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1. How to classify pediatric cataracts ?

1.1. Morphological classification

1.1.1. Anterior Polar Aniridia Pyramidal Severe form of anterior capsulo-lenticular opacities in the form of a pyramid protruding into the anterior chamber Retinoblastoma Ehlers–Danlos syndrome Subcapsular uveitis Trauma. Irradiation Atopic dermatitis Anterior lenticonus Bilateral condition Waardenburg syndrome Alport syndrome

1.1.2. Posterior Posterior lenticonus PHPV Posterior subcapsular Drug-induced Radiation induced Turner’s syndrome Fabry’s disease Bardet–Biedl syndrome Neurofibromatosis type 2 Mittendorf’s dot Oil- Droplet cataract Cerulean /Blue -Dot cataract total cataract Down’s syndrome and congenital rubella syndrome Early surgical intervention is mandatory to prevent the development of amblyopia. Membranous Disk-like opacities formed after spontaneous resorption of lens material Hallermann –Streiff syndrome congenital rubella Lowe syndrome PFV Diffuse Wedge-shape cataracts partial lenticular opacities Stickler syndrome Conradi–Hunermann syndrome Fabry’s disease Neurofibromatosis type 2

1.1.3. Central Nuclear Sutural visually insignificant opacities along the Y-sutures of the lens Seen in female carriers of Nance–Horan syndrome

1.2. Etiological classification

1.2.1. Unilateral Idiopathic Traumatic Other ocular abnormalities

1.2.2. Bilateral Idiopathic Systemic diseases What are the systemic infections associated with pediatric cataract? Hereditary Metabolic What are the metabolic disorders causing pediatric cataract? Maternal infections TORCH infections Ocular abnormalities Syndromic associations Down syndrome Congenital rubella syndrome Pierre-robin syndrome Hallermann- streiff syndrome Lowe syndrome Conradi- Hunermann syndrome Alport syndrome Stickler syndrome Fetal-alcohol syndrome Nance-Horan syndrome Smith-lemli-opitz syndrome Zellweger syndrome Craniofacial syndromes

