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 Table of Contents  
Year : 2018  |  Volume : 30  |  Issue : 3  |  Page : 162-171

Pediatric cataract

Department of Pediatric Ophthalmology, Little Flower Hospital, Angamaly, Kerala, India

Date of Web Publication17-Dec-2018

Correspondence Address:
C K Meena
Department of Pediatric Ophthalmology, Little Flower Hospital, Lakshmi, P. T Usha Road, Ernakulum - 682 011, Angamaly, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/kjo.kjo_85_18

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Pediatric cataract remains a very important and difficult problem to manage, in spite of dramatic advances that have occurred in the field over the past 10 years. Since early treatment is the most important factor in determining the visual outcome in congenital cataract, prompt detection and treatment in the neonatal period are the aims. This review is to update the reader on advances and present concepts on the topic.

Keywords: Anterior vitrectomy, intraocular lens, posterior capsule, prediction error, primary posterior capsulorhexis

How to cite this article:
Joseph E, Meena C K. Pediatric cataract. Kerala J Ophthalmol 2018;30:162-71

How to cite this URL:
Joseph E, Meena C K. Pediatric cataract. Kerala J Ophthalmol [serial online] 2018 [cited 2022 Oct 7];30:162-71. Available from: http://www.kjophthal.com/text.asp?2018/30/3/162/247597

  Definition and Epidemiology Top

Cataracts are opacities of the crystalline lens. Bilateral congenital cataract is the most common cause of treatable blindness in children, worldwide. The incidence varies in different countries.[1] In the developing countries, the prevalence of blindness from congenital cataract is higher, about 1–4/10,000 births. It accounts for 7.4%–15% of pediatric blindness and a significant avertable disability-adjusted life years.[2]

  Etiopathogenesis Top

The cause of bilateral congenital cataract in most cases is idiopathic. The most common etiologies include intrauterine infections such as rubella, toxoplasmosis, herpes simplex, and varicella [Figure 1].[3] About one-third of cases is hereditary, without a systemic disease. Inherited cataracts differ morphologically within the same pedigree. These are mainly autosomal dominant, but autosomal recessive and X-linked traits occur.
Figure 1: Bilateral rubella cataract

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Galactosemia and hypocalcemia are metabolic disorders with congenital cataract. Infants with classical galactosemia develop oil-droplet cataracts, and if left untreated, these progress to lamellar and then total cataracts due to the accumulation of galactitol in the lens. However, if galactose is eliminated from the diet of these children, cataracts may become transparent again. Hence, it is important to test for the presence of reducing substances in the urine after a galactose-containing meal [milk] in all infants with cataract. Enzymatic assays and DNA studies can then be used to confirm the diagnosis.

Hypocalcemia leads to seizures, failure to thrive, and irritability in children. Altered permeability of lens capsule results in cataract. These cataracts generally begin as fine white punctate opacities scattered throughout the lens cortex which may then progress to lamellar cataracts. Serum calcium and phosphorus levels should be measured in infants with congenital cataracts.

Cataracts are manifested in large number of syndromes such as Trisomy 21, Turner syndrome, Trisomy 13, Lowe syndrome, Alport syndrome, Nance–Horan syndrome, Martsolf syndrome, and Marinesco–Sjogren syndrome to name a few.[4] Associated mental retardation is common. Many genes involved in cataractogenesis have been identified. At present, there are about 39 genetic loci to which isolated or primary cataracts have been mapped, and the number is constantly increasing.[5] Of these, several are associated with additional abnormalities.

  Morphology Top

Nuclear cataract is usually present at birth and is nonprogressive.[6] In cases with dense cataracts present at birth, it is usually nuclear. The opacification is located in the embryonic and fetal nuclei between the anterior and posterior Y sutures and is usually very dense in the center. The eyes may be smaller than normal. The cataract is bilateral in 80% of cases, and inheritance can be demonstrated in 30%–50% of cases.

