2. Definition :
Developmental glaucomas are a group of disorders characterized by improper
development of ocular aqueous outflow system usually manifesting in infancy or
childhood
Importance :
• Though uncommon impact on visual development can be significant
• Early recognition and appropriate therapy can significantly improve child’s visual future
3. Classification :
1.True Congenital Glaucoma – Raised IOP during intra uterine
life
- Neonatal glaucoma
- Poor prognosis
2. Congenital / Infantile – Birth to 4 years
3. Late congenital – 4 to 10 years
4. Juvenile Galucoma – 10 to 35 years
4. Three main categories depending on pathogenesis :
1. Primary congenital glaucoma : Developmental anamoly restricted to maldeveloment
of TM
2. Glaucomas associated with specific ocular or systemic congenital anomalies
3. Glaucomas secondary to miscellaneous pediatric conditions encircling eye
5. Terminology :
• Congenital glaucoma = Developmental glaucoma
• Primary congenital glaucoma = Isolated maldevelopment of TM without other developmental ocular
anomalies or diseases
• Infantile glaucoma = Congenital glaucoma occurring in first couple of years of life
• Primary infantile glaucoma = Primary congenital glaucoma
• Juvenile glaucoma = Non specific term to any type of glaucoma occurring later in childhood ( > 5 years )
and through third and fourth decades
7. Congenital Glaucoma :
- Incidence is 1 in 10,000 live births
Primary Secondary
Sporadic Hereditary
- 90 % - AR
- 4 % - AD
8. Primary congenital Glaucoma :
• Most common glaucoma seen in infancy
• Bilateral in 75 % of cases
• Males are affected more than females by 65 %
• More than 80 % are evident before first year of life
9. Hereditary :
GLC3A and GLC3E - CYP1B1 gene
Required for normal oxidative homeostasis
loss of enzymatic
activity
Abnormal organization and function of
Trabecular meshwork
GLC3D - CTBP2 gene
- Produces ECM protein in TM and ciliary
body
- Has role in cell adhesion and serves as
structural component of microfibrils to
confer tissue elasticity
Normal development of anterior chamber
10. Pathogenesis :
Primary –
• Failure of development or Abnormal development of trabecular meshwork
• Iris may not be completely separated from cornea so angle remains closed by persistent embryonic
tissue
• As circulation is maintained by anterior ciliary veins and uveoscleral outflow rise of tension is neither
acute nor marked
• Neonatal has more malformations thus poor prognosis
11. Pathophysiology :
Normal development of infantile angle
• Mesenchymal mass of neural crest origin
• Three waves of tissue come between surface ectoderm and developing lens by 8th week
• First wave – primordial corneal endothelium and descements membrane
• Second wave – between epithelium and endothelium – corneal stroma
• Third wave - between cornea and lens – pupillary membrane and iris stroma
12. AC cavity :
• Slit in mesenchyme occurs between surface ectoderm and
developing iris
• Anterior to slit is corneal endothelium
• Posterior is the pupillary membrane
13. Angle of anterior chamber :
• Nest of loosely arranged undifferentiated neural crest derived mesenchymal cells form the
trabecular meshwork
• By 5th month of GA. AC cavity will be closed due to continuous layer of endothelial cells
• Anterior surface of iris will be present infront of primordial TM covering it called as fetal pectinate
ligament
14. By third trimester ( 7th month )
• Endothelial layer disappears from pupillary membrane and iris and cavitates over anterior chamber angle
and incorporates into TM
• Ciliary muscle and ciliary processes overlap TM being separated by loose connective tissue
• Angle recess deepens and peripheral uveal tissue moves posteriorly in relation to angle structures and TM
is exposed to anterior chamber
• Development of trabecular lamellae and intratrabecular spaces begin in inner aspect ( posterior ) of
primordial tissue and progress towards Schelmms canal and Schwalbes line
15.
