IOSR Journal of Dental and Medical Sciences (IOSR-JDMS)

e-ISSN: 2279-0853, p-ISSN: 2279-0861.Volume 16, Issue 5 Ver. V (May. 2017), PP 56-61
www.iosrjournals.org

Congenital Cataract- Approach and Management Review

Dr.Bhawesh Chandra Saha1, Dr.Rashmi Kumari2, Dr Bibhuti Prasanna Sinha3
1
Senior Resident,AIIMS ,Patna
2
Senior Resident,Regional Institute Of Ophthalmology,IGIMS ,Patna
3
Professor,IGIMS,Patna
All India Institute Of Medical Sciences ,Patna ,Indira Gandhi Institute Of Medical Sciences,Patna

Abstract: Childhood cataract remains a challenge to pediatric ophthalmologists despite recent major breakthrough in
surgical techniques and instrumentation. Pediatric cataract is one of the major causes of preventable childhood blindness,
affecting approximately 200,000 children worldwide, with an estimated prevalence ranging from three to six per 10,000 live
births Congenital cataracts usually are diagnosed at birth. If a cataract goes undetected in an infant, permanent visual loss
may ensue. The management of pediatric cataract is a team effort of ophthalmologist ,pediatrician,anaesthetist and parents
and should be customized depending upon the age of onset, laterality, morphology of the cataract, and other associated
ocular and systemic co-morbidities.This review attempts to summarize the available management options to these patients
along with some analytical recommendations to optimise the outcome.
Keywords: Pediatric cataract,childhood blindness

I. Introduction
Pediatric cataract is one of the major causes of preventable childhood blindness, affecting approximately 200,000
children worldwide, with an estimated prevalence ranging from three to six per 10,000 live births1-3. It may be congenital, if
present within the first year of life, developmental if present after infancy, or traumatic. Congenital cataracts occur in about 3
in 10 000 live births3. Two-thirds of cases are bilateral and most common etiology is genetic mutation, usually autosomal
dominant (AD)4; Others etiology are chromosomal abnormalities, metabolic disorders and intrauterine infections4,5,6.
Etiology of unilateral cases remain unclear but they are usually sporadic. 2,3.Early diagnosis and treatment are of crucial
importance to prevent the development of irreversible stimulus-deprivation amblyopia7,8. The management of pediatric
cataract is a team effort of ophthalmologist ,pediatrician,anaesthetist and parents and should be customized depending upon
the age of onset, laterality, morphology of the cataract, and other associated ocular and systemic co-morbidities.

Work up:

Fig 1.visually significant cataract

History-
Evaluation of a child with a cataract begins with a detailed history including family history; an elaborate antenatal
history including maternal drug use and febrile illnesses with rash; and birth history, especially birth weight.A
developmental history should be carefully assessed,to exclude metabolic or systemic etiologies. A history of onset of
lenticular opacities, laterality, and progression is also important. Unilateral cataracts are usually isolated, but they are most
commonly found to be associated with persistent fetal vasculature (PFV) or other ocular abnormalities, such as
lenticonus/lentiglobus.
Examination

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 Congenital Cataract-Approach And Management Review

Fig 2 Examination of an infant

Examining a neonate is not only different from adult but also very difficult and demanding job .It can be carried
out in the OPD with the child sitting comfortably on its mother lap but many a time examination under anaesthesia is
needed. Recent introduction of high quality portable slit lamp has made the examination of a neonate easier (fig 2) .Since
a formal estimate of visual acuity cannot be obtained in the preverbals, followlng features are noted in order to assess the
visual significance of the opacity.
 Fixation behaviour, fixation preference and objection to occlusion.
 Density,size,site and morphology of cataract- A red reflex test can be performed in a dark room with a direct and
indirect ophthalmoscope to assess the visual significance of the lens opacity. A very dense cataract precluding any
view of the fundus, central cataract larger than 3 mm in diameter, unilateral cataract associated with strabismus and
bilateral cataract with nystagmus are considered visually significant.Visual acuity in verbal cooperative children should
be noted along with glare test in cases of lamellar or posterior subcapsular cataracts. Morphology of the opacity can
give important clues to the underlying aetiology and helps in planning their management.
 Associated ocular pathology involving the anterior segment (corneal clouding, microphthalmos, glaucoma, persistent
fetal vasculature) or the posterior segment (chorioretinitis, Leber amaurosis, rubella retinopathy, foveal or optic nerve
hypoplasia) should be looked for.
 Measurement of intraocular pressures and corneal diameters .

