Incidence and Risk Factors for Glaucoma
Development After Bilateral Congenital Cataract
Surgery in Microphthalmic Eyes
JEONG-AH KIM, SANG-YOON LEE, KI HO PARK, YOUNG SUK YU, AND JIN WOOK JEOUNG

PURPOSE: To evaluate the long-term incidence and risk
of glaucoma after bilateral congenital cataract surgery in
microphthalmic eyes.

DESIGN: Retrospective, observational case series.

METHODS: SUBJECTS: Children with microphthalmic
eyes who had undergone surgery for bilateral congenital
cataract within 6 months of birth and been followed up
for at least 5 years. PROCEDURES: Review of medical re-
cords at our institution. MAIN OUTCOME MEASURES: Prob-
ability of an eye’s developing glaucoma after bilateral
congenital cataract surgery and associated risk factors.

RESULTS: Thirty-eight eyes of 19 children with bilat-
eral congenital cataract were included. The mean age at
surgery was 3.2 ± 1.7 months, and the mean follow-up
duration was 7.79 ± 2.61 years. After cataract surgery,
11 eyes (29.0%) developed glaucoma at the age of 4.0
± 1.4 years. Three of these eyes underwent Ahmed glau-
coma valve implantation surgery. The probability of an
eye’s developing glaucoma was estimated to be 32.0%
by 10 years after surgery. In a multivariate analysis, axial
length was significantly associated with glaucoma devel-
opment (odds ratio [ 0.364, P [ .025). Age at the
time of cataract surgery, corneal diameter, and aphakia
did not affect the risk of glaucoma (P > .10). Eyes
without glaucoma had a better final visual outcome than
those with glaucoma (0.75 ± 0.60 and 1.47 ± 1.10
logMAR, respectively, P [ .049).

