Purpose
To report the short-term outcomes and complications of implantation of scleral-fixated capsular tension rings and/or capsular tension segments with intraocular lenses (IOL) in pediatric patients with ectopia lentis.
Design
Retrospective, observational case series.
Methods
Thirteen consecutive pediatric patients (19 eyes) underwent placement of in-the-bag IOL with either a Cionni modified capsular tension ring or a capsular tension segment in conjunction with a conventional capsular tension ring between January 1, 2009 and March 30, 2013 by 3 anterior segment surgeons at a single academic center. The scleral fixation suture was 9-0 polypropylene in 16 eyes and CV-8 Gore-Tex (expanded polytetrafluoroethylene) in 3 eyes. Outcome measures included change in corrected distance visual acuity (CDVA) and complications.
Results
The mean age was 10.2 years ± 4.8 (SD) and the median follow-up, 23.4 months. A Cionni modified capsular tension ring was implanted in 5 eyes and a capsular tension segment with an unsutured capsular tension ring was implanted in 12 eyes. In 2 eyes, capsular tension segment alone was placed. The mean CDVA at the final follow-up (0.10 ± 0.11 logMAR, 18 eyes) was significantly better than preoperatively (0.58 ± 0.26 logMAR, 15 eyes) ( P < .001). The CDVA at the final follow-up was 20/40 or better in 18 eyes (94.7%). All IOLs were well centered. Posterior capsule opacification developed in 11 eyes (57.9%), 9 eyes (47.4%) required neodymium–yttrium-aluminum-garnet capsulotomy, and 3 eyes (15.8%) required pars plana vitrectomy and posterior capsulotomy. Other complications included broken suture (5.3%) (9-0 polypropylene at CTR eyelet, repaired with CV-8 Gore-Tex), conjunctival dehiscence (5.3%), suture exposure (5.3%) (trans-scleral 9-0 polypropylene), and vitreous strand at inferior paracentesis (5.3%).
Conclusions
Implantation of in-the-bag IOL with either a Cionni modified capsular tension ring or a capsular tension segment in conjunction with a conventional capsular tension ring appears to be a safe and effective technique for visual rehabilitation in pediatric ectopia lentis.
Crystalline lens subluxation in children has been associated with hereditary metabolic disorders such as Marfan syndrome, homocystinuria, and Weill-Marchesani syndrome, as well as trauma and idiopathic ectopia lentis. When the lens loses significant zonular support, a large refractive error, a partially phakic visual axis, and anisometropia can result; untreated, these conditions place children at risk for dense amblyopia. Loss of significant zonular support also increases the difficulty of cataract extraction with intraocular lens (IOL) implantation in children, which is already complicated by a high incidence of posterior capsule opacification (PCO), the likelihood of progressive zonular weakness, and a proclivity for inflammation. Additionally, surgical treatment for ectopia lentis carries a risk for serious complications such as glaucoma or retinal detachment. Previously, investigators have reported achieving good visual outcomes with no serious complications in children by performing limbal lensectomy with both anterior and posterior capsulotomies or pars plana lensectomy. However, these methods leave patients aphakic postoperatively, thus necessitating correction with spectacles or contact lenses. Lens-induced aniseikonia and social issues can lead to noncompliance with spectacles, while contact lenses for a child may be difficult to fit or poorly tolerated and predispose to complications such as keratitis.
The capsular tension ring has been used to effectively manage localized zonular weakness. However, it cannot provide adequate support or correct decentration of the capsular bag in the presence of extensive zonular dialysis. In 1998, the Cionni modified capsular tension ring (Morcher) was introduced to help manage profound zonular weakness. The Cionni modified capsular tension ring can be fixated to the sclera without compromising capsular bag integrity via 1 or 2 sutures; advantages are stabilization of the lens capsule and reduced risk of future IOL dislocation. Implantation of the Cionni ring with in-the-bag IOL placement in children with ectopia lentis has been reported to be safe with few short-term significant complications.
