POSTNATAL EYE PROBLEMS

OPHTHALMIA NEONATORUM

A.
Ophthalmia Neonatorum
B.
Retinopathy of Prematurity
C.
Congenital Glaucoma
D.
Retinoblastoma
occurring in infants younger than 4 wk of age
most common eye disease of newborns. Its many differentcausal agents vary greatly in their virulence and outcome.
Silver nitrate instillation may result in a mild self-limited chemical conjunctivitis
Neisseria gonorrhoeae and Pseudomonas are capable of causing corneal perforation, blindness, and death
The risk of conjunctivitis in newborns depends on:
o frequencies of maternal infections
o prophylactic measures
o circumstances during labor and delivery
o Postdelivery exposure to microorganisms

ETIOLOGY
1. Chemical
o Caused by silver nitrate drops instilled inconjunctival sac at birth for prophylaxis; not used anymore (Will cause a secondary chemical conjunctivitis)
o most apparent during 1st and 2nd day of life
o self-limited
o some advocate as the topical erythromycin and tetracycline
2. Chlamydial Infection
o most common identifiable infectious cause
o Onset bet. 5th and 14th day of life
o Giemsa stain: typical inclusion bodies in theepithelial cells of conjunctival scrapings andobserve under microscope confirms diagnosis
o systemic therapy + topical erythromycin
o parents should be treated since it is an STD
3. Bacterial Infection
o caused by:
Staphylococcus aureus
Hemophilus sp.
Streptococcus pneumonia
Neiserria gonorrhea
Pseudomonas sp.
o Presents between 2nd and 5th day of life
o Gonococcal conjunctivitis requires parenteral therapy
IM Ceftriaxone
Pen G (IV for penicillin-sensitivestrains)
Parents should be treated
o Topical therapy
Sodium sulfacetamide, Bacitracin, Tetracycline
4. Viral Infection

Herpes Simplex
Characteristic giant cells and viral inclusions on cytologic examination
Resolve spontaneously
May need antivirals severe cases

CLINICAL MANIFESTATIONS
Timing and character of the signs
- are somewhat typical for each cause of this condition, there is considerable overlap and physicians should not rely solely on clinical findings
Regardless of its cause, ophthalmia neonatorum is characterized by:
- Redness and chemosis (swelling) of the conjunctiva
- Edema of the eyelids
- Discharge which may be purulent
Infection may also have associated systemic manifestations that require treatment
- Therefore, any newborn infant who develops signs of conjunctivitis needs a prompt and comprehensive systemic and ocular evaluation to determine the
agent causing the infection and the appropriate treatment.
Onset of inflammation caused by silver nitrate drops
- Usually occurs within 6-12 hr after birth, with clearing by 24-48 hr.
.
Gonococcal conjunctivitis
Incubation period: 2-5 days

Chalmydial Conjunctivitis
Incubation period: 5-14 days

Begins with mild inflammation and a serosanguineous

discharge

Conjunctivitis caused by C. trachomatis (inclusion

blennorrhea) may vary from mild inflammation
to severe swelling of the eyelids with copious
purulent discharge.

Within 24 hr, the discharge becomes thick and

purulent, and tense edema of the eyelids with marked
chemosis occurs

The process involves mainly the tarsal

conjunctivae; the corneas are rarely affected.

Delayed treatment: the infection may spread to involve

the deeper layers of the conjunctivae and the cornea
Complications: include corneal ulceration and
perforation, iridocyclitis, anterior synechiae, and rarely
panophthalmitis

DIAGNOSIS
Evaluate for possible infectious cause: Conjuctivitis appearing after 48 hr

Conjunctivitis caused by Pseudomonas aeruginosa

is uncommon, acquired in the nursery, and a
potentially serious process
It is characterized by the appearance on days 5-18 of
edema, erythema of the lids, purulent discharge,
pannus formation, endophthalmitis, sepsis, shock,
and death

RETINOPATHY OF PREMATURITY

Gram stain of the purulent discharge

Viral: Culture media of virus isolation
Chalmydial infection: Giemsa stained epithelial cells scraped from the tarsal conjunctivae for the characteristic intracytoplasmic inclusions, by isolating the
organisms from a conjunctival swab using special tissue culture techniques, by immunofluorescent staining of conjunctival scrapings for chlamydial inclusions, or
by tests for chlamydial antigen or DNA.
is a bilateral proliferative retinopathy, occurring in premature infants with low birth weight who often have been exposed to high concentration of oxygen.
is a complex disease of the developing retinal vasculature in premature infants
It may be acute (early stages) or chronic (late stages)
Clinical manifestations range from mild (transient changes of the peripheral retina) to severe( progressive vasoproliferation, scarring, and potentially blinding retinal
detachment)
ROP includes all stages of the disease and its sequelae. Retrolental fibroplasia, the previous name for this disease, described only the cicatricial stages

PATHOGENESIS
16 wk of gestation:
- retinal angiogenesis normally proceeds from the optic disc to the periphery, reaching the outer rim of the retina (ora serrata) nasally at about 36 wk and extending
temporally by approximately 40 wk
- Injury to this process results in various pathologic and clinical changes
1.
-

Cessation of Vasculogenesis
first observation in the acute phase
Rather than a gradual transition from vascularized to avascular retina, there is an abrupt termination of the vessels, marked by a line in the retina
The line may then grow into a ridge composed of mesenchymal and endothelial cells

2.

