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Multi-national panel lends guidance in pediatric cataract cases

A panel of pediatric surgeons present preferred approaches to a newborn patient with occlusive cataract and relative microphthalmia.

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Age and surgery

Dominique Brémond-Gignac, MD: In patients with congenital occlusive cataract and microphthalmia, at what age do you perform cataract surgery?

Elie Dahan, MD: The best time to perform surgery is around 3 months of age but not before the baby has reached 3 kg (approximately 7 lbs). Although it is possible to perform surgery at 2 months, the risk from anesthesia is higher, and the surgery can be more difficult. It is important to note that before 3 months of age, the baby is asleep many hours a day so the visual deprivation is less important.

Surgery can still be performed up to 6 months of age with good results. After 6 months of age, visual deprivation nystagmus can develop. Thus, the window when surgery is indicated is between 3 and 6 months.

In bilateral congenital cataracts, simultaneous bilateral surgery is indicated. This includes cataract aspiration plus, electively, posterior capsulotomy plus, electively, generous anterior vitrectomy. The reason for bilateral surgery is to reduce the anesthetic risk and to prevent preference amblyopia.

Claude Speeg-Schatz, MD: I prefer to perform congenital cataract surgery before the child reaches 3 months of age.

Dr. Brémond-Gignac: For the best results, I prefer to operate before 3 months of age, but when cataract is diagnosed early it can be done by 1 month of age in the more affected eye and 1 week later in the second eye if the condition is bilateral.

I prefer early surgery because of the neuronal plasticity and for the development of binocular and color vision.

M. Edward Wilson, MD: For a newborn with a visually significant cataract, I operate between 4 and 6 weeks of age. An excellent article by Birch and Stager in 1996 provided evidence that no loss of visual prognosis occurs with delays in surgery up to the sixth week of life. We no longer operate in the first week or two of life, but we try not to delay past the sixth week in unilateral or the 10th week in bilateral neonatal cataract cases.

Technique

Dr. Brémond-Gignac: What type of surgical technique do you use — a posterior or an anterior approach?

Dr. Dahan: I prefer a limbal approach (anterior) in order not to disturb the retinal pigment epithelium. The posterior approach (pars plana lensectomy and vitrectomy) may sound elegant but can cause late retinal detachment because of the seeding of RPE cells in the vitreous and vitreous incarceration in the wound.

Dr. Speeg-Schatz: I use a corneal approach through a limbal incision with an anterior rhexis. I employ a posterior approach when possible or phakophagia though the pars plana mostly when there is pupillary seclusion.

Dr. Brémond-Gignac: For microphthalmia, I prefer a posterior approach with pars plana lensectomy and anterior vitrectomy with a posterior capsulorrhexis because of the lack of inflammatory reactions with this type of incision.

Dr. Wilson: I prefer an anterior approach to the cataract. If no IOL is to be placed, I perform the surgery using a bimanual technique through two 1-mm incisions using a 20-gauge Alcon/Grieshaber irrigation cannula (170.01) and a 20-gauge vitrectomy handpiece attached to the Alcon Accurus machine.

A vitrectorrhexis, lens aspiration, posterior vitrectorrhexis and anterior vitrectomy can all be done without having to remove the instruments from the eye. In newborns, if an IOL is implanted, I perform a posterior capsulectomy and anterior vitrectomy through the pars plana after the IOL has been implanted into the capsular bag.

Implantation

Dr. Brémond-Gignac: At what age and at what point, primarily or secondarily, do you insert an implant?

Dr. Dahan: I insert a pediatric IOL at the first surgery (at 3 months or whenever the child arrives at surgery) in unilateral congenital cataracts only. In bilateral congenital cataracts, there is no moral justification to implant an IOL near birth because there is no danger of rivalry amblyopia.

Furthermore, when bilateral IOL implantation is done in a baby, invariably there is a difference in the success of surgery between the two eyes. This difference causes amblyopia of the less successful eye, which means losing an eye. It is, therefore, easier to aspirate the two cataracts with elective vitrectomy and to wait 2 years until the eye has reached near-adult size for performing a secondary implantation of an IOL in the sulcus. During the first 2 years, glasses can adequately correct the aphakia (despite the temporary cosmetic problem), and it is a good way to keep close follow-up with the child.

Dr. Speeg-Schatz: I normally implant at around 10 months if the cataract is unilateral, 2 years if bilateral.

Dr. Brémond-Gignac: Implantation should occur 3 months after the lensectomy in unilateral cataract surgery, also in cases of microphthalmia, and at 2 years in bilateral cataracts, but this can be adjusted.

Dr. Wilson: I am comfortable implanting at any age and nearly always do so when the surgery is done after the first birthday. In infants, I discuss the options of an IOL now vs. an IOL later (aphakia initially followed by a secondary IOL at age 3 to 5 years or later) and make the decision with the parents’ input.

IOL preferences

Dr. Brémond-Gignac: What IOL styles do you prefer for these patients?