2. How is pediatric cataract different from adult cataract?

2.1. Pre-operatively

2.1.1. Difficult and delayed diagnosis

2.1.2. Timing of surgery

2.1.3. Higher syndromic and systemic as well as ocular associations

2.1.4. Need for G.A Set up and need for EUA

2.1.5. Difficulty and differences in IOL Power calculation

2.1.6. Short AL in children

2.1.7. High keratometry values

2.1.8. Shallow AC

2.1.9. Need for proper counselling of parents/ caregivers

2.1.10. Increased chances of myopic shift – need for planned under-correction of IOL Power pre-operatively

2.2. Intraoperatively

2.2.1. Risks of G.A

2.2.2. Smaller size of eye

2.2.3. Poor mydriasis

2.2.4. Low scleral rigidity

2.2.5. Difficult ACC –Highly elastic anterior capsule ,increased intra-lenticular and intravitreal pressure

2.2.6. Need for doing a controlled ACC

2.2.7. Need for PPC+AV

2.2.8. Need for high viscosity OVDs for capsular management

2.2.9. Thicker posterior capsular plaques- may need intraocular scissors along with anterior vitrector

2.2.10. Wound suturing – a must till 8-9 years age

2.2.11. Difficult IOL implantation

2.3. Postoperatively

2.3.1. High chances of PCO

2.3.2. Need for intensive steroid therapy post-operatively – due to post operative inflamation

2.3.3. Need for cycloplegics- a must

2.3.4. EUA and Suture removal- 1 week post-operatively

2.3.5. Need for Visual rehabilitation

2.3.6. Reduced compliance with post-operative medications

2.3.7. Frequent change in refraction

2.3.8. Need for repeated EUAs

2.3.9. Amblyopia therapy

2.3.10. Need for close Follow up

3. What is congenital cataract?

3.1. Congenital cataract: if present within the first year of life

4. What is developmental cataract?

4.1. Developmental cataract: if present after infancy

5. What is the prevalence of pediatric cataract?

5.1. Developing countries -1-4/10000

5.2. Developed countries - 0.1-0.4/10000

6. How to do the Pre Operative evaluation of pediatric cataract?

6.1. History Taking

6.1.1. Family history of cataract

6.1.2. H/O Parental consanguinity

6.1.3. Prenatal history-Maternal drug use,Vaccination,febrile illnesses,rash

6.1.4. Birth history-birth weight,NICU admission

6.1.5. Developmental milestones/Nutritional status/Immunisation status

6.1.6. H/O of onset of lenticular opacity:

6.1.7. H/O Trauma

6.1.8. Laterality

6.1.9. Progression

6.2. Detailed Ocular examination

6.2.1. Torch light examination

6.2.2. Slit lamp biomicroscopy

6.2.3. Size

6.2.4. Location

6.2.5. Density

6.2.6. Morphology

6.2.7. Measurement of HCD and VCD

6.2.8. Anterior segment evaluation and Gonioscopy

6.2.9. IOP measurement

6.2.10. Fundus examination and B-Scan (if needed)

6.3. Specific investigations

6.3.1. Routine Tests

6.3.2. Fasting Blood Sugar

6.3.3. Plasma Ca & P

6.3.4. Urine Reducing substances

6.3.5. Urine Amino Acids

6.3.6. TORCH titres

6.4. Preoperative evaluation

6.4.1. Unilateral cataract Complete ocular examination Complete ocular examination Look for PHPV, posterior lenticonus, ,Anterior segment dysgenesis

6.4.2. Bilateral cataract Family history- Inheritence pattern Hepatomegally, failure to thrive, diarrhoea Look for syndromes-60%

6.5. IOL Power calculation

6.6. General examination: to r/o syndromic/systemic associations

6.7. Assessment of visual acuity

6.7.1. Very young infant – Red reflex with direct ophthalmoscope/ undilated retinoscopy

6.7.2. Preverbal children – Fixation behaviour,preference and objection to occlusion

6.7.3. Older children – age appropriate methods

6.8. A-Scan , B-scan (in case of total cataract/hazy media), Slit-lamp photography

7. What are the challenges encountered during IOL Power calculation in children?

7.1. I.Myopic shift

7.2. II.Target refraction

7.3. III.AL/K measurement

7.4. IV.IOL power formula

7.5. Problems in using present formulas

7.5.1. Considerations for adult eye

7.5.2. Short AL in children

7.5.3. High keratometry values

7.5.4. Shallow AC

8. What is myopic shift and how to tackle it?

8.1. Myopic shift can be tackled by doing under-correction from the emmetropic IOL Power

8.1.1. 3 ways to under-correct as per age Dahan et al 20% under correction < 2 years 10% under correction 2–8 years Enyedi et al (Target refraction): Target post operative refraction- Age +Target Hyperopia = 7 1-2 yrs: +6 to +5 3-4yrs: +4 to +3 5-6yrs: +2 to +1

