Presentation
Bilateral progressive proptosis.
Patient Data
Abnormal expansion of the cranial nerves passing on both sides of the cavernous sinus, all of them exhibit low signal on T1, target sign on T2 and no post contrast enhancements. The thickened nerves cause marked an expansion of their exits via the skull base including the widening of the SOF, inferior orbital fissure and foramen ovale. The affected nerve roots continue through the orbit forming extra and intraconal serpiginous lesions. The infraorbital nerves traversing the anterior walls of the maxillary sinuses are affected as well. All of the affected nerves have a characteristic appearance of target sign in T2 images. Subsequent bilateral proptosis. Thinning of the left lateral rectus muscle. Localized abnormal bone marrow signal of the mandible intimately related to the supplying nerve termination. Normal both optic nerve and bilateral EOMS. Bilateral posterior globes staphylomas. The examined brain is unremarkable.
Case Discussion
Multiple intra or extraconal masses follow the course of multiple nerves of the orbit, with subsequent bilateral proptosis. The MR appearance of the lesions is suggestive of plexiform neurofibromata.
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