https://discovery.lifemapsc.

com/library/review-of-medical-embryology/chapter-167-the-eye-optic-
cup-and-lens-vesicle-retina-iris-and-ciliary-body

During the fourth week of gestation embryo (7,6-7,8 mm), while the lens

vesicle is forming, simultaneously the optic vesicle is converted into a double layered

optic cup. It appears from that, this has happened because the developing lens has

invaginated itself into the optic vesicle. However, this is not so. The conversion of the

optic vesicle to the optic cup is due to differential growth of the walls of the vesicle.

The margin of optic cup grow over the upper and lateral sides of the lens to enclose

it. However, such a growth does not take place over the inferior part of the lens, and

therefore walls of the cup show deficiency in this part.

CHANGES IN THE ASSOCIATED MESENCHYME

The developing neural tube (from which central nervous system develops) is

surrounded by mesenchme. Mesenchyme is a loose tissue constiting of stellate,

amoeboid mesenchymall cells embedded in a matrix rich in glycosaminoglycans.

Mesenchymall cells may be derived from serosal sources, namely mesoderm

(dermatome and sclerotome component of the somite or lateral plate mesoderm) or

neural crest. Thus this descriptive term mesencyme does not imply an origin from

any particular embryonic germ layer. The mesenchyme surrounding the neural tube

subsequently condenses to form mrninges.

An extension of this mesenchyme may be derrived from the cephalic neural crest

and indeed from crest cells detaching from the outer surface of the optic vesicle

itself. Later, this mesenchyme differentiates to form a superficial fibrous layer

(correspondiing to dura), which will form the sclera and cornea and a deeper

vascular layer (corresponding to piaarachnoid) which will form stroma of uveal tissue

with the formation of optic cup, part of the inner vascular layer of mesenchyme is
carried into the cup through the choroidal fissure. With the closure of this fissure, the

portion of mesenchyme which has made its way into the eye through the fissure is

cut off from surrounding mesenchyme gives rise to hyaloid system of the vessels.

The fibrous layer of mesenchyme surrounding anterior part of optic cup forms

the cornea. The corresponding vascular layer of mesenchyme becomes

iridopupillary membrane, which, in the peripheral region, attaches to the anterior part

of the optic cup to form iris. The central part of this lamina is pupillary membrane and

also forms the tunica vasculosa lentis. In the posterior part of the optic cup, the

surrounding fibrous mesenchyme forms sclera and extraocular muscles, while the

vascular ayer forms the choroid and ciliary body.

Neurosensori retina

Inner wall of the optic cup is a singled layered epithelium with an internal and an

external basement membrane. As development proceeds, this layer proliferates and

during 4th-5th week of gestation, the primitive retina formed is arranged in two

zones: an outer primitive zone (nuclear zone or germinal epithelium) filled with eight

to nine rows of nuclei and an inner marginal zone (layer of HIS) devoid of nuclei.

Dinding bagian dalam dari optic cup adalah satu lapisan epitelium dengan membran

basalis internal dan eksternal. Seiring proses perkembangan, lapisan ini

berproliferasi. Pada minggu ke 4 sampai ke-5 kehamilan, retina primitif yang

terbentuk disusun dalam dua zona: zona primitif luar (zona nuklir atau epitel

germinal) yang diisi dengan delapan hingga sembilan baris nuklei dan zona marjinal

dalam (lapisan HIS) tanpa inti.

Retinal pigment epithelium

Cells of the outer wall of the optic cup become pigmented around 6th week of

gestation. Its posterior part forms the retinal pigment epithelium (RPE) of the retina

and the anterior part continues forward in cilliary body and iris as their pigmented

epithelium.

Initially, the RPE compires a mitotically active pseudostratified columnar ciiated

epithelium. The cilia disappear as melanogenesis commences. The mitotic activity

causes by birth, thereafter growth of eye and consequently of the RPE itself is

accomodated by hypertrophy or enlargement of existing cells. The mature RPE cells

arre hexagonal in shape, homogenous in size and in section appear as simple

cuboidal epithelium.

Crystalline lens

Lens placode and vesicle. Lens placode, the thickened area of surface ectoderm

from which the lens develops, is identifable by 27 days of gestation (embryo 4-4.5

mm). The lens placode invaginates the sinus below the surface ectoderm to form

lens vesicle, which consists of a single layer of cells covered by a basal lamina.

Primary lens fibers, the cells of the posterior wall of the lens vesicle rapidly

elongate and get filled with protein called crystallines, which make them transparent.

These elongated transparent cels are known as primary lens fibers. The nuclei of the

lens fibers are present more anteriorly within the cells to form a line convex forward

called and the nuclear bow. The primary lens fibers now become attached to the

apical surface of anterior lens epithelium and their nuclei disappear. The primary

lens fiibers are formed up to the 3rd month of gestation and are preserved as the
compaact core of the lens, known as embryonic nucleus. The posterior aspect of the

lens, therefore, becomes devoid of epithelium.

The anterior and posterior chambers and the aqueous outflow pathway

Anterior chamber arises as a slit in the mesenchyme between the surface ectoderm
and developing iris. The mesenchyme anterior to the slit forms the corneal
endothelium and the posterior to the slit forms the primary pupillary membrane.

Angle of the anterior camber is occupied by a nest of loosely organized

undifferentiated neural crest derived mesenchymal cells that are destined to develop
into the trabecular meshwork

Schlemm’s canal develops by the end of third month of gestation from the cannels
derived from mesodermal mesenchyme. Thus, the embryogical origin of trabecular
cells (neural crest derived mesenchyme) is different from that of vascular endothelial
cells of schlemm’s canal (mesodermal mesenchyme).

