Oesophageal Atresia and Tracheoesophageal Fistula

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Last updated: April 6, 2026Bookmark

Oesophageal atresia and tracheoesophageal fistula are congenital anomalies, occurring during the 4th week of gestation due to failure of foregut separation into trachea and oesophagus.

An oesophageal atresia is a blind-ending oesophagus, while a tracheoesophageal fistula is an abnormal connection between the trachea and the oesophagus. They can be part of other midline congenital anomalies, such as the VACTERL association and CHARGE syndrome.

Classification

Type	Description
Type A (8%)	Oesophageal atresia only
Type B (1%)	Oesophageal atresia and proximal tracheoesophageal fistula
Type C (84%)	Oesophageal atresia and distal tracheoesophageal fistula
Type D (3%)	Oesophageal atresia, and proximal and distal tracheoesophageal fistula
Type E (4%)	Tracheoesophageal fistula only (H-type)

Causes
- Genetic associations: SHH, SOX2, CHD7, MYCN, FANCB
Associated conditions
- VACTERL association
- CHARGE syndrome
Signs and symptoms
- Respiratory distress
  - Shortly after birth
  - Worsens with feeding
- Regurgitation
- Excessive salivation
- Drooling due to proximal atresia
- Choking with feeding when there is a fistula (types B and D)
- Gastric distension and gastric perforation due to distal tracheoesophageal fistula (types C and D)
  - Especially with mechanical ventilation
- Signs of aspiration pneumonia
  - Fever
  - Tachypnea
  - Adventitious lung sounds
- Failure to pass a nasogastric tube
- Mother has a history of polyhydramnios with proximal atresia
- A physical examination of the anus and genitalia is required to screen for associated anomalies
Differentials
- Oesophageal strictures or webs
- Laryngotracheoesophageal cleft
- Oesophageal duplication
- Tracheal agenesis
- Severe GERD
Investigations
- Chest or Abdominal X-ray: This is the best initial test
  - Blind upper pouch (92%, the nasogastric tube is coiled before reaching the stomach
  - Absent gastric bubble
  - Air in the stomach suggests a distal tracheoesophageal fistula
- Upper gastrointestinal contrast studies to diagnose type E (H-type) fistulas
- Echocardiogram to screen for concurrent heart defects, since it is associated with the VACTERL sequence
- Renal function tests
- Renal ultrasound
- Spine and limb X-rays
Treatment
- Airway protection
  - Intubation if indicated
  - Continuous suction of the upper pouch
- Nil per os
- Intravenous fluid
- Intravenous antibiotics
- Intravenous nutrition or gastrostomy
  - Consider total parenteral nutrition
- Surgical repair
  - Staged repair for low birth weight (< 1500 g): fistula ligation → oesophageal atresia repair
  - Fistula ligation or primary esophageal anastomosis
  - Foker technique (traction lengthening) and esophagela replacement for a large gap
- NICU care
- Oesophagogram at 5 – 7 days
  - Feeds can be started if there are no leaks
Complications
- Anastomotic leak
- Recurrent fistula
- Recurrent laryngeal nerve injury
- Oesophageal stricture
- GERD
- Tracheomalacia
- Dysphagia
- Recurrent respiratory infections
- Barrett’s oesophagus and oesophageal cancer

