Tracheoesophageal fistula

Learning objectives

Describe tracheoesophageal fistula and esophageal atresia, and their classification
Define the associated congenital anomalies of tracheoesophageal fistulas
Anesthetic management of pediatric patients with a tracheoesophageal fistula and/or esophageal atresia

A tracheoesophageal fistula (TEF) is one of the most common congenital anomalies and is an abnormal connection (fistula) between the esophagus and trachea
TEF commonly occurs with esophageal atresia (EA), a related congenital malformation with a similar presentation to TEF
EA is an abnormal connection between the esophagus and stomach, the esophagus ends in a blind-ended pouch rather than connecting normally to the stomach, can occur with or without the presence of a fistula

Classification

Type A	No TEF, only EA → isolated EA The esophagus is divided into two parts with both portions ending in blind pouches	8% of all cases
Type B	Proximal TEF and distal EA The lower portion of the esophagus ends in a blind pouch and the upper portion is connected to the trachea by a TEF	2% of all cases
Type C	Proximal EA and distal TEF The upper portion of the esophagus ends in a blind pouch and the lower portion is connected to the trachea by a TEF	Most common, 85% of all cases
Type D	Both proximal and distal TEF TEF connects both the upper and lower portions of the esophagus and trachea	Rarest form, <1% of all cases
Type E (H-type)	Isolated TEF The esophagus connects to the stomach normally and is fully intact, a TEF connects the esophagus and trachea	4% of all cases

50% of babies with TEF/EA also have associated congenital anomalies

Prematurity (30%)
Congenital heart disease (30%): Ventricular septal defect, tetralogy of Fallot, patent ductus arteriosus, atrial septal defect, right-sided aortic arch
Gastrointestinal anomalies (14%): Diaphragmatic hernia, duodenal atresia, pyloric stenosis, imperforate anus, malrotation, omphalocele
Genitourinary anomalies (14%): Renal agenesis, hypospadias, horseshoe/polycystic kidney, ureteric/urethral abnormalities
Musculoskeletal anomalies (10%): Radial limb abnormalities, polydactyly, lower limb defects, hemivertebrae, rib defects, scoliosis
VACTERL syndromes (10%)
Respiratory anomalies (6%): Tracheobronchomalacia, pulmonary hypoplasia, tracheal agenesis/stenosis, tracheal upper pouch
Chromosome abnormalities (4%): Trisomy 13, 18, or Down syndrome

Surgical correction with resection of any fistula and anastomosis of any discontinuous segments
Usually repaired within 24 hours of birth to minimize the risk and complications of aspiration
Complications after surgery
- Anastomotic leaks
- Esophageal strictures (abnormal tightening)
- Damage to the laryngeal nerve
- Recurrence of the fistula
- Gastroesophageal reflux disease
- Dysphagia
- Asthma-like symptoms: Persistent coughing or wheezing
- Recurrent chest infections
- Tracheomalacia