Posterior chamber develops as a split in the mesenchyme posterior to the

developing iris and anterior to the developing lens. The anterior and posterior
chambers communicate when the pupilary membrane disappears and the pupil
formed

STRUCTURES DERIVED FROM THE EMBRYONIC LAYERS

Based on the above description, the various structures derived from the different
embryonic layers can be summarized as below

THE ANTERIOR AND POSTERIOR CHAMBERS AND THE AQUEOD OUTFLOW

PATHWAY

Anterior chamber arises as slit in the mesenchyme between the surface ectoderm
and developing iris. The mesenchyme anterior to the slit forms the corneal
endothelium and that posterior to the slit forms the primary pupillary membrane

Angle of the anterior chamber is occupied by a nest of loosely organized

undifferentiated neural crest derived mesenchymall cells that are deestined to
develop into the trabecular meshwork

Schlemm’s canal develops by the end of third month of gestation from the cannels
derived from mesodermal mesenchyme. Thus, the embryological origin of trabecullar
cells (neural crest derived mesenchyme) is different from that of vascular endothelial
cells of schlemm’s canal (mesodermal mesenchyme)
Posterior chamber develops as split in the mesenchyme posterior to the developing
iris and anterior to the developing lens. The anterior and posterior chambers
communicate when the pipillary membrane disappears and the pupil is formed.

Cornea

a. Epithelium is formed from the surface ectoderm. At about 40 days of gestation

(embryo 17-18mm), corneal epithelium consist of a superficial aquamous cell
layer and a basal cuboidal epithelial cell layer. By the time the eyelids oper at
5-6 months of gestation, the corneal epithelium attains an almost adult
appearance.
b. Endothelium and descemet’s membrane are formed from mesenchymal cells
derived from neural crest, which are situated at the margins of the rim of the
optic cup. These cells migrate into the developing eye beneath the basal
lamina of the corneal epithelium and form the primordial corneal endothelium.
At about 40 days gestation (17-18 mm embryo), the corneal endothelium
consists of two layers of flattened cells. By the third month (embry 63 mm),
the endothelium in the central region of cornea becomes a asingle layer of
flattened cells that rest on their intterupted basal lamina – the future
descemet’s membrane. The apices of the endothelial cells are joined by
zonulae occludentes in the middle of the 4th month, which corresponds to the
production of aqueous humour by the ciliary processes. At the 6th month of
gestation. Descemet’s membrane is demarcated
c. Stroma and bowman’s layer are derived from the mesenchymal cells that
insinuate between the surface ectoderm and the developing lens. Primary
corneal stroma is secreted by basal layer of epithelium and consists of fine
filaments, amorphous material and only a few collagen fibrils. At about 22-
24mm stage (7th week), the mesenchymal cells migrate into the primary
corneal stroma (between epithelium and endothelium) and contribute to the
further development of corneal stroma. The invading mesenchymal cells
differintiate into stromal fibroblasts or keratocytes that actively secrete the
type I collagen fibrils and the matrix of mature (secondary) corneal stroma.
Bowman’s layer starts forming by condensation of most superficial acellular
part of corneal stroma after 4 months of gestation and is fully developed at
birth. By 5 months, corneal nerves as present. It is important to note that the
fetal cornea is very hydrated compared to the adult and form and is, therefore,
translucent rather than transparent.

Sclera

Sclera is developed from the mesenchymal cells surrounding the optic cup
(corresponding to dura of CNS). The mesenchymal cells are derived ainly from the
neural crest. The process starts at the limbal equatorial region (future site of
extraocular muscle insertion), around 7th week of gestation and is completed by 5th
month
Choroid

Its mainlly derived from inner vascular layer of the mesenchyme that surrounds the
optic cup. Melanocytes of choroid originate from the neural crest

Ciliary body

a. Bothe epithelial layers of ciliary body develop from the anterior part of two
layers of optic cup (neuroectoderm). The ciliary epithelium undergoes a
convulating or folding movements to form about 70-75 ciliary process
b. Stromas of ciliary body, ciliary muscle and blood vessels are developed from
the vascular layer of mesenchyme surrounding the optic cup

Iris

a. Both layers of epithelium are derived from the marginal region of optic cup
(neuroectoderm)
b. Sphincter and dilator pupillae muscles are derived from the anterior epithelium
(neuroektoderm)
c. Stroma and blood vessels develop from vascular layer of mesenchyme
present anterior to the optic cup. Towards the end of gestation, the central iris
stroma (pupillary membrane) disappears forming the pupil. Sometimes a few
strands of this tissue are left as presistent pupillary membrane.

Vitreous

a. Primary or primitive vitreous is mesenchymal in origin and is a vascular

structure having the hyaloid system of vessels. It is present in the first month
of gestation. Surface ectodermally derived elements that surround the lens
during invagination are also thought to contribute to the primary vitreous. Thus
the primary vitreous may be of mixed ectodermal and mesenchymal origin
b. Definitive or secondary or vitreous proper is secreted by neuroectoderm of
optic cup from 2nd month of gestation onwards. This is an avascular
structure, basically an extracellular matrix, consisting mainly of a compact
network of type II collagen fibrils and primitive hyalocytes. The precise origin
of hyalocytes is presumed to be from the phagocytic monocytes of the primary
vitreous. The content of hyaluronic acid is very low during the prenatal period,
but increases after birth. When this vitreous fills the cavity by 5th to 6th month
of gestation, primitive vitreous is reduced to a smallcentral space, Cloquet’s
canal, which courses between the optic nerve head and the posterior surface
of the lens.
c. Tertiary vitreous is developed from neuroectoderm in the ciliary region during
4th month of gestation and is represented by the vitreous base and ciliary
zonules
DEVELOPMENT OF ACCESORY OCULAR STRUCTURE

Eyelids

Eyelids are formed reduplication of surface ectoderm above and below the cornea
during 2nd month of gestatio. The folds enlarge and their margins meet and fuse
with each other. The lids cut off a space called conjunctival sac. The folds thus
formed contain some mesoderm which would form the muscles of the lid and the
tarsal plate. The lids separate after seventh month of intrauterine life

 Tarsal glands are formed by ingrowth of a regular row of solid columns of

ectodermal cells from lid margins
 Ciliary glands are outhgrowths from ciliary follicles
 Cilia develop as epithelial buds from lid margins

Conjunctiva

It develops from the ectoderm lining of the lids and covering the globe

 Conjunctiva glands develop as growth of the basal cells of upper conjunctival

fornix. Fewer glands develop from the lower fornix

Lacrimal apparatus

Lacrimal gland is formed from about 8 cuneiform epithelial buds which grow by the
end of 2nd month of fetal life from the superolateral side of the conjunctival sac

Lacrimal sac, nasolacrimal duct and canaliculi

These structure develop from the ectoderm of nasolacrimal furrow. It extends from
the medial angle of the eye to the region of developing mouth. The ectoderm gets
burried to form a solid cord. The cord is later canalised. The upper part forms the
lacrimal sac. The nasolacrimal duct is derived from the lower part as it forms a
secondary connection with the nasal cavity. Some ectodermal buds arise from the
medial margins of eyelids. These buds later canalise to form the canaliculi. The
lower lacrimal canaliculus, as it extends laterally, cuts off part of the eyelid with its
components which forms caruncle and plica semilunaris.

