Surgical Approach To Microcornea With Macrophthalmia And Coloboma Syndrome Presenting With Pseudophakic Dislocated Lens And Mature Cataract

To describe a rare case of microcornea with macrophthlamia and coloboma syndrome presenting with unilateral pseudophakic dislocated lens and contralateral mature cataract. The altered corneal anatomy posts surgical challenges in wound creation and identifying landmarks necessary for secondary intraocular lens implantation. This report attempts to outline the ancillary diagnostics done and surgical approach for a successful outcome.

This is a case report from a tertiary hospital from a low income, third-world country.

A 50-year old Filipino male presented with bilateral blurring of vision. Visual acuity is counting fingers (CF) on the right eye (OD) and 20/200 on the left eye (OS). He has previously undergone uncomplicated cataract surgery on OS, but reports no history of trauma. Slit lamp exam shows microcornea of 7.5 x 7.5mm with patchy central stromal opacity, OD and 6x7mm  on OS, with 360 degrees pannus on both eyes. The right eye had mature cataract of LOCS grading NO3 NC3, while the OS had inferior iris coloboma and dislocated single piece lens. Biometry showed axial length of 25.91mm OD and 25.92 mm OS. Anterior segment ultrasound biomicroscopy was done to confirm the iris irregularities and determine positioning of corneo-scleral junction, as well as irregularities of the pars plana for preoperative planning. Left eye underwent pars plana vitrectomy, IOL explantation through a superior corneo-scleral tunnel. Followed by intrascleral fixation of 3 piece foldable IOL (Yamane technique). Right eye underwent standard phacoemulsification, adjusting the main corneal incision after focal peritomy, and a single piece foldable IOL was implanted at the posterior pole. Postoperative visual acuity remained to be CF, OD  due to corneal opacity and OS improved to 20/60. Posteriorly, OD was noted to have peripapillary staphyloma and macular thinning, while OS has peripapillary staphyloma, macular thinning and non-center involving coloboma. IOL on both cases remained to be stable and centered.

Microcornea, with macrophthalmia and coloboma is a rare condition compounded by multiple anatomic complications. These include poor corneal margins, iris and posterior pole coloboma that pose challenges for cataract intervention. Ancillary diagnostics such as ultrasound biomicroscopy can be used for confiriming landmarks in pre-operative planning, to ensure proper intraoperative decision making. Surgical approaches for cataract and secondary IOLs in rare ophthalmic conditions are varied and may be limited to local access of technology. As such, it is important to enrich our pool of knowledge for the management of these conditions.