Posterior cataract in infants and children is commonly associated with persistent fetal vasculature (PFV), and the affected eye is microphthalmic.[7] Cataract is a secondary event in PFV due to invasion of the vascular remnants, lenticular swelling, intralenticular hemorrhage, or calcification. PFV findings are seen in one-fifth of unilateral cases and less commonly in bilateral cataracts [Figure 2]. The retrolental vasculature may be in contact with the lens capsule and may bleed during surgery. Tractional retinal detachment and secondary glaucoma are common postoperative complications.
Figure 2: Cataract with persistent fetal vasculature

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Lamellar cataract usually develops after fixation is established. It is usually progressive and involves the lamellae surrounding the fetal nucleus, peripheral to the Y sutures [Figure 3]. Eyes are normal sized with normal corneas, and cataract is uniform bilaterally and has an autosomal dominant inheritance. Surgery can often be delayed and is undertaken when visual demands are compromised.
Figure 3: Lamellar cataract

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Other morphological types such as sutural cataract and anterior polar cataract have less influence on vision. Other associated ocular abnormalities include microphthalmos, lenticonus, cornea guttata, anterior segment dysgenesis, and Stickler syndrome.

  Preoperative Evaluation Top

Visual loss and development of amblyopia depend on the size, location, and density of the opacity. If the opacity is large enough to obscure fundus view through an undilated pupil, amblyopia development can be expected. If the retinal details such as the larger vessels can be distinguished through the central portion of the cataract, conservative treatment can be considered, but occlusion therapy is necessary in unilateral cases, and constant follow-up to evaluate the monocular and binocular visual behavior should be undertaken.

Visual assessment should be performed using patterns of fixation and supplemented when possible by preferential looking charts or pattern visual evoked potentials. Measurement of corneal diameter, intraocular pressure, pupillary reflexes, ultrasonography, and indirect ophthalmoscopy should be carried out.

  Investigations Top

In unilateral cases and in an otherwise healthy infant with one parent involved by the disease, an extensive preoperative investigation is not necessary to establish the cause for cataract.[3] Antibody titers for rubella, toxoplasmosis, herpes simplex, blood sugar, serum calcium, and urine examination for reducing substances should be done in all cases. Further investigations such as plasma electrolytes, amino acid studies, enzyme studies, and chromosome studies need be carried out only in appropriate cases and with the collaboration of a pediatrician and often are not rewarding.

  Timing of Surgery Top

Dense congenital cataract from the available data, it would appear that the optimal time to remove a dense congenital cataract in an infant and to initiate treatment is when the child is 4–8 weeks of life.[8] Cataract surgery before 4 weeks of age appears to increase the risk of secondary glaucoma, whereas waiting beyond 8 weeks of age compromise visual outcome.[9] The visual system which is immature at birth has a latent period of approximately 6 weeks before it becomes sensitive to visual deprivation, and binocular vision first appears at approximately 3 months of age. The pathophysiology of aphakic glaucoma is poorly understood. Its etiology has been attributed to the damage to the trabecular meshwork by inflammation, the loss of mechanical support of the trabecular meshwork, or a toxic substance gaining access to the trabecular meshwork from vitreous and may present as an early angle-closure glaucoma or late open-angle glaucoma and has been reported to occur after both limbal and pars plicata-based surgeries.

If the cataract is incomplete at birth, close follow-up is advised. Visual acuity should be followed, and a history about the visual interaction with parents should be noted. The evidence of squint or nystagmus is an indication for immediate intervention.[10] If the child has unilateral partial cataract, occlusion therapy should be considered.

In bilateral cataracts, whether to do simultaneous bilateral surgery or sequential unilateral surgery is still debated. It is advantageous to perform surgery in both eyes at the same intervention for simultaneous initiation of visual rehabilitation and reducing the exposure to general anesthesia. The second eye procedure needs new set of instruments and solutions. However, there is the fear of bilateral visual impairment and fear of endophthalmitis when both eyes are operated at the same time

  Surgical Management of Pediatric Cataracts Top

The management of pediatric cataract is surgical unless it is a nonprogressive opacification in the peripheral lens not obscuring the visual axis. Surgical management of children is different from that of adults since here the main issues are dealing with media opacities which can occur recurrently even on doing meticulous surgical clearing of the cataractous lens. The next important hurdle is the management of aphakia with refractive corrections or intraocular lens (IOL) implantation

