Journal of Pediatric Ophthalmology and Strabismus

The articles prior to January 2013 are part of the back file collection and are not available with a current paid subscription. To access the article, you may purchase it or purchase the complete back file collection here

Short Subjects 

Congenital Anterior Scleral Staphyloma in an Otherwise Normal Eye

Jerry A Shields, MD; Carol L Shields, MD; Judith Lavrich, MD

Abstract

INTRODUCTION

Staphyloma is a defect in the sclera or cornea through which there is a bulging of uveal tissue and other intraocular structures. It can be congenital or acquired. Congenital staphlyoma can occur anteriorly in the corneal region1·2 or posteriorly in the optic nerve region.2 Acquired staphyloma also can occur anteriorly from conditions such as rheumatoid scleritis3 or posteriorly in conditions such as high myopia.4

This article reports a congenital scleral staphyloma in an otherwise normal eye.

CASE REPORT

In November 2000, a healthy 7-year-old girl was first noticed to have a lesion on her left eye. She was the product of a normal pregnancy, labor, and delivery, and had developed normally. There was no history of prenatal amniocentesis or ocular trauma, and her physical examination was normal.

Her uncorrected visual acuity was 20/20 in each eye, and intraocular pressures were normal. The only ocular abnormality was a distinct blueblack lesion located 6 mm inferior to the corneoscleral limbus in the left eye (Figure). The lesion was 3.5X3.0 mm in diameter, elevated 1 mm from the scleral surface, covered by thin conjunctiva, and was fluctuant to manipulation with a cotton-tipped applicator. Transillumination demonstrated moderate transmission of light through the lesion. Ophthalmoscopy showed a normal fundus with no intraocular abnormalities in the area of the lesion. Specifically, there was no evidence of coloboma, tumor, or foreign body. It was elected not to do ultrasound biomicroscopy because of patient reluctance and because it would not have added to the diagnosis or management.

The clinical diagnosis was localized anterior scleral staphyloma. Repeat examination 1 year later revealed no change in the lesion.

DISCUSSION

Most anterior and posterior staphylomas are associated with other ocular abnormalities.15 The staphyloma reported here is unusual in that it occurred in a child with no concurrent ocular abnormality. We assume that it was a congenital lesion and was not detected earlier because of its location under the lower eyelid. Although a literature search revealed 178 citations to staphyloma, we were unable to identify a similar case.

The pathogenesis is unclear, but it may represent a developmental abnormality with a localized scleral defect, allowing uveal tissue to protrude through the opening. Congenital anterior scleral staphyloma must be differentiated from pigmented conjunctival tumor (nevus or melanoma), foreign body, and extraocular extension of ciliary body melanoma. It differs from such lesions by allowing transmission of light with transillumination techniques.

1. Laibson PR, Rapuano CJ. Diseases of die cornea. Corneal staphyloma and keloid. In: Nelson LB, ed. Harley's Pediatric Ophthalmology. 4th ed. Philadelphia, Pa: WB Saunders Co; 1998:230-231.

2. Lcff SR, Shields JA, Augsburger JJ, Sakowski AD, Blair CJ. Congenital corneal staphyloma. Clinical, radiological, and pathologic correlation. Br J OphthalmoL 1986;70:427-430.

3. Pfistcr DR, Krach mer JH. Adult rheumatoid arthritis. In: Gold DH, Weingeist TA, eds. Color Atlas of the Eye in Systemic Disease. Philadelphia, Pa: Lippincott, Williams and Wilkins; 2001:42-44.

4. Soubrane G, Coscas G, Kuhn D. Myopia. Posterior staphyloma. In: Guyer DR, Yannuzzi L, Chang S, Shields JA, Green WR, eds. Retina-Vttreous-Macuk. A Color Text Philadelphia, Pa: WB Saunders Co; 1998:189-191.

