Oesophageal Atresia

Question 1

What is oesophageal atresia?

Answer 1

Oesophageal atresia is a congenital interruption of the oesophagus, often accompanied by a fistula between the distal oesophagus and the trachea.

Question 2

What causes oesophageal atresia?

Answer 2

The exact cause is not fully understood, and debate about the embryopathologic process continues. No known human teratogens are associated with it.

Question 3

What is the recurrence risk of oesophageal atresia if a sibling is affected?

Answer 3

There is a 2% risk of recurrence when a sibling has been affected.

Question 4

What problems does oesophageal atresia cause in affected infants?

Answer 4

It prevents normal feeding, leading to pooling of saliva and potential aspiration. If a fistula is present, saliva or gastric secretions may enter the lungs.

Question 5

What is the incidence of oesophageal atresia?

Answer 5

Approximately 1 in 3,000 live births.

Question 6

What antenatal condition is associated with oesophageal atresia?

Answer 6

Maternal polyhydramnios occurs in about 30% of cases.

Question 7

How effective is ultrasound in diagnosing oesophageal atresia antenatally?

Answer 7

Ultrasound is not very sensitive, with findings like an absent or small stomach bubble and polyhydramnios present in about 40% of cases.

Question 8

How common is premature birth in infants with oesophageal atresia?

Answer 8

Approximately one-third of affected infants are born prematurely.

Question 9

What are common postnatal symptoms of oesophageal atresia?

Answer 9

• Excessive drooling or frothing of saliva from the mouth
• Respiratory distress after feeding (coughing, cyanosis from reflexive bronchospasm)
• Tachypnoea
• Aspiration pneumonia
• Stridor due to tracheomalacia

Question 10

What is the most common cause of stridor in infants with oesophageal atresia?

Answer 10

Tracheomalacia

Question 11

What proportion of infants with oesophageal atresia have associated congenital abnormalities?

Answer 11

Around 50% of cases have associated congenital abnormalities.

Question 12

What is the VACTERL association in relation to oesophageal atresia?

Answer 12

VACTERL is a group of congenital anomalies that may be associated with oesophageal atresia, particularly including cardiac anomalies.

Question 13

What chromosomal syndromes are associated with oesophageal atresia?

Answer 13

About 5% of cases are associated with chromosomal syndromes, such as trisomy 18 and trisomy 21.

Question 14

How is oesophageal atresia diagnosed?

Answer 14

Diagnosis is made by the inability to pass a firm, large-bore (size 8 French) nasogastric tube beyond approximately 10 cm from the nares.

Question 15

What radiological sign is indicative of oesophageal atresia?

Answer 15

The “coiled nasogastric tube” sign on a chest X-ray, showing the nasogastric tube coiled in the proximal atretic oesophageal pouch.

Question 16

What percentage of oesophageal atresia cases involve a distal tracheo-oesophageal fistula (TOF)?

Answer 16

Approximately 85% of oesophageal atresia cases have a distal TOF.

Question 17

How is a distal tracheo-oesophageal fistula identified?

Answer 17

It is indicated by the presence of air in the stomach below the diaphragm on imaging.

Question 18

How common is a proximal tracheo-oesophageal fistula, and how is it identified?

Answer 18

A proximal TOF is found in less than 3% of cases and is typically identified during surgery through pre-operative bronchoscopy.

Question 19

What is an isolated tracheo-oesophageal fistula (TOF)?

Answer 19

It is a very rare condition where the trachea and oesophagus are connected by a fistula, but the oesophagus itself remains patent (open).

Question 20

How is isolated TOF typically suspected?

Answer 20

It is suspected based on recurrent aspiration pneumonia and respiratory symptoms associated with feeding.

Question 21

How is an isolated TOF confirmed?

Answer 21

Confirmation is done through a contrast study ± bronchoscopy.

Question 22

What does a “TOF study” involve?

Answer 22

A TOF study involves injecting contrast via a nasogastric tube and slowly withdrawing it with the patient in a prone position under fluoroscopy to reveal the characteristic “H-shaped” fistula.

