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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 1
Patient Presentation: A 11-year-old male with a history of neurofibromatosis type 2 presented to a pediatric retina clinic for assessment of an atypical lesion in his posterior pole OD. The patient’s mother noted a painless decrease in vision over the last 2 years, but denied trauma, flashes, floaters or a visual field deficit. The patient’s NF2 was stable; his previous MRI brain and orbits was unremarkable. Best corrected distance visual acuity was 20/80 OD and 20/20 OS. IOP was within normal limits, and there was no RAPD.
Fundus photographs were taken and are shown below:
An OCT macula of the right eye was also conducted and is shown below:
Question 1: What findings are seen in the OCT macula above?
Question 2: Based on the history of NF2, fundus findings, and OCT macula imaging, what is the most likely diagnosis?
Question 3: Looking back at the OCT macula, what sign is demonstrated by this combined hamartoma of the retina and RPE?
Question 4: What is the prognosis for CHRRPE lesions?
Learning Objectives:
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To understand the OCT findings, including detection of the omega sign, for combined hamartoma of the retina and RPE.
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To understand the prognosis and follow-up process for CHRRPE lesions.
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