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Patient Presentation: A 23-year-old obese female was diagnosed with idiopathic intracranial hypertension (IIH) and referred to neurosurgery for ventriculoperitoneal shunt. A baseline ocular examination was performed prior to the procedure.
On examination, vision was 20/200 in the right eye, and 20/40 in the left eye. There was a right relative afferent pupillary defect. Slit lamp examination was normal.
A dilated fundus examination was performed demonstrating the following:
Patient Presentation: A 15-year-old asymptomatic male presented to clinic for abnormal visual fields (shown below). His visual acuity was 20/20 OU with IOP within normal limits.
Question: What visual field defects are present?
Question: OCT RNFL and Ganglion Cell Inner Plexiform Layer (GCIPL) Analysis were performed and shown above. What is the most significant finding?
Question: Fundus photography was performed and is shown above. What is the patient's most likely diagnosis?
Question: What maternal medical condition/finding is thought to be associated with optic nerve hypoplasia?
Question: What issues are patients with optic nerve hypoplasia at risk of developing?
1. Birkebæk NH, Patel L, Wright NB, Grigg JR, Sinha S, Hall CM, Price CA, Lloyd IC, Clayton PE. Endocrine Status in Patients with Optic Nerve Hypoplasia: Relationship to Midline Central Nervous System Abnormalities and Appearance of the Hypothalamic-Pituitary Axis on Magnetic Resonance Imaging. J Clin Endocrinol Metab. 2003;99(11):5281-6.
2. Mohney BG, Young RC, Diehl N. Incidence and Associated Endocrine and Neurologic Abnormalities of Optic Nerve Hypoplasia. JAMA Ophthalmol. 2013;131(7):898–902.
1. To recognize and diagnose optic nerve hypoplasia on OCT
2. To recognize the conditions commonly associated with optic nerve hypoplasia
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