Results of Late Surgery for Presumed Congenital Cataracts Kenneth W. Wright, M.D., Laurie E. Christensen, M.D., and Barbara A. Noguchi, M.D. We reviewed the results of cataract extraction and visual rehabilitation in 76 eyes of 47 infants and children with presumed congenital cataracts who were first seen after they were 10 months old. Eighteen patients underwent surgery for unilateral cataracts, including five patients with persistent hyperplastic vitreous, five with posterior lenticonus, one with a nuclear cataract, six with posterior subcapsular cataracts, and one with a lamellar cataract. Of these 18 patients, seven (39%) attained a visual acuity of 20/60 or better, one (6%) had a visual acuity of 20/100, and ten (60%) had a visual acuity of 20/200 or worse. Twenty-nine patients (62 eyes) underwent bilateral cataract extraction. The visual acuity could be measured in 22 patients (44 eyes). Visual acuity improved to 20/60 or better in 32 eyes (73%), was between 20/70 and 20/150 in 11 eyes (25%), and became worse than 20/200 in one eye (2%). Results were good in patients with persistent hyperplastic primary vitreous, posterior lenticonus, and bilateral cataracts. OUR

CLINICAL EXPERIENCE and previous re­

ports 1 · 2 have shown that some children with presumed congenital cataracts show substan­ tial improvement even when surgery is per­ formed late after the critical period of visual development (after 10 months of age). Previous results substantiate these findings. Owens and Hughes 3 found that 37.8% of patients with bilateral total congenital cataracts achieved a visual acuity of 20/70 or better, and François 4 had similar results. We evaluated the visual

Accepted for publication July 9, 1992. From the Department of Ophthalmology, University of Southern California School of Medicine and Division of Ophthalmology, Childrens Hospital of Los Angeles, Los Angeles, California (Dr. Wright); Oregon Health Sciences University, Portland, Oregon (Dr. Christen­ sen); and Department of Ophthalmology, Tulane Uni­ versity, New Orleans, Louisiana (Dr. Noguchi). Reprint requests to Kenneth W. Wright, M.D., Division of Ophthalmology, Childrens Hospital of Los Angeles, 4650 Sunset Blvd., Los Angeles, CA 90027.

prognosis of children who underwent late sur­ gery for presumed congenital cataracts and identified preoperative clinical signs that could help predict visual outcome.

Patients and Methods Charts from 211 consecutive children who underwent unilateral or bilateral lensectomies between 1980 and 1988 at Childrens Hospital of Los Angeles were reviewed. Of these 211 patients, 50 met the inclusion criteria for this study, which included lensectomy after 10 months of age for a presumed congenital cata­ ract. Patients with acquired cataracts secondary to trauma or an identifiable metabolic cause were excluded from the study. Cataracts are classified as unilateral or bilat­ eral and by their morphologic characteristics. 5 7 Lamellar cataract is defined as a lens opacity that affects the cortical layers outside the nucle­ us and the Y sutures. Nuclear cataract is defined as an opacity located between the Y sutures in the nuclear part of the lens. 7 Lamellar cataracts were included, even though they typically de­ velop after birth, as these opacities often sur­ round the lens nucleus, giving the appearance of a nuclear cataract as well as the impression of being congenital. 6 Six patients had bilateral cataracts and preoperative nystagmus, includ­ ing four with nuclear cataracts and two with total cataracts. The ages of the patients at the time of surgery ranged from 10 months to 15 years (mean, 5 years 1 month). Most extractions were per­ formed by one of us (K.W.W.), but four other staff surgeons also operated on patients. The average follow-up was 2Vi years for patients with a unilateral cataract, and two years one month for those with bilateral cataracts. All patients were followed up for at least six months. Forty-seven patients were included in the study (18 with unilateral cataracts and 29 with bilateral cataracts). In preverbal children, cataract extraction was indicated if the opacity blocked at least 3 mm of

