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Infantile Cataract: Where Are We Now?

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Infantile Cataract: Where Are We Now? Major Review Infantile cataract: where are we now? Praveen Kumar KV and Sumita Agarkar Correspondence to: Introduction disorder but also helps in planning the manage- Dr. Sumita Agarkar, Pediatric cataract is one of the major causes of pre- ment. Based on morphology, pediatric cataracts – Deputy Director Pediatric ventable childhood blindness affecting approximately can be classified into cataracts involving the Ophthalmology Department, 1 Sankara Nethralaya 200,000 children worldwide. In developing countries, entire lens, central cataracts, anterior cataracts, Medical Research Foundation the prevalence of blindness from cataractC is higher, posterior cataracts, punctate lens opacities, coral- 18, College Road, about one to four per 10,000 children. Early diag- line cataracts, sutural cataract, wedge shaped cata- Chennai - 600 006 nosis and treatment WWWis essential to prevent ract and cataracts associated with PFV. email: [email protected] the development of stimulus deprivation ambly- opia in these children. Cataract surgery in infants Preoperative evaluation poses greater challenges compared to young chil- History taking is an integral part in the evaluation dren. Primary implantation of an intraocular lens of an infant with congenital cataract. The history remains controversial for infants, and the selec- should include tion of an appropriate IOL power is difficult. The Family history of congenital or developmental management of infantile cataract has changed cataract, over the last decade. In this study, we present an 1. Antenatal history of maternal drug intake and overview of the changing concepts of cataracts in fever with rash. infants and its management. 2. Birth history should be specifically looked for Etiology of childhood cataract as bilateral congenital cataract is more The common causes of congenital cataract are common in preterm, low birthweight, small genetic, metabolic disorders, prematurity and intra- for gestational age children.5 uterine infections. Almost 60% of cases of congeni- 3. Developmental mile stones should be carefully tal cataract in developed countries are idiopathic.2 assessed. One-third of cases of congenital cataract are her- editary without any known associated systemic 4. History of visual interaction of the child with disease.3 The various causes of congenital cataract the family members should also be inquired are as it helps in determining the severity of visual dysfunction. 1. Heredity: These cataracts are usually auto- somal dominant but can be autosomal reces- 5. History of the onset of the opacities, progres- sive and x linked. sion and laterality is also important. Unilateral cataracts are isolated but are 2. Associated with genetic disorders: Seen in usually associated with other ocular abnor- downs and turners syndrome. malities like persistent fetal vasculature, lenti- 3. Metabolic disorders: Galactosemia, Hypocalcemia. conus, lentiglobus.6,7 4. Intrauterine infections: Toxoplasmosis, Rubella, Cytomegalovirus, Herpes, Varicella and Syphilis. Examination of the child Detailed ocular examination of the child can be 5. Associated Ocular conditions: Aniridia, Iris done either in an outpatient setting if the child is coloboma, lens coloboma, Lenticonus, cooperative or under general anesthesia when the Lentiglobus, Persistent fetal vasculature. child is being taken up for surgery. Quantification Next generation DNA sequencing shown prom- of visual acuity of the child as far as possible ising results in identifying mutations leading to should be done. In infants, fixation behavior, fix- metabolic abnormalities and cataracts. This new ation preference and resistance to occlusion gives technology has immense potential in identifying us a clue to the visual acuity. In young infants metabolic abnormalities and correct them with with poorly developed fixation, an undilated appropriate intervention. In future, this technology distant direct ophthalmoscopy can indicate whether is likely to become cheaper to allow testing in chil- the opacity is visually significant or not. Dense dren with non-syndromic bilateral cataracts with central opacities larger than 3 mm in diameter systemic manifestation and delayed development.4 usually need surgical removal.8 Examination of both the eyes has to be done to Morphology of pediatric cataract determine whether the cataract is unilateral or Morphology of pediatric cataract can not only bilateral. Unilateral cataract, even if mild can help in the diagnosis of specific associated cause irreversible deep amblyopia if not treated.9 126 Sci J Med & Vis Res Foun October 2015 | volume XXXIII | number 3 | Major Review Often the first symptom is a white or partially should be treated by appropriate