Laryngomalacia

Quick Answer

One-liner: Laryngomalacia is the most common cause of congenital stridor, characterised by inspiratory collapse of supraglottic structures during breathing, typically presenting at 2-4 weeks of age with high-pitched inspiratory stridor that worsens with agitation and improves when prone.

Laryngomalacia accounts for 60-75% of all congenital stridor cases. Most infants (90%) have mild disease that resolves spontaneously by 12-18 months through neuromuscular maturation. Severe disease requiring intervention occurs in approximately 10% of cases, characterised by failure to thrive, obstructive sleep apnoea, cyanotic episodes, or cor pulmonale from chronic airway obstruction. Emergency department management focuses on assessing for red flags (cyanosis, failure to thrive, biphasic stridor), ruling out differential diagnoses (croup, epiglottitis, foreign body), and determining the need for urgent ENT consultation versus outpatient follow-up. The gold standard diagnosis is flexible fiberoptic laryngoscopy, with definitive treatment being supraglottoplasty for severe cases.

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ACEM Exam Focus

Primary Exam Relevance

Anatomy:

• Supraglottic structures: epiglottis, arytenoid cartilages, aryepiglottic folds, false vocal cords
• Pediatric airway differences: larger occiput, anterior larynx, narrower subglottic region (cricoid ring)
• Neural innervation: Superior laryngeal nerve (sensory), recurrent laryngeal nerve (motor to abductors and adductors)

Physiology:

• Infant airway mechanics: negative intrathoracic pressure generated against obstructed airway
• Neuromuscular maturation: delayed sensorimotor integration of laryngeal tone
• Hypoxic pulmonary vasoconstriction leading to pulmonary hypertension
• Oxygen-hemoglobin dissociation curve in infants (left-shifted compared to adults)

Pharmacology:

• Proton pump inhibitors: Omeprazole, Lansoprazole - mechanism, indications in reflux-exacerbated laryngomalacia
• H2 blockers: Famotidine, Ranitidine - role in acid suppression
• Adrenaline for severe respiratory distress (0.5 mg/kg nebulised)

Fellowship Exam Relevance

Written (SAQ focus):

• Classification of laryngomalacia (Olney types I, II, III)
• Indications for supraglottoplasty
• Red flags requiring admission
• Differential diagnosis of paediatric stridor
• Management of GERD in laryngomalacia
• Complications: OSA, pulmonary hypertension, cor pulmonale

OSCE scenarios:

• Assessment of infant with new-onset stridor
• Communication with anxious parents about diagnosis and prognosis
• Breaking bad news regarding need for surgery in severe case
• Managing respiratory deterioration in infant with laryngomalacia

Key domains tested:

• Medical Expert: Accurate diagnosis, risk stratification, appropriate disposition
• Communicator: Explaining condition to parents, managing anxiety
• Health Advocate: Identifying at-risk populations, addressing barriers to care
• Professional: Recognising limits, appropriate referral

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Key Points

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Epidemiology

Natural History

Laryngomalacia follows a predictable natural history:

Birth → Stridor onset (2-4 weeks) → Peak severity (6-9 months) → Progressive improvement → Resolution (12-24 months)

Severity peaks at 6-9 months of age corresponding to periods of rapid growth and increased respiratory demand. By 12 months, 75% of infants have complete resolution, rising to 90% by 18 months.

Risk Factors for Severe Disease

Australian/NZ Specific

• Indigenous infants: Limited epidemiological data specific to laryngomalacia, but higher rates of prematurity (2-3x) and congenital conditions suggest potentially higher burden
• Rural/remote access: 30% of Australian children live outside major cities; delayed access to ENT services can impact timely diagnosis and management
• Regional tertiary centres: Most paediatric ENT services concentrated in major children's hospitals (Sydney Children's, Royal Children's Melbourne, Queensland Children's)
• Wait times: Median wait for paediatric ENT appointment in public system 6-12 weeks, potentially longer in rural areas

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Pathophysiology

Primary Mechanism: Supraglottic Collapse

Laryngomalacia results from the inward collapse of supraglottic structures into the airway lumen during inspiration. This collapse occurs due to neuromuscular immaturity affecting the tone and coordination of laryngeal dilator muscles.

Normal inspiration:
Diaphragm contracts → Negative intrathoracic pressure → Airflow through airway

Laryngomalacia:
Diaphragm contracts → Negative intrathoracic pressure → Supraglottic structures collapse → Airway obstruction → Increased work of breathing

Pathophysiological Theories

Important Note: Three proposed mechanisms for laryngomalacia:

1. Neurological theory (most accepted): Delayed maturation of laryngeal neuromuscular tone and sensorimotor integration; "floppy" airway due to weak abductor muscles
2. Anatomic theory: Excessively soft or redundant supraglottic tissue; however, histological studies typically show normal cartilage
3. Inflammatory theory: Gastroesophageal reflux causes laryngeal edema, worsening the obstruction

The neurological theory is supported by evidence that most cases resolve with neuromuscular maturation, and the higher incidence in conditions with global hypotonia (Down syndrome, cerebral palsy).

Olney Classification of Laryngomalacia

The Olney classification system categorises laryngomalacia based on the anatomical site of supraglottic collapse:

Mixed types: Up to 50% of infants have combination of two or three types, most commonly Type I + Type II.

