January 2025

OPHTHALMOLOGY

CASE REPORT: BILATERAL BLURRY VISION IN A TEENAGE PATIENT

background

A 15-year-old male presented with several weeks of painless, progressive blurry vision and intermittent headaches. Initial ophthalmic examination revealed bilateral disc edema, prompting immediate referral to the emergency department, where the patient was found to have an elevated blood pressure measuring 239/172 mmHg.
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The patient was then admitted to the pediatric ICU for further management and remained hypertensive despite the use of multiple antihypertensive agents. Ophthalmic evaluation at this time was notable for best corrected visual acuity of 20/120 OU. Fundus exam demonstrated grade 4 hypertensive retinopathy with OCT imaging revealing subretinal fluid.​

work-up

Further studies demonstrated largely unremarkable neuroimaging and normal lumbar puncture opening pressure. Of note, the patient had elevated normetanephrine and metanephrine levels, prompting abdominal imaging which showed a 7-centimeter right adrenal mass. This was promptly excised by the Pediatric Surgery team and confirmed by pathology studies as a paraganglioma, or extra-adrenal pheochromocytoma. Genetic studies were pursued which identified the SDHB c.423+1G>A mutation, diagnosing Hereditary Paraganglioma-Pheochromocytoma syndrome with associated risk for recurrent tumors.

Post-op Week 1 s/p Paraganglioma Excision

Post-op Month 3 s/p Paraganglioma Excision

​The patient’s blood pressure had normalized to 110s/70s, and he also reported improvement in vision. The patient’s best corrected visual acuity was 20/125 OD and 20/25 OS by Snellen. Fundus exam was notable for improved optic nerve edema with flattened macula. Subretinal fluid had resolved; however, there were irregular outer segments with persistent retinal exudates

conclusion

This case demonstrates the importance of recognizing rarer, systemic causes of progressive, bilateral blurry vision in pediatric patients, especially in the setting of recent hypertension diagnosis. While pheochromocytoma is a rare cause of hypertension in children, it may infrequently present as changes in vision.
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A visit to the ophthalmologist may lead to the diagnosis of this life-threatening tumor. 
Roya Zandi, MD, Ophthalmology Resident at Northwestern Medicine 
Janice Lasky Zeid, MD, Associate Professor of Ophthalmology at Northwestern Medicine
​Safa Rahmani, MD, Associate Professor of Ophthalmology at Northwestern Medicine

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