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Esophageal Atresia and Tracheoesophageal Fistula

  1. The esophagus and trachea derive from the primitive foregut. During the fourth and fifth weeks of embryologic development, the trachea forms as a ventral diverticulum from the primitive pharynx (caudal part of the foregut).
  2. A tracheoesophageal septum develops at the site where the longitudinal tracheoesophageal folds fuse together.
  3. This septum divides the foregut into a ventral portion, the laryngotracheal tube and a dorsal portion (the esophagus).
  4. Esophageal atresia results if the tracheoesophageal septum is deviated posteriorly.
  5. This deviation causes incomplete separation of the esophagus from the laryngotracheal tube and results in concurrent tracheoesophageal fistula.
  6. Esophageal atresia as an isolated congenital anomaly may occur, rarely. In these cases, the atresia is attributable to failure of the recanalization of the esophagus during the eighth week of development and is not associated with tracheoesophageal fistula.
  7. Most common type of Tracheo- Oesophageal Fistula is Esophageal atrresia with distal tracheoesphageal fistuls. (MAHE 07)
  8. Investigation:
    1. Prenatally – USG, may suggest EA or TEF                                                                        
    2. 1st perinatal investigation in a suspected EA for TEF is chest x-ray PAS lateral view.
    3. Inability to pass o rigid NG tube from mouth + stomach is diagnostic of EAS / or TEF,  but this should be confirmed with radiologic visualization.
    4. Radiological investigation of choice with EA is 3D CT it 100% sensitive with specific.Q
  9. Pathology
    1. The most common variant of this anomaly consists of proximal a blind esophageal pouch with a Fistula between the trachea and the distal esophagus, which is estimated to occur 84 percent of the time. (MAHE 07)
    2. The fistula often enters the trachea close to the carina.
    3. The second most common anomaly is pure atresia without tracheoesophageal fistula.
  10. Associated Defects
    1. Associated congenital anomalies are discovered in approximately 50%. The acronym VATER, or VACTERL (vertebral defect, anorectal malformation, cardiac defect, tracheoesophageal fistula, renal anomaly, radial dysplasia and limb defects), has been used to describe the condition of multiple anomalies in these infants.
    2. Cardiac anomalies are the most common especially ventricular septal defect & TOF.
  11. Clinical Presentation and Diagnosis
    1. The first sign of esophageal atresia in the fetus may be polyhydramnios in the mother. Q
    2. Classically, the neonate with esophageal atresia presents with copious, fine, white, frothy bubbles of mucus in the mouth and, sometimes, the nose.
    3. The infant may have rattling respirations and episodes of coughing, choking and cyanosis. These episodes may be exaggerated during feeding.
  1. Management and Treatment
    1. Greatest risk to a child with EA and/or TEA is aspiration.Measures should be taken to reduce the risk of aspiration.
    2. The oral pharynx should be cleared, and an 8 French sump tube placed to allow for continuous suctioning of   the upper pouch.
    3. The infant's head should be elevated. Intravenous fluids (10 percent dextrose in water) should be started.
    4. Oxygen therapy is used as needed to maintain normal oxygen saturation. In infants with respiratory failure,   endotracheal intubation should be performed.
    5. Bag-mask ventilation is not appropriate since it may cause acute gastric distention requiring emergency gastrostomy.
    6. If sepsis or pulmonary infection is suspected, broad-spectrum antibiotics should be administered.
    7. Before surgical correction, the infant must be evaluated thoroughly for other congenital anomalies.
    8. Gastrostomy for gastric decompression is reserved for use in patients with significant pneumonia or atelectasis, to prevent reflux of gastric contents through the fistula and into the trachea.
    9. Healthy infants without pulmonary complications or other major anomalies usually can undergo primary repair in the first few day
    10. Prior to surgical procedure, the position of the aortic arch should be confirmed & surgical approach should be opposite to the aortic arch.
    11. The most common complication include anastomotic leak may be recurrent fistula, stricture. 

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