马凡综合征伴晶状体异位患者睫状体的形态计量评估：超声生物显微镜的定量研究。

Morphometric Assessment of the Ciliary Body in Patients With Marfan Syndrome and Ectopia Lentis: A Quantitative Study Using Ultrasound Biomicroscopy.

机构信息

From the Eye Institute and Department of Ophthalmology, Eye and ENT Hospital, Fudan University; NHC Key Laboratory of Myopia (Fudan University), Key Laboratory of Myopia, Chinese Academy of Medical Sciences; and the Shanghai Key Laboratory of Visual Impairment and Restoration, Shanghai, China.

出版信息

Am J Ophthalmol. 2023 Jul;251:24-31. doi: 10.1016/j.ajo.2023.03.003. Epub 2023 Mar 21.

DOI:10.1016/j.ajo.2023.03.003

PMID:36948371

Abstract

PURPOSE

To explore the biometric characteristics of the ciliary body in patients with Marfan syndrome (MFS) and ectopia lentis (EL).

DESIGN

Cross-sectional study.

METHODS

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.

RESULTS

Average CMA2000, CMT1000, and CBTmax were 0.692 ± 0.015 mm, 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).

CONCLUSIONS

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

探讨马凡综合征（MFS）伴晶状体异位（EL）患者睫状体的生物测量特征。

设计

横断面研究。

方法

招募了 72 例连续的 MFS 伴 EL 患者和 72 名无眼部疾病的对照组患者。使用超声生物显微镜（UBM）从多个方向测量睫状体生物测量参数，包括距巩膜突 2000 µm 处的睫状肌横截面积（CMA2000）、距巩膜突 1000 µm 处的睫状肌厚度（CMT1000）和最大睫状体厚度（CBTmax）。还评估了睫状体参数与其他眼部特征之间的关系。

结果

MFS 组患者的平均 CMA2000、CMT1000 和 CBTmax 分别为 0.692±0.015mm、0.405±0.010mm 和 0.855±0.023mm，均显著小于对照组（均 P<.001）。MFS 组睫状体变薄的患病率为 22.2%，而对照组为 0（P<.001）；晶状体异位更严重的眼，CMA2000 更小（P=.050），CMT1000 更薄（P=.022），CBTmax 更短（P=.015）。伴有小眼球（MSP）的患者的 CMA2000 和 CMT1000 甚至更小（P=.033 和 P=.044）。最常见的半脱位方向是超鼻上象限（n=25；39.7%），这可能与下颞象限最薄的 CMT1000 有关（P=.005）。