Subluxation of the lens is associated with Marfan’s syndrome,1 homocystinuria,1 Weill–Marchesani syndrome2 and trauma.3 4 Capsular tension ring (CTR) implantation may be the solution for zonular weakness but does not correct capsular bag decentration.5^–7 Cionni and Osher8 reported using an endocapsular ring with a fixation hook in cases of zonular deficiency. The hook enables a trans-scleral fixation suture without violating the capsular bag.

We report on the visual outcomes, intraoperative and postoperative complications of Cionni endocapsular ring and acrylic intraocular lens (IOL) implantation in eyes with subluxation of the lens secondary to Marfan Syndrome.

PATIENTS AND METHODS

Fifteen eyes of 12 patients aged 19–56 years (mean 32.5±14.8) with a subluxated lens or zonular weakness secondary to Marfan’s Syndrome who had phacoemulsification with posterior chamber IOL implantation were retrospectively reviewed. Table 1 presents the patients’ data and characteristics (fig 1).

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Figure 1 Marfan’s syndrome subluxated lens. In the pupillary area, the inferior edge of the subluxated lens is visible.

The eyes were operated on by one surgeon (D R) using retrobulbar anesthesia in 8 cases and general anesthesia in 7 cases. Retrobulbar anesthesia consisted of an injection of a mixture of 2 cc of mepivacaine and 2 cc of bupivacaine. The surgery was performed as previously described by Moreno-Montans et al.9 10

Briefly a fornix-based conjunctival flap was prepared nasally and temporally and a partial-thickness scleral flap was dissected posteriorly with a crescent blade up to 2.0–3.0 mm posterior to the limbus. A 3.0-mm superior incision was then made. A continuous curvilinear capsulorhexis (CCC) was done with a cystotome and a Utrata capsulorhexis forceps. Five iris retractors (FCI Ophthalmics, Marshfield Hills, MA) were used at the CCC edge to support the bag. In all eyes, the Cionni ring (Morcher type 2L) was introduced after phacoemulsification.

One end of a double-armed 10–0 polypropylene (Prolene®) suture was knotted on the Cionni ring hook. A 25-gauge needle was introduced into the ciliary sulcus 1.5 mm posterior to the limbus and advanced into the posterior chamber. The needle of the 10–0 polypropylene suture was passed through the superior incision and captured in the barrel of a 25-gauge needle, which was then retracted through the sclera. A Cionni ring (2-eyelet) was implanted in the capsular bag. The eyelet was positioned anterior to the capsulorhexis and dialled horizontally. The Cionni ring was sutured to the sclera. The knot was buried in the scleral bed and covered with the scleral flap and conjunctiva. The second eyelet was treated similarly and sutured at 180° to the first one.

An AcrySof IOL10 was implanted in the capsular bag. Intraocular lens centration was confirmed.

Postoperatively, all patients received Tobradex (tobramycin and dexamethasone, Alcon Laboratories, Randburg) eye-drops 4 times daily during the first postoperative week and prednisolone eye-drops 4 times daily for 4 weeks.

RESULTS

Mean axial length measurement (A scan) was 25.1±0.78 mm. The mean follow-up duration was 14±9.49 months. In all eyes, capsular bag centration was excellent (fig 2).

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Figure 2 (A) A 21-year-old Marfan’s Syndrome woman 6 weeks following cataract extraction + cionni endocapsular ring implantation in the right eye. Note the excellent capsular bag centration, and cionni ring eyelet demonstrated against the red reflex after dilating the pupil. (B) Visante OCT photograph of the anterior segment of the same patient demonstrating the cionni ring eyelet anterior to the IOL, at the posterior chamber.

All eyes had improved best corrected visual acuity (BCVA) after surgery: preoperative visual acuity ranged from 20/50 to counting fingers, and improved to better than 20/40 in all eyes (table 2).

Table 2 shows the intraoperative and postoperative complications. In 2 eyes (2/15), posterior capsule tear and vitreous in the anterior chamber were noticed during surgery, before the insertion of cionni ring. Anterior vitrectomy was performed, and IOL was sutured in the sulcus using a 9/0 prolene suture.

In one eye (1/15), lens subluxation temporarily increased during ring rotation and implantation of the cionni ring. After an anterior vitrectomy and trans-scleral fixation of the Cionni ring were performed, capsular bag centration was excellent.

The most frequent complication over a mean follow-up of 14±9.49 months (range 3–27 months) was posterior capsule opacification (PCO), which developed in 3 eyes (3/15, 20%), 2 of which required a neodymium:YAG posterior capsulotomy. At the last follow-up visit, the Cionni ring provided excellent centration and positioning in all cases. No eye developed retinal detachment.

