Pupilloplasty in a patient with true polycoria: a case report

Pupiloplastia em um caso de policoria verdadeira

Handan Bardak Nimet Yesim Ercalik Murat Gunay Ruveyde Bolac Yavuz Bardak About the authors

ABSTRACT

Here we report a case of surgical pupilloplasty in an adult with true polycoria. A 44-year old man was referred to our clinic with a best-corrected visual acuity (BCVA) of 0.5 diopters (D) in his left eye. Biomicroscopy revealed two pupils within a 2.5-mm central zone, with diameters of 1.2 and 1.1 mm. Both pupils had real iris sphincters and responded to light and chemical stimulation. Therefore, we surgically cut the bridge between the two pupils without any intraoperative or postoperative complications. One month after the surgery, BCVA had improved to 0.9 D, and the final pupil was almost round, measuring 2.7 mm in diameter.

Keywords:
Iris/abnormalities; Pupil/abnormalities; Pupil/surgery

RESUMO

Relatamos um caso de pupiloplastia cirúrgica em um paciente adulto com policoria verdadeira. Um homem de 44 anos de idade foi encaminhado ao nosso serviço com acuidade visual melhor corrigida (BCVA) de 0,5 em seu olho esquerdo. Biomicroscopia revelou 2 pupilas, dentro de uma zona central de 2,5 milímetros com dimensões de 1,2 mm e 1,1 mm de diâmetro. Ambas as pupilas apresentavam esfíncteres irianos reais que respondiam à luz e a drogas. A ponte entre as 2 pupilas foi cortada cirurgicamente. Não houve complicações transoperatórias ou pós-operatórias. A BCVA melhorou para 0,9, e a pupila ficou quase circunferencial com 2,7 mm de diâmetro, um mês após a cirurgia.

Descritores:
Iris/anormalidades; Pupila/anormalidades; Pupila/cirurgia

INTRODUCTION

The prevalence of true polycoria is extremely low(11 Duke-Elder SS. System of ophthalmology. 5th ed. St Louis: Mosby; 1964. Congenital deformities. p.592-3.,22 Loewenfeld IE. The pupil. Anatomy, physiology and clinical applications. Detroit: Wayne State University Press; 1993. Vol 1. Iris damage. p.902-6.), and the mechanism underlying its development remains unknown. A few theories have been proposed to explain the phenomenon, including segregation of a portion of the pupil margin, partial closure of a coloboma, and differentiation of neural ectodermal cells into muscle fibers in abnormal situations(33 Jaffe NS, Knie P. True polycoria. Am J Ophthalmol. 1952;35(2):253-5.

4 Mann I. Developmental abnormalities of the eye. London. British Medical Association; 1957. The iris. p.252-4.
-55 Islam N, Mehta JS, Plant GT. True polycoria or pseudopolycoria? Acta Ophthalmol Scand. 2007;85(7):805-6.). Only a few cases of true polycoria have been reported in the literature, some of which have been associated with polar cataracts, glaucoma, and retinal detachment(33 Jaffe NS, Knie P. True polycoria. Am J Ophthalmol. 1952;35(2):253-5.,66 Foos RY, Kiechler RJ, Allen RA. Congenital nonattachment of the retina with hydrophthalmia hypoplastic vitreous body and true polycoria. Am J Ophthalmol. 1968; 65(2):202-10.). Although most patients with polycoria are not surgically treated because visual acuity is not significantly affected, dimmed vision can be an issue for some. In this report, we aim to present the details of a patient with true polycoria, who complained of a low vision quality and requested treatment for this condition, and to describe the surgical pupilloplasty procedure we performed for its correction.

CASE REPORT

A 44-year-old man was referred to our clinic complaining of poor vision in his left eye that had been present since childhood. He had no history of trauma or ocular surgery, no abnormal obstetric history, and no other systemic conditions. On examination, his best-corrected visual acuity (BCVA) was 1.0 diopters (D) (-1.00, -1.75, 105º) in the right eye and 0.5 D (-4.25, -1.75, 105º) in the left eye, with intraocular pressures of 16 mmHg and 17 mmHg in the right and left eyes, respectively. Specular microscopy revealed 2,817 cells/mm2 in the right eye and 2,882 cells/mm2 in the left eye. Fundus examination was normal in both eyes, as was the right pupil, which measured 3 mm in diameter (Figure 1 A). However, there were two pupils in the left eye within a 2.5-mm central zone and measuring 1.2 and 1.1 mm in diameter (Figure 1 B). Both pupils in the left eye had a true iris sphincter and pigment epithelium. Direct and indirect pupillary reflexes were normal in both eyes. After the application of tropicamide 1% drops, both pupils in the left eye dilated (Figures 2 A and 2 B).

Figure 1
A) Preoperative image of the right (normal) eye, before pupil dilatation; B) Preoperative image of the left eye with true polycoria before pupil dilatation.

Figure 2
A) Preoperative image of the right (normal) eye after pupil dilatation; B) Preoperative image of the left eye after pupil dilatation, showing true polycoria.

