Congenital Diaphragmatic Hernia (CDH)

Quick Answer

What is congenital diaphragmatic hernia?

CDH is a developmental defect where abdominal contents herniate into the thorax through a defect in the diaphragm, causing pulmonary hypoplasia and pulmonary hypertension. Key anaesthetic principles:

1. Primary pathology - Pulmonary hypoplasia (compression-induced lung underdevelopment) and pulmonary hypertension
2. Prenatal diagnosis - LHR (lung-to-head ratio) predicts severity; <1.0 indicates poor prognosis
3. Gentle ventilation - Avoid high pressures (<20-25 cmH₂O) to prevent barotrauma
4. Delayed surgery - Stabilise 48 hours to 7 days before repair
5. PPHN management - iNO (20 ppm), HFOV, ECMO for refractory cases

Clinical Pearl: CDH is a medical emergency masquerading as a surgical one. The priority is stabilisation, not immediate surgery.

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Clinical Overview

Definition

Congenital diaphragmatic hernia results from incomplete fusion of the pleuroperitoneal folds during the 8th week of gestation. This allows abdominal viscera (stomach, intestine, liver, spleen) to herniate into the thoracic cavity, compressing the developing lungs and causing:

1. Pulmonary hypoplasia - Reduced number of airway generations and alveoli
2. Pulmonary vascular abnormalities - Thickened arteriolar walls causing pulmonary hypertension
3. Cardiac displacement - Mediastinal shift affecting cardiac function

Epidemiology

• Incidence: 1 in 2,500-4,000 live births [1]
• Left-sided: 85% (Bochdalek hernia through posterolateral foramen)
• Right-sided: 13% (usually liver herniation)
• Bilateral: 2% (very poor prognosis)
• Males: Slight predominance (male:female ratio 1.5:1)
• Associated anomalies: 40-60% have additional defects [2]

Associated Anomalies

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Pathophysiology

Embryology and Timing

Normal diaphragm development:

• Week 4: Septum transversum forms
• Week 5: Pleuroperitoneal folds develop
• Week 6-7: Fusion begins
• Week 8: Fusion complete

CDH pathogenesis:

• Failure of pleuroperitoneal fold fusion
• Creates posterolateral defect (Bochdalek foramen)
• Occurs before week 8 - lung development already compromised
• Earlier defect = more severe lung hypoplasia

Pulmonary Hypoplasia

Mechanism of lung compression:

The herniated viscera occupy thoracic space during the critical period of airway branching (weeks 5-16):

Functional consequences:

• Reduced lung compliance (stiff lungs)
• Increased work of breathing
• Ventilation-perfusion mismatch
• Hypoxia and hypercapnia

Pulmonary Vascular Disease

Structural abnormalities:

Physiological consequences:

• ↑ Pulmonary vascular resistance (PVR)
• Persistent pulmonary hypertension of the newborn (PPHN)
• Right-to-left shunting through PDA and foramen ovale
• Right ventricular pressure overload and dysfunction

Factors exacerbating PPHN:

• Hypoxia
• Acidosis
• Hypothermia
• Hypovolaemia
• Hyperinflation/overdistension

Cardiac Effects

Hemodynamic consequences:

Other Organ Effects

Hypertension on abdominal organs:

• Reduced abdominal cavity volume (herniated contents)
• Intestinal malrotation common
• Gastric distension risk (N2O avoidance critical)

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Diagnosis and Severity Assessment

Prenatal Diagnosis

Ultrasound findings (18-22 week anomaly scan):

Severity markers:

Clinical Pearl: The O/E LHR is the most reliable prenatal predictor of survival in isolated left-sided CDH. Values <25% predict survival <25% [4].

Postnatal Diagnosis

Clinical presentation:

Imaging:

• Chest X-ray: Bowel loops in thorax, mediastinal shift, absent lung markings in affected hemithorax
• ECHO: Exclude cardiac anomalies, assess PPHN, RV function
• ECHO: Exclude cardiac anomalies, assess PPHN, RV function
• ECHO: Exclude cardiac anomalies, assess PPHN, RV function
• ECHO: Exclude cardiac anomalies, assess PPHN, RV function

Severity Classification

Risk stratification:

Risk categories:

• Low risk: 0-2 points (survival >80%)
• Moderate risk: 3-5 points (survival 50-80%)
• High risk: >5 points (survival <50%)

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Preoperative Stabilisation

The "Gentle Ventilation" Strategy

Historical context: Traditional aggressive ventilation (high pressures, hyperventilation) caused high rates of pneumothorax (50-60%) and poor survival (40-50%). The "gentle ventilation" approach, pioneered by Wung et al. in the 1990s, revolutionised outcomes [5].

