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TECHNIQUE

Novel double-flanged technique for managing Marfan syndrome and microspherophakia

Canabrava, Sergio MD; Canedo Domingos Lima, Ana Carolina MD; Arancibia, Ana Elisa Loyola MD; Bicalho Dornelas, Luiza Filgueiras MD; Ribeiro, Guilherme MD

Author Information
Journal of Cataract and Refractive Surgery: March 2020 - Volume 46 - Issue 3 - p 333-339
doi: 10.1097/j.jcrs.0000000000000116

Abstract

Ectopia lentis, the displacement of the crystalline lens within the eye, is a rare condition that can be inherited as an autosomal dominant disorder or associated with several systemic disorders. Marfan syndrome, Weill–Marchesani syndrome, and homocystinuria account for more than 75% of reported nontraumatic lens displacements.1 Patients with ectopia lentis invariably have generalized and, in some cases, progressive zonular fragility.2 Displacement of the lens bag complex, as well as zonular weakness, presents with several surgical challenges.3 In particular, lens implantation can be challenging and is associated with secondary lens dislocation. We present a novel method for stabilizing the capsular bag, which enables safe insertion of an intraocular lens (IOL) within the bag.

A variety of methods and devices have been described for stabilizing the lens bag in cases of zonular weakness.3 A standard capsular tension ring (CTR) is recommended in zonular weakness less than 120 degrees (<4 clock hours) or in generalized mild laxity.4 However, in more extensive zonular weakness and progressive pathologies such as Marfan syndrome, pseudoexfoliation, Weill–Marchesani syndrome, and sulfite oxidase deficiency, fixation of the capsular bag in the sclera with a modified CTR (m-CTR) or a capsular tension segment (CTS) is recommended.5,6 Previously described methods of fixation of an m-CTR or CTS, although a well-known and reproducible technique, require high surgical skill and expertise and increase surgical time.7 Furthermore, suture material should be permanent, and polypropylene 10-0 hydrolyzes over time with a 5- to 10-year survival time, resulting in pseudophacodonesis and late IOL decentration.8

We describe a new technique of stabilizing a subluxated capsular bag that avoids the need for direct suturing onto the sclera. We have successfully demonstrated this technique in 3 patients with lens subluxation. This technique can also be applied in other congenital and traumatic lens subluxations and other zonulopathies.

SURGICAL TECHNIQUE

Capsulorhexis is performed with a femtosecond laser (Catalys, Abbott Medical Optics, Inc.) to facilitate the procedure as described by Chee et al.9 A standard CTR (Morcher GmbH) is inserted to redistribute the capsular forces, expand the fornices, and decrease the flaccidity of the posterior capsule.

With this technique, it is recommended to hydroprolapse the nucleus into the anterior chamber to avoid further stress on the weak zonular fibers, followed by phacoemulsification above the iris plane. If the bag remains too unstable to perform surgery after insertion of the CTR, capsular hooks can be inserted to support the weak zonular area.

After phacoemulsification, a sclerotomy approximately 2.0 mm from the limbus in the same quadrant of the zonular weakness is made using a 26-gauge needle. The needle enters the anterior chamber between the anterior capsule and the posterior iris surface. When the needle is in the anterior chamber, 23-gauge microforceps are used through the corneal incision to place a 5-0 polypropylene monofilament (Ethicon Inc.) into the 26-gauge needle's inner cavity (Figure 1, A). Then, the needle is used as an external guide of the suture end within the sclera (Figure 1, B and C). After this maneuver, an adjustable 5-0 polypropylene suture is passed through the anterior chamber, with one end out of the corneal incision and the other end out of the sclera. The external corneal side of the 5-0 suture is placed in the suturing eyelet of the CTS (Madhu Instruments), heated, and shaped into a flange using a bipolar portable cautery outside the eye (Figure 1, D). After this procedure, the CTS/5-0-monofilament complex is created. The CTS is then placed in the same quadrant of the zonular weakness.

Figure 1
Figure 1:
A: A 5-0 monofilament is inserted in the lumen. B and C: The prolene suture is externalized through the sclera. D: The 5-0 is heated into a first flange. E and F: The second flange is created and inserted into the scleral tunnel.

