RESUMEN
A 32-year-old man presented with bilateral diminution of vision for 10 years. Visual acuity was light perception in both eyes with inaccurate projection in the oculus dextrus(OD) and accurate projection in the in oculus sinister (OS). Intraocular pressure was 6 and 12 mm Hg in the OD and OS, respectively. Slit-lamp examination revealed the presence of leucomatous corneal opacification with microcornea in oculus uterque (OU). Both eyes had a dislocated nucleus in the vitreous cavity on ultrasound B-scan with advanced cupping in the right eye. Ultrasound biomicroscopy revealed ciliary body atrophy in the OD and preserved ciliary processes (two quadrants) in the OS. In view of a partially preserved ciliary body function and a potential for gaining useful vision, we planned surgical intervention in the left eye. Deep anterior lamellar keratoplasty with pars planavitrectomy (PPV) and phacofragmentation of the nucleus was done. Donor lamellar graft was sutured after completion of PPV and nucleus fragmentation. The patient regained useful ambulatory vision postoperatively with clear corneal graft.