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Congenital glaucoma
1.
2. CONGENITAL GLAUCOMAS
They are a group of diverse disorders in which
abnormal high intraocular pressure results due to
developmental abnormalities of the angle of
anterior chamber obstructing the drainage of
aqueous humour.
5. Pathogenesis
Primary congenital glaucoma is due to failure or abnormal development
of the trabecular meshwork
Maldevelopment of trabeculum including the iridotrabecular junction
(trabeculodysgenesis) is responsible for impaired aqueous outflow resulting
in raised IOP.
Trabeculodysgenesis is characterized by absence of the angle recess with
iris having a flat or concave direct insertion into the surface.
The iris may not completely separate from the cornea that the angle
remains closed by persistent embryonic tissue.
6.
7. Pathogenesis of Glaucomatous Ocular
Damage
Main Theories are:
1 Mechanical changes due to the rise of IOP
2 Vascular perfusion of the optic nerve head
3 Defective autoregulation
8. Above is shown a typical nerve appearance with damage from
glaucoma. Note that the center of the nerve has an excavated or
"scooped out" appearance.
10. CLINICAL FEATURES
1. Photophobia, blepharospasm, lacrimation & eye
rubbing are often occur together.
These are thought to be caused by irritation of
corneal nerves, which occurs as a result of the
elevated IOP.
Photophobia is usually the initial sign, but is not
enough by itself to arouse suspicion in most cases.
11. 2. Corneal signs : Corneal signs include its oedema, enlargement and
Descemet’s breaks.
Corneal oedema – It is frequently the first sign which arouses suspicion
Corneal enlargement- It occurs along with enlargement of globe-
buphthalmos especially when the onset is before the age of 3yrs
.
Haab’s straie (Tears and break in Descement’s membrane)-
These occur because Descement’s membrane is less elastic than the
corneal stroma. Tears are usually peripheral and concentric with the
limbus.
12. 3. Sclera becomes thin and appears blue due to underlying uveal tissue.
4. Anterior Chamber becomes deep.
5. Iris may show iridodonesis and atrophic patches in late stage
6. Lens becomes flat due to stretching of zonules and may even subluxate.
7. Optic disc may show variable cupping and atrophy especially after third
year.
8. IOP is raised which is neither marked nor acute
9. Axial myopia may occur because of increase in axial length which may
give rise to anisometropic ambylopia.
16. The same infant as the previous
photo. Although the red reflex from
the flash can be partially seen, a
whitish haze covers the entire
corneal surface.
This infant presented with hazy
bilateral corneal opacities on the
initial newborn exam. The diagnosis of
was relatively easy as there was a
strong family history of congenital
glaucoma.
17. Examination under General
Anaesthesia
IOP
normal is 10-15 mm Hg under anaesthesia -
anaesthetics and preoperative dehydration lowers IOP.
Vernier callipers are used to measure the corneal diameter in infants.
Gonioscopy
anterior chamber deep with normal iris structure
angle open with concave or flat insertion of iris root with abnormal tissue
giving shagreen, glistening appearance
may also have absent angle recess, peripheral iris hypoplasia, tenting of
peripheral iris pigment epithelium, thickened uveal trabecular meshwork.
18. Stretch marks in the cornea
(Haab’s striae) from the high
intraocular pressure in a patient
with congenital glaucoma (need
cropping and arrows).
The left eye of this congenital glaucoma
patient is noticeably larger than the right
eye. The patient has buphthalmos of the
left eye
19. Examination under General
Anaesthesia
Optic nerve
- normally pink with small cup
- preferential neural loss of superior and inferior
poles
- cupping may be reversible if IOP lowered initially
Axial length
- enlarged but may reverse with reduced IOP
21. DIFFERENTIAL DIAGNOSIS
Corneal dystrophies and dysgenesis
Inborn errors of metabolism
Intrauterine inflammations (congenital syphilis and
rubella)
Optic nerve pit, coloboma, or physiologic cupping
22. TREATMENT
Surgical
mainstay treatment
goniotomy for clear corneas
trabeculotomy for hazy corneas ,success rates similar
trabeculectomy and shunt procedures only when
goniotomy or trabeculotomy fails
23. GONIOTOMY
Involves making a horizontal incision at the midpoint
of the superficial layers of the trabecular meshwork
May need to be repeated
Eventual success rate is about 85%
Results are poor if the corneal diameter is 14mm or
more because Schlemm’s canal obliterated.
24. The goniotomy surgery involves entering the
anterior chamber with a sharp goniotomy
knife and making an opening incision
through the abnormally developed
trabecular meshwork to allow greater
outflow of the aqueous fluid and thereby,
lower the IOP .
Often 120 degrees (out of 360 degrees
total) of the trabecular meshwork can be
treated with goniotomy in a single setting.
25. Goniotomy. A fine surgical knife is used to open the drainage angle
(trabecular meshwork) in order to lower the intraocular pressure.
26. TRABECULOTOMY
If corneal clouding prevents visualization of
the angle or when repeated goniotomy has failed
Partial thickness scleral flap is fashioned
Schlemm canal is found and a trabeculotome is inserted into Schlemm
canal and then rotated into the anterior chamber
Technically highly demanding, requires previous experience, and good
anatomical landmarks to achieve predictable results
Schlemm canal may be difficult to canalize because of hypoplasia or
angle anomaly
27. Trabeculotomy surgery involves making an external incision and
identifying the Schlemm’s canal from the outside, inserting a fine
instrument into the Schlemm’s canal, and breaking through the
trabecular meshwork to increase the aqueous outflow .
Typically, 120-140 degrees of trabecular meshwork can be treated
by trabeculotomy in a single surgery.
28. If one surgical technique is unsuccessful in decreasing the IOP, the
other technique can be utilized in a fresh area of the trabecular
meshwork (the area not previously operated upon) to increase the
success of the surgery.
Even after initial control of the intraocular pressure is established with
surgery, a periodic monitoring is necessary to ensure the IOP doesn’t
increase again and the glaucoma go out of control.
29. Trabeculotomy. A trabeculotome instrument is used to
open the drainage angle (trabecular meshwork) in
order to lower the intraocular pressure.
30. TREATMENT
Medical
Temporary measures to control IOP and to clear cloudy
cornea prior to surgery
Administer beta-adrenergic antagonists, CAI’s(carbonic
anhydrase inhibitors).
avoid alpha2-adrenergic agonists under 3 years
occlude nasolacrimal drainage system for 2 minutes
after administration.
31. •Medications can be used as an adjunct therapy either before or after the surgical
treatment.
• Medications may be utilized temporarily after the diagnosis until surgery can be
performed. If the initial surgery fails to completely control the IOP, topical
medications can be used to bring the glaucoma under control.
• The systemic side effects of topical medications are greater in infants than in
adults because of the smaller body mass.
•Because of potential systemic side effects, the first line of medications that are
commonly employed is the topical carbonic anhydrase inhibitors.
32. • After the CAI, the next choices are topical prostaglandin analogs or beta-
blockers .
•The prostaglandin analogs (e g.latanoprast) appear to be safe in children;
however, there are no long-term data on the safety of these medications in
children. Topical beta-blockers(eg .timolol , carvedilol) should be used
with caution in children because of the well-known systemic side effects.
•Finally, topical alpha-2 agonist (brimonidine) should be AVOIDED in
infants because it’s been associated with severe respiratory depression
(breathing difficulty).
33. PROGNOSIS
Good in asymptomatic patients
diagnosed before 24 months.
Guarded in symptomatic patients diagnosed after
24 months even if IOP controlled after surgery.
