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.