16. Deepening mechanisms :
• Atrophy and absorption of tissues
• Process of cleavage between two separate cell types – one forms TM
and other forms iris root and ciliary body
• TM is exposed to AC by means of posterior sliding of iris , ciliary muscle
and ciliary processes
• Major developments related to iridocorneal angle occur in third
trimester
• But embryonic insults in first 3 – 5 weeks following fertilization can also
manifest as anterior segment dysgenesis
17. Schlemms canal :
• Develops by the end of third month of
GA
• From mesodermal mesenchyme
Posterior chamber :
• Split in mesenchyme posterior to
developing iris and anterior to
developing lens
• Anterior chamber and posterior
chamber communicate when pupillary
membrane disappears and pupil is
formed
18. New born angle :
• Iris insertion into angle wall will be posterior to scleral spur ( SS ) with anterior extension of ciliary
body band ( CBB ) anterior to iris insertion
• Continuous posterior sliding of uveal tissue occurs during first 6 – 12 months of age
• At the end angle recess will be formed with apparent posterior insertion of iris root with ciliary body
• Normal AC angle is fully developed by 1 year of age
19.
20. Malformations :
• Iris and Ciliary body appear like eye in 7th or 8th GA
• Iris and ciliary body failed to recede posteriorly thus iris insertion and anterior ciliary body
overlaps posterior portion of TM
• Anterior insertion of ciliary body muscle
• Longitudinal and circular muscle fibres of ciliary muscles insert into TM rather than SS
• Root of iris can insert directly into TM
• Thickening of trabecular beams , thickened cords of uveal meshwork , compression of meshwork
with a resultant decrease of trabecular spaces
21. • Barkans membrane
• Schlemms canal – open in early cases of primary congenital glaucoma
• Obliterated in advanced cases due to secondary alteration caused by effect of pressure elevation on ocular
tissues
• Thickening of juxtacanalicular tissues
• Amorphous material in subendothelial area of internal wall of Schelmms canal leading to thickened cords of
uveal meshwork
• This holds iris anteriorly , prevents SS from rotationg posteriorly and preventing trabecular sheets from
separating normally
22.
23. Conclusion :
• The more narrower ciliary body band the more immature is the angle
• Obstruction to aqueous outflow is located at trabecular sheets
• Therefore in most cases goniotomy with incision at trabecular sheets normalizes IOP
24.
25. Isolated trabeculodysgenesis :
• Found in approximately 50 % of primary infants and juvenile cases
• No other congenital anomalies of iris or cornea except of abnormal insertion of iris into angle wall
• Occurs in two forms
First form :
• Iris inserts flatly into TM either at or anterior insertion to SS
• Ciliary body is obscured by this insertion although anterior ciliary body may be seen
26. • Invisibility of angle recess and ciliary body. - key distinction
• Surface of TM may appear as stippled , orange peel appearance
• Peripheral iris stroma may appear thinned and radial blood vessels are exposed
• If eye enlarges there will be more pronounced iris thinning
27. Second form :
• Iris inserts concavely into chamber angle wall
• Plane of iris is posterior to SS but anterior stroma sweeps upward over TM obscuring SS and inserting into
upper portion of TM just posterior to Schwalbes line
• Also called concave or wrap around insertion
• More common in brown irides and over all less common in children
• They present as raised IOP after first month of life
28. • Both forms may also appear in later juvenile forms of open angle glaucoma through third or fourth decades
of life
29. Iridodysgenesis :
Congenital developments of iris are associated with maldevelopment of
• Trabecular meshwork
• Anterior stroma
• Full thickness of iris
• Iris vessels
• Irido corneal adhesions or combination of these
30. • TM malformations are similar to isolated trabeculodysgenesis
Anterior stromal defects :
Hypoplasia of iris stroma Iris Hyperplasia
• Most common association with developmental glaucoma
• True hypoplasia – Clear malformation of collarette with
absence or marked reduction of crypts
• Distinguished from atrophy or thinning which is due to
stretching of iris from elevated IOP
• Pupillary sphincter may be prominent and have distinct
ring appearance or feathered outer border
• Thickened velvety pebbled appearance of anterior iris
stroma
• Seen in association with Sturge – Weber syndrome
31.
