American Journal of Medicine and Medical Sciences 2023, 13(11): 1795-1797
DOI: 10.5923/j.ajmms.20231311.43
Features of Management of Children with Congenal
Cataracts and Accompanying Visual Pathology
Abdurakhmanova Charos, Buzrukov Batir, Makhmudova Dilorom, Abdullaeva Durdona
Tashkent Pediatric Medical Institute, Tashkent, Uzbekistan
Abstract
An analysis of the case histories of 72 children (117 eyes) diagnosed with congenital cataracts, who were
hospitalized in the eye department of the TashPMI clinic for the period from 2016 to 2023 at the age of 3 months to 14 years,
was carried out. The study revealed that, children with congenital cataracts and concomitant pathology of the visual organ
require a differentiated approach to patient management: in the presence of microphthalmos, early surgical intervention is
necessary before 1 year - stage 1, including cataract extraction, followed by stage 2 - IOL implantation. Constant dynamic
monitoring is necessary, given the high percentage of postoperative complications.
Keywords
Congenital cataract, Concomitant pathology, Microphthalmia, IOL implantation
1. Introduction
One of the common causes of blindness and low vision in
children is congenital pathology of the lens, namely cataracts,
which account for up to 10-19.5% [5,7,9,16]. Congenital
cataracts (CC) are observed in 5 cases out of 100,000
newborns and cause 10-38% of cases of childhood blindness
[5]. Cataract is a persistent clouding of the substance of the
lens or its bag, accompanied by a decrease in visual acuity
from a slight weakening to light perception. Congenital cataracts
are often combined with other pathological changes in the
organ of vision, which are observed in 36.8-77.3% of children:
strabismus, nystagmus, microphthalmos, microcornea and
other anomalies of the cornea, as well as the vitreous div,
choroid, retina and optic nerve. Strabismus is observed in
30.2-83.3% of children with congenital cataracts. More often
it is converging, alternating, predominantly constant. Nystagmus
is observed in 14.0-58.8% of children with congenital
cataracts; it can be congenital or acquired, associated with a
sharp decrease in vision. More often it is pendulum-shaped,
horizontal, less often - jerky. Microphthalmos is a congenital
anomaly that is often associated with congenital cataracts in
22.5% of cases due to its characteristic structures including a
thickened sclera, a normal or slightly larger lens with a
relatively small eye volume, a higher lens-to-eye volume
ratio, and a shallow anterior chamber, narrow camera angle
and short ocular axis. Microphthalmos can cause glaucoma
and other complications affecting the retina and choroid.
Congenital cataract with complex microphthalmos is often
combined with ocular or systemic abnormalities, and patients
after surgery are prone to secondary glaucoma, corneal
Received: Oct. 22, 2023; Accepted: Nov. 20, 2023; Published: Nov. 25, 2023
Published online at http://journal.sapub.org/ajmms
opacification and other serious complications [10,11,14,15].
Depending on the size of the eye, there are 3 degrees of
microphthalmos: 1 degree - a decrease in one or two of the
above dimensions by 1.0-1.5 mm compared to the age norm;
2nd degree - decrease by 2.0-2.5 mm; 3rd degree - reduction
by 3mm or more. Features of congenital cataracts with
microphthalmia are predominantly complete opacities of
the lens - 66.7% (zonular are 2.7%), a high percentage of
capsulo-lenticular opacities - 67.1%, a narrow rigid pupil and
its various anomalies, even absence. The most common
change in the cornea in congenital cataracts is a change
in its size - microcornea. Colobomas of the iris are often
encountered, which vary widely in size and shape and are
often located in the lower segment [4]. Severe anomalies
are various manifestations of mesodermal dysgenesis.
Cataract is one of the elements of Rieger's syndrome
(dysgenesis of the iris and cornea) and Peters syndrome
(mesodermal-ectodermal dysgenesis of the cornea and iris).
CC is also part of the Persistent hyperplastic primary
vitreous (PHPV). PHPV is associated with a disruption of the
process of regression of the primary embryonic vitreous
div. Changes in the retina and optic nerve of varying
nature and severity, identified in 55% of cases, are one of the
reasons for low visual acuity after extraction of congenital
cataracts. More often, combined lesions were observed
(40.5%), less often - isolated changes in the retina (5.0%) or
optic nerve (9.5%). Pathologies of the optic nerve included
partial atrophy and developmental abnormalities (reduction
in size, change in disc shape, etc.). On the part of the retina,
macular hypoplasia, myelin fibers, central and peripheral
dystrophy, “old” chorioretinal lesions (multiple, small, in the
periphery, or large in the central region) were detected [2,3].
Treatment of congenital cataracts in children remains a serious
problem, which is associated not only with the anatomical
1796
Abdurakhmanova Charos
et al.
: Features of Management of Children
with Congenal Cataracts and Accompanying Visual Pathology
features of the structure of the child’s eye, but also with
the frequency of concomitant pathologies, as well as with
various complications caused by the hyperergic state of the
child’s immune system [7,6,8,16].
