Peripapillary Staphyloma

Abstract

To investigate the clinical features and visual outcome of patients with peripapillary staphyloma. Methods: The medical records of patients diagnosed as having peripapillary staphyloma at Seoul National University Children's Hospital, Seoul, Korea, between January 1, 1991, and December 31, 2003, were reviewed. Visual acuity and associated ocular and systemic abnormalities were recorded. Results: Nineteen patients (21 eyes) with peripapillary staphyloma were included in the study. The mean age at initial examination was 21 months, and 42% (8/19) of the patients were girls. Two patients (11%) had bilateral peripapillary staphylomas. Only 2 eyes had visual acuity better than 20/200 at the last examination, and 7 eyes had severe myopia of more than −6 diopters. After occlusion therapy, 1 patient achieved a visual improvement from light perception to 20/30. Congenital cataract and persistent pupillary membrane were present in 2 eyes each at initial examination. Complications observed during the follow-up period included acquired total cataract in 2 eyes and retinal detachment in 3 eyes. Scleral encircling buckling was performed in 1 eye with retinal detachment, and subsequently the retina was reattached. Only 2 patients had accompanying systemic abnormalities. Conclusions: Rarely, patients with peripapillary staphyloma can achieve significant visual improvement by occlusion therapy, although visual outcome is generally poor. Because associated ocular disease and refractive errors are not infrequent, complete ophthalmic examinations and regular follow-up are necessary.

A unique case of unilateral optic pit and coloboma without macular pathology: an asymptomatic 42-year-old female

“…In the differential diagnosis, peripapillary staphyloma typically appears as a deep peripapillary excavation surrounding a relatively normal-appearing optic nerve head, rather than as a focal defect confined within the disc itself [ 16 ]. Morning glory disc anomaly is characterized by an enlarged optic disc located within a funnel-shaped peripapillary excavation and is frequently associated with central glial tissue and radially arranged retinal vessels emerging from the disc margins [ 17 ].…”

A unique case of unilateral optic pit and coloboma without macular pathology: an asymptomatic 42-year-old female

“…In the differential diagnosis, peripapillary staphyloma typically appears as a deep peripapillary excavation surrounding a relatively normal-appearing optic nerve head, rather than as a focal defect confined within the disc itself [ 16 ]. Morning glory disc anomaly is characterized by an enlarged optic disc located within a funnel-shaped peripapillary excavation and is frequently associated with central glial tissue and radially arranged retinal vessels emerging from the disc margins [ 17 ].…”

Coexistence of Retinopathy of Prematurity With Peripapillary Staphyloma and Optic Nerve Hypoplasia in an Extremely Preterm Infant: Diagnostic and Therapeutic Challenges

“…Diagnosis often requires a meticulous clinical examination with ultrasonography and optical coherence tomography to differentiate it from other excavated congenital disk anomalies such as morning glory anomaly 4. Unlike several other congenital retinal anomalies, peripapillary staphyloma is generally not associated with systemic conditions 5,6. Visual prognosis in peripapillary staphyloma is generally poor and seems related to the excavation depth, although data remain scarce 4…”

“…4 Unlike several other congenital retinal anomalies, peripapillary staphyloma is generally not associated with systemic conditions. 5,6 Visual prognosis in peripapillary staphyloma is generally poor and seems related to the excavation depth, although data remain scarce. 4 The association between optic nerve anomalies and primary retinal nonperfusion in ROP is supported by emerging evidence.…”

Evaluation of baseline optic disc pit and optic disc coloboma maculopathy features by spectral domain optical coherence tomography

“…The optic nerve head is composed of retinal ganglion cell axons, blood vessels, glia, and connective tissue. The optic disc is widely regarded as the central location of impairment in congenital cavitary defects such as ODP, ODC, MGDA and megalopapilla and in acquired conditions like glaucoma and peripapillary staphyloma [ 5 , 18 , 19 ]. The observed disparities in our study’s findings can be attributed to different gross and histological anatomical characteristics, variations in lesion margins, and differing mechanisms underlying the onset of maculopathy in these two entities.…”

A unique case of unilateral optic pit and coloboma without macular pathology: an asymptomatic 42-year-old female