CONCLUSIONS: The long-term cumulative risk of post-
operative glaucoma development was 32.0% by 10 years
after bilateral congenital cataract surgery. Because the
risk of developing glaucoma persists for several years after
surgery, careful monitoring and control of intraocular
pressure is needed to preserve vision in such
Supplemental Material available at AJO.com.
Accepted for publication Aug 16, 2019.
From the Department of Ophthalmology, Hallym University College of
Medicine, Chuncheon Sacred Heart Hospital, Chuncheon, South Korea
(J.A.K.); Department of Ophthalmology, Seoul National University
College of Medicine, Seoul, South Korea (J.A.K., K.H.P., Y.S.Y.,
J.W.J.); Department of Ophthalmology, Jeju National University
College of Medicine, Jeju City, South Korea (S.Y.L.); and Department
of Ophthalmology, Seoul National University Hospital, Seoul, South
Korea (K.H.P., Y.S.Y., J.W.J.).
Inquiries to Jin Wook Jeoung, Department of Ophthalmology, Seoul
National University Hospital, Seoul National University College of
Medicine, 101 Daehak-ro, Jongno-gu, Seoul 03080, South Korea;
e-mail: neuroprotect@gmail.com
0002-9394/$36.00 © 2019 ELSEVIER INC. ALL
https://doi.org/10.1016/j.ajo.2019.08.005
patients. (Am J Ophthalmol 2019;208:265–272. Ó
2019 Elsevier Inc. All rights reserved.)
M
ICROPHTHALMIA IS A RARE CONGENITAL MAL-
formation resulting from abnormalities in the
development of the primary optic vesicle.1 It
has an incidence of 0.2-9 per 10,000 births, affects
3%-11% of blind children, and is associated with a high
prevalence of congenital cataract.2–5 Even for children
with microphthalmic eyes, early cataract surgery is
essential in order to prevent amblyopia and improve
visual outcomes, including stereopsis.6–9 However, despite
recent advances in pediatric cataract microsurgical
techniques, early surgical treatment in children with
microphthalmia remains challenging owing to several
intraoperative difficulties attributable to the crowded
anterior segment and the risk of postoperative
complications such as glaucoma.10–12
Glaucoma is one of the vision-threatening complica-
tions after congenital cataract surgery.13,14 However, its
incidence varies widely in the previous reports in the
literature, owing to differences among study populations
(eg, race, age at cataract surgery, etc) and in follow-up pe-
riods.15–21 Especially in rare cases of microphthalmia, the
long-term risk of glaucoma development after congenital
cataract surgery has not been well studied, and so disease
risk and the associated factors are still unclear. Since devel-
opment of glaucoma after cataract surgery takes consider-
able time, even in cases of microphthalmia, long-term
study is needed to evaluate the incidence of glaucoma
development and its risk factors after congenital cataract
surgery in microphthalmic eyes.
In the present study, we investigated the long-term risk
of glaucoma development and associated factors in micro-
phthalmic eyes of Korean children who had undergone
bilateral congenital cataract surgery within 6 months of
birth.
METHODS
PATIENTS DIAGNOSED WITH MICROPHTHALMIA WHO HAD
undergone bilateral congenital cataract surgery within
6 months of birth at Seoul National University Children’s
RIGHTS RESERVED. 265
Hospital between January 1997 and December 2008 were
consecutively included in this retrospective observational
cohort study. All of the patients were followed for at least
5 years after the initial visit. This study’s protocol was
approved by the Institutional Review Board of Seoul Na-
tional University Hospital, and its conduct was consistent
with the tenets of the Declaration of Helsinki.
Microphthalmia was defined based on a total axial
length at least 2 standard deviations below the mean for
age.22,23 Congenital cataract was defined as cataract
existing in patients younger than 6 months. Patients who
had retinopathy of prematurity, traumatic cataract,
aniridia, steroid-induced cataract, posterior persistent fetal
vasculature causing stretching of the ciliary process or trac-
tional retinal detachment, or glaucoma before cataract
extraction were excluded. Also, we excluded patients
with Down syndrome or systemic diseases such as
Hallermann-Streiff syndrome or Lowe syndrome, which
are independently associated with glaucoma.24–27
All of the cataract surgeries were performed under gen-
eral anesthesia and by 1 experienced pediatric surgeon
(Y.S.Y.) using a previously reported method.28,29 All of
the patients underwent simultaneous irrigation and
aspiration (I&A) of the lens, posterior capsulectomy, and
anterior vitrectomy in both eyes within 2 weeks of
diagnosis of congenital cataract. Preoperative anterior
segment and fundus examinations and B-scan
ultrasonography were performed with the patients sedated
by oral chloral hydrate. The corneal diameters of all of
the patients were measured during cataract surgery. A
precise fundus examination for glaucomatous optic disc
damage was performed immediately after cataract
extraction because the efficacy of preoperative fundus
examination was limited owing to dense cataract. Two
years after cataract extraction surgery, patients with eyes
having a corneal diameter > _9 mm were operated on for
secondary intraocular lens (IOL) implantations.
In each cataract surgery, mechanical anterior capsulor-
rhexis, I&A of lens, posterior capsulectomy, and anterior
vitrectomy were performed using a vitrector. Aphakic
correction with glasses was started 1 week after surgery.
Secondary IOL (1-piece polymethylmethacrylate; 811 B,
Pharmacia & Upjohn, Inc, Kalamazoo, Michigan, USA;
LK55 A, Lucid Korea Co, Ltd, Seoul, Korea) implantations
were performed through a 6-mm-sized scleral tunnel inci-
sion at 2 mm from the superior limbus. For adequate poste-
rior capsule support, the IOL was inserted into the capsular
bag or fixated in the ciliary sulcus. In all cases, sodium
hyaluronate 1.4% (Healon GV; AMO Inc, Santa Ana,
California, USA) was used, and the scleral tunnel incisions
were closed with interrupted 8-0 polyglactin sutures.
All of the patients were examined every 4 months until 4
years, and every 6 months until 8 years. Thereafter, they
were followed up annually. During the regular follow-up
visits after cataract surgery, visual acuity, manifest or
cycloplegicV refraction, and intraocular pressure (IOP)
266 AMERICAN JOURNAL OF OPHTHALMOLOGY
were measured. Visual acuity was measured with the
Snellen chart for patients over 3 years of age, then
converted to the logMAR scale for statistical analysis.
IOPs were measured using (1) a Goldmann applanation
tonometer (AT 900, Haag-Streit, Koeniz, Switzerland)
for children aged >_8 years old or (2) a noncontact tonom-
eter (Keeler Instruments, Inc, Broomall, Pennsylvania,
USA), Tono-Pen XL (Mentor, Santa Barbara, California,
USA), or rebound tonometer (Icare PRO; Icare Finland
Oy, Helsinki, Finland) for children aged younger than 8
years or those who were uncooperative. When 2 or more to-
nometers were used, the mean IOP value was used for the
analysis.
Glaucoma was diagnosed if the patient’s IOP was >_26 mm
Hg on at least 2 consecutive visits30,31 or if there was a
documented clinician’s decision for surgical intervention or
permanent medical glaucoma therapy.14,19,25 Gonioscopic
examination and optic nerve and visual field evaluations
were not uniformly performed in our study population
because patients could not cooperate consistently with
those examinations.
Patients diagnosed with glaucoma were categorized by
IOP control: IOP < _21 mm Hg on all post–cataract surgery
visits with topical medication (Group Bs) or >21 mm Hg
on at least 2 visits despite IOP-lowering medication or
IOP-lowering surgery required (Group Bf).32 During the
postoperative follow-up, patients with uncontrolled IOP
despite medical therapy underwent Ahmed valve implan-
tation (AVI) by 1 glaucoma specialist (K.H.P.).
All of the AVI surgeries were performed using the
Ahmed valve (model FP-7 or FP-8). A fornix-based
conjunctival incision was made in the superior temporal
quadrant or superior nasal area. After the sclera was
exposed, a partial-thickness limbal-based scleral flap was
cut in a rectangular shape. The tube was primed using
balanced salt solution, and the Ahmed drainage plate
was tucked under the subconjunctival space and sutured
to the underlying sclera with its anterior edge 6-8 mm
posterior to the limbus, using 2 8-0-nylon anchoring
sutures.

