Every Eye Deserves A Chance!

To show a rare case of bilateral microspherophakia and advanced glaucomatous optic nerve head damage in association with oromandibular facial anomaly. The patient showed marked restoration of vision following cataract extraction with pars plana vitrectomy and implantation of scleral fixated IOL.

Ruby Eye Hospital and PG institute 

A male patient in mid 20’s presented to us  with defective vision in both eyes for the last three years. On diffuse torchlight examination patient had shallow anterior chamber in BE. In the left eye, a significant relative afferent pupillary defect (RAPD) was detected, resulting in reduced visual acuity, with the patient only able to count fingers when held close to their face, albeit with accurate projection of rays (PR). Further examination revealed a faintly visible optic nerve head (ONH) with deep cupping and significant retinal nerve fiber layer (RNFL) loss. Anterior segment OCT was performed which revealed significantly shallow anterior with crowding in angle structures. Upon thorough examination, the patient's condition was diagnosed as bilateral microspherophakia, accompanied by advanced glaucomatous damage, with the left eye exhibiting more severe impairment than the right. On systemic evaluation, patient had a high-arched palate with dental malformations. Patient was scheduled for lenticular removal with and debulking of the anterior vitreous face for pushing the iris diaphragm back and opening up of trabecular meshwork.  A Pars Plana Vitrectomy with Pars Plana Lensectomy was undertaken, accompanied by a sutureless Scleral fixated Intraocular lens (IOL) implantation. The surgery proved successful, as the patient's vision improved to 6/24 post-operation. Additionally, the intraocular pressure (IOP) stabilized at 16 with the aid of two antiglaucoma medications.

Microspherophakia with mandibulofacial dystosis has not been reported in the literature. In cases of advanced optic nerve head damage with neuroretinal rim loss reduction of intraocular pressure reverses visual loss in young patients unlike the elderly. Pars plans vitrectomy with scleral fixated IOL is preferred over IOL inside the capsular bag as it deepens the anterior chamber allowing the trabecular meshwork to open.