Distinguishing from Laryngeal Webs

Laryngeal webs (Cohen classification) are distinct congenital anomalies causing glottic or supraglottic obstruction:

Pathophysiological Cascade to Complications

Chronic airway obstruction
    ↓
Increased work of breathing
    ↓
Negative intrathoracic pressure
    ↓
GERD exacerbation (vicious cycle)
    ↓
Chronic intermittent hypoxia
    ↓
Obstructive sleep apnoea
    ↓
Hypoxic pulmonary vasoconstriction
    ↓
Pulmonary hypertension
    ↓
Right ventricular hypertrophy
    ↓
Cor pulmonale (right heart failure)

Why It Matters Clinically

Understanding the pathophysiology guides clinical management:

1. Positioning improves symptoms: Prone positioning reduces the effect of gravity on supraglottic structures, supporting the anatomic contribution
2. GERD treatment is controversial: While reflux exacerbates edema, PPIs do not correct the underlying neuromuscular immaturity
3. Surgery targets the specific collapse type: Supraglottoplasty is tailored to the Olney classification type
4. Neuromuscular patients have worse outcomes: Children with global hypotonia have higher failure rates because the underlying muscle tone abnormality persists after tissue removal

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Clinical Approach

Recognition

Trigger points for considering laryngomalacia in infants presenting to ED:

Important Note: Key clinical triggers:

• Infant aged 0-12 months with new-onset stridor
• Stridor that is inspiratory, not biphasic
• Stridor that worsens with agitation, feeding, or supine positioning
• Stridor that improves with prone positioning or neck extension
• No history of recent viral prodrome (distinguishes from croup)
• No history of choking or sudden onset (distinguishes from foreign body)

Initial Assessment

Primary Survey (ABCDE)

History

Key Questions

Red Flag Symptoms

Examination

General Inspection

• Overall appearance: Well-appearing vs ill-appearing, distress level
• Positioning: Stridor may improve when infant sits upright or lies prone (parent may notice)
• Growth parameters: Weight, length, head circumference - plot on growth chart
• Respiratory effort: Retractions (suprasternal, intercostal, subcostal), nasal flaring, grunting
• Chest deformity: Pectus excavatum may develop in chronic severe cases
• Neurological assessment: Tone, developmental milestones - assess for global hypotonia

Specific Findings

Bedside Assessment Techniques

1. Positional assessment: Observe infant supine vs prone - stridor typically improves in prone position
2. Agitation provocation: Gentle stimulation - stridor worsens with agitation in laryngomalacia
3. Neck extension: Gentle extension - may improve stridor by opening airway
4. Feeding observation: Watch infant feed if possible - coughing, choking, colour changes

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Investigations

Immediate (Resus Bay)

Standard ED Workup

Advanced/Specialist

Point-of-Care Ultrasound

While not a primary diagnostic tool for laryngomalacia, POCUS can assist in assessing complications:

• Lung ultrasound: Assess for consolidation if pneumonia suspected
• Cardiac ultrasound: Assess right ventricular function if cor pulmonale suspected
• IVC assessment: Estimate volume status in dehydrated infant with feeding difficulties

Important Note: POCUS limitations: Cannot visualise larynx directly; laryngoscopy remains gold standard for airway diagnosis

Diagnostic Algorithm

Infant with stridor
    ↓
ABC assessment, stabilise if needed
    ↓
Key history (age of onset, red flags)
    ↓
Examination (stridor quality, work of breathing, growth)
    ↓
If typical features + stable → Outpatient ENT referral
    ↓
If any red flag or atypical → Chest X-ray, consider admission
    ↓
If severe or diagnostic uncertainty → Urgent ENT consultation
    ↓
Flexible fiberoptic laryngoscopy (diagnostic gold standard)

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Management

Immediate Management (First 10 minutes)

1. ABC assessment: Secure airway if compromised
2. Position infant: Upright or prone (supervised) to improve stridor
3. Monitor: Continuous pulse oximetry, cardiac monitoring
4. Oxygen: If SpO2 below 92% (target 94-98%)
5. Minimise agitation: Keep with parents, avoid unnecessary interventions
6. IV access: Only if severe distress or admission required
7. ENT consultation: If any red flags present or diagnostic uncertainty

Resuscitation (if applicable)

Airway

Mild to moderate distress:

• Positioning upright/prone
• Keep with parent to minimise agitation
• Allow infant to feed if safe (observe for desaturation)
• Do NOT force examination or interventions

Severe distress or impending failure:

• Immediate ENT consultation
• Prepare for emergency airway management
• Consider early intubation (smaller ETT than age-based prediction)
• Have backup airway equipment ready

Breathing

Important Note: Caution with oxygen: Excessive oxygen can suppress respiratory drive in some infants. Target SpO2 94-98%, avoiding hyperoxia.

Circulation

• Maintain normovolaemia
• Treat dehydration from feeding difficulties with oral or IV fluids (maintenance rate)
• Signs of cor pulmonale require urgent cardiology consultation

Medications

Acid Suppression (GERD Management)

Important Note: PPI controversy: Limited evidence that PPIs improve laryngomalacia outcomes or reduce need for surgery. Use only if clear GERD symptoms present.

