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Ocular Findings in Elderly Cases of Homozygous Sickle-Cell Disease in Jamaica

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Ocular Findings in Elderly Cases of Homozygous Sickle-Cell Disease in Jamaica Br J Ophthalmol: first published as 10.1136/bjo.60.5.361 on 1 May 1976. Downloaded from Brit. Y. Ophthal. (1976) 6o, 36I Ocular findings in elderly cases of homozygous sickle-cell disease in Jamaica P. I. CONDON* AND G. R. SERJEANT From the MRC Laboratories (Jamaica), University of the West Indies, Kingston, Jamaica Sickle-cell disease is characterized by recurrent one each, and was unknown in five patients. vascular occlusive episodes and a progressive Traumatic causes were more common in younger obliteration of capillary beds. This process can patients and macular degeneration more common in be directly observed in the eye and its occurrence the older group. in the peripheral retina has been well documented Tortuosity of the major retinal vessels was (Welch and Goldberg, I966; Goldberg, 1971; present in i8 (30 per cent) patients affecting the Condon and Serjeant, I972a, b, c). The effects of veins alone in 13 and the arteries and veins in five. such thrombotic episodes might be expected to Peripheral retinal vessel disease was classified as accumulate with age and to be most marked in previously described (Condon and Serjeant, I972a) elderly patients. The results of ocular examinations into three grades. Grade I consisted of narrowing in 6o patients aged over 40 years with homozygous of peripheral arterioles with tortuosity and abnormal sickle-cell (SS) disease in Jamaica are presented looping of peripheral venules. Grade II included below. tortuosity, dilatation, and microaneurysmal for- mation in the peripheral capillary network; coarsen- Patients and methods ing of the network with loss of some fine vessels; and abnormal branching of peripheral venules. The patients attended the sickle-cell clinic of the Grade III changes were arteriolar occlusions. The http://bjo.bmj.com/ University Hospital of the West Indies. Ocular exami- grade of vessel disease was usually identical in both nation of 56 out of 66 patients aged over 40 years eyes (37 patients), although differences between (born before i January 1935) attending the clinic was possible. In addition, the cases of four patients (cases eyes of one grade (I2 patients) or even two grades 2, 31, 47, 54) aged over 40 years at the time of previous (six patients) did occur. Grade I changes were examinations, but who had since died, were studied. present in 40 eyes, grade II in 21, and grade III in The overall group therefore consisted of 6o patients 40. The peripheral retinal vessels appeared normal (27 men, 33 women) whose ages ranged from 40-66 in I 5 eyes. Arterio-venous fistulae occurred in on September 30, 2021 by guest. Protected copyright. years. four eyes (four patients) and was associated with Homozygous sickle-cell disease was diagnosed on grade III vessel disease in all cases. Retinitis criteria reported elsewhere (Serjeant, I974). Methods of proliferans occurred in 12 eyes (eight patients), the ocular examination have been previously described lesions being often multiple and affecting large (Condon and Serjeant, I972a). Retinitis proliferans and angioid streaks were confirmed in each case by fluores- areas of the peripheral retina. In four of the eight cein angiography. patients the lesions had fibrosed, presumably after spontaneous vessel occlusion. In order to assess the effect of age on peripheral Results retinal vessel disease and retinitis proliferans the The findings are summarized in Table I. Visual group was divided into those aged 45 years or acuity was decreased in 40 eyes (25 patients) and under (29 patients) and those aged 46 years or was 6/6o or less in eight eyes (eight patients). The over (31 patients). The results are summarized in causes included astigmatic refractive errors in Table II, which shows that mild grades of vessel four patients, trauma in three, retinal detachment disease (I and II) were more common in the in two, macular degeneration of unknown origin younger groups and that the more severe grade III in seven, a toxoplasma lesion, long-standing optic and retinitis proliferans