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A Case of Peripapillary Staphyloma

A Case of Peripapillary Staphyloma A 35-YEAR-OLD African American woman was referred by her internist for an asymptomatic abnormality of the right optic nerve. She had a history of myopia but no chronic medical problems. On examination, her uncorrected visual acuity was 20/40 − 2 OD and 20/30 −2 OS. Her refraction was −1.00 + 0.75 × 10° OD with 20/20 + 3 visual acuity, and −0.50 + 0.75 × 5° OS with 20/20 + 3 visual acuity. External and slitlamp examination results were within normal limits. Intraocular pressures were 19 mm Hg and 18 mm Hg in the right and left eyes, respectively. Her posterior segment examination results were otherwise unremarkable except for a few vitreous strands in both eyes and a 2-mm staphyloma around the right optic nerve (Figure 1 and Figure 2). The optic nerve appeared relatively normal except for a whitish zone in the inferonasal substance of the nerve head with an overlying whitish membrane. The pigment epithelium around the staphyloma and in its walls was mottled, the fovea was near the edge of the staphyloma, and there was no evidence of choroidal coloboma. Figure 1. View LargeDownload Peripapillary staphyloma in the right eye, best viewed in stereo. Note that although the nerve head is recessed and surrounded by mottled pigment epithelium and choroid, it is relatively normal except for a whitish zone in the inferonasal substance with an overlying membrane. The normal vessel number and relatively normal morphology, lack of central glial tuft, and normal-sized disc rule out the morning glory disc. The left and right photographs are a stereoscopic pair. Figure 2. View LargeDownload Transverse B-scan ultrasound of the 9-o'clock meridian of the right eye. Note the 2-mm depth and 3.2-mm breadth of the staphyloma. The scale on the scan indicates millimeters. Comment Peripapillary staphyloma is an extremely rare nonhereditary condition in which the optic disc is relatively normal and sits at the base of a surrounding staphyloma whose walls, as well as the adjacent choroid and retinal pigment epithelium, contain atrophic changes.1-3 Staphylomas vary in depth, ranging up to 8 mm.1,4 They are usually unilateral, but 2 cases of bilateral staphylomas have been reported.1,5 Patients generally are nearly emmetropic, but may be myopic, especially with deep staphylomas.1,3 However, their visual acuity is usually markedly reduced1 and limited local retinal detachments may be present.4 Peripapillary staphylomas are generally not associated with other congenital defects1 and are thought to be the result of a developmental failure of the posterior sclera from neural crest cells occurring near the fifth month of gestation.1,3,6 Contractions of the staphyloma have been noted and seem to be caused by aberrant muscle.1,6 Peripapillary staphylomas are often confused with other lesions of the optic disc.2,3,6 The morning glory disc is shallower with a centrally located prepapillary glial tuft, an anomalous enlarged optic disc, and characteristic vessels.3,5-7 These vessels seem to be increased and to arise peripherally from the disc substance, radiating outward with an abnormally straight course.1-3,6 In a papillary coloboma the optic nerve contains the excavation.3,6,7 Myopic conus and staphyloma has a progressive, degenerative course and temporal predilection of pigmentary changes.1 This work was supported in part by Core Grant EY 01792 from the National Eye Institute, Bethesda, Md, by an unrestricted grant from Research to Prevent Blindness Inc, New York, NY, and by the Illinois Eye Fund, Chicago. Corresponding author: Norman P. Blair, MD, University of Illinois at Chicago Eye and Ear Infirmary, 1855 W Taylor St, Chicago, IL 60612 (e-mail: npblair@uic.edu). References 1. Brown GTasman W Congenital Anomalies of the Optic Disc. New York, NY Grune & Stratton1983;95- 191 2. Brodsky MC Congenital optic disc anomalies. Surv Ophthalmol. 1994;3989- 112Google ScholarCrossref 3. Brodsky MCBaker RSHemed LM Pediatric Neuro-Ophthalmology. New York, NY Springer-Verlag1996;49- 56 4. Gottlieb JLPrieto DMVander JFBrown GCTasman WS Peripapillary staphyloma. Am J Ophthalmol. 1997;124249- 251Google Scholar 5. Hodgkins PLees MLawson J et al. Optic disc anomalies and frontonasal dysplasia. Br J Ophthalmol. 1998;82290- 293Google ScholarCrossref 6. Pollock S The morning glory disc anomaly: contractile movement, classification, and embryogenesis. Doc Ophthalmol. 1987;65439- 460Google ScholarCrossref 7. Villalonga Gornes PTerraza AGGil-Gibernau JJ Opthalmoscopic evolution of papillary colobomatous malformations. J Pediatr Ophthalmol Strabismus. 1995;3220- 25Google Scholar http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Ophthalmology American Medical Association

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Publisher
American Medical Association
Copyright
Copyright © 2000 American Medical Association. All Rights Reserved.
ISSN
0003-9950
eISSN
1538-3687
DOI
10.1001/archopht.118.8.1138
Publisher site
See Article on Publisher Site

