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ORIGINAL ARTICLE
ARCH SOC ESP OFTALMOL 2007; 82: 37-42
PEDIATRIC CATARACTS: EPIDEMIOLOGY AND
DIAGNOSIS. RETROSPECTIVE REVIEW OF 79 CASES
CATARATAS PEDIÁTRICAS: ESTUDIO EPIDEMIOLÓGICO
Y DIAGNÓSTICO. ANÁLISIS RETROSPECTIVO
DE 79 CASOS
PERUCHO-MARTÍNEZ S1, DE-LA-CRUZ-BERTOLO J2, TEJADA-PALACIOS P2
ABSTRACT
RESUMEN
Purpose: To determine the epidemiology, diagnosis
and treatment features in a group of pediatric
patients with cataracts treated at our hospital. The
aim was to improve the visual prognosis in these
patients.
Methods: 79 children with cataracts were reviewed
retrospectively during an 18 year period (19862004). This involved patients with congenital cataracts and those who acquired them later. Most of the
latter group had a traumatic etiology (90%).
Results: The etiology of most cataracts was idiopathic (68%) for the congenital group and traumatic (90%) for the acquired group. Congenital cataracts were frequently nuclear in type (31%) with
56% being bilateral. 27% of the congenital group
were associated with dysmorphic eye features, the
most frequent being microphthalmos. The most frequent presenting feature was leucokoria, seen in
44% of patients. 75% of congenital cataracts were
diagnosed within one month of clinical manifestation. 58% of the congenital cataracts were treated
by surgery and 50% of these were performed within
one month of the diagnosis. 90% of the acquired
Objetivo: Determinar las características epidemiológicas, diagnósticas y terapéuticas de un grupo de
cataratas de nuestro medio con el propósito de
mejorar el pronóstico visual de estos pacientes.
Métodos: Estudio retrospectivo de 79 casos de
cataratas pediátricas durante un periodo de 18 años
(1986-2004). Hemos diferenciado dos grupos etiológicos de trabajo: cataratas congénitas y cataratas
adquiridas.
Resultados: La causa más frecuente entre las congénitas fue la idiopática (68%) y la más frecuente
de las adquiridas fue traumática (90%). La morfología más frecuente de las congénitas fue la nuclear
0,31 (31%). El 56% de las cataratas congénitas fueron bilaterales. El 27% de las cataratas congénitas
se asociaban a otras anomalías oculares y la más
frecuente fue el microftalmos. El signo clínico de
presentación más frecuente de las cataratas congénitas fue la leucocoria, en 0,44 (44%). El 75%
(0,75) de las cataratas congénitas de nuestro medio
tardan menos de un mes en diagnosticarse desde la
manifestación clínica. El 58% (0,58) de las cataratas congénitas se trataron con cirugía y el 50% de
Received: 27/2/06. Accepted: 17/1/07.
Ophthalmology Service. Hospital Universitario 12 de Octubre. Madrid. Spain.
1 Graduate in Medicine.
2 Ph.D. in Medicine.
Paper presented in part at the 17th Congress of the Spanish Strabology Society (Madrid 2004).
Correspondence:
Susana Perucho Martínez
C/. Cabo San Vicente, 10, 2.º C derecha
28924 Alcorcón (Madrid)
Spain
E-mail: [email protected]
PERUCHO-MARTÍNEZ S, et al.
cataracts were treated surgically, and 95% of these
were performed less than one month after diagnosis.
Conclusions: We attained a prompt diagnosis and
treatment in a high percentage of cases. Prompt
diagnosis and treatment will determine the visual
prognosis of these patients. We must continue
trying to shorten this period of time in order that all
children with this condition are diagnosed and treated urgently and efficiently (Arch Soc Esp Oftalmol
2007; 82: 37-42).
Key words: Cataract, pediatric, congenital, intraocular lens, pseudophakia, cataract extraction.
INTRODUCTION
Early congenital cataracts are frequent eye diseases, just like traumatic cataracts (1.4-6.3 for every
100.000 live births according to the population
being studied) and constitute an important cause of
visual acuity (VA) reduction in childhood (1). The
prevalence of blindness (VA with correction below
0.05) due to pediatric cataracts could be in the range
of 1 to 4/10,000 children in developing countries
and between 0.1-0.4/10,000 children in developed
countries (2-5). Said cataracts have particular characteristics and their management is completely different to that of adult cataracts.