Reference Intervals ›

Biochemistry

ACTH	P: <80 ng/L
ALT	P: 5–35 U/L
Albumin	P: 35–50 g/L
Aldosterone	P: 100–500 pmol/L
Alk. phosphatase	P: 30–130 U/L
α-Amylase	P: 0–180 IU/dL
α-Fetoprotein	S: <10 kU/L
Angiotensin II	P: 5–35 pmol/L
ADH	P: 0.9–4.6 pmol/L
AST	P: 5–35 U/L
Bicarbonate	P: 24–30 mmol/L
Bilirubin	P: 3–17 μmol/L
BNP	P: <50 ng/L
CRP	P: <10 mg/L
Calcitonin	P: <0.1 mcg/L
Calcium (ionized)	P: 1.0–1.25 mmol/L
Calcium (total)	P: 2.12–2.60 mmol/L
Chloride	P: 95–105 mmol/L
Cholesterol	P: <5.0 mmol/L
VLDL	P: 0.128–0.645 mmol/L
LDL	P: <2.0 mmol/L
HDL	P: 0.9–1.93 mmol/L
Cortisol AM	P: 450–700 nmol/L
Cortisol Midnight	P: 80–280 nmol/L
CK ♂	P: 25–195 U/L
CK ♀	P: 25–170 U/L
Creatinine	P: 70–100 μmol/L
Ferritin	P: 12–200 mcg/L
Folate	S: 2.1 mcg/L
FSH	P: 2–8 U/L ♂; >25 menopause
GGT ♂	P: 11–51 U/L
GGT ♀	P: 7–33 U/L
Glucose (fasting)	P: 3.5–5.5 mmol/L
Growth hormone	P: <20 mu/L
HbA1C (DCCT)	B: 4–6%
HbA1C (IFCC)	B: 20–42 mmol/mol
Iron ♂	S: 14–31 μmol/L
Iron ♀	S: 11–30 μmol/L
Lactate (venous)	P: 0.6–2.4 mmol/L
Lactate (arterial)	P: 0.6–1.8 mmol/L
LDH	P: 70–250 U/L
LH	P: 3–16 U/L
Magnesium	P: 0.75–1.05 mmol/L
Osmolality	P: 278–305 mosmol/kg
PTH	P: 0.8–8.5 pmol/L
Potassium	P: 3.5–5.3 mmol/L
Prolactin ♂	P: <450 U/L
Prolactin ♀	P: <600 U/L
PSA	P: 0–4 mcg/mL
Protein (total)	P: 60–80 g/L
Red cell folate	B: 0.36–1.44 μmol/L
Renin (erect)	P: 2.8–4.5 pmol/mL/h
Renin (recumbent)	P: 1.1–2.7 pmol/mL/h
Sodium	P: 135–145 mmol/L
TBG	P: 7–17 mg/L
TSH	P: 0.5–4.2 mU/L
T4	P: 70–140 nmol/L
Free T4	P: 9–22 pmol/L
TIBC	S: 54–75 μmol/L
Triglycerides	P: 0.50–2.3 mmol/L
T3	P: 1.2–3.0 nmol/L
Troponin T	P: <0.1 mcg/L
Urate ♂	P: 210–480 μmol/L
Urate ♀	P: 150–390 μmol/L
Urea	P: 2.5–6.7 mmol/L
Vitamin B12	S: 0.13–0.68 nmol/L
Vitamin D	S: 50 nmol/L

Arterial Blood Gases

pH	7.35–7.45
PaCO₂	4.7–6.0 kPa
PaO₂	>10.6 kPa
Base excess	±2 mmol/L

Urine

Cortisol (free)	<280 nmol/24h
Hydroxyindole acetic acid	16–73 μmol/24h
Hydroxymethylmandelic acid	16–48 μmol/24h
Metanephrines	0.03–0.69 μmol/mmol cr.
Osmolality	350–1000 mosmol/kg
17-Oxogenic steroids ♂	28–30 μmol/24h
17-Oxogenic steroids ♀	21–66 μmol/24h
17-Oxosteroids ♂	17–76 μmol/24h
17-Oxosteroids ♀	14–59 μmol/24h
Phosphate (inorganic)	15–50 mmol/24h
Potassium	14–120 mmol/24h
Protein	<150 mg/24h
Protein/creatinine ratio	<3 mg/mmol
Sodium	100–250 mmol/24h

Haematology

WCC	4.0–11.0 ×10⁹/L
RBC ♂	4.5–6.5 ×10¹²/L
RBC ♀	3.9–5.6 ×10¹²/L
Hb ♂	130–180 g/L
Hb ♀	115–160 g/L
PCV ♂	0.4–0.54 L/L
PCV ♀	0.37–0.47 L/L
MCV	76–96 fL
MCH	27–32 pg
MCHC	300–360 g/L
RDW	11.6–14.6%
Neutrophils	2.0–7.5 ×10⁹/L (40–75%)
Lymphocytes	1.0–4.5 ×10⁹/L (20–45%)
Eosinophils	0.04–0.44 ×10⁹/L (1–6%)
Basophils	0–0.10 ×10⁹/L (0–1%)
Monocytes	0.2–0.8 ×10⁹/L (2–10%)
Platelets	150–400 ×10⁹/L
Reticulocytes	0.8–2.0% / 25–100 ×10⁹/L
Prothrombin time	10–14 s
APTT	35–45 s

Paediatric

Pulse Rate (bpm)
Neonate	140–160
Infant <1yr	120–140
1–5 years	110–130
5–12 years	80–120
>12 years	70–100
Respiratory Rate (tachypnoea)
0–2 months	≥60/min
2–12 months	≥50/min
1–5 years	≥40/min
>5 years	≥30/min
Blood Pressure (mmHg)
Term	65/45
1 year	75/50
4 years	85/60
8 years	95/65
10 years	100/70
Weight Formulas
3–12 months	(a + 9)/2 kg
1–6 years	2a + 8 kg
>6 years	(7a − 5)/2 kg
Haemoglobin (g/dL)
Term newborn	13–20
1 month	11–18
2 months	10–15
1–2 years	10–13
>2 years	11–14
MUAC (6 months–5 years)
Obese	>17.5 cm
Normal	13.5–17.4 cm
At risk	12.5–13.4 cm
Moderate malnutrition	11.5–12.4 cm
Severe malnutrition	<11.5 cm
Developmental Milestones
Social smile	1.5 months
Head control	4 months
Sits unsupported	7 months
Crawls	10 months
Stands unsupported	10–12 months
Walks	12–13 months
Talks	18 months
CSF WBC (/mm³)
Term newborn	0–25
>2 weeks	0–5

Calculator ›

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