In 2002, Ahmed designed the capsular tension segment (Morcher), which is also intended for patients with significant zonular insufficiency. The capsular tension segment is a partial ring segment of 120 degrees; like the Cionni modified capsular tension ring, it has an anteriorly positioned eyelet, which enables scleral suture fixation. Compared to the Cionni modified capsular tension ring, the capsular tension segment can be inserted into the capsule bag with greater ease and less trauma because a dialing technique is not necessary. The capsular tension segment can be used alone or in combination with other endocapsular support devices such as the capsular tension ring to help manage extensive zonular loss. A case report has also reported the use of a capsular tension segment in conjunction with a capsular tension ring in a pediatric patient with microspherophakia. However, there is limited information in the literature regarding the outcome of the capsular tension segment in children with ectopia lentis. We present the short-term outcomes of 19 pediatric eyes after implantation of scleral-fixated capsular tension ring and/or capsular tension segment with IOL in pediatric patients with ectopia lentis.
Methods
A computer search of our patient database identified the records of all patients 18 years old or younger with ectopia lentis who received a capsular tension ring and/or a capsular tension segment between January 1, 2009 and March 30, 2013. One patient was excluded because of incomplete follow-up. Patients received either a scleral-fixated Cionni modified capsular tension ring (Morcher, FCI Ophthalmics, Marshfield Hills, Massachusetts, USA; type 1L or 2C) or a scleral-fixated capsular tension segment (Morcher, FCI Ophthalmics, Marshfield Hills, Massachusetts, USA; type 6D or 6E) in conjunction with a conventional unsutured capsular tension ring (Morcher, FCI Ophthalmics, Marshfield Hills, Massachusetts, USA; type 14, 14A, 14C). The Institutional Review Board of the Baylor College of Medicine approved this retrospective observational study.
In patients who were not able to be cooperative for awake measurements, IOL calculations were performed in the operating room using immersion A-scan ultrasonography for axial length measurements and keratometry was performed using a hand-held keratometer. All surgeries were performed under general anesthesia by 1 of 3 surgeons (D.D.K., M.P.W., M.B.H.). Patients were dilated preoperatively with topical cyclopentolate 1%, phenylephrine 2.5%, and tropicamide 1% or with lidocaine gel mixed with gatifloxacin, ketorolac, tropicamide, and phenylephrine drops. Paracenteses were created at 11 o’clock and 5 o’clock for the left eye, and at 1 o’clock and 7 o’clock for the right eye. In some patients, preservative-free 1% lidocaine was injected into the anterior chamber, followed by injection of sodium hyaluronate 3.0%–chondroitin sulfate 4.0% (Viscoat, Alcon, Fort Worth, Texas, USA) or a combination of Viscoat and sodium hyaluronate 1.4% (Healon GV, Abott/AMO, Abbott Park, Illinois, USA). A temporal clear corneal incision was made using a 2.4-mm blade; if necessary, iris hooks (Grieshaber, Alcon, Fort Worth, Texas, USA) were used to stabilize the capsular bag. A continuous curvilinear capsulorrhexis was created using a 27 gauge cystotome and a Utrata forceps. Hydrodissection and hydrodelineation were then performed with balanced salt solution. The cortical lens material was aspirated using bimanual irrigation/aspiration (I/A) with low aspiration parameters, and the posterior capsule was left intact. The capsular bag and anterior chamber were expanded with Healon and a capsular tension device was then inserted through the main incision into the capsular bag. Several combinations of capsular tension rings and/or segments were used: scleral-fixated Cionni modified capsular tension ring, scleral-fixated capsular tension segment with unsutured capsular tension ring, or scleral-fixated capsular tension segment alone. The choice of capsular tension device was determined intraoperatively by the surgeon’s assessment of the degree of zonular weakness. The Cionni modified capsular tension ring and capsular tension segment were positioned with the fixation eyelet anterior to the capsulorrhexis and in the area of maximum zonular weakness. Two scleral stab incisions or scleral flaps were made 2 mm posterior to the limbus in order to suture the eyelet of the capsular tension device to the sclera, with 9-0 polypropylene in 17 eyes and CV-8 Gore-Tex (expanded polytetrafluoroethylene) in 3 eyes.
