PURPOSE

This study is the first to evaluate the prevalence of retinal thinning and the correlation with papilledema and visual acuity (VA) in a large population with craniosynostosis.

DESIGN

Prospective clinical cohort study.

METHODS

All and complex patients syndromic and complex with craniosynostosis who visited the only national referral center between 2018 and 2020 were included. Retinal layers were segmented using optical coherence tomography. Patients were seen by an ophthalmologist for VA assessment and fundoscopy. Multivariate regression models were developed to evaluate correlations between retinal thickness, papilledema and VA.

RESULTS

We included 127 patients. Retinal thinning was most prevalent in the peripapillary retinal nerve fiber layer (≤38%). A longer duration of papilledema in early childhood correlated with an increased peripapillary retinal nerve fiber layer and total retinal thickness optic nerve head later in life (+0.3 ± 0.2, P = .04 and +1.0 ± 1.0, P = .003); however, its thickness was not correlated with the VA (P = .20 and P = .53). Macular retinal thinning was associated with a worse VA (P = .01); however, it was not correlated with the duration of papilledema (P = .95).

CONCLUSIONS

Following a preventative treatment strategy for syndromic and complex craniosynostosis, the prevalence of retinal ONH thinning is low. Although the prevalence of peripapillary retinal nerve fiber layer thinning is considerable, its thickness is not correlated with VA. In contrast, macular thinning is correlated with worse VA scores and should, therefore, be evaluated during follow-up. Future studies should evaluate the (1) causative mechanism for macular thinning, (2) correlation between the time to surgery and macular thinning, and (3) results of reactive treatment strategies and compare those results to the current study.

中文翻译：

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定量视网膜形态学及其与综合征和复杂颅缝早闭中视乳头水肿和视力的关联：光学相干断层扫描研究

目的

这项研究首次评估了颅缝早闭的大量人群中视网膜变薄的患病率以及与视乳头水肿和视力 (VA) 的相关性。

设计

前瞻性临床队列研究。

方法

纳入了 2018 年至 2020 年间访问唯一国家转诊中心的所有和复杂的颅缝早闭综合征和复杂患者。使用光学相干断层扫描对视网膜层进行分割。眼科医生对患者进行 VA 评估和眼底镜检查。开发了多变量回归模型来评估视网膜厚度、视乳头水肿和 VA 之间的相关性。

结果

我们纳入了 127 名患者。视网膜变薄在视盘周围视网膜神经纤维层最为普遍（≤38%）。儿童早期视乳头水肿持续时间较长与生命后期视乳头周围视网膜神经纤维层和视神经乳头总视网膜厚度增加相关（+0.3 ± 0.2，P  = .04 和 +1.0 ± 1.0，P  = .003）；然而，其厚度与 VA 无关（P  = .20 和P  = .53）。黄斑视网膜变薄与更差的 VA 相关（P  = .01）；然而，它与视乳头水肿的持续时间无关（P  = .95）。

结论

在对综合征和复杂颅缝早闭采取预防性治疗策略后，视网膜 ONH 变薄的患病率很低。尽管视乳头周围视网膜神经纤维层变薄的患病率相当高，但其厚度与 VA 无关。相比之下，黄斑变薄与较差的 VA 评分相关，因此应在随访期间进行评估。未来的研究应评估 (1) 黄斑变薄的致病机制，(2) 手术时间与黄斑变薄之间的相关性，以及 (3) 反应性治疗策略的结果，并将这些结果与当前研究进行比较。