Dr. Dahan: The most important aspect is the overall size of the implant rather than the material. The implant should preferably be a pediatric IOL with an overall diameter of 11 to 12 mm according to the size of the eye. As for the material, PMMA has the longest safety record in the human eye. Second best are the hydrophilic IOLs, and third best are the hydrophobic acrylic IOLs. Silicone IOLs should not be implanted in children’s eyes because of their poor biocompatibility.

Dr. Speeg-Schatz: I use PMMA in children with congenital cataract.

Dr. Brémond-Gignac: I use PMMA, 12 mm length, 6 mm diameter, surface-modified if possible, for unilateral cataracts in infants.

Dr. Wilson: I use acrylic implants in children. I have found the single-piece Alcon AcrySof IOL to be an ideal lens for children because it can be injected into even a small capsular bag with precision and it unfolds slowly. It also adapts to any size capsular bag without undue capsular stretch.

Calculating power

Dr. Brémond-Gignac: How do you perform the power calculation for the implant?

Dr. Dahan: In neonates and up to 2 years of age, I suggest implanting 80% of the power for emmetropia (the calculated power minus 20%). After 2 years of age, I use 90% of the power for emmetropia (minus 10%). After 12 years of age, I suggest 100% of the power for emmetropia.

It is extremely important to do biometry of both eyes in cases of unilateral congenital cataract in order to detect relative microphthalmia and to give the full cycloplegic refractive correction of the sound eye in order to reduce anisometropia.

Dr. Speeg-Schatz: In these cataracts operated on before 1 year, I undercorrect by 3 D or 4 D according to familial ametropia.

Dr. Brémond-Gignac: I suggest minus 20% of the calculated power for children under 2 years old and minus 10% of the calculated power for children under 6 years old. But this must be modulated with the degree of microphthalmia and the evolution of the visual deprivation in terms of acquired myopia.

Dr. Wilson: In infants I perform immersion A-scan ultrasound axial length and keratometry measurements under anesthesia at the time of cataract surgery. I use the Holladay formula to calculate the IOL power. I aim for a postoperative refraction of +8 when less than 8 weeks of age and +6 for ages 2 months to 6 months.

If glasses compliance is expected to be poor, then I piggyback two IOLs — one in the capsular bag “permanent” and one in the ciliary sulcus “temporary.” With piggyback IOLs I aim for emmetropia and manage increasing myopia rather than decreasing hyperopia. The temporary sulcus IOL is removed at age 2 to 4 years when sufficient eye growth has occurred.

Amblyopia

Dr. Brémond-Gignac: What type of occlusion or atropine therapy do you prescribe for amblyopia?

Dr. Dahan: From the time of surgery until age 5 years, the sound eye has to be patched daily for half of the waking hours of the child. Between 5 years and 8 years of age the patching time is reduced to 3 to 4 hours per day. (The patching should not be done during school time.) Between 8 years and 12 years, 1 hour a day is sufficient to keep the vision already achieved. Patching can be augmented by atropine penalization. In noncompliant patients, hospitalization of the child with his mother or guardian during a long weekend can be of great value to initiate and enforce good amblyopia treatment.

Dr. Speeg-Schatz: If the cataract is unilateral, occlusion of the good eye is prescribed 80% of the waking time; if the cataract is bilateral, total optical penalization is alternated with eyeglasses.

Dr. Brémond-Gignac: For unilateral cataracts, I prescribe occlusion of the normal eye 50% of the waking time with control of the fellow eye.

Dr. Wilson: For amblyopia treatment in the infant, I patch the normal eye 1 hour per day per month of age. For example, 2 hours per day at 2 months of age and 5 hours per day at 5 months – up to a maximum of 8 hours per day in the first year of life. I monitor the patching result using various visual fixation techniques such as the base-down prism fixation test.

For Your Information:

• Dominique Brémond-Gignac, MD, can be reached at the Department of Ophthalmology, Robert Debre Hospital, 48 Blvd. Serurier, Paris 75019 France; +33-1-40-03-22-10; e-mail: dominique.bremond@rdb.ap-hop-paris.fr. Ocular Surgery News was unable to confirm whether Dr. Brémond-Gignace has a direct financial interest in the products mentioned in this article or if she is a paid consultant for any companies mentioned.
• Elie Dahan, MD, can be reached at the Department of Ophthalmology, University of the Witwatersrand, Johannesburg, South Africa; e-mail: dahaneli@mweb.co.za. Dr. Dahan has no financial interest in any of the products mentioned.
• Claude Speeg-Schatz, MD, can be reached at Strasbourg University Eye Hospital, Strasbourg, France; e-mail: claude.speeg@chru-strasbourg.fr. Ocular Surgery News was unable to confirm whether Dr. Speeg-Schatz has a direct financial interest in the products mentioned in this article or if he is a paid consultant for any companies mentioned.
• M. Edward Wilson, MD, can be reached at Storm Eye Institute, 167 Ashley Ave., Charleston, SC 29425 U.S.A.; +1-843-792-7622; e-mail: wilsonme@musc.edu. Dr. Wilson has no financial interest in any of the products mentioned.

Reference:

• Birch EE, Stager DR. The critical period for surgical treatment of dense congenital unilateral cataract. Invest Ophthalmol Vis Sci. 1996;37(8):1532-1538.