predominated in the older atrophy, cataracts, and vitreous haemorrhage in group. Women also seemed to have more normal retinal vasculature and accounted for 5/7 (7I per *Present address: Regional Eye Department, Ardkeen Hospital, cent) patients with normal vessels and I2/14 Waterford, Ireland (86 per cent) of those with grade I vessel disease. Address for reprints: Ardkeen Hospital, Waterford, Ireland Chorioretinal scars known as 'sunburst' spots El Br J Ophthalmol: first published as 10.1136/bjo.60.5.361 on 1 May 1976. Downloaded from 362 British Journal of Ophthalmology Table I Octular findings in 6o patients aged 40 or over with homnozygous sickle-cell disease. Arabic figures refer to number of lesions in eye. See text Jor criteria for grading retinal vessel disease Tortuous Grade of Chorio- Micro- major retinal Arterio- retinal aneurysms Visual retinal vessel venous Retinitis sunburst of posterior Angioid Age acuity vessels disease fistulae proliferans lesions pole streaks Comments Case and (A =arterial, no. sex R L V = venous) R L R L R L R L R L R L 40 M 6/6 6/6 - II II - - - - 2 - - - - 2S 40 M 6/6 6/6o - II rr- - - (L) long-standing optic atrophy 3 40 F 6/6 6/6 V I I -- - 4 4I M 6/6 6/6 - III I -- - 5 4I M 6/6 6/6 AV If II -- - 6 4I M 6/6 I/6o V III -* - * - Traumatic maculopathy 7 4I M 6/6 6/6 - 8 4I F 6/6 6/6 - 9 42 M HM 6/6 V -* IrI -* - - Traumatic lesion (R) eye I0 42 M 6/6 6/6 V I I - - I I 42 M 6/6 6/6 V III II - 12 42 F 6/6 6/6 - I III -- - I3 42 F 6/9 6/I2 - I i-- - - Myopic astigmatism I4 42 F 6/6 6/6 - I III -- - 3- I I5 42 F 6/6 6/6 - II I -- -- i6 43 M 6/6 6/6 - - II I7 43 M 6/6 6/6 - II1 III - - - - I -.- I8 43 M 6/i8 6/24 - II III - - I9 43 M 6/36 6/6 - + + Healed toxoplasma lesion (R) macula 20 43 M 6/6 6/6 - II II - - I 4- + 2I 43 F 6/6 6/6 V I I -- - 22 44 F 6/6 6/6 - I I-- 23 44 F 6/6 6/6 V I I -- - 24 44 F 6/6 CF - I I -- - Traumatic lesion (L) eye 25 44 F 6/6 6/6 V III III -- 2 - I 26 44 F 6/6 6/6 - 27 45 F 6/6 6/6 - III II -- - - 28 45 M 6/6 6/6 I - - +-i.- http://bjo.bmj.com/ 29 45 F 6/6 6/9 V I I - - Macular degeneration -4- 30 46 M 6/I2 6/I2 - III III - - I + 3I 46 M 6/6 6/6 - II I -- - 32 46 F 6/I2 6/6 - II r - - I I - Macular degeneration 33 46 F 6/6 6/6 - 1 I - - 34 47 M 6/I2 6/I2 V I1 I - - Macular degeneration 35 47 F 6/6 6/6 - II 1II - - 36 48 M 6/I2 6/9 - III I - - I I 37 48 F 6/9 6/9 - I I- - 38 49 M 6/6 6/6 - 1II iii -- - 9 3 on September 30, 2021 by guest. Protected copyright. 39 49 M 6/6 6/6 - III III - 2 1 40 49 M 6/6 6/6 - I III - - 4I 49 F 6/6 6/6 - I I -- - 42 49 F 6/6 6/6 AV 43 49 F 6/i8 6/I8 - I I - - - Macular degeneration -* -* 44 50 M Nil 6/I2 - III -* I -* 3 -* I 4. - ?Retinal detachment (R) 45 53 M 6/6 6/6 V II I - - + 46 53 F 6/9 6/9 - - 2 47 53 F 6/I8 6/I2 - 1I IHI - - I - Macular degeneration 48 ss F 6/I2 6/I2 V II I - - - Myopic astigmatism 49 55 F 6/6 6/6 - I I - - so 56 F 6/6 6/24 AV III III - - 2 3 - (L) perimacular fibrosis from vitreous haemorrhage 50 57 F 6/6 6/6 - 52 58 F 6/24 6/6 - III III F--+ + Amblyopia F 53 59 6/6 Nil - III -. - -* 6 -s - -s - -+ - -, Retinal detachment (L) 54 59 F 6/36 2/6o - r III --. - Senile macular degener- ation and cataracts 55 59 F 6/I2 6/24 AV III III - - - - - - - - + (L) vitreous haemorrhage 56 6o M 6/6 6/6 - III III.--± 57 6o F 6/36 6/24 AV + Macular degeneration 58 6o F 6/6 6/6 - - 59 6i F 6/36 6/6o - Senile cataracts 6o 66 M 6/6 6/6 V III III. *Inapplicable: peripheral retina could not be seen Severity of lesion: +, ++, +++ Br J Ophthalmol: first published as 10.1136/bjo.60.5.361 on 1 May 1976. Downloaded from Homozygous sickle-cell disease 363 Table II Age distribution of peripheral retinal vessel disease and retinitis proliferans Eyes affected Grade of vessel disease Age Eyes Normal - ---. (years) No. observed* vessels I II III RP No. Percentage No. Percentage No. Percentage No. Percentage No. Percentage 40-45 29 56 7 12 22 38 13 22 14 24 I 2 46-60 31 6o 8 13 I8 29 8 I3 26 42 7 II *Excludes four eyes in which assessment of the peripheral retina was impossible occurred in 2o eyes (i6 patients). Microaneurysms are presumed to precede the development of at the posterior pole were seen in 12 eyes (nine retinitis proliferans, occurred mainly in the youn- patients).
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