Question 23

What proportion of infants with oesophageal atresia are born prematurely?

Answer 23

Approximately one-third of affected infants are born prematurely.

Question 24

What is the VACTERL association, and how common is it in oesophageal atresia cases?

Answer 24

VACTERL association is a group of congenital anomalies that occur together and is present in about 25% of oesophageal atresia cases.

Question 25

What does the acronym “VACTERL” stand for?

Answer 25

V: Vertebral defects • A: Anorectal malformations • C: Cardiovascular defects (35% of cases) • T: Tracheo-oesophageal defects • R: Renal anomalies (20% of cases) • L: Limb deformities (dysplasia)

Question 26

Which cardiovascular defects are commonly associated with oesophageal atresia?

Answer 26

Cardiovascular defects occur in approximately 35% of cases with oesophageal atresia.

Question 27

How common are renal anomalies in infants with oesophageal atresia?

Answer 27

Renal anomalies are present in about 20% of cases

Question 28

What types of limb deformities are associated with VACTERL syndrome?

Answer 28

Limb deformities often present as radial dysplasia.

Question 29

What is the overall incidence of associated anomalies in oesophageal atresia?

Answer 29

Approximately 50% of infants with oesophageal atresia have associated anomalies.

Question 30

What is the CHARGE association, and how common is it in oesophageal atresia cases?

Answer 30

The CHARGE association occurs in about 6% of oesophageal atresia cases and includes the following anomalies: • C: Coloboma • H: Heart defects • A: Choanal atresia • R: Retardation of development • G: Genital hypoplasia • E: Ear deformities

Question 31

What serious complication can arise from delayed detection and management of oesophageal atresia?

Answer 31

Aspiration pneumonitis, which can be fatal.

Question 32

How should secretions be managed in an infant with oesophageal atresia?

Answer 32

Suction the proximal oesophageal pouch with an 8F nasal tube every 15 minutes or continuously via a double-lumen Replogle tube.

Question 33

Should infants with oesophageal atresia be fed orally?

Answer 33

No, they should be kept nil per mouth to prevent aspiration.

Question 34

What is the optimal positioning to prevent aspiration in oesophageal atresia?

Answer 34

Position the infant laterally on the right side with the head elevated at 30°.

Question 35

How can hypoxia be prevented in these infants?

Answer 35

Early provision of oxygen and administration of broad-spectrum intravenous antibiotics (e.g., penicillin/ampicillin and gentamicin) to manage aspiration.

Question 36

Why should pre-operative endotracheal intubation be avoided if a tracheo-oesophageal fistula (TOF) is present?

Answer 36

Because air may preferentially enter the low-resistance TOF, causing stomach distension and impairing diaphragmatic breathing.

Question 37

What should be done if intubation is unavoidable in the presence of a TOF?

Answer 37

Perform deep tracheal intubation with the tip close to the carina, hoping the TOF is situated well above the carina.

Question 38

How is hypoglycaemia prevented in infants with oesophageal atresia?

Answer 38

Administer intravenous 10% dextrose-containing maintenance fluids.

Question 39

How should body temperature be maintained in these infants?

Answer 39

Maintain warmth using an incubator or kangaroo care.

Question 40

What is the priority regarding the transfer of an infant with oesophageal atresia?

Answer 40

Urgently transfer to a tertiary centre with a neonatal ICU and paediatric surgical expertise, along with any X-rays or blood results.

Question 41

What is the primary goal of pre-operative care in oesophageal atresia?

Answer 41

To continue supportive care until surgery can be performed safely.

Question 42

What important investigation is performed pre-operatively in oesophageal atresia?

Answer 42

A pre-operative cardiac echogram to assess for cardiac anomalies.

Question 43

How is oesophageal atresia typically repaired?

Answer 43

Through a right lateral thoracotomy with division and closure of the tracheo-oesophageal fistula (TOF) and end-to-end anastomosis of the oesophageal ends.

Question 44

What surgical approach is taken if there is a long gap between oesophageal ends?