STATISTICAL ANALYSIS: Descriptive statistics were
calculated for the individual-level characteristics of the
19 patients and for the eye-level characteristics of the
38 eyes. A Kaplan-Meier method was used to calculate
the probability of glaucoma development, as well as the
respective 95% confidence intervals, on a per-eye basis.
Inter-group comparisons were performed using the
Mann-Whitney U test or Fisher exact test. Factors asso-
ciated with the development of glaucoma were assessed
by logistic regression analysis. Variables with a statistical
significance of P < .10 in the univariate analysis were
included in the multivariate model. The analyses were
performed using SPSS software version 22.0 (IBM
Corp, Armonk, New York, USA). The cutoff for statisti-
cal significance was set at P < .05.
DECEMBER 2019

RESULTS

CLINICAL CHARACTERISTICS OF STUDY POPULATION:
From 1997 to 2008, 42 eyes of 21 patients with micro-
phthalmia underwent bilateral cataract surgery within
6 months of birth. Among them, 4 eyes of 2 patients
were excluded from our study because they had syndromes
known to be associated with increased risk of glaucoma
(Hallermann-Streiff syndrome, 2 eyes of 1 patient; Lowe
syndrome, 2 eyes of 1 patient). Finally, 38 eyes of 19 pa-
tients were enrolled in this study. The patients underwent
I&A of the lens, posterior capsulectomy, and anterior vit-
rectomy at the age of 3.2 6 1.7 months (range, 3-29 weeks);
only 1 infant underwent cataract surgery within 4 weeks of
birth. All of the patients were followed up for 7.8 6 2.6
(range, 5.2-14.4) years. Eleven patients (57.9%) were
male, and 10 (52.6%) had a family history of congenital
cataract. Preoperative IOP was 11.0 6 4.5 mm Hg, axial
length was 16.49 6 1.76 mm, and corneal diameter was
8.28 6 0.93 mm. After cataract surgery, 5 eyes underwent
removal of visual axis opacification and 12 eyes of 6 pa-
tients (31.6%) underwent secondary IOL implantation at
the age of 2.3 6 0.5 months. None of the eyes underwent
any other intraocular surgery except secondary IOL im-
plantation, glaucoma surgery, or visual axis opacification
removal. The final IOP and best-corrected visual acuity
were 17.3 6 3.8 mm Hg and 0.93 6 0.81 logMAR, respec-
tively (Table 1).