Five eyes (5/15, 33.3%) had undergone wavefront analysis of total eye aberration, tilt and high-order optical aberrations before and after the operation. Total eye aberration, tilt and high-order aberrations with a 6-mm pupil diameter decreased significantly after surgery in all three eyes: Total eye aberration decreased from a mean of 14.8±5.5 preoperatively to 2.1±4.3 microns after the operation. Tilt was decreased from 4.1±2.5 to 0.12±2.1 microns, and high-order aberrations decreased from 4.37±3.8 microns, before the operation, to 1.47±3.5 after the operation.

DISCUSSION

In the past, surgical intervention in eyes with subluxated lens was considered difficult, leading to many complications.3 The Cionni ring allows additional suture fixation of the bag to the eye wall, making posterior chamber IOL implantation in these cataract operations possible.

In this study, the Cionni ring enabled the capsular bag centration. However, similar to others’ experience with the cionni ring,11 there were some intraoperative limitations. First, with an unstable lens, a central capsulorhexis might be difficult, and it is often small. Second, in cases with the extensive subluxation, implantation can be difficult, as well. Third, we noticed that the capsular bag of the Marfan’s patient is sometimes smaller than that of a normal eye, so the standard cionni ring may be too large for this kind of bag, and implanting it increases the risk of a rip or tear of the bag. The same problem arises with younger Marfan’s patients (<6 years) than these studied here, in whom the size of the available Cionni ring does not fit with the size of the capsular bag.

Other surgical solutions for ectopia lentis have been reported: anterior chamber IOLs, open loop11 12 or iris fixated (Artisan)13 are simple and efficient techniques, but risks include iritis, pigment dispersion, corectopia, glaucoma, and endothelial loss. Zetterström et al14 found that trans-scleral fixation of a posterior chamber IOL is a valid procedure in young eyes. Tsai and Tseng15 performed pars plana lensectomy and trans-scleral fixation of a foldable IOL to treat a subluxated lens. However, the capsular bag is not preserved with either technique. The results in our series demonstrated comparable anatomic and visual outcomes of both techniques: cionni ring implantation or trans-scleral IOL fixation at the ciliary sulcus.

Another option to stabilise the capsular bag is an endocapsular tension ring.5 6 However, CTR implantation in eyes with a subluxated lens does not correct capsular bag decentration.7 Lam et al16 overcame this obstacle by implanting a CTR and then suturing it and the capsular bag to the sclera to improve capsule centration.

Table 3 presents previous studies published on ectopia lentis management in Marfan’s patients, the surgical procedure done, visual outcomes and complications.

Cionni et al reported in 200317 on the results of implantation of the modified capsular tension ring (MCTR) and a posterior chamber intraocular lens (PC IOL) in 90 patients with congenitally subluxated crystalline lenses (57 patients with Marfan Syndrome ). They concluded that the use of the MCTR resulted in centration of the capsular bag and PC IOL in all eyes. Fixation of a 9–0 polypropylene suture was recommended to decrease the risk for late suture breakage.

The most frequent postoperative complication in our series, as published by Cionni et al,17 was PCO, which occurred in 20% of cases. Previous studies demonstrated a reduction in secondary lens epithelial cell proliferation by the use of an endocapsular tension ring.18 19 However, the Cionni ring has a different structure with a hook that may allow the epithelial cells to proliferate. In our study, only 2 of the 3 eyes with PCO required an Nd:YAG laser posterior capsulotomy. In these cases, the Cionni ring remained stable after the posterior capsule was opened by the laser.

Two patients (13.3%) had glaucoma in our series, both of them developed an open angle glaucoma before the operation, and the glaucoma did not resolve afterwards. Marfan syndrome patients are known to have an increased risk for glaucoma, as well as retinal detachment (up to 11% of patients with Marfan syndrome, and 8–38% in those who have dislocation of the lens or have undergone lens surgery).20 However, no patient in our study developed retinal detachment. This could be an advantage of this operation, which preserved the posterior capsule, avoiding disruption of the vitreous in this myopic population (mean axial lens 25 mm). A longer follow-up and larger sample size should be done before any conclusion could be drawn.

Our study confirms previous studies’ findings about the safety and good visual outcomes of cionni endocapsular ring implantation in Marfan’s patients and is the first to demonstrate the significant improvement in total, tilt and high-order eye aberrations following this surgery. This can contribute to better quality of vision, although it was not studied here.

In conclusion, we suggest that Cionni ring implantation is a safe procedure to correct partial lens subluxation. However, this procedure requires a highly skilled surgeon and cannot always be completed.