1. Esophageal Atresia and Tracheoesophageal Fistula Pediatric Surgery Clinic

2. Successive stages in the development of the tracheoesophageal septum during embryologic development. (A) The laryngotracheal diverticulum forms as a ventral outpouching from the caudal part of the primitive pharynx. (B) Longitudinal tracheoesophageal folds begin to fuse toward the midline to eventually form the tracheoesophageal septum. (C) The tracheoesophageal septum has completely formed. ( (D) If the tracheoesophageal septum deviates posteriorly, esophageal atresia with a tracheoesophageal fistula develops

3. Esophageal atresia is a congenital abnormality in which the midportion of the esophagus is absent. Incidence is between 1 in 3,570 and Incidence is between 1 in 3,570 and 1 in 4,500.

4. Anatomic Variations 85% 85% Most common Most common VOGTtype3(b) VOGTtype3(b) GROSS type C GROSS type C

5. Anatomic Variations 6% Atresia alone, Atresia alone, no fistula no fistula Small stomach, Small stomach, gasless abdomen gasless abdomen Usually has a long Usually has a long gap between the gap between the esophagealends esophagealends VOGT types 1 and 2 VOGT types 1 and 2 GROSS type A GROSS type A

6. Anatomic Variations 2% 2% Proximal tracheo- Proximal tracheo- esophageal fistula esophageal fistula No distal fistula No distal fistula Small stomach, Small stomach, gasless abdomen gasless abdomen Often has a long Often has a long gap between the gap between the esophagealends esophagealends VOGT type 3(a) VOGT type 3(a) GROSS type B GROSS type B

7. Anatomic Variations l% l% Proximal and Proximal and distal fistulas distal fistulas ("double fistula") ("double fistula") VOGT type 3(c) VOGT type 3(c) GROSS type D GROSS type D

8. Anatomic Variations 6% 6% No atresia of No atresia of the esophagus the esophagus Congenital Congenital tracheoesophageal tracheoesophageal fistula fistula "H" or "N" fistula "H" or "N" fistula GROSS type E GROSS type E

9. Physiologic effects of distal tracheoesophageal fistula 1. Hyaline membrane disease may necessitate higher ventilator pressures, which encourage air to pass through the distal fistula. 2. A distended abdomen elevates and "splints" the diaphragm. 3 3. Gastric distension may result in gastric rupture and pneumoperitoneum. 4 4. Passage of air through a distal tracheoesophageal fistula diminishes the effective tidal volume.. (B) 1. Aspiration of gastric juices leads to soiling of the lungs and pneumonia 2. Gastroesophageal reflux 3 3. Direction of gastric fluid proximally through distal fistula. 4 4. Overflow of secretions or inadvertent feeding may contribute to aspiration and contamination of the airway..

10. Associated Abnormalities Incidence of Associated Anomalies in Esophageal Atresia. Anomaly Frequency (%) Anomaly Frequency (%) Congenital heart disease 25 Congenital heart disease 25 Urinary tract 22 Urinary tract 22 Orthopaedic (mostly vertebral and radial) 15 Orthopaedic (mostly vertebral and radial) 15 Gastrointestinal (e.g., duodenal Gastrointestinal (e.g., duodenal atresia,imperforate anus) 22 atresia,imperforate anus) 22 Chromosomal (usually trisomy 18 or 21) 7 Chromosomal (usually trisomy 18 or 21) 7 Total with one or more associated 58 Total with one or more associated 58 anomalies anomalies

11. Associated Congenital Anomalies Reported in Patients with Esophageal Atresia System affected Musculoskeletal MusculoskeletalGastrointestinalCardiacGenitourinary. Potential anomalies Hemivertebrae, radial dysplasia or amelia, polydactyly, syndactyly, rib malformations, scoliosis, lower limb defects Imperforate anus, duodenal atresia, malrotation, intestinal malformations, Meckel's diverticulum, annular pancreas Ventricular septal defect, patent ductus arteriosus, tetralogy of Fallot, atrial septal defect, single umbilical artery, right-sided aortic arch Renal agenesis or dysplasia, horseshoe kidney, polycystic kidney, ureteral and urethral malformations, hypospadias

12. DIAGNOSIS OF ESOPHAGEAL ATRESIA Antenatal Diagnosis (maternal polyhydramnios, a small stomach, a distended upper esophageal pouch, or abnormal swallowing) D Diagnostic suspicion is increased when abnormalities known to be associated with esophageal atresia are identified.

