- Email: [email protected]
Coats'-like retinitis pigmentosa variant and nanophthalmos
Correspondence centration of the drug within the site of infection, as with other, older, antifungals; these situations, indeed, predispose to a failure in the eradication of infection. Moreover, the excellent bioavailability of voriconazole allows an early switch from intravenous to oral therapy and makes it a reliable prophylactic option in situations that could be considered high risk, e.g., selected surgical interventions or the patient’s underlying status. For these reasons, it is our opinion that voriconazole should be considered, at present, the drug of choice in fungal endophthalmitis. REFERENCES 1. Pappas PG, Rex JH, Sobel JD, et al. Guidelines for treatment of candidiasis. Clin Infect Dis 2004;38:161–89. 2. Weinstein O, Levy J, Lifshitz T. Recurrent Candida albicans endophthalmitis in an immunocompromised host. Can J Ophthalmol 2007;42:154–5. 3. Hariprasad SM, Mieler WF, Holz ER, et al. Determination of vitreous, aqueous, and plasma concentration of orally administered voriconazole in humans. Arch Ophthalmol 2004;122:42–7. 4. Breit SM, Hariprasad SM, Mieler WF, Shah GK, Mills MD, Grand MG. Management of endogenous fungal endophthalmitis with voriconazole and caspofungin. Am J Ophthalmol 2005;139:135–40. 5. Durand ML, Kim IK, D’Amico DJ, et al. Successful treatment of Fusarium endophthalmitis with voriconazole and Aspergillus endophthalmitis with voriconazole plus caspofungin. Am J Ophthalmol 2005;140:552–4.
Leonardo Pagani, Greta Spoladore, Peter Mian, Claudio Vedovelli
eye (Fig. 1). There were also preretinal hemorrhages in the retinal vascular arcades together with some vitreous hemorrhage. Optical coherence tomography revealed cystoid macular edema with a central foveal thickness of 420 μm in the right eye and 450 μm in the left eye. Fluorescein angiography of the right eye confirmed the cystoid macular edema and presence of marked microvascular changes in the peripheral retina involving capillary nonperfusion, telangiectatic vessels, and leakage of fluorescein dye into the temporal fundus of the left eye (Fig. 2). Electroretinography was suboptimal bilaterally. The patient’s condition was diagnosed as nanophthalmos associated with Coats’-like retinitis pigmentosa (RP) variant; however, the patient refused any treatment and was lost to follow-up. In this case, exudative retinal detachment was most probably secondary to Coats’-like changes (telangiectatic vessels, subretinal lipid deposition, and exudative vasculopathy), which was probably associated with pigmentary retinopathy, as it is in Coats’-like RP. Coats’-like disease with retinal pigmentary changes was first reported by Zamorani1 in 1956. Today, it is estimated that 3.6% of RP cases have an associated Coats’ disease.2 The association of nanophthalmos and retinal pigmentary changes has also been described in previous literature.3–5 Nanophthalmos may result from mutations in retinal pigment epithelium-specific genes. Yardley et al.6 proposed that mutations in the vitelliform macular dystrophy gene might cause nanophthalmos associated with a generalized retinal dystrophy.
Unit for Hospital Antimicrobial Chemotherapy, Division of Infectious Diseases, Bolzano Central Hospital, Bolzano, Italy Correspondence to: Leonardo Pagani, MD; [email protected] Can J Ophthalmol 2007;42:876–7 doi:10.3129/i07-174
Coats’-like retinitis pigmentosa variant and nanophthalmos
A
34-year-old woman attended our clinic with a history of blurred vision in the left eye for over a week and complaint of progressive nyctalopia. Her family history was negative for any visual disturbances. She was markedly hyperopic (+16.00 diopters [D] in the right eye and +14.50 D in the left eye), and best corrected visual acuity was 20/100 OD and 20/400 OS. Slit lamp examination revealed bilaterally shallow anterior chambers. Intraocular pressure was 17 mm Hg OD and 15 mm Hg OS. Axial length was 16 mm OD and 16.5 mm OS. Examination of the posterior segment showed arteriolar attenuation and mid-peripheral “bone-spicule” pigmentary changes bilaterally. There was exudative retinal detachment with subretinal lipid exudation in the temporal periphery of the left
Fig. 1—Fundus photography, left eye, demonstrates preretinal and vitreous hemorrhages and exudative retinal detachment with subretinal lipid exudation in the temporal periphery.
