Khi Tri 2012
Khi Tri 2012
Khi Tri 2012
Monica R. Khitri, MD, Monte D. Mills, MD, Gui-Shuang Ying, PhD, Stefanie L. Davidson, MD,
and Graham E. Quinn, MD
PURPOSE
To evaluate and compare the visual acuity prognosis in the various pediatric glaucoma
subtypes and to determine risk factors for vision loss.
METHODS
The medical records of pediatric glaucoma patients from 2000 to 2010 at Childrens
Hospital of Philadelphia were retrospectively reviewed. Visual acuities, surgeries, glaucoma subtype, and etiology of vision impairment were recorded. Univariate and multivariate analyses were performed to determine the risk factors for visual impairment.
RESULTS
A total of 133 eyes (36.8% primary congenital glaucoma, 28.6% aphakic glaucoma, 12.0%
glaucoma associated with anterior segment dysgenesis, 12.0% Sturge-Weber glaucoma) of
88 patients were included. At last follow-up (median length, 5 years), 46.6% eyes achieved
excellent ($20/70) visual acuity. Of the glaucoma subtypes, primary congenital glaucoma
conferred the best visual prognosis, with 69.4% eyes with excellent ($20/70) visual acuity
at final follow-up. Factors most associated with visual impairment (\20/200) were unilateral disease, multiple surgeries, poor vision at diagnosis, and other ocular comorbidities.
The most common primary etiology for vision impairment was amblyopia (54.9%).
CONCLUSIONS
Patients with glaucoma early in life appear to have a better visual acuity prognosis than previously reported, with those with primary congenital glaucoma faring better than other
glaucoma subtypes. Recognition of risk factors for visual impairment can better guide
clinical management and counseling of patients. ( J AAPOS 2012;16:376-381)
376
this study was to determine the relationship among the various subtypes of pediatric glaucoma and their visual outcomes and to identify the factors that most affected visual
prognosis.
Methods
The medical records of all patients with pediatric glaucoma
treated between 2000 and 2010 at Childrens Hospital of Philadelphia were retrospectively reviewed. Institutional review board
approval was obtained for data review and analysis, and the study
conformed to the requirements of the Health Insurance Portability and Accountability Act. Eligible patients were identified
through queries of the medical billing record. Exclusion criteria
for the study included age .14 years at time of diagnosis, glaucoma related to trauma, and diagnosis and/or initial management
of glaucoma made at another hospital.
The following data were collected: type and laterality of glaucoma, age at diagnosis, ages at all surgeries, types of surgeries undertaken, visual acuities, and associated ocular and systemic
comorbidities. Visual acuities were measured using fixation or
Teller grating acuity cards for nonverbal children and Lea symbols, HOTV, and Snellen letters progressively for verbal children
as cooperation allowed. For those patients with subnormal final
visual acuities (\20/70) the primary etiology for the poor vision
also was determined. The cause of vision impairment in glaucomatous eyes can be multifactorial; however, we determined the single most important contributing factor for each eyes loss of vision
on the basis of the physical examination (ie, presence of optic
nerve pallor and/or cupping, retinal degeneration) and history
Journal of AAPOS
Khitri et al
2.11 (3.35)
0.36 (0.0, 13.8)
49 (36.8)
38 (28.6)
16 (12.0)
16 (12.0)
5 (3.76)
4 (3.01)
5 (3.76)
53 (39.9)
38 (28.6)
16 (12.0)
5 (3.76)
5 (3.76)
4 (3.01)
4 (3.01)
2 (1.50)
1 (0.75)
1 (0.75)
1 (0.75)
1 (0.75)
9 (10.2)
5 (5.7)
5 (5.7)
4 (4.6)
2 (2.3)
35 (26.3)
73 (54.9)
3 (2.26)
20 (15.0)
2 (1.50)
9.24 (6.87)
7.05 (1.28, 34.3)
7.14 (6.18)
4.95 (0.29, 27.9)
Journal of AAPOS
