It's Always Been Like That - Slides

An 18 year-old Caucasian boy presented with left upper eyelid ptosis, headaches, and iris heterochromia. The patient and his family reported the left iris being darker than the right since early childhood. Over the 5 years preceding his presentation, the patient's mother noted progressive ptosis of his left upper eyelid. A top student in middle school, his grades had declined through high school, and his family had noticed loss of motivation mixed with intermittent irritability and mood changes. The patient developed headaches 2 years prior that had been worsening steadily and culminated over the preceding week in severity with daily episodes of nausea and vomiting. He noticed increased thirst and voiding frequency and had felt fatigued. He gained 25 lbs in the preceding 3 months. The patient was initially prescribed sumatriptan and cyproheptadine for presumed migraines without symptomatic benefit. In the week prior to presentation, the left side of his face began to droop and he developed binocular, horizontal diplopia. A CT head was abnormal, and the patient was referred for evaluation and admission. External examination revealed ptosis of the left upper eyelid and slate grey circumferential coloration of the sclera (Figure 1). The right iris was hazel, and the left was dark brown. The pupils were equal and reactive without a relative afferent defect. Visual acuities were 20/20 OU without dyschromatopsia or metamorphopsia. Abduction of the left eye was mildly limited, and there was subtle left facial weakness. Automated visual field testing showed enlarged blind spots bilaterally. Funduscopic examination was significant for papilledema with peripapillary flame hemorrhages OU and a striking interocular diffuse choroidal pigmentation disparity with the left eye being darker than the right (Figure 2). The patient was treated with corticosteroids with improvement of his headache and resolution of diplopia. A diagnostic procedure was performed.VBskullbasetumor