The main cause of vision loss in congenital or
childhood cataracts is amblyopia. The first few
months of life are a critical period for the development of eyesight because the visual areas of the
brain experience fast maturation in response to
visual signals from the eyes (6,7). The fuzziness of
the retinal image in one or both eyes during this
critical period gives rise to irreversible amblyopia.
The early diagnosis of amblyopia is an international priority (8) because the visual prognosis of
these patients depends directly on it. This priority
has resulted in prevention and identification strategies in developed as well as developing countries
(9).
Seventy-nine cases of pediatric cataracts have
been analyzed in the pediatric ophthalmology practice of our hospital area, establishing its prevalence
in our region, its etiology and morphology, diagnostic and treatment characteristics in order to
improve the visual prognosis of patients.
38
ellas tardaron menos de 1 mes en operarse. El 90%
(0,9) de las adquiridas se trataron con cirugía y el
95% de ellas tardaron menos de un mes en operarse.
Conclusiones: En nuestra serie conseguimos un
diagnóstico y tratamiento precoz en un alto porcentaje de pacientes. El pronóstico visual de estos
niños viene determinado por la precocidad en el
diagnóstico y el tratamiento, es por ello que debemos continuar intentando acortar este período de
tiempo y conseguir que todos los niños sean diagnosticados y tratados precozmente.
Palabras clave: Catarata, pediátrica, congénita,
lente intraocular, pseudofaquia, extracción de catarata.
SUBJECTS, MATERIAL AND
METHODS
A retrospective study of 79 pediatric cataract cases in our environment during an 18-year period
(1986-2004) of diagnosis and follow-up. Two etiological working groups were set up:
— the congenital cataracts group, which comprises idiopathic and genetic cataracts as well as
those associated to syndromes and due to intrauterine infections, and
— the acquired cataracts group, comprising
traumatic and metabolic cataracts.
Each of the etiological groups analyzed the following parameters: sex, family history, location,
morphology and characteristics of the cataracts,
association with other ocular malformations, form
of expression, age of clinical expression, age at
diagnosis by pediatrician or ophthalmologist, surgical or conservative treatment and age at which
surgery was performed, if applicable.
The term «leucochoria» was utilized as a sign of
clinical expression and, in this paper, leucochoria is
utilized to denominate any lens opacity instead of
the typical whitish area (leucochoria) produced by
other types of pediatric ophthalmological pathologies such as retinoblastoma, PVPH, toxoplasmosis,
and the like.
A descriptive statistical analysis has been made,
which presents absolute and relative frequency distributions. The Chi-square test was utilized to compare the statistical significance of the various proportions. The statistical analysis software we used was
SAS v 8.02; SAS Institute Inc., (Cary, NC, USA).
ARCH SOC ESP OFTALMOL 2007; 82: 37-42
Pediatric cataracts
RESULTS
In the etiological group analysis, 59/79 (74.36%)
were congenital cataracts (i.e., idiopathic, hereditary, associated to syndromes and to intrauterine
infections) and 20/79 (25.64%) were acquired (i.e.,
traumatic and metabolic).
In the congenital cataracts it was found that 0.68
(68%) are idiopathic, 0.17 (17%) hereditary, 0.03
(3%) associated to intrauterine infections (two cases of congenital measles) and 0.12 (12%) associated to syndromes (9 cases associated to Down syndrome and on associated to the Rubinstein-Taybi
syndrome).
As for the acquired cataracts, 0.9 (90%) are of
traumatic origin and 0.1 (10%) are metabolic (3
cases with rheumatoid and kidney diseases treated
with high corticoid dosages).
In what concerns family histories, 0.27 (27.11%)
had congenital cataracts while 0.73 (72.8%) did not.
As regards the morphology of congenital
cataracts, 0.31 (31%) were nuclear cataracts, 0.15
(15%) were polar, 0.08 (8%) were subcapsular; the
rest of less frequent morphologies are summarized
in Table 1. In the group of acquired cataracts, 0.05
cases (5%) were of the nuclear type, 0,05 cases (5%)
were of the polar type, 0.35 cases (35%) were subcapsular, 0.35 (35%) cases had other expression and
0.2 cases (20%) were of undetermined morphology.
