Objective

Port-wine birthmark (PWB) is a common occurrence in the newborn, and general pediatricians, dermatologists, and ophthalmologists are often called on to make an assessment of risk for Sturge-Weber syndrome (SWS) due to workforce shortages in pediatric neurologists and MRI’s low sensitivity for SWS brain involvement in infants. We therefore aimed to develop a quantitative EEG (qEEG) approach to safely screen young infants with PWB for SWS risk and optimal timing of diagnostic MRI.

Methods

Forty-eight infants (prior to first birthday) underwent EEG recording. Signal processing methods compared voltage between left and right sides using a previously defined pipeline and diagnostic threshold. In this test sample, we compared sensitivity/specificity of the qEEG metric against MRI performed after the first birthday. We also used likelihood ratio testing to determine whether qEEG adds incremental information beyond topographical extent of PWB, another risk marker of brain involvement.

Results

qEEG helped predict SWS risk in the first year of life (p = 0.031), with a sensitivity of 50% and a specificity of 81%. It added about 40% incremental information beyond PWB extent alone (p = 0.042).

Conclusion

qEEG adds information to risk prediction in infants with facial PWB.

Significance

qEEG can be used to help determine whether to obtain an MRI in the first year of life. The data collected can assist in developing a predictive model risk calculator that incorporates both PWB extent and qEEG results, which can be validated and then employed in the community.

中文翻译：

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定量脑电图提高了对带有葡萄酒胎记婴儿 Sturge-Weber 综合征的预测

客观的

葡萄酒胎记 (PWB) 在新生儿中很常见，由于儿科神经科医生和 MRI 的劳动力短缺，普通儿科医生、皮肤科医生和眼科医生经常被要求对 Sturge-Weber 综合征 (SWS) 的风险进行评估婴儿 SWS 脑受累的敏感性低。因此，我们旨在开发一种定量脑电图 (qEEG) 方法，以安全地筛查患有 PWB 的小婴儿的 SWS 风险和诊断 MRI 的最佳时机。

方法

48 名婴儿（一岁之前）接受了脑电图记录。信号处理方法使用先前定义的管道和诊断阈值比较左侧和右侧之间的电压。在这个测试样本中，我们比较了 qEEG 指标与一周岁后进行的 MRI 的敏感性/特异性。我们还使用似然比测试来确定 qEEG 是否增加了超出 PWB 地形范围的增量信息，这是大脑受累的另一个风险标志。

结果

qEEG 有助于预测出生后第一年的 SWS 风险 ( p  = 0.031)，敏感性为 50%，特异性为 81%。它在 PWB 范围之外增加了大约 40% 的增量信息（p  = 0.042）。

结论

qEEG 为面部 PWB 婴儿的风险预测增加了信息。

意义

qEEG 可用于帮助确定是否在生命的第一年进行 MRI。收集的数据可以帮助开发一个预测模型风险计算器，该计算器包含 PWB 范围和 qEEG 结果，可以验证然后在社区中使用。