The capsular bag was reinflated with Healon (Abbott/AMO, Abbott Park, Illinois, USA), and an acrylic IOL was injected into the capsular bag. Patients received an acrylic 3-piece foldable IOL (Tecnis: ZA9003), an acrylic 1-piece foldable IOL (Tecnis: ZCB00; Alcon: AcrySofSN60WF or SN60AT), or an acrylic toric foldable IOL (Alcon: AcrySofSN6AT5 or SN6AT4); selection of lens was the surgeon’s preference. Residual Healon was aspirated using the I/A handpiece, and the lens was centered. The paracentesis and main incisions and the overlying conjunctiva were closed with 10-0 nylon suture. Postoperatively, all eyes were treated with gatifloxacin and prednisolone acetate 1% 4 times daily for the first week, followed by a taper of the topical prednisolone acetate 1% by 1 drop each week for 4 weeks.
All patients received complete preoperative and postoperative ophthalmic evaluations, including age-appropriate assessment of corrected distance visual acuity (CDVA) (Snellen, Allen pictures, HOTV, Alternates), refraction (cycloplegic refraction if not aphakic), slit-lamp examination, intraocular pressure (IOP) measurement (if cooperative in clinic), and dilated funduscopy. All patients underwent examination under anesthesia (EUA), including IOP measurement, prior to the surgical procedure if this information could not be obtained accurately in the clinic. Preoperative and postoperative IOL centration was assessed by the examiner on dilated slit-lamp examination. Subject parameters reviewed included sex; age; diagnosis; relevant medical history; preoperative, postoperative, and most recent CDVAs; length of follow-up; capsular tension ring and/or capsular tension segment model; IOL model; suture material and gauge; presence of amblyopia; intraoperative or postoperative complications; and need for reintervention.
Snellen CDVAs were converted to logarithm of minimal angle of resolution (logMAR) values for statistical analysis. Descriptive statistics and, when possible, the Wilcoxon signed rank test were used to support our findings and to compare subgroups. Significance was defined by a P value of less than .05.
Results
Nineteen eyes of 13 patients (3 female) met criteria for inclusion. Ten eyes had Marfan-associated ectopia lentis, 8 eyes had non-Marfan-associated ectopia lentis (either hereditary or idiopathic), and 1 patient suffered traumatic dislocation of a previously implanted IOL. Detailed individual patient data are given in Table 1 .
| Patient | Age (Y)/Sex | Eye | Diagnosis | Associated Ocular Condition | Surgical Procedure | IOL Model | Suture | MCTR Type | CTR Type | CTS Type |
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 15.8/M | L | Traumatic subluxed IOL a | — | CTS | ZA9003 | P | — | — | 6E |
| 2 | 7.0/F | R | Marfan syndrome | — | PE/IOL/CTS+CTR | SN60WF | P | — | 14 | 6E |
| 6.6/F | L | Marfan syndrome | — | PE/IOL/CTS+CTR | SN60WF | P | — | 14 | 6E | |
| 3 | 11.8/M | R | Familial ectopia lentis | — | PE/IOL/MCTR | SN6AT5 | P | 1L | — | — |
| 11.9/M | L | Familial ectopia lentis | — | PE/IOL/MCTR | SN68C5 | P b | 1L | — | — | |
| 4 | 14.2/M | R | Marfan syndrome | — | PE/IOL/CTR+CTSx2 | ZCB00 | P | — | 14 | 6D |
| 13.9/M | L | Marfan syndrome | — | PE/IOL/CTR+CTSx2 | ZCB00 | P | — | 14 | 6D | |
