Congenital et
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This document discusses congenital/essential infantile esotropia, which is a type of eye turning inward (esotropia) present from birth. Some key points: - It affects around 0.1% of the population and is characterized by a large angle esotropia (>30 prism diopters), constant crossing of the eyes, and asymmetry in optokinetic nystagmus. - Early surgery before 18 months of age results in better binocular vision outcomes compared to later surgery. Surgery involves recession of the medial rectus muscles, with multiple muscles recessed for larger angle deviations. - Early alignment through surgery improves stereoacuity outcomes by making patients less likely to need
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Congenital et
- 1. CONGENITAL / ESSENTIAL INFANTILE ESOTROPIA V.Chandrasekhar Reddy Hyderabad.India drvcreddy@gmail.com
- 2. Esotropia(Convergent Squint)..Cornea deviated nasally 4-5% of squint in gen population. 0.1% of population have CET 50% ocular deviations in paed age group are Esotropias 40% cong ET of all ET
- 3. Pseudo strabismusPseudo strabismus Epicanthal fold Wide nasal bridge Ectopia of macula Narrow IPD
- 4. Heredity: multifactorial genetic basis for congenital esotropia. suggested loci on regions 3p26.3-26.2 and 6q24.2-25.1 and may share alleles that underlie Duane retraction syndrome Unaffected parents are more likely to have slightly deficient stereopsis, but do not have OKN asymmetry.
- 5. pathogenesispathogenesis Worths sensory concept. Inborn and irreversible defect of fusion. Chavasse mechanical concept. Neural components necessary for normal binocular vision are present in strabismic individuals at birth, but the development of fusion is eventually impeded by abnormalities of optical input (eg, monocular cataracts) or muscular output (eg, cranial nerve palsies)
- 6. Certain risk factors . prematurity, family history, perinatal or gestational complications, systemic disorders, use of supplemental oxygen as a neonate, use of systemic medications. Awareness of these risk factors can lead to early detection and management of esotropia
- 7. CETCET Alignment is achieved by 4mths in the normal infant and stereopsis can be measured in the laboratory. Early misalignment constant or intermittent beyond 2-4 months is required to be sure of the diagnosis of congenital ET. OKN asymmetry present in all infants becomes symmetrical by 6 months in the normal. Patients with congenital ET retain OKN asymmetry
- 8. What to be seen in a patient with congenital esotropia? (Diagnostic criteria) * Neurologically normal 70% (except for ET) * Hyperopia less than +3.50 (A greater hyperopia does not rule out congenital ET) * Esotropia (30-70PD ± nystagmus)
- 9. A special characteristic of congenital esotropia - OKN asymmetry Temporal to Nasal (T/N).Smooth following, rapid and accurate Refixation Nasal to Temporal (N/T).Jerky, inaccurate movement with halting refixation
- 10. Congenital ETCongenital ET ET by 6 mths > 30 D ET & Stable Assymetry of OKN Cross fixation Abd restriction No clinical CNS involement Deviation same for D & N Consistant features
- 11. Associated featuresAssociated features IO overaction 60%, nystagmus,DVD 50% > 1yr Amblypoia (35%) AHP It can be associated with a systemic disease such as Down’s syndrome, albinism, cerebral palsy, or hydrocephaly.1-4
- 13. IO OA DVDIO OA DVD
- 14. Differential DiagnosisDifferential Diagnosis Congenital VIth Nervepalsy Duane Syndrome Type I Mobius Syndrome Nystagmus (blockage ) Syndrome Down Sydrome Albinism Cerebral Palsy etc.. Maybe associated with CNS and other systemic abnormalities.
- 15. CongenitalCongenital VIVIthth nerve palsynerve palsy Esotropia.
- 16. Management goalManagement goal To make the eyes as close to orthotropia with normal vision,develop some fusion to keep alignment May require multiple surgeries Alignment with 8-10d results in monofixation syndrome …comfortable surgical result
- 17. ManagementManagement Ruleout accomadation element with atropine. Treat refractive error Botax to keep fusion potential live Treat amblyopia surgery
- 18. Non surgical interventionNon surgical intervention Botulinum toxin To postpone surgery Not as effective as surgery Spects Acc ET Prisms Temp measure Small angle
- 19. SurgerySurgery 40d ET at 4 mths will not resolve spontaneously Must be as done early as possible to get binocular function. Large deviation…..correction is more ET + amb…more correction Fusion potential must be undercorrected
- 20. timing of surgerytiming of surgery Early surgery decreases the severity of DVD and lowers the need for additional operation for DVD oblique overaction. Yagasaki et al, Zak and Morin Early vs. Late Infantile Strabismus Surgery Study (ELISS), children operated at age 6-24 months had better gross stereopsis at age 6 years compared with those operated on later
- 21. Surgical alignment before 18 mos. better binocularity will even better binocularity be achieved with alignment before 12 mo. The Pediatric Eye Disease Investigator Group (PEDIG) in the Congenital Esotropia Observation Study (CEOS) determined that ET of 40 diopters or more present at 2 months persists until 7 months - diagnosis of congenital ET can be made ET of less than 40 prism diopters or intermittent ET at 2 mos. has a 50% chance of persisting at 7 mos.
- 22. Surgical options Recession of both MR Symmetrical or asymmetrical No of muscles depends upon amount of deviation Weakening of IO rece.wmv
- 23. Results to be expected fromResults to be expected from surgery for CETsurgery for CET Single muscle..Sparingly MR Recession Small angle (10-15pd),fusion potential,diplopia DRS Possible OVER CORRECTION
- 24. RESULT TO BE EXPECTED FROM SURGERY FOR CET Cong ET 50pd Adult ET 40pd Equal vision ET .>N more AC/A +Oblique muscle for AV,&IOOA BMR Recession Minimal 2.5mm MR for 15- 20pd Maxim.7mm for 40pd BLR Resection Div insufficiency. 20pd .5mm Res ET 40pd 9mm R&R CET ..poor vision one eye 2.5MR-5LR..20-25pd 5MR+9LR <1yr….50pd 7MR+10LR..>3yrs 50pd+ Two muscle surgery Measurement from insertion
- 25. 3 muscle surgery….50-70pd 5mmBMR+9mm LR…<1yr 7mm BMR+10mmLR..>3yr
- 26. 4 muscle surgery >75 pd. Not a routine 7mm BMR +10mm LR
- 27. Why does early alignmentWhy does early alignment improve stereoacuityimprove stereoacuity outcomes in CET?outcomes in CET? patients with stereo less likely to need a 2nd surgery [p=0.05] and less likely to have DVD (P <.001). CONGENITAL ESOTROPIA 27
- 29. 60 pd.Restriction of abd LE BMR Recession 6mm +LLR Resection 8mm
- 30. Management of CETManagement of CET Confirm diagnosis Fixation fusion Amblyopia Accomadation,Refraction Associated features Surgery as early as possible depending on anesthetist readiness
- 31. Thank u
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