Answer 44

A feeding gastrostomy is performed with delayed oesophageal anastomosis after 3–6 weeks. If this fails, an oesophagostomy is created, and oesophageal replacement surgery is planned when the child is older.

Question 45

What are the options for oesophageal replacement if delayed repair fails?

Answer 45

1. Colonic interposition graft on a vascular pedicle 2. Gastric tube 3. Gastric pull-up 4. Jejunal interposition graft

Question 46

What complication can arise from delayed repair of oesophageal atresia?

Answer 46

Lung damage from chronic aspiration can occur.

Question 47

When can feeds be gradually increased post-operatively?

Answer 47

Once a contrast meal confirms oesophageal patency, with no stricture or leak.

Question 48

What investigations are necessary for all babies post-operatively to assess for VACTERL anomalies?

Answer 48

1. Echocardiogram (may be done pre-operatively) 2. Kidney, ureter, and bladder (KUB) ultrasound 3. Spine MRI

Question 49

How long is the typical hospital stay for uncomplicated cases of isolated oesophageal atresia (OA) and tracheo-oesophageal fistula (TOF)?

Answer 49

Approximately 2 weeks.

Question 50

How long might infants with “long gap” oesophageal atresia require hospitalization?

Answer 50

3–4 months

Question 51

Why is long-term follow-up important in oesophageal atresia patients?

Answer 51

To manage any sequelae and associated VACTERL anomalies.

Question 52

What gastrointestinal complication is common in long-term follow-up, especially in cases with a “long gap”?

Answer 52

Gastro-oesophageal reflux disease (GERD), particularly if the distal oesophagus was placed under tension during anastomosis.

Question 53

What complication can occur at the anastomotic site, and how is it managed?

Answer 53

Anastomotic strictures can develop and typically require dilation.

Question 54

What respiratory complication can present with a barking cough and frequent chest infections?

Answer 54

Tracheomalacia, which usually improves over time but may require surgical treatment in some patients.

Question 55

What is the expected survival rate for oesophageal atresia with good care?

Answer 55

Survival rates exceed 90% with good care.

Question 56

What patient factors increase morbidity and mortality in oesophageal atresia and tracheo-oesophageal fistula (TOF)?

Answer 56

1. Premature birth 2. Other congenital abnormalities, especially cardiac disease (e.g., VSD, PDA, ToF) 3. Tracheomalacia 4. Genetic conditions, such as Trisomy 21 (Down syndrome) and Trisomy 18 (Edward syndrome) 5. Anatomic variations, including the length of the atretic oesophageal segments and the configuration of the TOF

Question 57

What are the most common cardiac anomalies associated with increased mortality in oesophageal atresia?

Answer 57

1. Ventricular septal defect (VSD) 2. Patent ductus arteriosus (PDA) 3. Tetralogy of Fallot (ToF)

Question 58

How does tracheomalacia affect morbidity and mortality in oesophageal atresia?

Answer 58

Tracheomalacia can lead to airway instability and recurrent respiratory complications, increasing morbidity and mortality.

Question 59

Why do anatomic variations in oesophageal atresia increase morbidity and mortality?

Answer 59

Variations in the length of atretic oesophageal segments and TOF configuration can make surgical repair more challenging and increase the risk of post-operative complications.

Question 60

Why is delivery outside a tertiary unit a risk factor for increased morbidity and mortality in oesophageal atresia?

Answer 60

Because the womb is the safest transport incubator for a baby with a congenital anomaly, and tertiary units are better equipped for immediate specialized care.

Question 61

How can antenatal detection of oesophageal atresia improve outcomes?

Answer 61

Antenatal ultrasound detection allows for planned delivery in a tertiary unit, ensuring immediate access to specialized care.

Question 62

What should clinicians be alert to if no antenatal diagnosis of oesophageal atresia was made?

Answer 62

A high index of suspicion is needed if the infant presents with: 1. Excessive drooling 2. Coughing or respiratory distress after feeds 3. Milk vomits

Question 63

How should oesophageal patency be checked in a suspected case of oesophageal atresia?

Answer 63

By attempting to pass a large-bore nasogastric tube to check for obstruction.