Finally, the external scleral side of the 5-0 monofilament is cut to approximately 2 mm to adjust the size and, consequently, to better position the CTS. Then, the monofilament is heated and shaped into a flange using a bipolar portable cautery (Figure 1, E). This second flange is inserted into the scleral tunnel using McPherson forceps. After centration of the capsular bag, the IOL is then implanted in the bag (Figure 1, F). The surgical procedure is also presented in video format (Video 1, available at http://links.lww.com/JRS/A23).

CASE REPORTS

The technique was performed in 3 eyes with lens subluxation: 1 patient with bilateral subluxation secondary to Marfan syndrome and in 1 eye of a patient with microspherophakia, both associated with ectopia lentis and zonulopathy greater than 120 degrees.

Case 1

A 38-year-old woman with Marfan syndrome had a progressive decline in distance and near vision in both eyes. At presentation, the patient's corrected distance visual acuity (CDVA) was 20/100 in the right eye and 20/70 in the left eye. Examination of the anterior segment showed superior temporal luxation of the lens in both eyes greater than 120 degrees (Figure 2, A and B). Because of the severity of subjective visual impairment and the patient's desire for surgical improvement, the double-flanged technique was performed on both eyes as previously described (Figure 3). There was a 4-month interval between surgeries. Capsulorhexis was performed by femtosecond laser, a standard CTR was inserted to redistribute capsular forces, and the nucleus was hydroprolapsed to the anterior chamber. Phacoemulsification was performed above the iris plane. Finally, a 5-0 polypropylene monofilament was used in a CTS to fixate the subluxated capsular bag in the sclera using a flanged method on both sides of the polypropylene without suture.

Figure 2
Figure 2:
Eye with a 180-degree disinsertion and folded capsular bag. (A) Catalys report image after capsulorhexis by femtosecond laser. (B) Intraoperative image showing the capsulorhexis performed by femtosecond laser.
Figure 3
Figure 3:
A: A 5-0 polypropylene monofilament is placed into the 30-gauge needle's inner cavity. B: A needle is used as an external guide of the suture end within the sclera. C: The external corneal side of the 5-0 suture is placed in the suturing eyelet of the CTS, heated, and shaped into a flange using a bipolar portable cautery outside the eye. D: The CTS is placed in the same quadrant of the zonular weakness. E: The external scleral side of the 5-0 monofilament is heated and shaped into a flange (red arrow) using bipolar portable cautery. F: Intraocular lens implanting (CTS = capsular tension segment).

The right eye presented with posterior capsule opacity at 30 days postoperatively. The capsulotomy was performed with a YAG laser to maintain the stability of the lens bag complex. The left eye needed no intervention after surgery.

Six months postoperatively, both eyes were free of complications. The CDVA was 20/40 in the right eye, 20/30 in the left eye, and 20/20 in both eyes. The IOLs were well centered on the capsular bags.

Case 2

A 46-year-old woman with a history of microspherophakia presented with a 4-year history of decreasing vision (Figure 4). The patient's right eye had previously been treated with scleral fixation by another group. The patient's CDVA was 20/40 in the right eye and 20/400 in the left eye. Examination of the anterior segment was notable for nuclear sclerosis in the left eye with inferotemporal subluxation, zonular disinsertion greater than 200 degrees, and lens tilt. The lens diameter was 7.9 × 7.8 mm.

Figure 4
Figure 4:
An eye with microspherophakia: lens diameter of 7.8 mm.

The new double-flanged technique was performed. Because of a large amount of zonulopathy and bag instability, fixation was accomplished with 2 CTSs. Capsulorhexis was performed using a femtosecond laser (Catalys), and a standard 10-8 mm CTR was inserted into the capsular bag. Four Mackool hooks were placed for bag stabilization, hydroprolapsed the nucleus to the anterior chamber, and the phacoemulsification was performed above the iris plane. Then, 1 sclerotomy was made 2.0 mm from the limbus in the quadrant of zonular weakness. A CTS/5-0 monofilament complex was created using bipolar portable cautery (Figure 5, A and B). After insertion of the CTSs, the second flange was created and inserted into the scleral tunnel (Figure 6, A and B). The same procedure was performed at 180 degrees from the first CTS. Finally, the 3-piece IOL was implanted in the bag. After 3 months, the patient's CDVA was 20/30, the complex lens bag was stable, and the IOL was centered (Figure 7). The final position of the IOL was confirmed with ultrasound biomicroscopy (Figure 8).