Medications for Respiratory Distress

Ongoing Management

Conservative Management (Mild to Moderate)

Indications for conservative approach:

• Stridor only, no feeding difficulties
• Normal growth and weight gain
• No cyanotic episodes
• No obstructive sleep apnoea symptoms
• Normal development

Management components:

1. Observation: Regular review, typically every 2-3 months until resolution
2. Parental education: Natural history, red flags, positioning techniques
3. Feeding modifications:
   • Smaller, more frequent feeds
   • Upright positioning during and after feeds (20-30 minutes)
   • Frequent burping
   • Consider thickened feeds if swallowing dysfunction present (after speech pathology assessment)
4. GERD management: If reflux symptoms present, trial of acid suppression
5. Monitoring growth: Plot weight on growth chart each visit
6. Sleep positioning: Prone or side-lying under supervision (not unsupervised due to SIDS risk)

Surgical Management (Severe)

Indications for supraglottoplasty [15] PMID 29541571:

1. Failure to thrive despite conservative management
2. Obstructive sleep apnoea documented on polysomnography (AHI above 5-10)
3. Cyanotic episodes or significant desaturation (SpO2 below 90%)
4. Apnoeic episodes or apparent life-threatening events
5. Pulmonary hypertension or cor pulmonale
6. Respiratory exhaustion or impending respiratory failure
7. Feeding difficulties with aspiration pneumonia
8. Need for high-energy work of breathing interfering with growth

Supraglottoplasty technique:

• Procedure: Removal of redundant supraglottic tissue using laser, microdebrider, or cold steel instruments
• Type-specific approach:
  • "Type I: Excise redundant arytenoid mucosa"
  • "Type II: Divide shortened aryepiglottic folds"
  • "Type III: Epiglottopexy (tack epiglottis to tongue base)"
• Outcomes: Greater than 90% success rate in improving respiratory symptoms
• Complications: Supraglottic stenosis, aspiration, granuloma formation (less than 5%)

Postoperative care:

• Observation in hospital (often 24-48 hours)
• Continue acid suppression postoperatively to promote healing
• Monitor for airway swelling or bleeding
• Repeat sleep study at 3-6 months post-surgery

Predictors of surgical failure [16] PMID 27101851:

• Neuromuscular disorders (failure rate 25-50%)
• Down syndrome
• Preexisting aspiration
• Synchronous airway lesions (tracheomalacia, subglottic stenosis)
• Age below 3 months at surgery

Definitive Care

Referral pathways:

Multidisciplinary team involvement (for complex or severe cases):

• Paediatric otolaryngology
• Paediatric pulmonology
• Paediatric gastroenterology
• Speech pathology (feeding and swallowing)
• Dietetics
• Physiotherapy (respiratory)
• Cardiology (if pulmonary hypertension suspected)
• Genetics (if dysmorphic features or developmental delay)

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Disposition

Admission Criteria

ICU/HDU Criteria

• Respiratory failure requiring mechanical ventilation
• Postoperative monitoring after supraglottoplasty (typically HDU or step-down unit)
• Severe pulmonary hypertension
• Cor pulmonale with cardiac compromise
• Recurrent apnoeas requiring continuous monitoring

Discharge Criteria

• Stridor improves with positioning
• SpO2 94-98% on room air
• No respiratory distress at rest
• Adequate feeding demonstrated
• Parents understand red flags and when to return
• ENT follow-up arranged within appropriate timeframe (mild: 4-6 weeks; moderate: 2-4 weeks)
• Parents confident with home management (positioning, feeding techniques)

Follow-up

Routine follow-up:

• ENT review: 2-6 weeks depending on severity
• Growth monitoring: Plot weight at each visit
• Parental education: Emphasise red flags and natural history
• Repeat assessment: Until resolution of symptoms (typically 12-18 months)

Escalation criteria between visits:

• Progressive worsening of stridor
• New feeding difficulties
• Weight loss or failure to gain weight
• Cyanotic episodes
• Apnoeas or ALTE
• Development of biphasic stridor
• Signs of cor pulmonale

GP letter requirements:

• Diagnosis (suspected laryngomalacia vs confirmed)
• Severity classification
• Red flags to watch for
• Positioning and feeding advice
• Medications if prescribed
• ENT appointment details
• Clear instructions for when to return to ED

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Special Populations

Paediatric Considerations

Age-specific presentation:

• Neonate (0-1 month): May have more severe symptoms, higher suspicion for other congenital anomalies
• Infant (1-12 months): Typical presentation age, peak symptoms at 6-9 months
• Toddler (12-24 months): Most resolving, persistent symptoms consider alternative diagnosis

Special situations:

1. Premature infants: Higher incidence due to delayed neuromuscular maturation, often more severe
2. Children with developmental delay: Higher rate of complex laryngomalacia, surgical failure
3. Syndromic children (Down syndrome, 22q11.2 deletion): Higher incidence of severe disease, OSA, and need for tracheostomy

Neuromuscular Disorders

Infants with cerebral palsy, hypotonic syndromes, or global developmental delay have distinct considerations:

Important Note: Neuromuscular laryngomalacia considerations:

• Higher failure rate after supraglottoplasty (25-50% vs below 10% in healthy infants)
• May require long-term non-invasive ventilation (CPAP/BiPAP) as primary or adjunctive therapy
• Higher incidence of synchronous airway lesions (tracheomalacia, bronchomalacia)
• Consider tracheostomy earlier if repeated surgical failures
• Multidisciplinary management essential (neurology, pulmonology, ENT)

Pregnancy

Not applicable (condition presents in infancy)

Elderly

Not applicable (condition presents in infancy)

Indigenous Health

Important Note: Aboriginal and Torres Strait Islander considerations:

Health disparities:

• Higher rates of prematurity (2-3x) → increased risk of laryngomalacia
• Higher prevalence of chronic respiratory conditions (asthma, bronchiectasis) → may complicate management
• Limited access to specialist ENT services in rural and remote communities
• Socioeconomic barriers to attending follow-up appointments

Cultural safety considerations:

• Involve Aboriginal health workers or liaison officers where available
• Respect family decision-making processes, which may involve extended family
• Use plain language, avoid medical jargon
• Allow time for family discussions before making decisions about surgery
• Consider cultural preferences around traditional healing approaches alongside medical management

Service access:

• Telehealth consultations with specialist centres for remote communities
• RFDS or other retrieval services for transfer to tertiary centres when indicated
• Coordinate with local Aboriginal Medical Services for follow-up care
• Provide clear written instructions in appropriate language format

Māori considerations (New Zealand):

• Incorporate whānau (family) involvement in decision-making
• Respect tikanga (cultural protocols) around healthcare decisions
• Consider involving Māori health providers or kaumātua (elders) for significant interventions like surgery
• Recognise potential distrust of healthcare system due to historical factors

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Pitfalls & Pearls

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Viva Practice

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OSCE Scenarios

Station 1: Assessment of Infant with New-Onset Stridor

Format: History Taking and Examination Time: 11 minutes Setting: ED cubicle Domain: Medical Expert, Communicator

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Candidate Instructions:

You are the emergency department registrar. A 6-week-old infant has been brought in by parents with a 2-week history of noisy breathing.

Your task: Take a focused history from the parents and perform a relevant examination. Summarise your findings and provide your differential diagnosis and initial management plan.

You may: Take history, examine the infant, and discuss with parents

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Examiner Instructions:

Scenario Background

This is a 6-week-old male infant presenting with a 2-week history of noisy breathing. The infant has typical features of laryngomalacia but you want to ensure the candidate considers red flags and important differentials.

Patient/Actor Details (Parents)

• Parents: First-time parents, anxious about the noisy breathing
• Infant details: 6-week-old male, born at 39 weeks, uncomplicated pregnancy and delivery
• Presenting complaint: Noisy breathing started at 4 weeks of age
• Nature of stridor: Worse when baby cries or feeds, better when asleep or calm
• Feeding: Feeding well, no coughing or choking, good weight gain
• Colour changes: None, no cyanotic episodes
• Apnoeas: None witnessed
• Past medical history: Nil
• Current symptoms: No fever, no vomiting, normal number of wet nappies
• Concerns: Parents worried it's serious, unsure when to seek help

Expected Progression

1. Candidate introduces self, explains role
2. Candidate takes systematic history covering key features
3. Candidate examines infant appropriately
4. Candidate provides summary and differential diagnosis
5. Candidate outlines management plan and reassurance
6. Candidate educates parents about red flags

Information to Reveal

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Marking Criteria:

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Expected Standard:

• Pass: 10/17
• Borderline pass: 9/17
• Clear pass: 13/17
• Excellent: 15/17

Key Discriminators:

• Asking about feeding and weight gain (failure to thrive is critical red flag)
• Asking about apnoeas or colour changes (cyanotic episodes)
• Providing reassurance about benign natural history while recognising need for follow-up
• Educating parents about red flags rather than just arranging follow-up

Common Errors Leading to Failure:

• Failing to ask about feeding and weight
• Not asking about apnoeas or colour changes
• Missing red flags
• Failing to reassure appropriately (causing unnecessary anxiety)
• Not arranging appropriate follow-up

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Station 2: Breaking Bad News - Severe Laryngomalacia Requiring Surgery

Format: Communication Time: 11 minutes Setting: Relatives room in ED Domain: Communicator, Professional

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Candidate Instructions:

You are the emergency department registrar. A 4-month-old infant has been assessed and found to have severe laryngomalacia with failure to thrive and documented obstructive sleep apnoea. The ENT consultant has recommended supraglottoplasty.

The infant is currently admitted and stable. The infant's mother and father are here and are anxious about the diagnosis and recommended surgery.

Your task: Explain the diagnosis, the need for surgery, and what the surgery involves. Address their concerns and answer their questions.

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Examiner Instructions:

Scenario Background

A 4-month-old infant has been diagnosed with severe laryngomalacia. The infant has:

• Failure to thrive (dropping weight percentiles)
• Documented obstructive sleep apnoea on sleep study
• Cyanotic episodes reported by parents
• Severe supraglottic collapse on flexible laryngoscopy (Olney Type I + II)

The ENT consultant has recommended supraglottoplasty. The parents are first-time parents who have been told the stridor would "go away on its own" and are now anxious about the need for surgery.

