Esophageal atresia/Tracheo-esophageal fistula

Definition

Congenital obstruction of the esophagus characterized by interrupted the continuity of the esophageal wall with one or more fistulae may be present between the malformed esophagus and the trachea.

Incidence

It is the most common congenital malformation of the esophagus.Incidence approximately 1 out of every 3,000 babies.

Embryology

• Esophageal atresia with TEF

• Posterior deviation of the septum which divides the foregut into ventral laryngeotracheal tube and dorsal esophagus

• Isolated esophgeal atresia :

• Failure of the recanalization of the esophagus during the eighth week of development and is not associated with tracheo-oesophageal fistula.

Types

Type A

Type B

Type C

Type D

Type E

Frequency of associated anomalies in esophageal atresia

Abnormality PercentageCardiac 25Gastrointestinal 22Vertebral/Skeletal 20Renal 15Chromosomal (Trisomy 18 & 21) 7

VACTERL syndrome

Vertebral anomalies

Anorectal malformation

Cardiac anomalies

Tracheo-esophageal fistula

Esophageal atresia

Renal anomalies

Limb anomalies

VACTERL syndrome

Vertebral anomalies

Anorectal malformation

Cardiac anomalies

Tracheo-esophageal fistula

Esophageal atresia

Renal anomalies

Limb anomalies

- 70 %of patients with VACTERL association -small (hypoplastic) vertebrae or hemivertebra -may put the child at risk for developing scoliosis or curvature of the spine.

Sacral agensis

VACTERL syndrome

Vertebral anomalies

Anorectal malformation

Cardiac anomalies

Tracheo-esophageal fistula

Esophageal atresia

Renal anomalies

Limb anomalies

- 55% of patients with VACTERL association -usually noted at birth and often require surgery in the first days of life.

VACTERL syndrome

Vertebral anomalies

Anorectal malformation

Cardiac anomalies

Tracheo-esophageal fistula

Esophageal atresia

Renal anomalies

Limb anomalies

- 75% of patients with VACTERL association -The most common heart defects seen are ventricular septal defect (VSD), atrial septal defects and tetralogy of Fallot. Less common defects are truncus arteriosus and transposition of the great arteries.

VACTERL syndrome

Vertebral anomalies

Anorectal malformation

Cardiac anomalies

Tracheo-esophageal fistulaEsophageal atresia

Renal anomalies

Limb anomalies

- 70 % of patients with VACTERL association, although it can frequently occur as an isolated defect.

VACTERL syndrome

Vertebral anomalies

Anorectal malformation

Cardiac anomalies

Tracheo-esophageal fistula

Esophageal atresia

Renal anomalies

Limb anomalies

- 50% of patients with VACTERL association. - Renal agensis or severe reflux

VACTERL syndrome

Vertebral anomalies

Anorectal malformation

Cardiac anomalies

Tracheo-esophageal fistula

Esophageal atresia

Renal anomalies

Limb anomalies

- 75% of patients with VACTERL association. - include hypoplastic thumb, extra digits (polydactyly) fusion of digits (syndactyly) and forearm defects such as radial aplasia.

- 75% of patients with VACTERL association. - include hypoplastic thumb, extra digits (polydactyly) , fusion of digits (syndactyly) and forearm defects such as radial aplasia.

- 75% of patients with VACTERL association. - include hypoplastic thumb, extra digits (polydactyly) , fusion of digits (syndactyly) and forearm defects such as radial aplasia.

- 75% of patients with VACTERL association. - include hypoplastic thumb, extra digits (polydactyly) , fusion of digits (syndactyly) and forearm defects such as radial aplasia.

CHARGE association

ColobomaHeart anomaliesAtresia choanaeRetarded growthGenital hypoplasiaEar anomalies

CHARGE association

ColobomaHeart anomaliesAtresia choanaeRetarded growthGenital hypoplasiaEar anomalies

CHARGE association

ColobomaHeart anomaliesAtresia choanaeRetarded growthGenital hypoplasiaEar anomalies

CHARGE association

ColobomaHeart anomaliesAtresia choanaeRetarded growthGenital hypoplasiaEar anomalies

CHARGE association

ColobomaHeart anomaliesAtresia choanaeRetarded growth Genital hypoplasiaEar anomalies

CHARGE association

ColobomaHeart anomaliesAtresia choanaeRracheo-esophageal fistulaGenital hypoplasiaEar anomalies

Diagnosis

Clinical picture Investigations

Antenatal

Postnatal

Symptoms

• Drooling of frothy saliva from the angle of the mouth.

• Attacks of chocking and cyanosis on feeding.

• Tachypnea and aspiration pneumonia.

Signs

• Passage of a semi-rigid PVC radio-opaque 10F catheter will be arrested and coiled back.