  Differences between Pediatric and Adult Eyes Top

Corneal diameter, anterior chamber depth, lens thickness, and axial length are quite different in pediatric eyes, and unlike in adult eyes, these values keep on changing with the aim of possible emmetropization. The ocular dimensions still become variable when any procedures are done on these growing eyes. The axial length at birth is approximately 17–17.5 mm with a 2.5–3.5 mm increase in the 1st year of life. Axial length reaches adult dimensions at approximately 5 years of age. Gordon and Donzis's cross-sectional biometric study of 148 normal eyes found on average that the axial length increased from 16.8 to 23.6 mm from birth to adulthood, whereas the refraction changed minimally, from +0.4 to −0.5 D.[11] This is primarily because the power of the crystalline lens declined from +34.4 to +18.8 D. In contrast, aphakic eyes have a decline in hyperopia of 10D over the same period.[12] As there is no proportionally changing lens to compensate for the ocular growth, aphakic eyes have a large myopic shift. The various ocular parameters in normal children in literature are shown in [Table 1] and [Table 2].[13] All these factors should be considered during procedures on pediatric eyes.
Table 1: Horizontal corneal diameter in normal eyes

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Table 2: Axial length, anterior chamber depth and Lens thickness in normal eyes

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  Basic Surgical Procedure for Pediatric Cataracts Top

Surgical steps in pediatric cataract differ from that of adults, especially in the management of posterior capsule. The anatomical statuses of a child's eye make the procedure more challenging. Furthermore, insertion of the IOL is yet another issue as in very young age, it is deferred. Discussing the procedure step by step, even draping and speculum application are challenging in children due to the small size. Incision in children is mainly dependent on the surgical procedure as if it involves only lens aspiration and no IOL as the whole procedure can be managed through side ports. Scleral incisions with conjunctival peritomy are rarely used unless we opt for a rigid IOL due to economic reasons or due to any contraindication for foldable IOLs. Scleral incisions would be ideally made 2 mm from the limbus, and care should be taken considering the thinner sclera in children. A longer scleral tunnel would be preferable as iris prolapse is possible, especially during the learning curve. The authors prefer clear corneal incision of 2.8 mm superior in small children and temporal as they age considering the factor of wound exposure and eye rubbing in children. A study comparing postoperative astigmatism following scleral tunnel and clear corneal incisions showed no statistically significant difference between the two. Furthermore, astigmatism reduced significantly 5 months postoperative in both groups.[14]

The next major step is anterior capsulorhexis. Capsule in children is quite thin and elastic in comparison to adults, and the major goal here is to obtain an ideal 5–6 mm rhexis. Trypan blue dye is commonly used for capsule staining here as it highlights the rhexis contours which is very essential for a good outcome, and even though one can get a good red glow under microscope, it would be better to stain the capsule as a mild disturbance of the cortex during the procedure itself can hinder your view. Kiel et al. reported use of trypan blue in the capsular bag after lens aspiration to detect residual epithelial cells and predicts that it helps to prevent postoperative media opacification.[15] It would be ideal to use capsule forceps which gives good control over the procedure as peripheral rhexis run is the major issue in this step [Figure 4]. Any suspicion of extension can be managed timely by making a small cut at the desired position of the run rrhexis with vitreous scissors and to continue on with forceps. All these instruments can be used through side ports which again help to maintain the anterior chamber well. Vitrectorhexis is also used by some. M Edward Wilson comparing various anterior rhexis techniques found no major difference in the outcome and concluded manual and vitrectorhexis as the most commonly used methods.[16]
Figure 4: Anterior capsulotomy

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Ophthalmic viscosurgical devices (OVDs) used in pediatric cataract also need attention. Although the commonly used dispersive OVDs itself can help you out for good anterior chamber maintenance and rhexis, many surgeons prefer cohesive OVDs such as sodium hyaluronate. A thorough wash of the viscoelastic agent at the end of the procedure is mandatory, and there are reports of postoperative intraocular pressure rise following improper wash of these agents in pediatric cataract surgeries.[17] Aspiration of lens matter is the easiest step in young cataracts and can usually be accomplished in the irrigation-aspiration mode as it is unlikely that one encounters a hard nucleus. Hydroprocedures should be done with caution and in a controlled manner as it is not rare to have a posterior capsular preexisting dehiscence in these cataracts, and vigorous hydroprocedures can enlarge the defect [Figure 5].
Figure 5: Pre-existing partial coherence dehiscence

