TRACHEO-ESOPHAGEAL FISTULA

ESOPHAGEAL ATRESIA

Three common anatomic variants:

1. Interruption of the esophagus in which the upper portion ends in a blind pouch and the lower portion connects to the trachea by a small fistular opening (3-5mm in diameter).
• Occurs in 85% of cases.
• No effective fetal swallowing through the esophagus.
• Fluid may reach the stomach via the fistula connection to the lung.
2. Complete interruption between the upper and lower esophagus with no connection to the trachea.
• Occurs in 5-8% of cases.
• Polyhydramnios.
• Absent stomach.
3. Relatively normal esophagus connecting to the trachea by a H-type fistular tract.
• Amniotic fluid normal.
• Stomach normal.

I – Esophageal atresia without a fistula.

II – Esophageal atresia with a fistula between the proximal pouch and trachea.

III – Esophageal atresia with a fistula from the trachea or main bronchus to the distal esophageal segment (most common type).

IV –Esophageal atresia with both proximal and distal fistulae.

V – H-type tracheoesophageal fistula without atresia (not seen in utero and usually presents with feeding difficulties in infancy).

ULTRASOUND

Link to Ultrasound

ASSOCIATED ANOMALIES

• Cardiac (especially VSD) in 37% (1).
• Other gastrointestinal anomalies (especially imperforate anus) in 21% (1).
• VATER / VACTERL association.
• Trisomy 18.
• Esophageal atresia may be associated with duodenal atresia (7% in one surgical series) (2).

The combination of both conditions are associated with several other conditions:

1. CHARGE association.

2. VACTERL association with duodenal atresia (3).

3. Trisomy 21.

4. Feingold syndrome (oculodigitoesophago-duodenal syndrome) (4):

- Microcephaly, tracheo-esophageal fistula and mesobrachyphalangy.

5. MODED (5): Microcephaly-oculo-digito-esophageal-duodenal syndrome.

REFERENCES

1. Grosfield JL, Ballantine TVN. Esophageal atresia and tracheoesophageal fistula: Effect of delayed thoracotomy on survival. Surgery 19878;84:394.
2. Andrassy RJ, Mahour GH. Gastrointestinal anomalies associated with esophageal atresia or tracheoesophageal fistula. Arch Surg 1979;114(10):1125-1128
3. Muraji T, Mahour GH. Surgical problems in patients with VATER-associated anomalies. J Pediatr Surg 1984;19(5):550-554.
4. Courtens W, Levi S, Verbelen F et.al. Feingold syndrome: report of a new family and review. Am J Med Genet 1997;73(1):55-60.
5. Frydman M, Katz M, Cabot SG et.al. MODED: microcephaly-oculo-digito-esophageal-duodenal syndrome. Am J Med Genet 1997;71(3):251-257.