CONGENITAL / DEVELOPMENTAL GLAUCOMA
CONGENITAL/DEVELOPMENTAL GLAUCOMA (BUPHTHALMOS)
- Due to failure or abnormal developmental of the trabecular meshwork.
- The iris may not be completely spared from cornea so that the angle remains closed by persistent embryonic tissue.
- If glaucoma is present at birth or within 3-4 years after birth it is called congenital or infantile glaucoma
- When it is present b/w 3-10 years it is c/d Juvenile glaucoma.
- Congenital glaucoma may or may not be associated with other syndrome.
- Accordingly congenital glaucoma is classified as following:
1. Primary congenital/ Developmental glaucoma:
- Except for glaucoma there is no other ocular or systemic anomaly.
- Congenital: Disease manifests at birth or within one month.
- Infantile: Presents b/w one month to 3-4 years of age.
- Developmental or Late congenital or Juvenile: Presents b/w 4-10 years of age.
2. Secondary congenital Glaucoma:
- Glaucoma associated with ocular anomalies & systemic syndrome.
- iridocorneal dysgenesis
- Struge-weber syndrome
- Von-Recklinghausen’s neurofibromatosis
- Lowe’s syndrome
- congenital microcornea
- Rubella syndrome
PRIMARY CONGENITAL GLAUCOMA
- Disease is bilateral
- Boys affected more than girls.
Common symptopms are:
- photophobia, blepharospasm
- lacrimation & eye rubbing
On examination following findings are there:
- 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 Descemet’s membrane)
- Cornea is hazy with frosted glass appearance.
- Corneal enlargement
- Thin & blue sclera
- Deep anterior chamber
- Lens is antero-posterior flat & may be subluxated backward.
- Iridodonesis (tremulous iris) & atrophic patch on iris.
- Large eye (Buphthalmos or hydrophthalmos)
- Variable cupping & atrophy of disc
- Raised IOP (niether acute nor marked)
- Axial myopia due to increased axial length which may give rise to anisometropic amblyopia.
TREATMENT OF CONGENITAL GLAUCOMA
- Initial treatment is often surgical.
- A drainage angle surgery is often recommended for congenital glaucoma.
Surgical procedures used are:
- Trabeculotomy→ can be performed with corneal clouding
- Combined trabeculotomy & trabeculectomy
- Medications are not very effective.
- IOP is lowered by use of hyperosmotic agents
- Acetazolamide & beta blockers till surgery is taken up.
- Congenital glaucoma presents as Photophobia.
- Incidence of congenital glaucoma is 1 in 10,000 births.
- In buphthalmos, lens is Anteroposterior flat.
- Habbs striae are seen in Buphthalmos.
- Photophobia in an infant could be due to Buphthalmos.
- Most common symptom in buphthalmos is Lacrimation.
- Initial treatment of buphthalmos is Goniotomy.
- The treatment of congenital glaucoma is Trabeculotomy with trabeculectomy.