CASE PRESENTATION
A 54-year-old woman presented with acute angle-closure glaucoma in her right eye. This was medically controlled, and bilateral Nd:YAG laser iridotomy was performed. Occludable drainage angles were present in both eyes. One year postoperatively, the patient presented with reduced vision, cortical lens opacities, and mild upwardly displaced crystalline lenses (Figure 1). These conditions were thought to be the cause of her original angle closure.
The patient was 6 feet tall with arachnodactyly, but investigations for Marfan syndrome were negative. She then underwent phacoemulsification and IOL implantation with a capsular tension ring (CTR). Six months postoperatively, however, she complained of blurred vision.
Figure 2 shows the patient's right eye, which demonstrated increased upward dislocation of the capsular bag, IOL, and CTR with the opacified anterior capsular rim in the papillary margin.
What surgical maneuver would you propose to address this patient's symptoms?
ROSA BRAGA-MELE, MD, MED, FRCSC
This patient presents with a subluxated in-the-bag IOL with a CTR. Significant features include the absence of obvious vitreous prolapse and lack of an Nd:YAG laser posterior capsulotomy.
First, I would confirm the absence of vitreous in the anterior chamber with either triamcinolone (Kenalog; Bristol-Myers Squibb Company, New York, New York) or trypan blue ophthalmic solution (Vision Blue; Dutch Ophthalmic Research Center International, Zuidland, Netherlands) prior to proceeding, as this will need to be managed appropriately.
There are three options for management. First, the lens and CTR implant may be explanted and an IOL implanted in the anterior chamber. This approach, however, runs the risk of disrupting the anterior vitreous face—which currently appears intact—and could potentially induce new complications. Furthermore, the removal and reimplantation of a lens could potentiate endothelial cell loss. The approach would also require a 6-mm incision and suture closure.
The second option is to reposition and refixate the subluxated IOL and CTR with iris sutures. This correction is also limited, as the additional bulk of the capsular-zonular complex and IOL would increase the lens' contact with the posterior aspect of the iris. This additional contact would increase the chances of pigment dispersion, uveitis, hyphema, and secondary glaucoma.
The third choice is ab externo scleral fixation of the CTR complex in the following manner. Ideally, the sutures are placed at the midpoint of the CTR subluxation arc (approximately at the 7:30-o'clock position). The first suture is placed at the section of the CTR that is subluxated toward the visual axis. After performing peritomy from the 6:30- to 8:30-o'clock position, cauterize the incision to achieve hemostasis. A vertical and circumferential scleral incision—1.5 mm posterior to the surgical limbus and 1.5 mm in length—should then be made at 50% thickness using a guarded diamond blade.
After creating a paracentesis at the 1:30-o'clock position, viscoelastic is injected into the anterior and posterior chambers in the area where the CTR is to be sutured; this will create the necessary space between the capsule and the iris. If possible, reform the capsular bag. Using a double-armed, 10-0 polypropylene suture on a CIF-4 needle (Ethicon Inc., Sommerville, New Jersey), a PC-7 needle (Alcon Laboratories, Inc., Fort Worth, Texas), or a 9-0 polypropylene suture on a CTC-6L needle (Ethicon Inc.) is appropriate. The latter suture is preferred because it lowers risk of late breakage. The needle is passed into the anterior chamber, over the optic, and under the CTR so that it penetrates the capsule. A second instrument (ideally, an Ahmed microforceps [Microsurgical Technologies, Redmond, Washington]) that could support the IOL/capsule complex through a second paracentesis would facilitate this maneuver. The needle must pass through the paracentesis, or a false passage will capture corneal tissue in the suture and prevent the loop from entering the anterior chamber.
After the successful passage of the suture under the CTR, a 26-gauge needle, bent at the hub, is placed into the posterior chamber at either of the lateral edges of the partial-thickness scleral incision. The tip of the suture needle is then captured in the lumen of the 26-gauge needle and externalized through the sclera. Repeat the process with the other end of the 10-0 polypropylene suture, passing it over the CTR and externalizing the suture on the other lateral edge of the scleral incision.
Tie the suture ends in a releasable fashion to allow for the titration of tension following the placement of the second suture. The applied tension on the suture centers the capsule/IOL complex, facilitating visualization of the second side of the CTR for suturing. After titrating both sutures (if needed) for tension and tying them in the usual fashion, rotate the knots so that they are located in the posterior chamber.
