1. Definition
2. Pathogenesis
3. Clinical Presentation
4. Differential diagnosis
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5. ManagementCongenital Glaucoma
? A condition of raised intraocular pressure in early
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childhood with developmental anomaly of the anteriorchamber angle region without any associated abnormality
of eye or adnexa, which obstructs the drainage of aqueous
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humour.
? It appears between birth and 4 yrs of age.
? Both eyes are usually involved, but to varying severity
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? Boys are more affected than girls.? Affects 1 in 10,000 births
? A hereditary factor is occasionally present (Autosomal recessive).
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The CauseTypes
1. Primary developmental/congenital glaucoma.
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2. Developmental glaucoma with associated ocularanomalies
3. Secondary congenital/Juvenile glaucoma
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Pathogenesis
? Failure/abnormal development of the anterior chamber angle and
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trabecular meshwork during intrauterine development.? Maldevelopment of trabeculum including the iridotrabecular
junction (trabeculodysgenesis)
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? Angle develops from mesodermal tissue
either in the form of failure of resorption of mesodermal sheet
Or
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abnormal cleavage of AC angleThis is responsible for impaired aqueous outflow resulting
in raised IOP.
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Figure1: The normal chamber angle: on the left is a
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histological cross-section; on the right is a drawing of the same
Figure 2: An underdeveloped chamber angle
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Clinical PresentationSymptoms:
? Photophobia
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? Blepharospasm? Lacrimation/watering
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Clinical Presentation
Signs:
? Enlargement of the globe (buphthalmos) is a result of elevated
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intraocular pressure.
? Bluish discoloration patches over sclera
[due to thinning of sclera underlying uveal tissue becomes visible]
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Clinical signs
? Corneal signs.
i. Corneal oedema.
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ii. Corneal enlargement. (corneal diameter > 13mm)iii. Tears and breaks in Descemet's membrane
(Haab's striae).
EPIPHORA
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CORNEAL EDEMAHIGH IRIS INSERTION
HAAB'S STRIAE
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ON GONIOSCOPY
Clinical signs
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? Anterior chamber becomes deep.? Iris may show iridodonesis and atrophic patches
in late stage.
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? Lens becomes flat due to stretching of zonules and may
even subluxate.
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Clinical signs
? IOP is raised which is neither marked nor acute.
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? Axial myopia may occur because of increase inaxial length which may give rise to anisometropic
amblyopia
? Optic disc may show variable cupping and thus damage to otic
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nerve in the form of atrophy
Diagnosis
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? Clinical clues
? Enlarged eyes; tearing, and photophobia (avoidance of light).
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? Often, babies also rub their eyes.? If CG is suggested, a thorough examination under general anesthesia
is necessary.
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? to avoid blepharospasm (spasmodic closure of the eyes). to prevent a
transient rise in the IOP.
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? Besides measuring the IOP, anesthesia al ows a thorough investigation of alsegments of the eye and, in particular, the optic disc
Diagnosis
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1. Measurement of IOP with Schiotz or preferably hand held Perkin's
applanation tonometer.
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2. Measurement of corneal diameter by callipers.3. Ophthalmoscopy to evalute optic disc.
4. Gonioscopic examination of angle of anterior
chamber reveals trabeculodysgenesis with either
flat or concave iris insertion as described in
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pathogenesis.Differential Diagnosis
? Cloudy Cornea: In unilateral cases the commonest
cause is trauma with rupture of Descemet's
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membrane (forceps injury).In bilateral cases causes may be trauma, mucopolysaccharidosis,
interstitial keratitis and corneal endothelial dystrophy.
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Differential Diagnosis? Large cornea due to buphthalmos should be
differentiated from megalocornea.
? Lacrimation in an infant is usually considered to
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be due to congenital nasolacrimal duct blockageand thus early diagnosis of congenital glaucoma
may be missed.
? Photophobia may be due to keratitis or uveitis.
Differential Diagnosis
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? Raised IOP in infants may also be associated
with
? retinoblastoma,
? retinopathy of prematurity,
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? persistent primary hyperplastic vitreous,? traumatic glaucoma and
? secondary congenital glaucoma seen in rubella, aniridia and Sturge-
Weber syndrome.
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Treatment
? The treatment is primarily surgical
(medical treatment is given temporarily- beta blockers,hyper osmotics,
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acetazolamide) .
? Different surgical procedures
(according to the degree of the maldevelopment and
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the clarity of the cornea)
? Goniotomy (85% success)
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? Trabeculotomy? Trabeculotomy + trabeculectomy (best results)
? Supplemental treatment options are
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? Medical therapy
? Implant surgery
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? Cyclodestructive proceduresTRABECULOTOMY
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Goniotomy
Trabeculotomy
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TrabeculectomyInfantile Glaucoma
? Infantile glaucoma is also congenital glaucoma
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? However, intraocular pressure starts to rise at some time during the firstyears of life.
? The cause for this IOP increase is basically the same as in congenital
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glaucoma, but it occurs later since the anterior chamber angle is more
mature than when glaucoma is present at birth.
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? The IOP may be normal during the first years of childhood and thengradually increase.
Juvenile Glaucoma
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? Juvenile glaucoma is an IOP increase that occurs in an older child oryoung adult (10- 35 yrs)and is often inherited.
? During a thorough examination, the ophthalmologist may find
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discreet evidence of an incomplete maturation of the chamber angle,
? The clinical features as well as treatment of juvenile glaucoma are
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quite similar to adult Primary Chronic Open-Angle Glaucoma(POAG)
Developmental glaucoma with associated
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anomalies
? Glaucoma with iridocorneal dysgenesis
? Glaucoma with aniridia
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? Glaucoma with ectopia lentis? With phakomatosis- Sturge weber syndrome, neuro fibromatosis
? Lowe's, naevus of ota, nanophthalmos, congenital ectopian uveae,
microcornea & rubella syndrome
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