Tracheo-Oesophageal Fistula (TOF)
TOF is a congenital anomaly where the trachea and oesophagus fail to separate during embryological development, creating abnormal connections. Oesophageal atresia (OA) is usually present. Key anaesthetic principles:
Clinical board
A visual summary of the highest-yield teaching signals on this page.
Urgent signals
Safety-critical features pulled from the topic metadata.
- severe aspiration
- gastric perforation
- severe pneumonia
- cardiac failure
Exam focus
Current exam surfaces linked to this topic.
- ANZCA Final Written
- ANZCA Final Viva
Editorial and exam context
Tracheo-Oesophageal Fistula (TOF)
Quick Answer
What is tracheo-oesophageal fistula?
TOF is a congenital anomaly where the trachea and oesophagus fail to separate during embryological development, creating abnormal connections. Oesophageal atresia (OA) is usually present. Key anaesthetic principles:
- Types: Gross C (OA with distal TEF) is most common (85%); Gross A (isolated OA) 8%; H-type 4%
- VACTERL association: 50-60% have associated anomalies; cardiac evaluation mandatory
- Airway risk: Positive pressure ventilation causes gastric distension via fistula → respiratory failure
- Spontaneous ventilation: Maintain until fistula identified and controlled
- ETT positioning: Tip must be distal to fistula (at carina or right main bronchus)
Clinical Pearl: TOF is a true airway emergency. The fistula is a direct communication between airway and stomach. Positive pressure ventilation without controlling the fistula = gastric overdistension = respiratory and cardiac arrest.
Clinical Overview
Definition
Tracheo-oesophageal fistula represents a spectrum of congenital anomalies characterised by:
- Oesophageal atresia (OA): Discontinuity of the oesophagus
- Tracheo-oesophageal fistula (TEF): Abnormal communication between trachea and oesophagus
These anomalies result from failed separation of the foregut into the respiratory (trachea) and gastrointestinal (oesophagus) tracts during the 4th week of gestation.
Epidemiology
- Incidence: 1 in 2,500-4,500 live births [1]
- Males: Slight predominance (male:female ratio 1.5:1)
- Familial recurrence: 1-2% if one sibling affected; higher if two affected
- Associated anomalies: 50-60% (VACTERL association) [2]
Embryology
Normal foregut development (week 4):
- Primitive foregut divides into ventral (respiratory) and dorsal (oesophageal) portions
- Tracheo-oesophageal septum forms and grows caudally
- Complete separation by week 5
Pathogenesis of TOF:
- Abnormal development of tracheo-oesophageal septum
- Exact mechanism unclear (mechanical, vascular, or genetic theories)
- Earlier disruption = more severe anomalies
Classification
Gross Classification
The Gross classification (modified) categorises TOF by anatomy:
| Type | Anatomy | Frequency | Diagram |
|---|---|---|---|
| C | OA with distal TEF (most common) | 85% | Upper pouch + fistula from distal oesophagus to trachea |
| A | OA only (no fistula) | 8% | Two blind-ending oesophageal pouches |
| E (H-type) | H-type TEF without OA | 4% | Fistula only, continuous oesophagus |
| B | OA with proximal TEF | 1% | Fistula from upper pouch to trachea |
| D | OA with proximal AND distal TEF | 1-2% | Two fistulae |
| F | Oesophageal stenosis | Rare | Narrowing without atresia |
Memory Aid: Type C is Common (85%). Think "C" for common, "A" for absent (fistula).
Clinical Implications by Type
Type C (OA + distal TEF):
- Gastric distension: Air flows through fistula into stomach during crying/ventilation
- Reflux aspiration: Gastric contents reflux through fistula into lungs
- Chemical pneumonitis: Acid aspiration causes lung injury
- High risk: Positive pressure ventilation without fistula control
Type A (Isolated OA):
- No fistula = no gastric distension risk
- Long gap between oesophageal pouches (often >2 vertebral bodies)
- May require staged repair (gastrostomy, delayed primary repair)
Type E (H-type):
- Present later (recurrent chest infections, feeding difficulties)
- Diagnosis challenging (may need bronchoscopy, contrast swallow)
- Surgical approach usually thoracoscopic or cervical
Associated Anomalies (VACTERL)
Definition
VACTERL is an acronym for associated congenital anomalies:
| Letter | Anomaly | Frequency in TOF |
|---|---|---|
| V | Vertebral anomalies | 20-30% |
| A | Anal atresia | 15-20% |
| C | Cardiac anomalies | 30-35% [3] |
| T | Tracheal anomalies | 10-20% |
| E | Esophageal atresia (TEF) | 100% (by definition) |
| R | Renal anomalies | 15-25% |
| L | Limb anomalies | 10-15% |
Clinical Pearl: Cardiac anomalies occur in 30-35% of TOF patients and are the major determinant of survival. ECHO is mandatory preoperatively.
