PERIPAPILLARY STAPHYLOMA WITH ASSOCIATED RETINOPATHY OF PREMATURITY Bryan M. Kim, MD, Michael J. Shapiro, MD, Marilyn T. Miller, MD, Michael P. Blair, MD
Purpose: To report a case of asymmetric retinopathy of prematurity associated with a peripapillary staphyloma. Methods: Case report. Results: A 1,545-g male infant was born at 34 weeks’ gestation. He was noted on initial examination to have a peripapillary staphyloma in the left eye and immature retinal vasculature in zone 2 of both eyes. Follow-up examination at 16 weeks of age showed a normal right eye with full vascularization and zone 2, stage 2 retinopathy of prematurity in the left eye. Conclusion: To our knowledge, this is the first reported case of peripapillary staphyloma in which only the affected eye developed retinopathy of prematurity. RETINAL CASES & BRIEF REPORTS 5:146–148, 2011
a left exotropia of 20 prism diopters. Dilated fundus examination demonstrated a normal right eye with full vascularization; the left eye had zone 2, stage 2 ROP without plus (Figure 3). B-scan ultrasonography was performed and confirmed the presence of a peripapillary staphyloma in the left eye (Figure 4). The patient was observed with repeat examinations and noted to have subsequent regression of the ridge in the left eye.
From the University of Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, Chicago, Illinois.
P
eripapillary staphyloma is a rare congenital anomaly that typically does not have ocular or systemic associations,1–5 although coexistent systemic anomalies have rarely been reported.4 To our knowledge, there are no reports of peripapillary staphyloma occurring in an eye with retinopathy of prematurity (ROP). We report on a patient who presented with unilateral peripapillary staphyloma and developed ROP only in the affected eye.
Discussion Congenital excavated optic nerve anomalies include peripapillary staphyloma, morning glory disk anomaly, and optic nerve coloboma. Peripapillary staphyloma is a unilateral, nonhereditary anomaly in which a well-defined optic disk lies at the base of an ectasia lined by retinal tissue.1–5 The excavation in peripapillary staphyloma is cup-shaped and deep, ranging from 3 mm to .9 mm in depth.5 The disk appears normal or displays temporal pallor.1,2 The vascular pattern is unremarkable other than reflecting the contour of the lesion.1,2 The normal appearance of the optic disk and vessels suggests that their development is fundamentally complete before staphyloma formation.1 Peripapillary staphyloma is thought to arise from faulty differentiation of posterior sclera from neural crest cells during the fifth month of gestation with subsequent herniation of unsupported ocular tissues through the defect once normal intraocular pressure is established.1
Case Report A 1,545-g male infant was born at 34 weeks from a monoamniotic twin gestation. He received supplemental oxygen for 18 days for respiratory distress syndrome. An initial ophthalmic examination at 4 weeks of age showed immature retinal vasculature in zone 2 of both eyes without ROP. The right eye was otherwise unremarkable (Figure 1); however, the left eye had a poorly developed fovea and peripapillary staphyloma (Figure 2). At age 16 weeks, vision was central, steady, and maintained with +0.75 sphere in the right eye; and uncentral, unsteady, and unmaintained with 25.00 + 1.50 3 095 in the left eye. There was All authors report no financial or proprietary interests. Reprint requests: Bryan M. Kim, MD, University of Illinois Eye and Ear Infirmary, 1855 West Taylor Street, Chicago, IL 60612; e-mail: [email protected]
146
PERIPAPILLARY STAPHYLOMA WITH ROP
147
Fig. 1. Photograph of the right eye showing a normal-appearing optic nerve, well-developed fovea, and normal retinal vasculature.
Fig. 3. Photograph of temporal peripheral retina in the left eye with a demarcation ridge (arrowhead) (stage 2 ROP).
Peripapillary staphyloma can be distinguished from morning glory disk anomaly and optic nerve coloboma ophthalmoscopically.1–5 Morning glory anomaly is characterized by an enlarged, orange-pink disk that lies within a shallow, funnel-shaped excavation.1,2 A tuft of glial tissue overlies the central portion of the disk, and an abnormally increased number of blood vessels arises from its periphery.1,2 In optic disk coloboma, a glistening, bowl-shaped excavation lies within the optic disk itself.1,2 The excavation is asymmetric and decentered inferiorly.1,2 Similar to peripapillary staphyloma, there are no glial or vascular anomalies and only mild pigmentary changes.1 Several aspects of our case are unique. The patient did not fit the usual characteristics of those at risk for ROP, due to his gestational age of 34 weeks and birth
weight of 1,545 g. Although the initial examination showed an immature retina in zone 2 of both eyes, only the eye with the peripapillary staphyloma later developed ROP. The patient’s identical twin had a similar birth weight and was also on supplemental oxygen for respiratory distress. He had an immature retina in zone 2 of both eyes without optic nerve anomalies on initial examination and did not develop ROP. To our knowledge, this is the first reported case of peripapillary staphyloma in which only the affected
Fig. 2. Photograph of the left eye showing a peripapillary staphyloma. The optic nerve seems relatively normal and lies at the base of the excavation. There are mild pigmentary changes around the nerve.
