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Anos (2004-2016) Cataratas emIdadePediátrica:EstudoRetrospetivode12 (2004 -2016) Pediatric : A RetrospectiveStudyof12 Years Recebido: 17desetembro de2016- Aceite: 13defevereiro 2017|  1. ServiçodeOftalmologia.HospitalPedroHispano. Matosinhos.Portugal. cataracts aremostlyidiopathic indevelopedcountries. Paediatric etc.). therapy, laser therapy, radiation (drugs, iatrogenic or injury in to secondary those from apart cataracts children, of causes leading the among are infections proximately 200,000childrenworldwide. ap affecting and blindness childhood avoidable of causes INTRODUCTION RESUMO Acta MedPort2017Mar;30(3):169-174 Paula TENEDÓRIO Jorge MOREIRA ABSTRACT Autor correspondente:[email protected] Palavras-chave: desafio aoplaneamentodeestratégiasprevenção. Conclusão: às descritasnaliteratura. inerentes a uma amostra pequena, o perfil epidemiológico das cataratas pediátricas, neste hospital, apresenta características similares Discussão: complicações pós-operatóriasregistaram-seem35%dascrianças,sendoaopacificaçãodacápsulaposteriormaiscomum(25%). outras anomalias oculares em 33,3% das crianças. Relativamente ao tratamento, 47,6% das crianças foram submetidas a cirurgia. As e a (59,5%), e casos dos 23,8% em sistémicas doenças (32,5%). frequente a nuclear associaram-se tipo cataratas do As cataratas das predomínio houve mais a foi idiopática etiologia A(26,3%). leucocória pela seguida (36,8%) visual acuidade da diminuição a foi bilaterais. eram cataratas as crianças, das A64,3% em e comum anos mais seis clínica de manifestação foi diagnóstico do momento Resultados: anos, noHospitalPedroHispano,Porto. Material e Métodos: implementação deprogramasprevençãoprimáriaediagnósticoprecocedascataratas. destas crianças depende de uma abordagem terapêutica atempada, sendo fundamental o conhecimento da sua epidemiologia para a Introdução: Keywords: Conclusion: described intheliterature. those to similar relatively characteristics has hospital our in cataracts with children of profile the size, sample small include study our Discussion: (25%). common most the was opacification capsular posterior and cases of 35% were in patients occurred complications surgically.of Postoperative treated 47.6% cases. of 33.3% in abnormalities ocular with and cases of 23.8% in diseases systemic with associated was (26.3%). The etiology was unknown in 59.5% of cases and there was a slight predominance of nuclear type (32.5%). Cataract leucocoria by followed (36.8%) sign presenting commonest the was acuity visual Decreased disease. bilateral had patients of 64.3% Results: in Porto. Material andMethods: primary preventionandearlydiagnosis. of programs of implementation the for importance great of is epidemiology cataracts pediatric Understanding approach. therapeutic Introduction: aaat hv be cniee a oe h main the one as considered been have Cataracts The first years of life are crucial for the development for arecrucial of life years first The intrauterine and diseases metabolic factors, Genetic We identified 42 cases of pediatric cataracts with an equal gender distribution. The mean age at diagnosis was 6 years and years 6 was diagnosis at age mean The distribution. gender equal an with cataracts pediatric of cases 42 identified We Adolescent; Cataract/epidemiology;Cataract/etiology;CataractExtraction;Child A descrição de 42 casos reflete, provavelmente, a baixa prevalência das cataratas nesta idade. Apesar das limitações das Apesar idade. nesta cataratas das prevalência baixa a provavelmente, reflete, casos 42 de descrição A O elevado número de cataratas de etiologia idiopática observado a nível global e neste estudo, em particular, constitui um As cataratas são consideradas uma das principais causas de cegueira evitável em idade pediátrica. O prognóstico visual The report of 42 cases is probably the result of the low prevalence of cataracts in this age. Although the limitations of limitations the Although age. this in cataracts of prevalence low the of result the probably is cases 42 of report The Foram diagnosticados 42 casos de cataratas pediátricas, com igual distribuição em ambos os géneros. A média etária no Giventhehighproportionofidiopathicpediatriccataracts,preventiondiseaseremainsachallengeworldwide. Cataracts are a major cause of preventable . Visual prognosis of these patients depends on a prompt  Adolescente; Criança;Catarata/epidemiologia; Catarata/etiologia;ExtraçãodeCatarata 1 1 , IsabelRIBEIRO

