En Face OCT of Type 2 Neovascularization: A Reappraisal of the Pitchfork Sign

BACKGROUND AND OBJECTIVES: To describe a new en face optical coherence tomography (OCT) imaging feature of type 2 choroidal neovascularization (CNV) and illustrate the OCT angiographic (OCTA) findings in these eyes. PATIENTS AND METHODS: Multimodal images of patients with type 2 CNV who displayed the typical "pitchfork" sign with cross-sectional OCT were reviewed. Corresponding en face structural OCT and OCTA images were analyzed to correlate the finding before and after anti-vascular endothelial growth factor (VEGF) therapy. RESULTS: Five eyes of five patients, including two females and three males with a median age of 13 years (range: 8 years to 84 years), were studied. The etiology for type 2 CNV was laser-induced maculopathy in two eyes, idiopathic in two eyes, and age-related macular degeneration in one eye. None of the eyes had evidence of inflammatory ocular disease. En face OCT displayed a characteristic wreath-like pattern of hyperreflective spikes surrounding the type 2 neovascular membrane that originated from the ellipsoid zone and extended into the outer nuclear layer. Wreath-like spikes resolved with intravitreal anti-VEGF injection with good visual outcomes and transformation of the neovascular lesion from a type 2 to type 1 morphology. CONCLUSIONS: Type 2 CNV associated with the pitchfork sign with cross-sectional OCT displayed a characteristic wreath-like pattern of hyperreflective spikes with en face OCT that resolved with anti-VEGF therapy. This form of type 2 neovascularization may occur in eyes with different underlying etiologies and without signs of intraocular inflammation and is not limited to pediatric patients.