Figure 5
Figure 5:
A: The external cornea side of the 5-0 suture is placed in the suturing eyelet of the capsular tension segment. B: The suture end is heated and shaped into a flange using a bipolar portable cautery outside the eye.
Figure 6
Figure 6:
A: The surgeon is required to pull the 5-0 polypropylene suture to place the second CTS in the weakness area of the capsular bag. B: Then, the surgeon should create the second flange in the first 5-0 polypropylene/CTS complex (CTS = capsular tension segment).
Figure 7
Figure 7:
The image shows a 2-week postoperative eye in the sliplamp. It is possible to see the 2 arms of the 2 capsular tension segments in the correct position when the pupil is dilated.
Figure 8
Figure 8:
Ultrasound Biomicroscopy image showing the intraocular lens in the right position. It is possible to see the 2 CTSs in the white arrows.

DISCUSSION

The lens is defined as subluxated when it is displaced but still contained within the pupillary space, whereas a subluxated lens is completely detached from the ciliary body and can be loose within either the anterior chamber or the vitreous cavity.1 The surgical management of a lens subluxation has evolved considerably in the past 2 decades.

Historically, surgical management involved intracapsular cataract extraction or pars plana vitrectomy/lensectomy. However, these techniques require a large corneal/scleral incision, which increases the intraoperative risk for iris prolapse and expulsive hemorrhage. Furthermore, these procedures result in significant postoperative corneal edema, making astigmatism more likely and leading to a relatively long recovery period.10 Furthermore, vitreous loss is also more likely with intracapsular cataract extraction, and the retinal detachment rate is higher. Finally, neither intracapsular cryoextraction nor pars plana lensectomy leaves capsular bag support for an IOL. Often, the resulting aphakia requires problematic aphakic spectacles or aphakic contact lenses.8,11,12

Today, several safer and more reproducible techniques are available, such as scleral-fixated posterior chamber IOLs, either directly or via capsular bag attachment to the sclera; anterior chamber IOLs; iris-fixated anterior and posterior chamber IOLs; and angle-supported IOLs.13

In cases of Marfan syndrome, open-loop anterior chamber IOL implantation should be avoided because of these patients' specific anterior chamber anatomy. They typically have abnormally deep anterior segments, where the standard open-loop anterior chamber IOLs do not fit appropriately, and its excessive movement leads to uveitis, glaucoma, corneal decompensation, IOL dislocation, and cystoid macular edema. Furthermore, these types of IOLs are not recommended in patients with long-life expectancy, as is often the case in conditions such as Marfan syndrome and microspherophakia.14

An alternative approach is to use anterior or posterior iris-fixated or iris-claw IOLs. Because of the different mechanism of fixation of these lenses, compared with open-loop anterior chamber IOLs, they are an excellent option for ectopia lentis management. Several authors have described their successful use in patients with subluxated lenses secondary to Marfan syndrome and also in patients with microspherophakia.15–18 Zheng et al. compared the clinical outcomes between iris-fixated IOLs and scleral-fixated posterior chamber IOLs in 71 eyes. The loss rate of the corneal endothelium in the scleral-fixated posterior chamber IOL compared with the iris-fixated anterior chamber IOL was not statistically significant in a 1-year follow-up period. However, in the scleral-fixated IOL, a 48.7% rate of IOL decentration was found at 1-year follow-up, whereas in the iris-fixated anterior chamber lenses, there were no cases.19

Our surgical method stabilizes the capsular bag in the setting of zonular weakness and then implants the lens into the capsular bag. To achieve this, it is important to complete a correctly sized and positioned capsulorhexis. One of the most challenging surgical steps in these cases is the capsulorhexis because puncturing and tearing the anterior capsule is difficult when zonular countertraction is compromised.4,20 On the other hand, the femtosecond laser does not depend on zonular support to create a capsulorhexis, and thus, it is able to create a circular capsulotomy despite lens subluxation.18 One of the advantages of laser software is the ability to manually reposition the capsulotomy and decrease its size. Thus, even in severely subluxated lenses, the femtosecond laser is effective in performing anterior capsulotomy.18,21,22 Femtosecond laser–assisted capsulorhexis can be challenging in cases of lens subluxation. However, successful use of the femtosecond laser in this setting is well described.9 The Liquid Optics Interface (Abbott Medical Optics, Santa Ana, CA, USA) feature of the Catalys laser system can be challenging when used with subluxated lenses; however, with the methodology described above, we did not find any complications.23