This technique minimizes the risk of conjunctival erosion over the knots, as well as erosion of the suture through the sclera. At this point, the IOL should be successfully recentered. If it is not, it should be repositioned with the use of viscoelastic and Kuglen hooks (Bausch & Lomb, Rochester, New York).
DANIEL ELIES, MD
This case emphasizes the relevance of clinical examination in ophthalmologic patients by ruling out clinical signs of Marfan or Weill-Marchesani syndrome in patients presenting with unexpected acute angle-closure glaucoma. In a patient with acute angle-closure glaucoma and Marfanoid phenotype (despite the lack of a Marfan syndrome diagnosis), one should have suspected an angle closure or a pupillary block, due to either a crystalline lens subluxation or very weak zonulas moving toward the crystalline lens.1 Once the acute angle-closure glaucoma has been controlled, confirm the diagnosis by looking for signs of phacodonesis, and examine under the cycloplegia to rule out the presence of a displaced lens.
Phacoemulsification is challenging in these cases, and a simple CTR placement usually fails in maintaining the IOL/capsular bag/CTR complex and, as in this case, leads to dislocation. In this scenario, I would recommend a sutured CTR as a first option, with one or two extra hooks (Cionni modified CTR; Morcher GmbH, Stuttgart, Germany)2 that are used to fix the CTR to the sclera in the direction of zonular weakness. Alternatively, an iris-supported IOL (eg, Artisan; Ophtec BV, Groningen, Netherlands, or Verisyse; Advanced Medical Optics, Inc., Santa Ana, California) can be implanted if the ectopia lentis is severe, with a profound or total loss of the zonular support.
Once the patient presents with a dislocated IOL/capsular bag/CTR complex, my surgical decision depends on the zonular support. In cases with some residual zonular support, I would suture the IOL/capsular bag/CTR complex.3-5 A single stitch is enough to reposition the lens, but I prefer to use two stitches placed 120º to 140º from the intact zonula in both directions to form a triangle with the zonula. This stabilizes the complex and avoids posterior IOL tilting. In the case of complete loss of zonular support, the entire IOL/capsular bag/CTR complex should be removed. An Artisan or Verisyse aphakic iris-supported IOL should be implanted. The presence of vitreous in the anterior chamber would require a dry anterior vitrectomy or the use of an ophthalmic viscosurgical device.
I strongly recommend a strict postoperative ophthalmic control of intraocular pressure (IOP) as well as inflammatory signs, and routinely prescribe a topical NSAID drug such as ketorolac tromethamine (Acular; Allergan, Irvine, California) for 6 weeks to prevent the development of cystoid macular edema.
CARLO FRANCESCO LOVISOLO, MD
I believe the original acute angle-closure glaucoma and the unstable capsule/IOL/CTR-zonule complex, postcataract extraction, was likely caused by ectopia lentis. Arachnodactyly or achromachia is often associated with certain medical conditions (eg, Marfan syndrome) where a mutation in the gene encoding the fibrillin—a glycoprotein essential for the formation of the elastic fibers (particularly abundant in the ciliary zonules)—is responsible for a weakened structural support of the crystalline lens. Because diagnosis is mainly clinical—no simple blood test or skin biopsy is currently available—the gene's incomplete penetrance bears no surprise when spider fingers occur with no underlying health problems. Careful examination of the fellow eye is important in these cases. Although the lens dislocation is not typical (supranasal rather than supratemporal), I would assume this case may be a form fruste of Marfan syndrome, where the deep instability of the capsule-zonule complex does not allow the surgeon the chance of maintaining a well-centered IOL. In these cases, CTR implantation gives little, if any, guarantee of preventing complete luxation over time.
Because the haptics and the CTR are enmeshed within the residual capsule and lens debris, the most conservative treatment option includes the transcapsular placement of one or more sutures to secure the temporal haptic and the portion of the CTR. This also allows for the quickest recovery of vision.
These sutures should then be secured to the ciliary sulcus or iris through an anterior approach with an ab-interno technique, using 10-0 polypropylene sutures. Different variations in technique are available.6 If the residual iris tissue is not atrophic after the acute IOP rise, the easiest method is to fixate the temporal haptic to the 10-o'clock peripheral iris using a polypropylene stitch passed through a paracentesis. This should then be tied using a Siepser sliding knot.