Fig. 4. B-scan ultrasound image of the left eye showing the peripapillary staphyloma (arrows). The depth of the excavation measured 4.5 mm.
148
RETINAL CASES & BRIEF REPORTS´ 2011 VOLUME 5 NUMBER 2
eye developed ROP. Of note, the affected child was otherwise at low risk for ROP, and his identical twin with normal optic nerves did not develop ROP. Although there are no previous reports of peripapillary staphyloma with ROP, two cases of markedly asymmetric ROP have previously been reported in association with optic nerve hypoplasia.6 Retinal vascular development involves multiple complex steps, including migration of astrocytes and vascular precursors through the optic nerve,7 and could therefore be affected by optic nerve anomalies. We believe possible factors involved in our case may include alterations in retinal vascular development caused by the nerve anomaly and the increased distance from the optic nerve head to the temporal retina as a result of the staphyloma. Key words: morning glory disk anomaly, optic nerve, optic nerve anomaly, optic nerve coloboma,
optic nerve hypoplasia, peripapillary staphyloma, retinopathy of prematurity. References 1. Pollock S. The morning glory disc anomaly: contractile movement, classification, and embryogenesis. Doc Ophthalmol 1987;65:439–460. 2. Brodsky MC. Congenital optic disk anomalies. Surv Ophthal mol 1994;39:89–112. 3. Blair MP, Blair NP, Rheinstrom SD, Jednock NJ. A case of peripapillary staphyloma. Arch Ophthalmol 2000;118: 1138–1139. 4. Dutton GN. Congenital disorders of the optic nerve: excavations and hypoplasia. Eye (Lond) 2004;18:1038–1048. 5. Gottlieb JL, Prieto DM, Vander JF, Brown GC, Tasman WS. Peripapillary staphyloma. Am J Ophthalmol 1997;124:249–251. 6. Arnold RW. Optic nerve hypoplasia potentiates retinopathy of prematurity. J Pediatr Ophthalmol Strabismus 2008;45: 247–249. 7. Gariano RF. Cellular mechanisms in retinal vascular development. Prog Retin Eye Res 2003;22:295–306.
Purpose: To report a case of asymmetric retinopathy of prematurity associated with a peripapillary staphyloma. Methods: Case report. Results: A 1,545-g male infant was born at 34 weeks’ gestation. He was noted on initial examination to have a peripapillary staphyloma in the left eye and immature retinal vasculature in zone 2 of both eyes. Follow-up examination at 16 weeks of age showed a normal right eye with full vascularization and zone 2, stage 2 retinopathy of prematurity in the left eye. Conclusion: To our knowledge, this is the first reported case of peripapillary staphyloma in which only the affected eye developed retinopathy of prematurity. RETINAL CASES & BRIEF REPORTS 5:146–148, 2011
a left exotropia of 20 prism diopters. Dilated fundus examination demonstrated a normal right eye with full vascularization; the left eye had zone 2, stage 2 ROP without plus (Figure 3). B-scan ultrasonography was performed and confirmed the presence of a peripapillary staphyloma in the left eye (Figure 4). The patient was observed with repeat examinations and noted to have subsequent regression of the ridge in the left eye.
From the University of Illinois Eye and Ear Infirmary, Department of Ophthalmology and Visual Sciences, Chicago, Illinois.
P
eripapillary staphyloma is a rare congenital anomaly that typically does not have ocular or systemic associations,1–5 although coexistent systemic anomalies have rarely been reported.4 To our knowledge, there are no reports of peripapillary staphyloma occurring in an eye with retinopathy of prematurity (ROP). We report on a patient who presented with unilateral peripapillary staphyloma and developed ROP only in the affected eye.