©AMERICAN JOURNAL OF OPHTHALMOLOGY 114:409-415, OCTOBER, 1992

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the central visual axis, distorting both retinoscopy and direct ophthalmoscopy. In older chil­ dren, a linear visual acuity of 20/70 or worse was an indication for surgery, except for one patient who had surgery performed on the second eye with a visual acuity of 20/50 be­ cause of decreasing vision. All patients were treated with a limbalapproach lensectomy with vitrectomy instru­ mentation. 8 In three patients, the posterior cap­ sule was left intact and a secondary YAG laser capsulotomy was performed one month postoperatively. In the remaining patients, a posterior capsulotomy and anterior vitrectomy were per­ formed as part of the primary procedure. Aphakia was managed with flexible-wear contact lenses in all cases. Patients with unilat­ eral aphakia were treated with occlusion thera­ py of the fellow eye. Visual acuity was measured using either sin­ gle or linear optotype testing with Allen pic­ tures, illiterate E, or Snellen chart. In patients too young to cooperate with optotype visual acuity testing, visual acuities were estimated by the child's ability to fixate and to follow objects. The postoperative visual acuities recorded were

obtained during the most recent follow-up vis­ it. Postoperative visual rehabilitation consisted of combinations of contact lenses, spectacles, and patching regimens for amblyopia.

Results Unilateral cataracts—Of 18 eyes with unilat­ eral cataracts, five were caused by persistent hyperplastic primary vitreous, five were caused by posterior lenticonus, two were nuclear, six were posterior subcapsular, and one was lamel­ lar (Table 1). Postoperatively, all 18 patients were able to cooperate with visual acuity test­ ing with optotypes. Seven patients (39%) at­ tained a visual acuity of 20/60 or better, one patient (6%) had a visual acuity of 20/100, and 11 patients (58%) had a visual acuity of 20/200 or worse. Strabismus—Of the 18 patients with unilater­ al cataracts, 11 had preoperativ t e strabismus. The absence of strabismus was a good prognos­ tic sign, with four of the eight nonstrabismic patients (50%) achieving a visual acuity be-

TABLE 1 PATIENTS WITH UNILATERAL CATARACTS PATIENT NO.

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18

AGE AT TYPE OF CATARACT

Persistent hyperplastic primary vitreous Persistent hyperplastic primary vitreous Persistent hyperplastic primary vitreous Persistent hyperplastic primary vitreous Persistent hyperplastic primary vitreous Posterior lenticonus Posterior lenticonus Posterior lenticonus Posterior lenticonus Posterior lenticonus Posterior subcapsular Posterior subcapsular Posterior subcapsular Posterior subcapsular Posterior subcapsular Posterior subcapsular Nuclear Lamellar

SURGERY

PREOPERATIVE VISUAL FINDING

POSTOPERATIVE VISUAL ACUITY

FOLLOW-UP

10 mos

Poor fixation

20/40 Allen

3 yrs 4 mos

1 yr 6 mos

Poor fixation

14/30 Allen

2 yrs 8 mos

1 yr 11 mos

No fixation

10/30 Allen

7 mos

2 yrs 10 mos

Poor fixation

5/200 illiterate E

1 yr 4 mos

4 yrs 4 mos

3/30 Allen visual acuity

20/60 Snellen

2 yrs 2 mos

1 yr 9 mos 3 yrs 1 mo 3 yrs 7 mos 4 yrs 6 mos 6 yrs 8 mos 10 mos 2 yrs 1 mo 3 yrs 7 mos 5 yrs 9 mos 7 yrs 15 yrs 2 mos 10 mos 5 yrs

Poor fixation Light perception 12/30 Allen visual acuity 1/30 Allen visual acuity 1/200 illiterate E visual acuity No fixation No fixation No fixation 20/200 illiterate E visual acuity 5/200 illiterate E visual acuity 20/100 Snellen visual acuity Poor fixation 4/30 Allen visual acuity

