Objective: 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.
Methods: 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.
Results: 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).
Conclusions: 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.
Keywords: case series; microvascular decompression; review; surgical technique; trigeminal neuralgia.