Analisámos os processos clínicos de doentes com cataratas diagnosticadas em idade pediátrica, nos últimos 12 We reviewed the clinical cases of pediatric cataracts diagnosed in the last 12 years at Hospital Pedro Hispano, ica da Ordem dos Médicos www m o c . a s e u g u t r o p a c i d e m a t c a w. w w s o c i d é M s o d m e d r O a d a c fi í t n e i C a t s i v e R ▪ 1

, ÁgataMOTA https://doi.org/10.20344/amp.8223 1 Copyright ©Ordemdos Médicos2017 1 , RitaGONÇALVES 1,2 - 169 rvnin f n mjr opiain Knowledge management in complication. impact positive a of has cataracts paediatric characteristics major clinical and epidemiological any regarding of prevention of sensory plasticity. period the over out carried is therapy adequate an as period. that over image retinal distorted and blurred by induced be can irreversible therefore and vision child’s a of iey igoi ad ramn ae rca fr the for crucial are treatment and diagnosis Timely 3,4 iin maret a sil e eetd s long as reverted be still may impairment Vision 1 , PedroCOELHO 1 , Tiago MAIO 1 ,

ARTIGO ORIGINAL Total n (%) 8 (19.0) 8 (19.0) 42 (100) 12 (28.6) 14 (33.3) n (%) 3 (7.1) 3 (7.1) 21 (50) Female 7 (16.7) 8 (19.0) Acta Med Port 2017 Mar;30(3):169-174 Port 2017 Acta Med Nuclear cataracts were most frequently found (32.5%), No statistically significant differences were found Cataracts were associated with other ocular Cataracts that were associated with other ocular Impaired (36.8%), followed by Idiopathic cataracts were most frequently found (59.5% in another patient cataract was associated with persistent cataract a with presented patient one and vasculature foetal caused by rubella Hereditary embryopathy. cataracts were found in four patients (9.5%), transmitted in an autosomal dominant in three and in an autosomal recessive pattern in 2). another patient (Table with patients eight to corresponding (25%, polar by followed posterior and cataracts) polar posterior with two and anterior are morphologies frequent less Other (22.5%). subcapsular 2. Table shown in between aetiology and laterality aetiology and morphology. as well as between abnormalities in 14 patients (33.3%) and microphthalmia being the most frequently ectopic found (21.4%), (14.3%), followed microcornea by (14.3%), (14.3%), coloboma congenital (7.1%), posterior lenticonus (7.1%) vasculature foetal persistent (7.1%), (7.1%), and persistent pupillary membrane (7.1%). the right eye). No differences were found between cataract age distribution. patient’s laterality and (26.3%) were the most common clinical manifestations. In 23.7% of the patients, cataracts were diagnosed in routine examinations and eye movement disorders were the first sign in 13.2% of the patients in 7.9%, all ( of the convergent type). No in clinical 5.3% and 2). regarding four patients (Table information was available syndromes genetic with associated were and patients) the of or metabolic diseases in 19% including two of patients with trisomy 21 and one patient the with patients (n = muscular Duchenne sclerosis, tuberous syndrome, Noonan 8), dystrophy, type-1 diabetes mellitus, each). Fabry patient (one syndrome polymalformative unclassified disease and patients Two developed cataract secondary to chronic oral corticosteroid therapy for a rheumatoid arthritis). An systemic exposure to chemical substances disease (juvenile during embryogenesis (alcohol) was found in one patient, 170 5 vs. Male n (%) 21 (50) 5 (11.9) 5 (11.9) 5 (11.9) 6 (14.3) standard deviation. Chi-square R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m ± Moreira J, et al. Pediatric cataracts: a retrospective study, study, a retrospective cataracts: J, et al. Pediatric Moreira - Distribution of patients with cataracts according with the age group Mean age at diagnosis was 6 ± 4.9 years (2 days - 17 In total, 27 patients (64.3%) presented with bilateral In total, 42 childhood cataracts (69 eyes) were diagnosed were eyes) (69 cataracts childhood 42 total, In SPSS version 21 software was used in statistical The following clinical variables were analysed: patient’s This was a retrospective study based on clinical 7 – 10 years > 10 years Total Age group ≤ 1 year 1 – 6 years Table 1 Table difference. No statistically significant differences as regards as differences significant statistically No difference. laterality were found in patients with in unilateral 7/15 and left the in cataract with presented patients (8/15 cataracts 1:1.8 was slight A found. male predominance was found in unilateral with patients in predominance female and bilateral cataracts, even though with no statistically significant ± 4.3 years) (Table 1). ± 4.3 years) (Table cataracts and a ratio of unilateral to bilateral cataracts of male). 5.4 ± (7 patients male in higher slightly was and years) (50% patients 42 to corresponding 2016, and 2004 between five. A 0.05 level of significance has been considered. five. RESULTS exact test has been used if more than 20% of the cells in the in cells the of 20% than more if used been has test exact contingency table regarding the analysis of the association between two categories had expected frequencies below variables through mean independence test was used association for between categorical the variables analysis and of Fisher’s the analysis. Categorical variables were absolute described (n) and through relative frequencies (%) and continuous parameters were assessed: age at diagnosis intra to and surgery, postoperative complications surgery, time from and pre and postoperative visual acuity. morphology, morphology, aetiology, presence of systemic abnormalities, family other history of cataract, ocular follow- up and time and treatment. In case of surgery, the following childhood (patients aged under 18 years). childhood (patients gender, age at diagnosis, presenting symptoms, laterality, records of patients attending the Hospital Pedro Hispano, Matosinhos between 2004 and 2016 with cataracts in MATERIAL AND METHODS MATERIAL improvement, improvement, particularly in favour of an early diagnosis outcome. and improved