The next stage of our method requires stabilization of the subluxated capsular bag. When the subluxation is less than 120 degrees or has generalized mild severity, a traditional CTR is usually adequate for overcoming the weakness.3 However, eyes with zonular dialysis or weakness greater than 120 degrees may require scleral capsular bag fixation for centration and long-term stabilization.24,25 Since Hara et al. first presented the standard CTR in 1991, several authors have proposed different approaches to treat zonular dialysis using variations of CTR.26–29 In 1995, Cionni and Osher reported good outcomes of phacoemulsification and CTR implantation in patients with compromised zonular fibers.8 CTRs seem to provide excellent IOL centration and positioning several months after operation. Furthermore, in experimental studies, the tendency of secondary lens epithelial migration was reduced by the use of an endocapsular tension ring in cataract surgery.30 Cionni and Osher in 1998 proposed an m-CTR designed to provide scleral fixation without violating the integrity of the capsular bag.2 The method uses a suture to fixate the CTR and lens bag to the sclera.2 The use of this method is now well documented, and several articles show its safety and stability, even in cases in which toric IOLs were implanted.22,31 An alternative to a CTR is the Cionni ring that allows additional suture fixation of the bag to the sclera, making posterior chamber IOL implantation possible in subluxated cases. However, we advise against this method because in patients with Marfan syndrome and microspherophakia, the capsular bag is sometimes smaller than in normal eyes; thus, the standard Cionni ring may be too large, increasing the risk for a capsular bag tear. This problem is exacerbated in pediatric patients.32

Ahmed proposed the CTS, which was designed to be used either intraoperatively to stabilize the capsular bag or to fixate it permanently postoperatively.33 Recently, it was proposed that the Ambati CTS, which has 2 eyelets close to each other (allowing the distribution tension to 2 points and avoiding too much stress at a single point on the anterior capsulotomy), and thus it reduces the risk for anterior capsule tear.34

Polypropylene 10-0 sutures are the traditional choice for fixating the capsular bag to the sclera. Usually, the suture is passed through the eyelet of an m-CTR or in a CTS and is fixated under a scleral flap. To achieve this, it is necessary to pass a needle with the monofilament through the capsular tension device eyelet, then through the anterior chamber, under the posterior iris surface, and through the scleral wall, exiting 2.0 mm posterior to the limbus at the area of zonular weakness. Then, the capsular tension device is implanted in the capsular bag. Once the capsular bag is centered, the sutures are tightened and tied, and the knot is buried by rotation under the scleral flap. However, the published literature has shown that 10-0 polypropylene sutures are susceptible to degradation over time, which can lead to long-term IOL dislocation.7,35 Therefore, techniques using a thicker monofilament could potentially minimize the risk for suture detachment.

Soosan Jacob proposed a sutureless technique, in which they used a capsular hook to stabilize the capsular bag during surgery, and then, this hook is anchored under a scleral flap, using fibrin glue, as proposed earlier by Agarwal.6,36 The disadvantage of this technique is that support is only 1 point of the capsule, whereas the flanged CTS complex supports at an approximately 140-degree extension, as the entire CTS is supported within the capsular bag, reducing the chance of IOL tilt in the postoperative follow-up.

In 2017, we described a knotless technique that uses a double-flanged 5-0 polypropylene haptic that was initially removed from a 3-piece IOL to fix the capsular bag in zonular dialysis greater than 120 degrees in a patient with traumatic zonular dialysis.37 The new modified technique presented in this article uses an adjustable 5-0 polypropylene suture instead of the 3-piece IOL haptic in patients with ectopia lentis. Because the 5-0 polypropylene suture is adjustable in size, it is easier to fix the bag compared with the use of the haptic. We call this method, the double-flanged adjustable transscleral bag fixation technique. After phacoemulsification, a sclerotomy is made transconjunctivally approximately 2.0 mm from the limbus in the same quadrant of the zonular weakness using a 26-gauge needle. The needle enters the anterior chamber anterior to the capsular bag without penetrating it. Twenty-three-gauge microforceps are used through the corneal incision to place a 5-0 polypropylene monofilament into the 26-gauge needle's lumen (Figure 1, A and B). This needle acts as a guide to the monofilament externally, avoiding the technically challenging passing of long needles across the anterior chamber as in the traditional approach using a 10-0 polypropylene or 7-0 polytetrafluorethylene suture (Figure 1, C).

