[Retinal findings in the hereditary persistence of fetal hemoglobin and glucose-6-phosphate dehydrogenase deficiency (G-6-PD deficiency)].

Peripheral arteriolar occlusions of the retinal vessels and the 'black sunburst sign' as the first stage of sickle-cell retinopathy (Goldberg 1976) were found in a 34-year-old black patient from West Africa with hereditary persistence of fetal hemoglobin (HPFH) and glucose-6-phosphate dehydrogenase deficiency (G-6-PD-D). The patient's son was found to have HPFH without G-6-PD-D, his daughter HPFH as well as G-6-PD-D. Ophthalmoscopic findings in the two children were normal. In the mother - a white German without clinical symptoms - neither HPFH nor G-6-PD-D were found. This is the first description of the typical picture of 'sickle-cell retinopathy' in a patient with HPFH combined with G-6-PD-D. HPFH is not associated with any retinal symptoms or hematological abnormalities. Hemolytic crises induced by fava beans or drugs in G-6-PD-D alone lead to different ocular complications from those found in this patient.