Esophageal Atresia and Esophageal Atresia and Tracheoesophageal FistulaTracheoesophageal Fistula

Pediatric Surgery Pediatric Surgery ClinicClinic

Successive stages in the development of the Successive stages in the development of the tracheoesophageal septum during tracheoesophageal septum during

embryologic developmentembryologic development. .

(A)(A) The laryngotracheal The laryngotracheal diverticulum forms as a diverticulum forms as a ventral outpouching from the ventral outpouching from the caudal part of the primitive caudal part of the primitive pharynx. pharynx.

(B)(B) Longitudinal Longitudinal tracheoesophageal folds begin tracheoesophageal folds begin to fuse toward the midline to to fuse toward the midline to eventually form the eventually form the tracheoesophageal septum. tracheoesophageal septum.

(C)(C) The tracheoesophageal The tracheoesophageal septum has completely septum has completely formed.formed.

(D)(D) If the tracheoesophageal If the tracheoesophageal septum deviates posteriorly, septum deviates posteriorly, esophageal atresia with a esophageal atresia with a tracheoesophageal fistula tracheoesophageal fistula develops develops

Esophageal atresia is a congenital Esophageal atresia is a congenital abnormality in which the abnormality in which the

midportion of the esophagus is midportion of the esophagus is

absent.absent. Incidence is between 1 in 3,570 and Incidence is between 1 in 3,570 and

1 in 4,500. 1 in 4,500.

Anatomic VariationsAnatomic Variations

85%85%

   Most commonMost common

   VOGTtype3(b)VOGTtype3(b)

   GROSS type CGROSS type C

  

Anatomic VariationsAnatomic Variations

6%6%Atresia alone,Atresia alone,   no fistulano fistula   Small stomach,Small stomach,   gasless abdomengasless abdomen   Usually has a longUsually has a long   gap between thegap between the   esophagealendsesophagealends   VOGT types 1 and 2VOGT types 1 and 2   GROSS type AGROSS type A

Anatomic VariationsAnatomic Variations 2%2%   Proximal tracheo-Proximal tracheo-  

esophageal fistulaesophageal fistula   No distal fistulaNo distal fistula  

Small stomach,Small stomach,   gasless abdomengasless abdomen   Often has a longOften has a long  

gap between thegap between the  

esophagealendsesophagealends   VOGT type 3(a)VOGT type 3(a)   GROSS type BGROSS type B  

Anatomic VariationsAnatomic Variations

l%l% Proximal andProximal and  

distal fistulasdistal fistulas  

("double fistula")("double fistula")   VOGT type 3(c)VOGT type 3(c)   GROSS type DGROSS type D  

Anatomic VariationsAnatomic Variations

6%6% No atresia ofNo atresia of

the esophagusthe esophagus   CongenitalCongenital

tracheoesophagealtracheoesophageal  

fistulafistula

"H" or "N" fistula"H" or "N" fistula   GROSS type EGROSS type E  

Physiologic effects of distal

tracheoesophageal fistula 1. Hyaline membrane disease 1. Hyaline membrane disease

may necessitate higher may necessitate higher ventilator pressures, which ventilator pressures, which encourage air to pass through encourage air to pass through the distal fistula.the distal fistula.

2. A distended abdomen elevates 2. A distended abdomen elevates and "splints" the diaphragm.and "splints" the diaphragm.

3. Gastric distension may result 3. Gastric distension may result in gastric rupture and in gastric rupture and pneumoperitoneum.pneumoperitoneum.

4. Passage of air through a distal 4. Passage of air through a distal tracheoesophageal fistula tracheoesophageal fistula diminishes the effective tidal diminishes the effective tidal volume. volume.