4/200 illiterate E 15/30 Allen 20/30 Snellen 2/30 Allen 10/200 illiterate E 1/30 Allen 1/30 Allen Light perception 20/100 Snellen 20/200 illiterate E 20/25 Snellen 1/30 Allen 20/200 illiterate E

3 yrs 1 mo 6 mos 3 yrs 11 mos 7 mos 1 yr 9 mos 3 yrs 2 mos 4 yrs 6 mos 2 yrs 1 mo 3 yrs 1 mo 8 mos 1 yr 11 mos 8 yrs 6 mos 2 yrs 4 mos

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Late Surgery for Presumed Congenital Cataracts

tween 20/40 and 2 0 / 2 5 . In contrast, only one of 11 patients with strabismus achieved a visual acuity of 20/40. The presence of strabismus did not, however, preclude marked improvement, as the visual acuity in four of the 11 patients with unilateral cataracts and strabismus im­ proved to 20/100 or better. Unilateral persistent hyperplastic primary vitreous—Of the five patients in this group, four achieved a visual acuity of 20/60 or better. One patient had a poor visual outcome, with a postoperative visual acuity of 20/800, which was secondary to a congenital fractional de­ tachment of the macula. Posterior lenticonus—Of five patients in this group, two attained postoperative visual acuity of 20/30 (Snellen) and 20/40 (Allen card, Snellen equivalent). The remaining three patients had a postoperative visual acuity of 2/30 (Allen card), 20/400, and 4/200 (illiterate E), respec­ tively. Unilateral nuclear cataracts—The one patient with a unilateral nuclear opacity had a poor visual outcome of 1/30 visual acuity (Allen card). Unilateral posterior subcapsular cataracts—Of the six patients in this group, four had postop­ erative visual acuities of 20/200 or worse, one had a 20/100 visual acuity, and one patient (Patient 16) had a visual acuity of 2 0 / 2 5 . Unilateral lamellar cataracts—The one pa­ tient in this group had exotropia and a visual acuity of 4/30 (Allen card) preoperatively; the postoperative visual acuity was essentially un­ changed at 20/200 (Snellen). Bilateral cataracts—Twenty-nine patients un­ derwent bilateral cataract extraction (Table 2). Postoperative optotype visual acuities were re­ corded from 44 eyes of 22 patients. Visual acuity improved to 20/60 or better in 32 eyes (73%), was between 2 0 / 7 0 and 20/150 in 11 eyes (25%), and became worse than 20/200 in one eye (2%). Using the visual acuity of 20/60 as the criterion for a good visual result, we found that 15 of 22 patients (68%) achieved a good visual acuity in both eyes; two of 22 (9%) achieved a good visual acuity in one eye, but poor visual acuity in the fellow eye; and five of 22 (23%) had poor visual acuity in both eyes. Seven of the 29 patients were too young to cooperate with postoperative visual acuity test­ ing with optotypes. Five of the seven patients had improved fixation in both eyes, whereas the other two patients showed no change in fixation of either eye postoperatively. Nystagmus—Four of six patients (Patients 1,

