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Pediatric Cataract Delhi J Ophthalmol 2015; 25 (3): 160-165 DOI ISSN 0972-0200 Major Review Pediatric Cataract Delhi J Ophthalmol 2015; 25 (3): 160-165 DOI: http://dx.doi.org/10.7869/djo.99 Bharat Patil, Reetika Sharma, Paediatric cataract is one of the most important surgically treatable causes of childhood blindness. Bhagbat Nayak, Treating pediatirc cataract has a large impact on society as ‘lost blind years’ can be saved. Various morphological types are known of which zonular has the best visual prognosis. Rubella accounts for Gautam Sinha, the most common preventive cause for pediatrc cataract. Intraoperative biometry plays an important Sudarshan Khokhar role though in coperative children optical biometry may be suitable option. Since the pediatric eye and Cataract, Refractive Surgery & pediatric lens is not a minature adult eye and lens repsectively, surgical steps needs to be modified. Glaucoma Services Intraocular lenses (IOL) provide the best available option for visual rehabilitation after removal of Dr Rajendra Prasad Centre for cataract because of the constant visual input provided. Poor intraocular lens power predictability, Ophthalmic Sciences, increased inflammation, postoperative complications and the technical difficulty of surgery are the All India Institute for Medical Sciences, main concerns for IOL implantation. Surgery is just a step towards management of pediatric cataract, New Delhi 110029, India amblyopia therapy, glasses correction, and log term follwup are essetinal for better outcomes. Keywords : pediatric cataract • Lens aspiration • Posterior capsulorhexis *Address for correspondence According to World Health metabolic disorders,8 prematurity,9 and Organisation (WHO), every minute a intrauterine infections.10-13 Significant child goes blind somewhere in the world.1 causes of childhood cataract in older Childhood blindness has a socioeconomic children include trauma,10-12 drug-induced impact over child, family and the society cataract,14 radiation therapy,15 and laser due to ‘blind years’. Cataract being therapy for ROP.16 Bharat Patil MD one of the leading causes of childhood Angra SK10 studied 200 cases of Senior Resident, blindness and being treatable, it is logical congenital cataract and found that 31% Cataract, Refractive & Glaucoma to think that an improved approach to were idiopathic, 14% were hereditary, and Services the management of childhood cataract 21% might have been due to rubella. They Dr Rajendra Prasad Centre for would have large impact on childhood didn’t examine parents and rubella was Ophthalmic Sciences, blindness.2-4 The control of childhood diagnosed on clinical grounds alone. Jain All India Institute of Medical Sciences, blindness has been identified as a priority 11 New Delhi 110029, India et al prospectively enrolled 76 children E-mail: [email protected] of the WHO global initiative for the with cataract from a general clinic over elimination of avoidable blindness by the 1 and 1/2 years and noted that 20% of year 2020.2 In India, lens related causes the cataracts were hereditary, 9% were accounts for 7.4-12.3% of childhood due to metabolic diseases, and 5% had blindness3-4global number being 14%.5 an associated syndrome and 46% were The prevalence of childhood cataracts idiopathic. Nearly 8% had a positive has been reported to vary between 1.2 and rubella titre but the disease may have been 13.6 cases per 10000 children.6 The wide acquired after birth and the significance range is due to variety of methods, case is questionable. Michael et al12 evaluated definitions, different age groups, as well 514 consecutive patients of congenital as true differences between populations. cataract in a hospital based study. Of the 366 children with nontraumatic cataract, Aetiology of Childhood Cataract 25% were hereditary, 15% were due to Though traditionally it has been congenital rubella syndrome, and 51% described that roughly one third were undetermined. In children under 1 childhood cataracts are inherited, one year of age 25% were due to rubella and third are associated with other diseases cataract of nuclear morphology had a 75% or syndromes, and the remaining one positive predictive value for congenital third are idiopathic; the aetiological rubella syndrome. classification of childhood cataract is not In approximately 50% of bilateral cases so simple and straight forward. Different and virtually all of the unilateral cases, the studies give varying results. Important underlying cause could not be determined. causes of childhood cataract in younger Approximately 20% of cases are associated kids include genetic aberrations,7 with a positive family history.17 About one 160 Del J Ophthalmol 2015;25(3) E-ISSN 0976-2892 Pediatric Cataract Major Review third of cases are hereditary, without a