13. Fetal MRI This 32 week fetus had esophageal atresia and an absent stomach, resulting in marked polyhydramnios This 32 week fetus had esophageal atresia and an absent stomach, resulting in marked polyhydramnios

14. Clinical Diagnosis Prematurity Any excessively drooling (copious, fine, white, frothy bubbles of mucus in the mouth and, sometimes, the nose).

15. . (A) Diagnosis of esophageal atresia is confirmed when a 10- gauge (French) catheter cannot be passed beyond 10 cm from the gums. (B) A smaller-caliber tube is not used because it may curl up in the upper esophageal segment, giving a false impression of esophageal continuity.. (A) Diagnosis of esophageal atresia is confirmed when a 10- gauge (French) catheter cannot be passed beyond 10 cm from the gums. (B) A smaller-caliber tube is not used because it may curl up in the upper esophageal segment, giving a false impression of esophageal continuity.

16. The chest radiograph A plain radiograph will confirm the tube has not reached the stomach A plain radiograph will confirm the tube has not reached the stomach

17. The Gasless Abdomen Absence of gas in the abdomen suggests that the patient has either atresia without a fistula or atresia with a proximal fistula only Absence of gas in the abdomen suggests that the patient has either atresia without a fistula or atresia with a proximal fistula only

18. Contrast studies should be performed by an experienced pediatric radiologist, or after transfer to the tertiary institution, and with the use of a small amount (0.5 to 1 mL) of water- soluble contrast. Care must be taken to avoid aspiration. should be performed by an experienced pediatric radiologist, or after transfer to the tertiary institution, and with the use of a small amount (0.5 to 1 mL) of water- soluble contrast. Care must be taken to avoid aspiration.

19. Management Measures should be taken to reduce the risk of aspiration(continuous suctioning of the upper pouch, the infant's head should be elevated). Measures should be taken to reduce the risk of aspiration(continuous suctioning of the upper pouch, the infant's head should be elevated). In infants with respiratory failure, endotracheal intubation should be performed. In infants with respiratory failure, endotracheal intubation should be performed. Transfer to a major tertiary pediatric institution is best not delayed. Transfer to a major tertiary pediatric institution is best not delayed.

20. Summary of Preoperative Investigations A plain radiograph A plain radiograph Renal ultrasonography and echocardiography are routine preoperative investigations Renal ultrasonography and echocardiography are routine preoperative investigations Endoscopy or a careful midesophageal contrast study performed in a tertiary center. In some centers, bronchoscopy is performed routinely in all infants with esophageal atresia. Endoscopy or a careful midesophageal contrast study performed in a tertiary center. In some centers, bronchoscopy is performed routinely in all infants with esophageal atresia.

21. Operative Repair of Esophageal Atresia Surgical repair is delayed (1-2days) in infants with low birth weight, pneumonia or other major anomalies. Surgical repair is delayed (1-2days) in infants with low birth weight, pneumonia or other major anomalies.

22. Operative Repair of Esophageal Atresia

24. Respiratory difficulty after feedings in a 3-day- old boy. Barium esophagogram clearly shows an H-shaped fistula between the trachea and the middle segment of the esophagus (arrowhead). Barium is filling the bronchi of the right lower lobe (arrows). Respiratory difficulty after feedings in a 3-day- old boy. Barium esophagogram clearly shows an H-shaped fistula between the trachea and the middle segment of the esophagus (arrowhead). Barium is filling the bronchi of the right lower lobe (arrows). Tracheoesophageal fistula without atresia (type E). without atresia (type E).

25. Tracheoesophageal fistula without atresia (type E). Esophagogram shows a fistula (arrow) arising from the anterior portion of the esophagus (e) and passing cephalad to the posterior portion of the trachea (t). Esophagogram shows a fistula (arrow) arising from the anterior portion of the esophagus (e) and passing cephalad to the posterior portion of the trachea (t).

26. Tracheoesophageal fistula without atresia (type E). Endoscopic diagnosis Endoscopic diagnosis

27. Congenital tracheoesophageal fistula

28. The end