377
9 (6.77)
124 (93.2)
2.41 (4.19)
0.55 (0.01, 19.8)
4.03 (4.80)
1.62 (0.01, 28.3)
89 (66.9)
20 (15.0)
11 (8.30)
2 (1.5)
2 (1.5)
44 (33.1)
38 (28.6)
21 (15.8)
8 (6.0)
6 (4.5)
7 (5.3)
46 (37.1%)
37 (29.8%)
14 (11.3%)
13 (10.5%)
3 (2.4%)
4 (3.2%)
3 (2.4%)
2 (1.6%)
2 (1.6%)
2.35 (2.01)
2
Patients were clinically managed by 1 of 3 pediatric ophthalmologists (MDM, SLD, GEQ). The surgeries were classified as angle
surgery (goniotomy, trabeculotomy), filtering surgery (trabeculectomy, placement of an aqueous drainage device), revision of filtering bleb, cyclophotocoagulation, peripheral iridectomy or
iridotomy, or other (anterior chamber washout, synechiolysis, revision of tube shunt for malposition). The aqueous drainage devices used were the Ahmed glaucoma valve implant (New World
Medical Inc, Rancho Cucamonga, CA), the Baerveldt implant
(Advanced Medical Optics, Santa Ana, CA), and the Molteno implant (Ophthalmic Ltd, Dunedin, New Zealand). The Ahmed
valve implant was placed in a single procedure. The Baerveldt
and the Molteno implants were placed in a staged process with
a 4- to 6-week interval between the plate placement and the tube
introduction. For the purposes of this study, these two-staged procedures were counted as one surgery; the date recorded was that of
the second procedure given that the IOP-lowering effect of the
surgery would not occur until after the second procedure. Besides
surgery, patients often were on topical or systemic glaucoma medications during the course of their treatment.
Statistical Analysis
The patients ocular characteristics at diagnosis and characteristics of surgical management were summarized by mean, SD,
378
Khitri et al
Results
A total of 133 eyes of 88 children (50 females [56.8%])
with glaucoma were eligible and included; 24 patients
were excluded for insufficient data resultant from significant treatment periods at other hospitals, 3 for age
.14 years at diagnosis and 2 for history of trauma.
The ocular and systemic presenting characteristics of
the patients at diagnosis are given in Table 1. Fortyfive (51.1%) patients had bilateral glaucoma. The median age at diagnosis was 0.36 years (range, 0-14 years).
The median age for each glaucoma group was as follows: 2.53 years (range, 0.07-12.2 years) for aphakic,
0.30 years (range, 0.00-2.89 years) for primary congenital glaucoma, 0.02 years (range, 0.00-5.93 years) for
Sturge-Weber glaucoma, 0.29 years (range, 0.00-13.8
years) for glaucoma associated with anterior segment
dysgenesis, and 5.50 years (range, 0.14-10.5 years) for
others. The most commonly encountered types of glaucoma were primary congenital glaucoma (36.8%) and
aphakic glaucoma (28.6%). Many of the patients presented with other ocular comorbidities, both related
and unrelated to their diagnosis of glaucoma. The
most common ocular comorbidity was corneal opacification (39.9%) followed by aphakia postcataract extraction
(28.6%). Notably, the vast majority of the eyes (81.2%)
had excellent or good vision at time of diagnosis, with
n (%)
62 (46.6)
18 (13.5)
12 (9.02)
35 (26.3)
6 (4.51)
23 (35.9)
31 (48.5)
10 (15.6)
39 (54.9)
18 (25.4)
10 (14.1)
2 (2.82)
1 (1.41)
1 (1.41)
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Khitri et al
379
Table 4. Multivariate analysis of risk factors associated with vision impairment at last follow-up
Risk factors
Laterality of glaucoma
Bilateral
Unilateral
Vision at diagnosis
Good ($20/200)
Poor (\20/200)
Glaucoma diagnosis
Aphakic glaucoma
Primary congenital glaucoma
Sturge-Weber glaucoma
Glaucoma associated with anterior
segment dysgenesis
Aniridia associated glaucoma, uveitic
glaucoma, other
No. surgeries
\2
2
.2
No. eyes
90
43
26 (28.9)
27 (62.8)
1.00
5.46 (1.83-14.0)
108
25
72 (33.3)
