This informative case series evaluates 4 eyes of 2 patients presenting with rapidly progressive vision loss secondary to cancer-associated retinopathy. The authors identified a distinct spectral-domain optical coherence tomography structural feature termed the angular sign of Henle fibre layer hyperreflectivity (ASHH). These bilateral, oblique hyperreflective bands extended from the outer plexiform layer to the ellipsoid and interdigitation zones, mirroring the anatomical angulation of Henle’s layer. Crucially, these specific lesions preceded extensive ellipsoid zone collapse and severe outer retinal degeneration characterised by granular hyperreflectivity. Full-field electroretinography demonstrated marked attenuation of both a- and b-waves, confirming widespread outer retinal and photoreceptor dysfunction. Serial fundus autofluorescence showed expanding macular hypoautofluorescence corresponding with progressive structural loss over time. Despite prompt, aggressive local and systemic immunosuppression using high-dose corticosteroids and bilateral intravitreal dexamethasone implants, retinal degeneration advanced relentlessly in all eyes. Final visual outcomes were poor, ranging from temporarily preserved central islands to hand motion and light perception. The study provides clear, detailed imaging characterisation and offers valuable clinical insight into the early presentation of autoimmune paraneoplastic processes. Recognising ASHH provides clinicians with a critical diagnostic window to prompt immediate oncologic investigation and initiate aggressive immunotherapy before irreversible ellipsoid zone collapse occurs. Although limited by a small sample size inherent to rare paraneoplastic conditions, the study’s findings on ASHH as a sensitive, early prognostic biomarker of acute outer retinal injury are highly credible, actionable and clinically relevant for everyday practice.
Early biomarker for paraneoplastic retinopathy
Reviewed by Anitha Priya Arun Shankar
Angular sign of henle fiber layer hyperreflectivity (ASHH) in cancer-associated retinopathy.
