Poorly differentiated small cell neuroendocrine carcinoma localized in three different endocrine glands: response to chemotherapy and octreotide LAR.

BACKGROUND AND PURPOSE: Superior orbital fissure syndrome (SOFS) is a rare sequela of craniofacial trauma caused by dysfunction of cranial nerves III, IV, V1, and VI. It typically presents with ophthalmoplegia, ptosis, pupillary abnormalities, and sensory deficits. Due to its rarity and heterogeneity, optimal management remains unclear. The purpose of this study was to systematically review the literature on traumatic SOFS, focusing on demographic profile, clinical presentation, diagnostic modalities, management strategies, and outcomes.

METHODS

A systematic review was conducted in accordance with PRISMA guidelines. Electronic databases (PubMed/MEDLINE, Scopus, Web of Science, and Google Scholar) were searched from inception to 31 December 2025. Studies reporting traumatic SOFS with adequate clinical and outcome data were included. Extracted variables included demographics, etiology, clinical features, imaging findings, treatment modalities, and outcomes.

RESULTS

A total of 23 studies comprising 61 patients were included. The mean age was 31.9 years (range: 7-75 years), with a male predominance (2.4:1). Most cases were associated with craniofacial trauma, particularly zygomaticomaxillary complex and orbital fractures, although some occurred without fractures due to edema or hematoma. The most common clinical features were ophthalmoplegia, ptosis, fixed dilated pupil, proptosis, and sensory deficits in the ophthalmic division of the trigeminal nerve. Diagnosis was primarily based on clinical examination and computed tomography, with magnetic resonance imaging used selectively. Management included observation, corticosteroid therapy (31.1%), and surgical intervention such as open reduction and internal fixation or decompression. Favorable outcomes were observed in most cases, with most patients demonstrating complete or partial recovery within 1-6 months. Steroid therapy was associated with higher recovery rate (78.9%) compared to non-steroid management (61.9%).

CONCLUSION

Traumatic SOFS is an uncommon but clinically significant condition with a generally favorable prognosis. Early diagnosis and individualized management remain essential for optimizing neurological and functional outcomes. Although a higher proportion of complete recovery was observed among patients receiving corticosteroid therapy, the available evidence is derived predominantly from heterogeneous, low-quality, observational, and non-comparative studies, precluding definitive conclusions regarding treatment efficacy or superiority. Accordingly, the current findings should be interpreted cautiously and considered primarily descriptive and hypothesis-generating.