Fig. 2—Fluorescein angiogram, left eye, shows leakage of fluorescein dye into the temporal periphery with capillary nonperfusion areas and telangiectatic vessels. CAN J OPHTHALMOL—VOL. 42, NO. 6, 2007
877
Correspondence To our knowledge, this is the first report of an association between nanophthalmos complicated by serous retinal detachment and retinal pigmentary changes. Coats’-like changes may accompany the pigmentary retinopathy, just as in RP.
men revealed intracytoplasmic electron-dense fibrillogranular “peculiar substance”1 (Fig. 1D), which confirmed the diagnosis of Meesmann’s corneal dystrophy.2
REFERENCES 1. Zamorani G. Una rara associazone di retinite di Coats con retinite pigmentosa. G Ital Oftalmol 1956;9:429–43. 2. Pruett RC. Retinitis pigmentosa: clinical observations and correlations. Trans Am Ophthalmol Soc 1983;81:693–735. 3. Ghose S, Sachdev MS, Kumar H. Bilateral nanophthalmos, pigmentary retinal dystrophy, and angle closure glaucoma—a new syndrome? Br J Ophthalmol 1985;69:624–8. 4. MacKay CJ, Shek MS, Carr RE, Yanuzzi LA, Gouras P. Retinal degeneration with nanophthalmos, cystic macular degeneration, and angle closure glaucoma. A new recessive syndrome. Arch Ophthalmol 1987;105:366–71. 5. Khairallah M, Messaoud R, Zaouali S, Ben Yahia S, Ladjimi A, Jenzri S. Posterior segment changes associated with posterior microphthalmos. Ophthalmology 2002;109:569–74. 6. Yardley J, Leroy BP, Hart-Holden N, et al. Mutations of VMD2 splicing regulators cause nanophthalmos and autosomal dominant vitreoretinochoroidopathy (ADVIRC). Invest Ophthalmol Vis Sci 2004;45:3683–9.
Berrak Urgancioglu, Sengul Ozdek, Berati Hasanreisoglu Department of Ophthalmology, Faculty of Medicine Gazi University, Ankara, Turkey Correspondence to: Berrak Urgancioglu, MD; [email protected] Can J Ophthalmol 2007;42:877–8 doi:10.3129/i07-170
Meesmann’s corneal dystrophy managed with an epithelial delaminator
A
50-year-old woman with a 3-year history of decreased visual acuity and irritative symptoms demonstrated numerous prominent corneal microcysts diffusely throughout the cornea, as well as basement membrane dystrophy in both eyes on slit lamp examination (Fig. 1A). Confocal microscopy revealed intraepithelial cysts containing hyper-reflective material (Fig. 1B). The patient underwent phototherapeutic keratectomy. An epithelial delaminator obtained an epithelial flap for histological and ultrastructural analysis. Histopathologic examination disclosed numerous intraepithelial cysts, many of which contained cellular debris, as well as a prominent, thick basement membrane (Fig. 1C). The material within many of the cysts stained positive with periodic acid–Schiff stain and demonstrated the presence of glycosaminoglycans with the colloidal iron and Alcian Blue stains. Electron microscopy of the speci-
878
CAN J OPHTHALMOL—VOL. 42, NO. 6, 2007
Fig.1—(A) The right cornea preoperatively demonstrates numerous epithelial microcysts. Grey, serpiginous lines (arrows) are representative of basement membrane dystrophy. Inset shows retroillumination of magnified epithelial microcysts. (B) Confocal microscopy reveals hyperreflective material inside intraepithelial cysts (arrows). (C) Corneal epithelium shows cysts containing cellular debris, most of which has extruded from the cyst on the right. Note the thick, periodic acid–Schiff positive basement membrane (arrows). (D) Electron micrographs disclose intracytoplasmic electron-dense fibrillogranular “peculiar substance” (arrows and inset).
Leonardo Pagani, Greta Spoladore, Peter Mian, Claudio Vedovelli
eye (Fig. 1). There were also preretinal hemorrhages in the retinal vascular arcades together with some vitreous hemorrhage. Optical coherence tomography revealed cystoid macular edema with a central foveal thickness of 420 μm in the right eye and 450 μm in the left eye. Fluorescein angiography of the right eye confirmed the cystoid macular edema and presence of marked microvascular changes in the peripheral retina involving capillary nonperfusion, telangiectatic vessels, and leakage of fluorescein dye into the temporal fundus of the left eye (Fig. 2). Electroretinography was suboptimal bilaterally. The patient’s condition was diagnosed as nanophthalmos associated with Coats’-like retinitis pigmentosa (RP) variant; however, the patient refused any treatment and was lost to follow-up. In this case, exudative retinal detachment was most probably secondary to Coats’-like changes (telangiectatic vessels, subretinal lipid deposition, and exudative vasculopathy), which was probably associated with pigmentary retinopathy, as it is in Coats’-like RP. Coats’-like disease with retinal pigmentary changes was first reported by Zamorani1 in 1956. Today, it is estimated that 3.6% of RP cases have an associated Coats’ disease.2 The association of nanophthalmos and retinal pigmentary changes has also been described in previous literature.3–5 Nanophthalmos may result from mutations in retinal pigment epithelium-specific genes. Yardley et al.6 proposed that mutations in the vitelliform macular dystrophy gene might cause nanophthalmos associated with a generalized retinal dystrophy.
