Derived from primitive foregut•4thweek of gestation tracheoesophageal

diverticulum forms from the laryngotracheal groove

•Tracheoesophageal septum develops during 4th-5thweeks, muscular + submucosal layer of T + E formed

•Elongates with descent of heart and lung•7thweek>>> reaches final length

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

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

VACTERAL and VATER syndrome:Vertebral anomalies: Anal: imperforate anusCardiac: VSD, PDA, TOF, coarction of aorta, ASDTrachea: TEFEsophageal: EARenal: renal agenesis,ureteral abnormalities, hypospadiasLimb: polydactyly, wrist/knee anomaliesCHARGE associationColoboma, Heart defects, Atresia of the choanae, developmental Retardation, Genital hypoplasia and Ear deformitiesEA has been observed occasionally in patients with CHARGE association. These patients have high mortality and morbidity rates

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

Prenatal Ultrasound =polyhydramnios, absent

stomach, MRI Reveals a blind distended esophageal

pouch

Postnatal A complete medical history physical examination

plain X-rayplain 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.

Pre-op treatment :Pre-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

Esophageal Atresia wit Distal Esophageal Atresia wit Distal tracheoesophageal Fistula (85%)tracheoesophageal Fistula (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 0.5–1.0 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

Isolated Esophageal AtresiaIsolated Esophageal Atresiao esophagostomy and gastrostomy are doneo Esophageal replacment are done at age of 1

yr of 9 kg with colon interposition or gastric pullup

Early complication :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 patient’s 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

Late complication :Late complication :The most common late complication is

gastro-esophageal 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