• Signs of respiratory distress

• Abdomen is distended in cases of large fistula

• Other anomalies eg. Imperforate anus, skeletal anomalies

Causes of respiratory distress in EA/TEF

1. Regurgitation of saliva2. Regurgitation of milk on feeding attempts3. Reflux of gastric acid through distal pouch4. Abdominal distention when large fistula is

present

InvestigationsAntenatal US • Polyhydramnios• Small stomach• Associated anomalies

InvestigationsPlain X-ray chest & abdomen

Blind upper pouch with a marker tube (coiled tube).

Signs of aspiration pneumonia.

Stomach full of air from the distal fistula.

Also, aortic arch location, presence of vertebral or rib anomalies can be detected.

InvestigationsPlain X-ray chest & abdomen

Blind upper pouch with a marker tube (coiled tube).

Signs of aspiration pneumonia.

Gasless abdomen indicates no distal fistula.

Investigations

Other anomalies

Spinal US EchocardiographyAbdominal USGenetic counselling

Treatment

Preoperative care

Neonatal ICU admission and incubation Head elevation at 45° Continuous suctioning IV fluids Antibiotics Intramuscular Vitamin K Ventilation if required ( avoid face mask )

Timing and selection of surgical intervention

Determined by:

The degree of prematurity Patient weight Overall clinical condition Other associated anomalies Length of the gap between the ends of the

esophagus

EA with TEF• In healthy near term or full-term infants without

other severe anomalies and minimal pneumonitis:

Primary repair

• Within the first 24 to 72 hours after birth.• Right transverse extrapleural thoracotomy• Lower pouch is disconnected from the trachea by

ligation & division of the fistula• End to end anastmosis between the 2 pouches• Rertropleural drain placement & closure

Primary anastmosis

Long gap atresia

• Livatidi’s circular myotomy• Slim spiral myotomy• Mucosa-muscular flap from the upper pouch• Foker’s technique• Staged procedure Oesophagostomy to drain saliva & prevent aspiration Gastrostomy for feeding Ligation of the fistula to prevent reflux to the lung Later, esophageal replacement commonly by retrosternal colon

transposition, less commonly by stomach pull or jejunal interposition

Mucosa-Muscular flap from the upper esophageal pouch

Esophageal replacement

Colon transposition

Gastric pull up

In infants with severe pneumonia, prematurity or other significant medical problems that increase the risk of

major surgery.

• Gastrostomy tube placement is an effective method to decompress the intestinal tract and allow for medical optimization prior to esophageal repair.

• Gastrostomy tube placement to allow enteral feeding and the overflow of saliva from the upper pouch is controlled by frequent suction

• Estimation of the distance between both both pouches radiologically and management is done accordingly

In infants with esophageal atresia without tracheesophageal fistula

Postoperative care

• Oral feeding starts on 3rd day• esophageal study at 5th day• Daily assessment of the retropleural drain• Post-operative check for leak – UGI study with

head up 30°, water-souble contrast [not barium] and NGT proximal to anastomotic site

Complications

• Gastresophageal reflux• Anastmotic leakage• Recurrent tracheo-esophageal fistula• Tracheomalacia• Stricture

Minor leakage

Fistula following repair

Anastmotic stricture

Impacted food

PrognosisWaterstone’s risk classification

Survival (%)

Birthweight > 2.5 kgotherwise healthy

100

Birthweight 2.0 – 2.5 kg orhigher mild pneumonia ormoderate cardiac anomalies

85

Birthweight < 2.0 kg or higher, severe pneumonia orsevere associated cardiac anomlalies

65

Summary

Esophageal atresia

Type

C/P

Investigation

Prognosis

Post op. complications

Proximal atresia & distal fistula

Failure to pass NGT

X-ray chest showing coiled NGT

Cardiac anomalies

GE Reflux

Quiz

• A 2.8-kg. neonate with excessive salivation develops respiratory distress. Attempts to pass an orogastric catheter fail because the catheter coils in the back of the throat. A chest film is obtained and shows right upper lobe atelectasis and a gasless abdomen. Which of the following types of EA would be consistent with this findings?

A. Type A B. Type BC. Type CD. Type DE. Type E

The initial treatment for a pure esophageal atresia is:

A. Repair of the esophageal atresiaB. Repair of the esophagcal atresia with

placement of a gastrostomyC. Repair of the esophageal atresia,

Nissen fundoplicalion and placement of a gastrostomy

D. Gastrostomy alone

A baby boy was delivered at 38-weeks gestation, who despite initial suctioning, appears to be salivating excessively. He has mild tachpnea, but is pink.

A) How can the cause be established?B) What are the other anomalies that

are likely to be present?C) What would you do before

proceeding to surgery?