DR. KEERTHI REDDY VONTELA

DR. VENKATA RATHNAM PERAM, DR.SUPERNA MAHENDRA, DR.PRATHIMA LINGA

Abstract

Purpose

To report a case of bilateral Microspherophakia-RE Aphakia with posterior dislocation of lens, LE secondary angle closure glaucoma with clear lens in Anterior-chamber.

Methods

A 14Y/F presented with LE pain, redness, nausea, vomiting : 1 week.

BCVA:RE 6/36,LE CF-1m.

BE-congenital ptosis

RE-Aphakia.

LE:circumcorneal congestion, corneal edema, dislocated clear lens compromising corneal endothelium.

IOP with GAT:RE 14mmHg, LE 42mmHg.

Gonioscopy:BE occludable angles.

BSCAN:RE Dislocated lens in Vitreous Chamber.LE normal.

Medical management-to control IOP & inflammation.

Plan

RE PPV+PPL+secondary IOL implantation.

LE Lens explantation+anterior vitrectomy f/b secondary IOL implantation at later date.

4 first degree relatives found to have Microspherophakia.

Conclusion

Management of Microspherophakia with Ectopia Lentis is multifaceted. Ocular examination has to be complemented with systemic,cardiac evaluation,genetic counselling including evaluation of first degree relatives.