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Management of posterior capsule and anterior vitreous is the main distinguishing feature of pediatric cataract surgery. Age limit for the same has individual preferences. However, 6 years of age has been kept as the limit by a few considering the early appearance of opacification of the capsule and difficulty in performing YAG capsulotomy in these small children.[18] We perform posterior capsulorhexis along with anterior vitrectomy up to 6 years of age, as also in older children who cannot cooperate for any procedure without anesthesia. Primary posterior capsulorhexis in children is easier to perform than the anterior mainly due to the ease of performing on a concave surface. However, one needs to be careful as to the size which should ideally be 1–2 mm less than the anterior [Figure 6]. Capsule forceps or vitrector can be used, but obviously, the forceps give a regular margin which ought to be safer during implantation. Anterior vitrectomy uses high cut rate with reduced vacuum to prevent any traction on the vitreous base and is mandatory at least up to 6 years or else the anterior vitreous face can act as a scaffold for epithelial growth [Figure 7].[19] It is common to have vitreous tags as you withdraw instruments following vitrectomy. This can be made out observing any irregularity or peaking of pupil and by sweeping the anterior chamber with iris repositor. There are surgeons who prefer the pars plana route for primary posterior capsulotomy and anterior vitrectomy which can be done pre or post-IOL insertion.[20]
Figure 6: Primary posterior capsulorhexis

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Figure 7: Anterior vitrectomy

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The next important step is insertion of intraocular lenses with its associated issues of age for implantation, power of the lens, etc.

  Intraocular Lens Power Calculations in Children Top

The ideal age for IOL implantation in children was thought to be around 2 years and above initially.[21] However, with the advent of better instrumentations and techniques whereby media opacities can be dealt with, IOLs are advised and inserted at a very younger age. Such situations are more when dealing with unilateral cataracts where early refractive correction and managing anisometropia are extremely important to prevent dense amblyopia. There are reports of IOL insertion even in the early months of infancy.[22] Authors prefer IOL insertion only from late infancy.

Four important factors to be considered in IOL insertion are age of the patient, corneal diameter, keratometric values, and axial length. Insertion in infancy has definite risks, and so, the indication at this age is mainly if the situation is demanding; for example, a baby with high risk for repeated anesthesia, doubtful postoperative follow-ups, unilateral cataracts where the anisometropia can be a very important amblyogenic factor. Still another indication is the surgeon factors such as refined surgical skills after years of experience and thorough assessment of the postoperative outcome of children who have gone through the procedure in infancy. Although infant aphakia treatment studies have shown IOL insertion before 7 months of age not to be beneficial, a long-term study of 14 eyes who underwent primary IOL implantation between 7 and 22 months was found to have a commendably good outcome.[23]

The second important consideration before IOL insertion is corneal diameter. A corneal diameter of <9.5 mm is a relative contraindication of IOL insertion. Since our ultimate aim is to achieve an acceptable visual acuity in adulthood, and as the implant used in children is of the same design and size as that of the adults, this should be taken as a key factor. Thus, if IOL is planned in an infant and corneal diameter on table found to be <9 mm, it would be ideal to postpone the same. However, if even at 2 years of age, the reduced diameter persists one can either defer IOL and opt for other refractive corrections or opt for custom made IOLs. This is based on the fact that the maximum increase in corneal diameter occurs in the first 5 months of life and is almost complete by 2 years of age.[24]

The next two important factors to be considered are similar to that of adult eyes, as keratometry and axial lengths are to be considered in any calculation formulae.