8.1.2. Refractive status of other eye: Anisometropia not more than 3.0D

8.1.3. Higher the power, more under correction needed

9. How to approach a case of pediatric cataract?

9.1. What do we need to ask?

9.1.1. Antenatal Fever Rashes Vaccination Drug intake

9.1.2. Perinatal Prematuriy Birth weight NICU admission

9.1.3. Postnatal Onset Developmental milestones Pedigree

9.2. What do we Look for?

9.2.1. General examination Built Gait Nails Skin Hairs

9.2.2. Systemic examination Craniofacial Oral cavity Ear Bony abnormality

9.2.3. Ocular examination Cataract morphology Microcornea Aniridia Fundus IOP

9.3. What do we Order?

9.3.1. Pediatrician consultation Syndromic features Developmental delay ECG BERA

9.3.2. Lab tests Reducing sugars IgG/IgM titres

9.3.3. Genetic analysis Parental consanguinity Family history Inheritence pattern and pedigree

10. What are the on-table procedures needed to be done in a case of pediatric cataract?

10.1. Measurement of HCD and VCD

10.2. Assessment of morphology of cataract

10.3. Axial length

10.4. Keratometry

10.5. ROPLAS –to r/o CNLDO

10.6. Fundus examination

10.7. CCT

10.8. IOP

10.9. Gonioscopy (in case of associated suspected congenital glaucoma/developmental glaucoma)

11. How to manage a case of pediatric cataract surgery post-operatively?

11.1. Topical medications

11.1.1. Topical Steroids – 10/8/6/5/4/3/2/1 times a day for 1 week each for 8 weeks

11.1.2. Antibiotic eye drops- 6 times a day for 1 week

11.1.3. Atropine/Homide eye drops-1 drop 3 times a day for 2 weeks

11.2. EUA -1 Week To 10 Days( B/L Cataract)

11.3. Other eye surgery- after 1-2 weeks

11.4. What are the parameters to be checked in EUA?

11.4.1. Suture removal

11.4.2. IOP,CCT measurement

11.4.3. Fundus evaluation

11.4.4. Refraction

11.4.5. Amblyopia therapy ,if needed

11.4.6. Glass prescription-mandatory

11.4.7. Referral to child-rehab clinic – vision stimulation exercises

11.5. What is the follow up schedule to be followed in a case of pediatric cataract?

11.5.1. 1 Month post-EUA

11.5.2. 3 Monthly follow up till 1 year – after that every 6 monthly

11.5.3. Repeat EUA at 6 monthly interval for children<3 yrs,aphakic(where HCD has to be measured)

11.5.4. Low vision therapy – if needed

11.6. What are the indications of secondary IOL implantation ?

11.6.1. Follow up the child with surgical aphakia every 3months on OPD basis/with EUA(if child is uncoperative)

11.6.2. Minimum HCD- 10.75 mm , with adequate bag

11.6.3. Adequate anterior capsular support for 3 piece IOL

11.6.4. Glued IOL- in case of lack of PC support

11.6.5. Secondary IOL- B/L – 5 years age

11.7. What are the contraindications of secondary IOL implantation?

11.7.1. Secondary glaucoma

11.7.2. Anterior segment dysgenesis

12. What is the ideal time to operate in a case of pediatric cataract?

12.1. Extraction of unilateral congenital cataracts by 4–6 weeks and bilateral congenital cataracts within the first 6–8 weeks of life can prevent the development of stimulus deprivation amblyopia, strabismus, and nystagmus

13. Which is the best type of IOL to be used in children and why?

13.1. AcrySof IOLs are considered better than PMMA IOLs

13.1.1. Greater biocompatibility and smaller incision size

13.1.2. Foldable design

13.1.3. Lower rate and late onset of PCO formation

13.1.4. Single piece IOLs for in the bag implantation and three piece IOLs for sulcus fixation

13.2. Which IOL is preferred in complicated cataract due to uveitis?

13.2.1. use of heparin-surface-coated PMMA IOLs : decreases postoperative inflamation

13.3. Why are Silicone IOLs not preferred?

13.3.1. Due to increased rate of capsule contraction

13.4. Are iris fixated lenses used in children?

13.4.1. Not preferably used As they cause major complications Retinal detachment Hyphema, synechiae, ectopic pupil, Fibrinous uveitis, and vitreous strands in the wound

13.5. Are multifocal IOLs to be used in children?

13.5.1. The indications for multifocal IOLs in children are debatable. Simultaneous distance and near vision without the aid of glasses or contact lenses can be achieved with the use of multifocal IOLs. What are the major concerns with multifocal IOLs in children? 1.Refractive shift during eye growth 2.Amblyopia due to loss of contrast sensitivity associated with multifocal IOLs are the main concerns What are the advantages of using multifocal IOLs in children? Improved stereopsis Spectacle independence

14. Which incision is preferred for pediatric cataract surgery-superior or temporal and why?

14.1. Superior incisions are commonly preferred

14.1.1. Less risk of injury and less chances of postoperative endophthalmitis

14.1.2. Less post-operative astigmatism

14.2. What are the indications of temporal incision?

14.2.1. Deep-seated eyes

14.2.2. In children with previous or planned filtration surgery superiorly

15. What is VAO ?

15.1. Visual axis opacification

15.1.1. What are the measures to be taken to prevent VAO? Doing a PPC+AV use of hydrophobic acrylic IOL, In the bag IOL implantation

15.1.2. How to manage if significant VAO occurs Nd:YAG laser capsulotomy Surgical membranectomy

16. What are the modalities of visual rehabilitation after pediatric cataract surgery?

16.1. IOL implantation

16.1.1. Best method of visual rehabilitation whenever possible.

16.2. contact lenses.

16.2.1. Contact lenses are usually well tolerated, and the power can be changed until the child is ready for IOL implantation with predictable postoperative refraction.

16.2.2. Rigid gas-permeable contact lenses are preferred by the majority of clinicians.

16.2.3. Good visual acuity can be achieved with contact lens fitting within 3 weeks of surgery in unilateral aphakes.

16.3. Aphakic glasses

16.3.1. Aphakic glasses are an efficient method for visual rehabilitation in children up to 4 years of age. The addition of a bifocal segment is needed in children 4 years of age or older.

16.4. Amblyopia therapy