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Strabismus, Amblyopia & Leukocoria. Dr. Hessah Alodan, Pediatric Opthalmology Dept, KAUH. Why Two Eyes ?.

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Strabismus amblyopia leukocoria

Strabismus, Amblyopia & Leukocoria

Dr. Hessah Alodan,

Pediatric Opthalmology Dept, KAUH

Why two eyes
Why Two Eyes ?

You can demonstrate to a patient the difference in their field or their child's field with one eye compared to two. With two eyes you can also demonstrate the peripheral field and the central fusion.

Why two eyes1
Why Two Eyes ?

Left Eye Monocular  

Right Eye Monocular  


Total binocular field is nearly 170 degrees (varies according to configuration of orbits)

Why two eyes2
Why Two Eyes ?

Two Pencils Test

The same person with one eye closed or with manifest strabismus or no stereopsis will miss the examiner's pencil initially and place it correctly only after the second or third try.

With both eyes open the patient who uses both eyes producing stereopsis can put his pencil accurately on the examiner's pencil if stereopsis is present

  • Visual Axis

    • Imaginary line between fovea and the object

  • Binocular Vision

    • If the visual axises from both eyes intersect at the object, binocular vision occurs

  • Sensory Fusion

    • Supper imposed images from each corresponding retinal area in binocular cells at the level of the occipital cortex

      • Same images

      • Similar in size

      • Similar in clarity

  • Motor Fusion

    • Ability to physically move the eyes so that they are pointing in the same direction allowing the corresponding areas of the retina in each eye to be pointing at the object of regard

  • Visual Axes Misalignment lead to:

    • Confusion

      • Superimposition of the two different images stimulating corresponding retinal points

    • Diplopia

      • One object stimulating two none corresponding retinal points

What is strabismus
What is Strabismus ?

Ocular misalignment due to abnormality in binocular vision or anomalies in neuromuscular control of ocular motility

  • Classification of Strabismus:

    • According to fusion status

      • Phoria

        • Latent tendency of the eye to deviate and controlled by fusional mechanism

      • Intermittent Phoria

        • Fusion control is present part of the time

      • Tropia

        • Manifest misalignment of the eye all the time

  • Classification of Strabismus:

    • According to fixation

      • Alternating

        • Spontaneous alternation of fixation from one eye to the other

      • Monocular

        • Preference of fixation with one eye

  • Classification of Strabismus:

    • According to type of deviation

      • Horizontal

        • Esodeviation

        • Exodeviation

    • Vertical

      • Hyperdeviation

      • Hypodeviation

  • Torsional

    • Incyclodeviation

    • Excyclodeviation

  • Combined

    • Classification of Strabismus:

      • According to variation of the deviation with gaze position or fixing eye

        • Comitant

          • Same deviation in different direction of gaze

        • Incomitant

          • Variable deviation in different direction of gaze usually in paralytic or restrictive type of strabismus

