Department of Ophthalmology, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Department of Anesthesiology, Critical Care and Pain Medicine, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts.
Ophthalmology. 2024 Mar;131(3):341-348. doi: 10.1016/j.ophtha.2023.09.022. Epub 2023 Sep 22.
To determine the sensitivity, specificity, and cutoff of macular ganglion cell layer (GCL) volume consistent with optic atrophy in children with syndromic craniosynostosis and to investigate factors independently associated with reduction in GCL volume.
Retrospective cross-sectional study.
Patients with syndromic craniosynostosis evaluated at Boston Children's Hospital (2010-2022) with reliable macular OCT scans.
The latest ophthalmic examination that included OCT macula scans was identified. Age at examination, sex, ethnicity, best-corrected logarithm of the minimum angle of resolution (logMAR) visual acuity, cycloplegic refraction, and funduscopic optic nerve appearance were recorded in addition to history of primary or recurrent elevation in intracranial pressure (ICP), Chiari malformation, and obstructive sleep apnea (OSA). Spectral-domain OCT software quantified segmentation of macula retinal layers and was checked manually.
The primary outcome was determining sensitivity, specificity, and optimal cutoff of GCL volume consistent with optic atrophy. The secondary outcome was determining whether previously elevated ICP, OSA, Chiari malformation, craniosynostosis diagnosis, logMAR visual acuity, age, or sex were independently associated with lower GCL volume.
Median age at examination was 11.9 years (interquartile range, 8.5-14.8 years). Fifty-eight of 61 patients (112 eyes) had reliable macula scans, 74% were female, and syndromes represented were Apert (n = 14), Crouzon (n = 17), Muenke (n = 6), Pfeiffer (n = 6), and Saethre-Chotzen (n = 15). Optimal cutoff identifying optic atrophy was a GCL volume < 1.02 mm with a sensitivity of 83% and specificity of 77%. Univariate analysis demonstrated that significantly lower macular GCL volume was associated with optic atrophy on fundus examination (P < 0.001), Apert syndrome (P < 0.001), history of elevated ICP (P = 0.015), Chiari malformation (P = 0.001), OSA (P < 0.001), male sex (P = 0.027), and worse logMAR visual acuity (P < 0.001). Multivariable median regression analysis confirmed that only OSA (P = 0.005), optic atrophy on fundus examination (P = 0.003), and worse logMAR visual acuity (P = 0.042) were independently associated with lower GCL volume.
Surveillance for optic atrophy by GCL volume may be useful in a population where cognitive skills can limit acquisition of other key ophthalmic measures. It is noteworthy that OSA is also associated with lower GLC volume in this population.
FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
确定与综合征型颅缝早闭患儿视神经萎缩一致的黄斑神经节细胞层（GCL）体积的敏感性、特异性和截断值，并探讨与 GCL 体积减少相关的独立因素。
回顾性横断面研究。
在波士顿儿童医院（2010-2022 年）接受评估的患有综合征型颅缝早闭的患者，且具有可靠的黄斑 OCT 扫描。
确定了最新的眼科检查，包括 OCT 黄斑扫描。记录了检查时的年龄、性别、种族、最佳矫正最小视角分辨率（logMAR）视力、睫状肌麻痹验光和眼底视神经外观，此外还记录了颅内压（ICP）原发性或复发性升高、Chiari 畸形和阻塞性睡眠呼吸暂停（OSA）的病史。谱域 OCT 软件对黄斑视网膜层进行了定量分割，并进行了手动检查。
主要结局是确定与视神经萎缩一致的 GCL 体积的敏感性、特异性和最佳截断值。次要结局是确定先前升高的 ICP、OSA、Chiari 畸形、颅缝早闭诊断、logMAR 视力、年龄或性别是否与较低的 GCL 体积独立相关。
检查时的中位年龄为 11.9 岁（四分位间距，8.5-14.8 岁）。58 名 61 名患者（112 只眼）的黄斑扫描可靠，74%为女性，代表的综合征分别为 Apert（n=14）、Crouzon（n=17）、Muenke（n=6）、Pfeiffer（n=6）和 Saethre-Chotzen（n=15）。确定视神经萎缩的最佳截断值为 GCL 体积<1.02mm，其敏感性为 83%，特异性为 77%。单变量分析表明，眼底检查发现视神经萎缩（P<0.001）、Apert 综合征（P<0.001）、ICP 升高史（P=0.015）、Chiari 畸形（P=0.001）、OSA（P<0.001）、男性（P=0.027）和 logMAR 视力更差（P<0.001）与黄斑 GCL 体积显著降低相关。多变量中位数回归分析证实，只有 OSA（P=0.005）、眼底检查发现视神经萎缩（P=0.003）和 logMAR 视力更差（P=0.042）与较低的 GCL 体积独立相关。
GCL 体积监测可能对认知能力有限而无法获得其他关键眼科测量的人群中视神经萎缩有用。值得注意的是，在该人群中，OSA 也与较低的 GLC 体积有关。
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