5. Brown GC, Tasman WS. Peripapillary staphyloma. In: Brown GC, Tasman WS, eds. Congenital Anomalies of the Optic Disc. New York, NY: Grune and Stratton; 1983:178-181.…

INTRODUCTION

Staphyloma is a defect in the sclera or cornea through which there is a bulging of uveal tissue and other intraocular structures. It can be congenital or acquired. Congenital staphlyoma can occur anteriorly in the corneal region1·2 or posteriorly in the optic nerve region.2 Acquired staphyloma also can occur anteriorly from conditions such as rheumatoid scleritis3 or posteriorly in conditions such as high myopia.4

This article reports a congenital scleral staphyloma in an otherwise normal eye.

CASE REPORT

In November 2000, a healthy 7-year-old girl was first noticed to have a lesion on her left eye. She was the product of a normal pregnancy, labor, and delivery, and had developed normally. There was no history of prenatal amniocentesis or ocular trauma, and her physical examination was normal.

Figure: Clinical appearance of inferior scleral staphyloma.

Figure: Clinical appearance of inferior scleral staphyloma.

Her uncorrected visual acuity was 20/20 in each eye, and intraocular pressures were normal. The only ocular abnormality was a distinct blueblack lesion located 6 mm inferior to the corneoscleral limbus in the left eye (Figure). The lesion was 3.5X3.0 mm in diameter, elevated 1 mm from the scleral surface, covered by thin conjunctiva, and was fluctuant to manipulation with a cotton-tipped applicator. Transillumination demonstrated moderate transmission of light through the lesion. Ophthalmoscopy showed a normal fundus with no intraocular abnormalities in the area of the lesion. Specifically, there was no evidence of coloboma, tumor, or foreign body. It was elected not to do ultrasound biomicroscopy because of patient reluctance and because it would not have added to the diagnosis or management.

The clinical diagnosis was localized anterior scleral staphyloma. Repeat examination 1 year later revealed no change in the lesion.

DISCUSSION

Most anterior and posterior staphylomas are associated with other ocular abnormalities.15 The staphyloma reported here is unusual in that it occurred in a child with no concurrent ocular abnormality. We assume that it was a congenital lesion and was not detected earlier because of its location under the lower eyelid. Although a literature search revealed 178 citations to staphyloma, we were unable to identify a similar case.

The pathogenesis is unclear, but it may represent a developmental abnormality with a localized scleral defect, allowing uveal tissue to protrude through the opening. Congenital anterior scleral staphyloma must be differentiated from pigmented conjunctival tumor (nevus or melanoma), foreign body, and extraocular extension of ciliary body melanoma. It differs from such lesions by allowing transmission of light with transillumination techniques.

REFERENCES

1. Laibson PR, Rapuano CJ. Diseases of die cornea. Corneal staphyloma and keloid. In: Nelson LB, ed. Harley's Pediatric Ophthalmology. 4th ed. Philadelphia, Pa: WB Saunders Co; 1998:230-231.

2. Lcff SR, Shields JA, Augsburger JJ, Sakowski AD, Blair CJ. Congenital corneal staphyloma. Clinical, radiological, and pathologic correlation. Br J OphthalmoL 1986;70:427-430.

3. Pfistcr DR, Krach mer JH. Adult rheumatoid arthritis. In: Gold DH, Weingeist TA, eds. Color Atlas of the Eye in Systemic Disease. Philadelphia, Pa: Lippincott, Williams and Wilkins; 2001:42-44.

4. Soubrane G, Coscas G, Kuhn D. Myopia. Posterior staphyloma. In: Guyer DR, Yannuzzi L, Chang S, Shields JA, Green WR, eds. Retina-Vttreous-Macuk. A Color Text Philadelphia, Pa: WB Saunders Co; 1998:189-191.

5. Brown GC, Tasman WS. Peripapillary staphyloma. In: Brown GC, Tasman WS, eds. Congenital Anomalies of the Optic Disc. New York, NY: Grune and Stratton; 1983:178-181.

10.3928/0191-3913-20030301-13

Sign up to receive

Journal E-contents