32. Iris vasculature anomalies :
Persistent tissue vasculosa lentis Irregularly wandering superficial iris vessels
• Regular arrangement of vessels looping into pupillary
axis either infront or behind the lens
• Attenuation and involution of vascular veil
• Pupil usually distorted
• Iris surface has whorled appearance with areas of
hypoplasia
• Have grave prognosis and require multiple surgeries
33.
34. Structural iris defects :
• Either as a small hole through iris without involvement of sphincter
• Full thickness coloboma involving sphincter
• Most severe - aniridia
35. Corneodysgenesis :
• May be peripheral , mid peripheral and central cornea or abnormalities of corneal size
• Mostly associated with iris abnormalities
Peripheral lesions :
• Bridging iris filaments or bands attach to prominent cord like Schwalbes line – posterior embryotoxon
• Extend no more than 2 mm. into clear cornea and involve 360 degree circumference
• Can be seen in 8 % of normal eyes Ex : Axenfeld Anamoly
36. Mid peripheral Lesions :
• Iris attached to cornea in broad areas of apposition which extend out to centre of cornea
• Cornea is opacified at that area
• Pupillary anamolies and holes of iris are common
Ex : Reigers anomaly
37. Central :
• Adhesions between collarette of iris and posterior aspect of central cornea
• Opacified centrally and thinned and fistulas develop occasionally
• Area of clear cornea between central defect and cornoeoscleral limbus is common
Examples : Peters anomaly
Posterior ulcer of Von Hippel
Posterior keratoconus
38. Abnormalities of size :
Microcornea :
• Seen as independent anamoly or associated with microphthalmos , nanophthalmos , Reigers anomaly ,
PHPV , congenital rubella syndrome
Macrocornea :
• Axenfeld syndrome , X linked recessive megalocornea
v Prognosis of glaucoma with corneodysgenesis is the worst
39. Raised IOP
Distensibility of sclera
cornea – 3 Y.
Stretching of cornea and sclera – Buphthalmos / Hydrophthalmos
sclera – 10 Y.
Lamina cribrosa stretch
Optic disc cupping ( Reversible if not gone into atrophy )
40. Symptoms : 1. Watering 2. Photophobia 3. Blepharospasm. And enlargement
of eyes
41. Signs :
Ø Buphthalmos – Vulnerable to lens subluxation and rupture with trivial trauma
Ø Cornea – haze is seen Raised IOP
Stretch of cornea
aqueous enters and swells
Stromal edema. - Ground / Frosted glass appearance
later stage
Haabs striae
42. Haabs striae –
Corneal stretch
DM and endothelium fractures , rips and breaks
Stromal edema and acute hydrops
Endothelial cells migrate over breaks and lay down new BM
Ridges along separate edges of DM – forms discrete opacities appearing as lines with
double contour – Double stria or Tram track appearance
43.
44. Keratoglobus ( AC hence deep ) – due to stretch of
junction of cornea and sclera
Lens flattened and displaced backwards – expansion
of ciliary region and stretch of suspensory ligament
Iridodonesis
Myopia ( but somehow less due to backward
displacement of lens )
IOP is along 30 ‘s
45. ü Angle abnormalities are seen
ü Optic disc cupping
ü Visual field defects
ü RNFL thinning
ü Amblyopic strabismus
47. Peters anomaly
Choristomas ( Dermoid and Dermis like )
Herpes infection
Large cornea - Megalocornea
High myopia
Microphthalmic fellow eye
48.
49. Epiphora
Conjunctivitis ,
aberrant lashes ,
Entropion
Delayed or failed
canalization of
eye
Photophobia
Conjunctivitis ,
iritis , trauma
Tears in DM
PPD ,
posterior corneal
vesicles
50. Juvenile
primary
Open
angle
Glaucoma
( JPOG ) :
Autosomal Dominant
Mutation of gene on long arm of ch 21
Abnormal protein TIGR / myocilin TM less
permeable to aqueous
Steroids if given – myocilin increases – raised IOP
On histopathology – thin imperforate membrane
covered AC angle structures and impeded aqueous
humor outflow - Barkans Membrane
Goniotomy useful
51.