Cardiac Anomalies
Common lesions:
| Lesion | Frequency | Impact on Surgery |
|---|---|---|
| VSD | 10-15% | May require separate surgery |
| ASD/PDA | 10% | Often minor |
| Tetralogy of Fallot | 5-8% | Major impact; staged repair |
| Right-sided aortic arch | 5% | Alters surgical approach (left thoracotomy) |
| Coarctation | 2-5% | May need prior repair |
Critical implications:
- Right-sided aortic arch: Requires left thoracotomy (rather than standard right)
- Severe cardiac lesions: May delay TOF repair
- Duct-dependent lesions: May require prostaglandin E1
Other Important Associations
| Anomaly | Significance |
|---|---|
| Duodenal atresia | "Double bubble" on X-ray; requires separate repair |
| Malrotation | May need Ladd's procedure |
| Anal atresia | Defunctioning colostomy may be needed |
| Vertebral anomalies | Scoliosis risk; may affect positioning |
| Tracheomalacia | Floppy trachea causing airway obstruction post-repair |
| CHARGE association | Coloboma, Heart defects, Atresia choanae, Retarded growth, Genital anomalies, Ear anomalies |
Pathophysiology
Respiratory Consequences
Proximal oesophageal pouch:
- Cannot swallow saliva or feeds
- Accumulation of secretions → aspiration risk
- Requires continuous suction (Replogle tube)
Distal tracheo-oesophageal fistula:
- Airway connection: Allows air into stomach
- Gastric distension: During crying or positive pressure ventilation
- Reflux pathway: Gastric contents reflux into lungs
- Aspiration pneumonia: Chemical and bacterial
Severity determinants:
| Factor | Effect |
|---|---|
| Fistula size | Larger = more air into stomach |
| Distal oesophageal muscle tone | Low tone = more reflux |
| Gastric outlet obstruction | Increases reflux risk |
| Prematurity | Poor respiratory reserve |
Gastric Consequences
Overdistension mechanisms:
| Mechanism | Pathway |
|---|---|
| Crying | Increased intrathoracic pressure forces air through fistula |
| Bag-mask ventilation | Positive pressure → fistula → stomach |
| Intubation attempts | Positive pressure during laryngoscopy |
| Controlled ventilation | If ETT above fistula |
Consequences of gastric distension:
- Elevated diaphragm → reduced lung compliance
- Impaired ventilation
- Vena caval compression → reduced venous return
- Reduced cardiac output
- Gastric perforation (rare but catastrophic)
Diagnosis and Presentation
Prenatal Diagnosis
Ultrasound findings:
| Finding | Sensitivity | Significance |
|---|---|---|
| Polyhydramnios | 60-70% | Absent foetal swallowing |
| Small/absent stomach bubble | 50% | Inability to swallow amniotic fluid |
| Pouch sign | 30% | Dilated proximal oesophageal pouch |
Limitations:
- Detection rates vary (30-60% diagnosed antenatally)
- Isolated H-type rarely detected
- Cannot determine presence of fistula reliably
Postnatal Presentation
Immediate presentation:
| Symptom | Mechanism |
|---|---|
| Drooling/excess secretions | Cannot swallow |
| Choking with first feed | Aspiration into airway |
| Cyanosis during feeds | Aspiration, airway obstruction |
| Respiratory distress | Aspiration pneumonia, gastric distension |
| Scaphoid abdomen | Air preferentially entering chest via fistula |
Diagnostic confirmation:
| Investigation | Finding |
|---|---|
| Unable to pass NG tube | NG tube coils in proximal pouch (pathognomonic) |
| Chest X-ray | NG tube in pouch (level T2-T4), gas in bowel (Type C), airless abdomen (Type A) |
| ECHO | Cardiac anatomy, right-sided arch |
