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 of the head. Membranous from about 5 years of age. For younger children, from about cataracts represent last stage of re-absorption of the lens, 3 years of age recognition acuity can be assessed using leaving either a disc of lens material or the anterior and symbols.46 Cardiff acuity test is useful for toddlers.47 Teller posterior capsules fused together. It is common in PFV,35 acuity test is recommended usually for infants.48 Visual trauma36 and congenital rubella.37 Zonular cataract is the evoked potential, fixation-refixation pattern and pupillary most common type of congenital cataract.38 It involves reflexes can give idea about the child’s visual potential. one or more layers or zones of the lens. These cataracts are usually idiopathic or inherited as an autosomal dominant Parent Counselling trait. Typically, they are bilateral but slightly asymmetrical, Parents should understand that surgery is one of the and are composed of a layer of minute white dots in a single steps in management of the childhood cataract; in fact or more layers of the lens. The embryonic nucleus is spared, treatment starts after surgery. They need to come for regular

www.djo.org.in 161 ISSN 0972-0200 Major Review Patil B et al follow-up visits and see that the child wears glasses or accuracy between SRKII, SRK-T, Holladay and Hoffer Q. contact lenses despite the IOL implantation; the child may Another study found that theoretic formulas were slightly also need occlusion therapy following surgery. Regular more accurate than regression formulas but no IOL power follow-up is essential for the successful management of calculation formula was satisfactory.57 Errors of IOL power childhood cataract. calculation are more if AL is <20 mm and age <36 months.58

Biometry and Keratometry Indications of Surgery Because of a lack of cooperation in the clinic setting, Indications for cataract surgery include visually axial length (AL) measurements of young children often significant central cataracts larger than 3 mm in diameter, must be obtained in the operating room under general dense nuclear cataracts, cataracts obstructing the examiner’s anesthesia. A-scan ultrasound biometry is the conventional view of the fundus and cataracts associated with strabismus, method for the measurement of AL in children. Many A-scan abnormal eye movements.59 instruments are available. For accurate measurements one must ensure calibration49 The instrument must have an Surgery oscilloscope screen such that true echo spikes are observed Paediatric eyes are not just miniature adult eyes. They in determining axiality.49 Instruments that merely give a differ from the adult eye in many aspects. numerical reading of the AL do not allow clinical decision making during the examination and are fraught with Applied Anatomy potential for errors. Ultrasound biometry can be done 1. Capsule: The anterior capsule is thinnest at birth, with either contact or immersion method. In the contact increasing in thickness with age. The young anterior method, the probe touches the cornea and may result lens capsule is strong and very elastic. The elderly in corneal compression and a shorter AL. Because of low anterior lens capsule is, by comparison, weak and corneal and scleral rigidity, corneal compression is more inelastic. Krag and coworkers60 found that anterior likely in paediatric eyes. Using the immersion technique, lens capsule extensibility was maximal in infancy and the ultrasound probe does not come into direct contact decreased about 0.5% per year throughout life. with the cornea, but instead uses a coupling fluid between 2. Anterior chamber (AC): It is shallow as compared to the cornea and the probe preventing corneal indentation. adults. Anterior chamber is shallower in the cataractous Immersion A-scan has been shown to be superior to contact eye in case of unilateral cataract.61 50 51 biometry in children. Trivedi and Wilson, proved that, 3. Sclera and vitreous AL measurements made with a contact technique were, on a. non liquefied vitreous and thin sclera with low rigidity the average, 0.24–0.32 mm less than measurements made results in vitreous up thrust. Thus to maintain chamber using an immersion technique. In spite of this advantage, stability and make a good anterior capsulorrhexis, indentation method is more commonly used, (82.4% vs. use of high viscosity ophthalmic viscosurgical device 51 17.6%). (OVD) is must. Optical biometry is based on partial coherence b. Thick vitreous gel gives protection against macular interferometry. There are currently two optical biometry edema. devices on the market and the use of both devices in children 4. Rapidly dividing lens epithelial cells (LECs), results 52,53 has been reported. Optical biometry has been used in in high incidence of visual axis opacification (VAO) cooperative children with reliability and accuracy. Though necessitating primary management of the posterior hand held keratometer is best for recording keratometry, capsule. manual keratometer and optical instruments can also be 54 used. Surgery: Key Considerations Wound construction: superior incision allows wound to IOL Power Calculation in Children be protected by eye lid and by Bell’s phenomenon in trauma Constant size of adult eye ensures stable refraction prone childhood years. Adult studies showed, corneal in postoperative period. However ocular growth has a tunnel incision has potential risk of endophthalmitis,62 but profound effect in children causing myopic shift. Surgeons in children the surgical incisions are sutured. This may can either choose hyperopia, with expectation of myopic shift diminish the risk of post op endophthalmitis. with time; or can opt for emmetropia to help with amblyopia Anterior capsule: Owing to the factor mentioned in management. Each of these approaches has advantages and applied anatomy section, anterior curvilinear capsulorrhexis disadvantages. We prefer to leave a child hyperopic, using a (ACC) is difficult in children as compared to adults. Though logical approach based on knowledge of how the refraction various techniques (Vitrectorhexis, Fugo blade, diathermy is likely to change with age. Various guidelines are available assisted, can opener etc) have been described for paediatric 55 on residual hyperopia and IOL power undercorrection. ACC, manual ACC is gold standard and most preferred No IOL power calculation formula was found to be superior technique.63 Hydro dissection: Advantages of cortical- over others and there is no general consensus over the choice cleaving hydrodissection, (ease, safety, and efficacy of 56 of formula. Andreo et al found no significant difference in removing lens matter) are well known. Mutiquadrant

