Esophageal Atresia

Question 1

Where is the esophagus and trachea derived from ?

Answer 1

Primitive foregut

Question 2

Different anatomical variants of EA/TEF are

Answer 2

1) EA with a distal TEF
2) Pure EA without a TEF
3) H-type fistula (TEF without EA)
4) EA with proximal and distal TEF
5) EA with proximal TEF

Question 3

What is EA And TEF

Answer 3

EA is a condition where the proximal and distal part of the esophagus do not communicate

TEF is an abnormal communication between the trachea and the esophagus

Question 4

The distal esophageal segment communicates with the trachea in TEF at where

Answer 4

Just above the carina

Question 5

Clinical manifestations

Answer 5

Drooling accompanied by choking , cough, sneezing
The patient maybe become cyanotic as the overflow of fluid from the blind pouch enters the trachea

Repeated attacks of choking, coughing during feeds

Question 6

Any newborn, infant, or child who presents with repeated attacks of choking and coughing during feeding, abdominal distension, and recurrent attacks of chest infection should raise suspicion of ?

Answer 6

congenital H-type TEF

Question 7

Diagnosis of EA can be confirmed by ?

Answer 7

1) Passing a nasogastric tube

Failure to pass the nasogastric tube and coiling of the tube in the upper pouch confirms the diagnosis of EA.

It is confirmed by plain chest and abdominal X-ray

Question 8

Prenatally , how can EA or TEF be suspected ?

Answer 8

ultrasonographic findings of polyhydramnios, proximal dilated upper pouch, and a small or absent gastric bubble is suggestive but not confirmatory sign

Question 9

Chest X-ray signs in EA with distal TEF?

Answer 9

The presence of gas in the stomach and intestines with a coiled nasogastric tube in the proximal pouch

Question 10

How to identify a H-type fistula ( diagnostics method

Answer 10

1) contrast-enhanced studies (pull out esophagogram) with fluoroscopic control.
2) Endoscopy and/or bronchoscopy
3) CT) scan
4) Measurement of intragastric oxygen tension

Question 11

Diagnostics of EA and TEF

Answer 11

Plain chest and abdominal X-ray ( passing a NG tube )

Contrast-enhanced studies with fluoroscopic control are necessary to identify or locate an H-type TEF

Endoscopy and/or bronchoscopy may be performed to locate or rule out H-type TEF.

An echocardiogram is important to establish associated cardiac anomalies and a normal left-sided aortic arch.

Question 12

Associated anomalies in esophageal atresia

Answer 12

1) CHD such as VSD, patent ductus arteriosus, or tetralogy of Fallot, right-sided aortic arch, ASD, Coarctation of aorta
2) Gastrointestinal anomalies, including imperforate anus, duodenal atresia, and malrotation, occur in approximately 15 % of infants, meeker diverticulitum , annular pancreas
3) Musculoskeletal defects like vertebral abnormalities and defects of the ribs and extremities.
4) Genitourinary - Renal agenesis, horseshoe kidney , poly cystic kidney

VATER OR VACTERL

Question 13

The acronym VATER means

Answer 13

vertebral defects, anorectal malformations, tracheoesophageal fistula, renal anomalies, renal dysplasia)

Question 14

The acronym VACTERL means

Answer 14

vertebral defect, anorectal malformation, cardiac defect, tracheoesophageal fistula, renal anomaly, radial dysplasia, and limb defects)

Question 15

Which chromosomal abnormalities are associated with EA?

Answer 15

trisomy 18 and 21

Question 16

Which cardiovascular anomalies are associated with EA

Answer 16

ventricular septal defect, patent ductus arteriosus, or tetralogy of Fallot

Question 17

Which gastrointestinal anomalies are associated with EA

Answer 17

imperforate anus, duodenal atresia, and malrotation,

Question 18

Which Urinary tract malformations are associated with EA?

Answer 18

hypospadias, horseshoe kidney, and renal agenesis, can also occur.

Question 19

Treatment of EA and TEF

Answer 19

Gastrostomy decompression if evidence of pneumonia or atelectasis , upper pouch suction, and central venous nutritional support

Surgery ( primary reconstruction)- joining the two ends of the esophagus

• This is usually done through a right thoracotomy and via an extrapleural approach.

Surgery involves division of the fistula after ligating its tracheal end, stretching the two ends of the esophagus and primary single layer end-to-end anastomosis.

Question 20

Surgery in EA and TEF involves

Answer 20

Surgery involves division of the fistula after ligating its tracheal end, stretching the two ends of the esophagus and primary single layer end-to-end anastomosis.

The anastomosis is done over a replogle tube which is passed into the stomach and can be used to decompress the stomach or for early postoperative feeding.

• An extrapleural chest tube is left after surgery to allow for drainage of secretions if a leak occurs at the surgical site

Question 21

Long gap EA is ?

Answer 21

defined as a gap length exceeding 4 cm, a gap greater than two vertebral bodies, or an upper pouch level above the thoracic inlet.

Question 22

How do you treat or solve a long Gap EA ?

Answer 22

1) delayed repair (waiting for the esophageal ends to grow closer) or
2) esophageal lengthening procedures such as the Kimura, Livaditis, Scharli, or Foker techniques.

Question 23

Treatment of H- type fistula

Answer 23

An open approach
Thoracoscopic approach
Endoscopic closure

Question 24

What is a replogle tube

Answer 24

A double lumen tube, where one lumen is for drainage of saliva and the other functions as an air vent.

Question 25

Treatment of Ea and TEF

Answer 25

Preoperative- Gastrostomy decompression if evidence of pneumonia or atelectasis , upper pouch suction, and central venous nutritional support

Operative