Unusual causes of papilledema: Two illustrative cases
A 16-year-old obese girl presented with a 2-week history of jaw pain, headaches, sore throat, and tactile fevers. One week after the onset of symptoms, she was diagnosed with acute sinusitis and treated with amoxicillin-clavulinate. Due to persistent symptoms, further tests were performed that revealed anemia (Hgb of 7.8 mg/dL) and thrombocytopenia (platelets of 15 × 103/mm3). Blasts were noted on the smear. A monospot test was negative. Leukemia was suspected and she was referred to our facility for further management.\nA review of the patient's systems revealed diffuse headaches, which were moderated by acetaminophen, mild neck pain, nausea with three episodes of emesis for 2 days, and bleeding when brushing her teeth. Bone marrow biopsy results were consistent with acute promyelocytic leukemia. She underwent induction therapy that consisted of all-transretinoic acid (ATRA) on days 1 through 28, daunomycin on days 3 through 6, and cytarabine (Ara-C) intravenously on days 3 through 9, and she also received four doses of intrathecal Ara-C. Her blood cell counts were monitored daily and transfusions of packed RBC and platelets were provided as needed.\nTwo days into her chemotherapy, the patient complained of progressive vision loss. An ophthalmology evaluation revealed visual acuity of 20/200 right eye and 20/200 left eye, bilateral 4+ optic nerve edema, and macular exudates. Intravenous acetazolamide (500 mg twice daily) was administered.\nA “high” opening pressure during lumbar puncture was reported. CSF studies revealed RBC of 16/mm3, WBC of 1/mm3, bands of 18%, neutrophils of 59%, lymphocytes of 14%, monocytes of 4%, and other cells of 2% (myelocytes, metamyelocytes). Myeloid cells were also noted in the cytopathology specimen consistent with the diagnosis of leukemia. MRI demonstrated multifocal irregular nodules, dural thickening, and gyral enhancement in the posterior fossa. In addition, the patient's optic nerve sheaths were dilated and demonstrated thick nodular enhancement diffusely [], consistent with leukemic infiltration of the optic nerve sheaths. Infiltration of the leptomeninges, and scalp were also noted. There was no evidence of cerebral venous sinus thrombosis or narrowing. An MRI of the spine was negative. We postulated that the patient's papilledema was secondary to leukemic infiltration of her optic sheaths.\nAcetazolamide did not lead to any improvement in the patient's condition. She urgently underwent a course of palliative craniospinal radiation therapy (12 daily treatments of 2160 cGy). Notably, her headaches and vision improved by the end of radiation treatment as evident by interval improvement in her papilledema with ongoing resolution of nerve hemorrhages and macula exudates. The patient continued taking acetazolamide until her papilledema resolved. A follow-up appointment at 8 months revealed visual acuity of 20/30 bilaterally and full ocular motility. Color plates continued to be decreased bilaterally, but were stable compared with prior exams. Mild optic nerve pallor was seen bilaterally, with no sign of optic nerve edema.