Sickle cell disease presenting with extensive peri-macular arteriolar occlusions in a nine-year-old boy

Nael A. Al-Abdulla, Tiffany A. Haddock, John B. Kerrison, Morton F Goldberg

Research output: Contribution to journalArticle

Abstract

PURPOSE: To report a dramatic occlusive event of the macula surrounding the foveal avascular zone, causing severe and permanent loss of vision in a child with sickle cell disease. METHODS: Case report. A nine-year-old boy with SS hemoglobinopathy and oculocutaneous albinism developed acute unilateral loss of vision. RESULTS: Ophthalmoscopy revealed a pale, milky white, thickened retinal lesion centered on the fovea in the right eye as well as foveal hypoplasia in the left eye. The presence of macular malformation associated with oculocutaneous albinism precluded formation of a cherry-red spot. Fluorescein angiography of the right eye demonstrated extensive occlusions of the arterioles surrounding the foveal avascular zone. The presence of occlusions surrounding the fovea from multiple directions suggested the possibility of central retinal artery occlusion with migration of microemboli downstream. CONCLUSION: The patient, the youngest case reported, developed an irreversible macular infarction that was not improved by an exchange erythrocyte transfusion. He was placed on a long-term monthly transfusion protocol to protect his unaffected eye.

Original languageEnglish (US)
Pages (from-to)275-276
Number of pages2
JournalAmerican Journal of Ophthalmology
Volume131
Issue number2
DOIs
StatePublished - 2001

Fingerprint

Sickle Cell Anemia
Oculocutaneous Albinism
Retinal Artery Occlusion
Ophthalmoscopy
Hemoglobinopathies
Erythrocyte Transfusion
Fluorescein Angiography
Arterioles
Infarction

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Sickle cell disease presenting with extensive peri-macular arteriolar occlusions in a nine-year-old boy. / Al-Abdulla, Nael A.; Haddock, Tiffany A.; Kerrison, John B.; Goldberg, Morton F.

In: American Journal of Ophthalmology, Vol. 131, No. 2, 2001, p. 275-276.

Research output: Contribution to journalArticle

Al-Abdulla, Nael A. ; Haddock, Tiffany A. ; Kerrison, John B. ; Goldberg, Morton F. / Sickle cell disease presenting with extensive peri-macular arteriolar occlusions in a nine-year-old boy. In: American Journal of Ophthalmology. 2001 ; Vol. 131, No. 2. pp. 275-276.
@article{195c40355ece408b9504b362d2a13b0e,
title = "Sickle cell disease presenting with extensive peri-macular arteriolar occlusions in a nine-year-old boy",
abstract = "PURPOSE: To report a dramatic occlusive event of the macula surrounding the foveal avascular zone, causing severe and permanent loss of vision in a child with sickle cell disease. METHODS: Case report. A nine-year-old boy with SS hemoglobinopathy and oculocutaneous albinism developed acute unilateral loss of vision. RESULTS: Ophthalmoscopy revealed a pale, milky white, thickened retinal lesion centered on the fovea in the right eye as well as foveal hypoplasia in the left eye. The presence of macular malformation associated with oculocutaneous albinism precluded formation of a cherry-red spot. Fluorescein angiography of the right eye demonstrated extensive occlusions of the arterioles surrounding the foveal avascular zone. The presence of occlusions surrounding the fovea from multiple directions suggested the possibility of central retinal artery occlusion with migration of microemboli downstream. CONCLUSION: The patient, the youngest case reported, developed an irreversible macular infarction that was not improved by an exchange erythrocyte transfusion. He was placed on a long-term monthly transfusion protocol to protect his unaffected eye.",
author = "Al-Abdulla, {Nael A.} and Haddock, {Tiffany A.} and Kerrison, {John B.} and Goldberg, {Morton F}",
year = "2001",
doi = "10.1016/S0002-9394(00)00778-9",
language = "English (US)",
volume = "131",
pages = "275--276",
journal = "American Journal of Ophthalmology",
issn = "0002-9394",
publisher = "Elsevier USA",
number = "2",

}

TY - JOUR

T1 - Sickle cell disease presenting with extensive peri-macular arteriolar occlusions in a nine-year-old boy

AU - Al-Abdulla, Nael A.

AU - Haddock, Tiffany A.

AU - Kerrison, John B.

AU - Goldberg, Morton F

PY - 2001

Y1 - 2001

N2 - PURPOSE: To report a dramatic occlusive event of the macula surrounding the foveal avascular zone, causing severe and permanent loss of vision in a child with sickle cell disease. METHODS: Case report. A nine-year-old boy with SS hemoglobinopathy and oculocutaneous albinism developed acute unilateral loss of vision. RESULTS: Ophthalmoscopy revealed a pale, milky white, thickened retinal lesion centered on the fovea in the right eye as well as foveal hypoplasia in the left eye. The presence of macular malformation associated with oculocutaneous albinism precluded formation of a cherry-red spot. Fluorescein angiography of the right eye demonstrated extensive occlusions of the arterioles surrounding the foveal avascular zone. The presence of occlusions surrounding the fovea from multiple directions suggested the possibility of central retinal artery occlusion with migration of microemboli downstream. CONCLUSION: The patient, the youngest case reported, developed an irreversible macular infarction that was not improved by an exchange erythrocyte transfusion. He was placed on a long-term monthly transfusion protocol to protect his unaffected eye.

AB - PURPOSE: To report a dramatic occlusive event of the macula surrounding the foveal avascular zone, causing severe and permanent loss of vision in a child with sickle cell disease. METHODS: Case report. A nine-year-old boy with SS hemoglobinopathy and oculocutaneous albinism developed acute unilateral loss of vision. RESULTS: Ophthalmoscopy revealed a pale, milky white, thickened retinal lesion centered on the fovea in the right eye as well as foveal hypoplasia in the left eye. The presence of macular malformation associated with oculocutaneous albinism precluded formation of a cherry-red spot. Fluorescein angiography of the right eye demonstrated extensive occlusions of the arterioles surrounding the foveal avascular zone. The presence of occlusions surrounding the fovea from multiple directions suggested the possibility of central retinal artery occlusion with migration of microemboli downstream. CONCLUSION: The patient, the youngest case reported, developed an irreversible macular infarction that was not improved by an exchange erythrocyte transfusion. He was placed on a long-term monthly transfusion protocol to protect his unaffected eye.

UR - http://www.scopus.com/inward/record.url?scp=0035138356&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0035138356&partnerID=8YFLogxK

U2 - 10.1016/S0002-9394(00)00778-9

DO - 10.1016/S0002-9394(00)00778-9

M3 - Article

C2 - 11228315

AN - SCOPUS:0035138356

VL - 131

SP - 275

EP - 276

JO - American Journal of Ophthalmology

JF - American Journal of Ophthalmology

SN - 0002-9394

IS - 2

ER -