Principles:

Physiological rationale:

• Hypoplastic lungs are non-compliant and easily overdistended
• High pressure causes barotrauma (pneumothorax, air leak)
• Overdistension compresses pulmonary vasculature → ↑ PVR
• Permissive hypercapnia avoids high-pressure ventilation
• Avoiding hyperoxia prevents pulmonary vasodilation at expense of systemic perfusion

Ventilator settings:

Clinical Pearl: "Gentle ventilation" reduced mortality from 60% to 20-30% in CDH. The lung is the limiting factor - don't try to make it do what it cannot [6].

Sedation and Analgesia

Goals:

• Minimise agitation (catecholamines increase PVR)
• Facilitate synchrony with ventilator
• Avoid respiratory depression

Strategy:

Note: Avoid ketamine (catecholamine release increases PVR).

Pulmonary Vasodilation

Inhaled nitric oxide (iNO):

ECMO criteria:

Other pulmonary vasodilators:

• Sildenafil: 0.5-1 mg/kg q6h (oral/NG); reduces PVR
• Milrinone: 0.25-0.75 mcg/kg/min (inodilator, reduces PVR)
• Prostacyclin: Epoprostenol 0.01-0.05 mcg/kg/min

Timing of Surgery

Historical error: Earlier practice was emergency surgery within hours of birth, based on the assumption that "reducing the hernia would fix the problem."

Current evidence:

• Delayed surgery (48 hours to 7 days) allows:
  • Pulmonary vascular reactivity to stabilise
  • PPHN to improve
  • Transition from fetal to neonatal circulation
  • Optimisation of haemodynamics

Emergency surgery indications:

• Strangulation of herniated viscera
• Perforation
• Severe obstruction unresponsive to medical management

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Intraoperative Management

Preoperative Preparation

Checklist:

Monitoring:

Induction and Maintenance

Transport to OR:

• Maintain gentle ventilation during transport
• Keep patient warm (forced air warmer)
• Have resuscitation drugs drawn up
• ECMO circuit ready if high risk

Induction strategy:

Maintenance:

• Opioid-based technique: Fentanyl infusion (5-10 mcg/kg/hr) + low-dose volatile (0.5-1 MAC)
• Avoid: N2O (expands bowel, increases PVR)
• Muscle relaxation: Continue to prevent ventilator asynchrony

Ventilation during surgery:

• Hand ventilation preferred (detect compliance changes)
• Minimise airway pressures (PIP <20-25 cmH₂O)
• Consider HFOV if available and effective preoperatively

Surgical Approach

Techniques:

Anaesthetic considerations during reduction:

Post-Reduction Physiology

Expected changes:

Abdominal closure assessment:

Clinical Pearl: If primary closure causes haemodynamic or respiratory compromise, leave the abdomen open with silo or vacuum-assisted closure. The patient will not die from an open abdomen; they may die from compartment syndrome.

Fluid and Inotrope Management

Fluid strategy:

• Expect higher than normal requirements (third-spacing, leaky capillaries)
• 100-150% maintenance fluids
• Blood products as needed

Inotrope support:

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Postoperative Management

Ventilation Strategy

Duration:

• Continue mechanical ventilation 24-72 hours minimum
• Premature extubation risks reintubation, barotrauma, PPHN crisis

Weaning:

• Gradual reduction of support
• Extubate only when:
  • Minimal ventilator settings (PIP <20, FiO2 <40%)
  • Haemodynamically stable
  • No signs of PPHN
  • Adequate analgesia (but not excessive respiratory depression)

Rebound PPHN:

• Can occur 12-48 hours postoperatively
• Monitor closely; may need to reinstitute iNO

Complications

Immediate postoperative:

Long-term:

ECMO

Indications:

• Refractory hypoxia (OI >40 for 4 hours or >60 for 1 hour)
• Severe cardiac failure
• Failure to respond to maximal medical therapy

Types:

Contraindications:

• Severe chromosomal abnormalities
• Irreversible brain damage
• Severe cardiac anomalies not amenable to repair
• Gestational age <34 weeks or weight <2 kg (usually)

Outcomes:

• Survival with ECMO: 40-60% [7]
• Higher survival in late-presenting CDH (diagnosed after 24 hours)
• Risk factors for poor outcome: need for ECMO, major cardiac anomalies, liver herniation

