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Managing IOLs in the first year of life
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Round table moderator was Rudolph S. Wagner, MD. Round table participants included Edward G. Buckley, MD; Scott R. Lambert, MD; and M. Edward Wilson Jr., MD.
Rudolph S. Wagner, MD: We are going to discuss case management of a 4-day-old, full-term, healthy newborn, who has been referred to you after the pediatrician noted a cataract in his right eye on discharge from the hospital. On examination, you find a dense unilateral zonular cataract in the right eye with only a peripheral red reflex noted. The left eye is normal, and both eyes have symmetric age-appropriate corneal diameters. Although visual axis is obstructed, indirect ophthalmoscopy can be performed around the opacity, and a normal optic nerve is present. How would you manage a unilateral cataract in an essentially normal-sized eye?
M. Edward Wilson Jr., MD: This appears to be a straightforward, unilateral, fetal nuclear congenital cataract, and three questions must be asked. One concerns timing of the surgery. How soon does this cataract have to come out? Second, what technique should be used to take it out? Third, what method of optical correction is going to work best in this infant?
Regarding timing, to my satisfaction no proof exists in the literature that surgery in the first 2 weeks of life produces better results than surgery at 3 to 6 weeks. I do think evidence exists that the prognosis for good vision may begin to drop if surgery is delayed longer than 6 weeks of age.
For that reason, I often consider letting the child get a little older or a little healthier. I do not want to wait too long, but I often schedule the surgery for these infants at 3 to 5 weeks of age, rather than seeing a 4 day old, and doing surgery at 5 days of age, which I have done. I am not sure operating at that age has any advantage; the surgery becomes a little harder. In addition, the anesthesiologists are happier if you delay just a few weeks.
Some might occlude both eyes while awaiting surgery. I have never done that, so I do not really know if that helps.
The second is the technique. I think most surgeons who operate on small babies would do a primary posterior capsulectomy and an anterior vitrectomy in this type of case. A mechanized surgical approach that would open the anterior capsule, take the cataract out, open the posterior capsule, and remove the vitreous works best.
The third is optical correction, and that depends a bit on the parents. If I am convinced that the parents can afford contact lenses, that they will be compliant, and that they will come back immediately when the contact lenses are displaced or lost, I might elect to do a lensectomy; leave the child aphakic, fit him or her with a Silsoft (Bausch & Lomb, Tampa, Fla.) silicone lens, and follow up with changes in the contact lenses as often as needed. However, I would quickly move to a secondary implant if contact lens failure occurs. I do not have much tolerance for uncorrected aphakia, and if we wait too long, with too many periods of time with uncorrected aphakia, we lose ground in the treatment of amblyopia. I may move a bit more quickly to refit the contact lens, and if that does not work, go to a secondary implant fast.
If a secondary implant became necessary, I would try to get that secondary implant within the capsule bag. And I prepare for that possibility at the initial surgery by leaving a fair amount of capsule remnant — the central opening should be about 4 mm. To keep that 4-mm opening from closing, you would do an adequate vitrectomy. I like to leave the anterior capsule and the posterior capsule open and the same size, so you may be able to get back into that doughnut of capsular bag and put a secondary lens in the bag.
If parents are not compliant, or the family is covered by Medicaid or is indigent — and I come from a state where 60% of the babies born are covered by Medicaid — I do not do well. In my practice, patients with unilateral congenital cataracts in the Medicaid population have worse than 20/200 vision virtually every time with the standard approach. In that group, I have been implanting IOLs, because in a unilateral setting, I do not believe I can get good vision otherwise.
With patients in those families, I operate in that 3-to-6-week window, placing an IOL in the bag, which I call the permanent lens. Then, I place a foldable acrylic temporary lens in the sulcus. I now have 13 eyes in which I have piggybacked lenses. To date, I have 10 infant eyes with piggyback lenses in place to try to get the child to emmetropia early, and to stay on the myopic side until the eye has grown sufficiently.
At that time, I remove the temporary lens, which is in the sulcus. To date, depending on the size of the eye, those lenses have been from 7 D to 14 D, but that leaves me with an in-the-bag lens that is a power that is not going to lead to as large a myopia when the child is older.
I use this approach for situations where I think the contact lens might fail and, so far, the eyes have tolerated the implants very well. I have been following them for up to 1 year. I have had one re-operation for cortex that reproliferated into the pupillary axis, but which aspirated out easily.
Edward G. Buckley, MD: Every time I hear Ed Wilson talk, I learn something new. Piggybacking in small eyes? Unbelievable!