glasses and patch- white reflex noted by the parents. Strabismus and ing. Small opacities can be managed by prescribing nystagmus should be specifically looked for in mydriatric agents to achieve a larger area of clear these children and sometimes these may be the visual axis. presenting signs. Strabismus is usually seen in children with unilateral cataracts and develops Timing of surgery when an irreparable visual loss has already Extraction of unilateral congenital cataracts by 4– occurred. The presence of manifest nystagmus at 6 weeks and bilateral cataracts within 6–8 weeks age of 2–3 months or elder generally indicates a of life can prevent the development of strabismus, very poor prognosis.10 The presence of either stra- nystagmus and amblyopia.14 bismus or nystagmus indicates that cataract is visually significant. How does an infant eye differ from the adult eye? Slit lamp biomciroscopy should be done to A child’s eye is unique and is different from an assess the size, location, density of the opacity. adult eye. The eyes are smaller in size at birth and Corneal diameters and intraocular pressure have have steeper corneas. The normal new born eye has to be measured with a tonopen or Perkins hand a mean axial length of 16.6–17 mm.15 It reaches a held applanation tonometer. Indirect ophthalmos- mean adult value of 23.6 mm at 15 years age. More copy can reveal persistent fetal vasculature or other than half of this growth in axial length occurs posterior segment abnormalities that may affect the before 1 year age and most axial elongation occurs visual outcome. In cases where the media opacity during the first 2 years of life. The corneal curva- precludes examination of the fundus, a B scan ultra- ture reduces from 51.2 D in new borns to 43.5 D in sonography has to be performed to rule out other adults.16 The sclera is thin and less rigid, the lens posterior segment pathologies that mimic congenital capsule is more elastic, and there is a risk of severe cataract. These conditions include retinoblastoma, postoperative inflammatory response. persistent hyperplastic primary vitreous, coats disease, ROP with retrolental fibroplasia, orgainzed Biometry in infants vitreous hemorrhage, congenital falciform fold, With advances in surgical techniques and instru- ocular toxocariasis and retinal hamartomas. mentation, several surgeons are implanting IOLs in Performing cataract surgery in these conditions is infants. Refractive growth after IOL implantation in disastrous and can lead one into medicolegal pro- infants cannot be predicted accurately and current blems. These children should be evaluated by a IOL formulae vary in their predictive outcomes. If r pediatrician to exclude systemic disorders or meta- target postoperative emmetropia, amblyopia treat- bolic causes causing cataract. ment is easier but this strategy results in high myopia in later life. If we aim for hyperopia, ambly- Laboratory workup opia therapy and refractive correction in initial Most children with congenital cataract do not phase is difficult but thisstrategy has the advantage need systemic work up. Unilateral, familial, iso- of potentially achieving either emmetropia or low lated cataracts with no systemic association do myopia later in adulthood. The amount of hyper- not need any systemic investigations. Trauma opia will vary depending on the age of the child at should be ruled out in all cases of unilateral cata- the time of surgery. Most surgeons prefer to leave ract. A child with peculiar facies or systemic mal- infants with hyperopia as it’s easy to titrate as chil- formations like microcephaly, deafness, cardiac dren grow. IATS recommended an hyperopia abnormalities, developmental delayneed systemic ranging from +6 D to +8 D depending on the age of workup. The work up usually includes fasting infant at the time of surgery. It is important to blood sugar, urine for reducing substances for counsel parents regarding need for glasses post- galactosemia, aminoacids for Lowes syndrome. operatively as well as perhaps through the life. Plasma phosphorous, calcium levels, RBC transfer- Three important things to be considered when ase and galactokinase levels have to be assessed. determining the IOL power to be implanted in TORCH titers have to be done to rule out infec- infants are tious causes of cataract.11,12 1. Anticipated refractive shift. Management 2. Age of the patient. Indication for cataract surgery in infants depends 3. Target refraction in the immediate postoperative on the extent of its effect on the visual function. period. Mere presence of a lenticular opacity does not warrant surgical removal. Cataract which is incom- Children who are younger at the time of plete at birth, peripheral lens opacities, punctate surgery, have a significantly greater myopic shift opacities with intervening clear zones,
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