| Renal ultrasound | Exclude renal anomalies |
| Spine X-ray | Vertebral anomalies |
Preoperative Management
Initial Stabilisation
Immediate priorities:
| Action | Rationale |
|---|---|
| NPO (nil per os) | Prevent aspiration |
| Head-up position (30-45°) | Reduce reflux |
| Replogle tube | Continuous suction of proximal pouch |
| Suction settings | Low pressure (-20 to -30 cmH₂O) |
Replogle tube technique:
- 10-12 Fr double-lumen tube
- Placed in proximal oesophageal pouch
- Connected to continuous low-pressure suction
- Prevents saliva accumulation and aspiration
Timing of Surgery
Primary repair (preferred):
- Timing: Within 24-48 hours if stable
- Advantages: Single procedure, earlier oral feeding
- Requirements: Stable cardiorespiratory status, <1500 g usually still attempted
Delayed repair:
- Indications:
- Severe pneumonia
- Significant cardiac anomaly requiring prior intervention
- Extreme prematurity (<1000 g)
- Long-gap oesophageal atresia (Type A)
- Approach: Gastrostomy for feeds, cervical oesophagostomy, delayed repair at 3-6 months
Gastrostomy indications:
- Severe aspiration pneumonia (prevents further aspiration)
- Severe cardiac disease delaying repair
- Long-gap atresia (>2 cm)
- Extreme prematurity
Preoperative Assessment Checklist
| System | Assessment |
|---|---|
| Respiratory | CXR for pneumonia, pneumonitis, gastric distension |
| Cardiac | ECHO mandatory (VACTERL association) |
| Renal | Ultrasound (VACTERL association) |
| Skeletal | Spine X-ray, limb examination |
| Anal | Patency of anus |
| Prematurity | Gestational age, weight, maturity |
| Nutrition | IV fluids, dextrose |
Anaesthetic Management
The TOF Airway Challenge
The fundamental problem:
- Positive pressure ventilation forces air through the fistula
- Gastric distension compromises respiratory and cardiac function
- The patient can die before surgery even begins
Anaesthetic priorities:
- Prevent gastric distension
- Secure airway below the fistula
- Control ventilation only after fistula isolated
Induction Strategy
The debate: Spontaneous vs controlled ventilation
| Approach | Advantages | Disadvantages |
|---|---|---|
| Spontaneous ventilation | No positive pressure → no gastric distension | Difficult to maintain during surgery; risk of movement |
| Controlled ventilation | Controlled conditions | Risk of gastric distension if ETT above fistula |
Current consensus:
- Maintain spontaneous ventilation until fistula identified and controlled
- Accept higher risk of aspiration from full stomach (vs guaranteed gastric distension with positive pressure)
- Exception: Severe aspiration pneumonia where positive pressure needed for oxygenation
Induction technique:
| Step | Action |
|---|---|
| 1 | Position: Head-up 30° (reduce reflux) |
| 2 | Replogle tube: Suction on, note quantity/quality of secretions |
| 3 | Preoxygenation: 3 minutes if stable |
| 4 | Induction: Gentle IV (propofol 2-3 mg/kg + fentanyl 2-3 mcg/kg) or inhalational (sevoflurane) |
| 5 | Critical: Maintain spontaneous breathing |
| 6 | Muscle relaxant: Avoid until airway secured below fistula (or surgeon present) |
Clinical Pearl: Some centres use awake intubation with the surgeon scrubbed and ready to identify the fistula immediately. This is the safest approach in experienced hands.
Intubation Technique
The critical step: The ETT must be positioned distal to the fistula to prevent air entering the stomach.