Basically, my routine is not much different from Dr. Wilson’s. I might argue a bit that, as far as timing is concerned, I would try to move as quickly as possible, only because some downtime occurs with postoperative care and getting the contact lens on.
I do not think it is an emergency situation, but I like to operate within 2 or 3 weeks. I do not like to wait as long as 5 or 6 weeks, because before you know it, it is 7 or 8 weeks and if a problem occurs anywhere along the line, you have to deal with that, as well.
As far as the technique is concerned, I think most of us use a two-port technique, using the vitrector instrument to do anterior capsulotomy and aspirate the cortical material, and then do a posterior capsulotomy. I like to do a relatively healthy opening, because I have had the unpleasant experience of some of those openings closing.
As far as the correction is concerned, I think the Silsoft contact lens is excellent. It is well tolerated, and in these very young infants, you have few problems. My experience has been that you start to have problems with contact lenses when the kids get to be 6 to 9 months old, and they start rubbing them out. If you can get the kid to 6 months, I think even a secondary IOL at that point is a much easier operation than a primary IOL.
And although this really young group does have its problems with lenses, this is my basic approach. If we are having trouble with the contact lenses, I do not hesitate to put a secondary lens in.
We should not forget aphakic spectacles, especially in this age group. If they are having problems with the contacts, make sure they have the aphakic correction as well; if the contact is out, the parents can slip the kid into aphakic spectacles, especially while they are doing the patching, which is definitely going to be necessary in this age group.
One of the things that may help us get over some of the problems we have with these very young infants and IOLs is a drug delivery system in the wings that can be implanted into the anterior chamber. There is an intense inflammatory response, and we tend to use a great deal of drugs, both systemically and topically. We are not too far off from slipping little pellets into the anterior chamber to give high doses of corticosteroids to the problem area.
Scott R. Lambert, MD: There are two ways I might approach this case. One is what I do now in my clinical practice. The second is what we do as part of the clinical trial we are currently organizing.
In terms of my current practice, I would want to do the surgery fairly soon. In Georgia, as in South Carolina, we have a lot of babies covered by Medicaid. Since it takes at least 10 working days in Georgia to obtain approval from Medicaid for surgery, we generally have to wait at least 2 weeks before we can perform surgery.
I also do a lensectomy using a two-port technique. I leave enough of the posterior and anterior capsule intact so that they will support a secondary IOL later on. I treat them with rigid gas-permeable (RGP) contact lenses and have been doing this for the past 10 years. RGP contact lenses have the advantages that they are less expensive than Silsoft lenses, and they come in a wider range of powers. Many of these children require a power greater than 32 D, which is the highest power available with a Silsoft lens. Generally, I measure the keratometry readings intraoperatively; the following day, a contact lens technician fits the contact lens and they are usually dispensed a few days later.
One of the things that we are looking at is the possibility of treating children like this with primary IOL implantation. In my opinion, it would be premature for people to start implanting IOLs in babies, unless they are part of a clinical trial.
I agree with Ed Wilson that a subset of children does poorly with contact lens correction. In fact, a group of us pooled all of our visual acuity data and found that only about 20% of the children whom we had treated in the past with unilateral congenital cataracts ended up seeing 20/40 or better. About one-half of these children see 20/200 or worse in their aphakic eyes. Although in some situations these children may have a good visual outcome, this is the exception rather than the rule. Only the child with a parent who can keep up with the contact lenses and the patching are likely to achieve a good visual outcome. If they have difficulty with contact lens wear, I move quickly to implant a secondary IOL. If they are doing well with the contact lens, I prefer to wait to implant the secondary IOL until they are 5 or 6 years of age to minimize their myopic shift.
As for patching therapy, many regimens have been recommended. In the pilot stage of our clinical trial, we have been patching the infants half time. We give parents the option of patching every other day, or half time every day.
Dr. Wagner: One of the advantages of having your three views is that you all have a great deal of experience treating this condition, especially when we are discussing IOLs.
People routinely ask me what is the youngest age you can implant an IOL. We can see from this discussion how that question must be answered.
What frequently happens is that surgeons who have been doing cataract surgery on adults or who have finished their residency feel proficient in implanting IOLs and want to perform this procedure in a young child.
Tell us what you feel is the most unique problem encountered when converting from cataract surgery on an adult — 8 or 9 years of age and older — to an infant, especially within the first year of life.
Dr. Wilson: The first obvious thing is that the eye is much smaller and the scleral rigidity is so reduced that, when the eye is opened, you get instability in the chamber, and scleral collapse — all things not dealt with in adult populations.
