Download MBBS (Bachelor of Medicine, Bachelor of Surgery) Ophthalmology PPT 23 Congeital Glaucoma Lecture Notes
Congenital glaucoma/ Buphthalmos
CONGENITAL GLAUCOMA
Learning Objectives:
1. Definition
2. Pathogenesis
3. Clinical Presentation
4. Differential diagnosis
5. Management
Congenital Glaucoma
? A condition of raised intraocular pressure in early
childhood with developmental anomaly of the anterior
chamber angle region without any associated abnormality
of eye or adnexa, which obstructs the drainage of aqueous
humour.
? It appears between birth and 4 yrs of age.
? Both eyes are usually involved, but to varying severity
? Boys are more affected than girls.
? Affects 1 in 10,000 births
? A hereditary factor is occasionally present (Autosomal recessive).
The Cause
Types
1. Primary developmental/congenital glaucoma.
2. Developmental glaucoma with associated ocular
anomalies
3. Secondary congenital/Juvenile glaucoma
Pathogenesis
? Failure/abnormal development of the anterior chamber angle and
trabecular meshwork during intrauterine development.
? Maldevelopment of trabeculum including the iridotrabecular
junction (trabeculodysgenesis)
? Angle develops from mesodermal tissue
either in the form of failure of resorption of mesodermal sheet
Or
abnormal cleavage of AC angle
This is responsible for impaired aqueous outflow resulting
in raised IOP.
Figure1: The normal chamber angle: on the left is a
histological cross-section; on the right is a drawing of the same
Figure 2: An underdeveloped chamber angle
Clinical Presentation
Symptoms:
? Photophobia
? Blepharospasm
? Lacrimation/watering
Clinical Presentation
Signs:
? Enlargement of the globe (buphthalmos) is a result of elevated
intraocular pressure.
? Bluish discoloration patches over sclera
[due to thinning of sclera underlying uveal tissue becomes visible]
Clinical signs
? Corneal signs.
i. Corneal oedema.
ii. Corneal enlargement. (corneal diameter > 13mm)
iii. Tears and breaks in Descemet's membrane
(Haab's striae).
EPIPHORA
CORNEAL EDEMA
HIGH IRIS INSERTION
HAAB'S STRIAE
ON GONIOSCOPY
Clinical signs
? Anterior chamber becomes deep.
? Iris may show iridodonesis and atrophic patches
in late stage.
? Lens becomes flat due to stretching of zonules and may
even subluxate.
Clinical signs
? IOP is raised which is neither marked nor acute.
? Axial myopia may occur because of increase in
axial length which may give rise to anisometropic
amblyopia
? Optic disc may show variable cupping and thus damage to otic
nerve in the form of atrophy
Diagnosis
? Clinical clues
? Enlarged eyes; tearing, and photophobia (avoidance of light).
? Often, babies also rub their eyes.
? If CG is suggested, a thorough examination under general anesthesia
is necessary.
? to avoid blepharospasm (spasmodic closure of the eyes). to prevent a
transient rise in the IOP.
? Besides measuring the IOP, anesthesia al ows a thorough investigation of al
segments of the eye and, in particular, the optic disc
Diagnosis
1. Measurement of IOP with Schiotz or preferably hand held Perkin's
applanation tonometer.
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
pathogenesis.
Differential Diagnosis
? Cloudy Cornea: In unilateral cases the commonest
cause is trauma with rupture of Descemet's
membrane (forceps injury).
In bilateral cases causes may be trauma, mucopolysaccharidosis,
interstitial keratitis and corneal endothelial dystrophy.
Differential Diagnosis
? Large cornea due to buphthalmos should be
differentiated from megalocornea.
? Lacrimation in an infant is usually considered to
be due to congenital nasolacrimal duct blockage
and thus early diagnosis of congenital glaucoma
may be missed.
? Photophobia may be due to keratitis or uveitis.
Differential Diagnosis
? Raised IOP in infants may also be associated
with
? retinoblastoma,
? retinopathy of prematurity,
? persistent primary hyperplastic vitreous,
? traumatic glaucoma and
? secondary congenital glaucoma seen in rubella, aniridia and Sturge-
Weber syndrome.
Treatment
? The treatment is primarily surgical
(medical treatment is given temporarily- beta blockers,hyper osmotics,
acetazolamide) .
? Different surgical procedures
(according to the degree of the maldevelopment and
the clarity of the cornea)
? Goniotomy (85% success)
? Trabeculotomy
? Trabeculotomy + trabeculectomy (best results)
? Supplemental treatment options are
? Medical therapy
? Implant surgery
? Cyclodestructive procedures
TRABECULOTOMY
Goniotomy
Trabeculotomy
Trabeculectomy
Infantile Glaucoma
? Infantile glaucoma is also congenital glaucoma
? However, intraocular pressure starts to rise at some time during the first
years of life.
? The cause for this IOP increase is basically the same as in congenital
glaucoma, but it occurs later since the anterior chamber angle is more
mature than when glaucoma is present at birth.
? The IOP may be normal during the first years of childhood and then
gradually increase.
Juvenile Glaucoma
? Juvenile glaucoma is an IOP increase that occurs in an older child or
young adult (10- 35 yrs)and is often inherited.
? During a thorough examination, the ophthalmologist may find
discreet evidence of an incomplete maturation of the chamber angle,
? The clinical features as well as treatment of juvenile glaucoma are
quite similar to adult Primary Chronic Open-Angle Glaucoma
(POAG)
Developmental glaucoma with associated
anomalies
? Glaucoma with iridocorneal dysgenesis
? Glaucoma with aniridia
? Glaucoma with ectopia lentis
? With phakomatosis- Sturge weber syndrome, neuro fibromatosis
? Lowe's, naevus of ota, nanophthalmos, congenital ectopian uveae,
microcornea & rubella syndrome
This post was last modified on 07 April 2022