Neovascularization: Cell division and differentiation may later resume, and vascularization of the retina may proceed
- Alternatively, there may be progression to an abnormal proliferation of vessels out of the plane of the retina, into the vitreous, and over the surface of the retina

3.

Cicatrization and traction on the retina may follow leading to retinal detachment

ETIOPATHOGENESIS
Low birth weight and decreased gestational age are now considered the primary causative factors.
Supplemental oxygen administration which was for a long time considered as the important causative factor is now considered only a risk factor
Based on the above facts, two important hypothesis are postulated to describe pathogenesis of disease:
1. Classical theory postulates that owing to exposure to high concentration of oxygen, there occurs obliteration of premature retinal vessels.
This is followed by neovascularisation and fibrous tissue proliferatiion which ultimately forms a retrolental mass.
2.

Spindle cell theory proposed recently postulates the induction of retinal and vitreal neovascularization by spindle cell insult in a premature retina.

Risk factors:
prematurity and the associated retinal immaturity at birth represent the
major factors
Oxygenation, respiratory distress, apnea, bradycardia, heart disease, infection, hypercarbia, acidosis, anemia, and the need for transfusion are thought by some to be
contributory factors.
Generally, the lower the gestational age, the lower the birthweight, and the
sicker the infant are, the greater the risk is for ROP.

Later in the course of the disease

peripheral hypoxia develops and vascular endothelial growth factors (VEGFs) are produced in the nonvascularized retina
These growth factors stimulate abnormal vasculogenesis, and neovascularization may occur. Because of poor pulmonary function, a state of relative retinal hypoxia
occurs. This causes upregulation of VEGF, which, in susceptible infants, can cause abnormal fibrovascular growth. This neovascularization may then lead to scarring and
vision loss
CLINICAL FEATURES
The condition has been divided into active ROP and cicatricial ROP

Clinically the evolution of the active ROP has been divided into five stages (Fig. 11.16):
Stage 1. It is characterised by formation of a demarcation line seen at the edge of vessels, dividing the vascular from the avascular retina.
Stage 2. The line structure of stage 1 acquires a volume to form a ridge with height and width.
Stage 3. It is characterised by a ridge with extraretinal fibrovascular proliferation into the vitreous. This stage is further subdivided into
mild, moderate and severe, depending on the amount of fibrovascular proliferation.
Stage 4a. It includes subtotal retinal detachment not involving the macula. It occurs as a result of exudation from incompetent blood vessels or traction from the fibrous
(cicatricial) tissue.
Stage 4b. It includes subtotal retinal detachment involving the macula.
Stage 5. It is marked by total retinal detachment which is always funnel-shaped

Retinal area (zones) of involvement in ROP

The retina is divided into 3 zones. The centre of the retinal map for ROP is the optic disc not the macula as in other retinal charts (Fig. 11.17).
Zone I. A circle drawn on the posterior pole, with the optic disc as the centre and twice the discmacula distance as the radius, constitutes zone I. Any ROP in this zone is usually
very severe because of a large peripheral area of avascular retina.
Zone II. A circle is drawn with the optic disc as the centre and disc to nasal ora serrata as the radius. The area between zone I and this boundary constitutes zone II.
Zone III. The temporal arc of retina left beyond the radius of zone II is zone III
Extent of involvement is denoted by the clock hours of retinal involvement in the particular zone
Plus disease
refers to presence of tortuous dilated vessels at posterior pole with any stage of ROP.
Associated with it is the engorgment and dilatation of iris vessels, which result in poor pharmacological
dilatation of pupil
Plus diseases signifies a tendency to progression.
Prethreshold disease is defined as ROP in:
Zone I, any stage, or Zone II, stage 2 with plus component, or Zone II or III, stage 3 with plus component but not reaching threshold clock hours.
Note: Pretheshold ROP needs very close observation as it can rapidly progress to threshold, which needs prompt treatment
Threshold disease
refers to stage 3 plus disease involving 5 continuous or 8 discontinuous clock hours. This stage needs laser or cryotherapy in less than 72 hours