One of the most important questions remains about the
timing of surgical intervention for congenital cataracts.
Currently, most authors are of the opinion that complete,
layered and central cataracts with an opacification area of
more than 2.5 mm should be operated on within the first
three months after the birth of the child [1,3], and for para
central and central cataracts less than 2.5 mm in diameter -
dynamic observation is possible. Proponents of a more
radical approach insist on surgical treatment of congenital
cataracts after a clinical diagnosis of the disease has been
established [12]. However, despite different views, in most
cases the problem of early surgical intervention is solved
individually in each case, taking into account not only the
degree of intensity of lens opacification, but also the
presence and severity of combined pathology of the visual
analyzer and the child’s div as a whole [13,14].
Purpose of the study.
To study the features of
management of children with congenital cataracts and
concomitant pathologies of the visual organ.
2. Material and Research Methods
An analysis of the case histories of 72 children (117 eyes)
diagnosed with congenital cataracts, who were hospitalized
in the eye department of the TashPMI clinic for the period
from 2016 to 2023 at the age of 3 months to 14 years,
was carried out. Boys made up 42%, girls 58%. A bilateral
process is observed in 45 (62%), unilateral in 27 (38%)
children. The patients underwent visometry, biomicroscopy,
keratometry, ophthalmoscopy, tonometry, echobiometry,
strabismus angle determination, and visual evoked potential
(VEP) study. Preoperative preparation also included
consultations with a pediatrician and neurologist; if necessary,
children were consulted by a geneticist, an ophthalmologist,
and other specialists.
3. Research Results and Discussion
According to the clinical and surgical classification of
congenital cataracts [5], “layered” (nuclear, zonular) were
observed in 22 (19%) eyes, “total” cataracts in 38 (32%),
“atypical” cataracts (semi-resolved, calcified, anteroposterior
capsular, posterior and anterior lenticonus) in 57 (49%)
children. In patients, the range of concomitant pathologies
was quite wide (Table 1).
Classical extracapsular cataract extraction (ECE) with
simultaneous implantation of a soft IOL made of hydrophobic
acrylic (Acrysof IQ, Acrysof) was performed in 84 (72%)
eyes. In 33 (28%) eyes, ECE was performed without
implantation due to the presence of high-grade microphthalmia
and complications that arose during implantation (unplanned
rupture of the posterior capsule), and the presence of
pathology from the fundus of the eye (hypoplasia of the optic
disc, stage IV-V, PHPV). In 72% of cases (42 eyes), the
operation was performed through a small tunnel sclerocorneal
incision; in 28% (17 eyes) of cases, tunnel self-sealing
corneal incisions were used (in children over 3 years old) in
the upper segment at 11-12 o'clock.
Table 1
Concomitant pathology of the organ of
vision
Number of eyes * (n=117)
Abs.
%
Pathology of the adnexal apparatus
- strabismus
- nystagmus
58
40
18
50
34
16
Microphthalmos
I degree
II degree
III degree
45
21
15
9
39
18
13
8
Refractive errors (myopia)
18
15
PHPV
7
6
Hypoplasia of the optic disc and macula
17
15
Atrophy of the optic disc
9
8
*note - in some cases several types of pathologies were observed in one eye
For children with microphthalmia, surgical treatment was
carried out as early as possible. At the first stage, ECE was
performed. After the first stage surgery, the patients'
condition was monitored. At the second stage, an IOL was
implanted with the lens placed in the ciliary sulcus if the eye
condition was suitable.
In the postoperative period, an inflammatory reaction was
observed in the form of a fibrous-exudative reaction that
occurred in the first days in 49% of cases, which was stopped
by frequent instillations of anti-inflammatory drugs. In 9%
of cases, subluxation of the IOL was observed, which
required repeated surgical intervention-reposition of the IOL.
In 18% of cases, an increase in IOP was observed, which was
stopped by the prescription of antihypertensive drugs.
The high percentage of complications can be explained by
the presence of concomitant pathology of the organ of vision,
especially microphthalmia and PHPV. Anatomical features
of congenital cataracts with complex microphthalmos include
reduced globe volume, reduced axial length (<18 mm),
predominantly spherical lenses, the presence of possible
microcornea and a shallow anterior chamber. Patients with
congenital microcornea often have a corneal diameter
<9 mm, corneal flattening, and are often associated with
cataracts and coloboma of the iris or choroid. Congenital
cataracts accompanied by complex microphthalmos or
microcornea are often accompanied by a rigid pupil. Due to
the structures of the eyeball characteristic of congenital
microphthalmia, intervention for cataracts is very difficult
and risky and can lead to postoperative complications such
as severe inflammatory reaction, glaucoma, IOL luxation,
retinal detachment and other serious complications.
American Journal of Medicine and Medical Sciences 2023, 13(11): 1795-1797
1797
Rehabilitation of children in the postoperative period included:
- Spectacle correction
- Treatment of amblyopia
- Conservative neuroprotective and nootropic therapy
(with concomitant pathology of the optic disc)
- YAG laser capsulotomy for the development of
fibrosis of the posterior capsule of the lens.
