Journal of Pediatric Ophthalmology and Strabismus

Whats Your Diagnosis 

Infantile “Jagged ‘Overdeveloped’ Pupil”…Recent “Iridocorneal Endothelial Syndrome”

Helen H. Yeung, MD

Abstract

A 19-year-old man was seen for evaluation of increased intraocular pressure (IOP) controlled with a progressive number of topical medications following recognition of an IOP of 28 mm Hg during a routine eye examination 3 years previously. At his initial ophthalmologic examination, an inferior ectropion uvea was described and gonioscopy reported 40% peripheral anterior synechiae. Further initial testing found normal Goldmann visual fields and endothelial cell counts of 2,933/2,857 mm 2 without pleomorphism. Stable cup–disc ratio was 0.7 in the right eye and 0.6 in the left eye and visual fields were observed repetitively with continued treatment for glaucoma secondary to the suspected iridocorneal endothelial syndrome. On examination, the patient reported good health with no ocular symptoms and there was no systemic or ocular evidence of neurofibromatosis-1 or family history of this disease. His mother recalled seeing a “jagged” pupillary defect in the right eye in infancy. Ophthalmologic examination determined 20/20 uncorrected visual acuity in both eyes and full eye movements. His eyelids showed 1 mm of ptosis with widening of the tarsal region in the right eye and a 2-mm reduction of vertical eyelid movement compared to the left eye. The corneas were clear and of equal size. No Haab striae or endothelial cell guttata were present in both eyes and the corneal thicknesses were 607/593 microns. The irides were equally and moderately pigmented in both eyes. The iris stroma in the right eye possessed no circumferential folds inferiorly and an easily seen pupillary ectropion uvea was present in the same iris sectors (Figure 1 ). The pupils were both central, round, and reactive to light. The iris stroma in the left eye was uniformly normal. Gonioscopy in the right eye revealed the temporal filtration angle to be normal and to resemble the normal angle in the left eye. Gonioscopy nasally in the right eye showed complete coverage of the filtration angle by iris stroma-like tissue extending anteriorly from the iris insertion to a prominent Schwalbe’s line circumferentially for 6 hours with no evidence of traction on the more central iris leaf. Bilateral central normal optic disc cupping was present. The need for continued use of pressure-lowering medications was confirmed by a trial of medications associated with an IOP in the right eye of 34 mm Hg.

For the correct answer, see page 101

The correct answer to What’s Your Diagnosis? is congenital iris ectropion syndrome.…

A 19-year-old man was seen for evaluation of increased intraocular pressure (IOP) controlled with a progressive number of topical medications following recognition of an IOP of 28 mm Hg during a routine eye examination 3 years previously. At his initial ophthalmologic examination, an inferior ectropion uvea was described and gonioscopy reported 40% peripheral anterior synechiae. Further initial testing found normal Goldmann visual fields and endothelial cell counts of 2,933/2,857 mm 2 without pleomorphism. Stable cup–disc ratio was 0.7 in the right eye and 0.6 in the left eye and visual fields were observed repetitively with continued treatment for glaucoma secondary to the suspected iridocorneal endothelial syndrome. On examination, the patient reported good health with no ocular symptoms and there was no systemic or ocular evidence of neurofibromatosis-1 or family history of this disease. His mother recalled seeing a “jagged” pupillary defect in the right eye in infancy. Ophthalmologic examination determined 20/20 uncorrected visual acuity in both eyes and full eye movements. His eyelids showed 1 mm of ptosis with widening of the tarsal region in the right eye and a 2-mm reduction of vertical eyelid movement compared to the left eye. The corneas were clear and of equal size. No Haab striae or endothelial cell guttata were present in both eyes and the corneal thicknesses were 607/593 microns. The irides were equally and moderately pigmented in both eyes. The iris stroma in the right eye possessed no circumferential folds inferiorly and an easily seen pupillary ectropion uvea was present in the same iris sectors (Figure 1 ). The pupils were both central, round, and reactive to light. The iris stroma in the left eye was uniformly normal. Gonioscopy in the right eye revealed the temporal filtration angle to be normal and to resemble the normal angle in the left eye. Gonioscopy nasally in the right eye showed complete coverage of the filtration angle by iris stroma-like tissue extending anteriorly from the iris insertion to a prominent Schwalbe’s line circumferentially for 6 hours with no evidence of traction on the more central iris leaf. Bilateral central normal optic disc cupping was present. The need for continued use of pressure-lowering medications was confirmed by a trial of medications associated with an IOP in the right eye of 34 mm Hg.

What’s Your Diagnosis?

For the correct answer, see page 101

The correct answer to What’s Your Diagnosis? is congenital iris ectropion syndrome.

References

  1. Dowling JL, Albert DM, Nelson LB, Walton DS. Primary glaucoma associated with iridotrabecular dysgenesis and ectropion uveae. Ophthalmology . 1985;92:912–921.
  2. Wilson ME. Congenital iris ectropion and a classification for anterior segment dysgenesis. J Pediatr Ophthalmol Strabismus . 1990;27:48–55.
  3. Salim S, Shields MB, Walton DS. Iridocorneal endothelial syndrome in a child. J Pediatr Ophthalmol Strabismus . 2006;43:1–3.

10.3928/01913913-20130122-01

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