Pronounced ectopic lenses in children can cause deprivation amblyopia.1 In addition, complete dislocation of the crystalline lens into the anterior chamber or vitreous can cause serious complications such as secondary glaucoma or retinal detachment.2 Therefore, surgery may be the only alternative in cases with ectopia lentis. In the past, surgical intervention has been considered extremely dangerous, leading to many complications and a poor visual outcome.3 With modern surgical techniques, lensectomy in eyes with ectopia lentis has become relatively safe.4,5
The refractive correction of the aphakia in children can be problematic. Contact lenses are frequently used after lensectomy to correct aphakia.6 However, they are associated with such problems as infection and vessel ingrowth in the cornea.7–9 Implanting an intraocular lens (IOL) in the capsular bag in the eye of a child older than 1 to 2 years10,11 is a safe and successful procedure. The possibility of implanting an IOL in a child's eye without a capsular bag has been questioned. The possibility of using anterior chamber lenses to correct aphakia in children has been raised. However, we have refrained from doing so because of the many complications reported after implantation of this kind of IOL.12,13
In this study, we did a long-term follow-up of 21 eyes of children with scleral-fixated IOLs in which the polypropylene knot was rotated, buried, and covered with conjunctiva.
Patients and Methods
This retrospective study was based on measurements obtained from 21 eyes of 3 girls and 10 boys. Seven eyes had Marfan's syndrome, 7 essential lens dislocation, 2 perforation with lens injury, and 5 spherophakia (Table 1). The scleral-sutured sulcus fixation was performed at the same time as the lensectomy in 16 eyes.
Preoperatively, pupils were dilated by a topical combination of cyclopentolate 0.85% and phenylephrine 1.5%. The operations were performed by the same surgeon (C.Z.). Lensectomy was done with a limbal approach in all cases. A 3.2 scleral pocket incision was made, and a small anterior continuous capsular opening was created with the aid of sodium hyaluronate (Healon GV®) 14 mg/mL.
After hydrodissection, irrigation/aspiration of the nucleus and cortex was done in the capsular bag. With Healon GV in the anterior chamber, the capsular bag was removed, at least from the pupil area. The wound was enlarged to 7.0 mm for scleral-sutured sulcus fixation of an Alcon CZ70BD poly(methyl methacrylate) (PMMA) IOL in 14 eyes (February 1994 to October 1995) or a heparin-surface-modified PMMA IOL in 7 eyes (Pharmacia & Upjohn 722Y, October 1995 to June 1996). The IOL power was based on axial length and K-readings performed before surgery. The IOL was selected to achieve emmetropia in older children; however, in younger patients, undercorrection was the goal to allow for eye growth. The IOL was sutured to the sclera 1.5 to 2.0 mm from the limbus with a 10-0 polypropylene suture with a straight needle. The knots were rotated, buried in the scleral bed, and covered with conjunctiva. No scleral flaps were used.14 The wound was closed with continuous 10-0 nylon sutures.
No eyes were patched after surgery. A standard postoperative dosage of topical dexamethasone (1 mg/mL) was given 3 times daily during the first postoperative week, twice daily for 2 to 4 weeks, and once daily for 4 to 6 weeks. Some patients were given cyclopentolate 1% for several weeks postoperatively.
Some weeks after surgery, retinoscopy was performed and the children were equipped with bifocal spectacles; in some cases, 1 eye was patched. All children were examined with a slitlamp 9 to 33 months after IOL implantation. Static refraction was measured, and visual acuity was tested using Teller-Dobson acuity cards or the Snellen optotype method. Compression tests of the 2 IOLs were performed at 11.0 mm compression according to the draft of ISO standard 11979-3.15
Mean patient age at the time of the study was 5.8 years ± 2.6 (SD) (range 1 to 11 years). Follow-up was between 9 and 33 months.
In all cases, mild postoperative inflammation occurred the day after surgery but resolved within 1 week. Two eyes had a mild fibrinoid reaction in the anterior chamber, and 4 eyes developed posterior synechias and cells on the IOL surface. No eye had evidence of suture exposure (Figure 1). No retinal complications (e.g., retinal detachment, cystoid macular edema) were found. In 2 eyes, the optic of an Alcon CZ70BD subluxated into the anterior chamber (Table 2, Figure 2). The subluxation was treated successfully in both cases with topical pilocarpine chloride 4% (Pilokarpin®) (Figure 3). In 1 of these cases, the IOL had rotated 180 degrees, probably after blunt trauma to the eye.
Best corrected visual acuity improved in most eyes. One child had the same visual acuity before surgery with contact lens correction as with a sutured IOL postoperatively. Four eyes had deprivation amblyopia and no improvement in visual acuity after surgery. One child was developmentally delayed, and measurement of the visual acuity could not be obtained (Table 1, Figure 4).
A comparison of postoperative complications between IOL models CZ70BD and 722Y is shown in Table 2. The difference between the 2 lenses in angle contact to hole at 11.0 mm compression is shown in Figures 5 and 6.
Our study found that transscleral fixation of a posterior chamber IOL is an acceptable alternative to correct aphakia in young eyes. This finding agrees with those in earlier studies of adults.14,16 Many cases of endophthalmitis caused by suture erosion were reported earlier.17 In our study, all knots were rotated, buried in the scleral bed, and covered with conjunctiva. No suture erosion or endophthalmitis occurred in our study or the study that first described this surgical technique of burying the knots in the scleral bed.14
Subluxation of the optic into the anterior chamber occurred in 2 eyes, both with the Alcon CZ70BD IOL. To our knowledge, this complication has not been reported in cases with transscleral fixation of a posterior chamber IOL. However, in adolescence, the pupils are at their largest.18 Between the ages of 5 and 15 years, many pupils have a diameter of 7.0 mm or more in darkness, and the optic diameter of the 2 IOLs used in our study were 6.5 and 7.0 mm. In contrast, it is rare to find a pupil diameter of 7.0 mm or more in patients older than 40 years.18
In 1 case in our study, an IOL rotated 180 degrees, probably because of asymmetrical suture configuration at the 2 haptics.19 Furthermore, the dimensions of the CZ70BD IOL probably make the lens easier to rotate. The contact between haptic and sulcus is more local, making it more suitable as an axis of rotation. The overall diameter is smaller, giving less support in a large eye. Also, the haptic angulation is smaller, providing less resistance to lens rotation.
The optical correction of aphakia in children continues to be a significant challenge to parents and ophthalmologists. Although children can wear spectacles, many will not accept them and thus they are impractical. Contact lenses are frequently used after lensectomy to correct aphakia in children.6,20 However, they are associated with many problems. For example, cleaning and handling the lenses are time consuming for the parents. Contact lens cases have been found to be contaminated with bacteria, fungi, and protozoa.7 Not surprisingly, keratitis8 and even corneal perforation are reported with contact lens wear.21 Corneal vascularization is usually found in eyes with contact lenses, particularly in those with continuous-wear soft lenses.9 Contact-lens-induced hypoxia is likely a risk factor for corneal vascularization.
In conclusion, our results suggest that transscleral fixation of a posterior chamber IOL in children with ectopia lentis is an acceptable procedure to correct aphakia.
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