    • Examination

      • History

      • Inspection

      • Assessment of monocular eye function

        • Visual acuity

          • Preverbal children

          • CSM

          • OKN

          • Preferential looking

          • Visual evoked potential

    • Examination

      • Assessment of monocular eye function

      • Visual acuity

        • Verbal children

        • Symbol tests

        • Single illiterate E

        • Allen pictures

        • H O T V letters

    • Examination

      • Assessment of binocular eye function

        • Hirschberg test

        • Krimski’s test

        • Cover test

        • Alternate cover test

        • Prism cover test

    • Examination

      • Fundoscopy

      • Cycloplegic refraction

        • Tropicamide

        • Cyclopentolate

        • Atropin

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    • Type of Strabismus

      • Esotropia

        • Pseudoesotopia

        • Infantile esotropia

        • Accommodative esotropia

        • Partially accommodative esotropia

    • Pseudoesotropia

      • Occur in patients with flat broad nasal bridge and prominent epicanthal fold

      • Gradually disappear with age

      • Hirschberg test differentiate it from true esotropia

    • Infantile Esotropia

      • Common comitant esotropia occur before six month of age

      • Deviation is often large more than 40 prism diopter

      • Frequently associated with nystagmus and inferior oblique over action

      • Treatment

        • Correction of refractive error

        • Treat amblyopia

        • Surgical correction of strabismus

    • Accommodative Esotropia

      • Occur around 2 ½ years of age

      • Start as intermittent then become constant

      • High hypermetropia

      • Treatment

        • Full cycloplegic correction

        • Treat amblyopia

    • Intermittent exotropia

      • Onset of deviation within the first year of age

      • Closing one eye in bright light

      • Usually not associated with any refractive error

      • Usually not associated with amblyopia

      • Treatment

        • Correction of any refractive error

        • Surgical correction of strabismus

    • Constant exotropia

      • Maybe present at birth or maybe progress from intermittent exotropia

      • Treatment

        • Correction of any refractive error

        • Correction of amblyopia

        • Surgical correction of strabismus

    • Sensory exotropia

      • Constant exotropia that occur following loss of vision in one eye e.g trauma

      • Treatment

        • Correction of any organic lesion of the eye

        • Correction of amblyopia

        • Surgical correction of strabismus

    • 6th Nerve Palsy

      • Incomitant esotropia

      • Limitation of abduction

      • Abnormal head position

    • 4th Nerve Palsy

      • Congenital or acquired

      • Hypertropia of the affected eye with excyclotropia

      • Abnormal head position

    • 3rd Nerve Palsy

      • Congenital or acquired

      • Exotropia with Hypotropia of the affected eye

      • In children caused by: trauma, inflammation, post viral and tumor

      • In adult caused by: aneurysm, diabetes, neuritis, trauma, infection and tumor

    • Duane Syndrome

      • Limitation of abduction

      • Mild limitation of adduction

      • Retraction of the globe and narrowing of the palpebral fissure on adduction

      • Upshoot or downshoot on adduction

      • Pathology faulty innervation of the lateral rectus muscle by fibers from medial rectus leading to co-contraction of the medial rectus and lateral rectus muscles

    • Brown Syndrome

      • Limitation of elevation on adduction

      • Restriction of the sheath of the superior oblique tendon

      • Treatment needed in abnormal head position or vertical deviation in primary position

    • Thyroid Ophthalmopathy

      • Restrictive myopathy commonly involving inferior rectus, medial rectus and superior rectus

      • Patients presents with hypotropia, esotropia or both

    • What is Amblyopia ?

      • Amblyopia refers to a decrease of vision, either unilaterally or bilaterally, for which no cause can be found by physical examination of the eye

    • Pathophysiology of Amblyopia

      • amblyopia is believed to result from disuse from inadequate foveal or peripheral retinal stimulation and/or abnormal binocular interaction that causes different visual input from the foveae

    Cortical ocular dominance columns representing amblyopic eye less responsive to stimulus and show changes microscopically

    Lateral geniculate layers subserving amblyopic eyes atrophic

    Afferent pupil response has been reported but not common

    No retinal changes - ERG OK

    • Amblyopia

    • Three critical periods of human visual acuity development have been determined. During these time periods, vision can be affected by the various mechanisms to cause or reverse amblyopia. These periods are as follows:

      • The development of visual acuity from the 20/200 range to 20/20, which occurs from birth to age 3-5 years.

      • The period of the highest risk of deprivation amblyopia, from a few months to 7 or 8 years.

      • The period during which recovery from amblyopia can be obtained, from the time of deprivation up to the teenage years or even sometimes the adult years

    • Amblyopia

      • Diagnosis of amblyopia usually requires a 2-line difference of visual acuity between the eyes

      • Crowding phenomenon: A common characteristic of amblyopic eyes is difficulty in distinguishing optotypes that are close together. Visual acuity often is better when the patient is presented with single letters rather than a line of letters

    An amblyopic eye with 20/70 full line vision

    may be able to see as well

    20/30 viewing a single optotype

    to 20/70

    • Causes of Amblyopia

      • Many causes of amblyopia exist; the most important causes are as follows:

      • Anisometropia

        • Inhibition of the fovea occurs to eliminate the abnormal binocular interaction caused by one defocused image and one focused image.