DEVELOPMENT OF GLAUCOMA AFTER BILATERAL
CONGENITAL CATARACT SURGERY: Eleven of 38 eyes
(29.0%) were diagnosed with glaucoma at the patient
age of 4.1 6 1.4 years. Of them, 4 patients had bilat-
eral glaucoma. The Kaplan-Meier survival analysis
result for risk of development of glaucoma was 32.0%
by 10 years after bilateral cataract surgery, and the
mean glaucoma-free time was 10.4 6 0.7 (95% confi-
dence interval, 9.0-11.9) years (Figure). All of the
eyes were of the late-onset open-angle type, without
family history of glaucoma. Only 1 eye among the glau-
coma group underwent secondary IOL implantation,
and 10 eyes of 6 patients remained in aphakia status
owing to their small corneal diameter (<9 mm) during
the follow-up period.
The mean IOP of the glaucoma group was 20.1 6 4.1
(14.4-29.7) mm Hg. In 6 of those eyes, IOP was well
controlled to below 21 mm Hg with topical glaucoma
medication (mean IOP, 17.73 6 2.06 mm Hg). The
remaining 5 eyes’ IOPs were poorly controlled during
the follow-up period (mean IOP, 22.90 6 4.34 mm
Hg). Consequently, 3 eyes underwent AVI surgery at
the patient ages of 5, 6, and 8 years, respectively. The
final IOP of the eyes in the glaucoma group was 19.3
6 3.3 (range, 14-23) mm Hg under topical glaucoma
medication; the average number of glaucoma medications
was 1.5.
VOL. 208 GLAUCOMA AFTER CONGENITAL CATARACT SURGERY IN MICROPHTHALMIA

COMPARISON OF EYES ACCORDING TO DEVELOPMENT
OF GLAUCOMA AND RISK FACTOR ANALYSIS FOR GLAU-
COMA DEVELOPMENT: The eyes were grouped according
to development of glaucoma: Group A, without glaucoma;
Group B, with glaucoma. Table 2 shows the clinical char-
acteristics of the patients. The eyes in Group B had a
shorter mean axial length at the time of cataract surgery
when compared with Group A (15.11 6 1.38 vs 16.95 6
1.66, P ¼ .036). In contrast, there were no significant
inter-group differences in the proportion of male patients
(55.6% vs 63.6%, P ¼ .729), the mean age of patients at
the time of surgery (3.3 6 1.7 months vs 3.0 6 1.7 months,
P ¼ .660), corneal diameter (8.37 6 0.99 mm vs 8.05 6
0.76 mm, P ¼ 0.334), or the mean follow-up period (7.31
6 2.09 years vs 8.96 6 3.46 years, P ¼ .278) (Table 2).
Group A had lower max and mean IOPs (19.9 6 4.6 mm
Hg vs 36.9 6 9.1 mm Hg and 13.5 6 2.2 mm Hg vs 20.1
6 4.1 mm Hg, respectively, P < .001) with better visual
outcomes (0.75 6 0.60 logMAR vs 1.47 6 1.10 logMAR,
P ¼ .049) than Group B. In the logistic regression analysis
to assess the factors associated with glaucoma development
after cataract surgery, only axial length was significantly
associated (P ¼ .025) (Table 3).
During the follow-up, 3 eyes of 3 patients in Group A
that showed temporary IOP elevation above 21 mm Hg
were not included in the glaucoma group, since the max
IOP was less than 26 mm Hg and dropped to within the
normal range without treatment.

INTRAOCULAR PRESSURE CONTROL AND VISUAL OUT-
COMES IN GLAUCOMA PATIENTS: In order to evaluate the
association between IOP control and visual outcomes, we
categorized eyes in Group B according to the success of
IOP control; successful IOP control (Group Bs) has been
defined as patient IOP <
_21 mm Hg regardless of glaucoma
medications and failed IOP control (Group Bf) as patient
IOP >21 mm Hg despite glaucoma mediation on at least
2 visits or when surgery for IOP control was needed.32 Of
the 11 eyes in Group B, 6 eyes were grouped in Bs and
the remaining 5 in Bf. In the analysis of visual outcome,
Group Bs had a significantly better final vision (0.69
6 0.42 logMAR) than did Group Bf (2.40 6 0.89
logMAR) (P ¼ .014). However, patients in Group Bs had
a comparable final visual acuity to those without glaucoma
(P ¼ .907) (Table 4).
DISCUSSION
WE EVALUATED THE LONG-TERM RISK OF GLAUCOMA AND
the visual outcomes in infants with microphthalmia who
had undergone bilateral congenital cataract surgery within
6 months of birth. In our study, 11 of 38 eyes (29.0%)
developed late-onset glaucoma after bilateral congenital
cataract surgery. The long-term cumulative incidence of
glaucoma development was 32.0% by 10 years of bilateral
267
 TABLE 1. Clinical Characteristics of Study Population (38
Eyes of 19 Patients)

