Congenital / Developmental Glaucoma

In this scenario signs and symptoms occur as a result of congenital glaucoma.

D i.e. Congenital Glaucoma

C i.e. Congenital glaucoma

A i.e. Large cornea; B i.e. Habb’s stria

C i.e. Small cornea

A i.e. Bupthalmos

C i.e. Trabeculotomy with trabeculectomy

–      Marked photophobia, blepharospasm, watering with blue and large eye (bupthalmos), hazy frosted glass large cornea with Habb’s striaeQ and backward subluxated anteroposteriorly flat lensQ are seen in congenital or infantile glucoma.

–      Congenital glaucoma is characterized by

–      Therefore, enlarged eye globe (causing lid lag) with enlarged corneal diameter (>_13 mm) with characteristic double contour round opacities concentric to limbus (i.e. Habb’s stria) suggest the diagnosis of congenital glaucoma.

–        5 Bs: Boys (more affected), Bilateral, Blepharospasm, Blue, Bull (ox or large) eye.

–    Photophobia & lacrimationQ

–    Cornea is large, hazy (frosted glass appearance) with Habb striae (double contoured opacities concentric to limbus) & diminished sensation.Q

–    Lens is anteroposteriorly flat & backward subluxated Q

• On the basis of cause

Primary congenital glaucoma is due to failure or abnormal development of trabecular meshwork e.g.

Presence of cellular Barkan’s membrane

Abnormal cleavage of anterior chamber

Iris may not completely separate from the cornea so that the angle remains closed by persistent embryonic tissue

Secondary congenital glaucoma is developmental glaucoma with associated ocular anomalies e.g.

– Aniridia (50%), iridocorneal dysgenesis

–         Ectopia lentis syndrome (eg Marfan/Weil-Marchesani syndrome, homocystinuria) & phakomatosis (Sturge

weber/ Von RecklingHusen syndrome)

– Neurofibromatosis

–         Sturge – Weber Syndrome (cavernous haemangiomas of eye & brain and cutaneous angioma of face)

– Rubella syndrome, Lowe’s syndrome, Congenital Microcornea, naevus of ota.

Mesodermal dysgenesis (Rieger’s & Peter’s anomaly)

Ans. 1 in 10,000 births

Ans. Anteroposterior flat

Ans. Photophobia

Ans. is ‘b’ i.e., Goniotomy

Treatment of congenital glaucoma

Unlike adult glaucoma. the initial treatment for congenital glaucoma is often surgical. A drainage angle surgery is often recommended for congenital glaucoma.

• Surgical procedures used are :-

1. Goniotomy
2. Trabeculotomy -+ can be performed with corneal clouding
3. Trabeculcctomy
4. Combined trabeculotomy and trabeculectomy

• Medications are not very effective. However, IOP is lowered by use of hyperosmotic agents, acetazolamide and beta- blockers till surgery is taken up. Miotics are of no use.

Ans. is ‘a’ i.e., Buphthalmos

Examination findings of primary congenital glaucoma (buphthalmos)

• First sign is corneal edema with watering of eye with marked photophobia
• Haab striae (Discrete corneal opacities appear as lines with double contour due to rupture in Descemets membrane)
• Cornea is hazy with frosted glass appearance.
• Corneal enlargement
• Thin & blue sclera
• Deep anterior chamber
• Lens is antero- posteriorly flat and may be subluxated backward
• Iridodonesis (tremulous iris) and atrophic patch on iris
• Large eye (Buphthalmos or hydrophthalmos)
• Variable cupping and atrophy of disc
• Raised IOP (neither acute nor marked)
• Axial myopia due to increased axial length which may give rise to anisometropic amblyopia

Ans. is ‘a’ i.e., Lacrimation 

Commonest symptom is watering”__ Mukherjee

In Buphthalmos

• Most common symptom →  Watering (lacrimation)
• 2^nd most common symptom →  Photophobia
• Most troublesome symptom →  Photophobia (Child avoids light) o First sign→ Corneal edema with watering
• Frosted glass appearance of cornea (hazy cornea)
• Haab striae
• Large cornea
• Deep anterior chamber
• Lens antero-posteriorly flat