  Keratometry Top

Pediatric corneas are steeper at birth and flatten to adult values at approximately 3 years of age. This reduction is very rapid in the first 6 months of life and gradual thereafter. This strongly affects the IOL power calculation. Hence, this factor should be considered gravely. Furthermore, eye growth can be altered after a procedure to the eye and may not follow the pattern of a normal eye. The surgical wound itself can influence the corneal curvature. Automated keratometers are commonly used in children [Figure 8]; however, in cooperative ones, optical biometry can be used [Figure 9].
Figure 8: Automated keratometry

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Figure 9: Optical biometry

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  Axial Length Top

This is the most important variable factor of pediatric eye. Axial length changes rapidly in the first 18 months of life. Hussain et al. in their study on axial lengths of apparently normal eyes of children found that the extrapolated mean axial length at birth was 16.8 mm, with a rapid initial axial growth, with a mean axial length of 20 mm at 12 months of age and 21 mm at age 4 years.[25] However, all eyes need not follow the norms. It is not rare to see eyes with myopic status, and increased axial lengths though what we anticipate in a pediatric eye are the hyperopic status of reduced axial lengths. Ultrasound biometry with corneal indentation or immersion method is being used of which the latter is the preferred one. Optical biometry, partial coherence interferometry (PCI), is extensively used in cooperative children. Axial length measurement error of 1 mm can cause a 2.5 diopter error in IOL power in adults; however, this increases to 3.75 D in short eyes. Studies comparing PCI with immersion conclude that PCI tends to give lower values, particularly in eyes with an axial length longer than 23.5 mm. Depending on the length of the eye, a 0.1 mm error in AL measurement could result in a 0.25–0.75 diopter difference in IOL calculation that could be clinically significant in some patients.[26]

  Intraocular Lens Power Calculation Top

This is the most challenging part of the decision-making in pediatric cataract as the implant is inserted with a decision for lifelong survival and least postoperative errors. As mentioned earlier, it is difficult to predict the eye growth postoperatively, and the power inserted is usually based on the existing formulae and personal experience of the surgeon.

  Formulae Used in Pediatric IOL Calculations Top

Unfortunately, we still do not have a customized formula and still follow adult formulas for IOL power calculations. To add on to this issue, the power obtained by these measurements is not the ultimate, we insert in children. We need to under correct these values with the aim of achieving emmetropia in adulthood. Hence, there is always a factor of guess and experiments based on individual experiences of the postoperative refractive status obtained.

Modified theoretical and regression formulae used in adults such as SRK T, Hoffer Q, Holladay, and SRK 2, respectively, are used in pediatric cases too. A study by Andreo et al. comparing the above-mentioned four formulae did not find a statistically significant difference between the formulae as regard to the outcome.[27] Furthermore, theoretical formulae do not outperform regression formula. Neely et al. in their study concluded that the accuracy of commonly used IOL calculation formulas is generally reasonable but highly variable within the pediatric population. Newer theoretic IOL calculation formulae did not outperform older regression models. Lens calculation errors predicted by each of the four formulae studied demonstrated a high degree of variability with the SRK II being the least variable and the Hoffer Q being the most variable, particularly among the youngest group of children with the axial lengths <19 mm.[28] The Holladay 1 and SRK/T formulae gave equally good results and had the best predictive value for infant eyes says the Infant Aphakia Treatment Study (IATS).[29] Vasavada et al. in their study comparing the various modern IOL formulae concluded that in pediatric eyes, SRK/T and the Holladay 2 formulae had the least PE. Personalizing the lens formula constant did reduce the PE significantly for all formulae except Hoffer Q. In extremely short eyes (AL <20 mm), SRK/T and Holladay 2 formulae gave the best PE.[30] Kekkunaya R in their study comparing the prediction error in children <2 years concluded that though all formulae gave high absolute prediction error, SRK 2 gave the most predictable results with least errors. The authors also have been following the SRK 2 and SRK T formulae.

