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Derived from primitive foregut 4thweek of gestation tracheoesophageal diverticulum forms from the laryngotracheal groove Tracheoesophageal septum

develops during 4th5thweeks, muscular + submucosal layer of T + E formed Elongates with descent of heart and lung 7thweek>>> reaches final length
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Esophageal atresia is when the upper part of the esophagus does not connect with the lower esophagus and stomach. Tracheoesophageal fistula (TEF) is a connection between the esophagus and the trachea or windpipe

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Incidence: 1/4000 20-30%: premature, BW<2000g M > F (25:3) Antenatal history: polyhydramnios (60%)

Esophageal Atresia with Distal Fistula (EA/TEF) This is the most common subtype, accounting for about 85% of EA anomalies ` Pure Esophageal Atresia ` Esophageal Atresia with Proximal Fistula ` Esophageal Atresia with Proximal and Distal Fistulas ` H-type Fistula without Esophageal Atresia
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VACTERAL and VATER syndrome: ` Vertebral anomalies: ` Anal: imperforate anus ` Cardiac: VSD, PDA, TOF, coarction of aorta, ASD ` Trachea: TEF ` Esophageal: EA ` Renal: renal agenesis,ureteral abnormalities, hypospadias ` Limb: polydactyly, wrist/knee anomalies CHARGE association ` Coloboma, Heart defects, Atresia of the choanae, developmental Retardation, Genital hypoplasia and Ear deformities ` EA has been observed occasionally in patients with CHARGE association. These patients have high mortality and morbidity rates

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cough, chocking, cyanosis, excessive salivation, Drooling Respiratory distress Very round, full abdomen (TEF) Unusually flat abdomen (isolated esophageal atresia) inability to pass NGT down the esophagus into the stomach

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Prenatal Ultrasound =polyhydramnios, absent stomach, MRI Reveals a blind distended esophageal pouch Postnatal A complete medical history physical examination

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plain X-ray
neck, the thorax, and the abdomen with NGT inserted. The approximate length of the upper pouch can be estimated by the length of the X-ray visible tube in it. Air below the diaphragm can be seen in the presence of a lower tracheoesophageal fistula and additional fluid levels indicate a duodenal or intestinal atresia. A gasless abdomen indicates a pure esophageal atresia without a lower fistula .A long distance between the segments is to be expected, but a tiny or secondary occluded fistula may be present extremely rarely. The translucency of the lungs provides the first information on whether aspiration pneumonia either from the saliva or from the refluxed gastric acid through the lower fistula is present.

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PrePre-op treatment : Emergency surgery is rarely required NPO Antibiotics IV fluids Continuous low suction to prevent aspiration of saliva The baby should be positioned upright to minimize gastro-esophageal reflux into the trachea and lungs via the lower fistula

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Esophageal Atresia wit Distal tracheoesophageal Fistula (85%) (85%) Under general anesthesia Rt thoracotomy incision at 4th intercostal space Either intra-plerual or intra-pleural approach Transfixion ligation of fistula Primary anastomosis is 1st choice In case of long gap the circular myotomy according to Livadatis can lengthen the upper esophageal pouch by 0.51.0 cm In case of failed primary anastomosis esophagostomy and gastrostomy are done Esophageal replacment are done at age of 1 yr of 9 kg with colon interposition or gastric pullup

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Isolated Esophageal Atresia esophagostomy and gastrostomy are done Esophageal replacment are done at age of 1 yr of 9 kg with colon interposition or gastric pullup

Early complication : recurrence of the tracheo-esophageal fistula (3%), which usually occurs following significant anastomotic leak anastomotic leak should be lower than 10% if no tension was on the anastomosis. In most of these cases the defects are small and clinically insignificant If the patients conditions are stable we may even start oral feeding because spontaneous closure of the fistula can be expected An anastomotic stenosis is a common finding Tracheo-malacia is a common finding after esophageal atresia patients with lower fistula
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Late complication : The most common late complication is gastroesophageal reflux causing feeding problems, vomiting,reduced weight gain or dystrophy, and recurrent respiratory tract infections, Reflux is very typical when the lower segment of the esophagus has to be pulled-up to be able to perform a primary anastomosis. Recurrent respiratory tract infections are also common problem
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