        • This type of amblyopia is more common in patients with anisohypermetropia than anisomyopia. Small amounts of hyperopic anisometropia, such as 1-2 diopters, can induce amblyopia. In myopia, mild myopic anisometropia up to -3.00 diopters usually does not cause amblyopia.

  • The patient favors fixation strongly with one eye and does not alternate fixation. This leads to inhibition of visual input to the retinocortical pathways.

  • Incidence of amblyopia is greater in esotropic patients than in exotropic patients

  • Alternation with alternate suppression avoids amblyopia

    • Causes of Amblyopia

      • Visual deprivation

        Amblyopia results from disuse or understimulation of the retina. This condition may be unilateral or bilateral. Examples include cataract, corneal opacities, ptosis, and surgical lid closure

    Deprivation Amblyopia

    Bilateral Deprivation Amblyopia

    • Causes of Amblyopia

      • Organic

        Structural abnormalities of the retina or the optic nerve may be present. Functional amblyopia may be superimposed on the organic visual loss

  • Uncorrected high hyperopia is an example of this bilateral amblyopia.

    • Treatment

      • The clinician must first rule out an organic cause and treat any obstacle to vision (eg, cataract, occlusion of the eye from other etiologies).

      • Remove cataracts in the first 2 months of life, and aphakic correction must occur quickly

      • Treatment of anisometropia and refractive errors must occur next

      • The next step is forcing the use of the amblyopic eye by occlusion therapy

    Causes of leukocoria
    Causes of Leukocoria

    • Cataract

    • Retinoblastoma

    • Toxocariasis

    • Coat´s disease

    • ROP

    • PHPV

    • Retinal detachment

    • Coloboma

    • Retinal dysplasia

    • Norrie´s disease


    • opacification of the lens.

    • Congenital cataracts usually are diagnosed at birth.

    • If a cataract goes undetected in an infant, permanent visual loss may ensue.

    • Bilateral cataracts are often inherited and associated with other diseases.

    • They require a full metabolic, infectious, systemic, and genetic workup.

    • The common causes are hypoglycemia, trisomy (eg, Down, Edward, and Patau syndromes), myotonic dystrophy, infectious diseases

    • (eg, toxoplasmosis, rubella, cytomegalovirus, and herpes simplex [TORCH]), and prematurity

    RETINOBLASTOMA with other diseases.

    • Retinoblastoma is the most common intraocular tumor of childhood.



    • Leukocoria (60%)

    • Strabismus (20%)

    • OTHER- Uveitis, Orbital cellulitis, Hyphaema, Heterochromia, Glaucoma, Bupthalmos

    RETINOBLASTOMA with other diseases.

    Retinoblastoma with other diseases.

    The disease is bilateral in approximately 30% of cases. The average age at diagnosis is 18 months and 90% of patients are diagnosed before the age of 3 years. Less than 10% of retinoblastoma suffers have a family history of the disorder, 90% of cases are sporadic. Of the sporadic cases, the responsible mutation is in a germ cell in 25% of cases and in a somatic cell in 75% of cases

    GENETICS with other diseases.

    • Retinoblastoma gene is a recessive oncogene of 180,000 kilobases.

    • Located chromosome- 13q14

    • Knudson two hit hypothesis:

    • Germinal cells have one defective and one normal RB gene.

    • A somatic mutation results in loss of the normal RB gene and hence retinoblastoma develops (somatic mutations occur frequently enough in the developing retina, therefore lesions usually affect both eyes)

    • In addition, the first child of a parent who had had a unilateral retinoblastoma has a 4% chance of developing the disease

    PATHOLOGY with other diseases.

    Arise in primitive photoreceptor cells.Characteristic histology:

    • Retinoblastomas are composed of poorly differentiated neuroblastic cells with scanty cytoplasm and prominent basophilic nuclei.

    • The tumour proliferates rapidly, with a tendency to outgrow its blood supply and undergo spontaneous necrosis. Necrotic tumour being eosinophilic stain pink.

