Our 54YO patient had a strong family history for macular problems. The fundus photos demonstrate radial cuticular drusen.
The OCT scans are even more dramatic with a saw-tooth pattern of drusen. Subretinal hyperreflective material (SHRM) is noted between Bruch’s membrane and the RPE and subretinal fluid is present in the right eye.
Learning Points:
Dominantly inherited radial basal laminar drusen (Doyne’s honeycomb macular dystrophy, Malattia Levantinese) is a rare genetic macular disorder. A defect in the EFEMP1 gene on chromosome 2p16 is thought to cause an abnormally thickened RPE basement membrane.
Centrally large, nodular and confluent drusen are noted, along with a temporal radiating pattern of smaller cuticular drusen. Later there may be variable amounts of RPE hyperplasia and fibrous metaplasia. Macular neovascularization may also develop.