Technique:
| Step | Action |
|---|---|
| 1 | Prepare: Surgeon present, suction ready, different ETT sizes |
| 2 | Size: 3.0-3.5 mm uncuffed for term neonate |
| 3 | Position: Advance beyond carina into right main bronchus OR position just above carina |
| 4 | Confirmation: Auscultation (right lung only if RMB intubation), EtCO2 |
| 5 | Once surgeon identifies and controls fistula: Withdraw ETT to trachea |
Right main bronchus intubation:
- Acceptable temporarily to ensure ventilation distal to fistula
- Risk of right lung overdistension, left lung collapse
- Once fistula controlled, withdraw to mid-trachea
Alternative Airway Strategies
Fogarty catheter occlusion:
- 3 Fr Fogarty catheter passed through mouth into fistula
- Balloon inflated to occlude fistula
- Allows controlled ventilation with ETT above fistula
- Disadvantages: Dislodgement risk, airway trauma
Bronchoscopy:
- Rigid or flexible bronchoscopy to identify fistula location
- Rarely used (adds time, risk)
- May be helpful for difficult anatomy or H-type fistula
Emergency gastrostomy:
- If severe gastric distension occurs during induction
- Surgeon creates gastrostomy under local anaesthesia
- Decompresses stomach, allows definitive repair later
Maintenance
Once fistula controlled:
| Parameter | Management |
|---|---|
| Ventilation | Convert to gentle positive pressure |
| Muscle relaxant | Rocuronium 0.6 mg/kg, maintain with boluses or infusion |
| Analgesia | Fentanyl 5-10 mcg/kg/hr (blunts stress response) |
| Monitoring | Arterial line (frequent gases), temperature |
N2O:
- Contraindicated - expands bowel, increases mediastinal pressure
Surgical Approaches
Thoracotomy:
- Standard: Right posterolateral thoracotomy (4th intercostal space)
- Alternative: Left thoracotomy if right-sided aortic arch
- Technique: Ligation of fistula, primary oesophageal anastomosis
Thoracoscopic repair:
- Increasingly used in stable patients
- Advantages: Less pain, better cosmesis
- Disadvantages: Longer operative time, CO2 insufflation challenges, steep learning curve
Anaesthetic considerations during surgery:
| Phase | Risk |
|---|---|
| Pleural opening | Loss of tidal volume, atelectasis |
| Fistula identification | Critical moment - surgeon identifies and occludes |
| Retraction | Impaired venous return, mediastinal compression |
| Oesophageal anastomosis | Risk of tracheal compression |
Extubation Strategy
Primary extubation (goal):
- Safe in most infants if:
- No severe pneumonia
- No significant cardiac disease
- Minimal leak at anastomosis
- Born >35 weeks
Deferred extubation:
- Severe aspiration pneumonia
- Significant cardiac disease
- Prematurity (<32 weeks)
- Technical difficulties with anastomosis
- Tracheomalacia documented
Postoperative ventilation:
- Minimise positive pressure on fresh anastomosis
- Paralyse if ventilated (prevent anastomotic disruption)
- Early extubation when criteria met
Postoperative Management
Immediate Postoperative
ICU admission:
- All patients require neonatal ICU observation
- Monitor for anastomotic leak, stricture, recurrence
Feeding:
- Timing: 48-72 hours after repair (contrast swallow first)
- Method: Start with small feeds, advance gradually
- Position: Head-up to reduce reflux
Analgesia:
- Paracetamol regularly
- Opioids as needed (morphine 0.05-0.1 mg/kg q4h)
- Regional techniques (paravertebral, caudal) if thoracotomy
Complications
Early complications (<7 days):
| Complication | Incidence | Management |
|---|---|---|
| Anastomotic leak | 10-15% | Conservative if contained; reoperation if mediastinitis |
| Recurrent fistula | 5-10% | Reoperation usually required |
| Anastomotic stricture | 10-30% | Balloon dilatation |
| Tracheomalacia | 10-20% | Aortopexy if severe |
| Chylothorax | 5% | Thoracic duct injury; dietary modification |
Late complications:
| Complication | Mechanism |
|---|---|
| Gastroesophageal reflux | 30-50%; anatomic distortion, motility issues |
| Oesophageal dysmotility | Abnormal innervation, surgical manipulation |
| Respiratory infections | Tracheomalacia, aspiration history |
| Growth failure | Chronic reflux, feeding difficulties |
| Oesophageal stricture | Scar tissue at anastomosis |
Long-Gap Oesophageal Atresia
Definition
Gap between oesophageal pouches >2 cm (approximately 2 vertebral bodies) or >10% of patient length.