    • Characteristic Flexner-Wintersteiner rosettes represent an attempt at retinal differentiation. Histologically, a ring of cuboidal cells is seen surrounding a central lumen. Cuboidaltumour cells with basally oriented nuclei arranged around a central lumen.

    Retinoblastoma with other diseases.

    Calcification is another feature of retinoblastomas, usually occurring in necrotic areas. Calcium stains with H&E. It is worth identifying calcium in suspect eyes by ultrasound, or CT scan to differentiate retinoblastomas from other tumours.

    MANAGEMENT with other diseases.



    • unilateral, poor visual prognosis

    • PLAQUE

    • 4-12mm +/- vitreous seeding


    • >12mm, multiple foci, only eye

    • LASER

    • consider- indirect, xenon arc

    • cryotherapy if <2dd in size

    • CHEMOTHERAPY, if intracranial extension

    Non retinoblastoma malignancies
    Non-Retinoblastoma Malignancies with other diseases.

    Unfortunately, children who have genetic retinoblastoma and survive their primary intraocular cancer have a substantially increased risk of death from one or more nonretinoblastoma malignancies over the course of their lifetimes, up to 35% of children who have had a bliateral retinoblastoma and external beam radiation therapy will develop a second cancer by age 25 years

    Congenital retinal telangiectasis coats disease
    Congenital retinal with other diseases. telangiectasis (Coats' disease)

    Congenital retinal telangiectasis (Coats' disease) is an idiopathic retinal vascular disorder that usually affects young male patients unilaterally in their first or second decade of life.. Patients may present with decreased vision, as well as strabismus or leukocoria in children. The hallmark feature of congenital retinal telangiectasis is localized fusiformaneurysmal dilations of the retinal vessels reminiscent of tiny light bulbs

    Persistent hyperplastic primary vitreous phpv
    Persistent hyperplastic primary vitreous (PHPV) with other diseases.

    Persistent hyperplastic primary vitreous (PHPV) is a congenital anomaly in which the primary vitreous fails to regress in utero. Highly vascular mesenchymal tissue nurtures the developing lens during intrauterine life. In PHPV, the mesenchymal tissue forms a mass behind the lens.

    Persistent hyperplastic primary vitreous phpv1
    Persistent hyperplastic primary vitreous (PHPV) with other diseases.

    A gray-yellow retrolental membrane may produce leukocoria, with the subsequent suspicion of retinoblastoma.

    In PHPV, the globe is white and slightly microphthalmic. Patients have no history of prematurity or oxygen administration.

    Retinopathy of prematurity rop
    RETINOPATHY OF PREMATURITY (ROP) with other diseases.

    • Vasoproliferative retinopathy affecting premature infants exposed to high oxygen


    • Prematurity (<32/40)

    • Birth weight (30% < 1000gm affected)

    • Oxygen duration

    • 90% ROP regresses spontaneously, 5% blindness

    RETINOPATHY OF PREMATURITY (ROP) with other diseases.

    • Signs include:

    • neovascularization,

    • fibrous bands

    • retinal detachments

    • vitreous hemorrhage

    • leukocoria

    Retinal detachment in childhood
    Retinal detachment in childhood with other diseases.

    Retinal detachment in childhood can be confused with retinoblastoma, and vice versa. The possibility of an underlying retinoblastoma should always be considered when a child presents with retinal detachment and vitreous hemorrhage, even when a history of trauma is obtained.

    Retinal detachment in childhood with other diseases.

    Risk factors:



    vitreous detachment

    high myopia

    retinal breaks or tears

    retinal vascular disease

    history of detachment in the other eye

    Retinal detachment in childhood with other diseases.


    flashes of light


    curtain-like decrease in vision

    COLOBOMA with other diseases.

    • Optic Disc Coloboma

    • Due to failure of closure of foetal fissure inferiorly

    • May be isolated disc or associated chorioretinalcoloboma

    • Usually sporadic, some AD

    • Can be bilateral

    • Visual acuity varies from normal to NPL.