Clinical and imaging features of SMACH

Abstract

Background/Obejectives To describe clinical and multimodal imaging features of patients with stellate multiform amelanotic choroidopathy (SMACH). Subjects/Methods This retrospective multicenter study included patients with SMACH at Severance Eye Hospital and Seoul National University Bundang Hospital from January 2005 to October 2024. Results The mean age of SMACH at initial diagnosis was 30.48 +/- 22.52 years from 8 eyes of 8 patients. The cohort comprised 3 males and 5 females. Fluorescein angiography (FA) demonstrated a core filled with angiomatous vessels accompanied by mild leakage, irrespective of the presence of subretinal fluid (SRF). Indocyanine green angiography (ICGA) revealed prominent filling defects corresponding to the choroidal lesions, and near-infrared reflectance imaging further showed marked hyperreflectivity along the surface of the dendritic mass lesions, which distinctly highlighted the stellate configuration of the tumors.Three patients (3/8) had SRF. Whether treated or not, the SRF was almost completely absorbed, and visual acuity fully recovered. Most patients with SMACH maintained good visual acuity above 0.1 (logMAR). Conclusions In patients with SMACH, typical findings in FA and ICGA are presented to facilitate differentiation from other diseases. Understanding the disease entities and favorable prognosis of SMACH should aid clinicians in differentiating these lesions from other tumor diseases.