An uncommon presentation of orbital apex syndrome

Bilateral total ophthalmoplegia secondary to a malignancy or infection of the cavernous sinus and orbital apex is an unusual presentation. Bilateral ophthalmoplegia as the initial sign of this type of rhinocerebral pathology is also uncommon.

A 34-year old Haitian woman presented with bilateral vision loss, ptosis, total ophthalmoplegia, and ocular pain. A physical and neurologic examination, laboratory analysis, chest x-rays, and neurologic imaging studies were requested. Cranial and facial computed tomography (CT) scans showed swelling of the soft tissues proximal to the sphenoid extending into the dorsal sella and prepontine cistern, obliteration of the nasopharyngeal reflection, opacification of the ethmoid and sphenoid sinus, bony destruction of the mid-skull base, and bilateral lymphadenopathy of the neck. Magnetic resonance imaging (MRI) showed the presence of a soft tissue mass causing destruction of the skull base. Involvement of the clivus, cavernous sinuses, and sella with nasopharynx extension was observed. Hypodense centers within nasopharyngeal tissues suggested the presence of necrosis. Differential diagnosis included nasopaharyngeal carcinoma, lymphoma, or an infectious process. The patient did not consent to a biopsy and refused treatment.

This presentation is a medical emergency. A combination of surgical, medical, or radiological intervention may be required to manage rhino-cerebral diseases responsible for orbital apex syndrome. Despite advances in neurologic imaging, histologic examination of tissue obtained from a biopsy may ultimately be necessary to obtain a precise diagnosis.