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Intravitreal avastin for choroidal neovascularization associated with stargardt-like retinal abnormalities in pseudoxanthoma elasticum.

Querques G, Bux AV, Prascina F, Noci ND - Middle East Afr J Ophthalmol (2010)

Bottom Line: A 57-year-old woman with diagnosis of angioid streaks, retinal flecks, and chorioretinal Stargardt-like atrophy due to PXE was referred to our department for sudden decreased vision in her RE (20/160).At the 1-month follow-up, visual acuity (VA) improved (20/40), and FA and OCT revealed the CNV closure.Further investigations are required to confirm our results.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Policlinico Riuniti di Foggia, University of Foggia, Foggia, Italy.

ABSTRACT
The aim of the study was to describe a patient with pseudoxanthoma elasticum (PXE), showing Stargardt-like retinal abnormalities, who underwent treatment with intravitreal bevacizumab for subfoveal choroidal neovascularization (CNV) of the right eye (RE). A 57-year-old woman with diagnosis of angioid streaks, retinal flecks, and chorioretinal Stargardt-like atrophy due to PXE was referred to our department for sudden decreased vision in her RE (20/160). Upon a complete ophthalmologic examination, including fluorescein angiography (FA), and optical coherence tomography (OCT), the patient was diagnosed with subfoveal CNV of the RE. Owing to the subfoveal localization of the CNV, the patient was submitted to intravitreal bevacizumab injection. At the 1-month follow-up, visual acuity (VA) improved (20/40), and FA and OCT revealed the CNV closure. Twelve months after the treatment, the patient's VA remained stable with no recurrence of active CNV. On the basis of our findings, a single intravitreal bevacizumab injection seems to induce total regression of CNV complicating PXE, in a patient showing Stargardt-like retinal abnormalities. Further investigations are required to confirm our results.

No MeSH data available.


Related in: MedlinePlus

Fundus autofluorescence shows retinal flecks and areas of welldefined retinal atrophy (A). Late phase fluorescein angiography of the RE shows macular leakage form subfoveal choroidal eovascularization (CNV), associated with cystoid macular edema (CME), within a large area of well-defined atrophy (B). The hypofluorescence of the retinal flecks, is still discernable despite the hyperfluorescent background due to retinal pigment epithelium changes. Optical coherence tomography scan demonstrates moderately reflective mass corresponding to the subfoveal CNV, associated with shallow neurosensory retina elevation and CME in the macular area (C)
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Figure 0001: Fundus autofluorescence shows retinal flecks and areas of welldefined retinal atrophy (A). Late phase fluorescein angiography of the RE shows macular leakage form subfoveal choroidal eovascularization (CNV), associated with cystoid macular edema (CME), within a large area of well-defined atrophy (B). The hypofluorescence of the retinal flecks, is still discernable despite the hyperfluorescent background due to retinal pigment epithelium changes. Optical coherence tomography scan demonstrates moderately reflective mass corresponding to the subfoveal CNV, associated with shallow neurosensory retina elevation and CME in the macular area (C)

Mentions: A 57-year-old woman diagnosed with angioid streaks, retinal flecks and chorio-retinal Stargardt-like atrophy due to PXE (homozygous nucleotide substitution in exon 9 of the ABCC6 gene [C1132T] introducing a stop codon at position 378 [Q378X]) was referred to our department for sudden decreased vision in her right eye (RE). The patient signed a comprehensive consent form according to Good Clinical Practice guidelines, before proceeding with all examinations and treatments. Her best-corrected visual acuity (BCVA) was 20/160 in the RE and 20/400 in the left eye (LE). On fundus biomicroscopy, the macula of the RE showed a subfoveal greenish-gray lesion associated with cystoid macular edema (CME), and the macula of the LE showed a very large fibro-atrophic lesion. Fluorescein angiography (FA) and optical coherence tomography (OCT-3, Humphrey-Zeiss, San Leandro, CA, USA) of the RE revealed subfoveal CNV associated with CME [Figure 1]. After discussing treatment options, including the option of intravitreal bevacizumab, the patient requested intravitreal bevacizumab for treatment. Intravitreal bevacizumab (1.25 mg/0.05 mL) was administered without complication. At the one-month follow-up, BCVA of the RE improved to 20/40, FA revealed closure of CNV and OCT revealed total resolution of the associated CME [Figure 2]. Twelve months after treatment, the patient’s BCVA remained 20/40 without recurrence of either active CNV or CME.Figure 1


Intravitreal avastin for choroidal neovascularization associated with stargardt-like retinal abnormalities in pseudoxanthoma elasticum.

