PEDIATRIC CATARACT...PEARLS AND PITFALLS

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PEDIATRIC CATARACT...PEARLS AND PITFALLS により Mind Map: PEDIATRIC CATARACT...PEARLS AND PITFALLS

1. What is congenital cataract?

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

2. What is developmental cataract?

2.1. Developmental cataract: if present after infancy

3. What is the prevalence of pediatric cataract?

3.1. Developing countries -1-4/10000

3.2. Developed countries - 0.1-0.4/10000

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

4.1. History Taking

4.1.1. Family history of cataract

4.1.2. H/O Parental consanguinity

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

4.1.4. Birth history-birth weight,NICU admission

4.1.5. Developmental milestones/Nutritional status/Immunisation status

4.1.6. H/O of onset of lenticular opacity:

4.1.7. H/O Trauma

4.1.8. Laterality

4.1.9. Progression

4.2. Detailed Ocular examination

4.2.1. Torch light examination

4.2.2. Slit lamp biomicroscopy

4.2.3. Size

4.2.4. Location

4.2.5. Density

4.2.6. Morphology

4.2.7. Measurement of HCD and VCD

4.2.8. Anterior segment evaluation and Gonioscopy

4.2.9. IOP measurement

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

4.3. Specific investigations

4.3.1. Routine Tests

4.3.2. Fasting Blood Sugar

4.3.3. Plasma Ca & P

4.3.4. Urine Reducing substances

4.3.5. Urine Amino Acids

4.3.6. TORCH titres

4.4. Preoperative evaluation

4.4.1. Unilateral cataract

4.4.1.1. Complete ocular examination

4.4.1.2. Complete ocular examination

4.4.1.3. Look for PHPV, posterior lenticonus, ,Anterior segment dysgenesis

4.4.2. Bilateral cataract

4.4.2.1. Family history- Inheritence pattern

4.4.2.2. Hepatomegally, failure to thrive, diarrhoea

4.4.2.3. Look for syndromes-60%

4.5. IOL Power calculation

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

4.7. Assessment of visual acuity

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

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

4.7.3. Older children – age appropriate methods

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

5. How to approach a case of pediatric cataract?

5.1. What do we need to ask?

5.1.1. Antenatal

5.1.1.1. Fever

5.1.1.2. Rashes

5.1.1.3. Vaccination

5.1.1.4. Drug intake

5.1.2. Perinatal

5.1.2.1. Prematuriy

5.1.2.2. Birth weight

5.1.2.3. NICU admission

5.1.3. Postnatal

5.1.3.1. Onset

5.1.3.2. Developmental milestones

5.1.3.3. Pedigree

5.2. What do we Look for?

5.2.1. General examination

5.2.1.1. Built

5.2.1.2. Gait

5.2.1.3. Nails

5.2.1.4. Skin

5.2.1.5. Hairs

5.2.2. Systemic examination

5.2.2.1. Craniofacial

5.2.2.2. Oral cavity

5.2.2.3. Ear

5.2.2.4. Bony abnormality

5.2.3. Ocular examination

5.2.3.1. Cataract morphology

5.2.3.2. Microcornea

5.2.3.3. Aniridia

5.2.3.4. Fundus

5.2.3.5. IOP

5.3. What do we Order?

5.3.1. Pediatrician consultation

5.3.1.1. Syndromic features

5.3.1.2. Developmental delay

5.3.1.3. ECG

5.3.1.4. BERA

5.3.2. Lab tests

5.3.2.1. Reducing sugars

5.3.2.2. IgG/IgM titres

5.3.3. Genetic analysis

5.3.3.1. Parental consanguinity

5.3.3.2. Family history

5.3.3.3. Inheritence pattern and pedigree

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

6.1. Topical medications

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

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

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

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

6.3. Other eye surgery- after 1-2 weeks

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

6.4.1. Suture removal

6.4.2. IOP,CCT measurement

6.4.3. Fundus evaluation

6.4.4. Refraction

6.4.5. Amblyopia therapy ,if needed

6.4.6. Glass prescription-mandatory

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

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

6.5.1. 1 Month post-EUA

6.5.2. 3 Monthly follow up till 1 year – after that every 6 monthly

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

6.5.4. Low vision therapy – if needed

6.6. What are the indications of secondary IOL implantation ?

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

6.6.2. Minimum HCD- 10.75 mm , with adequate bag

6.6.3. Adequate anterior capsular support for 3 piece IOL

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

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

6.7. What are the contraindications of secondary IOL implantation?

6.7.1. Secondary glaucoma

6.7.2. Anterior segment dysgenesis

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

7.1. 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

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

8.1. Superior incisions are commonly preferred

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

8.1.2. Less post-operative astigmatism

8.2. What are the indications of temporal incision?

8.2.1. Deep-seated eyes

8.2.2. In children with previous or planned filtration surgery superiorly

9. How to classify pediatric cataracts ?

9.1. Morphological classification

9.1.1. Anterior

9.1.1.1. Polar

9.1.1.1.1. Aniridia

9.1.1.2. Pyramidal

9.1.1.2.1. Severe form of anterior capsulo-lenticular opacities in the form of a pyramid protruding into the anterior chamber

