Angular Sign of Henle Fiber Layer Hyperreflectivity (ASHH) in Cancer-Associated Retinopathy.

Abstract

Purpose: To describe the longitudinal multimodal imaging findings of two patients with cancer-associated retinopathy (CAR) presenting with the angular sign of Henle fiber layer hyperreflectivity (ASHH) on spectral-domain optical coherence tomography (SD-OCT).

Methods: Retrospective case series.

Results: A total of two patients (an 88-year-old woman and a 63-year-old man, respectively) with systemic malignancy (endometrial cancer and metastatic prostate cancer, respectively) presented with bilateral ASHH lesions on OCT in the setting of rapidly progressive vision loss. Baseline visual acuity (VA) ranged from 20/30 to 20/60. In both cases, ASHH lesions preceded the development of extensive ellipsoid zone (EZ) loss and outer retinal atrophy with granular hyperreflectivity. Despite systemic therapy and local steroid injections, both patients showed poor anatomical and functional outcomes, with final VA ranging from 20/30 to light perception, and significant visual field constriction.

Conclusion: CAR eyes in our series who presented with ASHH lesions demonstrated a rapidly progressive course, culminating in severe EZ loss and outer retinal atrophy with characteristic hyperreflective granularity on OCT. The presence of ASHH lesions may serve as an early OCT biomarker of photoreceptor toxicity in paraneoplastic retinopathy and is a poor prognostic factor.