Discussion Congenital excavated optic nerve anomalies include peripapillary staphyloma, morning glory disk anomaly, and optic nerve coloboma. Peripapillary staphyloma is a unilateral, nonhereditary anomaly in which a well-defined optic disk lies at the base of an ectasia lined by retinal tissue.1–5 The excavation in peripapillary staphyloma is cup-shaped and deep, ranging from 3 mm to .9 mm in depth.5 The disk appears normal or displays temporal pallor.1,2 The vascular pattern is unremarkable other than reflecting the contour of the lesion.1,2 The normal appearance of the optic disk and vessels suggests that their development is fundamentally complete before staphyloma formation.1 Peripapillary staphyloma is thought to arise from faulty differentiation of posterior sclera from neural crest cells during the fifth month of gestation with subsequent herniation of unsupported ocular tissues through the defect once normal intraocular pressure is established.1
Case Report A 1,545-g male infant was born at 34 weeks from a monoamniotic twin gestation. He received supplemental oxygen for 18 days for respiratory distress syndrome. An initial ophthalmic examination at 4 weeks of age showed immature retinal vasculature in zone 2 of both eyes without ROP. The right eye was otherwise unremarkable (Figure 1); however, the left eye had a poorly developed fovea and peripapillary staphyloma (Figure 2). At age 16 weeks, vision was central, steady, and maintained with +0.75 sphere in the right eye; and uncentral, unsteady, and unmaintained with 25.00 + 1.50 3 095 in the left eye. There was All authors report no financial or proprietary interests. Reprint requests: Bryan M. Kim, MD, University of Illinois Eye and Ear Infirmary, 1855 West Taylor Street, Chicago, IL 60612; e-mail: [email protected]
146
PERIPAPILLARY STAPHYLOMA WITH ROP
147
Fig. 1. Photograph of the right eye showing a normal-appearing optic nerve, well-developed fovea, and normal retinal vasculature.
Fig. 3. Photograph of temporal peripheral retina in the left eye with a demarcation ridge (arrowhead) (stage 2 ROP).
Peripapillary staphyloma can be distinguished from morning glory disk anomaly and optic nerve coloboma ophthalmoscopically.1–5 Morning glory anomaly is characterized by an enlarged, orange-pink disk that lies within a shallow, funnel-shaped excavation.1,2 A tuft of glial tissue overlies the central portion of the disk, and an abnormally increased number of blood vessels arises from its periphery.1,2 In optic disk coloboma, a glistening, bowl-shaped excavation lies within the optic disk itself.1,2 The excavation is asymmetric and decentered inferiorly.1,2 Similar to peripapillary staphyloma, there are no glial or vascular anomalies and only mild pigmentary changes.1 Several aspects of our case are unique. The patient did not fit the usual characteristics of those at risk for ROP, due to his gestational age of 34 weeks and birth
weight of 1,545 g. Although the initial examination showed an immature retina in zone 2 of both eyes, only the eye with the peripapillary staphyloma later developed ROP. The patient’s identical twin had a similar birth weight and was also on supplemental oxygen for respiratory distress. He had an immature retina in zone 2 of both eyes without optic nerve anomalies on initial examination and did not develop ROP. To our knowledge, this is the first reported case of peripapillary staphyloma in which only the affected
Fig. 2. Photograph of the left eye showing a peripapillary staphyloma. The optic nerve seems relatively normal and lies at the base of the excavation. There are mild pigmentary changes around the nerve.
Fig. 4. B-scan ultrasound image of the left eye showing the peripapillary staphyloma (arrows). The depth of the excavation measured 4.5 mm.
148
RETINAL CASES & BRIEF REPORTS´ 2011 VOLUME 5 NUMBER 2
eye developed ROP. Of note, the affected child was otherwise at low risk for ROP, and his identical twin with normal optic nerves did not develop ROP. Although there are no previous reports of peripapillary staphyloma with ROP, two cases of markedly asymmetric ROP have previously been reported in association with optic nerve hypoplasia.6 Retinal vascular development involves multiple complex steps, including migration of astrocytes and vascular precursors through the optic nerve,7 and could therefore be affected by optic nerve anomalies. We believe possible factors involved in our case may include alterations in retinal vascular development caused by the nerve anomaly and the increased distance from the optic nerve head to the temporal retina as a result of the staphyloma. Key words: morning glory disk anomaly, optic nerve, optic nerve anomaly, optic nerve coloboma,
optic nerve hypoplasia, peripapillary staphyloma, retinopathy of prematurity. References 1. Pollock S. The morning glory disc anomaly: contractile movement, classification, and embryogenesis. Doc Ophthalmol 1987;65:439–460. 2. Brodsky MC. Congenital optic disk anomalies. Surv Ophthal mol 1994;39:89–112. 3. Blair MP, Blair NP, Rheinstrom SD, Jednock NJ. A case of peripapillary staphyloma. Arch Ophthalmol 2000;118: 1138–1139. 4. Dutton GN. Congenital disorders of the optic nerve: excavations and hypoplasia. Eye (Lond) 2004;18:1038–1048. 5. Gottlieb JL, Prieto DM, Vander JF, Brown GC, Tasman WS. Peripapillary staphyloma. Am J Ophthalmol 1997;124:249–251. 6. Arnold RW. Optic nerve hypoplasia potentiates retinopathy of prematurity. J Pediatr Ophthalmol Strabismus 2008;45: 247–249. 7. Gariano RF. Cellular mechanisms in retinal vascular development. Prog Retin Eye Res 2003;22:295–306.