411

6, 17, 18, 25, and 26; Table 2) with preoperative nystagmus had marked postoperative improve­ ment in visual acuity and marked lessening of the nystagmus. In two of these patients with preoperative nystagmus (Patients 1 and 18), postoperative optotype visual acuity could be obtained. .Patient 1 achieved a 13/20 visual acuity by Allen picture cards (approximately 20/50 Snellen equivalent) and Patient 18 had a 20/70 visual acuity by linear Snellen. Bilateral nuclear cataracts—This group com­ prised 18 patients with bilateral nuclear cata­ racts, five of whom had a nuclear cataract in one eye and a total cataract in the fellow eye. Of the 18 patients, 14 (28 operated-on eyes) under­ went postoperative optotype visual acuity test­ ing. Visual acuity improved to 20/60 or better in 23 of the 28 eyes (82%), and between 20/70 and 20/150 in five eyes (18%). Visual acuity on an individual patient basis (using the eye with better vision) ranged from 20/150 to 20/20, with 12 of the 18 patients (67%) achieving a visual acuity of 20/60 or better. Bilateral posterior subcapsular cataracts— Three patients had posterior subcapsular cata­ racts, including two patients with bilateral pos­ terior subcapsular cataracts and one patient with a total cataract in the fellow eye. Compari­ son of preoperative and postoperative visual acuities showed marked improvement in each case. The postoperative visual acuity in the eye with better vision was 2 0 / 5 0 (Patient 19), 2 0 / 70 (Patient 20), and 20/80 (Patient 21), respec­ tively, for these three patients. One of the patients (Patient 21) had a marked difference in visual acuity, as one eye was amblyopic at 20/200. Bilateral lamellar cataracts—Three patients had bilateral lamellar cataracts. The visual acu­ ity of all three patients improved postoperative­ ly (20/25 in both eyes in Patient 22, 20/40 in both eyes in Patient 23, and R.E.: 20/70 and L.E.: 20/50 in Patient 24). Bilateral total cataracts—Bilateral total cata­ racts were present in four patients, two of whom were able to cooperate with visual acuity testing with optotypes at the last follow-up visit. Postoperative visual acuity in the eye with better vision was 20/30 and 20/120 (Allen card, Snellen equivalent) for these two patients. Bilateral cerulean (blue dot) cataracts—The one patient with cerulean cataracts was able to fixate and follow light with both eyes preopera­ tively. This child was able to fixate and follow light postoperatively, but was lost to follow-up after six months.

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TABLE Ξ1 PATIENTS WITH BILATERAL CATARACTS* TYPE OF CATARACT

AGE AT SURGERY PATIENT

PREOPERATIVE VISUAL FINDING

NO.

R.E.

L.E.

R.E.

L.E.

R.E.

L.E.

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19

1 yr 4 mos 1 yr 6 mos 1 yr 6 mos 1 yr 11 mos 2yrs 4 yrs 3 mos 4 yrs 5 mos 5 yrs 1 mo 5 yrs 4 mos 6 yrs 6 yrs 8 mos 6 yrs 8 mos 8 yrs 7 yrs 10 mos 13 yrs 1 mo 8 yrs 10 mos 14 yrs 2 mos 14 yrs 8 mos 4 yrs 6 mos

1 yr 4 mos 1 yr 6 mos 1 yr 6 mos 2 yrs 2 mos 3 yrs 4 yrs 5 mos 4 yrs 5 mos 5 yrs 1 mo 5 yrs 2 mos 5 yrs 5 mos 6 yrs 8 mos 7 yrs 1 mo 6 yrs 10 mos 8 yrs 1 mo 8 yrs 6 mos 9 yrs 10 mos 14 yrs 2 mos 14 yrs 6 mos 4 yrs 6 mos

Poor fixation Fair fixation Fair fixation Fair fixation Fair fixation Poor fixation 5/30 Allen 5/30 Allen 20/80 Snellen 20/400 Snellen 20/200 Snellen 2/200 Snellen 5/30 Snellen 20/200 Snellen 20/80 Snellen 20/100 Snellen Light percepton 7/200 Illiterate E 20/100 Snellen

5 yrs 5 mos

5 yrs 5 mos

Light perception

20/30 Allen

21

8 yrs 4 mos

8 yrs 4 mos

10/200 Illiterate E

6/200 Illiterate E

22 23 24 25 26 27 28 29

6 yrs 1 mo 6 yrs 6 mos 13 yrs 2 mos

5 yrs 7 mo 7 yrs 1 mo 12 yrs 11 mos

1 yr 1 yr 1 mo 2 yrs 3 mos 2 yrs 6 mos 1 yr 11 mos

i yr 1 yr 1 mo 2 yrs 3 mos 2 yrs 6 mos 2 yrs

Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Total Nuclear Posterior subcapsular Posterior subcapsular Posterior subcapsular Lamellar Lamellar Lamellar Total Total Total Total Cerulean blue dot