15 (68.0)
1.00
4.68 (1.28-17.1)
38
49
16
16
19 (50.0)
11 (22.5)
5 (31.3)
10 (62.5)
1.00
0.25 (0.07-0.87)
0.07 (0.01-0.40)
1.60 (0.34-7.60)
0.03
0.003
0.56
14
8 (57.1)
0.64 (0.14-2.84)
0.19
55
37
41
12 (21.8)
12 (32.4)
29 (70.7)
1.00
3.30 (0.98-11.1)
32.3 (7.82-133)
P value
0.002
0.02
0.01
\0.0001
0.053
\0.0001
NLP
Poor
Fair
NLP
1
1
0
Poor
2
9
2
Fair
0
1
1
Good
3
20
6
Excellent
0
4
3
Percent of agreement 5 45/133 5 33.8%
Weighted kappa (95% CI) 5 0.23 (0.16-0.35)
Good
Excellent
0
2
0
11
5
0
5
1
33
23
Journal of AAPOS
380
Khitri et al
Discussion
In our pediatric patients with glaucoma, we found that at
least 60% of the glaucomatous eyes achieved good vision
(ie, $20/200) at final follow-up, and nearly one-half
(46.6%) of the eyes achieved a visual acuity of $20/70,
vision sufficient to qualify for a motor vehicle driving license in most states. Considering the patients vision
with both eyes open (functional visual outcome), more
than 84% of patients who could be tested in each eye had
at least one eye with final visual acuity of $20/200. These
results suggest outcomes better than those reported by earlier case series, which have reported good visual outcomes
in 29% to 41% of patients.6-9 The slightly greater
incidence of excellent vision in this study suggests that
progress that has been made during the last several years
in the medical and surgical management of pediatric
glaucoma, as others have suggested.3,10
We decided to group the various glaucoma subtypes in
a slightly different schematic than that reported by Yeung
and Walton4 to better reflect the specific subtypes most
commonly seen at our institution. Patients with primary
congenital glaucoma were all grouped together for the
purposes of this study despite the potential variability in
genotype because genetic testing was not routinely available. It became apparent that primary congenital glaucoma and Sturge-Weber glaucoma conferred a better
visual prognosis than the other subtypes studied. Specifically, only 22.5% and 31.3% of these groups, respectively,
had eyes with final visual acuities \20/200. Eyes with
other causes of glaucoma with additional concomitant ocular abnormalities (aphakia, anterior segment dysgenesis,
uveitis, aniridia, and other) have other additional factors
contributing to their poor visual outcome besides
glaucoma.
Poor vision at diagnosis appeared to portend a poor
visual prognosis. This correlation underscores the importance of early diagnosis to improved visual outcomesin
our study, patients who had already sustained considerable
vision loss by the time of presentation likely already suffered from amblyopia and/or glaucomatous optic neuropathy. Thus, the goal of effective treatment of pediatric
glaucoma may not be to improve patients vision but to
preserve the vision they present with at diagnosis.
IOP control in pediatric glaucoma is possible with
current surgical techniques and medications. The British
Infantile and Childhood Glaucoma (BIG) Eye study demonstrated that IOP control in pediatric glaucoma is successful in 94% of patients.11 However, good IOP control
does not necessarily predict a good visual outcome. Amblyopia proved to be the single most important factor contributing to poor visual outcome in our study. This finding is
similar to visual acuity outcomes associated with other pediatric ocular conditions, such as congenital cataracts.12-14
Successful management of pediatric glaucoma cannot
focus exclusively on IOP control but must also ensure
adequate amblyopia therapy.
Patients with unilateral glaucoma fared worse than
those with bilateral glaucoma, just as in their counterparts with congenital cataracts. In multivariate analysis,
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381