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Year : 1984  |  Volume : 32  |  Issue : 4  |  Page : 201-204

Ocular changes in sickle cell haemoglobinopathies

Indira Gandhi Medical College and Mayo Hospital, Nagpur, India

Correspondence Address:
S K Kate
31-Corporative Colony, North Ambazari Road, Nagpur-10
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Source of Support: None, Conflict of Interest: None

PMID: 6571499

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How to cite this article:
Kate S K, Segokar V E, Sutaria S, Mokadam P J. Ocular changes in sickle cell haemoglobinopathies. Indian J Ophthalmol 1984;32:201-4

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Kate S K, Segokar V E, Sutaria S, Mokadam P J. Ocular changes in sickle cell haemoglobinopathies. Indian J Ophthalmol [serial online] 1984 [cited 2023 Feb 5];32:201-4. Available from: https://www.ijo.in/text.asp?1984/32/4/201/27388

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Ocular changes in sickle cell anaemia were first described by Cook[1]. Shukla and Solanki[2] conducted sickle cell studies at Nagpur and found 22.2 per cent sickle cell trait in Mahar community in 450 individuals while Das et a1[3] found more prevalence of sickle cell disease in this community.

Since very little work has been done so far on this topic it was thought worth-while to study this topic.

  Material and method Top

Those patients who attended the hospital with clinical syndrome suggestive of haemo­globinopathy were subjected for Sickling test[4], Alkaline Denaturation Tests, Citrate Agar Electrophoresis[6]. Patients with hyper­tension and diabetes were excluded from the study.

Fifty cases were taken up for the study. The detailed history was taken of such sickle cell disease patients with emphasis on ocular symptoms. Thorough systemic examination of these patients was done. Detailed ocular examination was done including Visual acuity, colour vision field charting furndus examination, _ I.O.P. recording. Fluorescein angiography was done in patients having ophthalmoscopical evidence of ocular changes in the patients studied in this series.

  Observations Top

(1) Electrophoresis was done in fifty cases which revealed that 35 patients (70 per cent) had AS haemoglobinopathy, 10 patients (20 per cent) had SS haemoglobinopathy and 5 patients (10 per cent) had ASF haemoglob­inopathy. Haemoglobin `SC' was not obser­ved in the study.

(2) Ninety-two percent of the patients were from Mahar community, four per cent from Teli community and two patients were from Gond community.

(3) Male to-female ratio was 1:1

(4) Seventy-five per cent of the patients were in the age group of 11 to 30 years. Mean age was 26.4 years.

(5) Symptomatology : Seventy-four per cent of the patients had no ocular symptoms. Painful eye was observed in 12 per cent of the cases. Diminished vision was the complaint in 8 per cent of the patients. Watering of eyes was present in only two cases. On patient complained of black spot before eyes.

(6) Ocular findings : On external examina­tion eyebrows, eye lids, palpebral fissure revealed nothing abnormal.

Thirteen patients had icterus. Grade I con­junctival sign was observed in 13 patients, out of which 10 patients were having `SS' haemoglobinopathy. Three patients had `AS' haemoglobinopathy. Patients of haemo­globin 'ASF' had normal conjunctival vas­culature. Grade II, III and IV conjunctival signs were not observed in this study.

Cornea, anterior chamber were normal in all the cases. Iridocyclitis and iris atrophy was observed in one patient. One patient had traumatic cataract in the left eye. In 92 percent of the cases vision was not significantly affec­ted. Intraocular tension was observed to be within normal limits. Field of vision and colour vision was normal in 49 cases. One patient had cataract and vitreous opacity, hence could not be tested.

Thirty-four patients of `AS' haemoglobin­opathy had normal retina. One patient had vitreous haemorrhage. Seven patients having 'SS' haemoglobinopathy revealed normal retina, while whitening of retina was found in two patients. Arteriovenous anastomosis was found in one patient only. All the patients of 'ASF' haemoglobinopathy had normal retina [Table - 1].

