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Year : 2022  |  Volume : 2  |  Issue : 2  |  Page : 628

A well in the eye – A case of a peripappilary staphyloma

Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi, India

Date of Web Publication13-Apr-2022

Correspondence Address:
Asmita Mahajan
Dr. Rajendra Prasad Centre for Ophthalmic Sciences, AIIMS, New Delhi - 110 029
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2603_21

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How to cite this article:
Mahajan A. A well in the eye – A case of a peripappilary staphyloma. Indian J Ophthalmol Case Rep 2022;2:628

How to cite this URL:
Mahajan A. A well in the eye – A case of a peripappilary staphyloma. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2023 Jun 2];2:628. Available from: https://www.ijoreports.in/text.asp?2022/2/2/628/342971

A 25-year-old male presented with a history of poor vision in the left eye since childhood and inward deviation of the eye. On examination, visual acuity was FCCF PR accurate. The fundus showed a posterior staphyloma with a dysplastic disc in the staphyloma. The surrounding retina showed atrophic changes.

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 adjacent choroid and retinal pigment epithelium may be atrophic.[1]

It is generally unilateral and is accompanied by poor vision, although a bilateral case with normal visual acuity has been reported. Its exact pathophysiology is unknown. However, the clinical features of peripapillary staphyloma are suggestive of diminished peripapillary structural support, resulting from incomplete differentiation of the posterior sclera from neural crest cells during gestation.[2]

It should be differentiated from its differentials that include morning glory disc anomaly (MGDA) and optic nerve coloboma due to the associations of syndromes of the latter. This avoids unnecessary neuroimaging.

The appearance of MGDA is very characteristic with a central glial tuft, retinal vessels that exit in a radial fashion from the enlarged posterior scleral opening where the optic nerve tissue exits the globe, and a variable degree of peripapillary pigmentation.[3]

Optic nerve coloboma, on the other hand, appears as a white excavation that typically involves the inferior portion of the nerve and can extend inferiorly into the choroid and retina. Often, the superior rim of the optic nerve is spared.[4]

Here, we present an ophthalmic image of an eye with a dysplastic optic disc in a posterior staphyloma, likely peripapillary staphyloma [Figure 1]. It appears like a “well in the eye.”
Figure 1: A dysplastic disc in a posterior staphyloma

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Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Blair MP. A case of peripapillary staphyloma. Arch Ophthalmol 2000;118:1138. doi: 10.1001/archopht. 118.8.1138.  Back to cited text no. 1
Kim SH, Choi MY, Yu YS, Huh JW. Peripapillary staphyloma: Clinical features and visual outcome in 19 cases. Arch Ophthalmol 2005;123:1371-6.  Back to cited text no. 2
Lee BJ, Traboulsi EI. Update on the morning glory disc anomaly. Ophthalmic Genet 2008;2947-52.  Back to cited text no. 3
Brodsky MC. Congenital optic disk anomalies. Surv Ophthalmol 1994;39:89-112.  Back to cited text no. 4


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