Pulsating Exophthalmos due to Intraorbital Meningoencephalocele

Abstract

A 31-year-old woman initially presented to the ophthalmology clinic with a history of constantly pulsating exophthalmos in her right eye (Video clip). The patient's visual acuity was 20/25 in the affected eye. A. A slit-lamp examination showed Lisch nodules on the iris. B. The physical examination showed café-au-lait spots and cutaneous neurofibromas, as well as freckling in the armpits. C. Computed tomography showed right sphenoid dysplasia. D. Magnetic resonance imaging showed meningoencephalocele in the right orbit. The patient was diagnosed with neurofibromatosis type 1 (NF1). Her pulsating exophthalmos was secondary to intraorbital meningoencephalocele, which is a rare manifestation in NF1 patients with sphenoid dysplasia. The neurosurgeons and ophthalmologists advised surgical treatment, but she was reluctant to take risks. At the 6-month follow-up visit, there was no evidence of disease progression (Figure 1).