Extraocular muscles

The extraocular muscles are some of the few periocular tissue that have been shown
not to be of neural crest origin. Recently, they are thought to differentiate in situ from
the mesodermal derived mesenchymal tissue.

The four rectus muscles and the superior and inferior oblique muscles differentiate
from the mesenchyme in the region of developing eyeball (prechordal mesenchyme).
Originally represented as a single mass of mesenchyme, they later separate into
distinct muscles, first at their insertions and later still at their origins. The extraocular
muscle appear in approximately the following sequences: lateral rectus, superior
rectus and levator palpebral superioris (week 5), superior oblique and medial rectus
(week 6), followed by inferior oblique and inferior rectus (commond primordium).

During development, the extraocular muscles become associated with the axons of
the general somatic efferent neurons of cranial nerves III, IV and VI, which innervate
these muscles.

Orbit

The orbit develops around the eyeball. It is derived above from the mesenchyme that
encircles the optic vesicle, below and laterally from the maxillary processes, medially
by the frontonasal process and behind by the pre and orbitosphenoid. The orbital
bones are formed in the membrane except those belongin to the base of skull, which
develop in the cartilage. These bones differentiate during the 3rd month and later
undergo ossificiation, initially, the optic axes are directed laterally toward the side of
head; only later are they directed anteriorly. At birth, orbit is hemispherical. Its growth
corresponds with the growth of the eyeball. Although, the eyeball reaches the adult
size by 3 years of age, the orbit undergoes considerable alterations in shape and
grows progressively until puberty.

PERKEMBANGAN VISUS

kemampuan bayi untuk melihat dapat dinilai dengan mengukur ketajaman

visual dan sensitivitas kontras. perkembangan ketajaman visual dan sensitivitas
kontras pada bayi telah diteliti menggunakan teknik perilaku dan elektrofisiologi.

the electrophysiological method of evaluating visual acuity uses the visal

evoked potential (VEP). The VEP essentially tests the integrity of the visual pathway
from the retina to the cortex by recording brain activity through electrodes placed on
the scalp. The stimulus used is a pattern reversal checkerboard or grating and the
recorded transient response is time synchronised to the stimulation.

metode elektrofisiologi mengevaluasi ketajaman visual menggunakan potensi

Visual Evoked Potential (VEP). VEP pada dasarnya menguji integritas jalur visual
dari retina ke korteks dengan merekam aktivitas otak melalui elektroda yang
ditempatkan di kulit kepala.

In studies of acuity, the amplitudes of the VEP to stimuli of reducing size are
recorded. An estimation of visual acuity can then be made by extrapolating the
amplitude to zero as a function of the spatial frequency.

Dalam studi tentang ketajaman, amplitudo VEP terhadap rangsangan dicatat.

Estimasi ketajaman visual kemudian dapat dibuat dengan mengekstrapolasikan
amplitudo ke nol sebagai fungsi frekuensi spasial.
 The spatial frequency at which no VEP is recordable is the estimated visual
acuity. A variation of this, the sweep VEP technique, has also been developed
further to determine visual acuity in infants. In this technique. A rapidly reversing
stimulus is presented over a range, or sweep, of spartial frequencies and the steady
state response is recorded continually over the range. The visual acuity is then
estimated as the spatial frequency at which the VEP amplitude is zero.

Frekuensi spasial di mana tidak ada VEP yang dapat direkam adalah perkiraan
ketajaman visual. Variasi dari teknik sweep VEP ini, juga telah dikembangkan lebih
lanjut untuk menentukan ketajaman visual pada bayi. Dalam teknik ini. Ketajaman
visual kemudian diperkirakan sebagai frekuensi spasial di mana amplitudo VEP
adalah nol.

For behavioural estimation of visual acuity the technique of prefential looking (PL)
has been employed widely. This technique is based on the observation that an infant
would rather look at a pattern than a blank stimulus. An infant demonstates no
fixation preferennce when a blank field and the grating pattern is too small to be
resolved, which gives the estimate of visual acuity. Although these PL techniques
are dependent on the examiner’s ablility and the child’s attention, their repeatability
and robustness has been demonstrated. It is this technique that has been utilised to
develop, for clinical use, the teller and keeler acuity cards and the cardiff acuity cards
for slightly older children

Visual acuity is relatively poor at birth and undergoes rapid maturation within the first
year of life. There then appears to be a slow fine-tuning to reach adult levels by 3
years of age. Possible limiting factors on infant visual acuity may be optical or neural,
so development of these subssequently improves the transmission of spatial
information. The basic optical quality of the infant eye is good. With no evidence of
markedly greater aberration than in the adult eye, and hence this can not be
considered a major contributing factor.

Kemampuan bayi untuk melihat dapat dinilai dengan mengukur ketajaman visual

dan sensitivitas kontras. perkembangan ketajaman visual dan sensitivitas kontras

pada bayi telah diteliti menggunakan teknik perilaku dan elektrofisiologi. metode

elektrofisiologi mengevaluasi ketajaman visual menggunakan potensi Visual Evoked

Potential (VEP). VEP pada dasarnya menguji integritas jalur visual dari retina ke

korteks dengan merekam aktivitas otak melalui elektroda yang ditempatkan di kulit

kepala.
Orientation detection

Newborns can detect changes in the orientation of a stimulus, but show a preference
for horizontal rather than vertical gratings

Sensitivity to oblique orientation is not found until 6 week of age, and older infants
show slightly better acuity for vertical and horizontal gratings than for oblique
gratings. This is also true for adults, so this variation in acuity may be a response to
the orientation information within our environment, as we are exposed to more
horizontal and vertical information than oblique

Deteksi orientasi

Bayi baru lahir dapat mendeteksi perubahan dalam stimulus orientasi, tetapi
menunjukkan preferensi untuk grating horizontal daripada vertikal.

