THE PERIPAPILLARY RETINA: A COMMON JUNCTURE IN STARGARDT DISEASE AND IDIOPATHIC INTRACRANIAL HYPERTENSION

Area of Science:

• Ophthalmology
• Neuro-ophthalmology
• Medical Imaging

Background:

• Stargardt disease and idiopathic intracranial hypertension are individually rare conditions.
• Both diseases can affect vision and ocular structures.
• Understanding their combined presentation is crucial for diagnosis and management.

Purpose of the Study:

• To document the multimodal imaging and functional findings in a patient with co-occurring Stargardt disease and idiopathic intracranial hypertension.
• To investigate the potential interaction between these two conditions.

Main Methods:

• Multimodal imaging: color fundus photography, short-wavelength autofluorescence, spectral domain optical coherence tomography.
• Functional testing: Humphrey visual fields, full-field electroretinogram.
• Diagnostic confirmation: Magnetic resonance imaging, lumbar puncture, genetic testing.

Main Results:

• A 35-year-old woman presented with transient visual obscurations and optic disc edema.
• Imaging revealed bull's-eye maculopathy with flecks and peripapillary hyperautofluorescence.
• Idiopathic intracranial hypertension and Stargardt disease were confirmed.
• Peripapillary retina showed involvement, atypical for Stargardt disease.

Conclusions:

• The co-occurrence of Stargardt disease and idiopathic intracranial hypertension presents unique challenges.
• Disc edema in idiopathic intracranial hypertension may influence the typical presentation of Stargardt disease.
• Further research is needed to elucidate the relationship between these conditions.