Multimodal and clinical findings of three cases with stellate multiform amelanotic choroidopathy

Abstract

Purpose

To present the clinical and multimodal imaging features of three patients diagnosed with stellate multiform amelanotic choroidopathy (SMACH), a recently described, rare chorioretinal entity.

Methods

Case series.

Results

All patients, aged 5, 10, and 38 years, presented with unilateral, yellowish choroidal lesions extending from the fovea toward the temporal macula. Multimodal imaging was used for diagnosis. Structural disorganization of the subfoveal choroid with irregular, undulating hyperreflective alterations of the retinal pigment epithelium/Bruch's membrane complex with focal protrusions was noted in all patients, while subretinal fluid (SRF) was present in two patients at presentation on optical coherence tomography (OCT). No patient had systemic findings. The first and the third cases received intravitreal bevacizumab injections and the SRF persisted with fluctuations in both patients. In the second case, although there was no SRF initially, it occurred with fluctuations during follow-up. Throughout the follow-up periods, visual acuities remained stable in all patients without any additional treatment.

Conclusion

SMACH should be considered in the differential diagnosis of choroidal structural disorganization, particularly in young patients. Notably, one patient was 5 years old, which represents the youngest age reported to date. Multimodal imaging modalities are essential for the recognition of the disease and a confirmed diagnosis of SMACH may prevent unnecessary treatments such as intravitreal injections and photodynamic therapy.