Evaluation and Management of Tolosa-Hunt Syndrome in Children: A Clinical Update.

BACKGROUND: Tolosa-Hunt syndrome is a painful ophthalmoplegia caused by an inflammatory process of unknown etiology in the region of the cavernous sinus, orbital apex, or superior orbital fissure. This disease is rare in the pediatric population. The objective of this study was to provide a clinical framework for the evaluation and treatment of children with this disorder. A systematic approach to the diagnosis of painful ophthalmoplegia in children is proposed.

METHODS: We present a 15-year-old girl whose clinical presentation and neuroradiological findings support a diagnosis of Tolosa-Hunt syndrome as defined by the 2013 International Classification of Headache Disorders (Third Edition, ICHD-3 beta) diagnostic criteria. An exhaustive systematic literature search based on these criteria yielded 15 additional cases of Tolosa-Hunt syndrome in children. Clinical, demographic, and radiological features were retrospectively analyzed. The results and statistical analyses are reported.

RESULTS: A total of 16 individuals were included in the final analysis. This review summarizes the current knowledge and recommendations for the diagnosis and management of pediatric Tolosa-Hunt syndrome. It highlights demographic, clinical, and radiological features of this disease in children and underscores areas of the literature where evidence is still lacking.

CONCLUSIONS: Overall, Tolosa-Hunt syndrome seems to follow a similar course in children compared to adults. The diagnostic approach and treatment require specific considerations. New observations and possible features of pediatric Tolosa-Hunt syndrome are discussed. Further research is needed to optimize clinical detection and medical management of this disease.