Investigations:
Laboratory workup:
Extensive laboratory investigations are not usually indicated for unilateral cataracts as most of them are isolated,
nonhereditary, and without any systemic associations. In infants with bilateral cataracts,unless there is an established
hereditary basis for the cataracts, the investigations should include the following:

1 Serology for intrauterine infections(TORCH)

2 Urine. Urinalysis for reducing substance after drinking milk (galactosaemia) and
chromatography for amino acids (Lowe syndrome).
3 Other investigations include fasting blood glucose, serum calcium and phosphorus,
red blood cell GPUT and galactokinase levels.Children who have calcium and
phosphorus anomalies severe enough to cause cataracts are unwell.
4 Referral to a pediatrician may be warranted for dysmorphic features or suspicion of
other systemic diseases. Chromosome analysis may be useful in this context.

USG-B scan-
It is mandatory in every case of total cataract for posterior segement evaluation.

Ultrasound biomicroscopy
It can be informative in children with anterior-segment developmental anomalies and PFV and also in the
assessment of posterior capsular support while considering secondary IOL implantation.

Visual evoked potential

For preverbal, less cooperative, or developmentally delayed children, Flash VEPs may be useful in complete
cataracts to establish the gross integrity of the visual pathways.

Examination of the family members

A dilated slit-lamp examination of the parents and any sibling should be performed, which may reveal previously
undiagnosed lenticular changes indicative of an inherited cause for the child’s cataract . Female carriers of X-linked
conditions associated with males presenting with congenital cataracts may show lenticular changes.

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 Congenital Cataract-Approach And Management Review

II. Management
Non surgical management
Visually insignificant lenticular opacity like peripheral lens opacities, punctate opacities with intervening clear
zones, and opacities less than 3 mm in diameter can be observed closely and successfully managed by treating the associated
amblyopia by patching and glasses. In small central opacities, a larger area of clear visual axis can be achieved by
pharmacological dilatation. while conservative treatment can be used, it must be done so with caution, and ancillary tests
such as glare testing must be utilized to ensure that the lens opacity is not visually significant.

Surgical management
Indicated in all visually significant lenticular opacities. A critical period for visual development has been described
in the first 6 weeks of life, during which the vision is subcortically mediated and the infant is relatively resistant to
amblyopia. Timing of surgical intervention is crucial and the main considerations are as follows 7-10.
1.Bilateral dense cataracts require early surgery when the child is 4–6 weeks of age to prevent the development of
stimulus deprivation amblyopia. If the severity is asymmetrical, the eye with the denser cataract should be addressed first.
2. Bilateral partial cataracts may not require surgery until later if at all. In cases of doubt it may be prudent to
defer surgery, monitor lens opacities and visual function and intervene later if vision deteriorates
3. Unilateral dense cataract merits urgent surgery (possibly within days) followed by aggressive anti-amblyopia
therapy, despite which the results are often poor.11 The timing of intervention should be balanced by the suggestion that early
intervention (<4 weeks) may result in an increased risk of subsequent secondary glaucoma. If the cataract is detected after 16
weeks of age then the visual prognosis is particularly poor.