. (B) 1. Aspiration of gastric . (B) 1. Aspiration of gastric juices leads to soiling of the juices leads to soiling of the lungs and pneumonia lungs and pneumonia

2. Gastroesophageal reflux2. Gastroesophageal reflux 3. Direction of gastric fluid 3. Direction of gastric fluid

proximally through distal fistula.proximally through distal fistula. 4. Overflow of secretions or 4. Overflow of secretions or

inadvertent feeding may inadvertent feeding may contribute to aspiration and contribute to aspiration and contamination of the airway. . contamination of the airway. .

Associated AbnormalitiesAssociated Abnormalities

Incidence of Associated Anomalies in Esophageal Atresia.Incidence of Associated Anomalies in Esophageal Atresia. Anomaly Anomaly Frequency Frequency

(%)(%) Congenital heart disease Congenital heart disease 2525 Urinary tract Urinary tract 2222 Orthopaedic (mostly vertebral and radial) Orthopaedic (mostly vertebral and radial) 1515 Gastrointestinal (e.g., duodenalGastrointestinal (e.g., duodenal atresia,imperforate anus) atresia,imperforate anus) 2222

Chromosomal (usually trisomy 18 or 21) Chromosomal (usually trisomy 18 or 21) 77 Total with one or more associated Total with one or more associated 5858 anomaliesanomalies

Associated Congenital Anomalies Reported in Patients with Associated Congenital Anomalies Reported in Patients with Esophageal AtresiaEsophageal Atresia

System affectedSystem affected

MusculoskeletaMusculoskeletall

Gastrointestinal Gastrointestinal

CardiacCardiac

Genitourinary. Genitourinary.

Potential anomalies Potential anomalies

Hemivertebrae, radial dysplasia Hemivertebrae, radial dysplasia or amelia, polydactyly, or amelia, polydactyly, syndactyly, rib malformations, syndactyly, rib malformations, scoliosis, lower limb defectsscoliosis, lower limb defects

Imperforate anus, duodenal Imperforate anus, duodenal atresia, malrotation, intestinal atresia, malrotation, intestinal mmalformations, Meckel's alformations, Meckel's diverticulum, annular pancreasdiverticulum, annular pancreas

Ventricular septal defect, Ventricular septal defect, patent ductus arteriosus, patent ductus arteriosus, tetralogy of Fallot, atrial septal tetralogy of Fallot, atrial septal defect, single umbilical artery, defect, single umbilical artery, right-sided aortic archright-sided aortic arch

Renal agenesis or dysplasia, Renal agenesis or dysplasia, horseshoe kidney, polycystic horseshoe kidney, polycystic kidney, ureteral and urethral kidney, ureteral and urethral malformations, hypospadiasmalformations, hypospadias

DIAGNOSIS OF DIAGNOSIS OF ESOPHAGEAL ATRESIAESOPHAGEAL ATRESIA

Antenatal DiagnosisAntenatal Diagnosis ((maternal maternal polyhydramnios, a small stomach, a polyhydramnios, a small stomach, a distended upper esophageal pouch, distended upper esophageal pouch, or abnormal swallowing)or abnormal swallowing)

Diagnostic suspicion is increased Diagnostic suspicion is increased when abnormalities known to be when abnormalities known to be associated with esophageal atresia associated with esophageal atresia are identified.are identified.

Fetal MRIFetal MRI

This 32 week This 32 week fetus had fetus had esophageal esophageal atresia and an atresia and an absent absent stomach, stomach, resulting in resulting in marked marked polyhydramnios polyhydramnios

Clinical DiagnosisClinical Diagnosis

Prematurity Prematurity Any excessively drooling (cAny excessively drooling (copious, opious,

fine, white, frothy bubbles of mucus fine, white, frothy bubbles of mucus in the mouth and, sometimes, the in the mouth and, sometimes, the nosenose)). .

Clinical DiagnosisClinical Diagnosis

.. (A) Diagnosis of (A) Diagnosis of esophageal atresia is esophageal atresia is confirmed when a 10-confirmed when a 10-gauge (French) gauge (French) catheter cannot be catheter cannot be passed beyond 10 cm passed beyond 10 cm from the gums. (B) A from the gums. (B) A smaller-caliber tube is smaller-caliber tube is not used because it not used because it may curl up in the may curl up in the upper esophageal upper esophageal segment, giving a segment, giving a false impression of false impression of esophageal esophageal

continuitycontinuity..