Abstract

A 35-YEAR-OLD African American woman was referred by her internist for an asymptomatic abnormality of the right optic nerve. She had a history of myopia but no chronic medical problems. On examination, her uncorrected visual acuity was 20/40 − 2 OD and 20/30 −2 OS. Her refraction was −1.00 + 0.75 × 10° OD with 20/20 + 3 visual acuity, and −0.50 + 0.75 × 5° OS with 20/20 + 3 visual acuity. External and slitlamp examination results were within normal limits. Intraocular pressures were 19 mm Hg and 18 mm Hg in the right and left eyes, respectively. Her posterior segment examination results were otherwise unremarkable except for a few vitreous strands in both eyes and a 2-mm staphyloma around the right optic nerve (Figure 1 and Figure 2). The optic nerve appeared relatively normal except for a whitish zone in the inferonasal substance of the nerve head with an overlying whitish membrane. The pigment epithelium around the staphyloma and in its walls was mottled, the fovea was near the edge of the staphyloma, and there was no evidence of choroidal coloboma. Figure 1. View LargeDownload Peripapillary staphyloma in the right eye, best viewed in stereo. Note that although the nerve head is recessed and surrounded by mottled pigment epithelium and choroid, it is relatively normal except for a whitish zone in the inferonasal substance with an overlying membrane. The normal vessel number and relatively normal morphology, lack of central glial tuft, and normal-sized disc rule out the morning glory disc. The left and right photographs are a stereoscopic pair. Figure 2. View LargeDownload Transverse B-scan ultrasound of the 9-o'clock meridian of the right eye. Note the 2-mm depth and 3.2-mm breadth of the staphyloma. The scale on the scan indicates millimeters. Comment Peripapillary staphyloma is an extremely rare nonhereditary condition in which the optic disc is relatively normal and sits at the base of a surrounding staphyloma whose walls, as well as the adjacent choroid and retinal pigment epithelium, contain atrophic changes.1-3 Staphylomas vary in depth, ranging up to 8 mm.1,4 They are usually unilateral, but 2 cases of bilateral staphylomas have been reported.1,5 Patients generally are nearly emmetropic, but may be myopic, especially with deep staphylomas.1,3 However, their visual acuity is usually markedly reduced1 and limited local retinal detachments may be present.4 Peripapillary staphylomas are generally not associated with other congenital defects1 and are thought to be the result of a developmental failure of the posterior sclera from neural crest cells occurring near the fifth month of gestation.1,3,6 Contractions of the staphyloma have been noted and seem to be caused by aberrant muscle.1,6 Peripapillary staphylomas are often confused with other lesions of the optic disc.2,3,6 The morning glory disc is shallower with a centrally located prepapillary glial tuft, an anomalous enlarged optic disc, and characteristic vessels.3,5-7 These vessels seem to be increased and to arise peripherally from the disc substance, radiating outward with an abnormally straight course.1-3,6 In a papillary coloboma the optic nerve contains the excavation.3,6,7 Myopic conus and staphyloma has a progressive, degenerative course and temporal predilection of pigmentary changes.1 This work was supported in part by Core Grant EY 01792 from the National Eye Institute, Bethesda, Md, by an unrestricted grant from Research to Prevent Blindness Inc, New York, NY, and by the Illinois Eye Fund, Chicago. Corresponding author: Norman P. Blair, MD, University of Illinois at Chicago Eye and Ear Infirmary, 1855 W Taylor St, Chicago, IL 60612 (e-mail: npblair@uic.edu). References 1. Brown GTasman W Congenital Anomalies of the Optic Disc. New York, NY Grune & Stratton1983;95- 191 2. Brodsky MC Congenital optic disc anomalies. Surv Ophthalmol. 1994;3989- 112Google ScholarCrossref 3. Brodsky MCBaker RSHemed LM Pediatric Neuro-Ophthalmology. New York, NY Springer-Verlag1996;49- 56 4. Gottlieb JLPrieto DMVander JFBrown GCTasman WS Peripapillary staphyloma. Am J Ophthalmol. 1997;124249- 251Google Scholar 5. Hodgkins PLees MLawson J et al. Optic disc anomalies and frontonasal dysplasia. Br J Ophthalmol. 1998;82290- 293Google ScholarCrossref 6. Pollock S The morning glory disc anomaly: contractile movement, classification, and embryogenesis. Doc Ophthalmol. 1987;65439- 460Google ScholarCrossref 7. Villalonga Gornes PTerraza AGGil-Gibernau JJ Opthalmoscopic evolution of papillary colobomatous malformations. J Pediatr Ophthalmol Strabismus. 1995;3220- 25Google Scholar

Journal

Archives of OphthalmologyAmerican Medical Association

Published: Aug 1, 2000

Keywords: staphyloma

References

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