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Diseases of the Lens

Diseases of the Lens. Doç.Dr.Raciha Beril Küçümen Yeditepe University Department of Ophthalmology 2013 - 2014 Educational Year. AIM. Learning the diagnosis and treatment of lens diseases . Learning the relationship between lens diseases and other systemic diseases. OBJECTIVE.

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Diseases of the Lens

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  1. Diseases of the Lens Doç.Dr.Raciha Beril Küçümen Yeditepe University Department of Ophthalmology 2013 - 2014 Educational Year

  2. AIM • Learning the diagnosis and treatment of lens diseases. • Learning the relationship between lens diseases and other systemic diseases.

  3. OBJECTIVE • To be able to completely list the etiology, classification, signs and symptoms, and different types of cataract. • To be able to completely write the diseases and syndromes that cause lens dislocation. • To be able to completely list lens shape disorders. • To be able to completely list of cataract surgical techniques and technology used in modern cataract surgery.

  4. Cataract • Any congenital or acquired opacity in the lens capsule or substance is called cataract.

  5. Classification of cataract • Aetiological • Morphological • According to maturity • According to age of onset

  6. Aetiological • Senile • Presenile • Traumatic • Metabolic • Toxic • Complicated (secondary) • Maternal infection • Maternal drug ingestion • Syndromes • Hereditary

  7. Cataract • Age - related cataract is a very common cause of visual impairment in older adults. • In cross - sectional studies the prevalence of cataract is 50% in people between the ages of 65 and 74 increasing to 70% in those over the age of 75. • The pathogenesis of age - related cataract is multifactorial and not completely understood.

  8. Senile • Subcapsular: Anterior or posterior.Patients with posterior subcapsuler cataract have more diminished near vision than their distance vision. • Nuclear: Its frequently associated with myopia due to an increase in the refractive index of the lens and also with increased spherical aberration. Some individuals read without spectacles a condion referred as second sight • Cortical: Anterior, posterior, equatorial. • Christmas Tree cataract is uncommon , characterized by polychromatic, needle-like deposits in cortex and nucleus - myotonic dystrophy

  9. Subcapsular cataract Anterior Posterior

  10. Nuclear cataract Progression • Increasing nuclear opacification • Exaggeration of normal nuclear • ageing change • Causes increasing myopia • Initially yellow then brown

  11. Cortical cataract Progression Progressive radial spoke-like opacities Initially vacuoles and clefts

  12. Christmas Tree Cataract

  13. Traumatic: • Penetrating(intraocularforeign body, siderosisbulbi, chalcosis) • Concussion • İnfraredirradiation(glass-blowers’ cataract) • Electricalinjury • Chemicalinjuries • Ionizingradiation(oculartumorssuch as retinoblastoma) • Ultravioletradiation Trauma is themostcommoncause of unilateralcataract in youngindividuals.

  14. Causes of traumatic cataract Concussion ‘Vossius’ ring from imprinting of iris pigment Flower-shaped Penetration Other causes • Ionizing radiation • Electric shock • Lightning

  15. Metabolic: • Diabetes:Is associated with two types of cataract. • 1)Senile cataract which appears earlier and may progress more rapidly. • 2)True diabetic cataract which is due to osmotic overhydration of the lens and appears as bilateral white punctate or snowflake posterior or anterior opacities.

  16. Other causes of cataract - diabetes Juvenile Adult

  17. Metabolic: • Galactosaemia – Oildropletcataract • Hypoglycaemia • Galactokinasedeficiency • Mannosidosis • Fabry’sdisease - angiokeratomacorporisdiffusum • Lowesyndrome – Posteriorlenticonus • Wilson disease – Sunflowercataract • Hypocalcaemia- tetaniccataract-parathyroiddestruction • Myotonicdystrophy – ‘Christmastree’ cataract

  18. Toxic: • Corticosteroids • Chlorpromazine- (Largactil) sedative • Miotics • Busulphan - Myleran • Gold – Romatid arthritis • Amiodarone

  19. Corticosteroids: • Posterior subcapsular cataract • Safe dose? Children are more susceptible • Individual susceptibility (genetic?) • Less in intermittent therapy

  20. Drugs Systemic or topical steroids Chlorpromazine - initially posterior subcapsular - central, anterior capsular granules Other drugs • Long-acting miotics • Amiodarone • Busulphan

  21. Secondary: • Anterior uveitis: This is the most common cause of secondary cataract.The lens opacification seems to progress more rapidly in the presence of posterior synechia. • Hereditary retinal: Retinitis pigmentosa, Leber’s congenital amaurosis,Wagner’s and Stickler’s syndrome) • High myopia • Glaukomflecken – acute angle closure glaucoma

  22. Glaucoma and Cataract • Glaukomflecken Acuteangleclosureglaucoma

  23. Cataract and GlaucomaLens – Induced Glaucoma • PhacolyticGlaucoma mature /hypermaturecataracts: denatured lens protein leaksthroughintactcapsule, macrophagesingest lens protein , togethertheyclogthetrabecularmeshwork. • Lens ParticleGlaucoma Aftercataractsurgeryretainedcorticalmaterialparticlesobstructtrabecularmeshwork • PhacomorphicGlaucoma Intumescentcataractcausespupillaryblockandangleclosureglaucoma

  24. Lens – Induced Glaucoma Signs & Symptoms: Headache Nausea Impaired general condition Red eye Pain in the eye Visual loss High intraocular pressure Middilated pupil with no reaction Shallow anterior chamber Leukocoria Treatment: • Systemic & Topical antiglaucomatous agents • Tranquilizers • Laser iridotomy • Cataract Surgery

  25. Congenital CataractMaternal infections: • Rubella: Fetal infection is the direct result of maternal viraemia (which may be clinical or subclinical) with seeding of the virus in the plasenta. • Fetal infection is about 50% during the first 8 weeks,33% between week 9 and 12 and about 10% between 13 and 24. • Cataract, microphtalmos,retinopathy, glaucoma etc • Cataracts are present in about 50% of cases.After the gestastional age of 6 weeks the virus is incapable of transversing the lens capsule so that the lens is immune.

