Tracheo-oesophageal fistula

Classification according to ICD-10
Q39.2	Congenital tracheal esophageal fistula without atresia
Q39.1	Esophageal atresia with tracheal esophageal fistula
J95.0	Tracheostomy dysfunction - Tracheo-esophageal fistula after tracheotomy
ICD-10 online (WHO version 2019)

A tracheo-esophageal fistula , English tracheo-esophageal fistula , ( TEF ) , is a relatively common congenital or acquired abnormal fistula connection between the trachea and esophagus .

Cause and spread

Fistulas can arise as a complication after surgery or as a result of the infiltrative growth of malignant tumors. These are rare (around 0.5% after tracheostomy , around 4.5% in malignant esophageal tumors, and 0.3% in primary lung tumors).

These acquired forms, which can be very different in localization and symptoms depending on the cause, will not be discussed in the following. The vast majority of them are congenital fistulas with malformations of the esophagus and trachea. They occur in 1 in 2,000–4,000 live births ; additional malformations are present in 17–70% of patients.

Isolated fistulas occur in 1 in 80,000 births.

Classification

Depending on the anatomical course of this fistula connection, a distinction can be made: (see also classification of esophageal atresia according to Vogt )

Esophagotracheal fistula from the upper blind sac of an esophageal atresia into the trachea with constant ingestion of saliva and immediate aspiration when drinking
H-fistula between the esophagus and trachea with normal passage through the esophagus, congenital isolated fistula , and - depending on the size of the fistula - more or less frequent aspiration when drinking
Tracheo-oesophageal fistula from the tracheal system into the lower blind sac of an oesophageal atresia with reflux of gastric contents and severe symptoms.

Ro-contrast imaging of a tracheo-oesophageal fistula in a newborn

Clinical manifestations

If there is esophageal atresia, this determines the clinical symptoms.

If, on the other hand, it is an isolated fistula , the combination of coughing attacks , chronically recurrent aspiration pneumonia and meteorism is considered to be groundbreaking.

Other clinical signs of a fistula include:

Refusal to drink
Turn blue when trying to drink
Recurrent aspirations
Upper lobe atelectasis

diagnosis

H-fistulas run from the trachea predominantly at the level of C6 to C2 and are significantly higher than the fistulas in an esophageal atresia and run from the anterior wall of the esophagus obliquely cranial to the trachea.

Evidence is provided by x-rays during fluoroscopy using a contrast agent examination ( esophageal swallow ) through a probe in the esophagus.

therapy

Treatment consists of surgical ligation or resection of the fistula and, if necessary, treatment of the esophageal atresia.

Occurrence in syndromes

Such fistulas are found in the following syndromes :

Feingold syndrome (synonyms: Brunner-Winter syndrome; FGLDS; FS; finger anomalies - short eyelid slits - esophageal or duodenal atresia; MMT; MODED syndrome; microcephaly - intellectual disability - tracheo-esophageal fistula; microcephaly - oculo-digito-oesophago- duodenal syndrome; microcephaly-finger abnormalities-normal intelligence syndrome; ODED syndrome; okulo-digito-esophago-duodenal syndrome )
VACTERL association
Beta blocker embryopathy

history

The first description of a TEF in an esophageal atresia was made in 1697 by Thomas Gibson . The first successful operation of an esophageal atresia with fistula was carried out in 1943 by the surgeon C. Haight .

literature

L. Spitz, AG Coran (Editor): Rob & Smith's Operative Surgery. Pediatric Surgery, 5th Edition 1995, Chapman & Hall, ISBN 0-412-59110-3

Individual evidence

↑ Emedicine
^ ^A ^b ^c M. Bettex, N. Genton, M. Stockmann (Ed.): Pediatric Surgery. Diagnostics, indication, therapy, prognosis. 2nd edition, Thieme
↑ ^a ^b W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, ISBN 3-540-60224-0 .
↑ Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
^ Feingold syndrome. In: Orphanet (Rare Disease Database).
^ T. Gibson T: The anatomy of human bodies epitomized. Awnsham & Churchill, 1697, London
↑ C. Haight: Congenital atresia of the esophagus with tracheoesophageal fistula: Extrapleural ligation of fistula and end to end anastomosis of esophageal segments. In: Surgery, Gynecolology & Obstetrics , Vol. 76, pp. 672-688.

[emed-1] Emedicine

[Bettex-2] A ^b ^c M. Bettex, N. Genton, M. Stockmann (Ed.): Pediatric Surgery. Diagnostics, indication, therapy, prognosis. 2nd edition, Thieme

[Schuster-3] W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, ISBN 3-540-60224-0 .

[4] Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .

[5] Feingold syndrome. In: Orphanet (Rare Disease Database).

[6] T. Gibson T: The anatomy of human bodies epitomized. Awnsham & Churchill, 1697, London

[7] C. Haight: Congenital atresia of the esophagus with tracheoesophageal fistula: Extrapleural ligation of fistula and end to end anastomosis of esophageal segments. In: Surgery, Gynecolology & Obstetrics , Vol. 76, pp. 672-688.