Departments of1Neurosurgery and.
2Department of Neurosurgery, Section of Pediatric Neurosurgery, Oklahoma Children's Hospital; and.
J Neurosurg Pediatr. 2023 Jun 16;32(3):384-393. doi: 10.3171/2023.4.PEDS22494. Print 2023 Sep 1.
Trigeminal neuralgia (TN) is a rare disorder, affecting 4-13 per 100,000 people annually. Only 1%-1.5% of these cases are reported before the age of 18 years. The initial management of pediatric TN is based on symptomatic treatment, using first-line medications including carbamazepine, based on data from adult treatment studies. Many of these children are refractory to medication and undergo excessive workup and ineffective therapies before neurosurgical referral. The objective of this study was to perform a comprehensive review of literature-reported pediatric-onset TN, analyzing demographics, diagnostic practices, and complications in this population.
An institutional, retrospective chart review was performed to identify patients younger than 18 years old who were diagnosed with classic TN and referred for surgical evaluation at Oklahoma Children's Hospital (OCH). A systematic review of all pediatric patients undergoing microvascular decompression (MVD) for pediatric-onset TN was also performed.
Three patients from OCH were identified, ranging in age from 3 to 11 years. All 3 patients had received multiple ineffective medical therapies before referral for neurosurgical evaluation. In 2 cases, imaging demonstrated potential vascular compression of the trigeminal nerve. In the final case, imaging demonstrated no vascular compression, but compression was found at the time of MVD. All cases were successfully treated using MVD. The literature review identified 49 cases of pediatric TN treated using MVD. All 49 cases were identified as classic TN with neurovascular compression demonstrated either on imaging or found at the time of surgery. The average age at symptom onset was 11.72 years, while the average age at MVD was 19.6 years. The average duration of symptoms before undergoing MVD was 8.2 years. There was a female predominance in the literature review, with a female-to-male ratio of 1.88:1. The right side was involved in 56% of cases, the left side in 40%, and bilateral involvement occurred in 4% of cases. The most common distribution of TN was both V2/3 branches (38%) of the trigeminal nerve, followed by solely the V2 branch (22%). A successful outcome (Barrow Neurological Institute pain intensity score of I or II) was reported in 79% of patients at the last follow-up (mean 69.1 months).
There are currently no guidelines for diagnosing or treating pediatric TN. MVD is a safe and effective option in this patient population. Early evaluation for surgical intervention could be critical for early pain relief and reduced disease morbidity.
三叉神经痛（TN）是一种罕见疾病，每年每 10 万人中有 4-13 人发病。这些病例中只有 1%-1.5%发生在 18 岁之前。儿科 TN 的初始治疗基于对症治疗，使用卡马西平等一线药物，这些药物的数据来自成人治疗研究。这些儿童中有许多对药物治疗无效，在接受神经外科转诊之前，进行了过多的检查和无效的治疗。本研究的目的是对文献中报告的儿科发作性 TN 进行全面回顾，分析该人群的人口统计学、诊断实践和并发症。
对俄克拉荷马儿童医院（OCH）诊断为经典 TN 并接受手术评估的小于 18 岁的患者进行机构回顾性图表审查。还对所有接受微血管减压术（MVD）治疗儿科发作性 TN 的儿科患者进行了系统回顾。
在 OCH 发现了 3 名年龄在 3 至 11 岁的患者。所有 3 名患者在接受神经外科评估前均接受了多次无效的药物治疗。在 2 例中，影像学显示三叉神经可能受到血管压迫。在最后 1 例中，影像学显示无血管压迫，但在 MVD 时发现有压迫。所有病例均成功采用 MVD 治疗。文献复习发现 49 例儿科 TN 采用 MVD 治疗。所有 49 例均被确定为经典 TN，影像学显示或手术时发现有神经血管压迫。症状发作的平均年龄为 11.72 岁，而 MVD 的平均年龄为 19.6 岁。在接受 MVD 之前，症状的平均持续时间为 8.2 年。文献复习中女性比例较高，女性与男性的比例为 1.88:1。右侧受累占 56%，左侧受累占 40%，双侧受累占 4%。TN 最常见的分布是三叉神经的第 2/3 分支（38%），其次是第 2 分支（22%）。在最后一次随访（平均 69.1 个月）时，79%的患者报告了良好的结局（巴罗神经研究所疼痛强度评分 I 或 II）。
目前尚无诊断或治疗儿科 TN 的指南。MVD 是该患者群体的一种安全有效的选择。早期评估手术干预可能对早期缓解疼痛和降低疾病发病率至关重要。
