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

Abstract

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. [ Ophthalmic Surg Lasers Imaging Retina. 2019;50:719–725.]

Optical Coherence Tomography Features of Choroidal Neovascularization and Their Correlation With Age, Gender, and Underlying Disease

“…This seemed to confirm the usefulness of this sign to distinguish iCNV from AMD-CNV, as previously reported. 16–18 However, multiple linear regression analysis found no significant effect of the lesion type on the PFS prevalence after accounting for the subject age, and subjects with PFS were significantly younger than those without it in all groups. Furthermore, the presence/absence of a PFS did not affect the odds for a specific diagnosis in the logistic regression.…”

“…11 However, the PFS has also been described in CNV related to noninflammatory conditions, making the specificity of this sign a topic of debate. 5,18 There is a need to identify features on OCT that can help in differentiating CNV related to conditions such as AMD, uveitis, and myopia, the three most common causes. In this study, we have analyzed the OCT features from a large cohort of patients with iCNV, myopic CNV, and AMD-related CNV to characterize the specific OCT features that can help in identifying the underlying etiology.…”

“…11 However, the PFS has also been described in CNV related to noninflammatory conditions, making the specificity of this sign a topic of debate. 5,18…”

Retinal Optical Coherence Tomography Imaging Biomarkers: A Review of the Literature

“…39 Recent evidence, however, suggests that the pitchfork sign may not be specific to inflammatory type 2 CNV. 40,41 Disorders Relating to Toxicity Flying saucer sign. The flying saucer sign was first described by Chen et al 42 in patients with suspected hydroxychloroquine retinopathy.…”

Choroidal Neovascularization Associated with Rare Entities

“…Various retinal pathologies including macular hole, subhyaloid hemorrhage, sub-internal limiting membrane (sub-ILM) hemorrhage and outer retinal disruption following laser pointer injury have been described [78,79]. However, reports of laser pointer induced CNV are sporadic [80]. Fujinami et al [81] reported on an intellectually disabled child who suffered from CNV following presumably frequent and repeated exposure to a laser pointer as a toy.…”