Surgical technique of choice :

Phacoaspiration with primary posterior capsulotomy with or without anterior vitrectomy and in the bag implantation / optic
capture of IOL.12-16

Pecularities of child’s eye

Pediatric eyes are different from adult eyes in following aspects making the surgical management technically demanding

1 .Small size
2 .Thin sclera with decreased rigidity
3 .Increased vitreous pressure
4 .Elastic capsule with unstable anterior chamber.
5 .Changing axial length and corneal curvature
6 .Severe inflammatory response after surgery.
7. longer life span after cataract removal, with a potential for irreversible visual loss due to amblyopia.

Therefore, presurgical evaluation of a growing child’s eye is a little complex.

Biometry and IOL Power calculation14-17

Choosing an appropriate IOL power to prevent the unexpected refractive change in a pediatric case is still a
challenge though there is increasing concensus towards IOL implantation even in infants after the age of 6 months. Poor
patient cooperation and poor fixation most of the time mandates biometry to be performed under sedation or general
anesthesia (Fig 3). Immersion biometry has been shown to be more predictable than the contact method for IOL-power
calculation.

Fig 3 Examination under Anaesthesia

There is a general tendency of partial undercorrection at the time of surgery to balance the postoperative
anticipated myopic shift of the growing eyeball,The choice f IOL power should be individualized based on the child’s need
and refractive status of the other eye in unilateral cases.Different formulas have been used to calculate the desired IOL

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 Congenital Cataract-Approach And Management Review

power in children, with variable results. Various factors in children younger than 2 years like, smaller axial lengths,
shallower anterior chambers, and changes in corneal curvature and corneal thickness may lead to higher prediction errors.
The recent report of IATS ( Infant Aphakia Treatment Study ) recommended Holladay 1 and SRK/T formulae for infant
eyes. However, at 5 years, refractive errors ranging from +5.00 to −19.00D were detected. Iinability to predict axial
elongation in infantile eyes was the primary reason for such a wide range of refractive errors.
Refraction-based formulae by Hug, Khan and Algeed for estimation of secondary posterior-chamber IOL power
provide comparable results to those obtained by standard biometry-based formulas, and can be useful in difficult situations
when standard biometry cannot be performed or when it is not available in the operating room. Many surgeons use an
empiric rule whereby children under the age of 3 months are left +8.00 D, 3 months to 1 year +6.00 D, 1–2 years +4.00 D,
2–3 years +3.00 D, 3–5 years +2.00 D, 5–7 years +1.00 D, and 7 years and after +0.5 D until the age of 11 years. These
numbers may be modified in unilateral cases, depending upon the refraction of the other eye, so as to cause minimal
anisometropia 2 years after surgery.

Type of IOL14,15
Hydrophobic acrylic square edge IOLs are considered best to be used in pediatric cases because of greater
biocompatibility,foldable design,and lower rate of PCO formation and are currently IOL of choice for most pediatric cataract
surgeons.The indications for multifocal IOLs in children are debatable.Refractive shift during eye growth as well as
amblyopia due to loss of contrast sensitivity associated with multifocal IOLs are the main concerns of most pediatric cataract
surgeons.Although limited studies in children have shown improved stereopsis and spectacle independence with the use of
multifocal IOLs, studies with long-term follow-up are warranted.

Surgical Difficulties with Recent Innovations19-23

Cataract surgery in children is challenging, because of decreased sclera rigidity, increased capsular elasticity,
thicker corneas, increased risk of trauma from eye rubbing, less compliance with activity restriction, and most importantly
the effect of postoperative astigmatism on amblyopia

Wound22
Conventionally scleral tunnel incision was preferred by most pediatric cataract surgeons because it was thought to
induce less postoperative astigmatism but recent trend is towards clear corneal approach.Clinically insignificant difference
exists between the two types of incisions, with spontaneous regression of astigmatism over a period of time.Superior
incisions are more preferred as compared to the temporal approach, probably in view of less risk of injury and postoperative
endophthalmitis. To prevent wound leakage due to the reasons mentioned, suturing of all the wounds, including paracentesis
with either a 10-0 or 9-0 Vicryl or nylon suture, is recommended. Absorbable sutures are preferred, in order to avoid a
second visit. Research has shown that the use of 10-0 Vicryl caused astigmatism, but that this astigmatism dissipated after 6
weeks.

