The golden ring.

Case Presentation

Afemale in her mid-20s presented to the ophthalmologist with low vision in both eyes since childhood. On examination, she had a best-corrected visual acuity of 20/80 in the right eye with 17.00 Ds and 20/40 in the left eye with 14.00 Ds. Intraocular pressures were normal. On close inspection, both eyes [Figure 1] (Panel A – right eye, Panel B – left eye) had thick lenses with 360° golden ring around the lens on retroillumination. Posterior segment examination was unremarkable. Systemic examination revealed a short stature, brachydactyly, and joint stiffness.

Figure 1

Anterior segment photograph in retroillumination of the right (Panel a) and left (Panel b) eyes of a patient demonstrating a round spherical lens with a golden ring

Questions

What is the ocular diagnosis?

Subluxated lens

Microspherophakia

Keratoconus

Lenticonus

Which of the following syndromes are most commonly associated with this abnormality?

Marfan's syndrome

Alport syndrome

Weill–Marchesani syndrome

Ehlers–Danlos syndrome

What is the best surgical management for this patient?

Clear lens extraction with intraocular lens implantation

Laser-assisted in situ keratomileusis

Penetrating keratoplasty

Implantable Collamer Lens

Answers

b

c

a

Case Description

This is a case of microspherophakia with Weill–Marchesani Syndrome. Microspherophakia is a lenticular abnormality in which the existing zonules lack tension that directly contributes to the presence of a small spherical lens.[1] The laxity of the zonules may lead to anterior subluxation of the lens which can cause uneven shallowing of the anterior chamber, sometimes even leading to raised intraocular pressure.[23] In a dilated pupil, the margin of the crystalline lens can be seen as a golden ring on retroillumination. This condition must be ruled out in all cases of high myopic refractive error with a dilated slit lamp examination. Surgical intervention may be required in cases of high refractive error, lens subluxation or glaucoma. This patient was managed with sequential clear lens extraction with in-the-bag intraocular lens implantation and capsular tension segments.[4] She maintains an unaided vision of 20/30 in both eyes after 1-year follow-up.

Declaration of patient consent

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Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.