Sensitivitas terhadap orientasi oblique tidak ditemukan sampai usia 6 minggu, dan
bayi yang lebih tua menunjukkan ketajaman yang sedikit lebih baik untuk grating
vertikal dan horizontal daripada untuk grating oblique. Ini juga berlaku untuk orang
dewasa, jadi variasi dalam ketajaman ini mungkin merupakan respon terhadap
informasi orientasi dalam lingkungan kita, karena kita dihadapkan pada informasi
horizontal dan vertikal daripada oblique.

Motion detection

Infants under 2 months of age have poor sensitivity to motion of all velocities. VEP
responses to low velocity stimuli first appear at 10 weeks of age, with responses to
speeds four times as fast evident a 13 weeks

Deteksi gerakan

Bayi di bawah usia 2 bulan memiliki sensitivitas yang buruk terhadap gerak. Gerakan
(low velocity) pertama kali muncul pada usia 10 minggu dan respon terhadap
kecepatan empat kali lebih cepat pada usia bayi 13 minggu.

Eye movements

Normal movements of the eyes indicates normal visual development: visually

impaired infants demonstrate abnormal movements as a first sign. An infant is able
to demonstrate a range of eye movements. Although these are less sophisticated
than those of an adult. Depending on their level of interest. Infants can fixate on
stationary objects. Follow a moving target or move their eyes towards a stimulus in
the peripheral field

Gerakan mata

Gerakan mata yang normal menunjukkan perkembangan visual yang normal: tanda
pertama bayi dengan gangguan penglihatan adalah bayi yang menunjukkan gerakan
abnormal. Seorang bayi mampu menunjukkan berbagai gerakan mata meskipun
kurang sempurna seperti orang dewasa.

Saccades

While adults use small corrective eye movements of varying size to maintain fixation,
the newborn infant can only direct its eyes to a fixation target by a series of small
sacades that appear to be of a standard size

Saccade

Sementara orang dewasa menggunakan gerakan mata korektif kecil dengan

berbagai ukuran untuk mempertahankan fiksasi, bayi yang baru lahir hanya dapat
mengarahkan matanya ke target fiksasi oleh serangkaian kantung kecil yang
tampaknya menjadi ukuran standar.

An adult can usually achieve accurate fixation with one saccade, but while the
infant’s initial saccade towards the target is usually in the correct direction. It only
covers a fraction of the distance. The infant takes longer to reach the target and also
longer to initiate the first saccade. The ability to control the nerve impulses
accurately enough to vary the size of the sacades undergoes marked development
during the first 3 months of life, but efficient adult like saccades are not found until
the fifth month of life. Horizontal saccadic eye movements develop before vertical
eye movements, which are not seen until 4-6 weeks.

Orang dewasa biasanya dapat mencapai fiksasi akurat dengan satu saccade, tetapi
sementara saccade awal bayi menuju target biasanya dalam arah yang benar. Ini
hanya mencakup sebagian kecil dari jarak. Bayi membutuhkan waktu lebih lama
untuk mencapai target dan juga lebih lama untuk memulai saccade pertama.
Kemampuan untuk mengendalikan impuls saraf cukup akurat untuk memvariasikan
ukuran sakade mengalami perkembangan yang ditandai selama 3 bulan pertama
kehidupan, tetapi orang dewasa yang efisien seperti saccade tidak ditemukan
sampai bulan kelima kehidupan. Gerakan mata saccadic horizontal berkembang
sebelum gerakan mata vertikal, yang tidak terlihat sampai 4-6 minggu.
Smooth pursuit

Smooth pursuit movements were not generally though to be present untiil 2 months
of age. With the infant dependent on using a series of small saccades prior to that.
Studies have demonstrated that this is not the case and newborns can produce
smooth eye movements under certain conditions.

The target velocity must be low enough and with target of 12o or more, otherwise the
infant tracking breaks down into a series of saccades. The maximum speed at which
the moving target can be followed by smooth pursuit increases with age. By 10
weeks of age the ability to follow a moving target with reasonable accuracy has
developed

Gerakan smooth pursuit umumnya tidak ada hingga usia 2 bulan oleh karena
sebelumnya bayi tergantung pada gerakan sakadik. lahir dapat menghasilkan
gerakan smooth pursuit dalam kondisi tertentu. Gerakan smooth pursuit meningkat
seiring bertambahnya usia. Pada usia 10 minggu, kemampuan untuk mengikuti
target dengan akurasi yang wajar telah berkembang.

Optokinetic nystagmus

The slow and fast phases of optokinetic nystagmus (OKN) can be induced visually in
infants from birth, but an immaturity can be demonstrated in monocular OKN. In
monocular OKN there is an asymetry in direction of the OKN such that in infants
under 2 months of age it can be elicited only in a temporal to nasal direction.
Symmetry for both directions is not achieved until 5 months of age. Persistence of an
asymmetric response beyond this age indicated a problem in visual development

Nystagmus Optokinetik

Fase lambat dan cepat dari nistagmus optokinetik (OKN) dapat diinduksi secara
visual pada bayi sejak lahir, tetapi ketidakmatangan dapat ditunjukkan dalam
nistagmus optokinetik monokuler. Dalam nistagmus optokinetik monokuler ada
asimetri dalam arah dari nistagmus, sehingga pada bayi di bawah 2 bulan nistagmus
monokuler hanya dalam arah temporal ke hidung. Simetri untuk kedua arah tidak
tercapai sampai usia 5 bulan. Respon asimetris yang persisten di luar usia ini
menunjukkan masalah dalam perkembangan visual.
Vestibulo-ocular reflex

When a target is presented unexpectedly in the visual field, sacccadic eye

movements align the eye with the target and the head rotates towards the target. As
the head rotates, the semicircular canals of the vestibular system sense a movement
and initiate a reflex rotation of the eye in the opposite direction to maintain fixation.
This is the vestibulo-ocular reflex and is present at birth.