52. Glaucomas associated with other congenital abnormalities:
• Associated with malformation of one or more angle structures
• Most of them are of neural crest origin – Neurocristopathies
• May be due to
Trabeculodysgenesis
Iridodysgenesis
Corneodysgenesis
53. Axenfield – Reiger syndrome :
• Axenfield anomaly - Peripheral anterior segment defects
• Reiger anomaly - Axenfeld anomaly + Iris abnormality
• Reiger syndrome - Ocular plus systemic abnormalities
v Posterior embryotoxon of cornea – Axenfeld
v Mesoderm dysgenesis of cornea and iris - Reiger
54. Features :
• Autosomal Dominant
• Bilateral , M = F
• Diagnosed from birth to adulthood
• Structures involved are peripheral cornea , AC angle and iris
55. Pathogenesis :
Developmental arrest late in gestation
High insertion of anterior uvea onto Abnormal retention of primordial
posterior TM endothelial layer on portions of iris and angle
Incomplete maturation of TM and Iridocorneal strands
schlemms canal
Contraction on iris
Iris changes
56. Genetics :
- Three loci - 4q25 , 6p25 , 13q14
- Three types 1 . PITX2
2. 13q14
3. FOXC1
- Same for AR syndrome , AA , RA , RS , Iridodysgenesis anomaly and syndrome , Iris
hypoplasia , Familial glaucoma , IG dysplasia
57. Signs :
1. Cornea :
• Prominent anteriorly displaced Schwalbe line
• On slit lamp white line on posterior cornea near limbus ( upto temporal quadrant or 360 degrees )
• Sometimes normal and seen only on gonioscopy
• Also present in congenital glaucoma and ICE syndromes
• Isolated defect – posterior embryotoxon
• Megalo or microcornea and opacities may be present
58. 2. AC angle :
• Iridocorneal adhesions are present as strands ( thread like to broad bands ) one clock hour or more
• AC angle is open with visible trabecular meshwork
• Scleral spur is obscured due to insertion of peripheral iris into posterior portion of trabecular meshwork
59. 3. Iris :
• Mild stromal thinning to marked atrophy with hole
• Corectopia ( pupil displaced towards prominent peripheral tissue strand )
• Ectropion uvea
• Central iris changes may progress
• Sometimes normal
60. Glaucoma :
• At infancy or childhood or young adulthood
• Due to high insertion of peripheral iris into trabecular
meshwork
• Abnormality of trabecular meshwork and Schlemms
canal ( rudimentary or absent )
62. Systemic features :
• Dental - Decreased crown size ( micro odontia )
Decreased but evenly spaced number of teeth ( hypo odontia )
Focal absence of anterior maxillary or permanent central incisors ( oligo or
an odontia )
• Facial - Maxillary hypoplasia with flattening of mid face and receeding upper lip
Prominent lower lip ( associated with dental hypoplasia )
Hypertelorism , telecanthus , broad flat nose , micrognathia , mandibular
prognathism
65. Management :
Medical - Beta blockers
Carbonic Anhydrase inhibitors
Alpha agonists
• For resistant cases and for infantile glaucoma
Surgical - Goniotomy
Trabeculotomy
Trabeculectomy , Drainage devices and Cycloablation
66. Peters anomaly :
• Sporadic
• May be Autosomal recessive or Dominant
• Hallmark - central corneal abnormality
- Defect in DM , endothelium , stroma and Bowmanns
membrane
67. Clinical Features :
• Iris adhesions extend to border of this corneal defect
Not associated with kerato lenticular contact Associated with Kerato lenticular contact or cataract
Primary failure of corneal endothelial development Incomplete separation of lens vesicle from surface
ectoderm
Or from an intrauterine malformation Lens develops normally and secondarily pushed forwards
against cornea causing loss of DM
Von Hippel Internal corneal ulcer
• Both are associated with glaucoma due to Peripheral Anterior Synechiae
70. Treatment :
• Penetrating Keratoplasty with Trabeculectomy or drainage devices
• Cyclodestructive surgeries
71. Aniridia :
• Bilateral
• Misnomer because iris is partially absent with rudimentary stump
• Presents at birth ( or ) childhood ( or ) adulthood