Querques G, Bux AV, Prascina F, Noci ND - Middle East Afr J Ophthalmol (2010)

Fundus autofluorescence shows retinal flecks and areas of welldefined retinal atrophy (A). Late phase fluorescein angiography of the RE shows macular leakage form subfoveal choroidal eovascularization (CNV), associated with cystoid macular edema (CME), within a large area of well-defined atrophy (B). The hypofluorescence of the retinal flecks, is still discernable despite the hyperfluorescent background due to retinal pigment epithelium changes. Optical coherence tomography scan demonstrates moderately reflective mass corresponding to the subfoveal CNV, associated with shallow neurosensory retina elevation and CME in the macular area (C)
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2991464&req=5

Figure 0001: Fundus autofluorescence shows retinal flecks and areas of welldefined retinal atrophy (A). Late phase fluorescein angiography of the RE shows macular leakage form subfoveal choroidal eovascularization (CNV), associated with cystoid macular edema (CME), within a large area of well-defined atrophy (B). The hypofluorescence of the retinal flecks, is still discernable despite the hyperfluorescent background due to retinal pigment epithelium changes. Optical coherence tomography scan demonstrates moderately reflective mass corresponding to the subfoveal CNV, associated with shallow neurosensory retina elevation and CME in the macular area (C)
Mentions: A 57-year-old woman diagnosed with angioid streaks, retinal flecks and chorio-retinal Stargardt-like atrophy due to PXE (homozygous nucleotide substitution in exon 9 of the ABCC6 gene [C1132T] introducing a stop codon at position 378 [Q378X]) was referred to our department for sudden decreased vision in her right eye (RE). The patient signed a comprehensive consent form according to Good Clinical Practice guidelines, before proceeding with all examinations and treatments. Her best-corrected visual acuity (BCVA) was 20/160 in the RE and 20/400 in the left eye (LE). On fundus biomicroscopy, the macula of the RE showed a subfoveal greenish-gray lesion associated with cystoid macular edema (CME), and the macula of the LE showed a very large fibro-atrophic lesion. Fluorescein angiography (FA) and optical coherence tomography (OCT-3, Humphrey-Zeiss, San Leandro, CA, USA) of the RE revealed subfoveal CNV associated with CME [Figure 1]. After discussing treatment options, including the option of intravitreal bevacizumab, the patient requested intravitreal bevacizumab for treatment. Intravitreal bevacizumab (1.25 mg/0.05 mL) was administered without complication. At the one-month follow-up, BCVA of the RE improved to 20/40, FA revealed closure of CNV and OCT revealed total resolution of the associated CME [Figure 2]. Twelve months after treatment, the patient’s BCVA remained 20/40 without recurrence of either active CNV or CME.Figure 1

Bottom Line: A 57-year-old woman with diagnosis of angioid streaks, retinal flecks, and chorioretinal Stargardt-like atrophy due to PXE was referred to our department for sudden decreased vision in her RE (20/160).At the 1-month follow-up, visual acuity (VA) improved (20/40), and FA and OCT revealed the CNV closure.Further investigations are required to confirm our results.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Policlinico Riuniti di Foggia, University of Foggia, Foggia, Italy.

ABSTRACT
The aim of the study was to describe a patient with pseudoxanthoma elasticum (PXE), showing Stargardt-like retinal abnormalities, who underwent treatment with intravitreal bevacizumab for subfoveal choroidal neovascularization (CNV) of the right eye (RE). A 57-year-old woman with diagnosis of angioid streaks, retinal flecks, and chorioretinal Stargardt-like atrophy due to PXE was referred to our department for sudden decreased vision in her RE (20/160). Upon a complete ophthalmologic examination, including fluorescein angiography (FA), and optical coherence tomography (OCT), the patient was diagnosed with subfoveal CNV of the RE. Owing to the subfoveal localization of the CNV, the patient was submitted to intravitreal bevacizumab injection. At the 1-month follow-up, visual acuity (VA) improved (20/40), and FA and OCT revealed the CNV closure. Twelve months after the treatment, the patient's VA remained stable with no recurrence of active CNV. On the basis of our findings, a single intravitreal bevacizumab injection seems to induce total regression of CNV complicating PXE, in a patient showing Stargardt-like retinal abnormalities. Further investigations are required to confirm our results.

No MeSH data available.


Related in: MedlinePlus