Spheno-orbital fibro-osseous lesion with orbital, intracranial, and infratemporal extension with adamantinoma-like epithelial differentiation: Surgical management and Clinicopathological correlation

Craniofacial fibro-osseous lesions are benign disorders characterized by replacement of normal bone with fibrocellular tissue and immature woven bone. Involvement of the sphenoid bone is uncommon and may lead to orbital deformity, proptosis, and compression of adjacent neurovascular structures. Extensive lesions with simultaneous orbital, intracranial, and infratemporal extension are rare and present unique neurosurgical challenges. A 19-year-old male presented with progressive right-sided proptosis and intermittent headache for two months. Neurological examination revealed preserved visual acuity and intact cranial nerve function. Computed tomography demonstrated an expansile ground-glass lesion involving the right sphenoid wing with extension into the orbit, middle cranial fossa, and infratemporal fossa, producing mild temporal lobe compression without optic canal narrowing. The patient underwent right frontotemporal craniotomy with extradural drilling of the dysplastic sphenoid bone, orbital decompression, and reconstruction using an autologous split calvarial graft. Histopathological examination favored a benign craniofacial fibro-osseous lesion, likely within the ossifying fibroma spectrum, with focal cytokeratin-positive epithelial nests. The postoperative recovery was satisfactory, with improvement in proptosis and preserved visual function. Management should be guided by clinical findings, particularly regarding selective optic canal decompression. Adamantinoma-like epithelial nests may create diagnostic uncertainty and require multidisciplinary clinicopathological correlation. • Extensive spheno-orbital fibro-osseous lesions may cause intracranial mass effect without optic canal compression. • Selective rather than prophylactic optic canal decompression is appropriate when visual function is preserved. • Extradural skull base drilling and orbital decompression can provide effective early symptomatic relief. • Focal epithelial nests within fibro-osseous lesions may mimic adamantinoma and complicate diagnosis. • Multidisciplinary clinicoradiological and histopathological correlation is essential for accurate diagnosis and management.