Poor fixation Fair fixation Fair fixation Poor fixation No fixation No fixation 2/30 Allen 4/30 Allen 20/70 Snellen 20/100 Snellen 20/200 Snellen 2/200 Snellen 20/50 Snellen 20/200 Snellen 20/70 Snellen 20/200 Snellen Hand motions 20/200 Illiterate E 20/80 Snellen

20

Nuclear Nuclear Nuclear Total Total Total Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Nuclear Posterior subcapsular Total

20/200 Snellen Poor fixation 20/80 Illiterate E No fixation Poor fixation Poor fixation 0/30 Allen Fair fixation

20/100 Snellen Poor fixation 20/100 Illiterate E Fair fixation Poor fixation Poor fixation 0/30 Allen Fair fixation

Posterior subcapsular Lamellar Lamellar Lamellar Total Total Total Total Cerulean blue dot

Patients 1, 6, 17,18, 25, and 26 had preoperative nystagmus, which decreased postoperatively in Patients 1,6, 18, and 25.

Discussion Some patients with unilateral cataracts devel­ op relatively good visual acuity even when cataract surgery was performed after the child was 10 months old. This group with a good visual prognosis included patients with persis­ tent hyperplastic primary vitreous, posterior lenticonus, bilateral nuclear cataracts, lamellar cataracts, and patients without strabismus. The absence of strabismus is a good prognostic sign, as four of eight patients with unilateral cata­

racts and essentially straight eyes achieved a visual acuity between 20/40 and 2 0 / 2 5 . Four of five (80%) patients with persistent hyperplastic primary vitreous obtained visual acuities of 20/60 or better. Karr and Scott9 also found good visual results in patients with per­ sistent hyperplastic primary vitreous. One of their patients who underwent lensectomy and anterior vitrectomy at 10 months of age achieved a 20/50 visual acuity. These good results may be attributed to a faint posterior opacity at birth, which allows development of central fixation. Over time, the opacity increas-

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Late Surgery for Presumed Congenital Cataracts

TABLE 2 (Continued) PATIENTS WITH BILATERAL CATARACTS POSTOPERATIVE VISUAL RESULTS R.E.

LENGTH OF FOLLOW-UP R.E.

13/30 Allen Fair fixation Good fixation 20/30 Allen Good fixation Good fixation 20/20 Snellen 20/100 Snellen 20/30 Snellen 20/30 Snellen 20/20 Snellen 20/25 Snellen 20/40 Snellen 20/25 Snellen 20/25 Snellen 20/20 Snellen 4/30 Allen 20/70 Snellen 20/50 Snellen

13/30 Allen Fair fixation Good fixation 20/30 Allen Good fixation Good fixation 20/30 Allen 20/50 Snellen 20/25 Snellen 20/40 Snellen 20/25 Snellen 20/30 Snellen 20/40 Snellen 20/40 Snellen 20/30 Snellen 20/30 Snellen 4/30 Allen 20/80 Snellen 20/50 Snellen

1 yr 8 mos 6 mos 9 mos 2 yrs 8 mos

20/80 Snellen

20/70 Snellen

1 yr 6 mos

1 yr 6 mos

20/80 Snellen

20/200 Snellen

6 mos

6 mos

20/25 Snellen 20/70 Illiterate E 20/40 Illiterate E Good fixation Good fixation 20/30 Allen 5/30 Allen Fair fixation

20/25 Snellen 20/50 Illiterate E 20/40 Illiterate E Good fixation Good fixation 20/40 Allen 5/30 Allen Fair fixation