Refleks vestibulo-okular

Ketika target disajikan secara tak terduga di lapang visual, gerakan mata sacadic
menyelaraskan dan kepala berputar ke arah target. Ketika kepala berputar, kanalis
semisirkularis dari sistem vestibular merasakan gerakan dan memulai rotasi refleks
mata ke arah yang berlawanan untuk mempertahankan fiksasi. Ini adalah refleks
vestibulo-okular dan hadir saat lahir.

Accomodation

Early studies of accomodation by dynamic retinoscopy suggested that newborns had

a fixed focus and no acommodation. More recent studies have shown that, for
resolvable images, adult levels of accuracy in accommodation are present by 2-3
months, with newborns achieving accurate focus over distances of less than 75 cm

The accomodative inaccuraciesat 1-3 months are actually within the estimated depth
of focus of the visual system. This depth of focus is greater in infants because of the
smaller eye and pupil, and hence it appears that an infant has less sensory stimulus
to control accommodation accurately

Akomodasi

Studi awal akomodasi oleh retinoscopy dinamis menyarankan bahwa bayi yang baru
lahir memiliki fokus tetap dan tidak ada akomodasinya. Penelitian yang lebih baru
telah menunjukkan bahwa, untuk gambar yang dapat dipecahkan, tingkat akurasi
orang dewasa dalam akomodasi hadir selama 2-3 bulan, dengan bayi yang baru
lahir mencapai fokus yang akurat pada jarak kurang dari 75 cm

Ketidakakuratan akomodatif dalam 1-3 bulan sebenarnya berada dalam perkiraan

kedalaman fokus sistem visual. Kedalaman fokus ini lebih besar pada bayi karena
mata yang lebih kecil dan pupil, dan karenanya tampak bahwa bayi memiliki stimulus
sensorik yang lebih sedikit untuk mengontrol akomodasi secara akurat.
Convergence

There are two types of vergence, that driven by blur (accommodative) and that
driven by diplopia (fusional). Accomodative convergence to near target is present by
1 monthof age, but improves in precision by 2 months, and improves in accuracy
with age

Both vergence mechanism are involved in covergence, accommodative and disparity

detection, and involve pathways between the cortex and the oculomotor centres.
Immaturity in the pathways with in the visual system underlies poor perfomance in
the task.

Konvergen

Ada dua jenis vergen, akomodatif dan fusional. Konvergen akomodatif untuk target
dekat hadir saat usia 1 bulan, meningkat pada usia 2 bulan dan seperti dewasa pada
usia 7 bulan.

Binocular function

The development of binocular function has been assessed widely. Binocular function
can be classified into three levels, bifoveal fixation. Fusion and stereopsis. The
presence of these functions can be used to describe binocular function, but they are
not present at birth. Establishing their presence can provide useful information as to
development of binocular function.

Bifoveal fixation is prerequisite for binocular function. Inaccurate alignment of the

eyes results in degradation of stereopsis and fusion. Bifoveal fixation can only occur
in a state of orthoporia, which most normal infants achieve between 3 and 6 months
of age

Fungsi binokular

Fungsi binokular dapat diklasifikasikan menjadi tiga tingkat, fiksasi bifoveal, fusi dan
stereopsis. Kehadiran fungsi-fungsi ini dapat digunakan untuk menggambarkan
fungsi binokular, tetapi mereka tidak ada saat lahir.

Fiksasi bifoveal merupakan prasyarat untuk fungsi binokular. Mata yang tidak akurat
menghasilkan degradasi stereopsis dan fusi. Fiksasi bifoveal hanya dapat terjadi
dalam keadaan ortoporia yang didapat pada usia bayi normal yaitu antara 3 dan 6
bulan
Stereopsis

Stereopsis relates to the visual system’s ability to process information abouth depth
perception as a consequence of simultaneus, but slightly disparate, images
presented to the two eyes

Studies of stereopsis using a variety oof techniques give comparable results.

Behavioural responses to random dot and line stereogram techniques, VEP
techniques and eye movement analysis have all been used to investigate the
development of stereopsis.

Stereopsis berhubungan dengan kemampuan sistem visual untuk memproses

informasi tentang kedalaman persepsi sebagai konsekuensi simultan, tetapi sedikit
berbeda, gambar disajikan kepada dua mata

Studi stereopsis menggunakan berbagai teknik memberikan hasil yang sebanding.

Respon perilaku terhadap teknik dot dot dan garis stereogram secara acak, teknik
VEP dan analisis gerakan mata semuanya telah digunakan untuk menyelidiki
perkembangan stereopsis.

Consistent findings across all techniques place the development of stereopsis

beetween 2 and 6 months. Infants have binocular visually evoked responses to
random dot stereograms at 3-4 months

Assessed stereopsis development with uncrossed (behind the plane of fixation) and
crossed (in front of the plane of fixation) disparities and showed the crossed
emerged earlier (at 12 weeks) the development of stereopsis appears to be almost
entirely complete by 6 months, but this appears to take slightly longer when asessed
by behavioural methods. Once stereopsis emerges, the time course for improvement
in stereoacuicity is very rapid, and reaches adult levels of about 1 minute of are
within 5 weeks onset.

Perkembangan stereopsis terjadi antara 2 dan 6 bulan. Bayi memiliki respon

binokular visual terhadap stereogram pada usia 3-4 bulan.

Perkembangan stereopsis dengan uncrossed (di belakang bidang fiksasi) dan

crossed (di depan bidang fiksasi) dibedakan dan crossed muncul lebih awal (pada
12 minggu) perkembangan stereopsis tampaknya hampir seluruhnya selesai pada
usia 6 bulan.
Fusion

Fusion relates to the visual system’s ability to combine similar and perhaps non
identical information from two eyes into one image. Fusion development has been
investigated using both PL and VEP techniques, and is seen to develop over a
similar time course as that of stereopsis. No fusion can be demonstrated consistently
by 6 months. This parallel development of stereopsis and fusion is thought to be
function of the development of the visual cortex

Fusi

Fusi merupakan kemampuan sistem visual untuk menggabungkan informasi dari

dua mata menjadi satu gambar. Perkembangan fusi telah diselidiki menggunakan
teknik PL dan VEP, dan terlihat berkembang seiring waktu yang sama seperti
stereopsis. Tidak ada fusi yang dapat ditunjukkan secara konsisten selama 6 bulan.
Perkembangan stereopsis dan fusi yang paralel ini dianggap sebagai fungsi
perkembangan korteks visual

Colour vision

The emergence of colour vision between the ages of 1 and 3 months has been
demonstrated using PL and VEP Techniques. Infants as young as 2 months of age
can discriminate some wavelengths in the absence of luminance information and
develop trichromacy by 3 months.

