An Efficiently Reproductible Surgical Approach For Bilateral Ectopia Lentis Using Scleral Fixation Of A Cionni Modified Capsular Tension Ring

To describe a simple and reproductible surgical solution to pediatric ectopia lentis associated with Marfan Syndrome (MFS): usage of a Cionni modified capsular tension ring (MCTR) and scleral fixation using a 9-0 polypropylene (prolene) suture with straight atraumatic needle. Ectopia lentis is a Ghent nosology diagnosis criteria present in 60% of MFS patients with partial or complete displacement of the lens due to abnormalities in the incorporation of fibrillin into tissues such as the lens capsule, zonules, iris and sclera, associated with mutation in the fibrillin-1 gene (FBN1). Significant refractive errors, decreased corneal K values and thickness, anisometropia and high astigmatism may be present.

All clinical investigations and surgical procedures were performed in the Clinical Hospital for Ophthalmological Emergencies Bucharest.

An 8-year-old pediatric patient presented with marked decrease in visual acuity in both eyes and high myopic astigmatism (highest OD -5.25 Spherical Diopters, -9 Cylinder axis at 71°). MFS clinical features present were kyphoscoliosis, pectus carinatum, long slender limbs and arachnodactyly. Ectopia lentis was present in both eyes with temporal displacement and visualization of nasal zonules (Zech JC et all. classification grade IV: decreased fiber density, irregular inferior lens side, phacodonesis). We applied the same surgical technique in both eyes. Capsulorhexis was performed under viscoelastic using a fine forceps, starting clockwise from the center of the lens bag with the maneuver performed under the iris plane in the temporal region. A small scleral tunnel, corresponding to the middle of the zonular dehiscence zone, was constructed using a diamond blade and enlargened with a crescent blade. After aspiration of the masses a capsular hook was deployed. We proceeded with the scleral fixation of a Cionni MCTR. A 30-gauge insulin guide needle was used to perform a scleral tunnel 2mm behind the limbus and to extricate the 9-0 prolene atraumatic straight suture needle. After suturing the thread to the Cionni ring’s eyelet, the ring was loaded into an injector and very slowly implanted as to not pinch the posterior capsule. The scleral fixation thread ends were partially tensed and after implantation of the intraocular lens final suture adjustment and knots were made. 

Preservation of the lens bag is preferred in MFS patients with ectopia lentis to prevent post-surgical complications such as macular oedema, retinal detachment, or secondary glaucoma associated with lensectomy. Scleral fixation of a Cionni MCTR with 9-0 or 8-0 prolene suture threaded through the Cionni eyelet provides adequate capsular support for IOL implantation while also widely distributing tensional forces. Tightening of the suture after IOL implantation allows fine tuning of suture tension, avoiding excessive tension that could lead to later suture failure, while scleral fixation beneath a scleral pocket prevents subsequent conjunctival erosion. This method is easily reproductible and simply efficient.