ARTIGO ORIGINAL es IL ipatto ad h svn ains who patients and cataracts seven bilateral with the presented aphakic and remained implantation (IOL) or timefromdiagnosistosurgery. namely surgery at bilateral), age patients, operated of number the and regarding (unilateral groups both between two months (sevendays–ninemonths). was other the and eye one in surgery between time and bilateral cataracts diagnosis 12.6 ± 20.4 months upon from diagnosis; mean months 5.9 ± 6.8 operated average on was cataract Unilateral (Table3). months) 73 - days (4 years inpatientswithbilateralcataracts). 4.5 ± 4 and unilateral with patients in years 4.8 ± (8 years) [60%] male;7[35%]unilateral). 0.05). (61.5%; unilateral predominantly were abnormalities nical manifestations,aetiologyandmorphology Table2 Unavailabledata Sutural Cerulean Total Lamellar Mixed Subcapsular Polar Nuclear Morphology Persistentfoetalvasculature Rubella Exposuretochemicalsubstances Systemiccorticoidtherapy Hereditary Geneticsyndromesandmetabolicdiseases Idiopathic Aetiology Unavailabledata Routineexamination Nystagmus Strabismus Leukocoria Visual acuityimpairment Clinical manifestations Variable In total, 13 patients (65%) underwent primary intraocular found were differences significant statistically No Time from diagnosis to surgery was 10.5 ± 16.8 months 13.5 – days (23 years 5 ± 5.5 was surgery at age Mean (12 surgery underwent eyes) (33 patients 20 total, In - Characteristics of paediatric cataracts according with cli with according cataracts paediatric of Characteristics - Moreira J,etal.Pediatriccataracts:aretrospectivestudy, ica da Ordem dos Médicos www m o c . a s e u g u t r o p a c i d e m a t c a w. w w s o c i d é M s o d m e d r O a d a c fi í t n e i C a t s i v e R 10 (25.0) 13 (32.5) 25 (59.5) 10 (26.3) 14 (36.8) 9 (22.5) 8 (19.0) 9 (23.7) 1 (2.5) 1 (2.5) 1 (2.5) 2 (5.0) 3 (7.5) 1 (2.4) 1 (2.4) 1 (2.4) 2 (4.8) 4 (9.5) 2 (5.3) 3 (7.9) n (%) Total 2 4 p < - 171 referred for YAG laser capsulotomy on average 14 months 14 average on capsulotomy YAGfor laser referred patients were aged on average 9.5 years at surgery and these were capsulotomy; posterior primary undergo not did who patients in occurred and 25%) – patients (five complication frequent most the was opacification patients capsular posterior and the of 35% in occurred complications operative place 3.5yearsuponcataractsurgery, onaverage. IOL secondary at age took and years four was implantation subsequently underwent secondary IOL implantation. Mean whom of four months), 22 and days 23 between range age were aged under 2 years at surgery (mean age: six months; Table 3 children. people, visually-impaired even though there are no specific data on the prevalence in 35,000 including found, been have cataracts with people 170,000 estimated an Portugal, 4.0/10,000. and 2.2 between ranges prevalence this apart from real differences between populations. In Europe, worldwide, groups, age estimated and definition group methodologies, different been has cataracts of causes treatable blindness inchildren. leading the amongst causes. are preventable Cataracts to due mostly worldwide, children DISCUSSION underwent surgery. years and 7 ± 3.5 years upon surgery in the 20 patients who bilateral). unilateral in 0.1 ± (0.3 cataract unilateral with patients in found BCVAwas mean lower A0.4. ± 0.6 to corresponding patients, 18 in available BCVAwere data (BCVA)study,acuity the visual 0.2. of ± end 0.3 the was At regarding 13 patients and mean preoperative best-corrected the ageofthreemonths. at surgery underwent who microphthalmia with associated glaucoma in a three-year-old patient with bilateral cataracts included posterior synechiae (n = 1) and aphakic secondary surgery.complications initial Other the upon months) (2-24 > 12months 3 –12months 1 –3months ≤ 1month Time from diagnosistosurgery o nroeaie opiain wr fud Post- found. were complications intraoperative No Visually impairment from cataracts in children, apart from paediatric of rate prevalence 1-15/10,000 estimated An impaired visually million 1.5 estimated an are There 3.6 ± 6 was patients 42 the of time follow-up Mean available was acuity visual preoperative on Information Acta MedPort2017Mar;30(3):169-174 - Time fromdiagnosistosurgery 5 vs. 5 (25.0) 6 (30.0) 4 (20.0) 5 (25.0) n (%) Total . ± . in 0.1 ± 0.8 1 reflecting 1 In 1

