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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:

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Paediatric Ophthalmology

Case 4

Patient Presentation: A 14-year-old male was referred to a paediatric ophthalmology clinic for decreased vision in both eyes over the past 2 years. The patient denied previous red eye or visual field deficit. There was no past medical, ocular or family history of eye disease. Best corrected distance visual acuity was 20/100 OD and 20/150 OS. IOP was 12 OD and 13 OS; there was no RAPD. Fundus photos and OCT macula images were taken and are shown below:

Question 1: What is the main finding in the OCT macula images above?

A. Foveal atrophy

B. Subretinal fluid

C. Disorganization of inner retinal layers

D. Drusenoid deposits

Fundus autofluorescence images were also conducted and are shown below:

Question 2: Based on the fundus photos, OCT, and fundus autofluorescence images, what is the most likely diagnosis?

A. Retinitis pigmentosa

B. Central areolar choroidal dystrophy

C. Stargardt’s disease

Question 3: What gene is implicated in typical Stargardt’s disease?

Learning Objectives:

Understand the presenting signs of Stargardt’s disease
Learn the pathophysiology and genetic cause of Stargardt’s disease

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