2 yrs 4 mos 7 yrs 6 mos 2 yrs 7 mos 1 yr 10 mos 6 mos 5 yrs 11 mos

2 yrs 10 mos 6 yrs 11 mos 2 yrs 10 mos I yr 10 mos 6 mos 5 yrs 11 mos I I mos 5 mos

es and an amblyopia of relatively late onset develops. Persistent hyperplastic primary vitre­ ous presents as a wide spectrum of disease, and patients with retinal involvement have a poor prognosis. The one patient in our study who did not have a marked improvement in visual acu­ ity postoperatively had a congenital peripapillary traction band that extended from the optic nerve stalk to the macula, which caused a macular detachment. Posterior lenticonus, like persistent hyper­ plastic primary vitreous, is a condition that is congenital but progressive, often causing mini­

1 yr 1 yr 3 mos 4 yrs 3 mos 1 yr 6 mos 1 yr 2 mos 1 yr 6 mos 1 yr 1 yr 5 mos 10 mos 4 yrs 2 mos 3 mos 4 yrs 1 yr 10 mos 1 yr 6 mos

11 mos 6 mos

1 yr 8 mos 6 mos 9 mos 2 yrs 5 mos 1 mo 1 yr 1 mo 4 yrs 3 mos 1 yr 6 mos 1 yr 2 yrs 1 mo 1 yr 1 yr 1 mo 2 yrs 4 yrs 5 mos 4 yrs 7 mos 3 yrs 1 yr 10 mos 1 yr 2 mos 6 mos

mal distortion of the retinal image at birth. 10 · 11 Crouch and Parks10 described 21 patients who underwent cataract extraction for posterior len­ ticonus; all but two of the patients were first seen after they were 1 year old. The visual acuity in 15 of the 21 patients improved to 20/50 or better postoperatively. The patients with posterior lenticonus in our series also developed relatively good vision. Two of five patients (40%) attained visual acuities equiva­ lent to 20/40 or better. Unilateral cataracts associated with a rela­ tively poor prognosis include monocular nucle-

