Tracheo-Esophageal Fistula

Question 1

the most common congenital anomaly of the esophagus

Answer 1

Esophageal atresia (EA) is, with a prevalence of 1.7 per 10,000 live births. Of these, >90% have an associated tracheoesophageal fistula (TEF)

Question 2

The most common type of TEF

Answer 2

Type c is the upper esophagus ends in a blind pouch and the TEF is connected to the distal esophagus (type C)

Question 3

Risk factors for TEF

Answer 3

Advanced maternal age, 
European ethnicity, 
Obesity, 
Low socioeconomic status, and 
Tobacco smoking

Question 4

Prognosis of TEF

Answer 4

Survival rates of >90%, owing largely to improved neonatal intensive care, earlier recognition, and appropriate intervention

Question 5

High risk for mortality in TEF

Answer 5

Infants weighing <1,500 g at birth and those with severe associated cardiac anomalies

Question 6

Syndromic association with TEF

Answer 6

Fifty percent of infants with TEF
Most often associated with the vertebral, anorectal, (cardiac), tracheal, esophageal, renal, radial, (limb) (VACTERL) syndrome. Cardiac and vertebral anomalies are seen in 32% and 24%, respectively

Question 7

CHARGE syndrome

Answer 7

coloboma of the eye;
central nervous system anomalies;
heart defects;
atresia of the choanae;
retardation of growth and/or development;
genital and/or urinary defects [hypogonadism];
ear anomalies and/or deafness

Question 8

Other syndrome associated with TEF

Answer 8

Feingold syndrome (N-MYC)

Anophthalmia-esophagealgenital syndrome (SOX2)

Question 9

Most common presentation of TEF

Answer 9

The neonate with EA typically has frothing and bubbling at the mouth and nose after birth as well as episodes of coughing, cyanosis, and respiratory distress

Question 10

Presentation of H type fistula

Answer 10

Later in life with chronic respiratory problems, including refractory bronchospasm and recurrent pneumonias

Question 11

Diagnosis of H type fistula by ?

Answer 11

An esophagogram with contrast medium injected under pressure can demonstrate the defect

Question 12

Current standard approach of TEF

Answer 12

Surgical ligation of the TEF and primary end-to-end anastomosis of the esophagus via rightsided thoracotomy

Question 13

Contraindication for primary closure in case of TEF

Answer 13

In the premature or

Otherwise complicated infant,

If the gap between the atretic ends of the esophagus is >3 to 4 cm (>3 vertebral bodies)

Question 14

Temporary treatment for TEF In case delayed surgery

Answer 14

Fistula ligation and gastrostomy tube placement

options include using gastric, jejunal, or colonic segments interposed as a neoesophagus

Question 15

Complications of TEF

Answer 15

Complications of surgery include anastomotic leak, refistulization, and anastomotic stricture.

Gastroesophageal reflux disease, resulting from intrinsic abnormalities of esophageal function, often combined with delayed gastric emptying, contributes to management challenges in many cases