3. Introduction
Growth and development
• The eye of a normal full-term infant at birth is
approximately 65% of adult size
• The lens of a newborn infant is more spherical
• Its greater refractive power helps to compensate for the
relative shortness of the young eye.
• An infant's eye is somewhat hyperopic (farsighted).
• Tears often are not present with crying until after 1-3 mo
5/16/2023 3
4. Normal human visual development
Function Age
Intermittent visual fixation present birth
Fixates well on nearby human face 2- 3 months
Smooth following movements on nearby objects 3 months
Full accommodation possible 3-4 months
Onset of stereopsis 3-5 months
Fixates well on distant objects 6 months
Subjective acuity possible 3 years
Adult type visual acuity testing possible 5- 6 years
5/16/2023 4
Albert , pediatric ophthalmology 3rd edition
5. Physiology of Eye: Image formation
Refraction of light:
• Cornea refracts 75% of light transmitted
• The rest done by the lens
• Image is projected on the retina, inverted, minimized
and real
5/16/2023 5
6. Accommodation of lens :
• The lens is biconvex intensifies focusing power
• The lens is flexible and can change curvature to
accommodate according to light and object distance
5/16/2023 6
8. Definition: Is the clouding or opacification of a lens
which would result in vision loss
• Greek word – cataractos (water fall)
• It remains the leading cause of blindness in middle &
low income countries.
5/16/2023 8
9. EPIDEMIOLOGY
• Childhood cataracts ranges from 1 to 15 per 10,000
children
• Congenital cataract 1 to 3 per 10,000
• Cataracts are responsible for nearly 10% of all vision
loss in children worldwide
• In our country it is also the first cause of &
constitutes 40% of all blindness.?
5/16/2023 9
12. Classification of cataracts in children
Category Location Comment
Anterior pole Anterior pole , usually bilateral Often hereditary
Persistent fetal vasculature Posterior , unilateral microphthalmia and
glaucoma
Posterior pole Unilateral posterior pole
(Mittendorf dot)
Bilateral posterior pole defects in the posterior
capsule
Posterior
lentiglobus/lenticonus
Posterior capsule; mainly
unilateral
Usually sporadic
Posterior subcapsular
Immediately anterior to posterior
capsule
inflammatory
conditions,
glucocorticoids use,
ionizing radiation
Total (diffuse, complete) Involve entire crystalline lens Usually seen after
trauma
Zonular cataracts
5/16/2023 12
14. Congenital/infantile cataract
• congenital cataract : lens opacity present at birth.
• Lens opacities that develop during the first year of life
are called infantile cataracts
• 1/3rd are a component of a more extensive
syndrome(congenital rubella syndrome),
• 1/3rd occur as an isolated inherited trait, and one-third
result from undetermined causes
5/16/2023 14
15. CLINICAL FEATURES
Presentation :
• Progressive painless reduction of vision
• Glare
• Photophobia
• Vision deterioration in bright light
• Dulled color vision
Signs
• Reduced V/A
• Whitish opacity seen through the pupil
5/16/2023 15
16. MANAGEMENT
1. Conservative
• Children with good vision (20/50 or better),
• Small opacities (<3 mm), or
• Extra-axial opacities
• Refractive error is treated with spectacle or contact
lens correction
5/16/2023 16
17. cont….
2. Cataract extraction
Indications
• Bilateral complete cataracts.
• For children with incomplete cataracts
• Decreased visual response
• Reduced visual acuity (20/50 or worse)
• Opacity >3 mm in diameter
• Onset of strabismus and/or nystagmus, which indicate a
significant disruption in fusion
•
5/16/2023 17
18. Cont….
Timing of the Procedure
• Visually significant unilateral cataract should be
removed before age 6 weeks;
• Visually significant bilateral cataracts, before age 10
weeks.