  Intraocular Lens Power Adjustments Top

Selecting the ideal formula alone does not solve the problem as the IOL power has to be altered before insertion anticipating growth of the eye and the resultant myopic shift. Basically, most surgeons in their early career depend on one of the methods suggested by veterans for under-correction. However, with expertise, one gets a hang on the importance of various other factors involved and develops one's own methods. Methods followed by Dahan and Drusedau are being widely practiced.[31] The former suggested under-correcting biometry reading by 10% in children between 2 and 8 years. For children younger than 2 years, perform biometry and under-correct by 20% or use the axial length only [Table 3]. IOL power suggested for 21 mm is (22.00D), 20 mm (24.00D), 19 mm (26.00D), 18 mm (27.00D), and for 17 mm, axial length is 28.00D [Table 4]. Enyedi et al. recommended a postoperative refractive goal of + 6D for a 1-years old, +5D for a 2-years old, +4D for a 3-years old, +3D for a 4-years old, +2.0D for a 5-years old, +1D for a 6-years old, plano for a 7-years old, and −1 to −2D for an 8-years old and older [Table 5].[32] IATS aims for a target refractive error after IOL implantation of 8 diopter for infants 4–6 weeks of age and 6 diopter for infants 6 weeks to 6 months of age.[33] Pediatric IOL calculator can be downloaded from the AAPOS website. This can be used for IOL power calculation in both primary and secondary IOL implantation and needs the patient's age, a constant of the intended IOL to be implanted, AL and K values. The desired postoperative refraction can also be entered to get the optimum IOL power value.[34] However, in our experience, we go through the less complicated calculation of deducting 20%–25% in <2 years, 15%–20% in 2–5 years, and 5%–10% in 5–8 years.
Table 3: IOL power undercorrection based on age and biometry (Dahan et al)

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Table 4: IOL power based on axial length (Dahan et al)

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Table 5: Post operative refractive status (Enyedi et al)

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  Types of IOL and Its Insertion Top

Hydrophobic acrylic IOLs remain the favorite of majority of surgeons [Figure 10]. However, it is not rare that we insert rigid polymethyl methacrylate (PMMA) lenses where the situation demands. Aasuri et al. have reported that uveal inflammation and incidence of visual axis opacification were significantly less with acrylic IOLs in comparison to PMMA.[35] In our experience, the surgical steps were more important than the IOL type in predicting outcomes. In the bag, IOL is the procedure of choice, however, where the situation demands, like a large posterior capsular dehiscence in posterior polar cataract and secondary IOLs where it is difficult to open up the bag a sulcus insertion is preferred. The natural position of the lens is always the preferred one and it also prevents the problems of contact with highly vascular uveal tissue and the blood aqueous barrier [Figure 11].[36] A good centration of the IOL is necessary, and for this, an ideal-sized anterior and posterior capsulotomies and a vitreous-free anterior chamber are mandatory; optic capture is preferred by few to guarantee visual axis clarity.
Figure 10: Hydrophobic acrylic intraocular lens

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Figure 11: Intraocular lens in the bag

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Wound closure with sutures is a must in these fragile eyes. We prefer both main port and side port suturing, and 10-0 vicryl is preferred if no immediate anesthesia is planned or else 10-0 nylon is used which causes least scarring. Absorbable suture such as 10-0 Vicryl is preferred over nonabsorbable suture 10-0 nylon for suturing incisions in pediatric cataract surgery, to avoid subjecting the child to repeated anesthesia is the opinion of Matalia et al. in their study comparing the two sutures.[37]

  Secondary IOL Implantation Top

In children, left aphakic secondary implantation should be considered once the cornea has reached the acceptable diameter and usually is done within 18 months of age. In secondary insertion, usually rigid PMMA lenses or foldable acrylic three-piece lenses is used, as usually only sulcus fixation will be possible here.

  Premium Procedures in Pediatric Cataracts Top

Manual capsulotomy can be replaced by thermal power generated through a probe through a radiofrequency endodiathermy generating probe, or Fugo plasma blade can be used, especially in cases of persistent fetal vasculature and fibrotic capsules.[38],[39] The latest Zepto (precision pulse capsulotomy) can also be used. However, all these require more entry and exit into these immature eyes which when weighed against the benefits could not be justified always. However, endodiathermy does have an advantage in tackling the bleeds from the persistent fetal vasculature [Figure 12] and [Figure 13]. The current femtosecond can also be used in pediatric cataract, but it does have the hassle of shifting theaters which is not quite functional when it involves general anesthesia.
Figure 12: Persistent hyperplastic primary vitreous

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Figure 13: Endocautery

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Literature provides reports on multifocals and toric IOLs in children.[40] However, this still is not very popular among pediatric ophthalmologist probably due to the simple reason that these are young growing eyes and no one can authoritatively predict the sequence of growth in these eyes. However, in post amblyopic age, they do have a significant role, especially in unilateral cases.