Rehabilitation measures must also be carried out in
local clinics. Recommendations for improving the quality
of rehabilitation of children with CC in outpatient settings
include the following:
1. Increasing the level of knowledge of ophthalmologists
in city clinics and regional centers in matters of diagnosis,
treatment, clinical examination and rehabilitation of
children with congenital cataracts and concomitant
pathologies of the organ of vision.
2. Increasing the frequency of observations during the
rehabilitation period - after discharge, 2 times a month
for 2 months, then 1 time for 4 months, then as
indicated. If the process is stabilized, it is necessary to
examine children 1-2 times a year until they reach 15
years of age.
3. Increasing the frequency of studies performed during
the rehabilitation period - during examination, it is
necessary to monitor the density of lens opacity,
measure the diameter of the cornea, determine the
state of transparency of the lens capsule, determine
acuity and IOP.
4. Conclusions
Thus, children with congenital cataracts and concomitant
pathology of the visual organ require a differentiated approach
to patient management: in the presence of microphthalmos,
early surgical intervention is necessary before 1 year - stage
1, including cataract extraction, followed by stage 2 - IOL
implantation. Constant dynamic monitoring is necessary,
given the high percentage of postoperative complications. It
is also necessary to carry out the following rehabilitation
measures: glasses correction, measures aimed at combating
amblyopia, if necessary, nootropic therapy, YAG - laser
capsulotomy.
REFERENCES
[1]
Avetisov S.E., Ilyakova L.A., Voronin G.V. Correction of
aphakia after early surgery of congenital cataracts //
Congenital and hereditary eye diseases in children. M., 2003.
T. 100. No. 5. pp. 318-319.
[2]
Aznabaev R.A., Akmanova A.A. Implantation of IOLs in
children with congenital unilateral cataracts complicated by
deep amblyopia // Problems of ophthalmology: Results and
development prospects 2001. pp. 92-96.
[3]
Bobrova N.F. Basic principles of surgery for congenital
cataracts in children // Ophthalmology. – 2015. – No. 2 (02).
pp. 244-256.
[4]
Batkov E.N., Zotov V.V. A case of surgical treatment of
congenital cataract with a posterior capsule defect, lens
coloboma and atypical iris coloboma. Modern technologies of
cataract and refractive surgery. 2009; p. 1690.
[5]
Bobrova N. F. Classification of congenital cataracts (clinical
and surgical) // Russian pediatric ophthalmology. 2012. No. 2.
pp. 52
‑
57.
[6]
Vasilyev A.V., Egorov V.V., Smolyakova G.P. et al. Analysis
of the frequency and structure of complications in the
long-term period after aspiration of congenital cataracts with
IOL implantation in children of different ages during early
childhood. Russian Pediatric Ophthalmology 2011; 1, pp. 34-38.
[7]
Kruglova T. B. Results and prospects for the treatment of
children with congenital cataracts. Pediatric ophthalmology.
Results and prospects: Materials of scientific research. pract.
conf. M., 2006, pp. 45-49.
[8]
Neroev V.V. and others. Features of blood flow in the orbital
vessels in children with congenital microphthalmos of varying
degrees // Ros. pediatrician. ophthalmol. 2013. No. 1. pp. 4-7.
[9]
Neroev V.V., Khvatova A.V., Sudovskaya T.V. Unilateral
congenital cataracts in children (clinical classification,
indications for operations, timing of surgical treatment) //
Russian Pediatric Ophthalmology. 2009. No. 1. p. 8 13
[10]
Sirotina N.A. and others. Our experience in conservative
and surgical treatment of congenital anophthalmos and
microphthalmos // XXX. 2017. No. 1. pp. 30-33.
[11]
Sudovskaya T.V. Features of diagnosis, clinical presentation
and treatment of unilateral congenital cataracts with
microphthalmos of varying degrees in children. Ros.
pediatrician. ophthalmol. 2009. No. 3. p. 8.
[12]
Shilovskikh O.V., Shlyakhtov M.I., Fechin O.B. Rehabilitation of
visual functions in children after extraction of congenital
cataracts. Russian Pediatric Ophthalmology 2008; 2: 24 26
Apple DJ, Ram J, Foster A, et al. Elimination of cataract
blindness: a global perspective entering the new millenium.
Surv Ophthalmol
2000; 45: Suppl 1: pp. 1-96. [PubMed]
[13]
Khokhar SK, Dave V. Cataract surgery in infant eyes with
microphthalmos.
J Cataract Refract Surg
2009; 35: 1844–5.
doi:10.1016/j.jcrs.2009.05.040. [PubMed] [Google Scholar]
[14]
Sun J, Zhang J, Dai Y, Wan X, Xie L. Cataract surgery
contributes to ocular axis growth of aphakic eyes in infants
with complex microphthalmos. Medicine. 2020; 99: 39 (e22140).
[15]
Zetterstrom C., Lundvall A., Kugelberg M. Cataract in
children. J. Cataract. Refract. Surg. 2005; 31: 824-840.
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