Capsulotomy23-27
Continuous, smooth, and well-centered anterior capsulotomy is a prerequisite for safe lens implantation. For
proper centration of the IOL, the anterior capsulotomy should be smaller than the IOL optic
(4-5mm).24 Elasticity and thickness of the anterior capsule in young children makes manual CCC the most difficult
technique, with a steep learning curve .Use of high viscosity ophthalmic visco surgical devices and a soft shell technique
After coating the corneal endothelium with dispersive viscoelastic,the cohesive viscoadaptive agent is filled in the anterior
chamber and Balanced salt solution is used below the viscoadaptive agent, away from the incision, and creating a surgical
operating space with low viscosity. Anterior capsule staining is done using trypan blue by gently “painting” the anterior
capsule.It makes capsulorrhexis, hydrodissection and later,OVD removal,much easier.Currently used techniques for
pediatric anterior capsulotomy includes25-28
 Manual continuous curvilinear capsulorhexis (CCC)-Gold standard technique and produce the most extensible
capsulotomy and the smoothest edge with scanning electron microscopy evaluation in a porcine model. Multiple
anterior capsulorhexisotomies are advocated by some recent studies to prevent anterior-capsule phimosis.
 Vitrectorhexis- Done with vitrector and it is not robust compared to manual CCC but preferred by some surgeons in
very young infants.
 Plasma blade (Fugo blade), diacapsutom, and pulsed-electron avalanche knife, have been suggested to minimize
zonular tension and prevent peripheral CCC extension, but have not become popular.
 Femtosecond laser assisted anterior and posterior capsulotomies

Techniques for cataract extraction

Soft consistency of pediatric cataracts makes phacoemulsification unnecessary. Lens removal can be conducted
through an anterior approach by manual irrigation and aspiration or through the pars plana using a vitrector. In rare cases
with membranous cataracts or retrolenticular membranes in PFV, intraocular scissors are required. An anterior-chamber
maintainer used for continuous irrigation prevents the collapse of the anterior chamber during the surgery and aids in
pupillary dilatation with the use of 1:1,000 epinephrine added to the balanced salt solution. 28-30 The use of heparin in the
balanced salt solution and intracameral recombinant tissue-plasminogen activator has been shown to reduce the fibrinous
reaction and pigment deposits on the IOL but their routine use is not yet established. Recently, the use of a transconjunctival
25-gauge vitrectomy system has been gaining popularity for lens removal in pediatric patients.

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 Congenital Cataract-Approach And Management Review

Posterior-capsule management
Maintenance of a clear visual axis is critical for a good postoperative visual outcome after pediatric cataract
extraction. If the posterior capsule is left intact, 100% of eyes less than 4 years of age develop significant PCO. The anterior
vitreous face acts a scaffold for the proliferation of lens epithelial cells (LECs).Intraoperative measures taken to minimise it
are:
1.Primary posterior capsulotomy (PPC) with anterior vitrectomy 30,31in infants and young children or in older
children who are poor candidates for possible YAG laser capsulotomy.This step can be facilitated by using trypan blue and
triamcinolone acetonide for staining posterior capsule and vitreous respectively. The opening for PPC should be smaller than
the anterior capsulotomy.Two-incision push–pull technique has been shown to give consistent results without vitreous loss
during the procedurPrimary manual anterior and posterior continuouscurvilinear capsulorhexis (ACCC, PCCC) showing a
smooth and regular edge32-34
2.Optic capture of the IOL was believed to obviate the need for anterior vitrectomy and prevent the development
of PCO34. However,recent studies have proven that anterior vitrectomy is necessary with optic capture even in older
children.Moreover,it is a difficult technique to perform with single-piece IOLs without haptic angulation. Posterior vertical
capsulectomy with optic entrapment has promising results in children aged 2 months to 8 years, with clear visual axes for 5–
12 years postoperatively. Although this procedure is technically challenging, anterior vitrectomy is seldom required.
3.Subconjunctival or intracameral steroids are recommended to suppress inflammation in the immediate
postoperative period. Intracameral recombinant tissue-plasminogen activator has also been shown to reduce the fibrinous
reaction and pigment deposits on the IOL after pediatric cataract surgery, though there is a risk of hyphema.

