Pediatric Glaucoma

Pediatric Glaucoma

🔑 Definition

• กลุ่มโรค optic neuropathy → optic disc cupping + visual field loss
• อาจมีหรือไม่มี IOP สูง
• untreated → peripheral → central vision loss → blindness

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🧠 Key Differences จาก Adult

• ในเด็ก:
• ตา ยืดได้ → globe enlargement (buphthalmos)
• มีผลต่อ visual development → amblyopia, refractive error
• ต้อง diagnose & refer เร็ว เพื่อ preserve vision

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🧩 Classification (high-yield)

1. Primary glaucoma

👶 Primary infantile glaucoma

• onset: birth–2-3 ปี
• cause: trabeculodysgenesis
• ยิ่ง onset เร็ว → disease รุนแรงกว่า

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🧑 Juvenile glaucoma (JOAG)

• onset >4–5 ปี → young adult
• inheritance: autosomal dominant (MYOC mutation)
• คล้าย adult POAG

Clinical:

• asymptomatic
• IOP สูงมาก
• ไม่มี buphthalmos
• อาจมาด้วย vision loss / cupping

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2. Secondary glaucoma

แบ่งตาม cause

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🔍 Causes of Secondary Pediatric Glaucoma (ต้องจำ)

🧬 1. Angle anomalies / developmental

• Sturge-Weber syndrome
• Aniridia (PAX6)
• Anterior segment dysgenesis (Axenfeld-Rieger, Peters)

👉 กลไก: abnormal angle → ↓ outflow
👉 incidence glaucoma สูง (~40–60% ใน SWS)

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👁️ 2. Post-surgical

Aphakic glaucoma (หลัง cataract surgery)

• incidence:
• ~17% ใน 4–5 ปี
• ↑ ตามเวลาติดตาม
• risk:
• surgery early (<4 wk)
• ocular anomalies

👉 ต้อง follow lifelong

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🧠 3. Inflammatory

• uveitis (JIA, sarcoid)
• mechanism:
• trabecular blockage
• scarring

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👶 4. ROP (Retinopathy of prematurity)

• late complication
• mechanism:
• lens-iris displacement
• angle closure

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🧬 5. Tumor-related

• เช่น retinoblastoma
• mechanism:
• angle infiltration
• hemorrhage
• angle closure

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💥 6. Trauma

• hyphema → blockage
• angle damage → chronic glaucoma

⚠️ sickle cell → IOP สูงมาก even small hyphema → early surgery

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💊 7. Steroid-induced

• topical > systemic
• reversible เมื่อหยุดยา

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🔬 Diagnosis

Key components

• IOP measurement
• Fundoscopy → cupping
• Visual field (ถ้าทำได้)
• ± OCT

👉 ในเด็กเล็ก:

• ต้อง rely on:
• corneal size
• tearing / photophobia
• exam under anesthesia

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⚠️ Clinical Presentation

Infantile glaucoma (classic triad)

• tearing
• photophobia
• blepharospasm

• corneal enlargement

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Juvenile glaucoma

• asymptomatic
• progressive visual loss
• cupping

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🔪 Treatment (สำคัญมาก)

🥇 Mainstay = Surgery

• แตกต่างจาก adult

Primary / angle anomaly

• goniotomy / trabeculotomy (first-line)

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💊 Medical therapy

• role = adjunct
• ใช้ก่อน surgery หรือในบาง secondary cases

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🧩 Specific approach

Condition	Treatment
Primary infantile	surgery (angle surgery)
Juvenile glaucoma	similar to adult (med ± surgery)
SWS / angle anomaly	surgery เป็นหลัก
Aphakic glaucoma	trial medical → surgery
Uveitis	control inflammation + IOP
ROP	treat cause (± lensectomy)

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🔁 Monitoring

• lifelong follow-up
• monitor:
• IOP
• optic nerve
• refraction
• visual development

👉 aphakic glaucoma อาจเกิด หลายปีหลัง surgery

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🧠 Clinical Pearls Pediatric glaucoma = surgical disease early diagnosis = critical (prevent amblyopia + blindness) infant → buphthalmos + tearing + photophobia juvenile → silent like adult aphakia = high-risk → follow lifelong secondary causes ต้องคิดเสมอ (SWS, aniridia, ROP)

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Primary Infantile Glaucoma

🔑 Definition

• glaucoma onset birth–<2–3 ปี
• สาเหตุ: angle dysgenesis → ↓ aqueous outflow → ↑IOP
• ทำให้:
• optic nerve damage
• globe enlargement (เฉพาะเด็กเล็ก)

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📊 Epidemiology

• incidence ~ 1:10,000
• bilateral > 2/3 cases
• inheritance:
• mostly sporadic
• ~10–27% familial (AR, CYP1B1)

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⚙️ Pathophysiology (high-yield)

• abnormal trabecular meshwork development
• neural crest defect
• → ↑IOP

👉 เด็ก <2–3 ปี:

• sclera + cornea ยืดได้ → buphthalmos

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⚠️ Clinical Presentation

👶 Classic triad

• epiphora (tearing)
• photophobia
• blepharospasm

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🔍 Exam findings (must know)

1. Corneal enlargement

• 12 mm ใน infant = abnormal
• asymmetry >1–2 mm → suspicious

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2. Corneal changes

• edema → clouding
• Haab striae (Descemet membrane rupture)

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3. Optic nerve

• cupping >0.3 = abnormal in infant
• asymmetry >0.2 สำคัญมาก

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4. Globe enlargement

• buphthalmos
• → axial myopia

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5. Refractive / amblyopia risk

• astigmatism
• anisometropia
• deprivation amblyopia

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🔬 Diagnosis

Based on constellation:

• enlarged cornea
• corneal edema
• optic disc cupping

👉 IOP:

• supportive but not required

👉 บางรายต้อง:

• exam under anesthesia

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🔄 Differential Diagnosis (เจอบ่อย)

Symptom	DDx
tearing	NLD obstruction (ไม่มี photophobia)
corneal clouding	congenital endothelial dystrophy, MPS
large cornea	megalocornea (IOP ปกติ)
cupping	physiologic / optic nerve anomaly

 

 

 

                         
👉 key:

• glaucoma = มีครบ triad + corneal enlargement

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🔪 Treatment (core concept)

🥇 Mainstay = Surgery

First-line

• Goniotomy
• Trabeculotomy

👉 success ~80–90% (IOP <20)

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Second-line

• Trabeculectomy
• Drainage device
• Cyclodestruction

👉 success ~50–65%

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💊 Medical therapy

• role:
• bridge → pre-op
• adjunct → post-op

Drugs used

• beta-blocker
• CAI
• prostaglandin
• α2-agonist (⚠️ หลีกเลี่ยง <2 ปี → CNS depression)

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📈 Prognosis

• VA ≥20/60 ได้ใน ~50–80%
• poor outcome:
• early onset (<3 เดือน)
• amblyopia
• associated anomalies

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🔁 Follow-up

• lifelong
• risk:
• relapse years later
• need re-op ~20–30%

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🧠 Clinical Pearls

• infant glaucoma = surgical emergency (relative)
• triad: tearing + photophobia + blepharospasm
• cornea >12 mm in infant → urgent referral
• cupping >0.3 = abnormal (ต่างจาก adult)
• amblyopia = major cause of poor vision (ต้อง treat พร้อมกัน)
• IOP ไม่ใช่ key diagnosis → clinical features สำคัญกว่า

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