Causes:
- True Type A (isolated OA)
- Type C with proximal pouch that retracts superiorly
- "Gap" varies with respiratory excursion
Management Options
| Approach | Indication | Technique |
|---|---|---|
| Primary repair with traction | Gap 2-3 cm | Sutures on both pouches, daily traction |
| Delayed primary repair | Gap >3 cm | Growth with feeds via gastrostomy |
| Oesophageal replacement | Very long gap | Gastric pull-up, colon interposition, jejunal interposition |
| Foker process | Long gap | Extra-pleural traction sutures with daily tightening |
Anaesthetic implications:
- Multiple procedures over months
- Need for staged approaches
- Gastroscopy and bronchoscopy under GA for assessment
Indigenous Health Considerations
Aboriginal and Torres Strait Islander Families
Healthcare access challenges:
TOF management requires:
- Immediate access to paediatric surgery
- Prolonged ICU care
- Potential for multiple procedures (if complications)
- Long-term follow-up
| Challenge | Impact on TOF Care |
|---|---|
| Geographic distance | Prenatal diagnosis may be missed; late presentation |
| Retrieval logistics | Need urgent transfer to tertiary centre |
| Family separation | Extended ICU stay separates families |
| Communication | Complex surgical concepts difficult to convey |
| Cultural factors | Family decision-making, kinship obligations |
Late presentation:
- Limited access to detailed anomaly scans in remote areas
- Community births without immediate medical care
- Presentation with established aspiration pneumonia
- Higher morbidity due to delayed diagnosis
Cultural safety in TOF care:
-
Communication during crisis:
- Emergency nature limits time for extended family consultation
- Use Aboriginal Liaison Officers immediately
- Visual aids to explain anatomy
- "Teach-back" to ensure understanding of feeding restrictions
-
Family support:
- Patient-assisted travel schemes
- Accommodation near hospital
- Social work involvement for practical support
- Consideration of family income loss during prolonged stay
-
Postoperative discharge:
- Remote follow-up challenging
- Telemedicine for feeding assessments
- Local health service liaison for reflux management
- Recognition that "routine" follow-up may be difficult
Māori Health (Aotearoa New Zealand)
Health disparities: Māori infants face similar barriers:
- Geographic distance to Starship or other paediatric centres
- Higher rates of socio-economic deprivation
- Cultural expectations around whānau involvement
Whānau-centred care:
- Involve whānau in care decisions despite urgency
- Recognise stress of separation from extended family
- Respect for tikanga around birth and surgery
- Māori Health Workers to facilitate communication
Practical considerations:
- Whānau may need to travel from rural areas
- Accommodation and financial support
- Clear discharge instructions for rural GPs
ANZCA Professional Standards
Relevant Documents
| Document | Application |
|---|---|
| PS08 | Anaesthesia for the unwell neonate |
| PS09 | Emergency surgery (TOF is semi-urgent) |
| PS18 | Transport of critically ill (retrieval to surgical centre) |
| PS46 | Paediatric anaesthesia requirements |
TOF-Specific Requirements
Personnel:
- Anaesthetist with neonatal airway experience
- Surgeon experienced in neonatal thoracic surgery
- Paediatric ICU for postoperative care
Equipment:
- Neonatal airway equipment (Miller 0/1 blades, sizes 2.5-3.5 ETT)
- Ventilator capable of neonatal ventilation
- Fibreoptic bronchoscopy (if available)
- Fogarty catheters (3 Fr) for fistula occlusion
Environment:
- Operating theatre with warming facilities
- Paediatric ICU immediately available
- Blood products if needed
Assessment Content
Short Answer Questions (SAQs)
SAQ 1: TOF Classification and Airway Management (20 marks)
Question:
A term neonate is born with excessive oral secretions and fails to pass a nasogastric tube. A diagnosis of oesophageal atresia with tracheo-oesophageal fistula is made. Describe the classification of TOF and outline the specific airway management considerations. (20 marks)
Model Answer:
Classification (8 marks):
Gross types (5 marks):
- Type C (85%): Oesophageal atresia with distal tracheo-oesophageal fistula - most common
- Type A (8%): Isolated oesophageal atresia without fistula - long gap
- Type E/H-type (4%): Tracheo-oesophageal fistula without atresia - presents later
- Type B (1%): Oesophageal atresia with proximal fistula
- Type D (1-2%): Oesophageal atresia with both proximal and distal fistulae
Associated anomalies (3 marks):
- VACTERL association in 50-60% of patients
- Cardiac anomalies in 30-35% (ECHO mandatory)
- Right-sided aortic arch in 5% (alters surgical approach)
- Vertebral, renal, anal anomalies also common
Airway Management (12 marks):
Fundamental problem (3 marks):
- Distal fistula (Type C) connects airway directly to stomach
