Pediatric Glaucoma

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Pediatric Glaucoma: Intro & Types - Tiny Eye Pressure

  • Optic neuropathy in children, often from ↑ Intraocular Pressure (IOP), leading to irreversible vision loss; early detection is key.
  • Types:
    • Primary Congenital Glaucoma (PCG): Developmental anomaly of trabecular meshwork. Onset birth to <3 years.
      • True congenital: IOP elevated in utero.
      • Infantile: Presents by 1-2 years.
    • Juvenile Open-Angle Glaucoma (JOAG): Onset >3-4 years up to 35 years.
    • Secondary Glaucomas: Result from other conditions (e.g., uveitis, trauma, syndromes, steroid-induced). Pediatric Glaucoma: Buphthalmos and Corneal Haze

⭐ Haab's striae (breaks in Descemet's membrane due to corneal stretching) are a pathognomonic sign of PCG presenting before age 2-3 years an exam favourite!

Pediatric Glaucoma: Pathophysiology & Genetics - Faulty Faucets

  • Pathophysiology: Trabeculodysgenesis (maldeveloped aqueous outflow pathways).
    • Abnormal Schlemm's canal & trabecular meshwork.
    • Leads to ↑ Intraocular Pressure (IOP).
  • Genetics: Key genes involved:
    • CYP1B1: Most common in Primary Congenital Glaucoma (PCG).
    • LTBP2, TEK (TIE2): Also linked to PCG.
    • MYOC: Associated with Juvenile OAG (JOAG). Trabeculodysgenesis and angle anatomy

CYP1B1 mutations are the most common genetic cause for primary congenital glaucoma (PCG).

Pediatric Glaucoma: Clinical Features - Tears & Big Eyes

  • Classic Triad: 📌 EPB
    • Epiphora (persistent, watery eyes)
    • Photophobia (marked light sensitivity)
    • Blepharospasm (forceful eyelid closure)
  • Key Signs of ↑IOP:
    • Buphthalmos ("ox eye"): Globe enlargement. Corneal diameter > 12mm at birth (increases with age).
    • Corneal edema/haze: Cloudy cornea, may obscure iris details.
    • Haab's striae: Horizontal/curvilinear tears in Descemet's membrane.
    • Elevated IOP: Typically > 21 mmHg.
    • Optic disc cupping: Progressive, initially reversible.
    • Axial myopia and astigmatism.

⭐ Haab's striae are a hallmark of congenital glaucoma, indicating corneal stretching due to elevated IOP.

Pediatric Glaucoma: Diagnosis - Anesthesia Peeks

Examination Under Anesthesia (EUA) is key for diagnosis.

  • Tonometry: Perkins (gold standard), Tonopen. IOP > 21 mmHg or asymmetry > 5 mmHg suspicious.
  • Corneal Diameter: Horizontal. > 11 mm (birth), > 12 mm (1 yr), or > 13 mm (any age) abnormal.
  • Gonioscopy: Direct (Koeppe, Swan-Jacob) for angle structures.
  • Ophthalmoscopy: Optic disc. C/D ratio > 0.3, asymmetry > 0.2, progressive cupping.
  • Axial Length: A-scan. ↑ indicates globe stretching from ↑IOP.
  • UBM: If corneal opacity obscures anterior segment.

Gonioscopy and Tonometry in Pediatric Glaucoma EUA

⭐ Haab's striae (breaks in Descemet's membrane) are pathognomonic for congenital glaucoma.

Pediatric Glaucoma: Management - Surgical First

Surgery is primary; medical therapy is often adjunctive/bridging.

  • Angle Surgery (1st Line):
    • Goniotomy (clear cornea)
    • Trabeculotomy (cloudy cornea)
  • Alternatives (if angle surgery fails/unsuitable):
    • Combined Trabeculotomy-Trabeculectomy (CTT)
    • Trabeculectomy + antifibrotics (MMC/5FU)
    • Glaucoma Drainage Devices (GDDs)
  • Last Resort: Cyclodestructive procedures.
  • Medical Therapy:
    • Topical: Beta-blockers, CAIs.
    • Systemic: CAIs.
    • ⚠️ AVOID alpha-2 agonists (infants/young children) - CNS depression risk.

⭐ Goniotomy is preferred for clear corneas; trabeculotomy if cloudy, allowing direct Schlemm's canal access.

High‑Yield Points - ⚡ Biggest Takeaways

  • Buphthalmos (corneal diameter > 12 mm at 1 year) and Haab's striae (Descemet's breaks) are key signs.
  • Primary Congenital Glaucoma (PCG) is most common, often bilateral, with autosomal recessive inheritance.
  • Classic triad: epiphora, photophobia, blepharospasm; also corneal edema/haze.
  • Diagnosis: Elevated IOP (> 21 mmHg), progressive optic disc cupping (C:D > 0.3 or asymmetry).
  • Treatment: Primarily surgical; goniotomy or trabeculotomy are preferred procedures.
  • Associated syndromes include Sturge-Weber, Neurofibromatosis, and Axenfeld-Rieger.

Practice Questions: Pediatric Glaucoma

Test your understanding with these related questions

A child presents with unilateral white reflex (leukocoria) and raised intraocular pressure, raising suspicion of retinoblastoma. Which of the following investigations is the most appropriate to perform?

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Flashcards: Pediatric Glaucoma

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What is the visual acuity of a newborn?_____

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What is the visual acuity of a newborn?_____

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