Ocular Presentations of Sickle Cell Disease Patients in a Nigerian Hospital

Main Article Content

Chilota Chibuife Efobi
Obiora S. Ejiofor
Bernard C. Ochiogu
Chukwudi Charles Uzozie
Anthonia Udeaja
Adaora Amoge Onyiaorah


Introduction: Sickle Cell Disease (SCD) is an inherited haemolytic disorder that affects virtually all body systems including the eye. Ocular complications of SCD could have dire consequences if not detected early.

Objectives: We aimed at determining the prevalence of ocular complications of SCD in our patients.

Methods: This study was carried out on patients attending both the adult and Paediatric Sickle cell disease clinic at Chukwuemeka Odumegwu Ojukwu Teaching Hospital, Akwa, Anambra State, Nigeria, over an 8 month period. A structured questionnaire was administered to consenting patients to determine their sociodemographic variables and other characteristics. Visual acuity was determined with multiple optotype Snellen’s chart at 6 metres in natural daylight. Using pen-torch, the face, external eye examination, anterior segment examination and pupillary reaction to light were assessed. Magnified anterior segment examination was performed with slit-lamp biomicroscope.  All subjects underwent dilated fundoscopy (after instillation of tropicamide 0.5% eye drop) using Welch Allyn indirect ophthalmoscope. Results were analysed using SPSS version 22.0 Inc Chicago Illinois and represented in tables. Statistically significant P value was set at < 0.05.

Results: A total of 28 patients with age range of 6-42 years were seen. 17 (60.7%) were males, while 11 (39.3%) were females.  8 (28.6%) had at least one ocular anomaly comprising of refractory errors 3 (10.8%), sickle cell retinopathy 4 (14.3%) and glaucoma 2 (7.1%). Only 2 (7.1%) routinely visit eye clinic. SCD retinopathy was found to increase with age (P value= 0.005).

Conclusion: SCD retinopathy was found more in the older age group despite the small sample size at a prevalence rate of 14.3%. Early initiation of routine ocular examination for SCD patients will be beneficial to avert these complications. More efforts should be made at educating the patients. Larger studies will be required to determine the actual prevalence of ocular anomaly in SCD patients in Anambra State.

Sickle cell disease, eye manifestations, early detection.

Article Details

How to Cite
Efobi, C. C., Ejiofor, O. S., Ochiogu, B. C., Uzozie, C. C., Udeaja, A., & Onyiaorah, A. A. (2020). Ocular Presentations of Sickle Cell Disease Patients in a Nigerian Hospital. Journal of Advances in Medicine and Medical Research, 32(1), 95-102. https://doi.org/10.9734/jammr/2020/v32i130356
Short Research Article


Smith JS Diseases of the retina. In: Eye diseases in hot climate. 3rd Ed. Linancre Hoose, Jordan Hill: Jordan Hill, Oxford Boston Johannesburg. Button worth Heinemann. 1999;(Chpt 12):209-227.

Piel FB, Patil AP Howes RE, Nyangiri OA, Gething PW, Dewi M, et al. Global epidemiology of sickle hemoglobin in neonates. A contemporary geostatistical model-based map and population estimates, Lancet. 2013;381:142-151.
DOI: 10.1016/S0140-6736(12)61229-X

Epub 2012 Oct 25.

Diallo D. Tchernia G. Sickle cell disease in Africa. Curr Opia Hematol. 2002;9:111-116.
PMID: 11844993

Roberts 1, Dede montalembert M. sickle cell diseases as a paradigm of migration hematology. New challenges for hemato-logists in Europe. Haematologica 2007; 92(7):865-71.
PMID: 17606434
DOI: 10.3324/haematol.11474

Wealtheral DJ, Clegg JB. Inherited hemoglobin disorders; an increasing global health problem. World Health Organ. 2001; 79(8):704-12.
PMID: 11545326
PMCID: PMC2566499

Popma SE. ocular manifestations of sickle haemoglobinopathies. Clin Eye Vis care. 1996;8:111-17.
DOI: 10.1016/j.bjhh.2014.07.020 PMCID: PMC4318455 PMID: 25305162

Nagpal KC, Goldberg MF. Ocular manifestations of sickle cell hemoglobino-pathies. Surv Ophthalmic. 1977;21:391- 41.
PMID: 559355

To KW, Nadel AJ. Ophthalmologic complications in hemoglobinopathies. Hematol oncol Clin North. AM. 1991;5: 535-48.
PMID: 1864821

Fadugbagbe AO, Gurgel RQ, Mendonca CQ, Cipolotti R, Dos Santos AM, Cuevas LE. Ocular manifestations of sickle cell diseases. Ann Trop Paediatr. 2010;30:19-26.
PMID: 20196930

Khurana AK. Diseases of the retina: In comprehensive ophthalmology. 4th5th ed. Darayaganji. New Delhi: New Age International Ltd 20122. 2007;(Chpt11): 249-285.