Complications: 35,36
Cataract surgery in children carries a higher incidence of complications than in adults
1.Increased inflammatory response in children can lead to fibrinous reactions, pigment deposits on the IOL,
decentration of the IOL, and posterior synechiae. Intensive steroid therapy post operatively is utilised to combat this
problem.
2.Secondary glaucoma is the most feared complication of pediatric cataract surgery, and is commonly seen in infants.
 Closed-angle glaucoma may occur in the immediate postoperative period in microphthalmic eyes secondary to pupillary
block.
 Secondary open-angle glaucoma may develop years after the initial surgery; it is therefore important to monitor the
intraocular pressure long-term

Measurement of central corneal thickness in aphakic as well as pseudophakic eyes is essential to diagnose true
glaucoma and monitoring for glaucoma is mandatory for any child undergoing cataract surgery early in life.
3.Visual axis opacification remains the most common complication after pediatric cataract surgery despite doing
PPC, anterior vitrectomy, use of in the bag hydrophobic acrylic IOL. Severe capsular phimosis can also occlude the visual
axis. Nd:YAG laser capsulotomy or surgical membranectomy can be performed to clear the visual axis. Sealed-capsule
irrigation using either distilled water or 5-fluorouracil has been shown to be successful in reducing the incidence of visual
axis opacification in adult eyes by killing the LEC but have potentiai risk of endothelial loss.
4.Retinal detachment is an uncommon and usually late complication

Vision rehabilitation
With regard to optical correction for the aphakic child, the two main considerations are age and laterality of
aphakia.The modalities for visual rehabilitation after pediatric cataract extraction include the following:

1 Spectacles are useful for older children with bilateral aphakia.

2 Contact lenses provide a superior optical solution for both unilateral and bilateral aphakia. Tolerance is usually
reasonable until the age of about 2 years, although after this period problems with compliance may develop as
the child becomes more active and independent.
3 IOL implantation is increasingly being performed in younger children and appears to be effective and safe in
selected cases. Awareness of the rate of myopic shift which occurs in the developing eye, combined with
accurate biometry, allows the calculation of an IOL power targeted at initial hypermetropia (correctable with
spectacles) which will ideally decay towards emmetropia later in life. However, final refraction is variable and
emmetropia in adulthood cannot be guaranteed.
4 Occlusion to treat or prevent amblyopia is essential. Atropine penalization may also be considered.Compliance
with patching has been shown to be associated with good postoperative visual outcomes in unilateral and
bilateral cataracts. Educating the parents about the importance of amblyopia therapy postsurgery is crucial.

Recommendations and Future directions

Childhood cataract remains a challenge to pediatric ophthalmologists despite recent major breakthrough in surgical
techniques and instrumentation.We need to tackle this problem more seriously and sensitively.
1. Immunization programms for rubella and measles and neonatal screening should be more intensified in developing
countries as prevention of disease is always better than its cure.
2. Future research in developing formulas for accurate biometry predicting refractive surprises and techniques for
preventing PCO is warranted. Capsule-irrigating devices customized for pediatric eyes ,dexamethasone-coated
IOLs.are showing promising results but needs to be studied well.

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 Congenital Cataract-Approach And Management Review

3 Evaluation by a geneticist can be helpful in determining the inheritance pattern and to identify associated syndromes.
Understanding of molecular genetics have been a major breakthrough achieved in the field of congenital cataracts in
these recent years and these informations need to to be applied practically by genetic counsellors during marriages.
Source(s) of support-nil……Conflict of Interest:None

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