Variables Value

Sex (%)
Male 57.9%
Female 42.1%
Family history of congenital cataract 52.6%
Type of cataract (eyes), n (%)
Nuclear 21 (52.5%)
Total 15 (37.5%)
Anterior subcapsular 2 (5.0%) FIGURE. Kaplan-Meier plot of glaucoma-free survival in chil-
Age at initial visit (months) 2.6 6 1.6 dren with microphthalmic eyes after bilateral congenital cata-
Age at cataract extraction surgery (months) 3.2 6 1.7 ract surgery.
Preoperative ocular parameters
Axial length (mm) 16.49 6 1.76
Corneal diameter (mm) 8.28 6 0.93
Secondary IOL insertion surgery 12 eyes of were 18% and 32%, respectively.20 When we applied the
6 patients disease definition used in IATS to our data, the incidence
Age at surgery (years) 2.3 6 0.5
of glaucoma plus glaucoma suspect was 36.8%, which,
IOP
though higher than the IATS result, was not significantly
Preoperative IOP (mm Hg) 11.0 6 4.5
Mean IOP after cataract surgery (mm Hg) 15.4 6 4.1
different. In a study by Vasavada and associates,18 the
Final IOP (mm Hg) 17.3 6 3.8 risk of glaucoma development was 30.9% in 21 infants
Eyes diagnosed with glaucoma (eyes), n (%) 11 (29.0%) followed up for 25.6 6 11.3 months postoperatively,
Age at glaucoma diagnosis (years) 4.0 6 1.4 whereas Prasad and associates17 reported a lower incidence
Glaucoma surgery (eyes)a 3 (7.9%) of glaucoma: 13.5% of 37 microphthalmic eyes followed up
Age at glaucoma surgery (years) 6.3 6 1.5 for 18 6 5.1 months. This discrepancy might reflect differ-
Final BCVA (logMAR) 0.6 6 0.8 ences in the follow-up duration and patient population be-
Follow-up time (years) 7.8 6 2.6 tween the studies.
In our study, all of the patients with glaucoma had late-
BCVA ¼ best-corrected visual acuity; IOL ¼ intraocular lens;
IOP, intraocular pressure.
onset open-angle glaucoma and those with shorter axial
a
All cases underwent Ahmed valve implantation. length had a higher risk for developing glaucoma. Glau-
coma after congenital cataract surgery has a bimodal
onset33,34; early-onset glaucoma develops within a year of
cataract surgery, and late-onset open-angle glaucoma arises
much later.19,30 The mechanism of early-onset glaucoma
congenital cataract surgery. Although eyes without glau- might be related to genetic predisposing factors13,14,30,35
coma had better visual outcomes than those with glau- along with angle closure caused by pupillary block or
coma, glaucomatous eyes with well-controlled IOP had a inflammatory peripheral anterior synechiae (PAS)36–38;
comparable visual outcome to those without glaucoma. when glaucoma occurs, it tends to develop in both eyes
This study included patients on whom bilateral congen- (ie, bilaterally).14,30 In late-onset glaucoma, although the
ital cataract surgery had been performed, using modern sur- angle is usually open on gonioscopy, many cases have
gical techniques, by 1 experienced pediatric surgeon in abnormal pigment deposition and micro-PAS39 that might
order to exclude bias such as surgeon performance. More- be the result of early intermittent pupillary block and
over, only patients followed up for at least 5 years were chronic inflammation by remnant lens materials after sur-
included to achieve representative long-term results; in gery.33 Two possible mechanisms of late-onset glaucoma
our study, 10 of the 11 cases of glaucoma were diagnosed have been suggested. The first is the chemical theory that
within 5 years of surgery and the remaining 1 patient was may be associated with unique susceptibility of filtration
diagnosed at 8 years. Thus, this study can be considered angle to postoperative inflammation13 or toxic chemical
to have had a sufficient follow-up period for evaluation of factors from vitreous fluid38,40 to induce chronic
the long-term incidence of glaucoma development. trabeculitis15 that alter the microstructure of the trabecular
The incidence of glaucoma after congenital cataract sur- meshwork and may impair the normal maturation of angle
gery has been reported to be between 3% and 41%.15–20 In or acquire micro-PAS.39 The second is a mechanical the-
the recent prospective Infant Aphakia Treatment Study ory; loss of support to the trabecular meshwork in the
(IATS), the incidences of glaucoma and glaucoma plus aphakic eye induces a disorganization or collapse of the
glaucoma suspect after surgery by 5 years of follow-up trabecular meshwork that results in diminished function

