Should We Aggressively Treat Unilateral Congenital Cataracts?
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1120 Br J Ophthalmol 2001;85:1120–1126 Br J Ophthalmol: first published as 10.1136/bjo.85.9.1120 on 1 September 2001. Downloaded from CONTROVERSIES IN OPHTHALMOLOGY (Series editors: Susan Lightman and Peter McCluskey) Should we aggressively treat unilateral congenital cataracts? David Taylor, Kenneth W Wright, L Amaya, L Cassidy, K Nischal, Isabelle Russell-Eggitt View 1 Kenneth W Wright I do believe that visually significant congenital occurs. Even so, one might argue that this is cataracts should be treated aggressively with still only a “spare eye,” and that it is impossible early surgery! However, I advocate surgery to get the eyes to work together. only if the cataract is truly visually significant. Historically, patients with unilateral con- Small partial cataracts can often be treated genital cataracts had a very poor prognosis for with close observation or pupillary dilatation, obtaining binocular fusion and many studies possibly with part time occlusion therapy. have reported that 100% of patients with unilateral congenital cataracts develop strabis- mus.23However, in separate papers published Early surgery for unilateral congenital in 1992, Wright et al4 and Gregg and Parks5 cataracts—why? reported that good visual acuity, straight eyes, Some might protest that visual results are and good binocularity with stereopsis is possi- notoriously poor after surgery for a unilateral ble in patients with unilateral congenital congenital cataract. Why drag the family and cataracts. The key to the excellent outcomes child through all the turmoil required for the was the way the patients were treated. treatment of infantile cataracts for, at best, a Firstly, the patients had very early surgery “spare eye”? The answer is simple: two eyes are and immediate placement of an aphakic better than one. Two eyes give us a larger field contact lens usually by 4 weeks of age. The of view, a reserve if one eye is lost, and that case reported by Gregg and Parks5 had cataract wonderful trait of binocular vision with ster- http://bjo.bmj.com/ surgery with contact lens placement by the eopsis. Sure, you can get along with one eye but second day of life and ended up with 50 if there is any reasonable possibility for obtain- ing two functioning eyes, then we should go for seconds of arc of stereoscopic acuity and visual acuity of 20/25 in the aphakic eye. In the paper it! 4 I have both a personal and a professional of Wright et al, five of 13 (38%) patients with interest in unilateral cataract as my sister had unilateral congenital cataracts operated early technically successful unilateral congenital developed both good visual acuity and binocu- lar fusion. In both the Wright 4 and the on October 1, 2021 by guest. Protected copyright. 1 2 et al cataract surgery at 2 ⁄ years, followed by three 5 strabismus surgeries. She had a childhood of Gregg and Parks studies, extended wear being teased, an adult life of concern for her contact lenses were used not aphakic specta- one good eye and corrected vision of counting cles. Unilateral aphakic spectacles just do not fingers in the aphakic eye. do the job, as infants will never wear them full time and the unilateral magnification causes aniseikonia that disrupts binocular fusion. Can we realistically expect good visual Intraocular lenses have the theoretical advan- outcomes in children who have unilateral tage of providing a constant clear retinal image congenital cataracts? but early experience in newborns has been dis- With aggressive treatment the visual prognosis couraging, and they are not the standard of Pediatric is good, and there is even the possibility of care in infants. For me, optimal treatment is Ophthalmology obtaining binocular vision with stereoscopic early surgery and the contact lens placement Research and 1 Education acuity. Birch and Stager reported that a mean by 2 weeks of age. Cedars-Sinai Medical visual acuity of 20/60 (range 20/800–20/30) Secondly, both studies used immediate full Center, Cedars-Sinai was achieved if surgery was performed before 2 time binocular light occlusion when the Medical Towers, 8631 months of age, whereas surgery after 2 months cataract was first identified. Binocular occlu- West Third Street, of age resulted in poor visual acuity, ranging sion was continued until a clear retinal image Suite 304 E, Los from hand movements to 20/160. Thus, the was restored (that is, cataract surgery per- Angeles, CA 90048, USA prognosis for obtaining good visual function is formed and the contact lens placed—usually K W Wright possible if surgery is performed early, but hor- within 1 week). Binocular light occlusion or director rible if done late after irreversible amblyopia bilateral patching has been shown to preserve www.bjophthalmol.com Should we aggressively treat unilateral congenital cataracts? 