Retrolental fibroplasias
o fibrous membrane at the back of lens; rare
o stage 5 ROP total retinal detachment
o incomplete retinal revascularization
Retinal revascularization proceeds centrifugally from optic nerve
o ophthalmic artery and vein->optic nerve-> spreads in periphery of retina
Retinal vessel reaches the nasal ora serrata at 8 months and the temporal ora serrata at 9 months.
o Complete revascularization at 9 mos
o Born at 7 months: lack of blood vessels in peripheral retina
Usually bilateral but asymmetric
o ROP develops when the processes were disturbed

International Classification of Acute ROP Stages

Location:
o Zone II and III are based on convection rather than strict anatomy
o Zone I (posterior pole): circle with radius of 30 deg., twice disc-macula distance; direct ophthalmoscopy
o Zone II: from edge of zone I to point tangential to nasal ora serratia and around to the area near the temporal equator.
o Zone III: Residual crescent anterior to Zone II (at the temporal side)
Extent
o Specified as hours of the clock as the observer looks at each eye.
Staging (using Indirect Ophthalmoscopy):
o I- Demarcation line
thin yellowish line separating vascular area from avascular area of retina, flat line, no volume
o II- Ridges, +/- small tufts or the fibrovascular proliferation
o III- Ridge w/ extraretinal fibrovascular proliferation,
formation of new blood vessels on top of the ridge, it spreads in the substance of vitreous, contraction of which causes retinal detachment.
In an area of retinal ischemia, it will secrete VEGF that will stimulate growth of new blood vessels. But these new BV would grow into the vitreous
cavity. When it regresses, it contracts, forming membranes that will pull the retina, causing tractional retinal detachment
o IV-Subtotal retinal detachment
A- does not incude macula
B- includes macula or fovea
o V- Total retinal detachment
Tortuosity
o Dilatation of blood vessels
o Too much vascular shunting
o Plus Disease
o plus (+) is added when vascular shunting is so marked that the veins are enlarged and the arteries are tortuous in the posterior pole; more severe
o Makes the prognosis worse
Major Risk Factors
Decreasing gestational age
Decreasing birth weight
Its not the absolute weight, but its relation to the gestational age.
28 weeks, 800 grams= sometimes they dont develop ROP
30 weeks, 1000 grams= develop ROP (should consider both weight and gestational age)
Supplemental oxygen the higher the 02, the higher chance of RoP.
Acidosis, apnea, patent ductus arteriosus, septicemia, blood transfusion, intraventricular hemorrhage.

Screening Recommendations
Infants birth weight of less than 1500 grams or AOG of 32 wks or less, as well as selected infants with unstable clinical course, at least 2 fundus exams.
Should be performed by an ophthalmologist with sufficient regular experience and knowledge in the examination of preterm infants
st
st
rd
1 exam should normally be performed between 4-6 weeks (after birth) of chronological age or alternatively within the 31 to 33 week of post conceptual or
postmenstrual age (AOG), whichever is later.
E.g. if patient is 24 weeks old
24 + 4-6 weeks = 28-30 weeks it should be at least 31 weeks
st
rd
follow the 31 -33 rule
Screening recommendation in the Philippines: px born at 35 weeks and below, 2000 grams and below = examine 2 weeks after birth
Aggressive Posterior Type ROP progression in a week
Treatment should generally be accomplished within 72 hours of determination of threshold ROP.
o Threshold: stage 3+ ROP in 5 contiguous clock hours or 8 cumulative clock hours in either zone I or zone II.

CONGENITAL GLAUCOMA

Treatment of ROP
Retinal photocoagulation (laser treatment) ablate (burn) the avascular area
o Avascular areait is the problem; producesVEGF which produces abnormal blood vessels (neovascularization)
o Complicationlimited peripheral vision
Retinal cryotherapy not done anymore
Vitrectomy and lensectomy may be beneficial in cicatricial disease in stage 4 & 5
Anti-VEGF injections also used in treatment ofother ca like colon ca, slows down development of new blood vessels.
Manifest at birth in 50%, diagnosed in the first 6 months in 70%, and diagnosed by the end of the first year in 80%. The earliest and most commonsymptom is epiphora.
May be associated with other congenital lesions
Often bilateral
Signs and symptoms:
o Extreme photophobia - Infants avoidopening their eyes and if they do, they would suddenly close it because they feel extreme pain upon seeing bright lights
o Epiphora or Tearing
o Corneal haze or opacity
Increased corneal diameter
A later finding. Above 11.5mm is considered significant.
o Increased intraocular pressure (>10-20 mm Hg)
Is a cardinal sign
o Buphthalmus
TREATMENT: Surgical Immediate, may cause blindness

Glaucoma: is a general term used to indicate damage to the optic nerve with visual field loss that is caused by or related to elevated pressure
within the eye
Classification of glaucoma according to:
age of the affected individual at presentation
association of other ocular or systemic conditions.
Congenital glaucoma