  Postoperative Management Top

Topical antibiotics and prednisolone in tapering doses are preferred with mild cycloplegia with homatropine for a period of 4–6 weeks. Systemic antibiotics are given only in indicated cases such as associations of cardiac anomalies where conditions such as endocarditis are expected postoperatively

  Complications Top

Immediate complications include wound-related issues such as wound gape, suture breakage, and iris prolapse. Problems due to increased anterior chamber reaction are more common in association with infections such as rubella.

Repeated visual axis opacification even after a meticulous capsule and vitreous management is the most common long-term complication encountered by us. This is especially so in cases associated with a tendency for increased inflammation. Haargaard et al. reported 3% risk for retinal detachment in isolated cataracts and higher rates when associated with systemic and ocular problems.[41] Although glaucoma has been found to be a very major complication in IATS, we have rarely encountered secondary glaucoma postcataract surgery. The IATS investigators identified that the risk of developing glaucoma at 4.8 years was 17% and that for developing glaucoma or glaucoma suspect was 31%; however, there was not a statistically significant difference between those that received an IOL at the time of surgery and those that were left aphakic.[42] Grave complications such as IOL decentrations and endophthalmitis should also be considered in these eyes.

  Visual Rehabilitation and Parental Counseling Top

This is a very essential part of pediatric cataract surgery. Visual rehabilitation here implies the correction of aphakia in the very young where IOL is not inserted. This is ideally done with contact lenses, and the ideal one for children is the silicon hydrogel contact lenses which have an extended wearing property. However, in real-life situations, whatever be the quality of contact lens, maintaining the same in a child's eye is a herculean task for parents, and recurrent loss is quite common. The only other option until one goes for a secondary IOL would be aphakic glasses with a +3 add given to the power of the main lens assuming children to be more restricted to near viewing. The increased thickness and weight of the spectacles are the major drawbacks here. Once IOL is inserted too, they need to have regular refractive evaluation and prescription of appropriate power based on ocular growth. Post-IOL insertion, they are given bifocal or progressive near vision add of +2. 5-3 diopters. Regular assessment of vision is a must and detection of amblyopia and appropriate management with refractive correction and occlusion is a must, more so in unilateral cases.

Parental counseling is an unavoidable and definite step which is as important as a meticulous surgical step. This should start from the first clinic visit and ideally should be a continuing reminding process as to the need for recurrent follow-ups; contact lens care and spectacle use; and amblyopia therapies such as patching where and when needed.

  Conclusion Top

Dense congenital cataract requires prompt surgery, and the optimum time is 4–6 weeks of age. To remove cataract before 4 weeks appears to increase the risk of secondary glaucoma. While waiting beyond 8 weeks compromises visual outcome. Nystagmus/strabismus is indications for immediate intervention. The treatment regimen consists of surgery within 2 months combined with immediate optical correction. IOL implantation is safe in children older than 1 year of life. In all children aged 6 years and younger, primary posterior capsulorhexis and anterior vitrectomy are recommended. Peripheral cataracts not obscuring visual axis are followed up, and timing of surgery depends on the visual status. Visual rehabilitation, amblyopia therapy, and parental counseling are inevitable steps of pediatric cataract management.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

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Khokhar SK, Pillay G, Dhull C, Agarwal E, Mahabir M, Aggarwal P, et al. Pediatric cataract. Indian J Ophthalmol 2017;65:1340-9.  Back to cited text no. 4
[PUBMED]  [Full text]  
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Gordon RA, Donzis PB. Refractive development of the human eye. Arch Ophthalmol 1985;103:785-9.  Back to cited text no. 11
McClatchey SK, Parks MM. Myopic shift after cataract removal in childhood. J Pediatr Ophthalmol Strabismus 1997;34:88-95.  Back to cited text no. 12
Costa AM, Calixto N, Milhomens EG, Cronemberger S. Axial length, anterior chamber depth, lens thickness and horizontal corneal diameter in normal children. Investigative Ophthalmology and visual science 2005;46:671.  Back to cited text no. 13
Bar-Sela SM, Spierer A. Clinical study. Eye 2005;20:1044-8.  Back to cited text no. 14
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  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11], [Figure 12], [Figure 13]

  [Table 1], [Table 2], [Table 3], [Table 4], [Table 5]

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Axial Length
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