9.1.1.2.2. Retinoblastoma

9.1.1.2.3. Ehlers–Danlos syndrome

9.1.1.3. Subcapsular

9.1.1.3.1. uveitis

9.1.1.3.2. Trauma.

9.1.1.3.3. Irradiation

9.1.1.3.4. Atopic dermatitis

9.1.1.4. Anterior lenticonus

9.1.1.4.1. Bilateral condition

9.1.1.4.2. Waardenburg syndrome

9.1.1.4.3. Alport syndrome

9.1.2. Posterior

9.1.2.1. Posterior lenticonus

9.1.2.2. PHPV

9.1.2.3. Posterior subcapsular

9.1.2.3.1. Drug-induced

9.1.2.3.2. Radiation induced

9.1.2.3.3. Turner’s syndrome

9.1.2.3.4. Fabry’s disease

9.1.2.3.5. Bardet–Biedl syndrome

9.1.2.3.6. Neurofibromatosis type 2

9.1.2.4. Mittendorf’s dot

9.1.2.5. Oil- Droplet cataract

9.1.2.6. Cerulean /Blue -Dot cataract

9.1.2.7. total cataract

9.1.2.7.1. Down’s syndrome and

9.1.2.7.2. congenital rubella syndrome

9.1.2.7.3. Early surgical intervention is mandatory to prevent the development of amblyopia.

9.1.2.8. Membranous

9.1.2.8.1. Disk-like opacities formed after spontaneous resorption of lens material

9.1.2.8.2. Hallermann –Streiff syndrome

9.1.2.8.3. congenital rubella

9.1.2.8.4. Lowe syndrome

9.1.2.8.5. PFV

9.1.2.9. Diffuse

9.1.2.10. Wedge-shape cataracts

9.1.2.10.1. partial lenticular opacities

9.1.2.10.2. Stickler syndrome

9.1.2.10.3. Conradi–Hunermann syndrome

9.1.2.10.4. Fabry’s disease

9.1.2.10.5. Neurofibromatosis type 2

9.1.3. Central

9.1.3.1. Nuclear

9.1.3.2. Sutural

9.1.3.2.1. visually insignificant opacities along the Y-sutures of the lens

9.1.3.2.2. Seen in female carriers of Nance–Horan syndrome

9.2. Etiological classification

9.2.1. Unilateral

9.2.1.1. Idiopathic

9.2.1.2. Traumatic

9.2.1.3. Other ocular abnormalities

9.2.2. Bilateral

9.2.2.1. Idiopathic

9.2.2.2. Systemic diseases

9.2.2.2.1. What are the systemic infections associated with pediatric cataract?

9.2.2.3. Hereditary

9.2.2.4. Metabolic

9.2.2.4.1. What are the metabolic disorders causing pediatric cataract?

9.2.2.5. Maternal infections

9.2.2.5.1. TORCH infections

9.2.2.6. Ocular abnormalities

9.2.2.7. Syndromic associations

9.2.2.7.1. Down syndrome

9.2.2.7.2. Congenital rubella syndrome

9.2.2.7.3. Pierre-robin syndrome

9.2.2.7.4. Hallermann- streiff syndrome

9.2.2.7.5. Lowe syndrome

9.2.2.7.6. Conradi- Hunermann syndrome

9.2.2.7.7. Alport syndrome

9.2.2.7.8. Stickler syndrome

9.2.2.7.9. Fetal-alcohol syndrome

9.2.2.7.10. Nance-Horan syndrome

9.2.2.7.11. Smith-lemli-opitz syndrome

9.2.2.7.12. Zellweger syndrome

9.2.2.7.13. Craniofacial syndromes

10. How is pediatric cataract different from adult cataract?

10.1. Pre-operatively

10.1.1. Difficult and delayed diagnosis

10.1.2. Timing of surgery

10.1.3. Higher syndromic and systemic as well as ocular associations

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

10.1.5. Difficulty and differences in IOL Power calculation

10.1.6. Short AL in children

10.1.7. High keratometry values

10.1.8. Shallow AC

10.1.9. Need for proper counselling of parents/ caregivers

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

10.2. Intraoperatively

10.2.1. Risks of G.A

10.2.2. Smaller size of eye

10.2.3. Poor mydriasis

10.2.4. Low scleral rigidity

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

10.2.6. Need for doing a controlled ACC

10.2.7. Need for PPC+AV

10.2.8. Need for high viscosity OVDs for capsular management

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

