Airway Malformations in Children Laryngomalacia Subglottic Stenosis Congenital Lung Malformations and Foreign Body Aspiration in Paediatrics
Paediatrics

Airway Malformations in Children Laryngomalacia Subglottic Stenosis Congenital Lung Malformations and Foreign Body Aspiration in Paediatrics

Below is a concise but complete paediatrics reference for airway malformations and foreign body airway obstruction as requested (no images).


Airway Malformations and Foreign Body in Airway (Paediatrics)

1. Laryngomalacia

Definition

Laryngomalacia is the most common congenital laryngeal anomaly, characterized by collapse of supraglottic structures during inspiration, leading to stridor in infants.

Pathophysiology

Immature laryngeal cartilage and abnormal neuromuscular tone cause the epiglottis and arytenoid structures to collapse inward during inspiration, producing airway obstruction and inspiratory stridor.

Causes / Risk Factors

  • Congenital structural weakness
  • Neuromuscular immaturity
  • Associated conditions:

* Gastroesophageal reflux disease (GERD)

* Neurological disorders

* Prematurity

Clinical Features

  • Inspiratory stridor (appears at 2–4 weeks of age)
  • Worse when:

* Supine

* Feeding

* Crying

  • Improves when prone
  • Feeding difficulty
  • Failure to thrive (severe cases)
  • Apnea or cyanosis (rare)

Investigations / Diagnosis

  • Flexible laryngoscopy (gold standard)

* Omega-shaped epiglottis

* Collapse of arytenoids

  • Pulse oximetry
  • Sleep study if severe
  • Evaluate for GERD

Differential Diagnosis

  • Vocal cord paralysis
  • Subglottic stenosis
  • Tracheomalacia
  • Vascular rings
  • Foreign body aspiration

Management

Conservative

Most cases resolve by 12–18 months.

Non-pharmacologic:

  • Upright feeding
  • Thickened feeds
  • GERD management

Pharmacologic

Omeprazole

Indication

GERD associated with laryngomalacia

Mechanism

Proton pump inhibitor → blocks gastric acid secretion

Dose

Infants: 0.5–1 mg/kg/day

Adverse Effects

  • Diarrhea
  • Headache
  • Vitamin B12 deficiency (long term)

Monitoring

Symptoms and weight gain

Surgical

Supraglottoplasty

Indications:

  • Severe airway obstruction
  • Failure to thrive
  • Apnea
  • Hypoxia

2. Congenital Subglottic Stenosis

Definition

Narrowing of the subglottic airway below the vocal cords, either congenital or acquired.

Pathophysiology

Failure of normal airway recanalization results in reduced airway diameter, leading to airflow obstruction.

Clinical Features

  • Biphasic stridor
  • Respiratory distress
  • Recurrent croup
  • Difficulty breathing

Diagnosis

  • Rigid bronchoscopy (gold standard)
  • CT airway
  • Laryngoscopy

Severity Classification

Cotton-Myer grading:

| Grade | Airway obstruction |

| ----- | ------------------ |

| I | <50% |

| II | 51–70% |

| III | 71–99% |

| IV | Complete |

Management

Mild

  • Observation
  • Treat infections

Moderate

  • Endoscopic dilation
  • Steroid injection

Severe

  • Laryngotracheal reconstruction
  • Tracheostomy

3. Congenital Lobar Emphysema (CLE)

Definition

Overinflation of a pulmonary lobe due to air trapping from bronchial cartilage weakness.

Most Common Lobes

  • Left upper lobe
  • Right middle lobe

Pathophysiology

Air enters during inspiration but cannot exit during expiration → progressive hyperinflation.

Clinical Features

  • Neonatal respiratory distress
  • Tachypnea
  • Cyanosis
  • Hyperresonant chest
  • Mediastinal shift

Diagnosis

Chest X-ray:

  • Hyperlucent lobe
  • Mediastinal shift

CT scan

  • Confirms diagnosis

Differential Diagnosis

  • Pneumothorax
  • CPAM
  • Pulmonary sequestration

Management

Mild cases

  • Observation

Severe cases

  • Lobectomy

4. Congenital Pulmonary Adenomatoid Malformation (CPAM)

Previously called Congenital Cystic Adenomatoid Malformation (CCAM).

Definition

Developmental lung lesion characterized by abnormal cystic lung tissue replacing normal alveoli.