“…In the differential diagnosis, peripapillary staphyloma typically appears as a deep peripapillary excavation surrounding a relatively normal-appearing optic nerve head, rather than as a focal defect confined within the disc itself [ 16 ]. Morning glory disc anomaly is characterized by an enlarged optic disc located within a funnel-shaped peripapillary excavation and is frequently associated with central glial tissue and radially arranged retinal vessels emerging from the disc margins [ 17 ].…”

Coexistence of Retinopathy of Prematurity With Peripapillary Staphyloma and Optic Nerve Hypoplasia in an Extremely Preterm Infant: Diagnostic and Therapeutic Challenges

“…Diagnosis often requires a meticulous clinical examination with ultrasonography and optical coherence tomography to differentiate it from other excavated congenital disk anomalies such as morning glory anomaly 4. Unlike several other congenital retinal anomalies, peripapillary staphyloma is generally not associated with systemic conditions 5,6. Visual prognosis in peripapillary staphyloma is generally poor and seems related to the excavation depth, although data remain scarce 4…”

“…4 Unlike several other congenital retinal anomalies, peripapillary staphyloma is generally not associated with systemic conditions. 5,6 Visual prognosis in peripapillary staphyloma is generally poor and seems related to the excavation depth, although data remain scarce. 4 The association between optic nerve anomalies and primary retinal nonperfusion in ROP is supported by emerging evidence.…”

Evaluation of baseline optic disc pit and optic disc coloboma maculopathy features by spectral domain optical coherence tomography

“…The optic nerve head is composed of retinal ganglion cell axons, blood vessels, glia, and connective tissue. The optic disc is widely regarded as the central location of impairment in congenital cavitary defects such as ODP, ODC, MGDA and megalopapilla and in acquired conditions like glaucoma and peripapillary staphyloma [ 5 , 18 , 19 ]. The observed disparities in our study’s findings can be attributed to different gross and histological anatomical characteristics, variations in lesion margins, and differing mechanisms underlying the onset of maculopathy in these two entities.…”

A unique case of unilateral optic pit and coloboma without macular pathology: an asymptomatic 42-year-old female

“…In the differential diagnosis, peripapillary staphyloma typically appears as a deep peripapillary excavation surrounding a relatively normal-appearing optic nerve head, rather than as a focal defect confined within the disc itself [ 16 ]. Morning glory disc anomaly is characterized by an enlarged optic disc located within a funnel-shaped peripapillary excavation and is frequently associated with central glial tissue and radially arranged retinal vessels emerging from the disc margins [ 17 ].…”

Coexistence of Retinopathy of Prematurity With Peripapillary Staphyloma and Optic Nerve Hypoplasia in an Extremely Preterm Infant: Diagnostic and Therapeutic Challenges

“…Diagnosis often requires a meticulous clinical examination with ultrasonography and optical coherence tomography to differentiate it from other excavated congenital disk anomalies such as morning glory anomaly 4. Unlike several other congenital retinal anomalies, peripapillary staphyloma is generally not associated with systemic conditions 5,6. Visual prognosis in peripapillary staphyloma is generally poor and seems related to the excavation depth, although data remain scarce 4…”

“…4 Unlike several other congenital retinal anomalies, peripapillary staphyloma is generally not associated with systemic conditions. 5,6 Visual prognosis in peripapillary staphyloma is generally poor and seems related to the excavation depth, although data remain scarce. 4 The association between optic nerve anomalies and primary retinal nonperfusion in ROP is supported by emerging evidence.…”

Evaluation of baseline optic disc pit and optic disc coloboma maculopathy features by spectral domain optical coherence tomography

“…The optic nerve head is composed of retinal ganglion cell axons, blood vessels, glia, and connective tissue. The optic disc is widely regarded as the central location of impairment in congenital cavitary defects such as ODP, ODC, MGDA and megalopapilla and in acquired conditions like glaucoma and peripapillary staphyloma [ 5 , 18 , 19 ]. The observed disparities in our study’s findings can be attributed to different gross and histological anatomical characteristics, variations in lesion margins, and differing mechanisms underlying the onset of maculopathy in these two entities.…”