Tracheo-Oesophageal Fistula - History
Its incidence is about 1/4000. It was first described in 1670. Surgical correction was started only in the 1920s. The first survivors were reported in 1939. The extrapleural approach was reported in 1941.
Tracheo-Oesophageal Fistula - Embryology
- It occurs between the 21st and 34th days of fetal development.
- From the Ventral diverticulum of foregut, masses of endodermal cells divide foregut into trachea and oesophagus. It begins at carina and its imperfect division results in TOF.
- Explanation of Esophageal Atresia is less clear.
- Growth of trachea in caudal direction is so rapid that dorsal wall of oesophagus becomes incorporated into trachea.
- Other etiologies: "Vascular deficiency" Genetic basis.
Tracheo-Oesophageal Fistula - Classification
Gross divided this in to five groups:
- A: Esophageal atresia without fistula
- B: Atresia with upper segment communication
- C: Atresia with lower segment communication
- D: Atresia with both segments communicating
- E: Fistula with no disruption in continuity
- F: Esophageal stenosis
Presentation
ANTENATAL: Polyhydramnios
POST-NATAL: drooling, respiratory distress, choking, coughing, cyanosis
Associated Anomalies
Overall incidence of associated anomalies is 50-70%
- Cardiac (30%): PDA, VSD, ASD
- Gastrointestinal (12%): Imperforate anus, duodenal atresia, annular pancreas, pyloric stenosis
- VACTERL (25%)
Waterson Risk Groups and Current Survival Figures
Group |
Survival(%) |
Waterson classification |
A |
100 |
Birth Weight > 2500 g, well child |
B1 |
85 |
Birth Weight 1800-2500 g, well child |
B2 |
|
Higher weight with moderate associated anomalies |
C1 |
65 |
Birth Weight < 1800 g |
C2 |
|
Birth Weight < 1800 g, severe anomalies |
Tracheo-Oesophageal Fistula - Diagnosis
- Failure to pass NG tube
- Radiographic: Plain, Thin Barium Contrast
- Bronchoscopy
Pre-operative Management
Oral feeds should be discontinued. Baby should be kept in a semi-upright position to minimize regurgitation. The proximal segment should be suctioned continuously to prevent aspiration. The severity of pulmonary disease should be evaluated. Hypothermia should be avoided.
General Management
- Antibiotics
- Sump catheter in upper pouch
- Upright positioning
- Screening for any congenital anomalies
- Correction of fluid electrolyte status
- Gastrostomy, uncommon, usually in isolated EA
Surgical Management
Aim for one-stage repair
Problems with Tracheo-esophageal Fistula
General:
- Management of neonate
- Possible prematurity and low birth weight
Specific:
- Aspiration
- Chemical pneumonitis, pneumonia
- Gastric distention with diaphragmatic splinting
Intra-operative Problems
Lung retraction
Secretions and blood clots
Kinking of trachea
Post-operative Problems
- Tracheomalacia may result in life threatening cyanotic and apneic spells.
- Tracheostomy may be necessary to maintain patent airway without ETT.
- Leak
- Stricture
- Recurrent fistula
- Problems with dysphagia and recurrent respiratory infections
- Esophageal strictures might require dilatations
- Abnormalities in esophageal motility leading to repeated aspirations.