A cataract, 'a million cut' radial keratotomy, and Marfan syndrome February consultation #1

Abstract

A 56-year-old man with an ocular history of 20+ cut radial keratotomy (RK) in both eyes and Marfan syndrome presented with blurred vision in both eyes 2 years previously. He was intolerant of contact lenses and was correctable with spectacles for the past 10 years. His presenting photographs and corneal topographies are shown in Figures 1 and 2JOURNAL/jcrs/04.03/02158034-202102000-00022/figure1/v/2021-04-12T204757Z/r/image-tiffJOURNAL/jcrs/04.03/02158034-202102000-00022/figure2/v/2021-04-12T204757Z/r/image-tiff, respectively. His left eye had greater than 270 degrees of zonulopathy and a visually significant cataract. He underwent a planned pars plana lensectomy/vitrectomy and implantation of a scleral-fixated CZ70BD (Alcon Laboratories, Inc.) intraocular lens (IOL). He has enjoyed adequate vision in the left eye and now has a worsening cataract in his right eye. He is a practicing dentist and requested the fastest visual rehabilitation possible. His corrected distance visual acuity was 20/50 with a manifest refraction of +5.00-4.00 × 90 in the right eye and 20/25 with a manifest refraction of +1.75-2.50 × 180 in the left eye. Intraocular pressure (IOP) was measured at 16 mm Hg in both eyes, and extraocular motility, confrontational visual fields, and pupils were normal in both eyes. On slitlamp examination, he had mild ptosis in both eyes, the corneas had 20+ RK with multiple arcuate incisions at the 3- and 9-o'clock positions in both eyes, the anterior chamber (AC) was deep and quiet in both eyes, both irides had mild iridodonesis, the right lens had a 2 to 3+ nuclear sclerotic cataract with 6 clock hours of superotemporal zonulopathy that was only evident with dilation (Figure 3JOURNAL/jcrs/04.03/02158034-202102000-00022/figure3/v/2021-04-12T204757Z/r/image-tiff) and no phacodonesis. The left lens had a well-positioned CZ70BD IOL fixated at 6 and 12 o'clock without extrusion or exposure of the Gore-Tex suture. The posterior segment examination was unremarkable. What counseling would you provide for this patient in preparation for surgery? How would you plan the IOL calculations? What intraoperative techniques would you use to achieve the safest outcomes given his comorbidities?