  26. Maternal infections: • Altough the lens opacities (which may be unilateral or bilateral) are usually present at birth, they may occasionally develop several weeks or even months later. • The virus has been shown to be capable of persisting within the lens for up to 3 years postnatally.

  27. Maternal drug ingestion: • Thalidomide • Corticosteroids

  28. Leukocoria • literally means " white pupil « • Leukocoria can be caused by abnormalities in the lens (eg, cataract), vitreous (eg, hemorrhage), or retina (eg, retinoblastoma)

  29. Causes of Leukocoria • Retinoblastoma • Persistent fetal vasculature • Retinopathy of prematurity • Cataract • Coloboma (fissure or cleft) of choroid or optic disc • Uveitis • Toxocariasis • Coats disease • Vitreous hemorrhage • Retinal dysplasia

  30. Syndromes: • Down’s syndrome (mongolism) • Werner’ s syndrome • Rothmund’s syndrome

  31. Hereditary • 1/3 of all congenital cataracts are hereditary and unassociated with any of the metabolic or sistemic disorders. • The mode of inheritance is usually dominant.

  32. Morphological: • Capsular • Subcapsular • Nuclear • Cortical • Lamellar • Sutural

  33. According to maturity • Immature:Scattered opacities are seperated by clear zones. • Mature: Cortex is totally opaque • Intumescent: The lens has become swollen by imbibed water.It can be mature or immature • Hypermature: Mature cataract which has become smaller and has a wrinkled capsule due to leakage of water out of lens. • Morgagnian: Hypermature cataract in which total liquefaction of the cortex has allowed the nucleus to sink inferiorly.

  34. Classification according to maturity Immature Mature Hypermature Morgagnian

  35. According to the age of onset • Congenital • Infantile • Juvenile • Presenile • Senile

  36. Abnormalities of the lens shape: • Coloboma • Anterior lenticonus • Posterior lenticonus • Lentiglobus • Microphakia • Microspherophakia

  37. Coloboma Ocular associations Coloboma of choroid Giant retinal tear Coloboma of iris

  38. Lenticonus Posterior Anterior • Anterior axial bulge Central 3-4 mm of the lens • Posterior axial bulge • Unilateral - usually sporadic • Associated with Alport syndrome • Bilateral - familial or in Lowe • syndrome

  39. LOWE SYNDROME Oculocerebrorenal syndrome Error of aminoacid metabolism X-linked inheritance pattern Frontal prominence and sunken eyes Proteinuria Fanconi syndrome Renal tubular acidosis Areflexia Muscular hypotonia Mental retardation Glaucoma Posterior polar cataract + lenticonus

  40. ALPORT SYNDROME XLD inheritance Anterior lenticonus Pigmentary retinopathy Chronic renal failure Sensorineural deafness Hematuria

  41. Small lens Microphakia Microspherophakia • Small diameter and spherical • Small diameter • Systemic association • - Lowe syndrome • May be familial (dominant) • Systemic association • - Weill-Marchesani syndrome

  42. Ectopia lentis • Ectopia lentis is a displacement of the lens and may be congenital,developmental or acquired. • A subluxated lens is partially displaced from its normal position but remains in the pupillary area. • A luxated or dislocated lens is completely displaced from the pupil implying seperation of all zonular attachments.

  43. Ectopia lentis • Findings associated with subluxation of the lens include decreased vision,marked astigmatism,monocular diplopia and iridonesis. • Potential complications of ectopia lentis include cataract and displacement of the lens into the anterior chamber or into the vitreous.

  44. Ectopia lentis • Dislocation into the anterior chamber or pupil may cause pupillary block and angle –closure glaucoma.

  45. Ectopia lentis • Inherited Marfan’s syndrome, Weill-Marchesani syndrome, Homocystinuria Hyperlysinaemia Familial ectopia lentis Aniridia

  46. Marfan’ syndrome • Marfan’s syndrome is a heritable disorder with ocular,cardiac and skeletal manifestations. • From 50% to 80 % of patients with Marfan’s syndrome exhibit ectopia lentis. • The lens subluxation tends to be bilateral and symmetric and is usually superior and temporal.

  47. Marfan’s syndrome • The zonuler attachments usually remain intact but become streched and elongated. • Associated ocular abnormalities in Marfan’s syndrome include axial myopia and increased risk of retinal detachment. • Children with lens subluxation may develop amblyopia if there is significant asymmetry in their refractive error or if their refractive error is uncorrected in early childhood.

  48. Autosomal dominant Systemic features of Marfan syndrome • Arachnodactyly • Limb-trunk disproportion • Aortic dilatation, dissection • and regurgitation • Pectus excavatum • High-arched palate • Mitral valve prolapse

  49. Ocular features of Marfan syndrome Lens Retinal detachment • Axial myopia • Lattice degeneration • Upward subluxation • Zonule usually intact Angle anomaly and glaucoma Cornea plana Blue sclera

  50. Autosomal recessive Weill-Marchesani syndrome Systemic features Ocular features • Microspherophakia • Short stature • Anterior lens subluxation • Short stubby fingers (brachydactyly) • Angle anomaly and glaucoma • Mental handicap

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