As regards localization, it was found that in the
congenital cataracts group 0.56 (56%) were bilateral and 0.4 (44%) unilateral. However, in the group
of acquired cataracts, 0.85 (85%) were unilateral
and only 0.15 (15%) bilateral. No statistically significant results were found related to expression in
the right eye (RE) or left eye (LE) in the unilateral
cataracts cases.
In the congenital cataracts group, 0.2 (20%)
appeared in the RE and 0.24 (24%) in the left one.
Table I. Relative frequency of congenital cataracts based
on morphology
Morphology
Number
Percentage
Nuclear
Polar
Zonular
Subcapsular
Complete
Others
Unknown
TOTAL
18
9
1
5
2
7
17
59
30,50%
15,25%
1,69%
8,47%
3,38%
11,86%
28,81%
100,0%
In the group of acquired cataracts, 0.5 (50%) were
in the RE and 0.35 (35%) in the LE.
The association of congenital cataracts with other ocular abnormalities was studied, finding that
0.27 (27%) presented other associated anomalies.
The most frequent associated anomaly was
microphthalmus, which appeared in 0.44 cases
(44%). Other types of less frequent associated ocular anomalies were persistence of pupillar membrane, which appeared in 0.31 (31%) cases, congenital glaucoma in 0.2 cases (19%), persistence of
hyperplasic primary vitreous in 0.06 cases (6%).
In what concerns the form of clinical presentation of cataracts in our environment, it was seen
that the most frequent expression of congenital
cataracts was leukocoria (44%) and in the acquired
cataracts group the previous traumatism history
(table II).
We identified the age at which the first clinical sign
appeared. The following results were obtained: in the
congenital cataracts group, 17% of cases appeared at
birth, 40% during the first 3 months, 13.5% between
3-6 months, 5% between 6-12 months, 11.8%
between 12-48 months and 5% over 48 months. In the
group of acquired cataracts, not a single case
appeared at birth and only one (5%) appeared in the
first year of life, 30% of cases between 12-48 months,
20% between 48-96 months and 45% of cases
appeared at 96 months of life or more.
By knowing the age of cataracts clinical presentation and the age in which it is diagnosed it is possible to know how long it took to diagnose the
pathology in our region since its first clinical
appearance. In this way, we were able to see that in
the group of congenital cataracts, in 0.67 (70%) of
cases the diagnostic was made at the latest 2-3 first
weeks after clinical appearance, in 0.06 (7%) one
month, in 0.06 (7%) between 1-3 months, 0,01
(2%) between 3-6 months and in 0.13 (14%) over 6
months elapsed between the clinical appearance
and the diagnostic.
In acquired cataracts, it was seen that in 0.7
(70%) of cases there was no time compensation, in
0.1 (10%) one month elapsed, in 0.05 (5%) between
1-3 months elapsed, in 0.05 (5%) 3-6 months and in
0.1 (10%) over 6 months.
To end, we describe the type of treatment given
to cataracts in our study group. In the congenital
cataracts group, 58% received surgery. In the group
of acquired cataracts, 90% received surgical treatment (table III).
ARCH SOC ESP OFTALMOL 2007; 82: 37-42
39
PERUCHO-MARTÍNEZ S, et al.
Table II. Form of presentation of childhood cataracts based on etiological diagnostic:
congenital and acquired cataracts
Presentation
Etiological groups
Leukocoria
Number
Percentage
Number
Percentage
Number
Percentage
Number
Percentage
Number
Percentage
Number
Percentage
Number
Percentage
Nistagmus
Strabismus
VA reduction
No signs
UNKNOWN
TOTAL
Acquired
26
44,00%
7
11,80%
9
15,25%
2
3,38%
7
11,86%
8
13,50%
59
100,00%
2
10,00%
0
0,00%
0
0,00%
2
10,00%
16
80,00%
0
0,00%
20
100,00%
In congenital cataracts, 0.56 (56%) cases
required bilateral surgery while in 0.44 (44%) cases surgery was on one side only.
The period of time between cataracts diagnosis
and surgery in our environment was also analyzed.
