TY - JOUR
T1 - Macular cysts in retinal dystrophy
AU - Ganesh, Anuradha
AU - Stroh, Eliza
AU - Manayath, George J.
AU - Al-Zuhaibi, Sana
AU - Levin, Alex V.
PY - 2011/9
Y1 - 2011/9
N2 - PURPOSE OF REVIEW: To describe the entity of macular cysts in retinal dystrophy, differentiate it from cystoid macular edema (CME), and review the role of carbonic anhydrase inhibitors in management. RECENT FINDINGS: Macular cysts in retinal dystrophy are seen in retinopathies caused by mutations in the NR2E3 gene, juvenile X-linked retinoschisis (XLRS), and some other retinal dystrophies. These must be distinguished from CME. Optical coherence tomography can clearly demonstrate intraretinal cysts which may not be clinically detectable. Intravenous fluorescein angiography (IVFA) does not show macular hyperfluorescence (i.e. leakage). Molecular genetic testing aids in the diagnosis and elucidation of pathophysiology. Carbonic anhydrase inhibitors may promote resolution of the cysts resulting in visual improvement. SUMMARY: Non-CME macular cysts in retinal dystrophies can be differentiated from CME by a combination of clinical examination, IVFA, and molecular genetic testing to identify causative phenotype. Carbonic anhydrase inhibitors may be effective in promoting resolution.
AB - PURPOSE OF REVIEW: To describe the entity of macular cysts in retinal dystrophy, differentiate it from cystoid macular edema (CME), and review the role of carbonic anhydrase inhibitors in management. RECENT FINDINGS: Macular cysts in retinal dystrophy are seen in retinopathies caused by mutations in the NR2E3 gene, juvenile X-linked retinoschisis (XLRS), and some other retinal dystrophies. These must be distinguished from CME. Optical coherence tomography can clearly demonstrate intraretinal cysts which may not be clinically detectable. Intravenous fluorescein angiography (IVFA) does not show macular hyperfluorescence (i.e. leakage). Molecular genetic testing aids in the diagnosis and elucidation of pathophysiology. Carbonic anhydrase inhibitors may promote resolution of the cysts resulting in visual improvement. SUMMARY: Non-CME macular cysts in retinal dystrophies can be differentiated from CME by a combination of clinical examination, IVFA, and molecular genetic testing to identify causative phenotype. Carbonic anhydrase inhibitors may be effective in promoting resolution.
KW - NR2E3 retinopathy
KW - X-linked retinoschisis
KW - carbonic anhydrase inhibitors
KW - fluorescein angiography
KW - macular cyst
KW - retinal dystrophy
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U2 - 10.1097/ICU.0b013e328349229e
DO - 10.1097/ICU.0b013e328349229e
M3 - Review article
C2 - 21730849
AN - SCOPUS:80051800594
SN - 1040-8738
VL - 22
SP - 332
EP - 339
JO - Current Opinion in Ophthalmology
JF - Current Opinion in Ophthalmology
IS - 5
ER -