The capsule, especially the anterior capsule, is different in children; it handles differently. I think the manual continuous tear capsulorrhexis is the best edge, the gold standard, but very experienced phacoemulsification surgeons have told me they were humbled the first time they tried to operate on a child.
Someone who is just converting to pediatric cataract surgery should stay with the common practice of putting IOLs in patients older than 2.
After age 2, IOL implantation may be the most common way of dealing with the correction of aphakia in 2000. We are not, as a group, completely convinced that IOLs, especially in the first year of life, are going to become the standard. We are investigating it — and it should be investigated — but it should be examined carefully. I do not think surgeons who only rarely implant a child should deal with that age group.
In the Journal of Cataract and Refractive Surgery, we reported our experience at the Storm Eye Institute with implants in the first 2 years of life. We had an aged-matched group of aphakic patients, and we did not find an increase in re-operation rates.
I know in the pilot data that Scott Lambert put together — and we all contributed cases to that — quite a high re-operation rate was noted as we operated closer to birth.
My series is up to 27 eyes and 19 patients operated before their first birthday. I am watching those patients very carefully. Four patients needed re-operations for various reasons, but my young aphakic patients need re-operations too.
Dr. Wagner: What were some of the reasons for the re-operations?
Dr. Wilson: Cortex reproliferation. Without an IOL in place, the Soemmering’s ring seals better. The younger the child is when you operate, the more the lens epithelial cells produce cortex, and you get a Soemmering’s ring.
If the capsule edge is sealed to one another, it is trapped; it does not get into the pupillary space. In some of our patients, when an IOL is in place, the capsule does not seal as well to the IOL as it would to itself. Some of the new cortex from active lens epithelial cells occasionally can fluff out into the pupil, and although it aspirates very easily, nonetheless, it can end up in the pupil.
Dr. Buckley: A few things come to mind, with regards to how surgery in the pediatric population is a little different, even for the experienced nonpediatric surgeon. Certainly, the soft sclera is one. The eyes collapse at will.
Most ophthalmic surgeons do their surgery through a tunnel incision, or a clear cornea incision. The infusion and the instrument aspiration are all together, which makes for a fairly large opening. Doing it that way, you decrease the infusion rate just a little bit on your foot pedal, and everything starts collapsing. This makes for a rough surgical procedure.
Closing the wound is a little different in children. The adult patient usually is not going to manipulate the eye that much. He or she certainly is not going to squeeze and push on the eye, so you can get away with not putting in a suture.
Because of the nature of the sclera, those scleral wounds are difficult to self-seal in a child, whereas with an adult, a tunnel incision seals beautifully. In a child, the tunnel incision requires a suture. The two-port technique that most of us use, even when we are putting in lenses, has some benefit by keeping the infusion steady and doing the aspiration through a smaller opening.
The other difference affects the technical aspect. Most phaco surgeons bring the lens to the instrument. You hold the phaco tip in a relatively standard position, and you bring the material to the instrument. In kids, it is just the opposite. You do not bring anything to the instrument. Rather, you get the material with the instrument, which is a different approach, and if you are in a long tunnel, it is often much harder to get the peripheral cortex out, because you cannot manipulate it very well. Again, a two-port technique helps considerably in that maneuver.
Dr. Wagner: Do you still prefer to use a pars plana approach to remove the posterior capsule?
Dr. Buckley: If I am not putting a lens in, I do it all through the anterior limbal incision. However, if I am putting a lens in, I leave the posterior capsule until the lens is in position and the eye is sealed up. Then, I go pars plana to remove the posterior capsule. That is a safer way to do it. You can always stop if a problem occurs, without compromising your surgery.
Dr. Lambert: I have had some difficulty with the pupil constricting intraoperatively when implanting IOLs in infants and have found prostaglandin inhibitors helpful in preventing this from happening. In addition, I use 5 units per cc of heparin in the infusion solution, which reduces the amount of fibrin in the anterior chamber postoperatively.
This is not a common condition. Dr. Jugnoo Rahi has found the incidence of congenital cataracts in the United Kingdom to be 2.5 per 10,000 live births. Dr. Rahi found that about one-third of these cataracts are unilateral, and the disappointing statistic is that one-third of these cataracts are not even detected until these children are 1 year of age or older. In the United States, only about 300 children are born each year with a unilateral congenital cataract. As a result, many surgeons will only operate on one or two patients a year with this condition. These patients may be better off being cared for at regional centers that treat this condition more often.