The chest radiograph The chest radiograph

A plain radiograph A plain radiograph will confirm the will confirm the tube has not tube has not reached the reached the stomachstomach

The Gasless AbdomenThe Gasless Abdomen

Absence of gas in Absence of gas in the abdomen the abdomen suggests that the suggests that the patient has either patient has either atresia without a atresia without a fistula or atresia fistula or atresia with a proximal with a proximal fistula only fistula only

Contrast studiesContrast studies

should be performed should be performed by an experienced by an experienced pediatric radiologist, pediatric radiologist, or after transfer to the or after transfer to the tertiary institution, tertiary institution, and with the use of a and with the use of a small amount (0.5 to 1 small amount (0.5 to 1 mL) of water-soluble mL) of water-soluble contrast. Care must be contrast. Care must be taken to avoid taken to avoid aspiration.aspiration.

ManagementManagement

Measures should be taken to reduce the Measures should be taken to reduce the risk of aspirationrisk of aspiration((continuous suctioning of continuous suctioning of the upper pouchthe upper pouch,, tthe infant's head should he infant's head should be elevatedbe elevated)). .

In infants with respiratory failure, In infants with respiratory failure, endotracheal intubation should be endotracheal intubation should be performed. performed.

Transfer to a major tertiary pediatric Transfer to a major tertiary pediatric institution is best not delayedinstitution is best not delayed ..

Summary of Preoperative Summary of Preoperative InvestigationsInvestigations

A plain radiograph A plain radiograph Renal ultrasonography and Renal ultrasonography and

echocardiography are routine preoperative echocardiography are routine preoperative investigationsinvestigations

Endoscopy or a careful midesophageal Endoscopy or a careful midesophageal contrast study performed in a tertiary contrast study performed in a tertiary center. In some centers, bronchoscopy is center. In some centers, bronchoscopy is performed routinely in all infants with performed routinely in all infants with esophageal atresia.esophageal atresia.

Operative Repair of Operative Repair of Esophageal AtresiaEsophageal Atresia

Surgical repair is delayed Surgical repair is delayed (1-2days) (1-2days) in infants with low birth weight, in infants with low birth weight, pneumonia or other major anomalies. pneumonia or other major anomalies.

Operative Repair of Operative Repair of Esophageal AtresiaEsophageal Atresia

Operative Repair of Operative Repair of Esophageal AtresiaEsophageal Atresia

  Respiratory difficulty Respiratory difficulty after feedings in a 3-after feedings in a 3-day-old boy. Barium day-old boy. Barium esophagogram clearly esophagogram clearly shows an H-shaped shows an H-shaped fistula between the fistula between the trachea and the middle trachea and the middle segment of the segment of the esophagus esophagus (arrowhead). Barium is (arrowhead). Barium is filling the bronchi of filling the bronchi of the right lower lobe the right lower lobe (arrows). (arrows).

TracheoTracheoesophagealesophageal fistula fistula without without atresiaatresia (type E). (type E).

TracheoTracheoesophagealesophageal fistula without fistula without atresiaatresia (type E). (type E).

Esophagogram Esophagogram shows a fistula shows a fistula (arrow) arising from (arrow) arising from the anterior portion the anterior portion of the esophagus of the esophagus ((ee) and passing ) and passing cephalad to the cephalad to the posterior portion of posterior portion of the trachea (the trachea (tt). ).

TracheoTracheoesophagealesophageal fistula fistula without without atresiaatresia (type E). (type E).

Endoscopic Endoscopic diagnosisdiagnosis

CongenitalCongenital tracheoesophageal tracheoesophageal  

fistula fistula

The endThe end