1. Diseases of the Lens Doç.Dr.Raciha Beril Küçümen
Yeditepe University Department of Ophthalmology
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 Exaggeration of normal nuclear
ageing change
Increasing nuclear opacification
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 (intraocular foreign body,
siderosis bulbi, chalcosis)
Concussion
İnfrared irradiation (glass-blowers’ cataract)
Electrical injury
Chemical injuries
Ionizing radiation (ocular tumors such as retinoblastoma)
Ultraviolet radiation
Trauma is the most common cause of unilateral cataract in young individuals.

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 – Oil droplet cataract Hypoglycaemia
Galactokinase deficiency
Mannosidosis
Fabry’s disease - angiokeratoma corporis diffusum
Lowe syndrome – Posterior lenticonus
Wilson disease – Sunflower cataract
Hypocalcaemia- tetanic cataract-parathyroid destruction
Myotonic dystrophy – ‘Christmas tree’ 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 Other drugs
- 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
Acute angle closure glaucoma

23. Cataract and Glaucoma Lens – Induced Glaucoma
Phacolytic Glaucoma
mature /hypermature cataracts: denatured lens protein leaks through intact capsule, macrophages ingest lens protein , together they clog the trabecular meshwork.
Lens Particle Glaucoma
After cataract surgery retained cortical material particles obstruct trabecular meshwork
Phacomorphic Glaucoma
Intumescent cataract causes pupillary block and angle closure glaucoma
Denatured lens proteinsleak throuıgh intacta-permeable lens capsule macrophages ingest protein, trabecular meahwork is clogged both with lens proteins and engorged macrophages.

24. Lens – Induced Glaucoma
Treatment:
Signs & Symptoms:
Systemic & Topical antiglaucomatous agents
Tranquilizers
Laser iridotomy
Cataract Surgery
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

25. Congenital Cataract Maternal 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: 3 years postnatally.
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 Cataract 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
Werner premature aging, The appearance of affected individuals is abnormal. They exhibit growth retardation, short stature, premature graying of hair, alopecia (hair loss), wrinkling, prematurely aged face with a beaked nose, skin atrophy (wasting away) with scleroderma-like lesions, lipodystrophy (loss of fat tissues), abnormal fat deposition leading to thin legs and arms, and severe ulcerations around the Achilles tendon and malleoli (around ankles). Other medical signs include change in voice (weak, hoarse, high-pitched), atrophy of gonads leading to reduced fertility, bilateral cataract (clouding of lens), premature arteriosclerosis (thickening and loss of elasticity of arteries), calcinosis (calcium deposits in blood vessels), atherosclerosis (blockage of blood vessels), type 2 diabetes, osteoporosis (loss of bone mass), telangiectasia, and malignancies.[3][10] In fact, the prevalence of rare cancers, such as meningiomas, are increased in individuals with Werner syndrome.[14]
Rothmund–Thomson syndrome (RTS), also known as poikiloderma atrophicans with cataract or poikiloderma congenitale,[1][2] is a rare autosomal recessive[3][4] skin condition originally described by August von Rothmund (1830–1906) in Matthew Sydney Thomson (1894–1969) published further descriptions in 1936.
There have been several reported cases associated with osteosarcoma. A hereditary genetic basis, mutations in the DNA Helicase RECQL4 gene, has been implicated in the syndrome.[1][5][6]

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
Cıongenital cataract= lens opacities present at birth
İnfantile lens opacities that developbin the first year of life

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

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

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
Small diameter and spherical
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
Limb-trunk disproportion
Arachnodactyly
Aortic dilatation, dissection
and regurgitation
Pectus excavatum
High-arched palate
Mitral valve prolapse

49. Ocular features of Marfan syndrome
Lens
Retinal detachment
Upward subluxation
Lattice degeneration
Axial myopia
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

51. Ectopia lentis Acquired: Trauma(common)
Large eye(High myopia,buphthalmos)
Anterior uveal tumours
Chronic cyclitis(rare)
Hypermature cataract
Syphilis

52. Acquired ectopia lentis
Trauma
Stretched zonules
Buphthalmos
Megalocornea
Anterior uveal tumours
High myopia

53. Treatment The treatment of cataract is surgical Surgical Techniques
1. Intracapsular cataract extraction
(+ spectacles / contactlens / intraocular lens (IOL) )
2. Extracapsular cataract extraction + IOL
3. Phacoemulsification + IOL

54. Phacoemulsification Anesthesia (topical, local, general) Incision
Capsulorhexis
Hydrodissection
Phacoemulsification
Irrigation – aspiration
IOL Implantation
Removal of viscoelastic substance
Hydration of the incisions

55. Phacoemulsification + IOL Implantation
Anesthesia (topical, local, general)
Incision
Capsulorhexis
Hydrodissection
Phacoemulsification
Irrigation –aspiration
IOL Implantation,
Removal of viscoelastic substance
Hydration of the incisions

56. Complications of Cataract Surgery
MAJOR- EARLY
Endophthalmitis!!
Blurred vision- loss of vision
Severe ocular pain
Photophobia
Periorbital edema
Ciliary injection
Chemosis (conjunctival edema)
Corneal edema
Hypopyon - Collected white material in the anterior chamber
Treatment: URGENT!!
Vitreous tap-biopsy
Systemic antibiotics
Topical antibiotics-fortified
Intravitreal antibiotics
Vitrectomy (pars plana vitrectomy)

57. Complications of Cataract Surgery
MAJOR-LATE
Bullous Keratopathy
IOL Malposition
Cystoid Macular Edema
Retinal Detachment

58. Complications of Cataract Surgery
OTHER - EARLY
OTHER- LATE
Wound gape
Anterior Chamber Hemorrhage
İris Trauma
Zonular Rupture
Posterior Capsular Rupture
Vitreous Loss
Vitreous Hemorrhage
Choroidal Hemorrhage!!
Uveitis
Glaucoma (open angle/closed angle)
Posterior Capsule Opacification
IOL Dislocation