Heart of Darkness - Path PPT

An 18 year-old woman presented to the emergency department in October 2014 with three days of blurred vision in her left eye. Past medical history included a hypoplastic right heart status-post orthotopic heart transplant at 2 months of age, chronic kidney disease with BK viremia since 12 years of age, and recurrent post-transplant lymphoproliferative disorder (PTLD, diagnosed at 15 years of age, with two subsequent recurrences). Medications included tacrolimus, leflunomide, trimethoprim/sulfamethoxazole, atorvastatin, amlodipine, and 81 mg aspirin. Review of systems was notable only for mild nasal congestion and cough for the past four weeks. Visual acuity was 20/20 in the right eye, 20/40 in the left, with a mild APD. Ishihara plates were full in the right eye, 2/11 in the left. Automated perimetry revealed an inferonasal depression in the left eye. There was no proptosis or ptosis. The anterior segment examination was normal; posterior segment revealed tortuous vasculature in both eyes and mild nasal disc elevation in the left eye, along with a fan-shaped intraretinal hemorrhage nasal to the disc margin. MRI brain and orbits without contrast (contrast withheld in setting of renal dysfunction) demonstrated a 0.7 x 0.5 cm mass along the medial aspect of the left orbital apex, possibly contiguous with the optic nerve. Lumbar puncture on admission demonstrated a normal opening pressure with no malignant cells. Patient was started on dexamethasone and then rituximab for presumed PTLD recurrence. At 1 week, steroids were tapered and the exam was stable. At 2 weeks, visual acuity worsened to 20/200, and there was a 3+ rAPD. Repeat MRI was unchanged