Visi warna

Munculnya penglihatan warna terjadi antara usia 1 dan 3 bulan telah diteliti
menggunakan teknik PL dan VEP. Bayi usia 2 bulan dapat membedakan beberapa
panjang gelombang (dichromat) dan trichromat pada usia 3 bulan.
in infants and toddlers, fixation behavior is observed to qualitatively assess
vision. fixation and following (tracking) behavior is observed as the child's
attention is directed to the examiner's face or a small toy in the examiner's
hand. fixation preference is determined by observing a child's response to
covering 1 eye compared with covering the other. children typically resist
occlusion of the eye with better vision. it is also important to determine
whether each eye can maintain fixation through smooth pursuit or a blink;
strong fixation preference for 1 eye indicates decreased vision in nonpreferred
eye.

Pada bayi dan balita, fiksasi diamati untuk menilai visus secara kualitatif. Fixation
and following diamati dengan cara perhatian anak diarahkan ke wajah pemeriksa
atau mainan kecil di tangan pemeriksa. Preferensi fiksasi ditentukan dengan
mengamati respon seorang anak saat menutupi 1 mata dan dibandingkan dengan
saat menutupi mata yang lain. Penting bagi pemeriksaan ini untuk menentukan
apakah setiap mata dapat mempertahankan fiksasi dengan melakukan smooth
pursuit atau kedipan; Preferensi fiksasi kuat pada 1 mata menunjukkan penurunan
penglihatan pada mata yang yang lain.

visual acuity assessment requires different approaches depending on the age

and cooperativeness of the child. ideally, measurements at distance with
crowded snellen or sloan letter optotypes guide amblyopia diagnosis and
management. commonly, however, the child suspected of having amblyopia is
preverbal, preliterate, or not fully cooperative. in such cases, clinical options
include assessing fixation behavior and using preliterate eye charts. tablle 1-1
lists the expected acuity for various test a different ages. copies of whatever
optotypes are appropriate for the child (eg. allen cards, LEA symbols) can be
given to the parent for at home rehearsal to help distinguish not seeing the
test object from not understanding the test.

penilaian ketajaman visual membutuhkan pendekatan yang berbeda tergantung

pada usia dan kegotong-royongan anak. idealnya, pengukuran pada jarak jauh
dengan snellen yang penuh sesak atau optotip surat surut memandu diagnosis dan
manajemen amblyopia. umumnya, bagaimanapun, anak yang dicurigai memiliki
amblyopia adalah preverbal, preliterate, atau tidak sepenuhnya kooperatif. dalam
kasus seperti itu, pilihan klinis termasuk menilai perilaku fiksasi dan menggunakan
grafik mata preliterate. tablle 1-1 mendaftar ketajaman yang diharapkan untuk
berbagai tes pada usia yang berbeda. salinan optotip apa pun yang sesuai untuk
anak (mis. kartu allen, simbol LEA) dapat diberikan kepada orang tua untuk di rumah
latihan untuk membantu membedakan tidak melihat objek tes dari tidak memahami
tes.
various optotypes are available for testing preliterate children. LEA symbols
and the HOTV test are reliable for preschool-aged children. testability may be
improved by having a shy child point and match rather than verbalize the
optotypes. other optotypes such as the allen figures do not conform to
accepted parameters of optotype design and therefore provide less accurate
results. most pediatric opthalmologists consider letter (sloan or snellen) acuity
optotypes the most accurate, followed in decreasing order of accuracy by hotv
and lea symbols, the tumbling E, Allen figure, and fixation behavior. the
examiner should use the most sophisticated test that the child can perform

Berbagai optotypes tersedia untuk menguji anak-anak yang belum memiliki

kemampuan untuk membaca. Simbol LEA dan tes HOTV dapat digunakan untuk
anak usia prasekolah. Pada anak yang pemalu pemeriksaan dapat dilakukan
dengan cara menunjuk gambar yang sama dibandingkan anak tersebut harus
berbicara. Kebanyakan dokter mata mempertimbangkan bahwa pemeriksaan
dengan huruf (Sloan atau Snellen) adalah optotypes yang paling akurat.

most parent and many doctors doo not expect the newborn baby to see well,
so it is only when the child is not fixing and following by 2-4 months of age
that they are referred by the parents themselves, or their advisors, to the
ophthalmologist or pediatrician. the diagnosis of delayed visual maturation is
really done retrospectively, and by exclusion of visual system disease as far
as that is possible. it is essential for the diagnosis that the vision should
improve with time, but since delayed visual maturation may coexist eith ocular
or systemic disease, the eventual vision is not necessarily normal. it is
noteworthy that the patient who presents with delayed visual maturation in its
isolated form is the child with no apparent fixation and following reflexes, and
no strabismus. the patient thus appears distinctly different than the infant who
presents with poor visual function associated with a bilateral anterior visual
pathway disorderinwhich nystagmus is to be expected and in whom pupillary
abnormalities may be present. delayed visual maturation must be
distinguished from those infants who present with poor visual function as a
result of visual cortex or associated neurovisual pathway pathology. these
patients too may present with poor visual fixation and no nystagmus

Kebanyakan orang tua dan dokter tidak mengetahui bayi yang baru lahir untuk
melihat dengan baik, sehingga hanya ketika anak tidak memperbaiki dan mengikuti
dengan usia 2-4 bulan yang mereka dirujuk oleh orang tua sendiri, atau penasehat
mereka, ke dokter spesialis mata atau dokter anak. diagnosis kematangan visual
yang tertunda benar-benar dilakukan secara retrospektif, dan dengan pengecualian
penyakit sistem visual sejauh mungkin.

