Frontiers in Cardiovascular Medicine · Oct 30, 2026
Paracentral acute maculopathy (PAMM) associated with severe hypertriglyceridemia: a case report with a new therapeutic approach
By Sarah Kleemann, Charlotte Egbring, Jens Julian Storp et al.
Purpose: To report a case of severe hypertriglyceridemia in a young male patient who developed paracentral acute middle maculopathy (PAMM) followed by a second retinal arterial occlusion, presumably caused by lipogenic emboli. The case highlights the importance of interdisciplinary management and a multimodal therapeutic approach. Case Presentation: A 31-year-old man presented with unilateral visual loss. Best-corrected visual acuity was 0.8 in the right and 0.16 in the left eye. Anterior segment examination was unremarkable. Funduscopy revealed whitish intravascular deposits within the central retinal artery and its branches. Optical coherence tomography demonstrated hyperreflective bands in the inner nuclear layer of the superior macula, consistent with PAMM. Fluorescein angiography showed mild vascular leakage corresponding to the vascular deposits. Eight days later, the patient developed a second retinal ischemic event involving a macular branch of the central retinal artery in the inferior papillomacular bundle, resulting in a paracentral scotoma. Due to the short interval between events, an extensive diagnostic work-up was performed to exclude acute cerebral ischemia and major vascular occlusive disease. Cardiovascular risk assessment revealed persistently severe hypertriglyceridemia and minor metabolic abnormalities, prompting endocrinological evaluation and genetic testing for a possible familial lipid disorder. Therapy: Initial treatment consisted of lifestyle modification, including dietary changes and increased physical activity, combined with statin and fibrate therapy. Despite these measures, triglyceride levels remained markedly elevated and retinal findings showed little improvement, partly due to delayed adherence to dietary recommendations, particularly reduction of sugar and rapidly absorbable carbohydrates. Given the risk of permanent visual loss, treatment was escalated to five consecutive days of lipid apheresis and one session of plasmapheresis, accompanied by intensive nutritional counseling. This combined approach resulted in a reduction in triglyceride levels, disappearance of retinal embolic deposits, and substantial visual recovery. Final visual acuity in the affected eye improved to 0.8, with minor residual paracentral scotoma. Conclusions: Severe hypertriglyceridemia may be associated with recurrent retinal vascular occlusive events. Successful management required close interdisciplinary collaboration. While lipid apheresis and pharmacological therapy contributed to the favorable outcome, sustained lifestyle modification was essential. This multimodal treatment strategy prevented further retinal ischemic events and preserved visual function.