? Introduction
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? Embryology
? Epidemiology
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? Associated anomalies? Classification
? Diagnosis-
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?Antenatal diagnosis
?Postnatal diagnosis
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? Management-?Pre-operative management
?Operative management
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?Post-operative management
? Complications
Introduction
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? To anastomose the ends of an infant's esophagus, the surgeon must
be as delicate and precise as a skilled watchmaker. No other
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operation offers a greater opportunity for pure technical artistry.-Dr. Willis Potts(1950)
? The first successful repair was done in 1940.
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? Most-neonatal centres are performing repair now days with successup to 90%.
Embyrology
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? 4th week of gestation:
? the foregut --------->a ventral respiratory part
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--------->a dorsal esophageal partIncomplete fusion of the folds
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defective trachea-esophageal septum
abnormal connection between the trachea and esophagus
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EpidemiologyIncidence: 1 in 2500 to 3000 live births.
Slight male preponderance- 1.26:1
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? Chromosomal associations:
? Environmental associations:
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? DiGeorge syndrome? Methimazole in early pregnancy
? Trisomy 21, 13 and 18
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? Prolonged use of contraceptive
? Opitz syndrome
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pills? 13q, 17q and 16q24deletions.
? Progesterone and estrogen
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exposure
? Single gene mutations:
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? Maternal diabetes? Feingold syndrome
? Thalidomide exposure
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? CHARGE syndrome
? Fetal alcohol syndrome
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? Fanconi anaemia? Maternal phenylketonuria
Associated anomalies
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? The most frequent associated malformations encountered insyndromic EA are:
? Cardiac (13?34%)
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? Vertebral (6?21%)
? Limb (5?19%)
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? Anorectal (10?16%)? Renal (5?14%)
? Two syndromic associations:
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? VACTER-L: Vertebral, anorectal, cardiac, tracheo-esophageal, renal and limb
? CHARGE: Coloboma, heart defects, atresia of the choanae, developmental
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retardation, genital hypoplasia and ear deformities.Classification
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? Many classification system has been proposed:? Vogt
? Waterston
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? Ladd
? Gross
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? Gross classification is most commonly used.? Based on anatomic variation
Diagnosis
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? Antenatal diagnosis: Ultrasonography? Polyhydramnios
suggestive of EA
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? Absent or small stomach bubble
? Fetal MRI.
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Diagnosis? Postnatal:
? Premature babies.
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? Symptomatic within first few hours of life.
? Variants A, B, C and D- excessive drooling of saliva.
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? Choking, regurgitation and coughing on oral feeds.? Variants C and D- as the child cries, air goes into the stomach and causes abdominal
distension and respiratory distress.
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? Variants C, D and E- gastric juice may regurgitate through the fistula causing
chemical pneumonitis.
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? Inability to pass an orogastric tube into the stomach.
? X-ray:
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? Plain chest radiograph: dilated upper pouch.? If a soft feeding tube is used, the tube will coil in the upper pouch.
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Gasless abdomen:Pure esophageal atresia and proximal fistula
No route for gas to enter abdomen
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? Plain chest radiographs help in assessment of the gap between the
proximal and distal end.
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? Helps to plan for surgical management.
? Absence of air in abdomen is associated with long gap.
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? Gap < 2 vertebrae -Primary anastomosis
? Gap > 2and < 6 vertebrae -
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Delayed anastomosis
? Gap > 6 vertebrae -
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Esophageal replacementBronchoscopy
Pre-operative management
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? Oral suctioning through 10F double lumen Replogle tube.? Position of child: Propped up position- least gastric reflux.
? Intravenous access and I. V. antibiotics.
? I. V. fluids (10% dextrose and hypotonic saline)and Vitamin K
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administration.? If respiratory distress is present due to pneumonitis, mechanical
ventilation may be done at low pressures.
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? High pressure ventilation may cause gastric dilatation and gastric perforation
as well as diaphragmatic splinting.
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? Investigations for associated anomalies:? Echocardiography- to look for cardiac defects
? USG abdomen- to look for any renal abnormality.
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? X-rays spine- to look for any vertebral anomaly
? X-ray upper limbs- to look for any radial anomaly.
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Operative management
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? Open thoracotomy:? Through 4th intercostal space
? Extrapleural approach
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? Posterior mediastinum is exposed.
? Azygous vein is divided to reveal underlying TEF.
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? TEF is dissected circumferentially.? Attachment of the fistula to the membranous portion of the trachea is taken
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down.? Tracheal opening is closed with non absorbable interrupted sutures.
? Upper pouch of the esophagus is mobilised as much as possible.
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? The distal end of the upper pouch is opened and a nasogastric tube is passed
through the nares to upper esophagus to the lower esophagus and then to
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the stomach.? Anastomosis of the ends of the esophagus are done over the nasograstric
tube with absorbable sutures.
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? Wounds is closed after placement of chest drain.
? Thoracoscopic approach is also being used.
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? Special cases:
? H type fistula: division of the fistula done through the cervical approach.
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Long gap atresia
? Delayed closure with lengthening procedure (at about 12weeks of life)
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? Spontaneous growth? Bougienage
? Upper pouch mobilisation
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? Kimura's extrathoracic elongation technique
? Foker traction suture technique
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? Upper pouch myotomy and flaps? Esophageal replacement
? Stomach
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? Colon
? Jejunum
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? IleumComplications
? Anastomotic leaks
? Anastomotic stricture
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? Recurrent tracheoesophageal fistula? Tracheomalacia
? Disordered peristalsis/ gastroesophageal reflux/ esophageal cancer
? Vocal cord dysfunction
? Respiratory morbidity
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