Intraocular Lens Use in Children
• Aged 1–2 years and older is widely accepted
5/16/2023 18
19. Potential complications of cataract surgery
• Strabismus
• Glaucoma
• Swelling of the retina
• IOL may need to be repositioned or dislocated
5/16/2023 19
20. Prevention
• No effective way to prevent formation of cataract
• Secondary prevention
• Wearing sun glasses out side during the day time
• Vitamins ,minerals
• Healthy life style (do not smoke)
• DM ( tight blood control)
5/16/2023 20
22. • Heterogeneous group of diseases result from :
• Isolated congenital abnormality of the aqueous
outflow pathways (primary glaucoma) or
• Abnormalities affecting other regions of the eye
(secondary glaucoma)
• Childhood glaucoma is defined as intraocular
pressure (IOP)–related damage to the eye
5/16/2023 22
23. Classification of childhood glaucoma
1. Primary childhood glaucoma
• Primary congenital glaucoma(PCG
Neonatal
Infantile
late onset ( age above 2 year
• Juvenile open angle glaucoma (JOAG
2. Secondary childhood glaucoma
• Nonaquired systemic disease or syndrome
• Acquired condition
• Following cataract surgery
5/16/2023 23
24. Genetics
• Primary congenital glaucoma (PCG) usually occurs
sporadically, it may be inherited as an autosomal
recessive trait.
• The chance of an affected parent having a child with PCG
is approximately 2%
• Mutations in CYP1B1 (at the GLC3A locus) have been
shown to cause PCG.
• Juvenile open-angle glaucoma is inherited as an
autosomal dominant trait
5/16/2023 24
25. Primary Congenital Glaucoma
• Most common form of childhood glaucoma
• Males (65% of cases), and
• Bilateral in about two-thirds of patients.
• Diagnosis is made at birth in only 25%
• First year of life in more than 80% of cases
• Results in blindness in 2%–15% of cases, and
• visual acuity remains worse than 20/50 in at least 50%
5/16/2023 25
26. Pathophysiology
• Increased resistance to aqueous outflow through the
trabecular meshwork due to abnormal development of
neural crest–derived tissue of the anterior chamber
angle.
• The anomaly occurs late in embryologic development
5/16/2023 26
30. Secondary childhood glaucoma
• Associated With an Acquired Condition
• Corticosteroid use
• Uveitis
• Infection, or ocular trauma.
• Anticonvulsant and antidepressant medication
• Glaucoma Following Cataract Surgery
• Aphakic glaucoma 15% to 50%
5/16/2023 30
31. Treatment
• The primary treatment for most types of childhood
glaucoma is surgery.
• Angle surgery (Goniotomy or Trabeculotomy)
• Second-line surgical therapies
• Trabeculectomy,
• Implantation of drainage devices, and
• Cyclodestructive procedures
5/16/2023 31
32. Medical Therapy
• Lower success rates and greater risks than medical
therapy for adult
• Used in preoperative, postoperative, and long-term
management, particularly in childhood glaucoma
other than PCG
5/16/2023 32
33. Conjunctivitis
• The conjunctiva is transparent mucous membrane that
lines the inner surface of eyelids and anterior surface
of the globe
• The portion covering the globe is the "bulbar
conjunctiva," and the portion lining the lids is the "tarsal
conjunctiva."
• It is common in childhood and may be infectious or
noninfectious.
5/16/2023 33
35. Ophthalmia Neonatorum
• Occurring in infants younger than 4 wk of age, is the
• Most common eye disease of newborns.
• Acquired during vaginal delivery
• Reflects the sexually transmitted infections prevalent in
the community
• Gonococcal ophthalmia neonatorum has an incidence of
0.3/1,000 live births
• Chlamydia trachomatis incidence of 8.2/1,000 births.
5/16/2023 35
37. Gonococcal ophthalmia neonatorum
• Incubation period is 2-5 days,
• May be present at birth owing to PROM
• If after 5 days of life due to partial suppression by ocular
prophylaxis
• May also begin in infancy after inoculation by the
contaminated fingers of adults
• Incidence was reduced widespread use of silver nitrate
prophylaxis, prenatal screening, and treatment
5/16/2023 37
38. Clinical presentation
• Begins with mild inflammation and a serosanguineous
discharge
• Within 24 hr, the discharge becomes thick and purulent
• Tense edema of the eyelids with marked chemosis
occurs
5/16/2023 38
39. Diagnosis and Treatment
• Gram stain of the conjunctival exudate
• Gram-negative diplococcic
• Ceftriaxone (25 to 50 mg/kg, not to exceed 125 mg, IV or
IM.