Types (Stocker classification simplified)

| Type | Description |

| -------- | -------------------- |

| Type I | Large cysts |

| Type II | Multiple small cysts |

| Type III | Solid mass |

Clinical Features

  • Respiratory distress in neonates
  • Recurrent pneumonia
  • Asymptomatic (prenatal diagnosis)

Diagnosis

Prenatal

  • Fetal ultrasound

Postnatal

  • Chest CT

Complications

  • Infection
  • Pneumothorax
  • Malignancy risk (rare)

Management

  • Surgical resection (lobectomy)
  • Even if asymptomatic (often recommended)

5. Pulmonary Sequestration

Definition

A mass of nonfunctional lung tissue that does not connect to the tracheobronchial tree and receives blood supply from the systemic circulation (usually aorta).

Types

Intralobar

  • Within normal lung
  • Shares pleura

Extralobar

  • Separate pleura
  • Often diagnosed in infancy

Pathophysiology

Abnormal lung bud development leads to isolated lung tissue with systemic arterial supply.

Clinical Features

  • Recurrent pneumonia
  • Respiratory distress
  • Chronic cough

Diagnosis

CT angiography

Shows abnormal systemic artery from aorta

Management

  • Surgical resection
  • Endovascular embolization (selected cases)

6. Foreign Body in the Airway (Paediatrics)

Definition

Inhalation of a foreign object causing partial or complete airway obstruction.

Common Objects

  • Peanuts
  • Seeds
  • Toys
  • Coins
  • Food particles

Risk Age

1–3 years

Pathophysiology

Object lodges in airway causing:

  • Obstruction
  • Air trapping
  • Atelectasis
  • Infection

Most common location

Right main bronchus

Clinical Features

Acute phase

  • Sudden choking
  • Cough
  • Stridor
  • Wheezing

Later phase

  • Persistent cough
  • Recurrent pneumonia
  • Unilateral wheeze

Diagnosis

Chest X-ray

  • Air trapping
  • Atelectasis
  • Mediastinal shift

Definitive diagnosis

Rigid bronchoscopy

Differential Diagnosis

  • Asthma
  • Bronchiolitis
  • Pneumonia
  • Croup

Emergency Management

If Complete Airway Obstruction

Infant (<1 year)

  • 5 back blows
  • 5 chest thrusts

Child (>1 year)

  • Heimlich maneuver

Definitive Treatment

Rigid Bronchoscopy (Gold Standard)

Performed under general anesthesia to remove foreign body.

Complications if untreated:

  • Pneumonia
  • Lung abscess
  • Bronchiectasis
  • Death

Key Exam Points (High Yield)

  • Most common congenital laryngeal anomaly: Laryngomalacia
  • Most common congenital lung lesion: CPAM
  • Most common site for airway foreign body: Right main bronchus
  • Gold standard for diagnosis and treatment of foreign body: Rigid bronchoscopy
  • Congenital lung tissue with systemic blood supply: Pulmonary sequestration
  • Hyperinflated lung lobe in neonate: Congenital lobar emphysema

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Frequently Asked Questions

Laryngomalacia is the most common congenital laryngeal anomaly in infants in which the supraglottic structures collapse inward during inspiration, causing inspiratory stridor. It usually appears in the first few weeks of life and often resolves spontaneously by 12 to 18 months of age.
Typical symptoms include inspiratory stridor, noisy breathing that worsens when the infant is supine or feeding, improvement when the infant is prone, feeding difficulty, and in severe cases apnea or failure to thrive.
Congenital subglottic stenosis is a narrowing of the airway below the vocal cords present at birth. It causes biphasic stridor, recurrent croup, and respiratory distress due to reduced airway diameter.
The diagnosis is confirmed using rigid bronchoscopy or laryngoscopy, which directly visualizes narrowing of the subglottic airway. CT imaging may also help evaluate airway anatomy.
Congenital lobar emphysema is a developmental lung disorder characterized by overinflation of a lung lobe due to bronchial cartilage weakness or airway obstruction, leading to air trapping and respiratory distress in neonates.
The left upper lobe is the most commonly affected lobe, followed by the right middle lobe.
Congenital pulmonary adenomatoid malformation CPAM is a developmental lung lesion in which abnormal cystic lung tissue replaces normal alveoli, leading to respiratory distress, recurrent infections, or prenatal detection on ultrasound.
Pulmonary sequestration is a congenital lung anomaly in which a mass of nonfunctional lung tissue has no connection with the tracheobronchial tree and receives its blood supply from the systemic circulation, usually the aorta.
Foreign body aspiration occurs when a child inhales objects such as peanuts, seeds, small toys, or food particles into the airway, causing obstruction, coughing, wheezing, or respiratory distress.
The right main bronchus is the most common site because it is wider, shorter, and more vertical than the left bronchus.
Rigid bronchoscopy is the gold standard for both diagnosis and removal of airway foreign bodies in children.
If untreated, complications may include recurrent pneumonia, lung abscess, bronchiectasis, chronic cough, and in severe cases life threatening airway obstruction.