- Positive pressure ventilation forces air through fistula
- Gastric distension compromises respiration and circulation
Induction strategy (4 marks):
- Maintain spontaneous ventilation until fistula controlled
- Head-up position (30-45°) to reduce reflux
- Gentle IV induction (propofol + fentanyl) or inhalational
- Surgeon present and ready to identify fistula
Intubation technique (3 marks):
- Size 3.0-3.5 mm uncuffed ETT
- Position tip distal to fistula (at carina or right main bronchus)
- Verify position with auscultation and EtCO2
- Once surgeon isolates fistula, convert to controlled ventilation
Alternative strategies (2 marks):
- Fogarty catheter through fistula with balloon occlusion
- Awake intubation in experienced hands
- Emergency gastrostomy if severe distension occurs
SAQ 2: Gastric Distension Crisis (20 marks)
Question:
During induction of anaesthesia for TOF repair, despite attempts at spontaneous ventilation, the infant develops massive abdominal distension, desaturates to 60%, and becomes bradycardic. Describe the pathophysiology and immediate management. (20 marks)
Model Answer:
Pathophysiology (8 marks):
Mechanism (4 marks):
- Positive pressure ventilation (even gentle) forces air through fistula
- Air enters stomach via distal TEF
- Gastric overdistension occurs rapidly
- Elevated diaphragm reduces lung compliance and FRC
- Vena caval compression reduces venous return
- Reduced cardiac output and coronary perfusion
Clinical consequences (4 marks):
- Severe hypoxia (ventilation compromised)
- Hypotension (↓ venous return)
- Bradycardia (hypoxia + vagal response)
- Cardiac arrest risk if not immediately corrected
- Risk of gastric perforation if severe
Immediate Management (12 marks):
First response (6 marks):
- Stop positive pressure ventilation immediately
- Release cricoid pressure (if applied)
- Allow spontaneous breathing if possible
- Emergency gastric decompression:
- Attempt orogastric tube passage (often fails - obstruction)
- Surgeon to perform emergency gastrostomy (local anaesthesia if needed)
- Hand ventilate with minimal pressure once stomach decompressed
- Call for help and prepare for emergency surgery
Airway rescue (4 marks):
- Once stomach decompressed, position ETT distal to fistula
- Surgeon to identify and occlude fistula urgently
- Fogarty catheter occlusion if available
- Consider right main bronchus intubation temporarily
Cardiovascular support (2 marks):
- Volume bolus if hypovolaemic
- Atropine for bradycardia (20 mcg/kg)
- Adrenaline if cardiac arrest imminent
SAQ 3: VACTERL Association (20 marks)
Question:
A 2-day-old infant with TOF is found to have a right-sided aortic arch on preoperative ECHO. Discuss the VACTERL association, the implications of right-sided aortic arch for surgical planning, and the preoperative assessment required. (20 marks)
Model Answer:
VACTERL Association (8 marks):
Components (4 marks):
- Vertebral anomalies (20-30%): Hemivertebrae, scoliosis
- Anal atresia (15-20%): Imperforate anus
- Cardiac anomalies (30-35%): VSD, TOF, right-sided arch
- Tracheal anomalies (10-20%): Tracheomalacia
- Esophageal atresia with TEF (100% - by definition)
- Renal anomalies (15-25%): Hydronephrosis, agenesis
- Limb anomalies (10-15%): Radial abnormalities, polydactyly
Implications (4 marks):
- 50-60% of TOF patients have VACTERL features
- Cardiac anomalies are major determinant of survival
- Multiple systems may require surgical intervention
- Need coordinated multidisciplinary care
- Genetic counselling for families
Right-Sided Aortic Arch (6 marks):
Implications for TOF repair (4 marks):
- Standard approach is right thoracotomy
- Right-sided arch crosses right bronchus → obscures fistula
- Risk of aortic injury during dissection
- Surgical approach: Left thoracotomy required
- Increases operative complexity and risk
Anaesthetic implications (2 marks):
- Surgeon must be aware preoperatively
- Different positioning (left lateral)
- Potential for different pain distribution
- May require longer operative time
Preoperative Assessment (6 marks):
Mandatory investigations (3 marks):
- ECHO: Full cardiac anatomy, arch side, associated lesions
- Renal ultrasound: Exclude renal anomalies (VACTERL)
- Spine X-rays: Vertebral anomalies (VACTERL)
Additional assessment (3 marks):
- Anal examination for patency
- Limb examination for anomalies
- Review of CXR for pneumonia/pneumonitis
- Respiratory status and oxygen requirements
- Nutritional status and fluid balance
- Coagulation studies if indicated
Viva Voce Scenarios
Viva 1: Intraoperative Crisis (15 marks)
Scenario: You are anaesthetising a 1-day-old with TOF for repair. After induction, you are attempting to position the ETT below the fistula when the oxygen saturation suddenly drops to 50% and the abdomen becomes distended.