268 AMERICAN JOURNAL OF OPHTHALMOLOGY DECEMBER 2019

 TABLE 2. Comparison of Patients’ Clinical Features According to Glaucoma Development

Variables Group A (Without Glaucoma) Group B (With Glaucoma) P Valuea

Number of eyes 27 11
Male (%) 15 (55.6%) 7 (63.6%) .729
Right:left (% of right eye) 13:14 (48.1%) 6:5 (54.5%) 1.00
Age at cataract surgery (months) 3.3 6 1.7 3.0 6 1.7 .660
Corneal diameter (mm) 8.37 6 0.99 8.05 6 0.76 .334
Axial length (mm) 15.11 6 1.38 16.95 6 1.66 .036*
Secondary IOL implantation 11 eyes (40.7%) 1 eye (9.1%) .121
Intraocular surgery after cataract surgery 14 eyes (51.9%) 3 eyes (27.27%) .486
IOP
Max IOP (mm Hg) 19.9 6 4.6 36.9 6 9.1 <.001*
Mean IOP (mm Hg) 13.5 6 2.2 20.1 6 4.1 <.001*
Final IOP (mm Hg) 16.4 6 3.7 19.3 6 3.3 .034*
Final BCVA (logMAR) 0.75 6 0.60 1.47 6 1.10 .049*
Follow-up period (years) 7.31 6 2.09 8.96 6 3.46 .278

BCVA ¼ best-corrected visual acuity; IOL ¼ intraocular lens; IOP, intraocular pressure.
Asterisk (*) indicates statistical significance.
a
P values of continuous variables from Mann-Whitney U test, and those of categorical variables from Fisher exact test.

TABLE 3. Risk Factors Associated With Development of Glaucoma After Cataract Surgery

Univariate Analysis Multivariate Analysis

Variables Odds Ratio (95% CI) P Value Odds Ratio (95% CI) P Value

Age at cataract surgery, per 1 month older 0.896 (0.585-1.370) .611

Sex, female 0.714 (0.169-3.027) .648
Cornea diameter, per 1 mm thicker 0.676 (0.310-1.477) .326
Axial length, per 1 mm longer 0.364 (0.150-0.882) .025* 0.364 (0.150-0.882) .025*
Aphakia 6.875 (0.766-61.694) .085 0.803 (0.024-27.325) .903
Intraocular surgery after cataract extraction 0.469 (0.102-2.162) .331
surgery

Asterisk (*) indicates statistical significance.

CI ¼ confidence interval.
Variables with P < .1 in the univariate analysis were included in the multivariate model.

of trabecular meshwork as a filter and an active metabolic
tissue.41 Extremely early onset of cataract, subsequent sur-
gery in such young microphthalmic eyes, and associated
anomalies of the angle structures with peripheral crowding
might be risk factors for the development of glaucoma.42
These theories can be possible pathophysiological mecha-
nisms for the higher prevalence of postoperative glaucoma
in infants with microphthalmic eyes, and support the result
that eyes with shorter axis length had higher risk of glau-
coma after surgery in the present study.
Among the known risk factors for development of glau-
coma after cataract surgery in children, surgery at an early
age has been frequently identified in both normal and
microphthalmic eyes.17,18,20,21,33,35 However, in our
study, the age at surgery tended to be younger in the
glaucoma than in the nonglaucoma group, but it was not
statistically significant. This could have been due to the
facts that only 5 of 19 infants underwent surgery within
2 months of birth and that the number of eyes was too
small to conduct a meaningful statistical analysis.
Glaucoma after congenital cataract surgery remains a
major concern for microphthalmic eyes, and it needs timely
management to prevent optic nerve damage and subse-
quent vision loss. Although early surgery is associated
with a higher risk of subsequent glaucoma,21,33,35 timely
intervention is important to visual outcomes for the
following reasons: surgery after 6 weeks of age can
deprive the neonatal visual system of vital stimuli during

VOL. 208 GLAUCOMA AFTER CONGENITAL CATARACT SURGERY IN MICROPHTHALMIA 269

 TABLE 4. Visual Outcomes According to Postoperative Intraocular Pressure Control

Glaucoma (Group B)a

Variables Nonglaucoma (Group A) Group Bs Group Bf P Valueb Post Hoc Analysisc

Number of eyes 27 6 5
Mean IOP (mm Hg) 13.51 6 2.22 17.73 6 2.06 22.90 6 4.34 <.001* A < Bs < Bf
Final BCVA (logMAR) 0.75 6 0.60 0.69 6 0.42 2.40 6 0.89 .007* A ¼ Bs < Bf

BCVA ¼ best-corrected visual acuity; IOP ¼ intraocular pressure.

