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Management of congenital bilateral subluxated cataract with Cionni ring

Praveen, M. R. DOMS; Shah, Alpesh R. MS; Jani, Urvi D. DOMS; Raj, Shetal M. MS; Vasavada, Abhay R. MS

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Indian Journal of Ophthalmology: Jan–Mar 2006 - Volume 54 - Issue 1 - p 39-41
doi: 10.4103/0301-4738.21613
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A subluxated cataract is a difficult condition to treat and its surgical management has been challenging. Cionni and Osher1 reported the use of an endocapsular ring with a fixation hook in severe and progressive cases of zonular deficiency with better stability and centration of the capsular bag than the standard capsule tension ring (CTR). We report the clinical presentation and follow-up details of a patient with bilateral congenital subluxated cataracts who underwent phacoemulsification with implantation of posterior-chamber intraocular lens (IOL) and Cionni ring.

Case Report

A 30-year-old male patient presented with complaints of diplopia and decreased vision in both eyes. The best-corrected visual acuity (BCVA) was 20/60 in the right eye and 20/80 in the left eye. He had bilateral lens subluxation in the superior direction with nuclear sclerosis and posterior subcapsular cataract of grade 3, left eye more than the right eye. The extent of subluxation in both eyes was 210° between 3o′ and 10o′ clock position and was symmetrical with total absence of zonules at the site of subluxation (Figure 1). The history of diplopia in this patient was owing to simultaneous existence of phakic and aphakic portions in the pupil. There was no vitreous noted in the anterior chamber. The left eye was operated upon, followed by the right eye in a span of 3 months using the same technique. After maximum mydriasis, peribulbar and Van Lint anesthesia was given without any ocular massage. We followed the principles of closed-chamber technique. A scleral pocket was created in the area of zonular dehiscence. A two-plane clear corneal temporal incision was made with a 3-mm keratome (Alcon®), followed by entry into the anterior chamber. Healon GV® (Pharmacia) was placed in the anterior chamber and a 26-G bent needle used to perform a continuous curvilinear capsulorhexis. Two flexible iris retractors made up of nylon (Grieshabers, AG) were positioned on the capsulorhexis edge and used for retraction and expansion of the capsular bag. A gentle corticocleaving hydrodissection was performed and then rotation was attempted. Phacoemulsification (Alcon, Legacy® 20000[TM] series, with AdvanTec® software and the NeoSoniX®) was completed with Neosonix and Advantec with Kelman microtip of 30° bend using the “step-by-step chop in situ and lateral separation technique,”23 followed by “slow motion technique”4 for nuclear fragment removal. A Cionni ring was implanted before the removal of the residual cortex by inflating the bag with Healon GV®. The Cionni ring consists of an open, flexible polymethylmethacrylate filament. Insertion of ring began by preplacing a 10-0 prolene suture double-armed with two strong, straight needles (Ethicon STC-6, Johnson and Johnson Intl., Belgium) through the fixation hook. A stab incision, which is wide enough to permit fixation hook, was created 180° to the scleral pocket for implantation of Cionni ring. The fixation element remaining over the anterior capsular rim was dialed with a dialer toward the area of zonular weakness. The removal of the left over cortex was done by using a bimanual irrigation and aspiration with low vacuum, low aspiration flow rate, and minimal bottle height. After cortex removal, the capsular bag was inflated with Healon GV®. The iris hooks were removed. A 26-G needle bent at the hub on a 2 cc syringe was used to pierce the globe perpendicular to the sclera at the ciliary sulcus 2 mm from the limbus at the area of dialysis. Care was taken to ensure that the 26-G needle rests on the anterior capsule but behind the iris. The needle was advanced to midpupillary space in a bevel-up position. The straight needle of the double-armed 10-0 polypropylene suture (Ethicon STC-6) was passed through the paracentesis incision 180° from the area of dehiscence. It was captured through the barrel of the 26-G needle, which was then retracted through the scleral puncture site. The process was repeated for the other straight needle in a similar manner. Both the ends of the double-armed 10-0 polypropylene suture were pulled to the center of the capsular bag and a temporary knot was created (Figure 2). The capsulorhexis was enlarged by initial small and definitive large rhexis.5 A single-piece SA60AT AcrySof® IOL (Alcon®) was implanted in the capsular bag. The two ends of the 10-0 polypropylene suture were pulled to provide adequate tension for centration of the capsular bag and IOL. One end of the polypropylene suture was shortend at and the suture ends were tied to each other. The suture was tightened and buried into the sclera. The overlying conjunctiva, two paracentesis incisions, and the main incision were sutured. Residual viscoelastic was removed and intracameral 1-ml, 0.5% pilocarpine nitrate was used to constrict the pupil. This also ensured absence of vitreous in the anterior chamber. Finally, 1%, 0.3-ml vancomycin was injected intracamerally. A similar procedure was done in the fellow eye 3 months later. On the first postoperative day, in both eyes mild corneal edema was present and the anterior chamber was deep with 2+ cells. The IOL and capsular bag appeared centered and stable. The intraocular pressure with applanation tonometry was 14 mm Hg. in the left eye, at 18 months, and in the right eye, at 15 months, the IOL was clinically centered in the capsular bag following surgery in undilated pupil. Eighteen months after surgery, posterior capsular opacification (PCO) with Elsching pearl formation reaching the center of the IOL in the left eye [Figure 3 (A,B)] was noticed on slit-lamp examination. The diplopia disappeared in both eyes after the surgery. The BCVA was 20/30, N6 in both eyes. The fundus examination did not reveal clinical macular edema or retinal break anytime during the follow-up.

