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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. 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