• AD – two third cases and sporadic in one third cases
• Associated genes are 11p13 with PAX6 gene
73. Glaucoma :
• Presentation at birth or early childhood – abnormal AC angle structures
• Later in life – Previously functioning TM appears to be occluded due to anterior migration and
rotation of rudimentary iris stump
• Progressive obstruction of angle by contracture of tissue strands between peripheral iris and angle
wall
On Gonio
• Strands of tissue with fine blood vessels extending from iris root to trabecular meshwork
• Sometimes congenital anomalies in filtration angle
74. Others :
• Microcornea , Iridocorneal and keratolenticular adhesions
• Subluxated , reabsorbed or absent lens or cataract
• Choroidal colobomas , persistent pupillary membranes , sclerocornea
• Hallerman – Streiff syndrome , small optic nerve heads
• Strabismus , ptosis ,Retinoblastoma
• Marfans with cervical ribs and dental anomalies
• Delayed closure of anterior fontanelle
75. Management :
• Medical and trabeculotomy
• Glaucoma drainage devices away from corneal endothelium
• Cyclocryotherapy but more prone to pthisis
Cataracts and Corneal Opacities :
• Remaining anterior capsule often opacifies - Pseudo iris
• Insertion of IOL with peripheral black diaphragm
76. Familial hypoplasia of iris with glaucoma :
Features :
• Hypoplasia of anterior iris stroma
• Prominent pupillary sphincter
• Trabeculodysgenesis
• Glaucoma. From birth to adulthood
Ø Autosomal dominant inheritance….4q25 , 6p25 , 13q14 genes
Ø Management : Goniotomy or trabeculotomy
77. Developemental glaucoma with anomalous superficial iris vessels :
• Irregularly wandering iris vessels with distortion and absence of superficial iris stroma and distortion of
pupil
• Cornea is hazy and vessels are difficult to see
• Bilateral
• Seen in new born child with glaucoma
Management :
• Goniotomy or trabeculotomy
• Multiple surgeries are required
78. Sturge – Weber syndrome :
• Encephalo facial angiomatosis or Encephalo trigeminal angiomatosis
• Flat facial hemangiomas follow distribution of fifth cranial nerve
• Associated with intracranial hemangiomas
• Unilateral or bilateral
• Ocular features include choroidal and episcleral hemangiomas
• Leakage causes retinal edema
79. • Glaucoma occurs more in infancy
• Present when hemangioma involves lids and conjunctiva
Pathogenesis :
• If glaucoma at birth - abnormal angle structures
• Later - increased episcleral venous pressure
Management :
• Medical therapy tried
• If failed surgical therapies like goniotomy , trabeculotomy ,filtration surgeries or drainage devices
80. Other neural crest disorders involving episcleral vascular malformations thus causing glaucoma are
• Klippel – Trenaunay – Weber syndrome and Oculo dermal Melanocytosis ( Nevus of Ota )
• Phakomatosis pigmentovascularis ( oculodermal melanoctosis + nevus flammus ) is also a strong
predisposition for congenital glaucoma
• Cutis marmorata telangiectasia congenita involves periocular vascular anomalies associated with
regional or genelarized cutaneous marbling which is associated with infantile trabeculodysgenic
glaucoma
81. Neuro Fibromatosis :
• Autosomal dominanat
• Also called Von Reckinghausen disease
• Ophthalmologic features – Lisch nodules , Choroidal hematomas , Optic N. gliomas
• Glaucoma occurs in 50 % cases when involves upper eyelid or eye
Pathogenesis :
• Isolated trabeculodysgenesis
• Synechial closure by NF tissue posterior to iris
82. • NF infiltration of angle with synechial closure
• Late onset glaucoma due to ectropion uvea at pupillary margin
• Associated with ptosis without a palpable neurofibroma
• Pupil appears larger due to static iris hyperplasia of central iris pigment epithelium
Management :
• Goniotomy and trabeculotomy if iris adhesions are present
• In older children medical therapy is tried first followed by surgical therapy
85. Secondary Glaucomas :
