Emergent Lensectomy For Phacomorphic Angle Closure In Alport Syndrome With Anterior Lenticonus

To highlight surgical challenges and intraoperative considerations when performing cataract extraction in patients with anterior lenticonus associated with Alport syndrome.

This case was managed at Children’s Hospital of Michigan and Kresge Eye Institute, tertiary referral centers in Detroit, Michigan, USA.

An 18-year-old male with Alport syndrome and renal insufficiency presented with acute right eye pain, eyelid swelling, and headache after several months of progressive vision loss. He had been evaluated the day prior and diagnosed with a visually significant cataract. On presentation, best-corrected visual acuity was hand motion at 1 foot and intraocular pressure (IOP) was 59 mmHg. Slit-lamp examination showed diffuse microcystic corneal edema, a uniformly shallow anterior chamber, and a white cataract abutting the pupillary margin. The pupil was mid-dilated and fixed. Posterior segment view was obscured; B-scan ultrasonography was unremarkable. Gonioscopy demonstrated closed angles without visible angle structures. Despite maximal medical therapy and attempted laser peripheral iridotomy, IOP remained greater than 50 mmHg, and the patient underwent emergent lensectomy. Intraoperatively, the anterior capsule was noted to be spontaneously violated, consistent with capsular fragility associated with anterior lenticonus. Capsulorrhexis formation was avoided. The lens was removed using irrigation and aspiration, and a one-piece intraocular lens was implanted in the capsular bag with intact zonular support. Postoperative recovery was notable for delayed corneal epithelial healing. At two months, best-corrected visual acuity improved to 20/25. The fellow eye was scheduled for prophylactic lens extraction due to prior capsular fragility and risk of future angle closure.

Anterior lenticonus is a pathognomonic ocular manifestation of Alport syndrome caused by thinning and fragility of the anterior lens capsule. This structural weakness predisposes patients to spontaneous capsular rupture, cataract formation, and lens-induced angle closure. Cataract surgery in these patients requires careful surgical planning, including gentle capsulorhexis formation, cautious hydrodissection, and preparedness to modify lens removal techniques if capsular integrity is compromised. Early recognition and timely surgical intervention can result in excellent visual outcomes and prevent acute complications.