systemic disease.18,19 but sometimes involving the foetal nucleus, and with clear Autosomal dominant transmission is responsible for 75% cortex outside them. There is often a marked inter-ocular of congenital hereditary cataracts and affected children are and intra-familial variability.39 morphologicaly cataract is usually perfectly alright. Less commonly the inheritance often incomplete, and may have projections from their outer may be autosomal recessive and X-linked.20,21 Rare causes edges known as ‘riders’. The visual prognosis, especially in of childhood cataracts are metabolic disorders such as partial cataract, is better than in many other morphological galactosemia and hypocalcemia.22 Galactosemia causes types.34 characteristic oil ‘droplet cataracts’. About 10 – 30% of Sutural cataract is a special type of zonular cataract, in classic galactosemia cases develop cataracts in the first few which there are opacities around or involving the sutures. days or weeks of life, which usually clear once the baby is They are often familial and range from an increased density put on a galcatose free diet.20 Congenital cataracts may be of the sutures to a variety of whitish or cerulean dots associated with systemic abnormalities such as trisomy21 clustered around either or both sutures. They are visually and Turner’s syndrome. Mental retardation is commonly insignificant unless they progress to nuclear.38 Anterior polar associated with bilateral congenital cataract, and there are and anterior pyramidal cataracts represent abnormalities many inherited syndromes with this combination linked of lens vesicle detachment. Anterior pyramidal cataracts with other abnormalities such as craniofacial or skeletal are more likely to be visually significant and to progress deformities, myopathy, spasticity, or other neurological than anterior polar cataracts. They may become detached40 disturbances.23 Trisomy21 is the most common systemic and may form an anterior chamber foreign body. Anterior association of sporadic cataract, upto 13% cases of Down’s lenticonus may be sporadic or may be associated with other syndrome are associated with cataract.24 disorders. Bilateral anterior lenticonus is characteristic Several intrauterine infections (toxoplasmosis, feature of Alport’s syndrome. It may be a manifestation of rubella, cytomegalic inclusion disease, herpes infection, a basement membrane disorder.41 It can be congenital and varicella, and syphilis) can cause congenital cataracts.21,25-27 is found in about 10% of affected young children,42 but may Of these, rubella is the most important. Rubella cataract increase in frequency with time up to 30%.43 is usually bilateral but may be unilateral.13 Unilateral Posterior lenticonus is a unilateral or bilateral and congenital cataract is generally not associated with systemic asymmetrical, thinning and posterior bowing of the disease and is rarely inherited; in most cases, the cause is posterior lens capsule. Patient may either have a high degree idiopathic.28 Some cases are associated with lenticonus or of astigmatism, often irregular, without cataract. If cataract lentiglobus and persistent fetal vasculature (PFV).21 Trauma present it may be a progressive or localised It is not usually is one of the common causes of unilateral childhood cataract associated with any systemic disease, unlike anterior in developing countries.29,30 lenticonus. It can be sporadic, autosomal dominant44 or X linked.45 Morphology Morphology of the cataract is important for several Preoperative Workup reasons: it not only gives a clue to the age of onset, History heritability, and aetiology but may also be important for A good history can give a clue to aetiology. Presenting visual prognosis. Some morphological types have a better complaint may give clue to prognosis, as patient presenting visual prognosis than others, with lamellar cataracts and with leucocoria may have better prognosis as compared to posterior lenticonus doing well and dense central cataracts those with squint or abnormal eye movements. Similarly, relatively poorly.31-33 Total cataracts involving nearly the longstanding opacity hampers prognosis due to image entire lens occur in Trisomy 21, acute metabolic cataracts deprivation amblyopia. Any significant family, intranatal, and congenital rubella, but may be seen in familial or postnatal history should also be noted. History of associated sporadic cases.34 illness should also be elicited. Detailed history of trauma In congenital Morgagnian cataracts, the outer zones of can indicate the severity of damage. the lens become liquefied whilst the nucleus remains intact. This allows the nucleus to fall by gravity in any direction, Visual assessment Letter acuity assessment is normally possible in children depending on the position
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