1. Tracheoesophageal Fistula
　　　　　　R2趙怡芳/VS范守仁

2. Epidemiology Incidence: 1/4000
30-50%: congenital anomalies (35% cardiac; 30% musculoskeletal)
20-30%: premature, BW<2000g
VACTERAL and VATER syndrome:
Vertebral anomalies:
Anal: imperforate anus
Cardiac: VSD, PDA, TOF, coarction of aorta, ASD
Trachea: TEF
Esophageal: EA
Renal: renal agenesis,ureteral abnormalities, hypospadias
Limb: polydactyly, wrist/knee anomalies

3. Embryology
Esophagus and tracheal originate from the median ventral diverticulum of the forgut, separated by esophagotracheal septum
TEF: esophageal and tracheal failure to separate during divison of the endoderm
Esophageal atresia: tracheal structure assume most of the endoderm

4. Types of TEF

5. Diagnosis
During pregnancy: maternal polyhydramnios and premature labor
Newborn:
s/s: cough, chocking, cynosis, excessive salivation, drooling,
OG tube: inability to pass down the esophagus into the stomach
X-ray:
catheter curled up in the upper pouch of the esophagus
air/gas bubbles in the stomach and intestine
ultrasonography: diagnosis of any associated cardiac, musculoskeletal, GI or GU abnormalities

7. General management Primary repair of TEF is urgent
Associated anomalies: CV, GU, musculoskeletal
Protect the lungs from aspiration pneumonia
Avoidance of feeding
Positioning of the infant slightly head up to minimize regurgitation of gastric contents through fistula
Intermittent suction
Antibiotic therapy and physiotherapy in babies with contaminated lung

8. Surgical management One stage repair: optimal surgical management
Fistula is ligated, proximal and distal ends of the esophagus are anastomosed
Right posterolateral extrapleural thoracotomy
Left thoracotomy if the p’t has right aortic arch
Gastrostomy:
High risk infants unable to withstand a thoracotomy
Decompress the stomach and prevent regurgitation via fistula into the lungs
Local anesthesia:
general anesthesia: protect the lungs from aspiration during surgical manipulation

9. Surgical Managment Definitive repair
24-72 hours later, when infant can withstand both surgery and anesthesia
Method: right thoracotomy using a posterolateral extrapleural approach
Fistula is ligated, esophageal segments are anastomosed
If distal esophagus is too short
Fistula is ligated
exteriorization of the upper pouch through an esophagostomy is performed
wait till weight of 9kg or 1 y/o to perform anastomosis

10. Anesthetic Management
Operating room set up:
“keep warm”: warm room, blanket, overhead warmer
Release NE: vasoconstriction, increases metabolism, change degree of shunting
Affect anesthetic agents: likely overdosage, postop hypoventilation, apnea
Coagulopathy, metabolic acidosis
Standard monitoring: EKG, pulse oximetry, end tidal CO2, BP monitoring (A line in high risk infants), U/O

11. Anesthetic Management
Induction: “establish airway without pulmonary aspiration or gastric distension”
Suction, pre-oxygenation
Maintain spontaneous ventilation:
Avoid positive pressure ventilation:
Insufflation of the stomach via the fistula or loss of ventilation through the gastrostomy
Gastric distention: compromise ventilation, aspiration

12. Anesthetic Management Intubation/Airway
Awake intubation
Safe
Appropriate positiong of ETT w/o positive pressure ventilation
Difficult and traumatic in vigorous infants
Inhalation/IV anesthetic +/- muscle relaxant:
Aware of positive pressure ventilation
Avoid excessive insufflation of the stomach via fistula
Maintain spontaneous ventilation:
With assistance ventilation until fistula is ligated
Keep airway pressure low (10-15 cmH20)

13. Anesthetic Management: Intubation/Airway
ETT position
Below the fistula and above the carina
Right mainstem intubation, then withdraw
Proximal to carina, bevel facing anteriorly so that posterior wall can occlude the fistula
Confirmation
Fiberoptic bronchoscopy
Gastrostomy to water seal

14. Anesthetic Management
Occlusion of fistula:
Fogarty catheter via bronchoscope or gastrostomy
Intraop problems:
One lung ventilation: hypoxia, CO2 retension
ETT obstruction: blood clot, secretion,
kinking of trachea
Vagal response: tracheal manipulation, lead to bradycardia

15. Anesthetic Management: Postoperative problems
Post op extubation is desired, better suture line healing
1) degree of pulmonary dysfunction
2) presence of associated anomalies
Post op ventilation
Conditions:
Defective tracheal wall at the site of fistula
Contaminated lung
Problems associated with prematurity or associated anomalies
ETT positioned >1cm away from site of fistula repair
Distance btw lip and site of esophageal repair measured, avoid suction too deep

16. References
Smith’s Anesthesia for Infants and Children, 6th edition, 1996 Mosby
Clinical Cases in Anesthesia, 3rd edition, 2005 Elsevier
Pediatric Anesthsia:The Requisites in Anesthesiology, 2004 Mosby Elsevier
Yao & Artusio’s Anesthesiology; Problem Oriented Patient Management, 5th edition, 2003 Lippincott

17. Questions What are preoperative concerns in a patient with TEF?
How will you induce and maintain general anesthesia?
During ligation of the fistula, the infant’s oxygen saturation decreases to 86%, what will you do?
Will you extubate at the end of the surgery? Why or why not?