Neurological Manifestations and Diagnostic Delay in Pediatric Isolated Sphenoid Sinus Disease: A Systematic Review.
This systematic review of 11 observational studies encompassing 136 children found that isolated sphenoid sinus disease commonly presented with headache, sometimes with ocular or cranial nerve manifestations, and was frequently initially misdiagnosed, while tumors accounted for only five cases.
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This systematic review of 11 observational studies encompassing 136 children found that isolated sphenoid sinus disease commonly presented with headache, sometimes with ocular or cranial nerve manifestations, and was frequently initially misdiagnosed, while tumors accounted for only five cases.
Research significance
The evidence supports considering early CT or MRI when persistent or atypical pediatric headache is accompanied by ocular symptoms or cranial nerve deficits; it can only be inferred—not established—that reducing diagnostic delay could expedite treatment of rare sphenoid tumors and other lesions and limit neurological sequelae.
Source abstract
Background: Isolated sphenoid sinus disease (ISSD) in children is an uncommon but clinically significant condition that may present primarily with neurological or neuro-ophthalmological symptoms rather than typical sinonasal complaints. Owing to the anatomical proximity of the sphenoid sinus to the optic nerves, cavernous sinus, and multiple cranial nerves, even localized sphenoid pathology may produce symptoms that mimic primary neurological, neuro-ophthalmological, or headache disorders, contributing to diagnostic delay. Objective: To characterize the spectrum and frequency of neurological and neuro-ophthalmological manifestations, diagnostic pathways, treatment strategies, and reported clinical outcomes in pediatric ISSD, with particular emphasis on diagnostic delay and persistent neurological sequelae. Methods: A systematic review was conducted in accordance with the PRISMA 2020 guidelines. PubMed and Web of Science were searched from database inception to 3 January 2026. Observational studies reporting pediatric patients aged ≤18 years with radiologically confirmed ISSD were included. Single-patient case reports, reviews, and studies without extractable pediatric data were excluded. Owing to substantial clinical and methodological heterogeneity, findings were synthesized descriptively. Risk of bias was assessed using Joanna Briggs Institute (JBI) critical appraisal tools. Results: Eleven studies comprising 136 pediatric patients were included. Most cases were inflammatory lesions (n = 121, 89%), followed by mucoceles (n = 10, 7.3%) and tumors (n = 5, 3.7%). Headache was the most frequent neurological manifestation reported in 129 patients (94.9%). Ocular manifestations were present in 33/136 patients (24.2%), whereas otolaryngological symptoms were reported in 25/134 patients (18.7%). Cranial nerve involvement was documented in 10 out of 54 patients with available data (19%), most commonly affecting cranial nerves III, V, VI, and VII. Among 79 reported initial diagnoses, primary headache disorders were the most common (49.4%), whereas sphenoid sinusitis was correctly identified in only 20.3%. Diagnosis was established using CT and/or MRI in all included cases. Antibiotic therapy was the most common treatment for inflammatory lesions, while all patients with mucoceles and tumors underwent surgical management. Full recovery was reported in 81% of inflammatory cases and in all patients with mucoceles, although follow-up reporting was heterogeneous. Conclusions: Pediatric ISSD frequently presents with neurological and neuro-ophthalmological manifestations, whereas sinonasal symptoms may be absent or subtle. Persistent or atypical neurological symptoms, particularly headache accompanied by ocular symptoms or cranial nerve deficits, should prompt consideration of sphenoid sinus pathology and early cross-sectional imaging.