Congenital glaucoma

2021-02-02 12:00 AM

Opaque cornea: congenital keratitis (caused by ruby, herpes), congenital corneal endothelial dystrophy, mucopolysaccharide metabolic disorder, obstetric trauma.

Congenital glaucoma is the most common form of glaucoma in a group of glaucoma in children. Congenital glaucoma can be primary (due to structural abnormalities of the anterior angle) or secondary (with abnormalities in other parts of the eye). Early detection and treatment of congenital glaucoma can prevent blindness.

Congenital glaucoma is mostly detected late after birth, with a higher prevalence rate in boys (65%) compared with girls (35%). The disease is usually in the two eyes with different degrees. The most common symptoms are photophobia, watery eyes, and curled eyelashes. These symptoms often attract the attention of the patient's parents.

Signs of congenital glaucoma include

Enlarged cornea: High eye pressure causes the cornea to expand. The transverse diameter of the normal cornea in newborn babies is 9.5 to 10.5 mm and in children 1-year-old it is 10 to 11.5 mm. A diameter of the cornea 1 mm larger than normal is a sign of glaucoma.

Corneal edema: can come on suddenly or slowly. The edematous cornea becomes opaque. At first, edema is only present in the epithelium, then spread to the parenchyma. Corneal edema may be accompanied by cracks of the Cartesian membrane, which are lines in the horizontal direction or concentric with the margin (Figure 1.2). Descemet membranes can scar the cornea if parenchymal edema is persistent.

Thinning of the sclera: dilated eyeballs make the sclera thinner, revealing the blue color of the lower darker (bulging buffalo eye).

Other signs: the anterior chamber is often deeper than usual, and maybe nearsightedness or astigmatism, and vitreous distortion due to sclera.

Enlarged cornea (Congenital glaucoma)

Descemet membrance tear (Congenital glaucoma)

Diagnosis of congenital glaucoma

Corneal signs: cornea enlargement, possibly edema with cracks of the Cartesian membrane.

Eye pressure: usually high, between 30- and 40-mm Hg. The difference in eye pressure between the eyes is also a sign of congenital glaucoma. The eye pressure should be measured with a local anesthetic, as the anesthetic changes the eye pressure (usually lower).

Pre-room angle: in children, the premise is best with Koeppel contact lenses. In congenital glaucoma, the typical picture of the anterior chamber is the iris attaching to the anterior angle, the structures of the angle (ciliary band, raft, sclera) are more visible.

Neuropathic damage: larger disc concave than normal (3/10 disc/plate diameter ratio) or concave disc imbalance in 2 eyes

Differential diagnosis

Some other diseases may have congenital glaucoma-like signs and symptoms that require differential diagnosis, these are:

Enlarged cornea: congenital enlarged corneal disease, axial myopia.

Opaque cornea: congenital keratitis (caused by ruby, herpes), congenital corneal endothelial dystrophy, mucopolysaccharide metabolic disorder, obstetric trauma.

Large disc concave: visual disc defect, nerve atrophy, neurologic atrophy, wide physiological disc concave.

Watery eyes: due to congenital obstruction, damage to the corneal epithelium.


The pathogenesis of congenital glaucoma is due to the underdevelopment of the sclera-sclera (raft dysplasia) in which there is no anterior chamber and the iris attaches directly to the raft area, which may obscure a part or all of the raft area.

Dyspalasia of the raft area (Congenital glaucoma)


Specific congenital glaucoma forms

In addition to the primary congenital form of glaucoma, congenital glaucoma can also be associated with a number of iris and corneal dystrophy:

Axenfeld anomalies: the Schwalbe line is prominent and develops forward (posterior corneal ring), where the iris bands attach.

Rieger anomalies: Axenfeld anomalies accompanied by iris anomalies such as iris parenchymal dysplasia, pupil misalignment, multiple pupils (Figure 1.4), uveous flux (iris chromatographic epithelium flipped in front of near pupil).

Rieger's syndrome: Rieger's abnormality is accompanied by systemic abnormalities such as small and sparse teeth, dysplasia of the lower jaw, distant eyes.

Peters syndrome: central corneal cataract, iris sticking or vitreous to back cornea, small eyeball, iris minimization.

Disability without iris: partially or completely defect iris, accompanied by corneal cataract, small cornea, corneal iris stick.  

Atrophy of iris and Peter anomalies (Congenital glaucoma)

Treatment of congenital glaucoma is mainly surgical. Medication treatment is usually just in preparation for surgery or supplementation after surgery.

Surgical treatment

The following surgeries may be used for congenital glaucoma:

Goniotomy: An opening of the anterior chamber (during angular examination) with a small needle-shaped needle. This surgery can only be done while the cornea is still clear.

Trabeculectomy: an incision from the outside, exposing the Schlemm tube, inserting a wire-like device into the Schlemm tube, then splitting into the raft area. The purpose of surgery is to open the opening between the anterior chamber and the Schlemm tube. This surgery is usually indicated when the cornea is opaque and cannot see the anterior chamber angle.

Trabeculectomy: opening the opening between the anterior chamber and the submucosa. The opening is protected by a thin sclera. Raising surgery may be accompanied by the use of anti-fibrosis drugs (mitomycin C, 5-Fluorouracil) in cases where there is a risk of failure.

Other surgeries: in some special cases other surgeries may be used such as laser ablation, electrolysis or cryotherapy of the ciliary area, or insertion of aqueous drainage valves (Molten, Baer veldt, etc.).

Drug treatment

Possible antihypertensive drugs for congenital glaucoma include carbonic anhydrase inhibitors (oral acetazolamide or ophthalmic dorzolamide) and beta-blockers (such as timolol or betaxolol).

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