Congenital Glaucoma

History The discovery of the disease called glaucoma dates back to the 17th century. And it is the main cause of blindness

has been known since the 19th century world wide. In the Hippocratic Aphorisms the term "glaykoseis“ was used

to describe blindness that comes along with aging and a glazed appearance of the pupil.

Al- Tabari10th Century

Hippocratic treatmentDr. Richard Bannister

Oculist-1622

Definition & Anatomy

aqueous humor out flow in the anterior segment.

In people with glaucoma, the drainage canal is partially

or completely blocked. Fluid builds up in the chambers

and this increases pressure within the eye. High IOP can

damage the optic nerve and visual field.

Glaucoma is a pathogenic condition. And it is a group of eye diseases

in which the optic nerve is damaged leading to irreversible loss of

vision. In most cases, this damage is due to an increased pressure

within the eye.

Sequence Of Glaucoma

High pressure is not always dangerous, however in susceptible individuals high pressure can damage the optic

nerve, the nerve that transmit messages from the eyes to the brain. As the nerve fibers are damaged, the vision

begins to fail, starting with peripheral side vision. And if left untreated, may lead to total blindness.

Types of GlaucomaThere are several types of glaucoma. The two main types are open-angle

and angle-closure. These two types involve in increase of intraocular

pressure (IOP), or pressure inside the eye.

1- Open-angle glaucoma:

It caused by partial blockage of the drainage canal. The angle between the

cornea and the iris is "open", meaning the entrance to the drain is clear, but

the flow of aqueous humor is somewhat slow.

Types of Glaucoma

2- Close -angle glaucoma:

Caused by a sudden and complete blockage of aqueous humor

drainage. The IOP rises rapidly and may lead to total vision loss

quickly.

Demands immediate medical attention

Narrow Angle

Primary Congenital Glaucoma (PCG)

PCG refers to glaucoma resulting from maldevelopment of the aqueous outflow system. which has an isolated

poor development of the trabecular meshwork that is called : ISOLATED TRABECULODYSGENESIS

Barkan’s membrane theory - A thin, imperforate membrane covering the anterior chamber angle of the eyes

prevents aqueous humor outflow, and leads to increased IOP.

1- Flat anterior iris insertion 2- Concave iris insertion

Primary Congenital Glaucoma (PCG)

Signs and symptoms:1. Epiphoria

2. Photophobia

3. Blepharospasm

4. Buphthalmos

5. Haab striae

6. Corneal opacification

The optic nerve cupping may occur rapidly and early in infants

Also, it may be reversible with normalization of IOP.

Buphthalmos Corneal opacification

Haab striae

PCG Risk Factors

Although it is generally agreed that the IOP elevation in PCG is due to an abnormal development of the anterior chamber angle, there is no universal agreement on the subject. Below are some PCG abnormal theories:

Mann (1928)

Burian, and Braley (1955)

Maumenee (1958)

PCG resultfrom a developmental abnormality of anterior chamber angle

tissue derived from neural crest cells, leading to aqueousoutflow obstruction by one or more of several mechanisms.

PCG Treatments

1- Goniotomy:

is a surgical procedure in which the doctor uses a lens called a

goniolens to see the structures of the front part of the eye (A/C).

An opening is made in the trabecular meshwork, the new opening

provides a way for fluid to flow out of the eye.

PCG Treatments

2- Trabeculotomy:

A piece of tissue in the eye's drainage angle is removed to create

an opening. This new opening allows fluid (aqueous humor) to

drain out of the eye.

Trabeculotomy performed when cornea is cloudy.

Medications

Prostaglandin-related drugsXalatan® (latanoprost) 0.005% (qd)

Beta-blockersTimolol solution 0.25% (qd, bid)

Carbonic anhydrase inhibitorsDorzolamide 2% (bid, tid)

Medical therapy for pediatric glaucoma patients is often administered to reduce the IOP temporarily, to clear the cornea, and to facilitate surgical intervention.

Medication

the most serious systemic concerns associated with

topical glaucoma medication use in children.

Central nervous system (CNS) toxicity with alpha-2

agonists, Alphagan.

Post-treatment Some children with congenital glaucoma and elevated IOP respond to medical therapy. Which depend

on Structural defects ,Age, Systemic syndromes, Corneal clarity, Severity of glaucoma, and

Corneal diameter.

Between 3 and 6 weeks after surgery the postoperative control of the photophobia, tearing, and

Blepharospasm usually reflect the effectiveness of surgery.

Follow up visitation exams include, vision testing, external examination, refraction, ophthalmoscopy,

corneal assessment, and tonometry.

Visual rehabilitation is as important of in the management of disease as controlling IOP. Anisometropia

and Amblyopia must be manage to give the child best chance for good vision.

PCG Prevalence 60 % of pediatric glaucoma cases are diagnosed by the age of 6 months and 80% within the

first year of life. And 65% of patients are male.

PCG with elevated IOP occurs in about 1 out of 10,000 births and results in blindness in

approximately 10% of cases . And reduced vision (worse than 20/50) in about half of all

cases.

PCG is typically bilateral, although a significant IOP elevation may occur in only one eye in

25 to 30% of the cases.

In patient with clear cornea both procedure goniotomy & trabeculotomy yields a 70-80%

success rate.

Conclusion Glaucoma is one of the leading causes of blindness in the United States. It can occur at any age

but is more common in older adults.

Surgical therapy is the most effective and definitive form of treatment for the developmental

glaucoma.

The most common form of glaucoma has no warning signs in adult. In the other hand, the triad

signs in PCG should be considered seriously.

Elevated IOP – causes stretching and rupture of the zonules can cause lens subluxation. Blunt

trauma can cause hyphemas, retinal detachment, and rupture of the globe.