วันจันทร์ที่ 6 เมษายน พ.ศ. 2569

Congenital Laryngeal Anomalies / Laryngomalasia

Congenital Laryngeal Anomalies

🔎 Overview

  • เป็นความผิดปกติจาก embryogenesis error / intrauterine disruption
  • มัก present ด้วย
    👉 stridor + respiratory distress ตั้งแต่ neonatal period
  • ยิ่งโครงสร้างซับซ้อน → risk malformation สูง

🧠 Embryology (high-yield)

  • เริ่ม ~ day 25 → tracheobronchial groove
  • แยก trachea vs esophagus โดย tracheoesophageal septum
  • week 5–7 → larynx formed
  • Error → cleft, atresia, stenosis

📚 Common Congenital Laryngeal Disorders

1. Laryngomalacia (MOST COMMON)

🔑 Key points

  • ~ 2/3 ของ congenital laryngeal anomalies
  • Inspiratory stridor
  • Worse:
    • supine
    • feeding / crying
  • ดีขึ้นเมื่อ upright
  • onset: neonatal → peak infancy
  • หายเอง: 12–24 เดือน

🧪 Dx

  • Awake flexible laryngoscopy
    → supraglottic collapse

🩹 Tx

  • Mild → observe
  • Severe → supraglottoplasty

2. Vocal Cord Paralysis (2nd most common)

🔑 Presentation

  • Unilateral
    • weak cry
    • aspiration
  • Bilateral
    • biphasic stridor
    • respiratory distress (แต่ cry ปกติได้)

⚠️ Etiology

  • congenital / idiopathic
  • iatrogenic (cardiac surgery)
  • neurologic

🧪 Workup

  • Laryngoscopy + bronchoscopy
  • ± MRI brain / swallow study

3. Laryngeal Cysts

🟢 Vallecular cyst

  • location: base of tongue / epiglottis
  • sx:
    • stridor
    • feeding difficulty
    • FTT
  • severe → apnea / airway obstruction

🧪 Dx

  • flexible laryngoscopy ± CT

🩹 Tx

  • symptomatic → endoscopic excision / marsupialization

🟢 Saccular cyst / Laryngocele

Feature

Laryngocele

Saccular cyst

Content

Air

Fluid

Communication

Yes

No

Symptom

intermittent

constant
  • Tx → surgical excision

4. Laryngeal Webs & Atresia

🔑 Pathophysiology

  • failure of laryngeal recanalization

🧾 Presentation

  • stridor
  • abnormal cry / aphonia
  • severity ∝ % obstruction

🧪 Classification (glottic involvement)

  • Type 1: <35%
  • Type 2: 35–50%
  • Type 3: 50–75%
  • Type 4: >75%

⚠️ Associated

  • 22q11 deletion (DiGeorge)

🩹 Tx

  • mild → division
  • severe → reconstruction / tracheostomy

🚨 Laryngeal atresia (RARE, FATAL)

  • complete obstruction at birth
  • Dx prenatal: CHAOS syndrome
  • Tx:
    • EXIT procedure
    • emergent tracheostomy

5. Laryngeal Cleft

🔑 Pathophysiology

  • failure of fusion → communication larynx ↔ esophagus

🧾 Presentation

  • aspiration
  • dysphagia
  • recurrent pneumonia
  • ± stridor

🧪 Dx

  • VFSS (videofluoroscopic swallow study) / FEES (functional endoscopic evaluation of swallowing)
  • definitive: bronchoscopy

🧾 Classification (Benjamin)

  • Type I → mild
  • Type IV → severe (ถึง carina)

🩹 Tx

  • mild → conservative + GER control
  • severe → surgical repair

6. Congenital Subglottic Stenosis

🔑 Presentation

  • stridor infancy
  • recurrent croup
  • severe → respiratory distress

🧪 Grading (Myer-Cotton)

  • I: ≤50%
  • II: 51–70%
  • III: 71–99%
  • IV: complete

🩹 Tx

  • mild → observe
  • moderate → balloon dilation
  • severe → tracheostomy + LTR (laryngotracheal reconstruction)

7. Subglottic Hemangioma

🔑 Key features

  • vascular tumor (1–2%)
  • onset: infancy (rapid growth phase)

🧾 Presentation

  • stridor ± barking cough
  • ± cutaneous hemangioma (beard distribution)

🧪 Dx

  • laryngoscopy

🩹 Tx

  • Propranolol = first-line

🧠 Clinical Approach (high-yield summary)

👶 Neonate + stridor → think:

1.       Laryngomalacia (most common)

2.       Vocal cord paralysis

3.       Subglottic stenosis

4.       Hemangioma

5.       Structural anomaly (web, cleft)

🔍 Key differentiators

Clue

Suggest

Worse supine

Laryngomalacia

Weak cry

Vocal cord paralysis

Aspiration

Cleft

Progressive after birth

Hemangioma

Recurrent croup

Subglottic stenosis

🧪 Gold standard diagnosis

👉 Flexible laryngoscopy + bronchoscopy

🚨 Red flags

  • apnea
  • cyanosis
  • feeding intolerance
  • failure to thrive

🎯 Take-home

  • Congenital laryngeal disease = common cause of infant stridor
  • Laryngomalacia = most common & usually benign
  • ต้องแยก life-threatening lesions (atresia, severe stenosis, bilateral paralysis)
  • Diagnosis = direct airway visualization

🩺 Congenital Laryngomalacia

🔎 Definition & Epidemiology

  • Laryngomalacia = most common cause of chronic stridor in infants
  • กลไก: supraglottic tissue prolapse → glottic inlet ระหว่าง inspiration
  • คิดเป็น ~45–75% ของ infant stridor
  • พบ ~3–4/10,000 live births
  • ชาย > หญิง

⚙️ Pathophysiology (high-yield)

Multifactorial:

  • Neuromuscular immaturity (vagal reflex dysfunction)
  • Anatomic factors (redundant tissue, short aryepiglottic fold)
  • ± GER/LPR (พบร่วม 50–90%)

👉 core concept = supraglottic collapse during inspiration

🧠 Anatomy & Classification (Olney)

Type

Feature

Type 1

redundant arytenoid tissue

Type 2

short aryepiglottic folds + omega epiglottis

Type 3

posterior prolapse epiglottis

🧾 Clinical Features

👶 Typical presentation

  • Inspiratory stridor ตั้งแต่ newborn
  • peak: 4–8 เดือน
  • worse:
    • crying / feeding
    • supine
  • better: upright

👉 เสียงอาจ “wet” (สัมพันธ์กับ reflux)

⚠️ Not typical → ต้องคิด diagnosis อื่น

  • Expiratory stridor
  • Hoarseness

🍽️ Feeding-related symptoms

  • choking / coughing
  • regurgitation
  • poor feeding coordination

🚨 Severe features

  • apnea / cyanosis
  • retraction
  • failure to thrive

🧩 Associated Conditions

  • GERD / LPR (very common)
  • Neuromuscular disease
  • Genetic syndrome (Down, 22q11)
  • Other airway lesions (15–30%)

📈 Natural History

  • peak 4–8 เดือน
  • improve 12–18 เดือน
  • resolve ~<2 ปี (≈90%)

🧪 Diagnosis

✅ Clinical suspicion

  • Inspiratory stridor + worse supine + onset early

🔍 Gold standard

👉 Flexible laryngoscopy (awake)

Typical findings

  • omega-shaped epiglottis
  • supraglottic collapse

🧪 Additional evaluation (selected cases)

  • Swallow study → aspiration
  • Bronchoscopy → secondary lesion
  • PSG → sleep variant (older child)

📊 Severity Classification (clinical-based)

Severity

Features

Mild

intermittent stridor, growth OK

Moderate

persistent stridor + feeding difficulty

Severe

cyanosis, apnea, FTT, hypoxia

🩹 Management

🟢 Mild (MOST)

  • Observe
  • parental reassurance
  • feeding advice (upright, thickened feeds)

🟡 Moderate

  • multidisciplinary care
  • Medical management
    • acid suppression (H2RA/PPI)
    • feeding modification
    • nutrition support

👉 ~20–30% progress → surgery

🔴 Severe (ต้อง intervene)

Indications:

  • apnea / cyanosis
  • failure to thrive
  • respiratory distress
  • pulmonary HT / cor pulmonale

🔪 Surgery = Supraglottoplasty

  • ตัด redundant supraglottic tissue
  • success rate: 60–95%
  • improves:
    • breathing
    • feeding
    • growth

🛠️ Alternatives (selected cases)

  • CPAP / HFNC
  • NG/G-tube feeding

⚠️ Complications

  • aspiration
  • scarring
  • dysphonia (<10%)

🧠 Clinical Pearls (ใช้จริง)

  • Infant stridor → think laryngomalacia first
  • Inspiratory + worse supine = classic
  • ถ้ามี:
    • hoarseness
    • biphasic stridor
      👉 ต้องหา pathology อื่น
  • ต้อง exclude:
    • vocal cord paralysis
    • subglottic stenosis
    • hemangioma

🎯 Take-home

  • Most cases = benign, self-limited
  • Severity based on feeding + oxygenation
  • Flexible laryngoscopy = diagnostic
  • Surgery reserved for severe disease
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