Congenital cataract

Ophthalmology Project Paper Presentation

Congenital CataractR.Nandinii

080100332

Overview:1. Definition

2. Etiology

3. Epidemiology

4. Morphology

5. Diagnosis

6. Differential Diagnosis

7. Management

8. Complication

9. Prognosis

Definition:

• A congenital opacity of the crystalline lens. Cloudiness in the lens of the eye that is present at, or develops shortly after birth. Congenital cataracts are also the most frequent cause of leukocoria (white pupil) in children.

(Friedman, NJ & Kaiser, PK, 2009)

Etiology:BILATERAL

• Idiopathic (60%)

• Hereditary (30%)

• Intrauterine infection

• Associated with ocular disorders

• Tumor

• Metabolic

• Maternal drug ingestion/ malnutrition

• Trauma

UNILATERAL

• Idiopathic (80%)

• Intrauterine infection

• Ocular abnormalities (10%)

• Trauma (9%)

(Friedman, NJ & Kaiser, PK, 2009)

Epidemiology:

• The incidence is about 1 in 2000, and approximately one-third of congenital cataracts are familial, one-third are associated with a syndrome, and one-third are isolated.

(Friedman, NJ & Kaiser, PK, 2009)

Morphology:• The morphology of cataract is important for several reasons: it can give a clue to the age of onset,

to the visual prognosis, it may suggest heritability, and it may give a clue to the aetiology.

(Joseph, E., 2006)

Punctate (blue-dot) cataract

Anterior polar cataract

-pyramidal -reduplicated

Posterior polar cataract

(Embryonal) nuclear cataract

Coronary cataract Coralliform/Floriform cataract

Zonular (Lamellar) cataract

Total cataract-soft -membranous

(Basak, SK., 2007)

Morphology:

(Basak, SK., 2007)

Punctate (blue dot) cataract:

• Most common type

• Appears as multiple, tiny blue dots scattered all over the lens, especially in the cortex by oblique illumination with the slit lamp.

• Bluish color is due to the effects of dispersion of light, in the same way that the sky appears blue.

• Visual acuity is not affected

(Basak, SK., 2007)

Anterior Polar Cataract

• It is sharply demarcated opacity at the anterior lens capsule, & usually the size of a pin’s head.

• It may project forwards into the anterior chamber like a pyramid (pyramidal cataract); or subsequently, a sub-capsular cataract may develop, just behind it with a clear zone in between (reduplicated cataract)

• Due to delayed formation of the anterior chamber, during development of the lens.

• These opacities are stationary and rarely interfere with vision.

(Basak, SK., 2007)

Zonular (Lamellar) Cataract

• The commonest developmental cataract presenting with visual impairment.

• May be congenital/ occur 1st year of life.

• Usually bilateral & hereditary (autosomal dominant), without any ocular anomaly.

• Ass. with hypovitaminosis vit D or hypocalcemia & maternal malnutrition.

(Basak, SK., 2007)

Zonular (Lamellar) Cataract

• Consist of concentric, sharply demarcated zones(lamellae) of opacities surrounding a core, that is clear, & enveloped by the clear cortex externally.

• May be linear opacities, like spokes of a wheel (Called riders) that extends outwards towards the equator pathognomonic.

• Child present with photophobia (due to light scattering)

• Surgery when VA less than 6/18

(Basak, SK., 2007)

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) 

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 be done

under anaesthesia    

(Joseph, E., 2006) & (The Royal College of Ophthalmologists, 2010) 

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)

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 the

next day after birth.(Basak, SK., 2007)

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)

Visual Rehabilitation:• IOL Implantation

-Implanted with an IOL during surgery and the criteria of IOL implantation depend on the child’s age and whether

the cataract is unilateral or bilateral. It is perfectly safe and acceptable to perform primary implantation 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 is

mandatory. Silicon lenses or soft hydrogels are well tolerated.

• Spectacles

-In some children with bilateral aphakia spectacles are better tolerated than contact lenses. In addition a

secondary strabismus may be manipulated by prismatic effect of spectacles. . Bifocal glasses should be prescribed

when the child is about to start school.

(Joseph, E., 2006)

Post-Operative Complications:

• -Inflammation

• - Deposition of pigment on lens

• - Iris capture/ Lens decentration

• - Retinal Detachment

• - Glaucoma

(The Royal College of Ophthalmologists, 2010)

Prognosis:

• The visual outcome depends on:

-Unilateral /bilateral involvement

-Density of cataract during presentation

-Degree of visual impairment

(Basak, SK., 2007)

REFERENCES:

• Basak, SK., Essentials of Ophthalmology 4th edition, 2007. Current Books Int, India, 235-240.

• Bashour, M., et al., http://emedicine.medscape.com/article/1210837-clinical, 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: Paediatric Ophthalmology,

September 2010: 25-31.