• Where available, a single dose of cefotaxime (100 mg/kg,
IV or IM)
• The eye should also be irrigated initially with saline every
10-30 min, gradually increasing to 2-hr intervals until the
purulent discharge has cleared.
5/16/2023
39
40. Chlamydia trachomatis
• Exposure to an infected mother's genital flora during
vaginal birth
• The risk of acquiring C. Trachomatis in an infant born
vaginally to a woman with chlamydial cervicitis is
approximately 50 percent
5/16/2023 40
41. Clinical presentation
• The incubation period is 5 to 14 days after delivery.
• Presentation before five days is unusual
• Mild swelling with a watery eye discharge, which
becomes mucopurulent, to marked swelling of the
eyelids with red and thickened conjunctivae (chemosis
5/16/2023 41
42. cont….
Culture
• “Gold standard" for diagnosing neonatal C.
trachomatis conjunctivitis and pneumonia
Treatment
• Erythromycin (50 mg/kg per day given orally in
four divided doses) for 14 days
• Azithromycin (20 mg/kg given orally once daily
for a three-day course)
5/16/2023 42
43. TRACHOMA
• Leading cause of preventable irreversible blindness in the world.
• Direct contact with eye, nose, and throat secretions
• The house fly is an important vector.
• Features of the disease are divided into 2:
Active trachoma: usually in children.
• Follicular conjunctivitis, inflammation, discharge
Cicatricial trachoma: in middle age.
• Conjunctival scar, Herbert pits, Trichiasis, .
• Corneal vascularization and opacification.
43
45. WHO simplified trachoma grading:
• TF : > 5 follicles ,each >0.5mm,in the upper tarsal
conjunctiva.
• TI :pronounced inflammatory thickening of upper tarsal
conjunctiva that obscures more than half of normal deep
vessels.
• TS : presence of scarring in the upper tarsal conjunctiva.
• TT : at least one eyelash touching the cornea
• CO : easily visible corneal opacity over the pupil.
45
51. TREATMENT
(The “SAFE” strategy)
• Surgery: For Trichiasis and entropion.
• Antibiotics: For active trachoma
• 1% TTC BID for 6 weeks.
• Azithromycin 20mg/kg PO Stat for mass
treatment.
• Alternatively, topical azithromycin (1.5%) for
three days
• Facial cleanliness
• Environmental improvement: Acess to water,
sanitation and fly control.
51
52. Bacterial conjunctivitis
• Commonly caused by
• Staphylococcus aureus,
• Streptococcus pneumoniae,
• Haemophilus influenza,
• Moraxella catarrhalis
• Spread by direct contact with the patient and his or
her secretions or with contaminated objects and
surfaces.
• It is highly contagious
5/16/2023 52
53. Clinical presentation
• Complain of redness and discharge in one eye, can
also be bilateral
• Purulent discharge , thick and globular, it may be
yellow, white, or green
• The affected eye often is "stuck shut" in the morning
5/16/2023 53
55. Hyperacute bacterial conjunctivitis
• Neisseria species, N. Gonorrhoeae
• Severe and sight-threatening,
• Transmitted from the genitalia to the hands and then to
the eyes.
• Concurrent urethritis is typically present.
• Profuse purulent discharge present within 12 hours of
inoculation
5/16/2023 55
56. Cont ….
• Rapidly progressive other symptoms include
• Redness, irritation, and tenderness to palpation
• Marked chemosis, lid swelling, and
• Tender preauricular adenopathy
• Gram negative diplococcic can be identified on Gram
stain of the discharge.
5/16/2023 56
57. Investigation
• Mostly clinical, but
• Gram staining in sever cases to exclude gonococcal
infection.
Treatment
• Topical antibiotics: usually 4x per day for 1 week.