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Fetal Intervention

Fetal Endoscopic Tracheal Occlusion (FETO)

Rationale:

• Tracheal occlusion stimulates lung growth (fluid accumulation expands lungs)
• Reversal before birth allows transition to neonatal breathing

Patient selection:

• Isolated left CDH
• O/E LHR 25-35% (severe but not lethal)
• Gestational age 27-30 weeks for occlusion

Procedure:

1. Tracheal occlusion with detachable balloon at 27-30 weeks
2. Balloon removed at 34 weeks or earlier if preterm labour
3. Delivery at tertiary centre with ECMO capability

Outcomes:

• Improves survival in severe CDH from 20-30% to 50-60%
• Risk of preterm delivery (balloon complications)
• Requires EXIT procedure if balloon cannot be removed before delivery [8]

EXIT Procedure

Ex utero intrapartum treatment:

• Partial delivery by C-section
• Placental circulation maintained
• Airway secured while on placental support
• Indicated for FETO patients or severe airway obstruction

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Indigenous Health Considerations

Aboriginal and Torres Strait Islander Families

Healthcare access challenges:

Aboriginal families living in remote or rural Australia face significant barriers to accessing paediatric surgical care:

Late presentation:

• Higher rates of postnatal diagnosis in remote areas
• Limited access to detailed anomaly scans
• Transport to tertiary centres may be delayed
• Cultural factors may influence acceptance of surgery

Cultural safety considerations:

1. Family involvement:

   • Extended family (kinship) involvement in decision-making
   • Elders may need to be consulted for major decisions
   • Respect for Sorry Business if complications occur

2. Communication:

   • Use Aboriginal Liaison Officers
   • Medical Interpreter services if English is not first language
   • Visual aids and diagrams helpful
   • "Teach-back" method to confirm understanding

3. Supporting families:

   • Patient-assisted travel schemes may not cover whole family
   • Financial burden of travel, accommodation, lost income
   • Consider social work involvement early
   • Maintain connection to Country where possible

4. Discharge planning:

   • Remote follow-up challenging
   • Telemedicine options for postoperative review
   • Clear written instructions in plain language
   • Local health service liaison

Māori Health (Aotearoa New Zealand)

Health disparities:

Māori infants face similar geographic and cultural barriers:

• Higher proportion living in rural areas
• Lower access to tertiary paediatric services
• Cultural expectations around whānau involvement

Whānau-centred care for CDH:

• Involve whānau in all care decisions
• Recognise that "family" includes extended whānau
• Respect for tikanga around birth and intensive care
• Māori Health Workers facilitate communication

Te Tiriti obligations:

• Equitable access to paediatric surgery
• Culturally safe care environments
• Māori input into care planning

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ANZCA Professional Standards

Relevant Guidelines

Neonatal Anaesthesia Requirements

Per PS46:

Personnel:

• Anaesthetist with paediatric and neonatal training
• Availability of paediatric anaesthesia 24/7 for emergencies
• Maintenance of paediatric skills through regular practice

Equipment:

• Neonatal-specific airway equipment (size 2.5-3.5 ETT, Miller 0/1 blades)
• Paediatric monitoring (probes, cuffs)
• Temperature control (incubator, warmers)
• Vascular access equipment (UVC/UAC, 24G cannulae)

Environment:

• Paediatric ICU with ECMO capability for CDH
• Cardiac surgery on-site or rapid transfer pathway
• Blood bank with neonatal products available

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Assessment Content

Short Answer Questions (SAQs)

SAQ 1: CDH Pathophysiology and Gentle Ventilation (20 marks)

Question:

A term neonate is born with antenatally diagnosed left-sided congenital diaphragmatic hernia. Outline the pathophysiology of CDH and explain the principles of "gentle ventilation" for this infant. (20 marks)

Model Answer:

Pathophysiology (10 marks):

Lung hypoplasia (4 marks):

• Failure of pleuroperitoneal fold fusion at 8 weeks gestation
• Abdominal viscera herniate into thorax, compressing developing lungs
• Reduced airway branching (normal 23-24 generations; CDH 15-20)
• Decreased alveolar number (normal 50 million; CDH 20-30 million)
• Surfactant deficiency from Type II pneumocyte hypoplasia

Pulmonary vascular disease (4 marks):