Then, the 5-0 polypropylene suture is passed through the anterior chamber, with one end external to the corneal incision and the other end passing through the sclera. The suture end at the corneal side is placed in the suturing eyelet of the CTS. By using a bipolar portable cautery, the suture end is heated to shape it into a flanged shape, which creates the CTS complex (Figure 1, D). Finally, the CTS/5-0 monofilament complex is placed in the same quadrant of the zonular weakness, and the scleral flanged part is buried into the scleral tunnel (Figure 1, E and F). The scleral fixation of the polypropylene should be as radial as possible from the point of maximal zonular weakness. However, as the flanges in the eyelet of the CTS can move to set the best position, the surgeon can adjust its exit to find the best capsular bag centration, without excessive tension. We recommend that the CTS should only be inserted once phacoemulsification is completed. Before this, if extra support is required during nuclear disassembly, traditional CTR and capsular hooks can be inserted before phacoemulsification to stabilize the capsular bag. The CTR can either be inserted using a Sinskey hook to guide its entry and reduce tension in the capsular bag, or it can be introduced alone, depending on the capsular bag fragility. In our experience, we choose to hydroprolapse the nucleus to the anterior chamber and make the phacoemulsification above the iris plane to avoid further stress on the weak zonular fibers. Therefore, capsular support during phacoemulsification is unnecessary. However, if the surgeon prefers to center the nucleus inside the capsular bag, we suggest capsular hooks or using the CTS from the beginning of surgery.

To secure the CTS, we chose a 5-0 polypropylene monofilament. This was chosen because 5-0 polypropylene monofilaments are approximately 10 times thicker than 10-0 monofilaments and so have greater tensile strength.37,38

It is likely, based on this and the enlarged diameter of the suture, that the 5-0 monofilament has greater resistance to long-term failure. Yamane proposed the IOL-flanged technique and showed its safety and stability in IOL secondary implants. This long-term follow-up shows that the flanges seem to be safe from the spread of postoperative infections. Therefore, we can infer that the 5-0 polypropylene flanged suture to fixate the capsular bag in the scleral can be safe too.39 However, we recognize the potential risk for late IOL subluxation as a result of a single 5-0 polypropylene suture. For that reason, long-term follow-up data will need to be obtained both on the longevity of the suture and its fixation to the CTS and sclera using the heated flange technique. This technique does not use a scleral flap, which theoretically may facilitate the spread of postoperative infections. Further studies with more patients and longer follow-up are necessary to assess the exact outcomes.

In conclusion, we describe a novel method for stabilizing the capsular bag to the sclera in eyes with ectopia lentis that require cataract surgery using a CTS/5-0 polypropylene complex. The absence of tied sutures, flaps, and glue in this approach ensures that it is faster to perform and induces less ocular tissue trauma. Surgery in the setting of lens subluxation is always challenging; however, we think that, despite it involves using thicker suture and creating appropriate flanges in different meridians, our method presents less technical difficulty than previously described surgical techniques. Furthermore, by using a single 5-0 polypropylene suture, this technique minimizes traditional complications associated with suture degradation and subsequent IOL misalignment or dislocation over time. However, the very long-term stability of thermal created flanges has not been yet confirmed, and further studies with longer follow-up are needed to establish the safety data.

Experience with this new technique in clinical practice has shown IOL centration and bag stability outcomes similar to those achieved with conventional suture-held CTSs or those sutured with double-flanged 5-0 polypropylene haptic by 12 months postoperatively (Figure 8—Ultrasound Biomicroscopy). However, as a new technique, further studies with more patients and longer follow-up are necessary to assess the exact outcomes it can achieve among patients with zonular weakness of different etiologies.

WHAT WAS KNOWN

  • Cataract surgery in patients with Marfan syndrome and/or with zonular weakness or dialysis may be problematic, and these problems are currently managed by anterior chamber intraocular lenses (IOLs), an iris-fixed posterior chamber IOL, a scleral-fixed IOL, or an IOL placed in a capsule centered with capsular tension rings and capsular tension segments sutured to the sclera with polypropylene or Gore-Tex sutures.

WHAT THIS PAPER ADDS

  • The double-flanged transscleral bag fixation technique is a new knot-free option for managing patients with Marfan syndrome and zonular weakness or dialysis in eyes undergoing cataract surgery.
  • This new technique provides a new, less expensive, faster, and easier way to treat subluxated cataracts.

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