Juvenile glaucoma

Primary glaucoma

Secondary glaucoma

Glaucoma that begins within the 1st 3

years of life

that which begins between

the ages of 3 and 30 yr

indicates that the cause is an isolated

anomaly of
the drainage apparatus of the eye

other ocular or systemic abnormalities

are associated, even if a similar
developmental defect of the trabecular

(trabecular meshwork). More than

50% of infantile cases are primary
glaucoma.

meshwork is also present

CLINICAL MANIFESTATIONS
Classic triad
1. epiphora (tearing)
2. photophobia (sensitivity to light)
3. Blepharospasm (eyelid squeezing)
Each can be attributed to corneal irritation. Only approximately 30% of affected infants demonstrate the classic symptom complex. Signs of glaucoma include corneal edema,
corneal and ocular enlargement, and conjunctival injection
An increase in intraocular pressure (IOP): expansion of the globe, including the cornea, and the development of buphthalmos (ox eye)
Enlargement of cornea:
Breaks occur in th endothelial basement membrane which may lead to permanent corneal scarring
These breaks in Descemet membrane (Haab striae) are visible as horizontal edematous lines that cross or curve around the central cornea.
They rarely occur beyond 3 yr of age or in corneas <12 mm in diameter.
The cornea also becomes edematous and cloudy, with increased IOP.
Corneal edema
leads to tearing and photophobia.
Cupping of the optic nerve head
is detected by ocular examination
Deep, central cupping readily occurs and may regress with normalization of pressure
Treatment
Procedures used to treat glaucoma in childreninclude surgery to establish a more normal anterior chamber angle (goniotomy and trabeculectomy), to create a site for aqueous
fluid to exit the eye (trabeculectomy and seton surgery), or to reduce aqueous fluid production (cyclocryotherapy and cyclophotocoagulation)

RETINOBLASTOMA

is the most common primary malignant intraocular tumor of childhood

Fatal if untreated
o Compatible with life as long as there are no optic nerve and intracranial extensions.
90% of cases diagnosed before 3 years old.
o Most of them are actually diagnosed before age 1.
30% are bilateral
o If bilateral and multifocal usually the hereditary type
o If unilateral with one mass inside the eye usually secondary to mutation.
Due to loss of normally protective dominant allele at single locus within chromosome band 13q14.
Common signs: leukocoria and strabismus
Screen for children of families affected by retinoblastoma
o The chance of an infant having retinoblastoma if he has an older sibling with retinoblastoma is increased compared to those with no history.

Enucleation is the treatment of choice

o Used to be the treatment of choice before when it has grown too big and has no chance to be salvaged
o In advanced countries: Globe salvage is the treatment of choice
o Mean age of diagnosis in US: 15-18 months
o Mean age of dx in Philippines: later than 2 years
Other treatment modalities: Radiotherapy, photocoagulation, cryotherapy.
o With the advent of chemoreduction,enucleation is less. Usually used nowadays for smaller masses. 3 cycles of chemotherapy is given to decrease the mass.
Laser surgery is done afterwards to photocoagulate the mass.
o If mass is >1/2 of the globe, there is no choice but to remove the eyeball.
o If no recurrence at age 7, patient willmost likely have normal life expectancy.

CLINICAL MANIFESTATIONS
The clinical manifestations of retinoblastoma vary, depending on the stage at which the tumor is detected
Leukocoria
the initial sign in the majority of patients is a white pupillary reflex (leukocoria)
Leukocoria results because of the reflection of light off the white tumor.
Strabismus
The second most frequent initial sign of retinoblastoma
Less-frequent presenting signs include:
Pseudohypopyon (tumor cells layered inferiorly in front of the iris) caused by tumor seeding in the anterior chamber
of the eye
hyphema (blood layered in front of the iris) secondary to iris neovascularization
vitreous hemorrhage
signs of orbital cellulitis
On examination, the tumor appears as:
white mass, sometimes small and relatively flat, sometimes large and protuberant.
It may appear nodular. Vitreous haze or tumor seeding may be evident.
2/3 to of children with retinoblastoma have unilateral tumors, with the remainder having bilateral retinoblastoma.
Hereditary form: associated with loss of function of the RB1gene located on chromosome 13q14 and encodes the retinoblastoma protein (Rb),a tumor suppressor
protein that controls cell-cycle phase transition and has roles in apoptosis and cell differentiation.
Knudsons two-hit model of oncogenesis - two mutational events are required for retinoblastoma tumor development. In the hereditary form of retinoblastoma, the first
mutation in the RB1gene is inherited through germinal cells and a second mutation occurs subsequently in somatic retinal cells.
Imaging studies are not diagnostic, and biopsies are contraindicated. Indirect ophthalmoscopy with slit-lamp evaluation can detect retinoblastoma tumors
Occasionally, a pineal area tumor is detected in a child with hereditary retinoblastoma, a phenomenon known as trilateral retinoblastoma