• Abnormal pulmonary vascular development
• Medial hypertrophy of arterioles
• Muscularisation extends to periphery
• Hyperreactive vasculature causing pulmonary hypertension
• Right-to-left shunting through PDA and foramen ovale

Cardiac effects (2 marks):

• Mediastinal shift compresses left heart
• Right ventricular pressure overload from PPHN
• Reduced cardiac compliance from external compression

Gentle Ventilation (10 marks):

Principles (4 marks):

• Hypoplastic lungs are non-compliant and easily overdistended
• High airway pressures cause barotrauma (pneumothorax)
• Overdistension compresses pulmonary vasculature, increasing PVR
• Goal is adequate gas exchange without lung injury

Specific parameters (4 marks):

• Peak inspiratory pressure (PIP) <20-25 cmH₂O
• PEEP 3-5 cmH₂O
• Respiratory rate 40-60 breaths/min
• Permissive hypercapnia: PaCO2 45-60 mmHg acceptable
• SpO2 target 80-95% (avoid hyperoxia)
• pH >7.25 acceptable

Rationale for outcomes (2 marks):

• Gentle ventilation reduced mortality from 60% to 20-30%
• Lower rates of pneumothorax (10-20% vs 50-60%)
• Improved survival through reduced barotrauma and better PVR management

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SAQ 2: CDH Anaesthetic Management (20 marks)

Question:

A 3-day-old infant with CDH is scheduled for surgical repair. Describe the preoperative assessment, intraoperative management, and specific complications you must anticipate. (20 marks)

Model Answer:

Preoperative Assessment (6 marks):

Clinical stability (3 marks):

• Minimum 48 hours stabilisation period
• pH >7.25, lactate <3 mmol/L
• Stable on gentle ventilation settings
• ECHO: no major cardiac anomalies, assess RV function
• Pre- and post-ductal SpO2 documented

Risk stratification (3 marks):

• Birth weight >1,800 g
• Best PaCO2 <60 mmHg
• Absence of major cardiac anomalies
• Liver position (intrathoracic = worse prognosis)
• O/E LHR if antenatally assessed

Intraoperative Management (8 marks):

Monitoring (2 marks):

• Arterial line (pre- and post-ductal if possible)
• Central venous pressure
• Temperature (maintain normothermia)
• 5-lead ECG

Induction and maintenance (3 marks):

• Opioid-based technique (fentanyl 5-10 mcg/kg)
• Avoid ketamine (↑ catecholamines, ↑ PVR)
• Avoid N2O (expands bowel, ↑ PVR)
• Muscle relaxation (rocuronium)
• Low-dose volatile (0.5 MAC) if needed

Ventilation (3 marks):

• Continue gentle ventilation principles
• Hand ventilation preferred
• PIP <20-25 cmH₂O
• Be prepared for sudden changes during reduction

Complications (6 marks):

Immediate (3 marks):

• Pneumothorax (10-20%) - chest drain
• Rebound PPHN post-reduction
• Hypotension from sudden increase in capacitance
• Bleeding from liver manipulation
• Abdominal compartment syndrome if forced closure

Postoperative (3 marks):

• Chylothorax (thoracic duct injury)
• Surgical emphysema
• Rebound PPHN (12-48 hours)
• Gastroesophageal reflux (50-70%)
• Recurrent hernia (5-10%)

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SAQ 3: PPHN and iNO in CDH (20 marks)

Question:

A neonate with CDH has persistent pulmonary hypertension despite gentle ventilation. Discuss the pathophysiology of PPHN in CDH and the role of inhaled nitric oxide (iNO) in management. (20 marks)

Model Answer:

PPHN Pathophysiology in CDH (8 marks):

Structural abnormalities (4 marks):

• Abnormal pulmonary vascular development
• Medial hypertrophy of pulmonary arterioles
• Reduced arterial number
• Muscularisation extends to arteriolar level
• Reduced cross-sectional area of pulmonary vascular bed

Functional abnormalities (4 marks):

• Hyperreactive vasculature
• ↑ PVR due to structural changes
• Hypoxia and acidosis exacerbate vasoconstriction
• Right-to-left shunting through PDA and foramen ovale
• Right ventricular pressure overload and dysfunction
• Systemic hypoxia despite high FiO2

iNO Role (12 marks):

Mechanism (3 marks):

• Selective pulmonary vasodilator
• Diffuses into vascular smooth muscle
• Activates guanylate cyclase → ↑ cGMP → vasodilation
• Effect limited to ventilated lung segments (matched V/Q)
• Minimal systemic effect (rapidly bound by haemoglobin)

