Cataract, glaucoma, possible Marfan syndrome, and conception aspirations: January consultation #1

A 36-year-old highly myopic woman was referred for management of both cataract and glaucoma. Her ocular history included retinal detachment repair in each eye, 9 years earlier in the right eye and 7 years earlier in the left eye. Although the patient did not remember specific details of the retinal surgery, she recalled that she had a “gas bubble” postoperatively in the right eye, but not the left eye. She also had a very dense nuclear cataract in the right eye, but only mild nuclear sclerosis in the left eye. At presentation, the patient's corrected distance visual acuity (CDVA) was 20/125 in the right eye, with a large myopic shift (−18.25 + 2.00 × 175). Her CDVA in the left eye was 20/20 (−11.00 + 1.00 × 20). It is notable that she is contact lens–intolerant. Her angle was wide open in each eye, and each optic nerve had severe myopic saucerization and cupping. The axial length was 28.5 mm and 28.7 mm in the right eye and left eye, respectively. The intraocular pressure (IOP) at presentation was 18 mm Hg in the right eye and 20 mm Hg in the left eye; each eye was treated with a topical β-blocker, α-2 agonist, and a prostaglandin. The highest IOP measurements before treatment were 27 mm Hg and 25 mm Hg in the right eye and left eye, respectively. The pachymetry was 545 μm in the right eye and 540 µm in the left eye. Her visual fields and nerve fiber layers on optical coherence tomography (OCT) are shown in Figures 1 and 2, respectively. JOURNAL/jcrs/04.03/02158034-202001000-00025/figure1/v/2023-08-19T002529Z/r/image-tiff JOURNAL/jcrs/04.03/02158034-202001000-00025/figure2/v/2023-08-19T002529Z/r/image-tiff Cataract surgery was scheduled along with a coincident glaucoma procedure. It is noteworthy that intraoperatively, the right capsular bag was very loose. Indeed, the capsular bag could not be penetrated with the cystotome, which only dimpled the capsule severely but would not penetrate it. Accordingly, a super-sharp, #15 blade was used to pierce the capsule and initiate the capsulotomy. Whereas the zonule was obviously loose, the remainder of the procedure was completed without incident and the intraocular lens (IOL) placed in the capsular bag with perfect centration. It was unclear whether the loose zonule was a consequence of the patient's vitreoretinal surgery or whether there was a systemic cause for her zonulopathy. Although it was not suspected before the surgery, in retrospect, this patient had the classic body habitus of Marfan syndrome. Moreover, subsequent surgery in the fellow left eye found the zonule to be quite loose, but not as severe as in the right eye. How would you manage this patient's glaucoma? Given the finding of very loose zonular fibers, would you initiate a workup for Marfan syndrome? Certain microinvasive glaucoma surgery (MIGS) procedures are labeled for mild-to-moderate glaucoma. How strictly do you adhere to such labeling? Do you ever use a MIGS device in severe glaucoma?