Persistent Primary Hyperplastic Vitreous :
• Unilateral seen in a microophthalmic eye at birth
• Due to failure of atrophy of primary vitreous and vascular structures
• Retrolental fibrovascular membrane attaches to posterior aspect of lens and elongated ciliary
processes drawing them into pupillary spaces
• Membrane appears as white mass into pupil - leukocoria
86. Glaucoma :
• Progressive opacification and swelling of lens
• Contraction of retrolentral membrane pushing lens forward angle closure glaucoma
• Haemorrhages
Management :
• Cataract extraction
• Berveldt implant for post cataract glaucoma
87. Retinopathy Of Prematurity :
• Bilateral and symmetrical
• Retrolental fibrous membrane causes forward displacement of lens and iris
Angle closure with some degree of pupillary block
Management :
• Lens removal + removal of retrolental membranes
88. Lens related :
Aphakic Glaucoma :
• According to Infant Aphakia Treatment Study – incidence of glaucoma related adverse
effects following congenital cataract surgery are
@ 1 year – 12 %
@ 5 years – 18 %
• Most common related to lens and second most common overall
89. Etiology :
• Blockage of Posterior Trabecular
Meshwork with pigments and
synechiae
• Near constant ( 96 % ) filtration angle
deformity
Risk factors :
• Congenital cataract with micro cornea
• Perhaps cataract surgery unmasks a
marginally functional and
maldeveloped AC angle
• Family history and early age of surgery
90. • Glaucoma occurs 15 – 30 months following surgery
Treatment :
• Goniotomy and trabeculotomy are contraindicated due to presence of synechial angle
closure leading to haemorrhage
• Trabeculectomy or Drainage implants are recommended
• Posterior IOL implantation may provide benefit by reducing iris apposition against
angle and facilitating active pumping mechanism of trabecular tissue
91. Marfans syndrome :
• AD and sporadic
• Ocular abnormalities include ectopia lentis , micropthalmos , myopia , hyperplasia of
iris stroma and dilator muscle , RD , glaucoma
Glaucoma :
1. Pupillary block due to malposition of lens ( upward subluxation may dislocate into pupil
or anterior chamber )
Management – Dilatation of pupil. PI or lens extraction
92. 2. Abnormal anterior chamber angle causing open angle glaucoma in adulthood
• Pectinate iris processes bridge the angle recess and insert anterior to SS
Management – Medical therapy followed by trabeculotomy / trabeculectomy
3. Post surgical glaucoma
93. Homocystinuria :
• AR . 1 in 50000 births
• Due to deficiency of cystathione beta synthase enzyme
• Ocular features are lens dislocation , RD , ectopia lentis
Glaucoma :
• Inferior subluxated lens moves out causing pupillary block
• Angle closure appears by age 5
94. Management :
• Dilatation of pupil and PI
• Lens extraction if dislocated into AC
• Dietary and vitamin supplementation may delay the onset of ectopia lentis
95. Microspherophakia :
• Seen in Weill Marchesani syndrome and GEMSS syndrome
• Weill Marchesani syndrome is autosomal dominant
• Microspherophakia with loose zonules dislocated downwards is seen
• High degree of myopia with shallow AC is a feature
• Angle closure can occur in the absence of lens dislocation
Management :
• Prophylactic Argon Iridoplasty
• Lens extraction + IOL implantation + Shunt surgery
97. Tumors :
Mechanism of glaucoma :
• Obstruction or invasion of TM by tumor cells. - leukemia
• Inflammatory and pigment debris obstruction. - Iris rhadomyosarcoma
• Increased volume of posterior segment. Forward displacement of lens – iris diaphragm
angle closure. - Choroidal metastasis / medulloepithelioma
• Rubeosis irides. NVG. - Retinoblastoma
98. Retinoblastoma :
• Rubeotic glaucoma due to NVG
• Non rubeotic due to extensive serous retinal detachment with ciliary rotation of iris – lens
diaphragm leading to secondary angle closure
Juvenile Xanthogranuloma :
• Glaucoma due to tumor involvement of iris and ciliary body or hemorrhage into anterior