Dosing (2 marks):

• Start at 20 ppm
• Wean gradually (20→10→5→2→1 ppm)
• Monitor methaemoglobin levels (risk with prolonged use >50 ppm)

Indications in CDH (3 marks):

• OI >20 despite gentle ventilation
• Refractory hypoxia with evidence of PPHN
• Right-to-left shunting documented on ECHO
• Response typically within hours (improved oxygenation)

Evidence and outcomes (2 marks):

• ~50% of CDH patients respond to iNO
• Improves oxygenation and reduces need for ECMO in responders
• Non-responders may still require ECMO

Limitations (2 marks):

• Expensive and requires special delivery system
• Not effective if structural vascular disease too severe
• Rebound PPHN can occur on withdrawal
• Does not address underlying lung hypoplasia

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Viva Voce Scenarios

Viva 1: Prenatal Counselling and Delivery Planning (15 marks)

Scenario: A 28-year-old woman at 22 weeks gestation has an antenatal ultrasound showing left CDH with liver herniation and O/E LHR of 30%. She is being counselled about delivery planning.

Examiner Questions:

Q1: "What factors determine the prognosis for this fetus?" (5 marks)

Model Answer:

• O/E LHR: 30% = moderate risk (25-35% = poor prognosis, survival 50-80%)
• Liver position: Intrathoracic liver = worse outcome (independent predictor of mortality)
• Side: Left-sided (most common, better prognosis than right)
• Associated anomalies: Need detailed anatomy survey, genetic testing
• Gestational age at diagnosis: Earlier diagnosis = more severe
• Fetal MRI: Can assess lung volume more accurately
• Cardiac anomalies: Present in 15-25% and worsen prognosis

Q2: "Where should delivery occur and why?" (5 marks)

Model Answer:

• Tertiary centre with paediatric surgery and ECMO capability
• Immediate access to:
  • Neonatal intensive care (ventilation, iNO)
  • Paediatric surgery
  • ECMO if needed
  • Cardiac surgery if associated anomalies
• Retrieval after birth high-risk
• Prenatal transfer of mother preferred to postnatal transfer of neonate

Q3: "Would you recommend fetal intervention?" (5 marks)

Model Answer:

• FETO considered for O/E LHR 25-35%
• This patient at threshold (30%)
• Requires referral to fetal medicine centre
• FETO involves tracheal occlusion at 27-30 weeks
• Balloon removal at 34 weeks or if preterm labour
• Improves survival from 20-30% to 50-60%
• Risks: Preterm delivery, balloon complications, need for EXIT
• Requires careful counselling and informed consent

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Viva 2: Intraoperative Crisis (15 marks)

Scenario: You are anaesthetising a 2-day-old with CDH for repair. During reduction of the hernia, the oxygen saturation suddenly drops from 85% to 50%, and the patient becomes hypotensive.

Examiner Questions:

Q1: "What is your differential diagnosis?" (5 marks)

Model Answer:

• Pneumothorax (most likely) - tension pneumothorax from barotrauma or surgical manipulation
• PPHN crisis - sudden increase in PVR
• Cardiac compression - mediastinal manipulation compromising heart
• Severe hypovolaemia - bleeding or sudden capacitance increase
• Airway problem - ETT displacement, obstruction, kinking
• Equipment failure - disconnected circuit, oxygen failure

Q2: "What are your immediate management steps?" (5 marks)

Model Answer:

• Call for help and communicate with surgeon
• Check ETT position (displacement, obstruction)
• Hand ventilate with 100% O2
• Check for pneumothorax (asymmetry, decreased breath sounds) - if suspected, immediate chest drain
• Fluid bolus 10-20 mL/kg if hypovolaemic
• Inotropes (adrenaline bolus or increase infusion)
• iNO if available and not already running
• Consider tension pneumothorax - if suspected and patient unstable, immediate needle decompression (2nd intercostal space mid-clavicular line)

Q3: "How do you prevent this complication?" (5 marks)

Model Answer:

• Gentle ventilation throughout - never exceed PIP 20-25 cmH₂O
• Hand ventilation during surgical manipulation (detect compliance changes)
• Communication with surgeon during critical phases (reduction, liver manipulation)
• N2O avoidance (increases PVR, expands bowel)
• Adequate depth before surgical stimulation
• Prophylactic chest drain on ipsilateral side (some surgeons place prophylactically)
• Close monitoring of airway pressures and SpO2 trends

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