Congenital cataract


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  • Bilateral cataracts: Idiopathic (60%), hereditary (30%; usually autosomal dominant, associated with or without systemic abnormalities), intrauterine infection (TORCHS syndromes (toxoplasmosis, rubella, cytomegalovirus, herpes, syphilis), mumps, vaccinia), associated with ocular disorders ( Leber’s congenital amaurosis, retinitis pigmentosa (RP), persistent hyperplastic primary vitreous (PHPV), retinitis of prematurity, aniridia, Peter’s anomaly, ectopialentis, posterior lenticonus, uveitis, tumor (retinoblastoma, medullaepithelioma)), metabolic (diabetes, galactosemia, hypocalcemia, Lowe’s syndrome, congenital haemolytic jaundice, hypoglycaemia, mannosidosis, Alport’s syndrome, Fabry’s disease), maternal drug ingestion/ malnutrition, and trauma.Unilateral cataracts: Idiopathic (80%), intrauterine infection (rubella 33% unilateral), ocular abnormalities (10%), and trauma (9%).
  • Congenital cataract

    1. 1. Overview:1. Definition2. Etiology3. Epidemiology4. Morphology5. Diagnosis6. Differential Diagnosis7. Management8. Complication9. Prognosis
    2. 2. Definition:• A congenital opacity of thecrystalline lens. Cloudiness in thelens of the eye that is presentat, or develops shortly afterbirth. Congenital cataracts are alsothe most frequent cause ofleukocoria (white pupil) inchildren.(Friedman, NJ & Kaiser, PK, 2009)
    3. 3. Etiology:BILATERAL• Idiopathic (60%)• Hereditary (30%)• Intrauterine infection• Associated with ocular disorders• Tumor• Metabolic• Maternal drug ingestion/malnutrition• TraumaUNILATERAL• Idiopathic (80%)• Intrauterine infection• Ocular abnormalities (10%)• Trauma (9%)(Friedman, NJ & Kaiser, PK, 2009)
    4. 4. Epidemiology:• The incidence is about 1 in 2000, andapproximately one-third of congenital cataractsare familial, one-third are associated with asyndrome, and one-third are isolated.(Friedman, NJ & Kaiser, PK, 2009)
    5. 5. Morphology:• The morphology of cataract is important for several reasons: it can give a clue to the age ofonset, to the visual prognosis, it may suggest heritability, and it may give a clue to the aetiology.(Joseph, E., 2006)Punctate (blue-dot)cataractAnterior polarcataract-pyramidal-reduplicatedPosterior polarcataract(Embryonal) nuclearcataractCoronary cataract Coralliform/FloriformcataractZonular (Lamellar)cataractTotal cataract-soft -membranous(Basak, SK., 2007)
    6. 6. Morphology:(Basak, SK., 2007)
    7. 7. Punctate (blue dot) cataract:• Most common type• Appears as multiple, tiny blue dotsscattered all over the lens, especiallyin the cortex by oblique illuminationwith the slit lamp.• Bluish color is due to the effects ofdispersion of light, in the same waythat the sky appears blue.• Visual acuity is not affected(Basak, SK., 2007)
    8. 8. Anterior Polar Cataract• It is sharply demarcated opacity at theanterior lens capsule, & usually the size of apin’s head.• It may project forwards into the anteriorchamber like a pyramid (pyramidalcataract); or subsequently, a sub-capsularcataract may develop, just behind it with aclear zone in between (reduplicatedcataract)• Due to delayed formation of the anteriorchamber, during development of the lens.• These opacities are stationary and rarelyinterfere with vision.(Basak, SK., 2007)
    9. 9. Zonular (Lamellar) Cataract• The commonest developmentalcataract presenting with visualimpairment.• May be congenital/ occur 1st year oflife.• Usually bilateral & hereditary(autosomal dominant), without anyocular anomaly.• Ass. with hypovitaminosis vit D orhypocalcemia & maternalmalnutrition.(Basak, SK., 2007)
    10. 10. Zonular (Lamellar) Cataract• Consist of concentric, sharplydemarcated zones(lamellae) ofopacities surrounding a core, that isclear, & enveloped by the clearcortex externally.• May be linear opacities, like spokesof a wheel (Called riders) thatextends outwards towards theequator pathognomonic.• Child present with photophobia(due to light scattering)• Surgery when VA less than 6/18(Basak, SK., 2007)