Penting untuk mengetahui bahwa penglihatan harus membaik seiring berjalannya

waktu, tetapi karena kematangan visual yang tertunda ataupun penyakit mata
lainnya, penglihatan akhirnya tidak selalu normal.
normal visual fixatiion and tracking usually develop in infants within the first 3
months of life. if this does not occur, the condition is referred to as delayed
visual maturation (DVM), or cortical inattention. ophthalmologic and systemic
examination of infants with DVM usually reveals a cause. they are 3 subgroups
of infants with DVM: otherwise healthy infants, infants with systemic/
neurologic abnormalities, and infants with structural eye anomalies

fiksasi visual normal berkembang pada bayi di 3 bulan pertama kehidupan. jika ini
tidak terjadi, kondisi ini disebut sebagai kematangan visual yang tertunda (DVM),
atau kurangnya perhatian kortikal. pemeriksaan oftalmologi dan pemeriksaan
sistemik pada bayi dengan DVM biasanya mengungkapkan penyebabnya. Ada 3
subkelompok bayi dengan DVM: bayi sehat, bayi dengan kelainan sistemik /
neurologis, dan bayi dengan kelainan struktural mata.

in an otherwise healthy infant with dvm, the following findings suggest a good
visual and neurologic prognosis : some reaction to light, normal pupillary
responses, no nystagmus, and normal ocular structures. if the visual behavior
does not progress toward normal by 4-6 months of agee, further investigation
(neuroimaging or electrophysiologic testing) is warranted

Pada bayi sehat dengan DVM, menunjukkan prognosis visual dan neurologik yang
baik: beberapa bereaksi dan merespon cahaya, respon pupil normal, tidak ada
nistagmus, dan struktur okular normal. jika perilaku visual tidak berkembang, akan
menuju normal pada usia 4-6 bulan, tetapi harus ada pemeriksaan yang lebih lanjut.

pregeniculate visual impairment denotes visual deficits resulting from

pathology anterior to the lateral geniculate nucleus (the pregeniculate visual
pathways). congenital sensory nystagmus may be a clinical indicator of
bilateral pregeniculate visual impairment. strabismus is commonly seen with
these disorders. cause of pregeniculate visual impairment in infants include
corneal and other anterior segment abnormalities, glaucoma, retinal disorders
and optic nerve abnormalities

Gangguan visual pregeniculate menunjukkan defisit visual yang dihasilkan dari

patologi anterior ke lateral nukleus geniculate (jalur visual pregeniculate). Nistagmus
kongenital mungkin merupakan indikator klinis dari gangguan visual pregeniculate
bilateral. Strabismus juga sering terlihat pada gangguan ini. Penyebab lain
gangguan penglihatan pregeniculate pada bayi termasuk kelainan kornea dan
kelainan segmen anterior lainnya seperti glaukoma, gangguan retina dan kelainan
saraf optik.
optic nerve abnormalities are frequent causes of pre geniculate visual
impairment. the most common of these is optic nerve hypoplasia, which may
be associated with central nervous system anomalies such as septo-optic
dysplasia and schizencephaly. other optic nerve disorders are optic atrophy,
morning glory disc anomaly, optic disc colobma, and staphyloma. causes of
optic nerve atrophy include hydrocephaluss, brain tumors, trauma, hypoxic-
ischemic injury, metabolic storage diseases, and inherited optic neuropathies
such as behr optic atrophy

kelainan saraf optik sering menyebabkan gangguan penglihatan pregeniculate. yang

paling umum adalah hipoplasia saraf optik, yang mungkin berhubungan dengan
anomali sistem saraf pusat seperti displasia septo-optik dan schizencephaly.
gangguan saraf optik lainnya adalah atrofi optik, morning glory disc anomaly,
colobma diskus optik, dan staphyloma. penyebab atrofi saraf optik termasuk
hidrosefalus, tumor otak, trauma, cedera hipoksik-iskemik, penyakit penyimpanan
metabolik, dan neuropati optik yang diturunkan seperti atrofik optik penglihatan

babies who are very premature, who have severe intercurrent ilness early in
their life, may present with delay in visual development, but this usually
improves in the same way as in group I patients, with residuual defects only
related to their ilness. most patients in this group in this group have severe
mental retardation. it is most frequently seen in children who have infantile
spams, or other seizure disorders in relationship to severe birth asphxia,
hypoglicemia, hypocalcemia, tuberous sclerosis, aicardi syndrome, and so on.
in most cases, these are diagnostic clues to the underlying cause and the
neurophysiological (EEG). vision appears to imrpove with the control of
seizures in these children. children with other causes of mental retardation
without seizures, such as hydrocephalus or brain malformations, may also
exhibit delayed visual maturation often to a lesser degree. the vision is
variable and may be stimulated or excited by sound as well as visual
stimulation
Retrogeniculate visual impairment, or cerebral visual impairment

Cerebral visual impairment (CVI; also termed retrogeniculate visual

impairment) is the most frequent cause of childhood visual impairment in
develop countries. It denotes visual deficits resulting from pathology posterior
to the lateral geniculate nucleus (the retrogeniculate visual pathways). CVI is
also often referred to as cortical visual impairment, but the former term is
preferred because subcortical visual impairment causes vision problems that
are difficult to differentiate from those due to cortical visual impairment. The
pathology may involve the optic radiations as well as the occipital cortex.
Depending on the etiology, the visual impairment can be transient or
permanent and can be an isolated finding or associated with multiple
neurologic deficits.

Gangguan penglihatan retrogeniculate, atau gangguan penglihatan cerebral

Gangguan penglihatan cerebral/ Cerebral Visual Impairment (CVI; juga disebut

gangguan visual retrogeniculate) adalah penyebab gangguan penglihatan masa
kanak-kanak yang paling sering di negara berkembang. Defisit visual dapat
dihasilkan dari patologi posterior ke nukleus geniculate lateral (jalur visual
retrogeniculate). CVI juga sering disebut sebagai gangguan penglihatan kortikal.
Patologi mungkin melibatkan radiasi optik serta korteks oksipital. Tergantung pada
etiologi, gangguan penglihatan dapat bersifat sementara atau permanen dan dapat
terkait dengan beberapa defisit neurologis.