However, the asymmetrical fixation—one on the side of the sulcus or peripheral iris and one on the side of the capsular bag—carries a significant risk of IOL instability and tilt. For the scleral suturing, the significant potential for posterior placement in the pars plicata of the knotted haptic may cause localized pushing of the iris with segmental reocclusion of the iridocorneal angle, intraocular inflammation, vitreous incarceration with the potential for choroid hemorrhages, macular pathology, and retinal detachments. The blind maneuver behind the iris cannot guarantee the precise placement in the sulcus.7 Given the relatively young age of the patient, another concern is the longevity of the sutures. Long-term scleral suture failure and erosion with potential for endophthalmitis (secondary to biodegradation of polypropylene) are serious complications. Iris friction and chafing may cause prolonged iritis with CME and pigment dispersion with or without elevated IOP. In our hands, however, iris fixation seems to carry a smaller chance of deleterious consequences if compared with scleral suturing.8
Removing the entire capsular bag/IOL/CTR complex is especially challenging. Still, it should be carefully evaluated in the cost-benefit analysis and thoroughly discussed with the patient in the informed consent process. The patient must be aware that no one can precisely predict the long-term behavior of the unstable capsule/implant complex.
Once we decide to sacrifice the complex, I do not like grasping and removing it through an anterior segment wound, due to the risk of inadvertent vitreous incarcerations and tractions. Instead, I would perform a posterior approach to cut the entire IOL complex and clean the vitreous adherences using the maximum control of the vitreous base via pars plana vitrectomy. I would then take advantage of the posterior-assisted levitation with a viscoelastic gel to anteriorly float the complex. This step will retrieve the pieces from a sutureless 3.5-mm limbal incision. I make every attempt not to leave the eye aphakic throughout this process.
High anisometropia and contact lens intolerance are things of the past. The absence of the essential prerequisite (ie, a normal anatomy of the anterior chamber) precludes the positioning of an angle-fixated implant.9 Therefore, I would implant an iris-fixated acrylic Binderflex IOL (Iolution GmbH, Itzehoe, Germany),10 which in my practice has now substituted the inverted enclavation of an iris-claw lens (ie, the aphakic Artisan or Verisyse), because it can be inserted through a sutureless incision and guarantees more stability due to the loose iris diaphragm. The 6-mm optic is folded and introduced in a standard fashion. The long C-haptics—15 mm in overall diameter—are designed to rest on the ciliary sulcus. Special anchors are then secured (buttonholed) into the iridotomies. These are performed preoperatively with the Nd:YAG laser or intraoperatively with the vitrector at the 3- and 9-o'clock positions.
Drawbacks include a moderately steep learning curve and the fact that the instruments have not yet been perfected.
LARRY BENJAMIN, DO, FRCS, FRCOPHTH
To address this patient's problem, a suture was placed via a corneal wound across the anterior chamber, through the edge of the capsular bag and out of the eye under a scleral flap. The loop of the suture was then placed over the inferior haptic of the IOL and used to pull the capsule/IOL complex into a more central position (Figure 3). The suture was then tied under the scleral flap.
Two years postoperatively, the lens was still well centered, and the patient's unaided visual acuity was 20/20.
Rosa Braga-Mele, MD, MEd, FRCSC, is Associate Professor in the Department of Ophthalmology, Faculty of Medicine at the University of Toronto, Canada. She states that she has no financial interest in the companies or products mentioned. Dr. Braga-Mele may be reached at rbragamele@rogers.com.
Daniel Elies, MD, is a cornea, cataract, and refractive surgery specialist at the Instituto de Microcirugia Ocular (IMO) in Barcelona, Spain. He states that he has no financial interest in the companies or products mentioned. Dr. Elies may be reached at danielies@hotmail.com.
Carlo Francesco Lovisolo, MD, is Medical Director at Quattroelle Eye Centers in Milano, Italy. He states that he has no financial interest in the companies or products mentioned. Dr. Lovisolo may be reached at carlo.lovisolo@quattroelle.org or loviseye@fastwebnet.it.
Larry Benjamin, DO, FRCS, FRCOphth, is in the Department of Ophthalmology at Stoke Mandeville Hospital, in Aylesbury, UK. He is a member of the CRST Europe Editorial Board. Dr. Benjamin may be reached at larry.benjamin@btopenworld.com.
