Esophageal Atresia Flashcards
Where is the esophagus and trachea derived from ?
Primitive foregut
Different anatomical variants of EA/TEF are
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
What is EA And TEF
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
The distal esophageal segment communicates with the trachea in TEF at where
Just above the carina
Clinical manifestations
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
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 ?
congenital H-type TEF
Diagnosis of EA can be confirmed by ?
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
Prenatally , how can EA or TEF be suspected ?
ultrasonographic findings of polyhydramnios, proximal dilated upper pouch, and a small or absent gastric bubble is suggestive but not confirmatory sign
Chest X-ray signs in EA with distal TEF?
The presence of gas in the stomach and intestines with a coiled nasogastric tube in the proximal pouch
How to identify a H-type fistula ( diagnostics method
1) contrast-enhanced studies (pull out esophagogram) with fluoroscopic control.
2) Endoscopy and/or bronchoscopy
3) CT) scan
4) Measurement of intragastric oxygen tension
Diagnostics of EA and TEF
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.
Associated anomalies in esophageal atresia
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
The acronym VATER means
vertebral defects, anorectal malformations, tracheoesophageal fistula, renal anomalies, renal dysplasia)
The acronym VACTERL means
vertebral defect, anorectal malformation, cardiac defect, tracheoesophageal fistula, renal anomaly, radial dysplasia, and limb defects)
Which chromosomal abnormalities are associated with EA?
trisomy 18 and 21
Which cardiovascular anomalies are associated with EA
ventricular septal defect, patent ductus arteriosus, or tetralogy of Fallot
Which gastrointestinal anomalies are associated with EA
imperforate anus, duodenal atresia, and malrotation,
Which Urinary tract malformations are associated with EA?
hypospadias, horseshoe kidney, and renal agenesis, can also occur.
Treatment of EA and TEF
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.
Surgery in EA and TEF involves
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
Long gap EA is ?
defined as a gap length exceeding 4 cm, a gap greater than two vertebral bodies, or an upper pouch level above the thoracic inlet.
How do you treat or solve a long Gap EA ?
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.
Treatment of H- type fistula
An open approach
Thoracoscopic approach
Endoscopic closure
What is a replogle tube
A double lumen tube, where one lumen is for drainage of saliva and the other functions as an air vent.
Treatment of Ea and TEF
Preoperative- Gastrostomy decompression if evidence of pneumonia or atelectasis , upper pouch suction, and central venous nutritional support
Operative