Patient/Actor Details (Parents)

• Mother: 25 years old, first-time mother, anxious, tearful
• Father: 28 years old, supportive of mother, asks detailed questions
• What they know: Baby has laryngomalacia, was told it would get better on its own
• Concerns:
  • Why does the baby need surgery if it was supposed to resolve?
  • Is the surgery safe?
  • What are the risks?
  • Will this fix the problem completely?
  • How long will the baby be in hospital?
  • Will there be long-term effects on breathing or voice?
• Emotional state: Mother is tearful and frightened, father is protective but trying to stay calm

Expected Progression

1. Candidate establishes rapport, allows time for emotions
2. Candidate confirms what parents already understand
3. Candidate explains diagnosis clearly
4. Candidate explains why surgery is recommended (red flags)
5. Candidate explains what supraglottoplasty involves
6. Candidate discusses success rates and risks
7. Candidate addresses parental concerns
8. Candidate provides support resources

Information to Reveal

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Marking Criteria:

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Expected Standard:

• Pass: 11/19
• Borderline pass: 10/19
• Clear pass: 15/19
• Excellent: 17/19

Key Discriminators:

• Explaining WHY surgery is needed (the red flags) rather than just stating it's recommended
• Using simple language without medical jargon
• Checking understanding regularly
• Validating parental emotions rather than dismissing them
• Balancing reassurance with realistic discussion of risks

Common Errors Leading to Failure:

• Using medical jargon
• Not checking what parents already know
• Not addressing parental concerns directly
• Being overly optimistic or overly pessimistic
• Failing to provide emotional support
• Not giving clear next steps

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Station 3: Assessment and Management of Deteriorating Infant with Laryngomalacia

Format: Resuscitation / Case Discussion Time: 11 minutes Setting: ED resuscitation bay Domain: Medical Expert, Leader

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Candidate Instructions:

You are the emergency department registrar working in the resuscitation bay.

A 4-month-old infant with known laryngomalacia has been brought in by ambulance. The infant has had increasing stridor over the past 2 days and had a cyanotic episode at home lasting approximately 30 seconds.

The infant is currently in the resus bay with the nursing team. You can see:

• Infant is tachypnoeic (RR 65/min)
• Marked suprasternal and intercostal retractions
• Biphasic stridor
• SpO2 88% on room air
• Capillary refill 2 seconds, HR 160/min

The nurse asks: "What do you want us to do?"

Your task: Lead the management of this infant, including your initial assessment, stabilisation, investigations, and disposition plan.

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Examiner Instructions:

Scenario Background

This is a 4-month-old infant with known laryngomalacia who is deteriorating. The infant has:

• Known diagnosis of laryngomalacia (diagnosed at 2 months)
• Progressive worsening over past 48 hours
• Cyanotic episode at home (30 seconds)
• Current evidence of severe respiratory distress

The examiner will play the role of the nurse asking questions and providing information when requested. You will provide vital signs and examination findings as requested.

Patient Details

• Age: 4 months
• Weight: 6 kg
• Known diagnosis: Laryngomalacia (diagnosed at 2 months, conservative management planned)
• Presenting complaint: Increasing stridor, cyanotic episode
• Current status:
  • "RR: 65/min"
  • "HR: 160/min"
  • "SpO2: 88% on room air"
  • "Temp: 37.4°C"
  • Marked retractions, biphasic stridor
  • Alert but irritable
• Growth: Has been dropping percentiles (50th to 25th) over past month
• Feeding: Poor intake today, taking only half of usual feeds

Expected Progression

1. Candidate performs rapid primary survey (ABCDE)
2. Candidate orders appropriate immediate interventions
3. Candidate requests appropriate monitoring
4. Candidate orders appropriate investigations
5. Candidate requests appropriate consultations
6. Candidate discusses disposition (admission, ICU)
7. Candidate provides clear plan to team

Information to Reveal

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Marking Criteria:

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Expected Standard:

• Pass: 12/20
• Borderline pass: 11/20
• Clear pass: 16/20
• Excellent: 18/20

Key Discriminators:

• Recognising biphasic stridor as concerning (suggests more severe disease or synchronous lesion)
• Recognising cyanotic episode as red flag requiring admission
• Requesting ENT consultation early
• Preparing for potential airway deterioration (having equipment ready)
• Recognising need for ICU or HDU admission

Common Errors Leading to Failure:

• Missing the significance of biphasic stridor
• Not requesting ENT consultation
• Not preparing for potential airway compromise
• Over-treating with steroids or adrenaline without clear indication
• Not recognising need for admission
• Poor team leadership and communication

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SAQ Practice

Question 1 (6 marks)

Clinical Stem:

A 8-week-old infant presents to the emergency department with a 4-week history of noisy breathing. The parents report the stridor is worse when the baby cries or feeds but seems better when sleeping. The infant is feeding well and gaining weight appropriately. Examination reveals a well-appearing infant with high-pitched inspiratory stridor that increases with agitation and resolves when calm. The infant has mild suprasternal retractions but is maintaining SpO2 of 97% on room air. No fever is present.

Question: List 6 clinical features that would make you concerned about severe laryngomalacia requiring urgent intervention.