Akinsola FB, Kehinde MO. Ocular findings in sickle cell disease patients in Lagos. Niger Postgrad Med J. 2004;11: 203-6.
PMID: 15505651

Moriarty BJ Acheson RW, Condon PI, Serjeant GR. Patterns of visual loss in untreated sickle cell retinopathy. Eye. 1988;2:330-5.
PMID: 198862

Harlan JB Jr. Goldberg MF. Management and Therapy of Eye Disorders in Sickle Cell Diseasel; 2000.
Available:http://sickle.bwh.harvard edu/eye

Fox PD, Dunn DT, Morris JS. Risk factors for proliferative sickle retinopathy. Br. J. Ophthalmic. 1990;74(3):172-176.
PMCID: PMC1042042
PMID: 2322516
DOI: 10.1136/bjo.74.3.172

Traore J, Boitre JP, Bogoreh IA, Traore L, Diallo A, sickle cell disease and retinal damage; a study of 38 cases at the African tropical ophthalmology institute (IOTA) in Bamko (in French) Med Trop (Mars). 2006; 66:252-4.
PMID: 16924816

Penman AA, Talbot IF, Chuang EL, Thomas P, Serjeant GR. Bird AC. New classification of peripheral retinal vascular changes in sickle cell disease. Br J. Ophthalmic 1994;78:681-9.
PMID: 7947547
PMCID: PMC504905
DOI: 10.1136/bjo.78.9.681

Downes SM, Hambleton IR, Chuang EL, Lois N, Serjeant GR, Bird AC. Incidence and natural history of proliferative sickle cell retinopathy observations from a cohort study. Ophthalmology. 2005;112: 1869- 75.

Fox PD, Dunn DT, Morris JS, Serjeant GR. Risk factors for proliferative sickle retino-pathy. Br J Ophthalmol. 1990;74(3):172- 6.

Garcia CA, Fernandes MZ, Uchôa UB, Cavalcante BM, Uchôa RA. Achados fundoscópicos em crianças portadoras de anemia falciforme no estado do Rio Grande do Norte. Arq Bras Oftalmol. 2002;65(6):615-8.

Allisson Mário dos Santos, Gustavo Baptista de Almeida Faro, Marcus Vinicius Melo do Amaral, Cristiano de Queiroz Mendonça, Bruno Campelo Leal, Rosana Cipolotti. Retinal impairment in young individuals with sickle cell anemia (Hemoglobin SS disease) in university hospital in Northeastern of Brazil. Arq. Bras. Oftalmol. 2012;75(5):313-5.

Kcent D, Arya R, Aclimondos WA, Bellighan AJ. Bird AC. Screening for ophthalmic manifestations of sickle cell disease in United Kingdom. Eye. 1994;8: 618-22.

George IO, Cookey SAH. Eye manifestation of children with homozygous sickle cell disease in Nigeria. J med. Med. Sci. Journal of Medicine and Medical Sciences. 2012;3(5):302-5305.

Osafo-Kwaako A, Kimani, Ilako D, Akafo S, Ekem I, et al. Ocular manifestations of sickle cell disease at the korle-bu hospital, accra, Ghana. Eur J Ophthamol. 2011; 21(4):484-89489.

Rautray K, Tidake P, Acharya S, Shukla S. Ocular manifestations in sickle cell disease. A preventable cause of blindness? J. appl. Dent. Med. Sci. 2015; 14:11-16.

Ravindra C, Nishikant T, Sangeeta C. Ocular manifestations in sickle cell disease (SCD) in children. (IJRTSAT) International Journal of Recent Trends in science and Technology. 2015;16(2):454-458.

Shukla P, Verma H, Patel S, Patra Pk, Bhaskar LVKS. Ocular manifestations of sickle cell disease and genetic suscepti-bility for refractive errors. Taiwan J Ophthalmos. 2017;7(2):89-93.

Chizzoti MT, Lavezzo MM. Sickle cell retinopathy: Diagnosis and treatment. Arq Bras Oftalmol. 2013;76(5):320-7.

Oluseye TS. Patterns and presentations of sickle cell retinopathy in Ibadan. J Clin Exp Opthalmol. 2012;3(9):1-3.

Bergren RL, Brown GC. Neovascular glaucoma secondary to sickle-cell retino-pathy. Am. J. Ophthalmol. 1992;113(6): 718-719.

Ahmad SS. Glaucoma suspects: A practical approach.Taiwan J Ophthalmol. 2018;8(2):74-81.

Ahmad SS. Glaucoma suspect: A practical approach.Taiwan J Ophthalmol. 2018;8(2): 74-81.

Kornmann HL, Gianconi JA. Treatment initiation in glaucoma suspects. US Ophthalmic Rev. 2014;7:45-9.

Goldberg MF: Cytological diagnosis of phagolytic glaucoma utitlizing Millipore filtration of the aqueous. Br J Opthalmol. 1967;51:847-53.

Riva I, Legramandi L, Katsanos A, Oddone F, Rulli E et al. Influence of socio-demographic factors on disease characteristics and vision related quality of life in primary open angle glaucoma patients: The Italian Primary Open Angle Glaucoma Study. J Glaucoma. 2018;27(9): 776-84.