CASE REPORT

Isolated Congenital Cataract in Dizygotic Twin Babies

Focusing on Comprehensive Management of Cataract and
Visual Rehabilitation : A Case Report

Abstract
INTRODUCTION: Pediatric cataract is one of major causes of childhood blindness. Prevalence of congenital
cataract has been reported from 1 to 15 per 10.000 children worldwide. Congenital cataract in twin babies is a
rare case. This case will report the outcome of management of congenital cataract in dizygotic twin babies.
CASE PRESENTATION: Eight month old dizygotic twin babies came to our clinic with main complaint of whitish
appearance in the pupil of both eyes since birth. They were born aterm with sc delivery and the birth weight
was 2700 gram and 3100 gram. They were 4 thand 5th children in the family. There is no family history of
congenital cataract in the family. The visual acuity both eyes were response to light and had blink reflex
positive. The cataract were bilaterally and densed on both babies. Fundus reflex were negative. Torch
examination were negative and USG of retina were normal on both babies .
DISCUSSION: Isolated congenital cataract in twin babies is an unusual condition that should be treated
comprehensively. Congenital cataracts produce deprivation amblyopia, refractive amblyopia,and leading to
lifelong visual impairment. Successful management is dependent on early diagnosis and referral for surgery
when indicated. Here we present a case of isolated bilateral cataracts in a dizygotic twin babies.
Comprehensive management is needed to follow up the visual outcome after cataract surgery.
CONCLUSION: Isolated congenital cataract in dizygotic twin babies is a rare case. Early diagnostic and
comprehensive management including cataract surgery and visual rehabilitation should be done early to visual
result.

Keywords: congenital cataract; dizygotic twin babies; visual rehabilitation

Introduction lenses or intraocular lenses (IOL) implantation

A pediatric cataract is one of the major were highly recommended.1,2,3
causes of preventable childhood blindness, Isolated congenital cataract in dizygotic
affecting approximately 200.000 children twin babies is a rare case. To the best of our
worldwide. The prevalence of congenital knowledge there is no case report in our
cataracts has been reported from 1 to 15 per hospital about it. This case report covers
10.000 children worldwide, with an estimated clinical condition, initial management, and
prevalence ranging from three to six per follow-up visual rehabilitation management.
10.000 live births. In the United States, the Comprehensive treatment is needed for these
incidence is 2.0 per 10,000 births. In China, this case. Early detection and prompt
the incidence of congenital cataract is treatment provide a satisfactory result of
approximately 5.0 per 10,000 births, and 22% visual outcome
to 30% of childhood blindness is attributed to
congenital cataract in the absence of
appropriate treatment, with delayed
presentation to hospitals and late surgical
treatments found to be the major causes of
blindness and visual impairment. The
morphology of congenital cataracts was
genetic etiology, specific metabolic disorders,
associated ocular anomalies or systemic
findings, and optical correction for congenital
cataracts with aphakic glasses or contact
Case presentation

Eight month old dizygotic twin babies medication of dexamethasone 0.5 mg tablet
came to our clinic with complained of whitish were given every eight hours orally, and
appearance in the pupil of both eyes since paracetamol syrup 5 ml were given every
birth. There was also nystagmus on both eight hours orally. Optical visual rehabilitation
babies, since they were three months old. for these babies were aphakic glasses with the
These babies born aterm with section prescription for twin 1 (MCL) +16.00 D and
caesarean delivery. The birth weigh was 2700 twin 2 (MVL) +17.00 D. Since these babies
gram and 3100 gram. They were the 4 th and 5th were only eight monts old, implantation of
child of the family. There is no history of intra ocular lens is postponed until they
congenital cataract in the family. The visual become 2 years old.
acuity of the right and a left eye was fix and
follow to light stimuli. Anterior segment
examination reveals bilateral dense cataract
on both babies. Fundus reflex and posterior
segment of the eyes was unable to evaluate.
TORCH serology examination was negative
and Ultrasonography (USG) of both eyes was
normal on both babies.
Cataract surgeries on both babies were
performed with the technique of simple
aspiration without IOL implantation. Figure 1
was anterior segment and figure 2 was the
biometry of both babies. From Examination
under anesthesia, twin 1 cornea diameter RE
horizontal and vertical were 11.5 mm and LE
horizontal and vertical were 11 mm and twin 2
RLE horizontal and vertical were 12 mm. IOP
twin 1 RLE were 14.6 mmHg and twin 2 RE
was 12.2 mmHg and LE was 17.3 mmHg.Twin
1 keratometri RE K1 7.99/ K2 7.50 and LE K1
8.10 / K2 7.58 and twin 2 keratometri RE K1
6.92 / K2 6.41 and LE K1 7.58 / K2 7.12. Streak
twin 1 RLE +16.00 D and twin 2 RLE +17.00 D.
From anterior segment examination RLE on
both babies were found Fibrotic posterior
capsule in central and RLE posterior polaris
cataract. For Posterior segment examination
RLE on both babies were normal.
Visual acuity after surgery were 2 cpcm
(57 cm) ~ 2 cpd ~ 6/90. Visual rehabilitation
start with prompt post surgical medication.
These twin babies were treated with
tobramycin antibiotic and dexamethasone
steroid eyedrop every four hours for the right
eye dan every six hours for the left eye,
anticholinergic homatropine 2% eyedrop two
drops every eight hours for both eye. Oral
 RE RE LE
LE