1121 Table 1 Patching scheme for unilateral congenital Br J Ophthalmol: first published as 10.1136/bjo.85.9.1120 on 1 September 2001. Downloaded from cataracts after early surgery (surgery by 1 month of age) Age (months) Patching 0–1 No patching 1–2 1–2 hours/day 2–4 2–4 hours/day 4–6 50% awake hours 6–12 Up to 80% as indicated based on vision assessment visual cortical plasticity in the animal model and to be safe in human infants.6–8 Binocular occlusion is a way of postponing visual development until a clear retinal image can be established. I recommend binocular occlusion before surgery during the critical period of visual development (birth to 2 months of age) and for a duration of no longer than 2 weeks. Thirdly, after surgery and contact lens fitting, Wright et al 4 and Gregg and Parks5 used part time monocular occlusion of the good eye (less than 50%) to treat amblyopia rather than the standard treatment of full time occlusion. For the first month or two, virtually no patch- ing was performed, allowing for the develop- ment of binocular fusion. Full time monocular occlusion during early infancy destroys bin- ocular visual development and guarantees development of strabismus.3910 In addition, there is evidence that too much patching can actually reduce vision in the good eye (that is, Figure 1 This is my patient who had a dense congenital the phakic eye).11 Full time patching may be cataract, left eye, identifited at 1 day of age and had urgent necessary in older children with a cataract and surgery 48 hours after birth. The patient was treated aggressively with bilateral light occlusion from day 1 when strabismus (>1 year of age), but part time the cataract was identified, until a contact lens was fitted at patching is preferred in infants who have a 1 week of age. The patient wears a contact lens full time, left clear retinal image established during the first eye, and the right eye is being patched 4 hours a day. As pictured above, the patient is now 1 year old, has excellent few weeks of life (Table 1). fixation in each eye, straight eyes, and the family is very The papers by Wright et alT4 and Gregg and happy that aggressive treatment was initiated. Parks5 in 1992 and more recently by Brown et al12 in 1999, clearly show that good vision and prognosis include lamellar cataracts, posterior binocular fusion are not mutually exclusive, lenticonus, and persistent hyperplastic primary vitreous. These types of cataracts are often par- and that full time occlusion is not necessary if http://bjo.bmj.com/ a clear retinal image is established in early tial at birth and can progress over time, thus infancy. These papers are important as they allowing for early visual development. Another document that good visual acuity and binocu- indication for a good prognosis was the lar vision are obtainable through aggressive presence of straight eyes in these children. treatment (Fig 1). Approximately 50% of patients with a pre- sumed unilateral congenital cataract who had What about older children with presumed straight eyes and no strabismus obtained final unilateral congenital cataracts? visual acuity of 20/40 or better. The presence on October 1, 2021 by guest. Protected copyright. When an older child presents with a unilateral of straight eyes indicates that the cataract was cataract it can be diYcult to know whether the only partially obstructing the visual axis during cataract was there during the critical period the critical period of visual development and, if so, to what extent the cataract interfered indicating a relatively good visual prognosis. with visual development. Often, the clinician is unsure whether it is worthwhile removing the Indications for surgery—is the cataract cataract in a child who presents late after the visually significant? critical period of visual development. Should It is often diYcult to determine whether a par- we be aggressive in treating these children who tial cataract is visually significant in preverbal present late with presumed unilateral congeni- children. Neonates less than 8 weeks of age tal cataracts? This time the answer is some- normally have poor smooth pursuit eye move- times. Kushner13 reported relatively good ments and have not yet developed central fixa- results after surgery for monocular juvenile tion. Because of this, testing for fixation is not cataracts of undetermined onset. In the study very useful. Infant vision tests such as preferen- of Wright et al14 all patients presented after 10 tial looking and pattern visual evoked potential months of age and were treated with lensec- are also unreliable and diYcult to obtain tomy and aphakic contact lenses. Eighteen during the neonatal period. Neonates do not patients underwent surgery for a unilateral fully accommodate and it is diYcult to cataract and, of these, approximately 40% maintain their attention on the pattern stimu- obtained a visual acuity of 20/60 or better.