Dr. Buckley: I do think there is a major difference in lens implantation between kids who are 2 or 3 years of age and kids who are younger. The younger you go, the more complicated the whole process becomes.
Dr. Wilson: The reason Scott Lambert suggested that we study unilateral congenital cataracts first, with this trial, is that our standard of treatment is really not all that good. We all remember our one success, but the average is probably closer to 20/100 or 20/200, even with an enormous amount of effort. The study should contain a relatively small number of centers. The trial would be in cases up to 6 months of age only, and will include a contact lens arm and a single IOL arm.
I sometimes get patients who are diagnosed late, and some of my piggyback lens cases have been 6 months old. With unilateral congenital cataracts, where the situation is much more desperate, I try to improve my early detection rate so I can enroll in the study.
Dr. Wagner: Are you actively recruiting centers? What is the current status?
Dr. Lambert: We are finishing up the pilot studies and plan to apply for a U-10 clinical trial grant next year. We are hoping to begin the study in 2001.
We are currently trying to determine how many cataracts each participating center sees each year and determine how these patients have done historically. We also are studying some cases where IOLs were implanted, and trying to get some pilot data on the types of complications these patients are experiencing.
We still have many questions about the best lens power to put in these eyes and the best type of IOL to implant. We hope that this clinical trial will answer many of these questions.
Dr. Wagner: I think the pediatric ophthalmologist must hear that even people like yourselves, who do more cases than most, are not rushing this procedure.
Dr. Wilson: How young [of a patient would I operate] is such a bad question. It depends on the situation. It depends on the child, the family and when the detection occurred.
Most of us will state the youngest patient that we have done, but we may not want to repeat that every time we see someone. Therefore, if I give a specific age, I am sure to be clear that this is the youngest I will do in a dire situation only.
Dr. Wagner: That question usually comes from a surgeon inexperienced in pediatric cataract surgery who wants some positive feedback that it is okay to do it, and that may not necessarily be right.
Dr. Wilson: No one should be criticized for choosing to make a child younger than 2 aphakic. I would really hate to see surgeons criticized because they chose not to put in an IOL. We are investigating this procedure in order to obtain some measurable outcomes.
For Your Information:Reference:
- Rudolph S. Wagner, MD, can be reached at Children’s Eye Care Center of New Jersey, 495 N. 13th St., Newark, NJ 07107; (973) 485-3186; fax: (973) 497-5674; e-mail: wagdoc@aol.com. Dr. Wagner has no direct financial interest in any of the products mentioned in this article, nor is he a paid consultant for any companies mentioned.
- Edward G. Buckley, MD, can be reached at Duke Eye Center, P.O. Box 3802, Durham, NC 27705; (919) 684-3957; fax: (919) 684-6096; e-mail: buckl002@mc.duke.edu. Dr. Buckley has no direct financial interest in any of the products mentioned in this article, nor is he a paid consultant for any companies mentioned.
- Scott R. Lambert, MD, can be reached at Emory University Eye Center, 1365 Clifton Road, Atlanta, GA 30322; (404) 778-3420; fax: (404) 778-4143; e-mail: slamber@emory.edu. Dr. Lambert has no direct financial interest in any of the products mentioned in this article, nor is he a paid consultant for any companies mentioned.
- M. Edward Wilson Jr., MD, can be reached at Storm Eye Institute, Medical University of South Carolina, 171 Ashley Ave., Charleston, SC 29425-2236; (843) 792-7622; fax: (843) 792-1166; e-mail: wilsonme@musc.edu. Dr. Wilson has no direct financial interest in any of the products mentioned in this article, nor is he a paid consultant for any companies mentioned.
- Hutchinson AK, Wilson ME, Saunders RA. Outcomes and ocular growth rates after intraocular lens implantation in the first 2 years of life. J Cataract Refract Surg. 1998;24:846-852.
- Rahi JS, Dezateux C. National cross sectional study of detection of congenital and infantile cataract in the United Kingdom: role of childhood screening and surveillance. BMJ. 1999;318:362-365.
- Robb RM, Mayer DL, Moore BD. Results of early treatment of unilateral congenital cataracts. J Pediatr Ophthalmol Strabismus. 1987;24: 178-181.
- Wagner RS, Nelson LB. Problems in pediatric cataracts—IOL implantation. J Pediatr Ophthalmol Strabismus. 1997;34:332.
- Wright KW. Pediatric cataracts. J Pediatr Ophthalmol Strabismus. 1987;24:178-181.
Editor’s note:
This article originally appeared in the Journal of Pediatric Ophthalmology & Strabismus, a SLACK, Incorporated publication.