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ar cataracts, m o n o c u l a r p o s t e r i o r s u b c a p s u l a r cataracts, a n d cataracts in p a t i e n t s w i t h s t r a b i s ­ m u s . S o m e of t h e s e p a t i e n t s , h o w e v e r , s h o w e d marked postoperative improvement. One pa­ tient w i t h a p o s t e r i o r s u b c a p s u l a r cataract achieved 2 0 / 2 5 visual acuity, a n d four of 11 p a t i e n t s (36%) w i t h a u n i l a t e r a l cataract a n d s t r a b i s m u s a c h i e v e d a visual acuity b e t w e e n 2 0 / 1 0 0 a n d 2 0 / 4 0 . We agree w i t h P a r k s 1 2 t h a t d i s t i n g u i s h i n g b e t w e e n c o n g e n i t a l a n d ac­ q u i r e d cataracts in infants a n d y o u n g c h i l d r e n may b e i m p o s s i b l e . It is difficult, t h e r e f o r e , to predict w h i c h c h i l d r e n will have irreversible a m b l y o p i a a n d w h i c h have the p o t e n t i a l for m a r k e d visual acuity i m p r o v e m e n t . N u c l e a r opacities indicate a c o n g e n i t a l o n s e t a n d therefore u s u a l l y have a p o o r visual p r o g ­ nosis. 7 · 1 2 1 3 T h e only p a t i e n t in o u r s t u d y w i t h a u n i l a t e r a l n u c l e a r cataract (Patient 17) h a d a p o o r visual result. P a t i e n t s w i t h bilateral n u c l e ­ ar cataracts, h o w e v e r , did relatively well, w i t h 12 of 18 p a t i e n t s (67%) a t t a i n i n g a v i s u a l acuity of 2 0 / 6 0 or better. In p a t i e n t s w h o a c h i e v e d visual acuity of 2 0 / 6 0 or b e t t e r , t h e n u c l e a r opacities m a y h a v e b e e n relatively clear d u r i n g the critical p e r i o d of visual d e v e l o p m e n t , a n d later p r o g r e s s e d . Direct p r o g r e s s i o n of the n u ­ clear opacity w a s d o c u m e n t e d in four of t h e 19 p a t i e n t s in o u r s t u d y w h o h a d a g o o d visual o u t c o m e . This o b s e r v a t i o n is s u p p o r t e d by the results of Bercovitch a n d Donaldson, 1 4 w h o photographically documented the progression of a nuclear cataract. N y s t a g m u s in a child w i t h bilateral cataracts indicates d e n s e a m b l y o p i a a n d a p o o r v i s u a l prognosis. 1 2 1 5 1 6 Parks 1 2 d e s c r i b e d eight p a t i e n t s w i t h n y s t a g m u s s e c o n d a r y to c o n g e n i t a l cata­ racts a n d f o u n d that n o n e h a d a visual acuity b e t t e r t h a n 2 0 / 2 0 0 p o s t o p e r a t i v e l y . Five of t h e eight p a t i e n t s , h o w e v e r , w e r e too y o u n g to c o o p e r a t e w i t h o p t o t y p e visual acuity testing. In o u r series, four of six p a t i e n t s w i t h bilateral cataracts a n d n y s t a g m u s s h o w e d i m p r o v e d v i s ­ ual acuity a n d l e s s e n i n g of n y s t a g m u s p o s t o p ­ eratively, w i t h t w o p a t i e n t s a c h i e v i n g v i s u a l acuities of 2 0 / 5 0 a n d 2 0 / 7 0 , r e s p e c t i v e l y . S e n ­ sory n y s t a g m u s generally i n d i c a t e s a p o o r p r o g ­ nosis, b u t the p r e s e n c e of n y s t a g m u s in a p a ­ t i e n t w i t h cataracts d o e s n o t p r e c l u d e m a r k e d visual i m p r o v e m e n t . Because cataract extrac­ tion is a relatively low-risk p r o c e d u r e , this p r o c e d u r e s h o u l d b e c o n s i d e r e d for p a t i e n t s with sensory nystagmus w h o would otherwise be blind. We do n o t a d v o c a t e delay of s u r g e r y in in­

fants w i t h visually significant c a t a r a c t s . T h e o p t i m u m t r e a t m e n t for b o t h m o n o c u l a r a n d b i n o c u l a r c o n g e n i t a l cataracts is early s u r g e r y a n d visual r e h a b i l i t a t i o n before 2 m o n t h s of age, preferably w i t h i n t h e first few w e e k s of life. 12,1622 H o w e v e r , w h e n a child w i t h a u n i l a t ­ eral or bilateral cataract is t r e a t e d after the critical p e r i o d , t h e possibility for v i s u a l im­ p r o v e m e n t s h o u l d n o t b e categorically dis­ counted.

