Bilateral sphenoorbital hyperostotic meningiomas with proptosis and visual impairment: a therapeutic challenge. Report of three patients and review of the literature.

OBJECTIVE

Bilateral hyperostotic sphenoorbital meningiomas are extremely uncommon. Due to extensive infiltration of the orbits and the frontotemporal skull base, often only a subtotal tumor resection is feasible. Thus far, no treatment algorithms have been suggested for this rare tumor entity. We report on the surgical management of 3 patients.

METHODS

All 3 patients underwent a pterional approach for surgical resection. Surgery was performed in two stages, primarily treating the most affected side. Treatment consisted of microsurgical resection of the infiltrated sphenoid wing and orbital walls, intraorbital tumor removal and optic nerve decompression. Orbital wall reconstruction was performed using titanium mesh allografts. Radiation therapy was administered in 1 patient with residual tumor infiltration of the cavernous sinus.

RESULTS

Our series includes 2 women (51 and 68 years old) suffering from simultaneous progressive bilateral loss of vision and proptosis and 1 woman (69 years old) who developed contralateral disease after surgical resection of a hyperostotic sphenoorbital meningioma 16 years earlier. After optic nerve decompression, vision improved in 2 cases after surgery. Initial visual deterioration was observed in 1 case but improved on longterm follow-up. The degree of proptosis was reduced in all treated eyes.

CONCLUSION

In bilateral hyperostotic sphenoorbital meningiomas we propose staged surgery when clinical and radiological progression is observed. Subtotal tumor resection with the aim of optic nerve decompression and subsequent orbital reconstruction provides satisfactory results. The most affected eye should be treated first. In case of additional cavernous sinus infiltration, focal radiation therapy can be considered.