Unusual causes of papilledema: Two illustrative cases
A 14-year-old boy with Crohn's disease complained of “spots” in his vision for a 24-hour period and severe headaches during the prior several weeks. An initial evaluation revealed bilateral retinal hemorrhages and disc edema. Laboratory studies demonstrated severe anemia with hemoglobin of 3.0 mg/dL, white blood cells (WBC) of 1.8 × 103/mm3, and platelets of 101 × 103/mm3. He was transfused with packed red blood cells (RBC) and referred to our facility.\nOne to two days before admission, the headaches were described as retro-orbital and bilateral, accompanied by multiple spots in both visual fields. On admission, the spots had consolidated into one large spot in the center of his vision. The patient also admitted to intermittent diplopia, pulsatile tinnitus, and several episode of emesis over the previous 2 weeks. He had noted bloody stools the previous day. His medications before admission included 6-mercaptopurine (6-MP), mesalamine, and vitamin D supplements. He had been taking 6-MP to treat Crohn's disease for several months. On retrospective chart review, we noted that the patient's blood counts had gradually declined since 6-MP therapy had been initiated.\nAn ophthalmologic assessment at our institution demonstrated visual acuity of 20/800 right eye and 20/400 left eye, bilateral 4+ optic disc edema, bilateral central scotomas, nerve fiber layer hemorrhages, and bilateral scattered peripheral intraretinal hemorrhages. Computed tomography (CT) scan and magnetic resonance imaging/magnetic resonance venography (MRI/MRV) were unremarkable []. The patient underwent a lumbar puncture, which revealed an opening pressure in excess of 55 cm H2O. Other CSF studies were also unrevealing.\nWe postulated that the patient's severe anemia may have precipitated the elevated ICP, causing his symptoms. He was placed on oral acetazolamide 5 mg/kg/dose daily to reduce ICP. His 6-MP was discontinued as this was thought to be the proximate cause of anemia, and he received a transfusion of packed RBC to increase his hemoglobin to 8.5 g/dL.\nThe patient's clinical course showed improvement by hospital day 3 []. His visual acuity improved to 20/40 right eye and 20/40 left eye with a central bitemporal field loss; papilledema was reduced to 2-3 + OD, 2 + OS; and confrontation to visual fields still showed persistent but incomplete binasal central scotomas. His headaches gradually improved during hospitalization. His blood counts slowly increased after 6-MP was discontinued. One week after admission, his visual acuity remained 20/40 bilaterally with resolution of his partial central scotoma while disc edema improved 1.5-2 + OD, 1.5 + OS. A follow-up visit 2 weeks later revealed flat optic discs with good color, no hemorrhages, and visual acuity 20/40 right eye, 20/50 left eye.