Prevalence: approximately 1/3,000 to 1/4,000 live births, without ethnic dominance but with a small male predominance  (3/2.5). Congenital malformation characterized by an interruption of the esophagus between its distal portion (which is atretic) and its proximal portion which is dilated and positioned between the 2nd and 4th thoracic vertebrae. A tracheo-esophageal fistula is a variant of esophageal atresia characterized by an abnormal communication between the trachea and esophagus. The fistula can be located at any level between the cricoid cartilage and the carina. There is one form of tracheo-esophageal fistula not associated with esophageal atresia: it is called the H-type fistula. 

There are several classifications of esophageal atresia and  tracheobronchial fistulas. The most commonly used describes 5 types:

-         type I (or A): 'pure' esophageal atresia , no communication with the trachea (6 - 8 % of cases) 

-         type II (or B): esophageal atresia with a fistula between the proximal end of the esophagus and the trachea, the distal end of the esophagus being blind (1 - 3 % of cases) 

-         type III (C): esophageal atresia with a fistula between the distal end and the trachea, the proximal part of the esophagus being blind: it is the usual form (84 % of cases); the fistula typically connects posteriorly to the trachea , usually in its distal portion (43 %) or 1-2 cm above the carena (33 %) but it can sometimes be at the level of the carina (17 %) or of a main bronchus (2 %);

-         type IV (or D): a double tracheal fistula with esophageal atresia (proximal end and distal end) (3 % of cases) 

-         type V (or type E, or H-type fistula): fistula between the trachea and oesophagus but the latter is not interrupted although it is often stenotic (2,5 - 4 % of cases).

Some authors add a type VI (or F) type in which there is only a partial esophageal stenosis, a rare form that may not be discovered until adulthood.

The frequency of the malformation slightly increases with maternal age. Diabetes, alcohol, a maternal phenylketonuria and methimazole intake are possible teratogenic factors. Genetic factors do not appear to play an important role in the development of the malformation except for Edward syndrome (trisomy 18)  and some well-identified syndromes:

-        Feingold syndrome (or oculo-digito-tracheo-duodenal syndrome) [MIM 164 280]. Autosomal dominant transmission of mutations of the MYCN or miR-17-92 genes. Combination in variable proportions  of microcephaly, malformations of the limbs, duodenal, and esophageal atresia and more or less marked mental retardation

-        the  AEG or Rogers syndrome [MIM 206 900] (acronym for "Anophthalmia-Esophageal-Genital", i.e. "anophthalmia esophageal atresia, genital malformations"); mutations in the SOX2 gene

-        CHARGE syndrome (acronym for 'Coloboma Heart defect, choanal Atresia, Retarded growth and development, Genital hypoplasia, Ear anomalies/deafness '[MIM 214 800]; mutations on the CHD7 gene in 70% of cases

-         Fanconi anemia [MIM 227 650, 300 514, 227 645, 605 724, 603 467, 602 996]: esophageal atresia is present in approximately 15 % of the cases

-        the Guion Almeida type mandibulofacial dysostosis [MIM 610 536] which combines acrodysostosis, microcephaly, facial dysmorphism and intellectual retardation; mutations in the EFTUD2 gene

The most likely cause of malformation is an incomplete separation of the anterior intestine sepment occurring between the 4th and the 6th week of intrauterine life.

Neonates with esophageal atresia are often premature and LBW (birth weight < 1500 g in 20 to 30 % of cases). In half of the cases, the malformation is isolated (non-syndromic atresia). In the other cases (syndromic atresia), there are various associated abnormalities:

-        isolated cardiac malformations (25 %): VSD, ASD, atrioventricular canal, tetralogy of Fallot, coarctation of the aorta.

-        complex malformations (13 %), in particular the  VACTERL association (Vertebral, Anorectal, Cardiovascular, Tracheoesophageal, Renal, genitourinary and Limbs anomalies) [MIM 192 350]; a rare form of VATER is transmitted either in a recessive or X-linked way and includes  hydrocephalus (VACTER - H) [MIM 276 950, 314 390]

-        persistence of the left superior vena cava.