Asterisk (*) indicates statistical significance.
a
The success of IOP control (Group Bs) was defined as patient’s IOP <
_21 mm Hg regardless of glaucoma medications and the failure of IOP
control (Group Bf) was defined as patient’s IOP >21 mm Hg despite glaucoma mediation in at least 2 visits or when reoperation for glaucoma
was needed.
b
Kruskal-Wallis test.
c
Post hoc test by Mann-Whitney U test.

the critical period,43 and cataract surgery before 2 months
of age has produced the best visual outcomes.44,45 In our
study, the surgeon’s concern about postoperative
glaucoma did not delay cataract surgery, even in patients
with bilateral cataracts. The surgery was performed at 3.2
6 1.7 months, similarly to the findings reported by
Vasavada and associates (4.0 6 2.6 months)18 and Prasad
and associates (3.8 6 2.3 months).17 Most of the patients
in the current study underwent surgery within 2 weeks of
initial diagnosis of the disease, regardless of their age, and
they were more likely to be operated on earlier when
referred earlier. However, some patients who had a delayed
parental consent underwent cataract surgery later.
The present study included children with microphthal-
mia who underwent bilateral congenital cataract surgery
because there were no patients with unilateral disease
who met our inclusion criteria. Because the number of pa-
tients who performed surgery was not correlated with the
prevalence of the disease, we speculate that the reasons
for this are as follows: (1) infants with unilateral disease
might be diagnosed and operated on later than those
with bilateral cases because visual signs (ie, decrease in vi-
sual acuity) in preverbal infants are less helpful for early
detection of cataract in cases of unilateral disease; and
(2) the most frequent ocular abnormality in cases of unilat-
eral cataract and microphthalmia is persistent fetal vascu-
lature.2,46,47 In such condition, patients’ parents often
refuse to consent to surgery owing to its poor prognosis,
especially when fellow eyes are normal. Since bilateral
congenital cataracts in microphthalmia are more likely to
be syndromic or have other systemic findings that could
influence the prevalence of glaucoma, our findings are
relevant only to patients who have surgery for bilateral
congenital cataracts. Therefore, further study with
unilateral congenital cataract surgery in microphthalmia
is needed.
This study has several limitations. First, it is a retrospec-
tive review of a small sample size without a control group.
Second, we analyzed the long-term outcomes for a homoge-
neous cohort of microphthalmic eyes that had undergone
bilateral congenital cataract surgery within 6 months of
birth; all of the patients were Korean. Though the results
from this cohort can provide useful data for this population,
it may not apply as accurately to other racial or ethnic
groups. Therefore, further prospective study with a larger
sample size and a more diverse study population, along
with comparison of these eyes with nonmicrophthalmic
eyes, is needed. Third, given the present study’s limitations
in terms of the capacity to evaluate glaucomatous anatomic
and functional damage, the mechanism of glaucoma and
the severity of glaucomatous structural and functional dam-
age could not be comprehensively evaluated. Lastly,
because we included patients who were followed up for at
least 5 years postoperatively, some cases, especially uncom-
plicated ones, could not be included owing to insufficient
follow-up. Thus, there is a possibility that our data overes-
timate the risk of developing glaucoma.
In summary, the long-term cumulative risk of glau-
coma development by 10 years after bilateral congenital
cataract surgery was 32.0% in patients with microphthal-
mia. Since the risk of glaucoma development persists for
many years after surgery, regular IOP monitoring and
thorough IOP controls are needed in order to preserve
patients’ vision.

FUNDING/SUPPORT: THIS STUDY WAS SUPPORTED BY GRANT NUMBER 0420170680 (2017-1182) FROM THE SNUH RESEARCH FUND.
Financial Disclosures: All authors have no financial disclosures. All authors attest that they meet the current ICMJE criteria for authorship.

270 AMERICAN JOURNAL OF OPHTHALMOLOGY DECEMBER 2019

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