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

10.2.11. Difficult IOL implantation

10.3. Postoperatively

10.3.1. High chances of PCO

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

10.3.3. Need for cycloplegics- a must

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

10.3.5. Need for Visual rehabilitation

10.3.6. Reduced compliance with post-operative medications

10.3.7. Frequent change in refraction

10.3.8. Need for repeated EUAs

10.3.9. Amblyopia therapy

10.3.10. Need for close Follow up

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

11.1. I.Myopic shift

11.2. II.Target refraction

11.3. III.AL/K measurement

11.4. IV.IOL power formula

11.5. Problems in using present formulas

11.5.1. Considerations for adult eye

11.5.2. Short AL in children

11.5.3. High keratometry values

11.5.4. Shallow AC

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

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

12.1.1. 3 ways to under-correct as per age

12.1.1.1. Dahan et al

12.1.1.1.1. 20% under correction < 2 years

12.1.1.1.2. 10% under correction 2–8 years

12.1.1.2. Enyedi et al (Target refraction): Target post operative refraction- Age +Target Hyperopia = 7

12.1.1.2.1. 1-2 yrs: +6 to +5

12.1.1.2.2. 3-4yrs: +4 to +3

12.1.1.2.3. 5-6yrs: +2 to +1

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

12.1.3. Higher the power, more under correction needed

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

13.1. Measurement of HCD and VCD

13.2. Assessment of morphology of cataract

13.3. Axial length

13.4. Keratometry

13.5. ROPLAS –to r/o CNLDO

13.6. Fundus examination

13.7. CCT

13.8. IOP

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

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

14.1. AcrySof IOLs are considered better than PMMA IOLs

14.1.1. Greater biocompatibility and smaller incision size

14.1.2. Foldable design

14.1.3. Lower rate and late onset of PCO formation

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

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

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

14.3. Why are Silicone IOLs not preferred?

14.3.1. Due to increased rate of capsule contraction

14.4. Are iris fixated lenses used in children?

14.4.1. Not preferably used

14.4.1.1. As they cause major complications

14.4.1.1.1. Retinal detachment

14.4.1.1.2. Hyphema, synechiae, ectopic pupil,

14.4.1.1.3. Fibrinous uveitis, and vitreous strands in the wound

14.5. Are multifocal IOLs to be used in children?

14.5.1. 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.

14.5.1.1. What are the major concerns with multifocal IOLs in children?

14.5.1.1.1. 1.Refractive shift during eye growth 2.Amblyopia due to loss of contrast sensitivity associated with multifocal IOLs are the main concerns

14.5.1.2. What are the advantages of using multifocal IOLs in children?

14.5.1.2.1. Improved stereopsis

14.5.1.2.2. Spectacle independence

15. What is VAO ?

15.1. Visual axis opacification

15.1.1. What are the measures to be taken to prevent VAO?

15.1.1.1. Doing a PPC+AV

15.1.1.2. use of hydrophobic acrylic IOL,

15.1.1.3. In the bag IOL implantation

15.1.2. How to manage if significant VAO occurs

15.1.2.1. Nd:YAG laser capsulotomy

15.1.2.2. 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