In the congenital cataracts group, 0.5 (50%) cases
were diagnosed and treated within 1 month, in 0.26
(26%) between 1-2 months. In the group of
acquired cataracts, in 0.95 (95 %) cases said period
of time was under 1 month (table IV).
DISCUSSION
Pediatric cataracts are an important cause of VA
loss in children, mainly congenital cataracts and
those which develop in the first stages of life. They
constitute the first cause of preventable blindness in
the world in the pediatric population (10).
The objective of this study is to determine with
greater detail the pediatric cataracts in our populaTable III. Treatment of childhood cataracts according to
etiological diagnosis: congenital and acquired
cataracts
Surgical
Conservative
Total
40
Etiological groups
Congenital
Acquired
Number
Percentage
Number
Percentage
Number
Percentage
34
58%
24
42%
58
100%
18
90%
2
10%
20
100%
28
35,44%
7
8,86%
9
11,39%
4
5,06%
23
29,11%
8
10,12%
79
100,00%
tion in order to improve as far as possible an early
diagnosis and treatment. The most important purpose is to reduce the risk of development of secondary amblyopia which will have a direct influence in the visual prognosis of this population.
We carried out epidemiology, diagnostic and
therapeutic approach studies of infant cataracts in
our environment, finding similar results to those of
other populations (8,9,11,12).
As regards etiology, in the group of congenital
cataracts, the most frequent ones are the isolated
cataracts (68%). 17% are hereditary, although this
percentage should be higher because some cataracts
which were classified as isolated could be primary
mutations of hereditary cataracts.
The most frequent syndrome associated to
cataracts is the Down syndrome, and it seems there
Table IV. Period of time elapsed between diagnostic
of surgical childhood cataracts and the
surgery
Time interval
Under 1 month
Treatment
Total
Congenital
1-2 months
2-6 months
Over 6 months
Total
ARCH SOC ESP OFTALMOL 2007; 82: 37-42
Etiological groups
Congenital
Acquired
Number
Percentage
Number
Percentage
Number
Percentage
Number
Percentage
Number
Percentage
17
50%
9
26,5%
0
0%
8
23,5%
34
100%
17
94,5%
1
5,5%
0
0%
0
0%
18
100%
Pediatric cataracts
is an increased prevalence of congenital cataracts in
this population (2-6%) (13). In our series, the two
cases of intrauterine infections associated to
cataracts were congenital measles.
In the group of acquired cataracts, the most frequent cause is trauma (perforating traumatisms).
There are two cases of acquired cataracts secondary
to corticoid treatment which are also the two only
cases of bilateral acquired cataracts. The majority
of bilateral cataracts are congenital and more
specifically hereditary.
The most frequent morphology of congenital
cataracts is nuclear, followed by polar. In acquired
cataracts, the morphology depends on the nature of
the trauma with the exception of metabolic
cataracts which, as can be expected, are subcapsular posterior. 27% of congenital cataracts exhibited
other associated ocular abnormalities, the most frequent of which was microphthalmus followed by
the persistence of pupillar membranes. It is very
important to take into account microphthalmus as
the most frequent associated abnormality because it
can hinder surgical treatment and subsequent visual
rehabilitation. In addition, it is considered to be a
factor of negative visual prognosis for these patients
(10).
The most frequent clinical sign of congenital
cataracts is leucochoria followed by strabismus.
These clinical expressions are vitally important
because pediatricians increasingly recognize them
due to the inclusion of basic ophthalmological
explorations in their daily practice which provide
early diagnosis.
In what concerns the age of presentation, we can
refer two age peaks which correspond to each one
of the cataracts group. The peak age for congenital
cataracts is around 3 months whereas acquired
cataracts is around 8 years in our series.
Some of the most important factors in the visual
prognosis of these patients is diagnostic and early
treatment of this pathology, mainly in small children (14). The term «early» is understood to be
cataracts diagnosed before week 4-6 of their
appearance. In our series we have seen that the
majority of congenital cataracts (as well as acquired
cataracts) are diagnosed before one month, and surgical cataracts are operated also within the month.
These are encouraging data which show that exploration techniques for early detection in primary care
centers and specialized centers in our environment
are proceeding efficiently.
However, we must not cease in our endeavor to
have all children diagnosed and treated at an early
stage. For this, the cooperation of parents, pediatricians and ophthalmologists is required.
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