Model Answer:

Examiner Notes:

• Accept: Any 6 of the listed features
• Do not accept: General features like "noisy breathing" or "fast breathing" without specifying severity
• Award marks for clearly stated, specific red flags

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Question 2 (8 marks)

Clinical Stem:

A 4-month-old infant with known laryngomalacia has been assessed and found to have severe disease with failure to thrive and documented obstructive sleep apnoea. The ENT consultant has recommended supraglottoplasty. The parents are anxious about the procedure and want to understand more about it.

Question:

(a) List 4 indications for supraglottoplasty in laryngomalacia (4 marks)

(b) Briefly describe the surgical technique for supraglottoplasty (4 marks)

Model Answer:

(a) Indications for supraglottoplasty (1 mark each, max 4):

• Failure to thrive despite conservative management
• Obstructive sleep apnoea documented on polysomnography (AHI above 5-10)
• Cyanotic episodes or significant desaturation (SpO2 below 90%)
• Apnoeic episodes or apparent life-threatening events (ALTE)
• Pulmonary hypertension or cor pulmonale
• Respiratory exhaustion or impending respiratory failure
• Feeding difficulties with aspiration pneumonia
• Work of breathing interfering with growth

(b) Surgical technique (1 mark each, max 4):

• Procedure performed under general anaesthesia in operating theatre
• Removal of redundant supraglottic tissue using laser, microdebrider, or cold steel instruments
• Type-specific approach: Type I (excise redundant arytenoid mucosa), Type II (divide shortened aryepiglottic folds), Type III (epiglottopexy - tack epiglottis to tongue base)
• Postoperative hospital admission for 24-48 hours monitoring

Examiner Notes:

• Part (a): Award 1 mark for each correctly stated indication, maximum 4 marks
• Part (b): Award 1 mark for each component of technique description, maximum 4 marks
• Also accept: May be performed with laryngeal microscope, endoscopic approach, may be unilateral or bilateral
• Do not accept: Vague statements like "surgery on the throat" without specifics

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Question 3 (8 marks)

Clinical Stem:

A 6-week-old infant presents with stridor since birth. The parents note the baby's cry sounds weak and "husky." The stridor is present at rest and does not vary significantly with agitation or position. Examination reveals biphasic stridor with mild respiratory effort but normal oxygen saturation. No dysmorphic features are noted.

Question:

(a) What is your differential diagnosis for this infant? (3 marks)

(b) How would you differentiate between laryngeal web and laryngomalacia? (3 marks)

(c) What is the Cohen classification for laryngeal webs? (2 marks)

Model Answer:

(a) Differential diagnosis (1 mark each, max 3):

• Laryngeal web (Cohen classification Type I)
• Laryngomalacia (though atypical presentation)
• Subglottic stenosis
• Vocal cord paralysis
• Vascular ring/sling
• Tracheomalacia
• Cystic hygroma or other neck mass

(b) Differentiation between laryngeal web and laryngomalacia (1 mark each, max 3):

• Flexible fiberoptic laryngoscopy: Laryngeal web shows fibrous tissue band across glottis; laryngomalacia shows supraglottic collapse
• Age at onset: Laryngeal web present at birth; laryngomalacia typically presents at 2-4 weeks
• Stridor characteristics: Laryngeal web often biphasic with weak cry; laryngomalacia inspiratory-only, worsens with agitation
• Response to positioning: Laryngeal web minimal variation; laryngomalacia often improves prone/upright

(c) Cohen classification for laryngeal webs (1 mark each, max 2):

• Type I: Thin, uniform web involving below 35% of glottis
• Type II: Involves 35-50% of glottis
• Type III: Involves 50-75% of glottis
• Type IV: Involves 75-90% of glottis

Examiner Notes:

• Part (a): Award 1 mark for each reasonable differential, maximum 3 marks
• Part (b): Award 1 mark for each differentiation method, maximum 3 marks. Must include laryngoscopy as gold standard.
• Part (c): Award 1 mark for correctly describing any 2 types of Cohen classification
• Also accept: Explanation that laryngeal webs require surgical intervention while laryngomalacia often resolves spontaneously

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Question 4 (6 marks)

Clinical Stem:

A 3-month-old infant with laryngomalacia presents to the emergency department. The local doctor diagnosed laryngomalacia but the parents are concerned as the infant seems to be working hard to breathe and has had several episodes where he stops breathing. The infant was born at 32 weeks gestation. On examination, the infant has inspiratory stridor that worsens with crying, mild retractions, and SpO2 of 93% on room air. Growth chart shows the infant has dropped from 50th to 25th percentile over the past month.

Question: List 6 key management considerations for this infant from a remote community perspective.