Surgery

We performed pupilloplasty under retrobulbar anesthesia after the pupil was dilated with 1% tropicamide. Following two 1-mm limbal incisions, the anterior chamber was filled with viscoelastic material, and a spatula was inserted through a side port. The iris tissue between the two pupils was then elevated with the spatula to avoid contact with the lens. We then cut the iris tissue using vitreoretinal scissors inserted through the other side port (Figures 3 A and 3 B). After aspirating the viscoelastic material, limbal incisions were closed with stromal hydration, and the operation was completed with the application of intracameral cefuroxime. No serious intraocular inflammation was reported during the early postoperative period. Postoperatively, the patient was treated with antibiotics and steroid drops for 4 weeks. At the first follow-up 1 month after surgery, the BCVA had increased to 0.9 D (-3.75, -2.50, 95º), the intraocular pressure was 18 mmHg, and the lens was clear. The pupil was almost round and measured 3.5 mm in diameter on the first postoperative day (Figure 4 A), but had reduced to 2.7 mm one month after the surgery. The patient was satisfied with his improved vision and better quality of life.

Figure 3
A) Intraoperative image showing cutting of the iris (upper part); B) Intraoperative image showing cutting of the iris (lower part).

Figure 4
A) Image showing the result at the end of the postoperative day 1; B) Image showing the results after 1 month.

DISCUSSION

Pseudopolycoria is distinguished from true polycoria by the passive constriction of the accessory pupil when the true pupil is dilated(55 Islam N, Mehta JS, Plant GT. True polycoria or pseudopolycoria? Acta Ophthalmol Scand. 2007;85(7):805-6.) and is characteristic of essential iris atrophy that can be associated with Seckel syndrome, posterior polymorphous dystrophy, and juvenile glaucoma(77 Robbin DS. Seckel's syndrome with pseudopolycoria. Ophthalmic Paediatr Genet. 1985;6(3):135-9.

8 Patel AK, Loh RS, Morrell AJ. Posterior polymorphous dystrophy with polycoria and corectopia. Eye (Lond). 2004;18(8):856-7.
-99 Rodrigues MM, Spaeth GL, Weinreb S. Juvenile glaucoma associated with goniodysgenesis. Am J Ophthalmol. 1976;81(6):786-96.). In contrast, the extra pupil in true polycoria retains an intact sphincter muscle, is reactive to light, and synchronously contracts and dilates in response to medication(33 Jaffe NS, Knie P. True polycoria. Am J Ophthalmol. 1952;35(2):253-5.). These findings were observed in our patient, supporting the diagnosis of true polycoria. Although extra pupils typically present at some distance from the principal pupil(22 Loewenfeld IE. The pupil. Anatomy, physiology and clinical applications. Detroit: Wayne State University Press; 1993. Vol 1. Iris damage. p.902-6.), the extra pupil was within a 2.5-mm central zone in our patient.

Polycoria can decrease visual acuity. In one previous case report, a patient with true polycoria was noted to exhibit dimmed vision and decreased retinal illumination. Both these phenomena can be explained by the Airy disc effect or diffraction rings and interference fringes induced by the second pupil(55 Islam N, Mehta JS, Plant GT. True polycoria or pseudopolycoria? Acta Ophthalmol Scand. 2007;85(7):805-6.). It has been reported that, in true polycoria, visual acuity linearly decreases with pupil diameter for pupils with diameters >1.5 mm(1010 Miller SD, Judisch GF. Persistent pupillary membrane: successful medical management. Arch Ophthalmol. 1979;97(10):1911-3.). This trend may account for our patient's visual acuity of 0.5, considering that his pupils measured 1.2 and 1.1 mm in diameter. Although our patient was displeased with his low vision before surgery, he did not complain of either diplopia or glare symptoms. In our opinion, if his pupils had been spaced together more closely, he may have experienced these conditions. It was rewarding that his visual acuity increased following surgery and that he was satisfied with the outcome.

In conclusion, we have shown that a pupilloplasty procedure for true polycoria was effective in our case. However, the efficacy of the procedure remains unknown in the wider population. We anticipate more case reports to validate our surgical approach in cases with true polycoria.

  • Funding: No specific financial support was available for this study.

REFERENCES

  • 1
    Duke-Elder SS. System of ophthalmology. 5th ed. St Louis: Mosby; 1964. Congenital deformities. p.592-3.
  • 2
    Loewenfeld IE. The pupil. Anatomy, physiology and clinical applications. Detroit: Wayne State University Press; 1993. Vol 1. Iris damage. p.902-6.
  • 3
    Jaffe NS, Knie P. True polycoria. Am J Ophthalmol. 1952;35(2):253-5.
  • 4
    Mann I. Developmental abnormalities of the eye. London. British Medical Association; 1957. The iris. p.252-4.
  • 5
    Islam N, Mehta JS, Plant GT. True polycoria or pseudopolycoria? Acta Ophthalmol Scand. 2007;85(7):805-6.
  • 6
    Foos RY, Kiechler RJ, Allen RA. Congenital nonattachment of the retina with hydrophthalmia hypoplastic vitreous body and true polycoria. Am J Ophthalmol. 1968; 65(2):202-10.
  • 7
    Robbin DS. Seckel's syndrome with pseudopolycoria. Ophthalmic Paediatr Genet. 1985;6(3):135-9.
  • 8
    Patel AK, Loh RS, Morrell AJ. Posterior polymorphous dystrophy with polycoria and corectopia. Eye (Lond). 2004;18(8):856-7.
  • 9
    Rodrigues MM, Spaeth GL, Weinreb S. Juvenile glaucoma associated with goniodysgenesis. Am J Ophthalmol. 1976;81(6):786-96.
  • 10
    Miller SD, Judisch GF. Persistent pupillary membrane: successful medical management. Arch Ophthalmol. 1979;97(10):1911-3.

Publication Dates

  • Publication in this collection
    Nov-Dec 2016

History

  • Received
    09 Dec 2015
  • Accepted
    07 Jan 2016
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