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?
To understand the OCT findings, including detection of the omega sign, for combined hamartoma of the retina and RPE.
To understand the prognosis and follow-up process for CHRRPE lesions.