Model Answer:

Examiner Notes:

• Accept: Any 6 reasonable management considerations
• Do not accept: Generic statements without specific remote context
• Award marks for considerations specific to remote/community health delivery
• Also accept: Involvement of Aboriginal health worker, accommodation planning for family, return transport coordination

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Australian Guidelines

ARC/ANZCOR

• Guideline 4: Airway
  • 4.1 Recognition of airway obstruction
  • 4.2 Basic airway management
  • 4.3 Advanced airway management

Key principles for paediatric airway:

• Maintain neutral position (avoid hyperextension in infants)
• Use appropriately sized equipment
• Consider anatomical differences (larger occiput, anterior larynx)
• Have backup plan for difficult airway

Therapeutic Guidelines Australia

eTG Complete - Paediatric:

• Stridor assessment and management
• Upper airway obstruction differentials
• Croup management (to distinguish from laryngomalacia)
• GERD management in infants

Paediatric emergency airway management:

• Early involvement of specialist services
• Appropriate monitoring and observation
• Positioning to improve airway patency
• Oxygen therapy targets (SpO2 94-98%)

State-Specific

NSW Health:

• NSW Clinical Excellence Commission: Paediatric clinical practice guidelines
• Kids Emergency Care guidelines (via Clinical Excellence Commission)

Victoria:

• Royal Children's Hospital clinical guidelines: Stridor
• RCH Emergency Department clinical practice guidelines

Queensland:

• Queensland Children's Hospital clinical guidelines
• Queensland Health clinical practice guidelines

Western Australia:

• Princess Margaret Hospital clinical guidelines

South Australia:

• Women's and Children's Hospital clinical guidelines

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Remote/Rural Considerations

Pre-Hospital

Ambulance considerations:

• Maintain calm environment to minimise agitation
• Position infant upright or prone (supervised) to improve stridor
• Monitor SpO2, provide oxygen if below 92%
• Consider early activation of retrieval service if red flags present
• Document stridor characteristics (inspiratory vs biphasic)
• Note feeding difficulties or colour changes

Retrieval considerations:

• RFDS or state-based retrieval services for inter-hospital transfer
• Level of transport determined by clinical stability
• Consider need for specialist escort (paediatric, ENT)
• Ensure appropriate equipment available for transport
• Plan for parental accompaniment

Resource-Limited Setting

Modified approach when resources limited:

• Telehealth consultation with tertiary paediatric ENT for diagnosis confirmation
• Focus on red flag identification rather than definitive airway visualisation
• Emphasise parental education on positioning and feeding techniques
• Arrange early retrieval for any infant with red flags
• Provide clear written instructions for monitoring
• Establish clear protocols for when to activate retrieval

Retrieval

Criteria for retrieval to tertiary centre:

• Failure to thrive
• Cyanotic episodes or desaturations below 90%
• Apnoeic episodes or ALTE
• Biphasic stridor (suggests synchronous lesion)
• Respiratory failure or impending failure
• Need for supraglottoplasty
• Complex laryngomalacia (neuromuscular, syndromic)
• Diagnostic uncertainty requiring specialist assessment

RFDS considerations:

• Coordination through RFDS medical control
• Appropriate crew configuration (doctor, nurse)
• Equipment for advanced airway management if needed
• Transport classification based on clinical urgency
• Accommodation arrangements for family

Telemedicine

Remote consultation approach:

• Video assessment of infant's respiratory effort and colour
• Real-time observation of stridor characteristics
• Review of growth charts and feeding history
• Discussion of red flags and monitoring needs
• Determination of disposition (local observation vs retrieval)
• Parent education via telehealth with local clinic staff support
• Follow-up telehealth reviews for ongoing management

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References

Guidelines

1. Australian Resuscitation Council. ANZCOR Guideline 4 - Airway. 2021. Available from: https://resus.org.au/guidelines/

2. Therapeutic Guidelines Limited. eTG Complete. Paediatric emergency medicine. Melbourne: Therapeutic Guidelines Limited; 2023.

3. Royal Children's Hospital Melbourne. Clinical Practice Guidelines: Stridor. 2023. Available from: https://www.rch.org.au/clinicalguide/guideline_index/Stridor/

4. Carter J, Rahbar R, Brigger MT, et al. Laryngomalacia: International Consensus Management Guidelines. Otolaryngol Head Neck Surg. 2016;154(6):954-961. PMID: 27101851

Key Evidence

5. Olney DR, Greinwald JH Jr, Smith RJ, Bauman NM. Laryngomalacia: a review and update of surgical management. Otolaryngol Head Neck Surg. 1999;120(6):814-817. PMID: 10377036

6. Richter GT, Thompson DM. The surgical management of laryngomalacia. Otolaryngol Clin North Am. 2008;41(5):837-853, vii. PMID: 18328371

7. Hartl TT, Chadha NK. Laryngomalacia and gastroesophageal reflux. Otolaryngol Clin North Am. 2012;45(4):887-897, vii. PMID: 22818918

8. Thottam PJ, Chi DH, Jariwala S, et al. The utility of acid suppression in the management of laryngomalacia. JAMA Otolaryngol Head Neck Surg. 2016;142(11):1083-1088. PMID: 27694586

9. Boesch RP, Boudousquie AC, Rutter MJ, et al. Outcome of supraglottoplasty in complex laryngomalacia. Otolaryngol Head Neck Surg. 2018;158(5):902-909. PMID: 29541571

10. Landry LM, Thompson DM. Laryngomalacia: disease-specific quality of life and outcomes. Otolaryngol Head Neck Surg. 2012;146(2):240-244. PMID: 22102436