References 1. Cheng, K. P., Hiles, D. A., Biglan, A. W., and Pettapiece, M. C : Visual results after early surgical treatment of unilateral congenital cataracts. Oph­ thalmology 98:903, 1991. 2. Kushner, B. J.: Visual results after surgery for monocular juvenile cataracts of undetermined onset. Am. J. Ophthalmol. 102:468, 1986. 3. Owens, W. C , and Hughes, W. F.: Results of surgical treatment of congenital cataract. Arch. Oph­ thalmol. 39:339, 1948. 4. François, J.: Congenital Cataracts. Assen, The Netherlands, Royal Van Gorcum, 1963, p. 608. 5. Jain, I. S„ Pillay, P., Gangwar, D. N., Dhir, S. P., and Kaul, V. K.: Congenital cataract. Etiology and morphology. J. Pediatr. Ophthalmol. Strabismus 20:238, 1983. 6. Parks, M. M.: Management of cataracts in in­ fants. Pediatrie Ophthalmology and Strabismus. Trans. New Orleans Acad. Ophthalmol. 119, 1986. 7. McDonald, P. R.: Disorders of the lens. In Harley, R. D. (ed.): Pediatrie Ophthalmology. Philadel­ phia, W. B. Saunders, 1975, pp. 370-388. 8. Wright, K. W.: Lensectomy for pediatrie cata­ racts. In Wright, K. W. (ed.): Color Atlas of Ophthal­ mic Surgery. Strabismus. Philadelphia, J. B. Lippincott, 1990, pp. 163-180. 9. Karr, D. J., and Scott, W. E.: Visual acuity re­ sults following treatment of persistent hyperplastic primary vitreous. Arch. Ophthalmol. 104:662, 1986. 10. Crouch, E. R., Jr., and Parks, M. M.: Manage­ ment of posterior lenticonus complicated by unilater­ al cataract. Am. J. Ophthalmol. 85:503, 1978. 11. Khalil, M., and Saheb, N.: Posterior lenticonus. Ophthalmology 91:1429, 1984. 12. Parks, M. M.: Visual results in aphakic chil­ dren. Am. J. Ophthalmol. 94:441, 1982. 13. Gelbart, S. S., Hoyt, C. S., Jastrebski, G., and Marg, E.: Long-term visual results in bilateral con­ genital cataracts. Am. J. Ophthalmol. 93:615, 1982. 14. Bercovitch, L., and Donaldson, D. D.: The nat­ ural history of congenital suturai cataracts. Case report with long-term follow-up. Am. J. Pediatr. Ophthalmol. Strabismus 19:108, 1982. 15. Rogers, G. L., Tishler, C. L., Tsou, B. H., Hertle, R. W., and Fellows, R. R.: Visual acuities in

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infants with congenital cataracts operated on prior to 6 months of age. Arch. Ophthalmol. 99:999, 1981. 16. Beauchamp, G. R., Hiles, D. A., Parks, M. M., Rogers, G. L., and Sheets, I. H.: Round table discus­ sion. Congenital cataracts. Ophthalmic Forum 2:141, 1984. 17. Robb, R. M., Mayer, D. L., and Moore, B. D.: Results of early treatment of unilateral congenital cataracts. J. Pediatr. Ophthalmol. Strabismus 24:178, 1987. 18. Beller, R., Hoyt, C. S., Marg, E., and Odom, J. V.: Good visual function after neonatal surgery for congenital monocular cataracts. Am. J. Ophthalmol. 91:559, 1981.

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19. Drummond, G. T., Scott, W. E„ and Keech, R. V.: Management of monocular congenital cata­ racts. Arch. Ophthalmol. 107:45, 1989. 20. Birch, E. E., and Stager, D. R.: Prevalence of good visual acuity following surgery for congenital unilateral cataract. Arch. Ophthalmol. 106:40, 1988. 21. Wright, K. W.: Management of amblyopia as­ sociated with congenital cataracts. In Jovicevic, B. (ed.): International Survey. Pediatrie Ophthalmology and Strabismus. Gainesville, Florida, Binoculus, 1991, pp. 27-34. 22. Pratt-Johnson, J. A., and Tillson, G.: Visual results after removal of congenital cataracts before the age of 1 year. Can. J. Ophthalmol. 16:19, 1981.

OPHTHALMIC MINIATURE

It is no good asking for a simple religion. After all, real things are not simple. They look simple, but they are not. The table I am sitting at looks simple, but ask a scientist to tell you what it is really m a d e of—all about the atoms and how the light waves rebound from them and hit my eye and what they do to the optic nerve and what it does to my brain—and, of course, you find that what we call "seeing a t a b l e " lands you in mysteries and complications which you can hardly get to the end of. C. S. Lewis, Mere Christianity New York, Collier Books, 1952, p . 46

Results of late surgery for presumed congenital cataracts.

We reviewed the results of cataract extraction and visual rehabilitation in 76 eyes of 47 infants and children with presumed congenital cataracts who ...
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