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

Primary congenital glaucoma(PCG) :

Characterized by elevated intraocular pressure (IOP), enlargement of the globe (buphthalmos), edema, and opacification of the cornea with rupture of Descemet\’s membrane (Haab’s striae), thinning of the anterior sclera and iris atrophy, anomalously deep anterior chamber, and structurally normal posterior segment except for progressive glaucomatous optic atrophy.

It can be classified as follows:

• True congenital glaucoma (40%) in which intraocular pressure (IOP) is elevated during intrauterine life.
• Infantile glaucoma (55%), which manifests prior to age 3.
• Juvenile glaucoma, the least common, in which IOP rises between 3 and 16 years of age.
• Several genes have been implicated, prominently CYP1B1.

Treatment

• Management is essentially surgical.
• Goniotomy
• Trabeculotomy
• Other procedures when angle surgery fails include trabeculectomy, tube shunt implantation, and ciliary body ablative procedures.

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

Primary congenital glaucoma(PCG) :

• Characterized by elevated intraocular pressure (IOP), enlargement of the globe (buphthalmos), edema, and opacification of the cornea with rupture of Descemet\’s membrane (Haab’s striae), thinning of the anterior sclera and iris atrophy, anomalously deep anterior chamber, and structurally normal posterior segment except for progressive glaucomatous optic atrophy.

It can be classified as follows:

• True congenital glaucoma (40%) in which intraocular pressure (IOP) is elevated during intrauterine life.
• Infantile glaucoma (55%), which manifests before age 3.
• Juvenile glaucoma, the least common, in which IOP rises between 3 and 16 years of age.
• Several genes have been implicated, prominently CYP1B1.

Treatment

• Management is essentially surgical.
• Goniotomy
• Trabeculectomy
• Other procedures when angle surgery fails include trabeculectomy, tube shunt implantation, and ciliary body ablative procedures.

D i.e. Congenital Glaucoma

>This is a rare condition that may be inherited, caused by incorrect development of the eye’s drainage system before birth. This leads to increased intraocular pressure, which in turn damages the optic nerve.

C i.e. Congenital glaucoma

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

Primary congenital glaucoma(PCG) :

Characterized by elevated intraocular pressure (IOP), enlargement of the globe (buphthalmos), edema, and opacification of the cornea with rupture of Descemet\’s membrane (Haab’s striae), thinning of the anterior sclera and iris atrophy, anomalously deep anterior chamber, and structurally normal posterior segment except for progressive glaucomatous optic atrophy.

It can be classified as follows:
 True congenital glaucoma (40%) in which intraocular pressure (IOP) is elevated during intrauterine life.
 Infantile glaucoma (55%), which manifests prior to age 3.
 Juvenile glaucoma, the least common, in which IOP rises between 3 and 16 years of age.

 Several genes have been implicated, prominently CYP1B1.

Treatment
 Management is essentially surgical.
 Goniotomy
 Trabeculotomy
 Other procedures when angle surgery fails include trabeculectomy, tube shunt implantation, and ciliary body ablative procedures.

 Ans.B i.e. Habb\’s striae

• Haab’s striae are horizontal/circumferential breaks in Descemet’s membrane seen in buphthalmos.
• Buphthalmos (Bull-like eyes) is enlargement of the eyeball seen in true congenital and infantile glaucoma, usually when the disease onset is before 3 years.
• The classical symptoms of congenital and infantile glaucomas include tearing, photophobia, and irritability. 
• The parents may notice a hazy cornea or an increase in the size of the cornea. 
• On examination, clinicians notice that there is increased corneal diameter, deep anterior chamber, and an increase in the size of the globe.
•  Corneal examination reveals the presence of corneal edema, with ruptures of Descemet\’s membrane known as Haab\’s striae. 
• The intraocular pressure is elevated, and there may be optic disc cupping.

Image demonstrating Haab\’s striae:

Note:

• Buphthalmos is usually not seen in glaucoma with onset after the age of 3 years. That is why juvenile onset (3 years to teenage years) glaucoma is not associated with buphthalmos.
• Other conditions causing buphthalmos: Aniridia, Neurofibromatosis type 1, Sturge-Weber syndrome.

C i.e. Small cornea

A i.e. Bupthalmos

Buphthalmos (Bull-like eyes) is enlargement of the eyeball seen in true congenital and infantile glaucoma, usually when the disease onset is before 3 years.

• The classical symptoms of congenital and infantile glaucomas include tearing, photophobia, and irritability.
• The parents may notice a hazy cornea or an increase in the size of the cornea.
• On examination, clinicians notice that there is increased corneal diameter, deep anterior chamber, and an increase in the size of the globe.
• Corneal examination reveals the presence of corneal edema, with ruptures of Descemet\’s membrane known as Haab\’s striae.
• The intraocular pressure is elevated, and there may be optic disc cupping.
• Stretched sclera – thin and translucent (appears blue)
• Deep Anterior Chamber (AC)
• Zonular fibers stretch and lens subluxate
• Axial myopia can cause amblyopia if untreated.

Management is essentially surgical  due to significant anatomical anomaly of the anterior drainage angle, medical treatment has a limited role in controlling the IOP in PCG,
 Goniotomy: Goniotomy reduces IOP by cutting into the abnormal trabecular meshwork, allowing the iris to drop posteriorly to deepen the angle recess.A clear cornea is a prerequisite.
 Trabeculotomy: Trabeculotomy or goniotomy ab externo, lowerS the IOP through inserting a trabeculotome into the Schlemm’s canal, which then tears through trabecular meshwork into the anterior chamber.

Combined Trabeculotomy-Trabeculectomy: Trabeculotomy is combined with trabeculectomy and is performed, in PCG patients, with trabeculectomy performed after trabeculotomy.

 Other procedures when angle surgery fails to include tube shunt implantation, and ciliary body ablative procedures.

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 :-

* Goniotomy

* Trabeculotomy  can be performed with corneal clouding

* Trabeculcctomy

* 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 

The commonest symptom is watering\”

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 the cornea (hazy cornea)
• Haab striae
• Large cornea
• Deep anterior chamber
• Lens anteroposteriorly flat