Examiner Questions:
Q1: "What has happened and why?" (5 marks)
Model Answer:
- The ETT tip is positioned above the fistula (not below as intended)
- Positive pressure ventilation (from hand bagging or spontaneous breaths against closed glottis) is forcing air through the fistula
- Air is entering the stomach, causing rapid gastric overdistension
- Elevated diaphragm is reducing lung compliance and functional residual capacity
- Vena caval compression is reducing venous return and cardiac output
- This is the feared complication of TOF anaesthesia
Q2: "What are your immediate management steps?" (5 marks)
Model Answer:
- Stop positive pressure ventilation immediately - release any manual ventilation
- Allow spontaneous breathing if the patient is trying to breathe
- Reposition the ETT - advance it distally, potentially into the right main bronchus temporarily
- Check for gastric decompression - attempt orogastric tube (though likely to coil in pouch)
- Call the surgeon immediately to prepare for urgent gastrostomy if distension doesn't resolve
- Hand ventilate gently with minimal pressure only once ETT repositioned
Q3: "How would you prevent this complication?" (5 marks)
Model Answer:
- Maintain spontaneous ventilation until the surgeon is present and ready to identify the fistula
- Have the surgeon scrubbed and ready before induction in high-risk cases
- Consider awake intubation in experienced hands
- Fogarty catheter - pass through the mouth into the fistula and inflate to occlude it
- Surgical decompression - if severe distension occurs, emergency gastrostomy under local
- Close communication with surgeon throughout induction
Viva 2: Type A Oesophageal Atresia (15 marks)
Scenario: A 3-day-old infant has been diagnosed with isolated oesophageal atresia (Type A, "long gap") on chest X-ray showing an airless abdomen and NG tube coiled at T2.
Examiner Questions:
Q1: "How does Type A differ from the more common Type C?" (5 marks)
Model Answer:
- Type A: Isolated oesophageal atresia with NO fistula (8% of cases)
- Type C: Oesophageal atresia WITH distal tracheo-oesophageal fistula (85% of cases)
Key differences:
- No fistula in Type A = no risk of gastric distension with ventilation
- Airless abdomen on X-ray (no air passing to bowel)
- Often "long gap" between oesophageal segments (>2 cm)
- Cannot do immediate primary repair if gap too long
- No reflux of gastric contents into lungs (lower aspiration risk)
Q2: "What is your anaesthetic approach for this baby?" (5 marks)
Model Answer:
- Much lower airway risk - no fistula to cause gastric distension
- Standard neonatal anaesthetic technique
- Controlled ventilation is safe (no fistula)
- However: Still need careful approach
- Prematurity, associated anomalies (still VACTERL association)
- May need gastrostomy first, then delayed repair
- If primary repair attempted, may be prolonged
- Standard induction: IV or inhalational
- Muscle relaxant safe once airway secured
- Postoperative ventilation likely (fresh anastomosis under tension)
Q3: "What are the management options for long-gap atresia?" (5 marks)
Model Answer:
- Delayed primary repair: Growth with gastrostomy feeds, repair at 3-6 months
- Traction techniques: Foker process (daily traction sutures), growth induction
- Oesophageal replacement: If gap too long for primary repair
- Gastric pull-up
- Colon interposition
- Jejunal interposition
- Staged approach: Cervical oesophagostomy (spit), gastrostomy feeds, later reconstruction
- Choice depends on gap length, surgeon preference, patient factors
References
-
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Spitz L. Oesophageal atresia. Orphanet J Rare Dis. 2007;2:24. PMID: 17498282
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Chittmittrapap S, Spitz L, Kiely EM, et al. Anastomotic leakage following surgery for esophageal atresia. J Pediatr Surg. 1992;27(1):29-32. PMID: 1552406
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Pedersen RN, Calzolari E, Husby S, et al. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child. 2012;97(3):227-232. PMID: 21857020
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Beasley SW, Qi BQ. Understanding the embryology and pathogenesis of esophageal atresia. J Pediatr Surg. 2004;39(9):1428-1430. PMID: 15300533
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