Abstract：
To explore the biometric characteristics of the ciliary body in patients with Marfan syndrome (MFS) and ectopia lentis (EL).
Cross-sectional study.
Seventy-two consecutive patients with MFS and EL and 72 nondiseased control subjects were recruited. Ciliary body biometric parameters such as ciliary muscle cross-sectional area at 2000 µm from the scleral spur (CMA2000), ciliary muscle thickness at 1000 µm from the scleral spur (CMT1000), and maximum ciliary body thickness (CBTmax) were measured from multiple directions with ultrasound biomicroscopy (UBM). The relationship between ciliary body parameters and other ocular characteristics was also evaluated.
Average CMA2000, CMT1000, and CBTmax were 0.692 ± 0.015 mm2, 0.405 ± 0.010 mm, and 0.855 ± 0.023 mm in eyes of patients with MFS, respectively, and were significantly smaller than these values in control subjects (all P < .001). The prevalence of ciliary body thinning was 22.2% in the MFS group vs 0 in the control group (P < .001); eyes with more severe EL had smaller CMA2000 (P = .050), thinner CMT1000 (P = .022), and shorter CBTmax (P = .015). Patients with microspherophakia (MSP) had even smaller CMA2000 (P = .033) and CMT1000 (P = .044) than those without MSP. The most common subluxation direction was in the superonasal quadrant (n = 25; 39.7%), which probably correlates with the thinnest CMT1000 in the inferotemporal quadrant (P = .005).
Patients with MFS and EL had thinner ciliary muscles, shorter ciliary processes, and a higher prevalence of ciliary body thinning, especially those with MSP. Both the extent and direction of subluxation were associated with ciliary body biometry..
Cross-sectional study.
Seventy-two consecutive patients with MFS and EL and 72 nondiseased control subjects were recruited. Ciliary body biometric parameters such as ciliary muscle cross-sectional area at 2000 µm from the scleral spur (CMA2000), ciliary muscle thickness at 1000 µm from the scleral spur (CMT1000), and maximum ciliary body thickness (CBTmax) were measured from multiple directions with ultrasound biomicroscopy (UBM). The relationship between ciliary body parameters and other ocular characteristics was also evaluated.
Average CMA2000, CMT1000, and CBTmax were 0.692 ± 0.015 mm2, 0.405 ± 0.010 mm, and 0.855 ± 0.023 mm in eyes of patients with MFS, respectively, and were significantly smaller than these values in control subjects (all P < .001). The prevalence of ciliary body thinning was 22.2% in the MFS group vs 0 in the control group (P < .001); eyes with more severe EL had smaller CMA2000 (P = .050), thinner CMT1000 (P = .022), and shorter CBTmax (P = .015). Patients with microspherophakia (MSP) had even smaller CMA2000 (P = .033) and CMT1000 (P = .044) than those without MSP. The most common subluxation direction was in the superonasal quadrant (n = 25; 39.7%), which probably correlates with the thinnest CMT1000 in the inferotemporal quadrant (P = .005).
Patients with MFS and EL had thinner ciliary muscles, shorter ciliary processes, and a higher prevalence of ciliary body thinning, especially those with MSP. Both the extent and direction of subluxation were associated with ciliary body biometry..
摘要：
目的：探讨马凡氏综合征（MFS）和扁桃体外翻（EL）患者睫状体的生物特征。
方法：横断面研究。
方法：招募72例连续的ELMFS患者和72例非疾病对照。睫状体生物特征参数，例如距巩膜骨刺2000μm处的睫状肌横截面积（CMA2000），距巩膜骨刺1000μm处的睫状肌厚度（CMT1000），用超声生物显微镜（UBM）从多个方向测量最大睫状体厚度（CBTmax）。还评估了睫状体参数与其他眼部特征之间的关系。
结果：平均CMA2000，CMT1000和CBTmax为0.692±0.015mm2，0.405±0.010mm，和0.855±0.023mm的MFS眼睛，分别,并且显著小于对照组(所有p<0.001)。MFS组睫状体变薄的患病率为22.2％，对照组为0（p<0.001）；EL较严重的眼睛CMA2000较小（p=0.050），较薄的CMT1000（p=0.022）和较短的CBTmax（p=0.015）。与没有MSP的患者相比，患有微球（MSP）的患者的CMA2000（p=0.033）和CMT1000（p=0.044）甚至更小。最常见的半脱位方向是在鼻上象限（25，39.7%），这可能与下时象限中最薄的CMT1000相关(p=0.005)。
结论：患有EL的MFS患者睫状肌变薄，较短的纤毛过程，睫状体变薄的患病率更高，尤其是那些MSP。半脱位的程度和方向均与睫状体生物特征有关。
方法：横断面研究。
方法：招募72例连续的ELMFS患者和72例非疾病对照。睫状体生物特征参数，例如距巩膜骨刺2000μm处的睫状肌横截面积（CMA2000），距巩膜骨刺1000μm处的睫状肌厚度（CMT1000），用超声生物显微镜（UBM）从多个方向测量最大睫状体厚度（CBTmax）。还评估了睫状体参数与其他眼部特征之间的关系。
结果：平均CMA2000，CMT1000和CBTmax为0.692±0.015mm2，0.405±0.010mm，和0.855±0.023mm的MFS眼睛，分别,并且显著小于对照组(所有p<0.001)。MFS组睫状体变薄的患病率为22.2％，对照组为0（p<0.001）；EL较严重的眼睛CMA2000较小（p=0.050），较薄的CMT1000（p=0.022）和较短的CBTmax（p=0.015）。与没有MSP的患者相比，患有微球（MSP）的患者的CMA2000（p=0.033）和CMT1000（p=0.044）甚至更小。最常见的半脱位方向是在鼻上象限（25，39.7%），这可能与下时象限中最薄的CMT1000相关(p=0.005)。
结论：患有EL的MFS患者睫状肌变薄，较短的纤毛过程，睫状体变薄的患病率更高，尤其是那些MSP。半脱位的程度和方向均与睫状体生物特征有关。