162 Del J Ophthalmol 2015;25(3) E-ISSN 0976-2892 Pediatric Cataract Major Review hydrodissection (at least 3 quadrants) is preferred method spectacles, contact lens and epikeratophakia (outdated now in paediatric group. It not only helps in lens aspiration but due to complications). Children in the preschool-age may be also ensures removal of LECs.64 provided near add incorporated in the retinoscopy where Posterior capsulotomy and anterior vitrectomy: unlike as older children should be given bifocal glasses. Need of adults, Visual Axis Opacification, VAO is more common glasses should be explained to parents. This also requires in children. Anterior vitreous face is more closely linked repeated refraction and change of glasses over time. to posterior capsule and more reactive in children. It Postoperative amblyopia therapy should be instituted acts as a scaffold for proliferating LECs and metaplastic meticulously. Occlusion therapy for unilateral cataract after pigment cells. Anterior vitrectomy breaks this scaffold, surgery should be started early as these children are at thus preventing VAO formation. Decision about Posterior higher risk of developing amblyopia. curvilinear capsulorrhexis (PCC) and vitrectomy is based on age of patient at surgery. Posterior caapsulotomy is must Post-operative Complications for all patients less than 8 years of age. Vitrectomy can be Glaucoma following cataract surgery in children is deferred after 5 years of age.65 IOL implantation: Primary well documented. It may occur in the early postoperative IOL implantation is preferred, if not contraindicated.66 The period or as late as decades after surgery. Various studies problem lies in the sizing of IOLs. Adult size IOLs are known have found glaucoma to be observed in 10-25% of children to cause capsular bag ovaling, bag distension syndrome after paediatric cataract surgery.68-75 Early age at surgery and displacement. Ovaling was seen least with single piece and presence of microphthalmia are high risk factors. acrylic hydrophobic lenses.67 Acrylic hydrophobic IOLs Trivedi et al8 have found the incidence of glaucoma to be have been found to be efficacious, but long-term results are comparable between pseudophakic and aphakic eyes. In unknown.66 contrast, no glaucoma was reported by Cassidy et al76 who found a protective role of primary lens implantation against Post-operative Management glaucoma. Visual axis opacification is the most common Postoperative management of Paediatric cataract complication after pediatric cataract surgery with or surgery is unique, surgery is only a part of the management without IOL surgery.77,78 If not treated on time it may cause and success of the depends on the postoperative follow visual deprivation amblyopia. Primary posterior continuous up, multiple examinations under anesthesia (EUA) as curvilinear capsulorrhexis and anterior vitrectomy or applicable, amblyopia management and early detection and posterior capsulotomy with endodiathermy of capsule or treatment of complications. posterior capsulorrhexis with optic capture have been used in paediatric population and have shown to decrease VAO Post-operative Medications formation.79-82 In a thick VAO, surgical management in the The regular medications post-surgery in a case of form of posterior capsulotomy combined with anterior paediatric cataract surgery comprise of antibiotic eyedrops, vitrectomy is required to prevent amblyopia. Nd: YAG laser steroid eyedrops and cycloplegics. Inflammatory response has also been used.83 following cataract surgery is more intense in children and Postoperative uveitis is a common complication due hence requires intensive topical steroids. The frequency and to increased tissue reactivity. This can result in posterior duration for their use needs to be tailored depending on the synechia formation leading to seclusio pupillae, iris bombe preoperative diagnosis and postoperative response. Usually and subsequent secondary angle closure glaucoma and it is tapered over a period of six to eight weeks. Topical VAO. Due toimproved surgical techniques and frequent antibiotics are instilled three times a day for two to three use of topical steroids and cycloplegics in the postoperative weeks. Cycloplegics stabilize the blood-aqueous barrier, period uveitis and its related complications have been seen to relieve ciliary spasm and dilate the pupil. Hoamatropine decrease. Pupillary capture among children is high, varying eye drops 2% four times a day or atropine eye ointment 1% from 8.5% to 33%.38,83 In-the-bag IOL fixation decreases the thrice a day can be used. incidence of this complication.85 IOL decentration is also common which can be prevented by in the bag placement Follow up of IOL.84 These children require life-long follow up. They are Retinal detachment is a rare but sight threatening seen on the first postoperative day then first week and then complication, more common in eyes with Persistent foetal as and when required depending on surgeon’s protocol. vasculature Cystoid macular oedema following congenital EUA is planned after six to eight weeks for suture removal cataract surgery has been described but is self-limiting and if non absorbable suture has been used. At each visit, visual usually not problematic. acuity, slit lamp examination, intraocular pressure and fundus should be noted. Financial & competing interest disclosure The authors do not have any competing interests in any product/ Correction of Refractive error procedure mentioned in this study. The authors do not have any financial Residual refractive error should be corrected as early interests in any product / procedure mentioned in this study. as possible. Options for correction of refractive error include

www.djo.org.in 163 ISSN 0972-0200 Major Review Patil B et al

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