The causes of CVI can be congenital or acquired. Prenatal and perinatal

causes include periventricular leukomalacia (a prominent cause of visual
impairment in premature children), intrauterine infection, hypoxia, intracranial
hemorrhage, structural central nervous system abnormalities, seizures, and
hydrocephalus. Acquired causes include accidental trauma, abusive head
trauma, meningitis and encephalitis

Penyebab CVI bisa bawaan atau didapat. Penyebab prenatal yaitu leukomalasia
periventrikel (penyebab utama gangguan penglihatan pada anak prematur), infeksi
intrauterin, hipoksia, perdarahan intrakranial, kelainan sistem saraf pusat struktural,
kejang, dan hidrosefalus. Penyebab yang didapat yaitu trauma yang tidak disengaja,
trauma kepala yang berat, meningitis dan encephalitis
Infants with CVI show varying degrees of visual inattentiveness. Both the
family and the ophthalmologist may be uncertain as to whether the baby can
see. Examination reveals normal ocular structures, normal pupillary
responses, and variable levels of visual fixation, from midly decreased to
roving eye movements. Nystagmus is typically not present. Descending optic
atrophy (from transsynaptic degeneration) may coexist. In preterm infants,
optic disc cupping resembling that seen in glaucoma can occur as a result of
transsynaptic degeneration, most commonly secondary to periventricular
leukomalacia.

Bayi dengan CVI menunjukkan variasi tingkat ketidaksadaran visual. Baik keluarga
maupun dokter mata mungkin tidak yakin apakah bayi dapat melihat. Pemeriksaan
menunjukkan struktur okular normal, respon pupil normal, dan tingkat fiksasi visual
yang bervariasi. Nistagmus biasanya tidak ada. Pada bayi prematur, cup disc optik
menyerupai cup disc glaukoma.

Vision rehabilitation should be recommended when a child has a visual

impairment that affects his or her ability to perform visual tasks (as occurs
with best corrected visual acuity worse than 20/40 in better seeing eye,
decreased visual field, central field loss, or reduced contrast sensitivity). At
the time of diagnosis and throughout the suubsequent years, the
ophthalmologist plays an important role in recommending that children with
low vision receive and child on a course to achieve optimal visual performance
and to make a successful adjustment to vision loss

Rehabilitasi penglihatan harus direkomendasikan ketika seorang anak memiliki

gangguan penglihatan yang mempengaruhi kemampuannya untuk melakukan tugas-
tugas visual (seperti yang terjadi dengan ketajaman visual koreksi yang lebih baik
lebih buruk daripada 20/40 dalam mata yang lebih baik, bidang visual menurun,
kehilangan lapangan pusat, atau berkurang sensitivitas kontras). Pada saat
diagnosis dan sepanjang tahun-tahun berikutnya, dokter mata memainkan peran
penting dalam merekomendasikan bahwa anak-anak dengan penglihatan low vision
dan anak pada kursus untuk mencapai kinerja visual yang optimal dan untuk
membuat penyesuaian yang sukses terhadap kehilangan penglihatan.

Vision rehabilitation for children often involves pediatric ophthalmologist,

vision rehabilitation clinicians, occupational therapists, teachers, orientation
and mobility specialists, technology experts, state societies, and other
professionals and organizations. An individualized education plan (IEP)
outlines the needs of an individual child in the school setting. The child’s need
in the home and in other nonacademic settiings must be considered as well. A
variety of aids are available to assist patients with low vision, ranging from
simple telescopes to braille literacy. Because most children have large
accommodative amplitudes that allow them to hold an object closer than
normal to enlarge its image, magnifiers may not be necessary for pediatric
patients with low vision. Existing and future technologies, including e-readers,
audio books, and text to speech technology, offer continually expanding
opportunities for these children.

Rehabilitasi visi untuk anak-anak sering melibatkan dokter mata anak, dokter
rehabilitasi visi, ahli terapi okupasi, guru, spesialis orientasi dan mobilitas, ahli
teknologi, masyarakat negara, dan profesional dan organisasi lainnya. Rencana
pendidikan individual (IEP) menguraikan kebutuhan seorang anak di lingkungan
sekolah. Kebutuhan anak-anak di rumah dan di tempat-tempat non-akademik
lainnya juga harus dipertimbangkan. Berbagai alat bantu tersedia untuk membantu
pasien dengan penglihatan rendah, mulai dari teleskop sederhana hingga membaca
huruf braille. Karena kebanyakan anak memiliki amplitudo akomodatif besar yang
memungkinkan mereka untuk memegang objek lebih dekat dari biasanya untuk
memperbesar gambarnya, kaca pembesar mungkin tidak diperlukan untuk pasien
anak dengan penglihatan rendah. Teknologi yang ada dan yang akan datang,
termasuk e-reader, buku audio, dan teknologi text to speech, menawarkan peluang
yang terus berkembang untuk anak-anak ini.

major congenital anomalies occur in 2%-3% of live births. Causes include

chromosomal anomalies, multifactorial disoreders, enviromental aggents, and
idiopathic etiologies. Regardless of etiology, from a developmental point of
view, congenital anomalies may be categorized as follows (ocular examples
are given in pharentheses)

Anomali kongenital terjadi pada 2-3% kelahiran hidup. Penyebabnya termasuk

anomali kromosom, gangguan multifaktorial, faktor lingkungan, dan penyebab
idiopatik. Terlepas dari etiologi, dari sudut pandang perkembangan, Anak-anak
dengan penyakit okular dini memiliki penglihatan yang jauh lebih buruk daripada
yang diperkirakan. anomali kongenital dapat dikategorikan sebagai;

 agenesis: kegagalan perkembangan (anophthalmos)

 hypoplasia: hypoplasia saraf optik
 hyperplasia: perkebangan yang berlebihan (dictichiasis)
 dysparisia: kegagalan untuk menyatu (choroidal coloboma)
 Kegagalan untuk menyalurkan (obstruksi ductus nasolakrimal kongenital)