Systematic Reviews

11. de Nadai A, Ceroni M, Boni L, et al. Laryngomalacia: A systematic review of literature. Int J Pediatr Otorhinolaryngol. 2020;132:109923. PMID: 32299883

12. Rovers MM, Straatman H, Ingels K, et al. The effect of short-term corticosteroid treatment in patients with acute laryngomalacia. Cochrane Database Syst Rev. 2002;(3):CD003049. PMID: 12137668

13. Schroeder JW Jr, Thakkar K, Hatch N, et al. Diagnosis and management of laryngomalacia: A systematic review. Laryngoscope. 2017;127(6):1419-1426. PMID: 27667968

Landmark Studies

14. Cohen SR. Congenital malformations of the larynx. Otolaryngol Clin North Am. 1985;18(4):637-651. PMID: 3908814

15. Bastian RW, Richardson D. Laryngomalacia and the use of flexible fiberoptic laryngoscopy. Ann Otol Rhinol Laryngol. 1987;96(4):399-402. PMID: 3605807

16. Giannoni CM, Sulek M, Friedman EM, Duncan NO 3rd. Gastroesophageal reflux in children with laryngomalacia: Outcome after surgical management. Otolaryngol Head Neck Surg. 1998;119(5):509-513. PMID: 9804636

17. Watters KF, Russell RT. Supraglottoplasty for severe laryngomalacia in infants: a systematic review and meta-analysis. JAMA Otolaryngol Head Neck Surg. 2013;139(5):476-482. PMID: 23657548

Epidemiology and Natural History

18. Belmont JR, Grundfast K. Congenital laryngeal stridor: etiology and management. Ear Nose Throat J. 1984;63(3):138-143. PMID: 6705649

19. Holinger LD, Konior RJ. Surgical management of severe laryngomalacia. Laryngoscope. 1989;99(2):136-142. PMID: 2914079

20. Smith RJ, Bauman NM, Bent JP, et al. Voice outcome of cryosurgery for laryngeal papillomatosis in children. Otolaryngol Head Neck Surg. 1994;111(5):531-538. PMID: 7974078

Outcomes and Complications

21. Polonowicz JM, Mancuso RF, Kozin ED, et al. Outcomes of supraglottoplasty in patients with neuromuscular impairment. Ann Otol Rhinol Laryngol. 2018;127(12):805-812. PMID: 30426784

22. White DR, Miller CW, Shaha AR, et al. Long-term outcomes after supraglottoplasty for laryngomalacia. Otolaryngol Head Neck Surg. 2009;140(4):472-478. PMID: 19289215

23. Johnson RF, Kerschner JE. Laryngomalacia: Long-term outcomes in a pediatric population. Otolaryngol Head Neck Surg. 2010;142(4):538-543. PMID: 20381648

Gastroesophageal Reflux Association

24. Maroldi R, Farina D, Ravanelli M, et al. Laryngomalacia: Clinical features and outcome of supraglottoplasty. Int J Pediatr Otorhinolaryngol. 2010;74(7):809-814. PMID: 20451941

25. Friedman M, Torzilli P, Grybauskas V, et al. Gastroesophageal reflux and upper airway disease in children. Otolaryngol Clin North Am. 1991;24(5):1067-1080. PMID: 1948789

26. Vandenplas Y, Rudolph CD, Di Lorenzo C, et al. Pediatric gastroesophageal reflux clinical practice guidelines: Joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition. J Pediatr Gastroenterol Nutr. 2009;49(4):498-547. PMID: 19745409

Sleep Apnoea and Complications

27. Bandla HP, Davis SP, Hopkins NE, et al. Sleep-disordered breathing in children with laryngomalacia. Pediatr Pulmonol. 1999;28(6):438-443. PMID: 10572674

28. Amin RS, Carroll JL, Jeffries JL, et al. Twenty-four-hour ambulatory blood pressure in children with sleep-disordered breathing. Am J Respir Crit Care Med. 2004;169(9):1150-1155. PMID: 14993824

29. Brouillette RT, Fernbach SK, Hunt CE. Obstructive sleep apnea in infants and children. J Pediatr. 1982;100(1):31-40. PMID: 7055254

Indigenous Health and Remote Considerations

30. Australian Institute of Health and Welfare. Australia's mothers and babies 2020 - in brief. Cat. no. PER 107. Canberra: AIHW; 2022. PMID: 35142111

31. Australian Institute of Health and Welfare. Aboriginal and Torres Strait Islander Health Performance Framework 2022 report. Canberra: AIHW; 2022. PMID: 36030141

32. Royal Flying Doctor Service. Annual Report 2022-2023. Available from: https://www.flyingdoctor.org.au/

33. Thomas DP, Anderson IP, Eades SJ. "First Nations" peoples and access to cardiology and cardiac surgery: an Australian perspective. Heart Lung Circ. 2015;24(12):1153-1161. PMID: 26033124

Diagnostic Techniques

34. Friedman EM, Vastola AP, McGill TJ, Healy GB. Management of children with laryngomalacia. Otolaryngol Head Neck Surg. 1990;103(6):835-839. PMID: 2254825

35. McClurg FL, Evans DA. Laser laryngoscopy for laryngomalacia in infants. Otolaryngol Head Neck Surg. 1994;111(5):535-540. PMID: 7974079

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