• Flouroquinolones: Ciprofloxacin, ofloxacin,
moxifloxacin
• Aminoglycosides: Gentamicin, neomycin
• Chloramphenicol, Tetracycline
• Macrolides: erythromycin, azithromycin
5/16/2023 57
58. Cont..
• Systemic antibiotics: in the following cases..
• Gonococcal: IV/IM ceftriaxone
• H. influenzae in children: PO Augmentin
• Preseptal or Orbital cellulitis
• General measures: lid hygiene (irrigation), hand
washing and avoidance of towel sharing
5/16/2023 58
59. Viral conjunctivitis
• Adenovirus most common,
• Less common causes HSV, VZV, HIV and
molluscaum contagiosum
• Highly contagious
• The second eye usually becomes involved within 24
to 48 hours
5/16/2023 59
60. Clinical presentation
• watery or mucoserous discharge, burning, gritting or
sandy feeling
• Systemic symptoms like sore throat or cold are common
• Viral conjunctivitis is a self-limited process
• Symptoms frequently get worse for the first three to five
days gradual resolution over the following one to two
weeks
5/16/2023 60
63. Epidemic keratoconjunctivitis
• Particularly fulminant, causing a keratitis in addition
to conjunctivitis.
• Caused by adenovirus types 8, 19, and 37
• Typical symptoms of viral conjunctivitis plus
Foreign body sensation and multiple corneal
infiltrates
5/16/2023 63
64. Molluscum contagiosum conjunctivitis
• Human specific double- stranded DNA ,poxvirus
• Peak incidence b/n 2 and 4 years,
• Chronic unilateral ocular irritation and mild mucoid
discharge
• Self-limiting in the immunocompetent
• Removal is often necessary to address secondary
conjunctivitis or for cosmetic reasons
5/16/2023 64
66. Treatment of viral conjunctivitis
• Usually self limiting: 2-3 weeks.
• Patients should be instructed to use cold
compresses and lubricants for comfort.
• Prophylaxis antibiotic eye drops may be given as
necessary to prevent bacterial super infection.
• Reduce transmission.
5/16/2023 66
67. Allergic conjunctivitis
• Acute conjunctival reaction to an environmental allergen,
usually pollen
• It is a classic type I (IgE)-mediated hypersensitivity
• Typically presents as bilateral redness, watery discharge,
redness, chemosis
• Itching is the cardinal symptom of allergy,
• Patients often have a history of atopy, seasonal allergy, or
specific allergy (eg, to cats).
5/16/2023 67
69. Retinopathy of Prematurity(ROP)
• Is a developmental vascular proliferative disorder
that occurs in the retina of preterm infants with
incomplete retinal vascularization
70. EPIDEMIOLOGY
• The incidence and severity increase with decreasing GA
and BW
• Most common in GA <28 wks.
• The overall incidence of ROP of any severity was
reported to be 66% ( moderate to severe 18%; and severe
6%)
Fanaroff & Martins
71. PATHOGENESIS
• Retinal vascularization normally begins at 15 to 18
weeks GA & completes 36 -40 weeks.
• It involves two phases
Phase 1: cessation of normal vascular growth (as well
as obliteration of immature vessels with high oxygen
use)
Phase 2: pathological vessel growth .
73. Risk factors
• Prematurity
• Low BW
• Assisted ventilation for longer than one week,
• Surfactant therapy,
• High blood transfusion volume,
• severe sepsis
• Low caloric intake,
• Hyperglycemia, and insulin therapy
74. Classification of ROP
Four features
• Zone
• Stage
• Extent
• Presence or absence of plus disease, the most
important indicator of disease severity.
75. ZONE
STAGE
Stage 1 : Flat white line demarcates vascular
& avascular retina
Stage 2: A ridge of fibrous tissue protrudes
into the vitrous b/n vascular and avascular
Stage 3: New blood vessels and fibrous tissue
grow along the ridge and often extend to vitrous
Stage 4: Partial retinal detachment
Stage 5 : Total retinal detachment
76. Cont..
• Plus disease
• Vitrous haze
• Engorgement of the iris vessels
• Poor dilation of the pupil
• High-risk prethreshold "type I ROP."