| 5 | 15.3/M | L | Idiopathic | — | PE/IOL/CTS+CTR | SN60WF | G | — | 14A | 6E |
| 6 | 6.6/F | R | Marfan syndrome | — | PE/IOL/CTS+CTR | ZCB00 | P | — | 14 | 6D |
| 7 | 8.8/M | R | Marfan syndrome | — | PE/IOL/MCTR | SN60WF | P | 2C | — | — |
| 8.8/M | L | Marfan syndrome | — | PE/IOL/MCTR | SN60WF | P | 1L | — | — | |
| 8 | 11.1/M | R | Marfan syndrome | — | PE/IOL/CTS+CTR | SN60WF | P | — | 14A | 6E |
| 9 | 2.9/M | R | Familial ectopia lentis | Amblyopia, glaucoma, X(T) | PE/IOL/CTS+CTR | SN60WF | P | — | 14 | 6D |
| 2.8/M | L | Familial ectopia lentis | Amblyopia, glaucoma, X(T) | PE/IOL/CTS | SN60WF | P | — | — | 6D | |
| 10 | 17.9/M | R | Marfan syndrome | — | PE/IOL/CTS+CTR | ZCB00 | P | — | 14A | 6E |
| 18.3/M | L | Marfan syndrome | — | PE/IOL/CTS+CTR | ZCB00 | P | — | 14A | 6E | |
| 11 | 3.6/F | R | Idiopathic | Amblyopia | PE/IOL/MCTR | SN60AT | G | 1L | — | — |
| 12 | 11.9/M | L | Idiopathic | — | PE/IOL/CTS+CTR | ZCB00 | P | — | 14C | 6D |
| 13 | 5.3/M | L | Idiopathic | Amblyopia, E(T) | PE/IOL/CTS+CTR | SN60AT | P | — | 14 | 6D |
Mean age at the time of original IOL placement was 10.2 years (median, 11.1 years; range, 2.8–18.3 years), and the average length of follow-up was 19.3 months (range, 0.7–46.6 months). Six patients had bilateral surgery and 7 had unilateral surgery. Two patients underwent patching treatment for amblyopia.
All eyes received in-the-bag implantation of a posterior chamber IOL with a scleral-fixated capsular tension ring and/or capsular tension segment. A Cionni modified capsular tension ring was implanted in 5 eyes (26.3%), and a capsular tension segment with an unsutured capsular tension ring was implanted in 12 eyes (63.2%). In 2 eyes (10.5%), capsular tension segment alone was placed. In 1 eye, there was a peripheral extension of the continuous curvilinear capsulorrhexis; owing to the increased risk of capsular tension ring slippage, capsular tension segment alone was placed. In 1 eye, traumatic dislocation of a previously implanted IOL occurred; capsular tension segment alone was placed to minimize unnecessary manipulation of the IOL/capsule complex. Both eyes that received capsular tension segment alone did well during the follow-up period with IOLs remaining centered; both developed PCO, which were successful treated by neodymium–yttrium-aluminum-garnet (Nd:YAG) capsulotomy or pars plana vitrectomy and posterior capsulotomy. All IOLs were well centered. Of the 19 eyes, 11 eyes developed secondary cataract (57.9%), 9 eyes (47.4%) required Nd:YAG capsulotomy, and 3 eyes (15.8%) required pars plana vitrectomy and posterior capsulotomy. Mean time to PCO development from the time of surgery was 1.40 ± 0.38 years. One eye had a recurrence of PCO following Nd:YAG capsulotomy and thus received pars plana vitrectomy and posterior capsulotomy as well. One patient with a Cionni modified capsular tension ring suffered breakage of a 9-0 polypropylene suture after sports-related trauma; the IOL was noted to be slightly subluxated nasally at the time of the examination. The suture was replaced with CV-8 Gore-Tex; the patient has done well since the subsequent surgery, and the IOL has remained centered. There was 1 case each of conjunctival dehiscence, suture exposure (trans-scleral 9-0 polypropylene), and vitreous strand at inferior paracentesis.
