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Audrey A. Grossen, Courtney R. Iser, Amy B. Leming, Virendra R. Desai, and Deepti G. Chrusciel

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.

开云体育世界杯赔率

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

目前没有诊断指南r 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.

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兰斯·m·维伦纽夫Nangorgo j . CoulibalySyed M. Raza, Brittany Poinson, Deepti Chrusciel, and Virendra R. Desai

OBJECTIVE

Occipital neuralgia (ON) is a rare headache disorder characterized by sharp pain in the distribution of the greater occipital nerve (GON), lesser occipital nerve, or third occipital nerve. ON is commonly associated with traumatic injury, and effective identification and diagnosis can be difficult given the infrequent presentation and similarities to other pediatric headache disorders. While GON decompression has been well described in adults for refractory pain, there is a paucity of data in the pediatric population, with no previously published series on ON. The primary aim of this study was to identify the characteristics of pediatric patients with ON prior to surgical intervention and to describe the natural history of postoperative outcomes after decompression or neurectomy in a pediatric population.

开云体育世界杯赔率

A single-center retrospective case series was performed to evaluate factors predisposing children to refractory ON and the surgical efficacy of GON decompression or neurectomy. Six patients (mean age 15.0 ± 2.2 years) were identified for inclusion from October 2021 to October 2022. All patients had refractory ON as diagnosed by a pediatric neurologist. After medical therapy and repeated occipital nerve blocks failed, the patients were referred for GON decompression. Five patients had a history of trauma.

RESULTS

Six patients were identified and treated in our cohort, highlighting the infrequency of this pathology. All had at least one occipital nerve block, with 83% receiving varied relief. All underwent bilateral decompression or neurectomy of the GON and experienced relief, reporting improved visual analog scale scores (mean 8.3 ± 0.9 preoperatively to 1.0 ± 2.2 postoperatively, p = 0.0009). The patients were followed for an average of 10 months, and their mean number of medications decreased from 2.7 ± 0.5 preoperatively to 0.8 ± 0.7 postoperatively (p = 0.019). Each patient reported numbness or tingling in the GON distribution postoperatively, which spontaneously resolved over time. Two patients had recurrent pain in a delayed fashion.

CONCLUSIONS

GON decompression and neurectomy are efficacious treatments of refractory ON in the pediatric population.

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Audrey Grossen, Theresa Gavula, Deepti Chrusciel, Alexander Evans, Rene McNall-Knapp, Ashley Taylor, Benay Fossey, Margaret Brakefield, Carrick Carter, Nadine Schwartz, Naina Gross, Andrew Jea, and Virendra Desai

OBJECTIVE

Neurocutaneous syndromes have variable multisystem involvement. The multiorgan involvement, potential pathologies, and various treatment options necessitate collaboration and open discussion to ensure optimal treatment in any given patient. These disorders provide quintessential examples of chronic medical conditions that require a lifelong, multidisciplinary approach. The objectives of this study were to 1) perform a systematic review, thoroughly assessing different multidisciplinary clinic layouts utilized in centers worldwide; and 2) characterize an institutional experience with the management of these conditions, focusing on the patient demographics, clinical presentation, complications, and therapeutic strategies seen in a patient population.

开云体育世界杯赔率

A systematic review of studies involving multidisciplinary clinics and their reported structure was performed according to PRISMA guidelines using the PubMed database. Then a retrospective chart review of patients enrolled in the Oklahoma Children’s Hospital Neurocutaneous Syndromes Clinic was conducted.

RESULTS

A search of the PubMed database yielded 251 unique results. Of these, 15 papers were included in the analysis, which identified 16 clinics that treated more than 2000 patients worldwide. The majority of these clinics treated patients with neurofibromatosis (13/16). The remaining clinics treated patients with von Hippel–Lindau syndrome (n = 1), tuberous sclerosis complex (n = 1), and multiple neurocutaneous syndromes (n = 1). The most commonly represented subspecialties in these clinics were genetics (15/16) and neurology (13/16). Five clinics (31%) solely saw pediatric patients, 10 clinics saw a combination of children and adults, and the final clinic had separate pediatric and adult clinics. The retrospective chart review of the Neurocutaneous Syndromes Clinic demonstrated that 164 patients were enrolled and seen in the clinic from April 2013 to December 2021. Diagnoses were made based on clinical findings or results of genetic testing; 115 (70%) had neurofibromatosis type 1, 9 (5.5%) had neurofibromatosis type 2, 35 (21%) had tuberous sclerosis complex, 2 (1%) had von Hippel–Lindau syndrome, 2 (1%) had Gorlin syndrome, and the remaining patient (0.6%) had Aarskog-Scott syndrome. Patient demographics, clinical presentation, complications, and therapeutic strategies are also discussed.

CONCLUSIONS

To the best of the authors’ knowledge, this is the first detailed description of a comprehensive pediatric neurocutaneous clinic in the US that serves patients with multiple syndromes. There is currently heterogeneity between described multidisciplinary clinic structures and practices. More detailed accounts of clinic compositions and practices along with patient data and outcomes are needed in order to establish the most comprehensive and efficient multidisciplinary approach for neurocutaneous syndromes.

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