• Any stage ROP with plus disease in zone I
• Stage 3 ROP without plus disease in zone I
• Stage 2 or 3 ROP with plus disease in zone II
77. Treatment
• Treatment is generally indicated for eyes meeting
criteria for type I ROP
• Options
• Peripheral ablative therapy (with laser
photocoagulation) or
• Intravitreal injection of an anti-vascular
endothelial growth factor (VEGF) agent
• Surgical (scleral buckling or vitrectomy
78. Schedule for first indirect ophthalmoscopy in
premature infants
WHO
– All infants 30 weeks’ gestation or less, or weighing less than 1500 g at
birth
– Infants born at 1500 to 2000 g who have a medically unstable course
WHEN
– By the later of 31 weeks’ postmenstrual age or 4 weeks after birth
– Recommend first examination before discharge from the hospital
Fanaroff and Martinis
80. Retinoblastoma
• Most common malignant intraocular tumor of childhood
• It occurs in two forms
Heritable retinoblastoma
• Germline mutations (RB1) gene
• Bilateral disease
• Multifocal disease
• Positive family history,
• Those with known germline mutations
5/16/2023 80
81. Nonheritable retinoblastoma
• Somatic mutations in the RB1 gene
• Unilateral
• Unifocal disease and
• Tend to be diagnosed at a later age
• 15% of unilateral retinoblastoma cases are also due to
germline mutations and are therefore heritable
• Most cases of heritable retinoblastoma result from de novo
mutations and the family history is positive in only
approximately 25 % .
5/16/2023 81
82. EPIDEMIOLOGY
• It occurs in approximately 1/15,000 live births
• Accounts for 13 % of cancer in the first year of life
• 90% of cases are diagnosed before 3 years of age
• The median age at diagnosis is 18 to 20 months
• An average of 12 months for children with bilateral disease and
24 months for children with unilateral disease
5/16/2023 82
83. PATHOGENESIS
• Usually is caused by mutational inactivation of both alleles of the
retinoblastoma (RB1) gene maps to chromosome 13q14
Heritable form
• a germline mutation at the RB1 locus (most common) or
• deletion of chromosome 13q (containing the RB1 gene locus)
• And a second "hit," occurring later in development
Nonheritable retinoblastoma
– both allelic mutations arise spontaneously in a single somatic cell
of the retina
5/16/2023 83
84. Clinical presentation
• Leukocoria in 60%
• strabismus in 20%,
• Nystagmus, and a red inflamed eye
• Less common presentations -Decreased vision , ocular
inflammation
• Massive necrosis of the tumor can cause a presentation mistaken for
orbital cellulitis
• Metastatic disease: weight loss, vomiting, headache
5/16/2023 84
86. Trilateral retinoblastoma
• Consists of unilateral or bilateral retinoblastoma associated
with an intracranial tumor that is histologically similar
• Three-fourths of cases arise in the pineal gland but tumors
may also occur in the suprasellar or parasellar regions.
• Intracranial tumors occur in approximately 5 percent of
patients with bilateral or heritable retinoblastoma
• In Nonheritable unilateral retinoblastoma, occurring in <0.5%
5/16/2023 86
87. Diagnosis
• Indirect ophthalmoscopic examination
– chalky, off-white retinal mass with a soft, friable consistency.
• MRI- assess for extraocular extension and intracranial disease
• Aspiration of ocular fluids: special condition
• Pathology is not necessary to confirm the diagnosis.