Figure 1:
Preoperative congenital superior subluxated cataract
Figure 2:
Implantation of Cionni ring within the capsular bag. 1, scleral pocket; 2, stab incision; 3, main incision; 4, area of maximal zonular dialysis; 5, Cionni element implanted through stab incision
Figure 3:
Fifteen and eighteen months postoperative follow-up of right and left eye with Cionni ring and AcrySof implantation


Endocapsular phacoemulsification (posterior plane emulsification) with our phaco technique allowed nucleus division with minimal stress on the capsular bag and produced multiple, small nucleus fragments that were easy to consume in the central space. Using the stop-chop-chop and stuff technique and slow motion phaco4 with smaller fragments helped keep the flow and vacuum rates at safe levels, thus preventing inadvertent capsule touch and vitreous prolapse. Even though the patient was young, the cataract was firm enough to warrant the use of the technique of chopping. This technique of chopping was originally described for black and brunescent cataracts, but we employ this technique as a routine for chopping in all grades of cataract except for soft cataracts. Villarkuri and Osher reported that suturing the CTR to the capsular bag was useful.67 Lam et al.8 reported implanting a CTR and then passing a double-ended 10-0 polypropylene suture through the capsular bag to the sclera to improve capsule centration. The key to the success of these surgeries lies in maintaining capsular bag integrity while suturing. However, the integrity of the capsule is threatened by the above techniques while suturing. We believe it is difficult to implant the ring immediately after rhexis with in the bag filled with lens substance. In our experience, trying to implant at this stage stressed the capsular bag. Classically, Cionni ring implantation is done through the main incision in which the ring is relatively easy to insert and the fixation hook can be easily dialed and placed at the area of maximal zonular dialysis. Later, the two prefixed straight needles are introduced into the anterior chamber through the main incision and brought out of the eye between the anterior capsule ring and posterior iris surface and through the scleral wall, exiting 2.0 mm posterior to the corneoscleral junction at the area of maximal zonular weakness. In this particular case, we implanted Cionni ring through the corneal stab incision (which is large enough to permit fixation hook) opposite the meridian of maximal zonular dialysis. This was done because we found maneuvering of the Cionni element with two prefixed needles easier and not cumbersome when implanted through the corneal stab incision than when placed through the main incision. This was done because the maximal zonular dialysis was not exactly opposite from the main incision. The prefixed straight needle traverses from the same site of implanted ring from the corneal stab incision and comes out of the eye through the scleral pocket. Care was taken in the initial stages of implantation of the ring, to prevent the possibility of tear in the anterior capsule with the fixation hook. The other modified CTRs such as the capsule tension segment, a modified form of Cionni ring designed for localized zonular dialysis.9 A novel, flexible endocapsular ring has been manufactured with multiple islets.10 Despite using AcrySof IOL, we noted PCO in the left eye. We speculate that the incidence of PCO is higher owing to younger age group and increased activity of lens epithelial cells. We believe that these eyes are compromised and to some extent may contribute to posterior capsule opacification. After extracapsular cataract extraction, Jamal and Solomon11 reported pearling to be 32% and 18% in patients under and over 50 years of age, respectively. Our own results12 on development of central PCO in young patients less than 50 years of age after phacoemulsification using three-piece AcrySof IOL on three year follows up was reported to be 22.2%.12

In conclusion, this Cionni ring demonstrated the ability to provide centration of, and stability to the capsular bag without violating its integrity. Preoperatively, the case reported here had zonular weakness with iridodonesis and phakodonesis. Postoperatively, we achieved retention of the capsular bag with IOL centration. There was complete absence of iridodonesis and pseudophakodonesis postoperatively, thereby confirming the increased level of stability.

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11. Jamal SA, Solomon LD. Risk factors for posterior capsular pearling after uncomplicated cataract extraction and Plano-convex posterior chamber lens implantation J Cataract Refract Surg. 1993;19:333–8
12. Sheena AD, Vasavada AR, Singh R. Prospective evaluation of phacoemulsification in adults below 50 years J Cataract Refract Surg. 2005

Cionni ring; congenital; phacoemulsification; subluxated cataract

© 2006 Indian Journal of Ophthalmology | Published by Wolters Kluwer – Medknow