chamber due to blunt trauma or spontaneously
• Management – Medical – tumor supressing therapy with subconjunctival steroids
99. Other causes of NVG :
Ø Eales disease
Ø Coats disease
Ø X linked familial exudative retinopathy ( Norries disease )
100. Inflammations
• Infective – rubella , herpes , syphilis
• Non infective – Sarcoidosis , Juvenile Rheumatoid Arthritis
• Glaucoma due to synechial angle closure due to chronic iridocyclitis
• Congenital Rubella Syndrome may have isolated trabeculodysgenesis
Management :
• Medical – anti glaucoma medications , anti inflammatory medications and cycloplegics
• Surgical - Trabeculodialysis may be tried
101. Steroid induced Glaucoma :
• Intensive exposure to topical or systemic steroids cause ocular hypertension and frank glaucoma in
susceptible individuals on genetic basis
Risk factors :
• Use of nasal and inhalational steroids ( for Bronchial Asthma )
• Intra vitreal triamcinolone for intractable uveitis or Diabetic Retinopathy
Pathogenesis – Trabecular metabolic dysfunction
Management – Medical measures + Removal of Intravitreal drug with PPV or standard trabeculectomy
102. Trauma :
• Birth injury due to forceps delivery
• Blunt or penetrating injuries
Features:
1. Angle recession > 180 degree
2. Traumatic cataract any two present post concussion glaucoma
3. Iris injuries
4. Lens displacement
103. Diagnosis :
Three steps :
I. Confirming or excluding diagnosis of glaucoma
II. Determining the cause of glaucoma
III. Gathering information vital to plan management
104. According to Abbreviated CGRN / WGA classification of childhood glaucomas :
Definition of Glaucoma : 2 are required
1. IOP > 21mm. of Hg.
2. OD cupping – progressive increase in cup disc ratio ( > 0.3 )
Cup disc asymmetry > 0.2
Focal rim thinning
3. Progressive myopia or myopic shift with increase in ocular dimensions
105. 4. Corneal findings - Haabs striae
Diameter > 11 mm. in newborn
> 12 mm. less than 1 year
> 13 mm. in anyone
5. Reproducible VF defect consistent with glaucomatous optic neuropathy
106. Glaucoma suspect : atleast one is required
1. IOP > 21 mm. Hg. on two separate occasions
2. Suspicious OD appearance for glaucoma
3. Suspicious VF for glaucoma
4. Increased corneal diameter or axial length with normal IOP
107. Ocular Examination :
ü Visual acuity. Visual fields
ü External examination Cycloplegic refraction
ü Slit lamp biomicroscopy Optic N. and fundus examination
ü Tonometry Ultrasonography
ü Gonioscopy Fundus photography
ü Pachymetry OCT
108. Visual examination :
• Central maintained fixation behavior and absent nystagmus. - Good prognosis
• Glaucoma , Amblyopia and optic disc changes causes decreased vision
External examination :
• For signs of associated abnormalities
• Buphthalmos , facial hemangiomas and neurofibromas are noted
109. Slit lamp examination :
Cornea - Enlargement
Edema
Scarring
Haabs striae and syndromic anomalies are noted
Corneal diameter measurements :
• Done with calipers
• Both horizontal and vertical diameters are taken
• Increased. Diameters
110. Age Normal Susoicious of
glaucoma
Birth to 6 months 9.5 – 11.5mm. > 12mm.
1 – 2 years 10 – 12 mm. > 12.5mm.
> 2 years < 12 mm. > 13 mm.
111. • AC depth , any inflammation or bleeding
• Iris changes
• Pupillary changes are noted
Tonometry :
• For children less than five years Examination Under Anaestheia ( EUA ) is done
• Mild sedative – Chloral hydrate syrup in a dose of 100mg./kg. to maximum of 3 gms. can be used in a
healthy full term greater than 1 month of age
112. • Different tonometers can be used like
Applanation - Goldmanns , hand held Perkins
Indentation - Schiotz
Hybrid - Pneumatotonometer
Non contact air puff - Keeler pulsair
Electronic - Tono pen or Mackay Marg
• Pneumotonometer provides accurate measurements
• Tonopen over estimates while Perkins underestimates IOP
113. Normal IOP readings according to age are
Age in years IOP in mm. Hg.