• Biopsy is contraindicated because of the risk of tumor seeding
• The characteristic histologic features is small round blue cell tumor
with flexner-wintersteiner rosette formation
5/16/2023 87
88. International classification of intraocular retinoblastoma
Group Description Specific features
A Very low risk Tumor ≤3 mm in basal dimension or thickness
B Low risk • Macular location ≤3 mm to foveola
• Juxtapapillary location ≤1.5 mm to disc
• Clear subretinal fluid ≤3 mm from margin
C Moderate • Subretinal seeds ≤3 mm from tumor
• Vitreous seeds ≤3 mm from tumor
• Less than one quadrant of subretinal fluid in the fundus
D High risk • Subretinal seeds >3 mm from tumor
• Both subretinal and vitreous seeds >3 mm from tumor
• Greater than one quadrant of subretinal fluid in the fundus
E Very high risk • Neovascular glaucoma
• Opaque media from hemorrhage in anterior chamber, vitreous
• Invasion of postlaminar optic nerve, choroid (>2 mm), sclera, orbit
• Diffuse infiltrating tumor
• Phthisis bulbi or orbital cellulitis
5/16/2023 88
89. Treatment
• Determined by its size and location
• Team approach among pediatric oncologists, radiologists, and
ophthalmologists
• If limited to the eye, 98% of patients can expect 5-year disease-
free survival
• Hematogenous spread to the bone and liver,& brain, indicates a
very poor prognosis.
5/16/2023 89
90. Cont..
Local treatment
• laser photocoagulation or cryotherapy
• Small retinoblastoma tumors (Group A &B)
Systemic chemotherapy
• Spare vision for larger tumors
• Often used in cases of bilateral retinoblastoma
• With extension beyond the globe
Enucleation
• If likelihood of salvaging vision is low
• Advanced unilateral retinoblastoma
5/16/2023 90
91. Screening children at risk
• At-risk infants should initially be evaluated by an
ophthalmologist within the first 8 weeks of life
• During the first 3 years of life:
• Screening initially every 1-2 months and every 3 months
if there are no concerning findings.
• Age 3-7 years:
• Screening examinations are performed every 4-6 months.
• If genetic testing reveals that the child does not have a
germline RB1 mutation, screening can be discontinued.
5/16/2023 91
93. • is a functional reduction in visual acuity caused by abnormal
visual development early in life
• Usually unilateral or, less commonly
• Primarily a defect of central vision; the peripheral visual field is
usually normal.
5/16/2023 93
94. EPIDEMIOLOGY
• Estimated to be between 1 and 4 percent
• Equal frequency in boys and girls
• Left eye was more commonly affected than was the right,
particularly in cases of anisometropic amblyopia .
5/16/2023 94
95. Risk factors
• Prematurity
• Small size for gestational age
• First-degree relative with amblyopia
• Neurodevelopmental delay
5/16/2023 95
96. Pathophysiology
• Failure of normal neural development in the immature visual
system and abnormal visual experience early in life
– Abnormal binocular interaction
– Retinal image blur or both
Three “D”s classification
1. Deviated e.g. strabismus
2. Defocused e.g. anisometropia, ametropia
3. Deprived e.g. cataract,corneal opacity,etc
5/16/2023 96
97. Vision screening: findings indicative of amblyopia
Screening test finding
Preverbal children
• Fixation reflex Failure to initiate or maintain fixation
• Occlusion objection Asymmetric objection
• Vertical prism test Fixation preference or inability to maintain fixation
Older children
• Best-corrected VA
•Unilateral amblyopia:
≥2-line difference between eyes
•Bilateral amblyopia:
• Age ≤3 years: VA worse than 20/50 in either eye
•Age ≥4 years: VA worse than 20/40 in either eye
5/16/2023 97
101. Prognosis
• The Younger the child, the better the prognosis.
• Deprivation amblyopia carries poor prognosis.
• Strabismic amblyopia has best prognosis
• Patching is most effective than the other methods.
• ¼ recurrence within the first year after stopping therapy
5/16/2023 101
103. Messages
• Referral to an ophthalmologist is essential for
children in whom with signs and symptoms of
serious eye diseases or complications
• Ocular findings can also assist in the diagnosis of a
systemic illness thorough evaluation is mandatory
• Improving practice of using appropriate oxygen
target in preterm at NICU
104. The eye is the light of the body; therefore if the
eye is good, then the whole body will be full
of light, but if the eye is bad, then the whole body
will be full of darkness.”
FARUK H. ÖRGE, F.H & GRIGORIAN, F