ARTIGO ORIGINAL In fact, Sensory 23 24 18 16,17 Microphthalmia was most even though no statistically 7 9,13,14

13 21,22 19 Primary intraocular lens (IOL) implantation 20 15 Acta Med Port 2017 Mar;30(3):169-174 Port 2017 Acta Med In this study, postoperative complications In were this found study, in Surgery timing and an adequate visual rehabilitation Surgery is aimed at the promotion and maintenance Apart from intraoperative challenges, postoperative The morphology or laterality of childhood cataracts Ocular abnormalities related to cataracts were found in Specific anatomic and physiologic characteristics of stronger inflammatory response. Visual axisopacification is secondary the most frequent complication. the opacification of the posterior capsule, in the absence of primary posterior capsulotomy seems to be universal, particularly in children aged less than four years. 35% of the patients and the opacification of the posterior capsule was the most frequent complication (25%), in Additionally, normal Additionally, eye development, particularly over the first 18 months of life, has a relevant impact. are crucial in The order approach to to cataracts depends avoid on including different the irreversible factors degree of amblyopia. , patient’s age, laterality, size, location and morphology of and concomitant eye the or systemic abnormalities. cataract deprivation caused by cataracts soon as removed occurring be must and relevant very is over life of months the first as possible. of visual axis transparency. Phacoaspiration with primary is vitrectomy anterior without or with capsulotomy, posterior currently the most frequently used technique for childhood cataracts. has been increasingly used, challenging, be may implantation IOL even an selecting However, in younger children. the to due age, of years two than less children in particularly changes of refractive status in children, with an additional risk for complications. complications are more frequent in children, due to a heterogeneity heterogeneity may impair the morphological classification. In addition, different morphologies may prevalent, most the were cataracts polar and nuclear study, coexist. In this other studies. in line with may reflect their aetiology, significant were differences when found those in this study, variables were analysed. 33.3% of the patients predominantly in unilateral cataracts, in line with other studies. frequently found, impairing surgery and subsequent visual rehabilitation, therefore corresponding to a poor outcome factor. child’s eye have a negative impact childhood on cataracts. Apart from the smaller, child’s eyes approach have to higher elasticity, capsule higher , rigid less and thinner vitreous pressure, changes in corneal curvature and higher predisposition to postoperative inflammatory reactions.

