Ocular abnormalities and complications in anterior megalophthalmos: a case series

Abstract

Objectives: To describe the clinical and Scheimpflug imaging features in cases of anterior megalophthalmos (AM).

Methods: Retrospective record review was performed for patients with AM who presented between June 2017 and May 2018. Clinical history, slit lamp examination, Scheimpflug imaging indices (Pentacam-HR, Oculus, GmbH), dilated fundus examination and treatment records were reviewed.

Results: The study included eight eyes of four male patients (mean age 6.5 years, range 4-10 years). Corrected distance visual acuity ranged from finger counting to 20/80. The mean horizontal corneal diameter, central corneal thickness, steep keratometry, flat keratometry, anterior chamber (AC) angle, AC depth, maximum pupil diameter and axial length were 13.8 ± 0.5 mm, 538.7 ± 68.9 µm, 42.8 ± 1.6 D, 41.4 ± 0.9D, 47.0 ± 4.2 degree, 3.8 ± 0.3 mm, 3.9 ± 0.1 mm, and 24.9 ± 0.9 mm, respectively. Posterior dislocation of crystalline lens, vitreous degeneration and rhegmatogenous retinal detachment (consequent of retinal dialysis/atrophic hole/lattice with hole) were noted in seven, eight and five eyes, respectively. Pigment dispersion glaucoma was noted in both eyes of one patient, which was managed with topical anti-glaucoma medication. Vitrectomy with silicone oil tamponade was successful in retinal reattachment in all three eyes that underwent surgery.

Conclusion: Scheimpflug imaging helps in demonstrating the corneal and anterior segment pathological changes in AM. The disease extends to involve the zonules, vitreous and retina as well. Ophthalmologists should be able to identify this disorder, recognise and manage the associations and complications.

Fig. 1

Slit lamp colour photograph of the anterior segment of right and left eye of a 4-year-old boy with anterior megalophthalmos (case 1) shows large clear corneas, well-defined corneo-scleral junction, diffuse iris atrophy and aphakia (a, b). Ultra-wide field pseudo-colour fundus image of the right eye (c) shows a posteriorly dislocated crystalline lens and a total rhegmatogenous retinal detachment with few subretinal bands along the supero-temporal arcade and in temporal mid-periphery. A posteriorly dislocated clear crystalline lens was noted in the left eye as well (d). The left eye retinal examination was unremarkable

Fig. 2

Slit lamp photograph of the anterior segment of right and left eye of a 6-year-old boy with anterior megalophthalmos (case 3) shows large clear corneas, well-defined corneo-scleral junction, diffuse iris atrophy and aphakia (a, b). In addition, trans-illumination defects are seen in the inferior half of iris in the left eye (b). Ultra-wide field fundus images show posteriorly dislocated crystalline lens and attached retina in both eyes with an atrophic hole in the temporal periphery in the right eye (c) and inferior equatorial radial lattice degeneration in the left eye (d). Glaucomatous cupping can be noted in both eyes, more so in the left eye