    11. 11. Diagnosis:• History:• 1. Duration• 2. Family history of congenital cataracts• 3. Visual status: Ambulation in familiar & unfamiliar surroundings.• 4. Behavioural pattern & school performance.• Birth history:• 1. History & Degree of consanguinity• 2. History of maternal infection during 1st trimester• 3. Gestational age & birth weight• 4. Birth trauma• 5. Supplemental O2 therapy in perinatal period.• 6. Developmental milestone(Joseph, E., 2006) & (The Royal College of Ophthalmologists, 2010)
    12. 12. Diagnosis:• Ocular examination:• 1. Measurement of visual acuity• (An up to date refraction should be available as part of the optometrist’s report)• 2. Pupil examination• 3. External eye examination including lids and lashes.• 4. Measurement of intraocular pressure• 5. Slit lamp examination• -associated congenital anomalies of iris & lens.• -type of cataract• -iridodenesis/ phacodonesis• 6. Dilated examination of the cataract and fundus• 7. Biometry if the child is old enough to cooperate. If not this may need to bedone under anaesthesia(Joseph, E., 2006) & (The Royal College of Ophthalmologists, 2010)
    13. 13. Differential Diagnosis: -retinoblastoma -toxoplasmosis -toxocariasis -retinal detachment -retinitis of prematurity - persistent hyperplastic primary vitreous (PHPV) -Coat’s disease -coloboma -mylinated nerve fibers -retinal dysplasia -Norrie’s disease -incontinentia pigmenti -retinoschisis -cyclitic membrane(Friedman, NJ & Kaiser, PK, 2009)
    14. 14. Management:• Indications for surgery:-They are interfering with a person’s quality of life-There is a reasonable prospect that surgery will lead to a significant improvement in vision.-All dense cataract( unilateral/bilateral) & partial cataract with vision less than 6/18.• Timing for surgery-The critical period for development of fixation reflex is between 6 weeks & 6 months of age.So, best time for surgery is before this period to prevent stimulus-deprivation amblyopia.-In dense monocular cataract, the surgery should be done as early as possible, may be on thenext day after birth.(Basak, SK., 2007)
    15. 15. Surgical Techniques:• Options available for children are:-Lensectomy-Lens aspiration with IOL-Lens aspiration with Primary Posterior Capsulotomy (PPC) and IOL-Lens aspiration with PPC, Anterior Vitrectomy (AV) and IOL-Lens aspiration with PPC, AV, IOL and Posterior capsular optic capture(PCOC)(Basak, SK., 2007)
    16. 16. Visual Rehabilitation:• IOL Implantation-Implanted with an IOL during surgery and the criteria of IOL implantation depend on the child’s age andwhether the cataract is unilateral or bilateral. It is perfectly safe and acceptable to perform primaryimplantation in a child older than one year. A foldable acrylic hydrophobic IOL is the most biocompatible IOL.• Contact Lens-If no IOL is implanted, contact lenses are given as early as possible to prevent stimulus deprivation amblyopia.Frequent retinoscopy should be performed to decide the power of CL and an overcorrection of +2 to +3D ismandatory. Silicon lenses or soft hydrogels are well tolerated.• Spectacles-In some children with bilateral aphakia spectacles are better tolerated than contact lenses. In addition asecondary strabismus may be manipulated by prismatic effect of spectacles. . Bifocal glasses should beprescribed when the child is about to start school.(Joseph, E., 2006)
    17. 17. Post-Operative Complications:• -Inflammation• - Deposition of pigment on lens• - Iris capture/ Lens decentration• - Retinal Detachment• - Glaucoma(The Royal College of Ophthalmologists, 2010)
    18. 18. Prognosis:• The visual outcome depends on:-Unilateral /bilateral involvement-Density of cataract during presentation-Degree of visual impairment(Basak, SK., 2007)
    19. 19. REFERENCES:• Basak, SK., Essentials of Ophthalmology 4th edition, 2007. Current Books Int, India, 235-240.• Bashour, M., et al.,, 07 August 2007.Congenital Cataract. [Assessed on 08-06-2013]• Friedman, NJ & Kaiser, PK, Essentials of Ophthalmology, 2009. Saunders Elsevier, India, 92-95.• Joseph, E., Management of Congenital Cataract, Kerala Journal of Ophthalmology, 2006; 18(3):224-230.• Taylor, D., Congenital cataract: the history, the nature and the practice, Journal of Eye, 1998, 12:9-36.• The Royal College of Ophthalmologists, Cataract Surgery Guidelines: PaediatricOphthalmology, September 2010: 25-31.