° to 6. 2 ± 4. 3 ° in the myectomy group(P 【0.001, paired t-test) but there were no significant changes in the myotomy group(P =0.093, Wilcoxon signed rank test). Multivariable linear regression analysis showed that preoperative torsional angle, degree ofinferior oblique overaction, and age at surgery independently and significantly affected postoperative torsional angle.CONCLUSION: Mean torsional angle decreased after inferior oblique myectomy. Degree of preoperative torsional angle, inferior oblique overaction, and age at surgery influence postoperative torsional angle.
