Juvenile trabecular ossifying fibroma of the sphenoid sinus causing acute visual loss in a 14-year-old girl with Turner syndrome: a case report.

INTRODUCTION AND

IMPORTANCE

Juvenile trabecular ossifying fibroma (JTOF) is a rare, locally aggressive, fibro-osseous tumor that predominantly affects the jaw and rarely involves the sphenoid sinus. When optic neuropathy develops, an urgent yet carefully planned surgical intervention is required.

PRESENTATION OF CASE: A 14-year-old girl with Turner syndrome presented with acute visual deterioration. Imaging revealed a large expansile fibro-osseous mass occupying the sphenoid sinus and compressing both optic nerves. Endoscopic biopsy revealed a hypervascular lesion consistent with JTOF. Because of the marked vascularity and limited endoscopic visualization, immediate radical decompression was considered high risk. Therefore, a staged endoscopic endonasal approach was used. Definitive tumor resection was subsequently performed in collaboration with a skull base surgeon, achieving gross total tumor removal with controlled bleeding. Postoperatively, the visual acuity and visual field function improved markedly.

CLINICAL DISCUSSION: Sphenoid sinus JTOF poses significant diagnostic and therapeutic challenges owing to its rarity, aggressive behavior, and proximity to critical neurovascular structures. Preoperative biopsy plays a crucial role in confirming the diagnosis and guiding surgical planning, particularly for highly vascular lesions. A staged endoscopic approach can provide a safer operative field, while also minimizing the risk of excessive hemorrhage and optic nerve injury.

CONCLUSION

Patients with JTOF of the sphenoid sinus may present with acute compressive optic neuropathy and pronounced hypervascularity. In such high-risk cases, early pathological confirmation and flexible staged endoscopic surgical planning are essential to achieve complete resection while preserving visual function. This case has provided a practical surgical strategy for managing aggressive fibro-osseous lesions of the skull base.