Potential Effects of Hormone Therapy in Type 2 Idiopathic Macular Telangiectasia

Abstract

Purpose: To investigate the influence of hormone therapy with tamoxifen or estrogens on morphological changes in macular telangiectasia (MacTel) type 2 patients as revealed clinically in multiple imaging modalities. Methods: Patients with a history of tamoxifen or estrogen use were selected from the cohort of the MacTel Study. A race-, age- and best-corrected visual acuity-matched group of MacTel participants not under hormone therapy served as the comparison group. The frequencies of typical features of the MacTel phenotype apparent in color fundus, red-free, fluorescein angiographic and optical coherence tomographic images were graded and analyzed statistically. Results: Thirty-nine MacTel patients were included in the analyses, of whom 13 were receiving tamoxifen, 13 estrogens and 13 patients no hormone treatment. Patients treated with estrogens showed significantly fewer breaks in the ellipsoid zone on optical coherence tomography (7 eyes, 29.1%, vs. tamoxifen: 14 eyes, 53.8%, and vs. controls: 14 eyes, 53.8%, p = 0.04 in both analyses, Fisher exact test). Retinal crystalline deposits were significantly more frequent in patients receiving estrogens (12 eyes, 16.2%, vs. 2 eyes, 2.7%, p = 0.003, Fisher exact test). No significant between-group differences were apparent with regard to other features of the phenotype (extent of retinal low reflective spaces, late hyperfluorescence on fluorescein angiography or retinal thickness). Conclusions: Tamoxifen treatment does not seem to accentuate structural changes in patients with MacTel type 2. Treatment with estrogens may exhibit a neuroprotective effect as suggested by the decreased frequency of ellipsoid zone breaks in corresponding patients, although corroborative studies are warranted to confirm these exploratory data.

Tamoxifen retinopathy and macular telangiectasia: A comparative case series

“…There is another set of publications with contrasting results on the progression of MacTel in patients with and without history of tamoxifen use. 7,8 Behrens et al 7 supported the theory that concurrent tamoxifen use in a type 2 MacTel patient could accentuate the fovea atrophy while another paper by Wolf-Schnurrbusch et al suggested that simultaneous use of tamoxifen in a type 2 MacTel did not hasten the structural changes on OCT in type 2 MacTel cases. 8…”

“…There is another set of publications with contrasting results on the progression of MacTel in patients with and without history of tamoxifen use. 7,8 Behrens et al 7 supported the theory that concurrent tamoxifen use in a type 2 MacTel patient could accentuate the fovea atrophy while another paper by Wolf-Schnurrbusch et al suggested that simultaneous use of tamoxifen in a type 2 MacTel did not hasten the structural changes on OCT in type 2 MacTel cases. 8 In this retrospective study, we intended to compare the clinical and imaging findings of a subset of patients with and without tamoxifen retinopathy and another subset of patients with type 2 MacTel with and without history of tamoxifen use.…”

“…7,8 Behrens et al 7 supported the theory that concurrent tamoxifen use in a type 2 MacTel patient could accentuate the fovea atrophy while another paper by Wolf-Schnurrbusch et al suggested that simultaneous use of tamoxifen in a type 2 MacTel did not hasten the structural changes on OCT in type 2 MacTel cases. 8 In this retrospective study, we intended to compare the clinical and imaging findings of a subset of patients with and without tamoxifen retinopathy and another subset of patients with type 2 MacTel with and without history of tamoxifen use. Through this study, our main objective was to diagnose cases with tamoxifen retinopathy and also suspect cases with possible tamoxifen use in patients with type 2 MacTel.…”

Improvements in visual acuity and macular morphology following cessation of anti-estrogen drugs in a patient with anti-estrogen maculopathy resembling macular telangiectasia type 2: a pathogenic hypothesis

“…Findings such as intraretinal crystals, RPE abnormality, and foveal hypo-reflective space on optical coherence tomography (OCT), as seen in patients with tamoxifen retinopathy, may also be observed in patients with macular telangiectasia type 2 (MacTel-2), showing similarity of clinical findings between the two diseases [5]. A previous study of 17 cases with medical history of tamoxifen administration reported MacTel-2-like findings [5–7]. However, the relationship between anti-estrogen drugs and MacTel-2 is poorly understood.…”