SPONTANEOUS REGRESSION (AUTOINFARCTION) OF PROLIFERATIVE SICKLE RETINOPATHY KRISHAN C. NAGPAL, M.D., DIMITRIOS PATRIANAKOS, M.D., GEORGE K. ASDOURIAN, M.D., MORTON F. GOLDBERG, M.D., MAURICE RABB, M.D., AND LEE JAMPOL, M.D. Chicago, Illinois Spontaneous regression of proliferative retinopathy is well documented in "diabetes mellitus 1 ' 2 and retrolental fibroplasia, 3 '* al- though the factors responsible for such vascular involution are not known. 5 Davis 8 studied the role of vitreous detachment in proliferative diabetic retinopathy and found that complete vitreous detachment may result in regression of retinopathy with fewer hemorrhages and disappearance of many new vessels. Our initial experience 7 sug- gested that spontaneous regression of new vessels (sea fans) was extremely rare in proliferative sickle retinopathy. Although Okun 8 and later Condon, and Serjeant 9 showed isolated cases of what we now con- sider to be autoinfarcted retinal sea fans in sickling diseases, the concept of auto- infarction has never been stressed in this disease. SUBJECTS Of 365 patients who were screened, 152 patients were observed periodically (every six to 12 months). Forty-five patients had proliferative sickle retinopathy (stages III, IV, and V). 7 Each of these patients had a detailed fundus drawing, fundus photog- raphy, and fluorescein angiography. Of these 45 patients, nine (20%) showed autoinfarc- tion of retinal sea fans. In one patient, sea fan infarction occurred twice during the period of observation. In the others, autoinfarction had apparently From the Sickle Cell Eye Clinic, University of Illinois Eye and Ear Infirmary, Chicago. This study was supported in part by contract NIH72 29S6B and IP 18 HL 15168-03 from the National Heart and Lung Institute, Bethesda, Maryland. Reprint requests to Morton F. Goldberg, M.D., University of Illinois Eye and Ear Infirmary, 1855 W. Taylor St., Chicago, IL 60612. occurred before our first examination. Pa- tients were 13 to 55 years of age with an average age of 31.2 years. The age range of the nine patients with autoinfarction of retinal sea fans was 26 to 54 years with an average age of 39.2 years. The difference of years between the two averages suggests a fairly long interval during which sea fans were actively perfused. However, we ob- served autoinfarction that occurred within two days (Case 1). Of these patients, 25 were male and 20 female; five men and four women had spontaneous involution of pro- liferative sickle retinopathy. Most patients with proliferative sickle rctinnpathv had sirklp rpll hemoglobin C disease (SC) and sickle cell anemia (SS) (Table). Of those patients with autoinfarc- tion of sea fans, eight had hemoglobin SC disease, and one had sickle cell thalassemia (Sthal). CASE REPORTS Case 1—A 26-year-old black woman with hemo- globin SC disease was seen in 1972 because of vit- TABLE HEMOGLOBIN ELECTROPHORESIS OF PATIENTS WITH PROLIFERATIVE SICKLE RETINOPATHY* AND SPONTANEOUS REGRESSION Hemoglobin Typef Patients Patients with Spon- with taneous Regression PSR of PSR AS AC ss SC Sthal 2 1 9 29 4 0 0 0 8 1 * PSR indicates proliferative sickle retinopathy. t AS indicates sickle cell trait; AC, hemoglobin C trait; SS, sickle cell anemia; SC, sickle cell hemoglobin C disease; and Sthal, sickle cell thal- 885