Birth 9.6
0 - 1 10.6
1 – 2 12.0
2 - 3 12.6
3 - 5 13.6
5 - 7 14.2
7 - 9 14.2
9 -12 14.3
12 - 16 14.5
114. Gonioscopy :
• Also done under anaesthesia
• In older children - Zeiss or Sussman ( Indirect lenses ) are used
• In infants or in theatre – Koeppe direct gonio lenses
• Trabeculodysgenesis , Iris insertion and other specific related features are noted
115. Pachymetry :
• Central corneal thickness ( CCT ) is noted
• In 6 – 23 months. It is 540 microns.
• In older children - 550 to 560 microns.
• Thinner CCTs are risk factors for developing glaucoma independent of IOP correction
• Also important for accurate AT readings
Thin Thick
Congenital glaucoma Aniridia
Aphakic glaucoma
116. Visual fields :
• Older children perimetry done
• Confrontation tests show severe nasal field loss
• Humphrey VF testing and Frequency doubling perimetry is used
• In Sturge Weber syndrome there will be underlying hemifield loss
117. Cycloplegic Refraction :
• Done after therapeutic normalization of IOP
• To correct significant difference in refractive error of both eyes
• To prevent Anisometropic and strabismic amblyopia as well as myopic astigmatism
Ultrasonography :
• A scan – Axial length is measured
• B scan – retinal status in opaque media
Patency of glaucoma drainage devices when bleb cannot be seen
118. • Anterior segment U/S – AC depth measurement
Optic N. and fundus examination :
• Infants and Younger children – 28 D or 30 D IO
• Older children – 78 or 90 D or 14 D IO
• Glaucomatous cup : More often round , steep walled , central with notable circumferential enlargement
• Sometimes oval cup can also be seen
• Scleral canal enlarges with rise in IOP so more cupping is seen compared to actual loss of neural tissue
119. • Reversible cupping with decrease in IOP is an important feature of congenital glaucomas because of
greater amount of elastin amidst the connective tissue of infantile optic N. head
• Other disc changes are comparable to adults
• C:D > 0.3. or asymmetry of more than 0.2 are signs
• Other features in fundus are. - Foveal hypoplasia , choroidal hemangiomas , peripheral retinal
pathology or vitreous stranding
120. Treatment :
• In congenital glaucomas surgery is the main stay of treatment
• Early surgery is essential
• Damage like permanent compression and adhesion of trabecular sheets is
increasingly likely , the longer the elevated IOP is maintained
• Surgery is advisable as soon as possible after making the diagnosis and is often
performed on second or third day of life in patients with glaucoma present at birth
121. Medical therapy :
• Given pre op to permit clearing of corneal edema and improve visualization of angle at the
time of diagnostic examination and surgery
• Beta blockers , prostaglandins , aderenergic agonists , topical or oral carbonic anhydrase
inhibitors , miotics can be used
• Prostaglandin analogues ( Latanoprost ) are usually well tolerated without demonstrable
toxicity in pregnants or children
• Timolol ( 0.25 % ) can cause apneic spells and asthmatic symptoms
• Brimonidine is avoided as it causes bradycardia , hypotension , hypothermia and apnea in
infants
• All antiglaucoma drugs are used in the dose of 1 drop for every 12 hours
122. Surgery :
• EUA done to determine extent of glaucoma and patients ocular health
• Main surgeries are goniotomy and trabeculotomy
• Goniotomy done in primary congenital glaucoma with a clear cornea
• In the case of two failure goniotomies or cloudy cornea or as a primary procedure
trabeculotomy is done
• Both have high success rates
123. Goniotomy :
• Incision made across the trabecular meshwork under direct gonioscopic visualization
using a goniotomy knife