Tolosa–Hunt syndrome in children and adolescents: A systematic review
Authors: Ahmed H. Shafeeq , Jayaram Purva Reddy , Khar Sukriti 
Published: January 2025

Objective

This systematic review aims to consolidate and analyze the existing evidence on Tolosa–Hunt syndrome (THS) in the pediatric population, focusing on clinical features, diagnostic challenges, treatment outcomes, and prognosis.

Background

Tolosa–Hunt syndrome is a rare headache disorder caused by idiopathic inflammation of the cavernous sinus, orbital apex, or orbit, resulting in neuro‐ophthalmological manifestations. It is uniquely characterized by cranial nerve palsies and often responds well to steroids.

Methods

A comprehensive literature search was conducted using three databases along with the gray literature. We followed the Preferred Reporting Items for Systematic Reviews and Meta‐Analyses guidelines, and the review protocol was registered on International Prospective Register of Systematic Reviews (PROSPERO identifier: CRD42024576802). The review included case reports and case series published in multiple languages that documented pediatric or adolescent cases of THS. We excluded any cases that were irrelevant, had insufficient details, were unsure of the diagnosis, or were later re‐diagnosed with another condition. Data on clinical presentations, imaging findings, treatment modalities, and outcomes were extracted and analyzed using Microsoft Excel 2021.

Results

The initial literature search provided 325 articles of which 55 articles discussing 61 unique pediatric patients were included. The median (interquartile range [IQR]) age was 11 (8–15) years, with a female predominance (70% [43/61]). Common symptoms included unilateral headache (48% [29/61]), retro‐orbital pain (56% [34/61]), and cranial nerve palsies, predominantly involving the oculomotor nerve (66% [40/61]). The median (IQR) duration of symptoms was 14.5 (5–35) days. Imaging often revealed contrast enhancement on magnetic resonance imaging with cavernous sinus/orbital apex lesions. Steroid therapy was the mainstay of treatment, with 91% (52/57) of patients receiving corticosteroids. High‐dose steroids ranged from 500–1000 mg/day, with some cases requiring combined therapy (typically intravenous methylprednisolone followed by oral prednisolone) and subsequent tapering. A few patients (5% [3/61]) experienced spontaneous improvement without steroids. Recurrence was noted in 33% (20/61) of patients, often necessitating prolonged or repeated corticosteroid therapy, and some cases required additional immunosuppressive therapies (infliximab/adalimumab) for management. The median (IQR) time to symptom resolution was 14 (4.5–38.5) days, while the median (IQR) duration of follow‐up was 730 (195–1095) days.

Conclusion

Tolosa–Hunt syndrome in children presents significant diagnostic and management challenges due to the complexity of symptoms and the rarity of the condition. Accurate diagnosis and prompt steroid therapy are crucial after ruling out other causes, although recurrence remains a considerable risk. The present systematic review relies heavily on case reports and case series and is therefore at high risk of publication bias. Further research is needed to establish standardized treatment protocols and improve long‐term outcomes in this population.