Twin 1 MCL Twin 2 MVL

Figure 1. Twin anterior segment, The cataract were bilaterally and densed on both eyes

RE LE RE LE

USG Twin 1 MCL USG Twin 2 MVL

Figure 2. Twin USG for both eyes were normal

Table 1 The biometry of the twin.

Biometry Twin 1 MCL Biometry Twin 2 MVL

 RE LE RE LE

Twin 1 MCL Twin 2 MVL

Figure 3. Twin Anterior segment in first operation (on May 2021) with right eyes were
opaque lens, and left eyes were aphakia.

RE RE LE
LE
Twin 1 MCL Twin 2 MVL

Figure 4. Twin Anterior segment in second operation (on June 2021) with right and left eyes
were aphakia.

Twin 1 MCL Twin 2 MVL

Figure 5. Twin chromosomal examination were normal

Discussion and conclusions two fetuses. A pediatric cataract is one of the

Cataracts present at birth or develop
within the first year of life are called
congenital or infantile cataracts. Congenital
cataract in twin babies is an unusual condition
that should be assessed for the causes. About
one-third of the cases congenital cataract fall
into the group inherited without systemic
abnormality. Congenital hereditary cataracts
in a dizygotic twin pregnancy with discrepancy
in body weight and affected status between
major causes of preventable childhood
blindness. Congenital cataracts can be
classified by morphology, presumed or
defined genetic etiology, specific metabolic
disorders, or associated ocular anomalies or
systemic findings. There are some classified
causes of congenital and infantile cataract,
intrauterine infection or maternal infection
embryopathies such as rubella,
cytomegalovirus, varicella-herpes zoster,
herpes simplex, toxoplasmosis, syphilis,
Epstein–Barr virus, measles, poliomyelitis
intrauterine drug exposure, intrauterine
ionizing radiation, a prenatal or perinatal
metabolic disorder such as galactosemia,
hereditary without associated systemic
disorder such as autosomal dominant,
autosomal recessive, or X-linked recessive,
Hereditary with the associated systemic
disorder or multisystem dysmorphic
syndrome such as chromosomal trisomy 21,
turner’s syndrome, trisomy 13–15, trisomy
16–18, deletion chromosome 5, or idiopathic
(without a recognized cause). From this case,
twin chromosomal examination were normal
and the TORCH examination results were
negative on both babies. So we can conclude
that the cataract on these dizygotic twin
babies was isolated. To assure the successful
of the surgical results, parents education
should be given. Parents should be informed
that surgery is a starting point and not the
endpoint of treatment. and need long time
follow up and compliance with all of the visual
rehabilitation management including wearing
aphakic glasses and occlusion therapy with
eye patch. Appropriate postoperative
management, including an immediate optical
correction in the form of aphakic glasses or
contact lenses or intraocular lens (IOL)
implantation at the appropriate age (>1 year),
is highly recommended. Timing of surgery is
critical for congenital cataract prognosis, it
has also been reported that the presence of
other associated ocular abnormalities, such as
nystagmus, strabismus, PHPV (persistent
hyperplastic primary vitreous, CPPM
(congenital persistant pupillary membrane),
small eyes, and small corneal disorders, also
has a significant effect on prognosis. After
surgery, amblyopia treatment and periodic
follow-up examinations should be started as
soon as possible to achieve a satisfactory
visual outcome. 1-8
Twin Babies was examined under
anesthesia and anterior segment finding
were fibrotic posterior capsule in central and
right-left eye posterior polaris cataract. Twins
will recipe aphakic glasses while waiting for
IOL implantation. The first and second surgery
were aphakia for eyes twin babies because, in
children younger than two years, the baby eye
has smaller axial lengths, shallower anterior
chambers, corneal curvature changes, and
corneal thickness changes lead to higher
prediction errors. Various factors, including
inaccuracies of axial length measurements in
the supine position, biometry technique,
keratometry values, phenomenon of pseudo
accommodation, variability in IOL position due
to capsule fibrosis, and increased
corneoscleral elasticity in children, have been
suggested to contribute to prediction errors. 2
Twin babies will do four times surgery
for two times simple aspiration for the
congenital cataract and become aphakia
because in children younger than two years,
smaller axial lengths, shallower anterior
chambers, and changes in corneal curvature
and corneal thickness may lead to higher
prediction errors and two times surgery the
other for implantation IOL for the twin babies.
For visually significant, unilateral cataract was
recommend cataract extraction at 6 weeks
and bilateral cataract extraction between 6
