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Klin Monbl Augenheilkd 2017; 234(04): 474-477
DOI: 10.1055/s-0042-121290
Der interessante Fall
Georg Thieme Verlag KG Stuttgart · New York

The Connection between HbS Heterozygosity and Unilateral Vision Loss in a Child with Congenital Glaucoma

Die Beziehung zwischen der HbS-Heterozygotie und dem einseitigen Verlust des Sehvermögens bei einem Kind mit angeborenem Glaukom
R. I. Bojinova
1   Canada Department of Medicine, McGill University, Montreal, Canada (Chair: Dr. James Martin)
,
M. G. Todorova
2   Department of Ophthalmology, University of Basel, Switzerland (Chair: Prof. Hendrik Scholl)
› Author Affiliations
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Publication History

Publication Date:
13 February 2017 (online)

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Background

Hemoglobin-S (HbS) trait, or HbS heterozygosity, is present in 300 million people worldwide [1]. Among the risk factors for HbS sickling crisis have been reported to be dehydration, cold, fever, physical exertion [2], [3], or other factors [4]. To our knowledge this is the first report linking HbS heterozygosity state with visual loss. In our patientʼs case, we identified mechanical stress on the ocular vascular system, produced by an increased intraocular pressure from congenital glaucoma, to be an important pathologic factor in the genesis of occlusive retinal disease and vision loss.

 

  • References

  • 1 Derebal VK, Lazarus JM. Sickle trait in African-american hemodialysis patients and higher erythropoesis-stimulating agent dose. J Am Soc Nephrol 2014; 40: 819-826
    PubMedGoogle Scholar
  • 2 Tsaras G, Owusu-Ansah A, Aboateng FO. et al. Complication associated with sickle cell trait: a brief narrative review. Am J Med 2009; 122: 507-512
    CrossrefPubMedGoogle Scholar
  • 3 Al-Abdulla NA, Haddock TA, Kerrison JB. et al. Sickle cell disease presenting with extensive peri-macular arteriolar occlusions in a nine-year-old boy. Am J Ophthalmol 2001; 131: 275-276
    CrossrefPubMedGoogle Scholar
  • 4 Fine LC, Petrovic V, Irvine AR. et al. Spontaneous central retinal artery occlusion in hemoglobin sickle cell disease. Am J Ophthalmol 2000; 129: 680-681
    CrossrefPubMedGoogle Scholar
  • 5 Gupta V, Jha R, Srinivasan G. et al. Ultrasound biomicroscopic characteristics of the anterior segment in primary congenital glaucoma. J AAPOS 2007; 11: 546-550
    CrossrefPubMedGoogle Scholar
  • 6 Roche O, Beby F, Dufier JL. et al. Descemet membrane rupture accompanied by stromal clefting in congenital glaucoma. Arch Ophthalmol 2008; 126: 1163-1164
    CrossrefPubMedGoogle Scholar
  • 7 Todorova MG, Parsa CF, Grieshaber MC. Ciliary body clefting accompanied by rupture of the trabecular meshwork in congenital glaucoma. Arch Ophthalmol 2012; 130: 534
    CrossrefPubMedGoogle Scholar
  • 8 Stefansson E. Ocular hypotony: what is the mechanism of effusion and oedema?. Acta Ophthalmol Scand 2007; 85: 584-585
    CrossrefPubMedGoogle Scholar
  • 9 Sears DA. The morbidity of sickle cell trait: a review of the literature. Am J Med 1978; 64: 1021-1036
    CrossrefPubMedGoogle Scholar