Papilledema Associated with Puberty

research-article2014 CPJXXX10.1177/0009922814554503Clinical PediatricsSun and Horton Resident Round Papilledema Associated with Puberty Clinical Pediatrics 2015, Vol. 54(5) 504­–506 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/0009922814554503 cpj.sagepub.com Lynn W. Sun, BA, PhD 1 and Jonathan C. Horton, MD, PhD 2 Case Report A 9-year-old girl was brought by her mother to a hospi- tal emergency room because of severe headache and vomiting. The symptoms began 2 weeks earlier while at summer camp. The child’s examination was benign, and she was discharged with a diagnosis of migraine. The ocular fundi were not examined. The headaches contin- ued and were sometimes associated with nausea, photo- phobia, and tinnitus. She was evaluated 2 weeks later in our pediatric ophthalmology clinic because of intermit- tent horizontal diplopia and blurred vision. There was no relevant past medical history. The patient did not take tetracycline antibiotics or any other medications. The height was 142.5 cm and the weight was 58.0 kg, for a body mass index (BMI) of 28.6 kg/m 2 (obese). Physical development was at Tanner stage II. The visual acuity was 20/20 in each eye without cor- rection. Pupils were equal, round, and briskly respon- sive to light. There was no afferent pupillary defect. The extraocular eye movements were full. Ocular alignment was orthotropic by cover/uncover test. Stereopsis was intact to a hidden random dot butterfly pattern. Slit lamp examination was normal. Dilated fundus examination revealed acutely swollen optic nerves (Figure 1). Clinical Course Magnetic resonance (MR) imaging of the brain was normal, except for protrusion of the optic discs into the vitreous cavities, consistent with the findings on oph- thalmoscopy (Figure 1). There was also a partially empty sella, a finding that sometimes occurs from raised intracranial pressure. An MR venogram showed no occlusion of the cerebral dural sinuses. A lumbar puncture under sedation yielded an opening pressure of 370 mm H 2 O in the lateral decubitus posi- tion. Cerebrospinal fluid analysis showed no bacteria, 2 white cells, 0 red cells, protein 20 mg/dL, and glucose 52 mg/dL. A panel of laboratory tests for hematologic, autoimmune, and inflammatory diseases was negative. The patient was treated with acetazolamide 250 mg twice per day. Two weeks later she reported lessening of headache. Fundus examination showed slight reduction of her papilledema. Over the next 6 months her symp- toms gradually resolved and she stopped attending clinic appointments. Two years after her original presentation, she returned to the eye clinic for a routine examination. On her own initiative, she had discontinued treatment with acetazol- amide 18 months earlier. She denied headache or blurred vision. The height was 152.4 cm and the weight was 71.67 kg, for a BMI of 30.9 kg/m 2 (obese). She had regu- lar menstrual periods. The visual acuity was 20/20 in each eye without correction. Pupils, eye movements, ocular alignment, visual fields, and slit lamp examination were normal. The papilledema had resolved (Figure 2). Final Diagnosis Idiopathic intracranial hypertension Discussion The term pseudotumor cerebri was coined by Max Nonne in 1904 to describe the curious occurrence of elevated intracranial pressure in healthy patients without brain tumor. 1 The term benign intracranial hypertension was later adopted to emphasize the absence of malig- nancy. 2 This name has been criticized because the dis- ease course in some patients is hardly benign, inasmuch as irreversible vision loss may occur. 3 For this reason, the name was revised again to idiopathic intracranial hypertension. 4 In adults, the major risk factors for pseudotumor cerebri are female gender and obesity, suggesting that the underlying pathophysiology may involve estrogens and endocrinologically active adipose tissue. 5 In adult Loyola University Chicago, IL, USA University of California, San Francisco, CA, USA Corresponding Author: Jonathan C. Horton, Beckman Vision Center, Program in Neuroscience, University of California, San Francisco, 10 Koret Way, San Francisco, CA 94143-0730, USA. Email: hortonj@vision.ucsf.edu Downloaded from cpj.sagepub.com at UCSF LIBRARY & CKM on August 7, 2015