-        tracheal stenosis below the fistula

The risk of mortality at one month is about 5,1 % in developed countries: it is almost zero in infants born at term and without associated anomaly but increases:

-        if weight is less than 1000 g (x 8.6),.

-        if gestational age is less than 30 weeks

-        in case of a major cardiac defect (x 6)

-        in syndromic cases (x 1.8 in case of VACTERL, and x 3.9 in case of trisomy 21)

-        and, paradoxically, in case of prenatal diagnosis (more often associated with other anomalies)

Anesthetic implications: 

• neonatal surgery

-        malformation assessment includes verifying the side of the aortic arch (if on the right side, a left thoracotomy was classically recommended but this point is now discussed !): echocardiography, anal examination, morphology...

-        neonatal anesthesia: glycemia, temperature, BP, NIRS, pre- and postductal SpO2

-        thoracotomy or thoracoscopy ?

-        in case of respiratory distress: keep in mind that a gastrostomy may worsen  the situation because part of the ventilation might leak) through it

-        induction while keeping  a spontaneous breathing to prevent gastric distension

-        many teams perform a tracheoscopy (flexible or rigid) before surgery while keeping spontaneous breathing to localize the fistula and to exclude the presence of a second fistula, a laryngeal cleft (2,5 %) and/or tracheomalacia (85 % of which 30 % of severe forms: > 80 % of tracheal collapse during expiration) or of a tracheal stenosis; this exam should be followed with rigid esophagoscopy if no fistula is observed during tracheoscopy in order to exclude the diagnosis of an esophageal atresia without fistula (type I) or of an iatrogenic pharyngeal perforation at birth.

-        intubation: careful positioning of the distal end of the tracheal tube in order to occlude the tracheal opening of the fistula; one can catheterize the fistula from the trachea with a Fogarty catheter to facilitate ventilation until its closure. This procedure is very useful in case of fistula at the level of the carina or of severe tracheal stenosis below the fistula (in which case it can be intubated, above the stenosis, with a tube of normal diameter)

-        patient in left lateral decubitus and compression of the upper lung: risk of hypoxemia: constant communication with the surgeon is important

-        azygos vein ligation is no more recommended

-        post-operative: either elective intubation during 24-48 h or extubation at the end of the surgery (paravertebral block or (caudo)-thoracic epidural catheter )

-        in case of too large distance between the two esophageal segments ('long-gap'), the corrective surgery is postponed: gastrotomy tube, sometimes esophagostomy or pharyngostomy, and possibly insertion of traction wires on the esophageal dead-ends (Focker's technique) to favor their growth; sometimes replacement of the esophagus by an ileo-, gastro- or coloplasty.

• post-operative

-        risk of anastomotic leak or early refistulisation (5 %) with mediastinitis

-        anastomotic stenosis (20 %) requiring sessions of esophageal dilation or emergency extraction of blocked food: a 'full' esophagus situation !

-        risk of recurrence (3 %)

-        in case of severe tacheomalacia, aortopexy or, better, posterior trachopexy can be performed

-        gastro-esophageal reflux

-        airway hyperreactivity: tracheomalacia, secondary to GERD

-        rarely: presence of a diverticulum of the tracheal wall at the level of the closed fistula: it can result in difficult ventilation if the distal end of the tracheal tube enters it.

References : 

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An audit of anesthetic management and complications of tracheo-esophageal fistula and esophageal atresia repair. 
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Search for the azygos : a lesson learnt from a case with left superior vena cava, esophageal atresia and tracheo-esophageal fistula. 
Pediatr Surg Int 209 ; 25 : 121-2

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Preserving the azygos vein when repairing esophageal atresia and tracheoesophageal fistula accompanied by interrupted inferior vena cava.
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Tracheoesophagel fistula and associated congenital heart disease: implications for anesthetic management and survival. 
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A&A Practice, in press

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Updated: October 2021