Unit for Hospital Antimicrobial Chemotherapy, Division of Infectious Diseases, Bolzano Central Hospital, Bolzano, Italy Correspondence to: Leonardo Pagani, MD; [email protected] Can J Ophthalmol 2007;42:876–7 doi:10.3129/i07-174
Coats’-like retinitis pigmentosa variant and nanophthalmos
A
34-year-old woman attended our clinic with a history of blurred vision in the left eye for over a week and complaint of progressive nyctalopia. Her family history was negative for any visual disturbances. She was markedly hyperopic (+16.00 diopters [D] in the right eye and +14.50 D in the left eye), and best corrected visual acuity was 20/100 OD and 20/400 OS. Slit lamp examination revealed bilaterally shallow anterior chambers. Intraocular pressure was 17 mm Hg OD and 15 mm Hg OS. Axial length was 16 mm OD and 16.5 mm OS. Examination of the posterior segment showed arteriolar attenuation and mid-peripheral “bone-spicule” pigmentary changes bilaterally. There was exudative retinal detachment with subretinal lipid exudation in the temporal periphery of the left
Fig. 1—Fundus photography, left eye, demonstrates preretinal and vitreous hemorrhages and exudative retinal detachment with subretinal lipid exudation in the temporal periphery.
Fig. 2—Fluorescein angiogram, left eye, shows leakage of fluorescein dye into the temporal periphery with capillary nonperfusion areas and telangiectatic vessels. CAN J OPHTHALMOL—VOL. 42, NO. 6, 2007
877
Correspondence To our knowledge, this is the first report of an association between nanophthalmos complicated by serous retinal detachment and retinal pigmentary changes. Coats’-like changes may accompany the pigmentary retinopathy, just as in RP.
men revealed intracytoplasmic electron-dense fibrillogranular “peculiar substance”1 (Fig. 1D), which confirmed the diagnosis of Meesmann’s corneal dystrophy.2
REFERENCES 1. Zamorani G. Una rara associazone di retinite di Coats con retinite pigmentosa. G Ital Oftalmol 1956;9:429–43. 2. Pruett RC. Retinitis pigmentosa: clinical observations and correlations. Trans Am Ophthalmol Soc 1983;81:693–735. 3. Ghose S, Sachdev MS, Kumar H. Bilateral nanophthalmos, pigmentary retinal dystrophy, and angle closure glaucoma—a new syndrome? Br J Ophthalmol 1985;69:624–8. 4. MacKay CJ, Shek MS, Carr RE, Yanuzzi LA, Gouras P. Retinal degeneration with nanophthalmos, cystic macular degeneration, and angle closure glaucoma. A new recessive syndrome. Arch Ophthalmol 1987;105:366–71. 5. Khairallah M, Messaoud R, Zaouali S, Ben Yahia S, Ladjimi A, Jenzri S. Posterior segment changes associated with posterior microphthalmos. Ophthalmology 2002;109:569–74. 6. Yardley J, Leroy BP, Hart-Holden N, et al. Mutations of VMD2 splicing regulators cause nanophthalmos and autosomal dominant vitreoretinochoroidopathy (ADVIRC). Invest Ophthalmol Vis Sci 2004;45:3683–9.
Berrak Urgancioglu, Sengul Ozdek, Berati Hasanreisoglu Department of Ophthalmology, Faculty of Medicine Gazi University, Ankara, Turkey Correspondence to: Berrak Urgancioglu, MD; [email protected] Can J Ophthalmol 2007;42:877–8 doi:10.3129/i07-170
Meesmann’s corneal dystrophy managed with an epithelial delaminator
A
50-year-old woman with a 3-year history of decreased visual acuity and irritative symptoms demonstrated numerous prominent corneal microcysts diffusely throughout the cornea, as well as basement membrane dystrophy in both eyes on slit lamp examination (Fig. 1A). Confocal microscopy revealed intraepithelial cysts containing hyper-reflective material (Fig. 1B). The patient underwent phototherapeutic keratectomy. An epithelial delaminator obtained an epithelial flap for histological and ultrastructural analysis. Histopathologic examination disclosed numerous intraepithelial cysts, many of which contained cellular debris, as well as a prominent, thick basement membrane (Fig. 1C). The material within many of the cysts stained positive with periodic acid–Schiff stain and demonstrated the presence of glycosaminoglycans with the colloidal iron and Alcian Blue stains. Electron microscopy of the speci-
878
CAN J OPHTHALMOL—VOL. 42, NO. 6, 2007
Fig.1—(A) The right cornea preoperatively demonstrates numerous epithelial microcysts. Grey, serpiginous lines (arrows) are representative of basement membrane dystrophy. Inset shows retroillumination of magnified epithelial microcysts. (B) Confocal microscopy reveals hyperreflective material inside intraepithelial cysts (arrows). (C) Corneal epithelium shows cysts containing cellular debris, most of which has extruded from the cyst on the right. Note the thick, periodic acid–Schiff positive basement membrane (arrows). (D) Electron micrographs disclose intracytoplasmic electron-dense fibrillogranular “peculiar substance” (arrows and inset).