and 8 weeks of age with a 1–2 week period
between lensectomy for each eye. For infants
less than 6 months of age, ophthalmologist
was not routinely IOL implant and for children
at least 2 years of age, ophthalmologist was
implant IOL because there was sufficient
capsular support. Cataract surgery in children
had been rapidly evolving with improved
surgical techniques, instrumentation and
newer designs of IOLs. IOL selection in
children is challenging due to normal ocular
growth, low predictability of lens calculators
for shorter eyes, and difficulty attaining IOL
parameters in awake children. Current lens
formulas require axial length and corneal
curvature (K) measurements to predict final
refractive outcome. For young and
uncooperative patients who need sedation for
biometric measurement are more likely to
show greater biometric error because of lack
of fixation. It has been reported that in eyes
with shorter axial length and steeper cornea,
Hoffer-Q formula tends to be more sensitive
to the biometric errors than other theoretical
formulae such as SRK/T, Holladay I and Haigis
formula, while SRK/II has an invariable
sensitivity to the size of biometric error.
Nihalani and Van der Veen have reported that
among patients who underwent IOL
implantation for pediatric cataract before 18
years of age, Hoffer-Q is the most predictive
formula but Lee et al study have reported that
SRK/II was the most predictive formula across
all age subgroups, and Hoffer-Q was the least
predictive one for patients who received IOL
implantation surgery during their first decade
of life. In Lee et al study also reported that
age at IOL implantation has significant
correlation with prediction error calculated by
SRK/II, SRK/T and Hoffer-Q formula. After 10
years of age, all three formula have equally
satisfactory predictability. However, when
choosing IOL calculation formula in patients
with congenital cataract before 10 years of
age, SRK/ II formula consistently provides the
most relevant prediction among the three
formula evaluated. Most pediatric surgeons
implant hydrophobic one-piece acrylic lenses
into the capsular bag or three-piece lenses
into the ciliary sulcus. Although multifocal and
accommodating lenses can provide good
vision at distance and near, myopic shift in
infants prevents the refractive accuracy
necessary for appropriate IOL selection. The
use of IOLs for correction of surgical aphakia
after 2 years of age has become a standard
practice for most ophthalmologists. Primary
IOL implantation when combined with
primary posterior capsulorhexis and limited
anterior vitrectomy is now showing promising
results in children below 2 years of age. Visual
axis opacity is a common complication
following pediatric cataract surgery. In efforts
to reduce the high incidence of visual axis
opacity, lensectomy routinely involves
posterior capsulotomy with limited anterior
vitrectomy in patients less than 4 years of age.
When planning IOL implantation especially in
younger children, it is important to discuss
with the parents regarding the variations from
the predicted final refractions and the need
for subsequent IOL exchange. In a multicentric
Delphi consensus study by Serafino et al, the
target refraction was slightly higher, 6–10 D
for children < 6 months, 4–6 D for 6 months–1
year, 4 D for 1–3 years and 3 D for 3–4years. if
a high hyperopic refractive error is chosen in
the immediate postoperative period, the
visual rehabilitation and occlusion can
become quite challenging both for the
surgeon and for the parents because high
hyperopia might prevent IOL exchanges, but
at the same time causes a denser amblyopia
and worse visual acuity outcome. Primary IOL
gives good structural and functional results in
children with congenital cataract. The visual
outcome is affected by the time of
presentation and postoperative compliance to
amblyopia therapy. In addition to IOL
implantation, it is equally important to
carefully monitor and correct the refractive
error in the postoperative period to prevent
amblyopia. They also were examined
chromosomal disorders to detect morphology
causes of hereditary congenital cataracts but
from this cases, chromosomal examination
was normal. Holistic treatment is needed for
these cases from cataract division and
pediatric ophthalmology to follow up the
visual outcome and visual rehabilitation after
cataract surgery. Holistic management can
provide a satisfactory result. 4-7
Congenital cataract in twin babies is an
uncommon condition. Awareness of the
specific causes and visual condition of the
twin babies must be diagnosis and treated
earlier. Appropriate and holistic management
can provide satisfactory results and would
give a good result.
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