172 10 Plano Hereditary 2,7-9 This phenotypic and even though 12 7,8

Therefore, knowledge on the 6 R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m Moreira J, et al. Pediatric cataracts: a retrospective study, study, a retrospective cataracts: J, et al. Pediatric Moreira ), cataracts caused by an intrauterine an by caused cataracts ), a predominance of bilateral cases (64.3%) 7,8 8,11 Morphological presentations of paediatric cataract As expected, due to the inclusion of rubella immunization immunization rubella of inclusion the to due expected, As Cataracts are usually isolated or associated with Impaired visual acuity was the most frequent symptom Different factors may have contributed to the formation The results regarding cataract laterality are in line with This study aims at the evaluation of clinical and Due to its relevance, the ‘Vision 2020’ initiative was affect affect the nucleus or the surrounding cortex while in other patients there is an abnormal capsule an corresponding opacification to of the cortical lamella. usually range from a punctiform opacity to the opacification of the whole lens. The opacification of the lens may only into the Portuguese National Vaccination Program ( Nacional de Vacinação rubella infection were rare (only one patient). in children with Down syndrome ranged between 13%. 1 and and were bilateral in 80% of these, in line with literature. Down syndrome was predominant in patients with genetic syndromes. In other studies, the prevalence of cataracts these were metabolic syndromes or diseases. In this study, associated with systemic diseases in 23.8% of the patients X-linked cataracts, autosomal dominant cataracts most frequently found. were autosomal dominant; the prevalence of hereditary cataracts hereditary of prevalence the dominant; autosomal ranges from 6.8 to 29% of the patients there have been patients with autosomal recessive and simple task. Idiopathic cataracts were the most frequently found (59.5%), in frequently most line patients, the of 9.5% with in found were cataracts other studies. ability to verbalize these complaints. of a cataract, even though in many patients this is not a at age mean that considering expected was which (33.3%), diagnosis was six years, corresponding to children with the other studies, found been has eyes both between distribution even an and in patients with unilateral cataracts. children attending our hospital over the past 12 years; in included in the study (50% male). total, 42 patients were to define the best strategies for this type of program. to define the best strategies epidemiological characteristics of cataracts diagnosed in (IABP) with the main aim at the elimination of blindness by avoidable the year 2020. order in crucial is country each within reality epidemiological jointly developed by the World Health Organization (WHO) and the International Agency for Blindness Prevention with a major social and economic burden and became a concern. public health having having a relevant impact on the psychomotor development as well as on child and family quality of life, is associated

ARTIGO ORIGINAL 12. 11. 10. 9. 8. 7. 6. 5. 4. 3. 2. 1. 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ARTIGO ORIGINAL Acta Arq Bras Oftalmol. 2004;67:921-6. Oftalmol. Arq Bras Acta Med Port 2017 Mar;30(3):169-174 Port 2017 Acta Med Magnusson G, Abrahamsson M, Sjöstrand J. Glaucoma following submetidos a lensectomia. a lensectomia. submetidos congenital cataract surgery: an 18-year 2000;78:65-70. Ophthalmol Scand. longitudinal follow-up. 27. 174 R e v i s ta C i e n tífi c a d a Or d e m d o s M é d i c o s w w w.a c ta m e d i c a p o r tu g u e s a .c o m Moreira J, et al. Pediatric cataracts: a retrospective study, study, a retrospective cataracts: J, et al. Pediatric Moreira Oliveira ML, Di Giovanni ME, Porfírio Neto Jr F, Tartarella MB. Catarata Tartarella Oliveira ML, Di Giovanni ME, Porfírio Neto Jr F, Katina Katina JH, Lyra JM, Souza CD, Trindade FC. Tratamento cirúrgico da catarata pediátrica. Arq Bras Oftalmol. 2002;65:193-7. catarata pediátrica. congénita: aspectos diagnósticos, clínicos e cirúrgicos em pacientes 26. 25.

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