Ophthalmology Spot Diagnosis: Buphthalmos

buphthalmos glaucoma

A. Synoynms: Hydrothalmos, Congenital glaucoma

B. Definiton: Buphthalmos is the enlargement of the eye due to congenital glaucoma.

C. Classification:

  1. True congenital glaucoma (Increased intraocular pressure (IOP) during intrauterine life)
  2. Infantile glaucoma (Birth to 3 years)
  3. Juvenile glaucoma (>3 years)

D. Pathogenesis:

  1. Isolated trabeculo-dysgenesis: Absence of angle recess; iris inserted directly into surface of trabeculum
    • Revealed by Gonioscopy as Barkan’s membrane
  2. Flat or concave iris insertion

E. Clinical features:

1. Corneal haze: due to corneal epithelial/stromal edema

  • With lacrimation, photophobia and blepharospasm

2. Buphthalmos: In birth to 3 years

  • Enlarged eye due to stretching when IOP increases
  • Stretched sclera – thin and translucent (appears blue)
  • Deep Anterior Chamber (AC)
  • Zonular fibers stretch and lens subluxate
  • Axial myopia can cause amblyopia if untreated

3. Haab striae:

  • Horizontal or circular curvilinear lines
  • Healed breaks in Descemet’s membrane

4. Optic disc cupping may regress if treated early:

  • Cup to disc ratio > 0.3
  • Scleral canal enlarges
  • Lamina cribrosa may bow posteriorly

Possible causes of vision loss in Buphthalmos:

  1. Optic damage
  2. Corneal scarring
  3. Amblyopia
  4. Cataract
  5. Lens subluxation

F. Differential diagnoses:

1. Cloudy cornea:

  • Mucopolysaccharidoses
  • Birth trauma
  • Congenital hereditary endothelial dystrophy
  • Sclerocornea
  • Keratitis (Rubella)

2. Megalocornea (Myopia)

3. Lacrimation (Nasolacrimal duct obstruction)

4. Secondary infantile glaucoma:

  • Retinoblastoma
  • Juvenile Xanthogranuloma
  • Persistent Hyperplastic Primary Vitreous (PHPV)
  • Retinopathy of Prematurity (ROP)
  • Trauma
  • Ectopia lentis

G. Evaluation:

  1. IOP with Perkin’s tonometer/tonopen
  2. Corneal diameter (to rule out megalocornea)
  3. Gonioscopy with a koeppe lens
  4. Retinoscopy
  5. Optic disc evaluation

H. Surgical treatment:

  1. Medical treatment before surgery:
    • Beta-blockers and ACE inhibitors
    • Avoid alpha 2-agonists (may cause sleep apnea and respiratory failure)
  2. Goniotomy, Trabeculotomy
  3. Trabeculectomy + Trabeculotomy
  4. Aqueous drainage implants
  5. Trans-scleral cyclophotocoagulation

I. Follow up:

  1. Life-long
  2. Appropriate refractive and amblyopia therapy
  3. Treatment of media opacities like corneal scars and cataracts

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