In 13 patients who had ophthalmoscopical evidence of sickle cell retinopathy Flouores­cein Angiography was done. Venous tor­tuosity and venous fullness was observed in 24 per cent of the cases. Altered AV ratio in two per cent, macular and paramacular exudates in six per cent and choroidal sclerosis was observed in 2 per cent of the patients in the present study [Table - 1][Table - 2].

  Discussion Top

This study reveals that the incidence of retinopathy is paractically non-existant in heterozygous (AS), while it is greater in homozygous (SS). Similar findings were reported by Rubinstein[7].

It is obserned that 74 per cent patients were having normal conjunctival vasculature (Grade 0), 26 per cent of the patients were hav­ing only occasional abnormal long linear dilatation of vessels still connected to vas­cular net work (Grade I). Grade II, III and IV conjunctival signs were not seen in the pre­sent study. Further analysis of our data and compar ison is made with study of Nagpal[3]. Higher percentage of involvement of con­junctival vasculature observed by Nagpal is because his data contains more number of patients, having haemoglobin `SS' and `SC'. Haemoglobin `SC' is not observed in our series. Patients having haemoglobin 'ASF' had normal conjunctival vasculature. It can be concluded that conjunctival vasculature was observed to be normal in heterozygous, while there is involvement in homozygous patients.

In our series one patient had iris atrophy. None of the patients had changes in lens. Pallor of optic disc was seen in two cases.

Macular infarct, microaneurysm as described in literature were not observed in this study:

Goldberg 10 classified it into five stages and further sub-stages depending on the extent of fundus. He described 'veno-occlusive disease due to sickling' is the initiative event. Though high incidence of retinal involvement is reported in the literature, the retina was almost intact in majority of the cases. In our series less number of cases having `SS' haemoglobin is included. haemoglobin `SC' is riot observed.

  Summary Top

Twenty-six per cent of the patients of sickle cell disease had ocular changes in the present series, out of which 20 per cent were homozygous (Haemoglobin `SS') and 6 per cent were heterozygous (Haemoglobin `AS'). The incidence of ocular changes is practically non-existant in heterozygous, while it is greater in homozygous. Ocular manifes­tations may be seen in conjunctiva, uvea and retina although they are not severe enough to affect the vision.

  Acknowledgement Top

The authors are thankful to Dr. S.N. Patil and Dr. Yeshwant Kole for their help and also to Dr. P.Y. Deshmukh, Dean, Indira Gandhi Medical College, Nagpur, for permission to publish this paper.[9]

  References Top

Cook. W.C'., 1930. Jour. Med. 11:541.  Back to cited text no. 1
Shukla, R.N. and Solanki, B.R., 1958. Lancet, 1: 297.  Back to cited text no. 2
Das, S.R.: Bhattacharya, P.N.; Sastry, D.P. and Kumar, N., 1961, Jour. Roy. Anthrop. Instt. 91:Pt2.  Back to cited text no. 3
Donald. G.A. and Castle, W.B., 1948, Jour. Lab. & Clin. Med. 33:1082.  Back to cited text no. 4
Singer, K.: Chernoff. A.I. and Singer. I., 1953, Blood, 6:413.  Back to cited text no. 5
Paul, F.M.; Goolen, H.M.. 1975, Amer. Jour. Clin. Path. 64:63.  Back to cited text no. 6
Ruhinstein, K. 1967, Brit. Jour. Ophthal. 51:217.   Back to cited text no. 7
Nagpal, K.C.; Asiourian, G.; Goldberg. M.G. and Huamonte, 1976. Brit. Jour. Ophthal. 60:31.  Back to cited text no. 8
Goldberg, M.F., 1971, Amer. Jour. Ophthamol. 71: 469.  Back to cited text no. 9


  [Figure - 1], [Figure - 2], [Figure - 3]

  [Table - 1], [Table - 2]


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