Journal of Neuro-Ophthalmology, March 2008, Volume 28, Issue 1

Multiple Intracranial Meningiomas Causing Papilledema and Visual Loss in a Patient with Nevoid Basal Cell Carcinoma Syndrome

A 27-year-old man with nevoid basal cell carcinoma syndrome (NBCCS, Gorlin syndrome) who had undergone craniospinal irradiation for a childhood brain stem medulloblastoma complained of progressive binocular visual loss. Ophthalmologic examination disclosed subnormal visual acuity and visual fields in both eyes attributed to chronic papilledema. Brain MRI demonstrated mass effect from multiple large meningiomas. After embolization and surgical resection of the largest meningioma, papilledema disappeared and visual dysfunction resolved partially. This is the sixth reported patient with NBCCS, medulloblastoma, and craniospinal radiation who has developed intracranial meningioma, further documenting the fact that such patients have a relatively high likelihood of developing meningiomas, especially multiple meningiomas. Because patients with NBCCS are often mentally impaired and because papilledema can progress silently before causing irreversible visual loss, periodic ophthalmologic examination is advisable after craniospinal radiation.