Mean CDVA significantly improved from preoperatively (0.58 ± 0.26 logMAR, 15 eyes) to the most recent postoperative follow-up (0.10 ± 0.11 logMAR, 18 eyes) ( P < .001) ( Table 2 ). The CDVA at the final follow-up was 20/40 or better in 18 eyes (94.7%). Four eyes were fix-and-follow prior to surgery, and 3 of the 4 had measurable vision at the most recent follow-up, with 2 eyes measured at 20/40 and 1 eye measured at 20/25. The final patient maintained fix-and-follow at the most recent visit and had no noted complications. Mean preoperative spherical equivalent and astigmatism were −9.56 ± 6.34 diopters (D) (range −22.00 to −0.50 D) and 4.15 ± 3.73 D (range 0.00–11.00 D), respectively ( Table 3 ). Mean spherical equivalent and astigmatism at postoperative week 3 were −1.22 ± 2.34 D (range −7.50 to 1.50 D) and 0.88 ± 0.51 D (range 0.00–2.00 D), respectively.
Patient | Eye | F/U (M) | Preoperative CDVA | Postoperative CDVA | Complications/Comments | ||||
|---|---|---|---|---|---|---|---|---|---|
| 1 M | 3 M | 1 Y | 2 Y | Final F/U | |||||
| 1 | L | 39.4 | 20/40 | 20/40 | 20/20 | 20/20 | 20/20 | PCO-Nd:YAG capsulotomy, prior PE/IOL | |
| 2 | R | 27.6 | 20/200 | 20/30 | 20/25 | 20/40 | 20/20 | 20/20 | PCO-Nd:YAG capsulotomy |
| L | 32.7 | 20/200 | 20/60 | 20/30 | 20/60 | 20/20 | 20/20 | PCO-Nd:YAG capsulotomy | |
| 3 | R | 37.7 | 20/60 | 20/40 | 20/40 | 20/30 | 20/30 | 20/20 | PCO-Nd:YAG capsulotomy |
| L | 37.0 | 20/40 | 20/25 | 20/20 | 20/25 | 20/20 | 20/20 | IOL subluxation, resuturing of IOL, PCO-Nd:YAG capsulotomy | |
| 4 | R | 0.8 | 20/100 | 20/25 | 20/25 | ||||
| L | 3.2 | 20/60 | 20/30 | 20/20 | 20/20 | Transcleral suture exposure | |||
| 5 | L | 0.7 | 20/150 | 20/25 | 20/25 | Conjunctival dehiscence | |||
| 6 | R | 3.9 | 20/80 | 20/40 | 20/40 | Vitreous strand at inferior paracentesis | |||
| 7 | R | 22.5 | 20/100 | 20/40 | 20/20 | 20/20 | PCO-Nd:YAG capsulotomy | ||
| L | 24.1 | 20/160 | 20/60 | 20/40 | 20/25 | 20/25 | PCO-Nd:YAG capsulotomy | ||
| 8 | R | 46.6 | 20/40 | 20/25 | 20/20 | 20/30 | PCO-Nd:YAG capsulotomy | ||
| 9 | R | 23.4 | F&F | 20/150 a | 20/60 b | 20/40 b | 20/40 b | PCO-PPV | |
| L | 25.3 | F&F | 5/300 b | 20/100 a | 20/100 b | 20/40 b | 20/40 b | PCO-PPV, extended capsulorrhexis | |
| 10 | R | 2.8 | 20/50 | 20/30 | 20/20 | 20/20 | |||
| L | 0.9 | 20/60 | 20/32 | 20/32 | |||||
| 11 | R | 27.5 | F&F | 20/125 | 20/125 b | 20/50 b | 20/30 | 20/25 b | PCO-Nd:YAG capsulotomy, PCO-PPV |
| 12 | L | 0.7 | 20/30 | 20/25 | 20/25 | ||||
| 13 | L | 9.7 | F&F | CUSM c | CUSM c | F&F | |||
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