Esophageal atresia

Pediatric surgery. For mbbs undergraduates

1. Oesophageal Atresia

2. Objectives Definition. Epidemiology. Embryology . Types. Associated anomalies. Pathophysiology. Diagnosis & Investigations Management Complications

3. Esophageal Atresia Congenital discontinuity of esophageal lumen is known as esophageal atresia. Tracheoesophageal Fistula Abnormal communication between trachea and esophagus.

4. Epidemiology 1 per 2500 – 3000 live births. 50% have 1 or more associated anomalies: Cardiac, Anorectal , Genitourinary, vertebral/skeletal & others. 2% risk of recurrence is present when sibling is affected. Increased in advanced maternal age.

5. Embryology Both the esophagus and trachea originate from the primiti ve foregut

6. Stages in the development The laryngotracheal diverticulum forms as a ventral outpouching from the caudal part of the primitive pharynx. Longitudinal tracheoesophageal folds begin to fuse toward the midline to eventually form the tracheoesophageal septum. The tracheoesophageal septum completely forms.

7.  At week 4, the tracheoesophageal diverticulum developed to tracheoesophageal septum . There is a failure to separation in the septum leading to fistula.  During week 8, the primitive gut failure of recanalization leads to atresia .

8. Pathogenes is unk nown Occur sporadica lly Multifa ctorial . . . . . multiple genes and complex gene- environment intera ction

9. THEORI ES 1 . E s o p h a g e a l occlusion and fai lure of recanali zation 2 . S p o n t a n e o u s deviatio n of septum 3 T e r a t o g e n . . Doxorubicin

10. Classification According to the system formulated by Gross:- Type A - Esophageal atresia without fistula or pure esophageal atresia (10%) Type B - Esophageal atresia with proximal TEF (<1%) Type C - Esophageal atresia with distal TEF (85%) Type D - Esophageal atresia with proximal & distal TEFs (<1%) Type E - TEF without esophageal atresia or H-type fistula (4%) Type F - Congenital esophageal stenosis (<1%)

11. Associated Anomalies V ertebral defects. A norectal malformations. C ardiovascular defects. R enal anomalies. L imb deformi t y

12. Associated Congenital Anomalies Reported in Patients with Esophageal Atresia

13. P athophysiolo g y A fetus with esophageal atresia cannot effectively swallow amniotic fluid. Polyhydramnios Premature labour Aspiration of saliva or milk, if the baby is allowed to suckle, can lead to an aspiration pneumonitis.

14. In a baby with esophageal atresia and a distal TEF, the lungs may be exposed to gastric secretions. Also, air from the trachea can pass down the distal fistula when the baby cries, strains, or receives ventilation. This condition can lead to over distension of abdomen which ultimately may cause acute gastric perforation, which is often lethal.

15. D I AGNOS I S  Antenatal Diagnosis of esophageal atresia is d o n e du r ing routine maternal Ultra s onography   Polyhydramnios  A small stomach  A distended upper esophageal pouch

16. Clinical Diagnosis Prematurity Excessive drooling ( copious, fine, white, frothy bubbles of mucus in the mouth and sometimes, the nose ) If these signs go unnoticed the first feed is almost always accompanied by coughing, choking and cyanosis . After birth these babies often appear to blow bubbles when attempting to swallow mucous and saliva. Sign & Symptoms of respiratory distress due to aspiration pneumonia.

17. Diagnosis of esophageal atresia is confirmed when a 10-gauge (French) catheter cannot be passed beyond 10 cm from the gums. A smaller-caliber tube is not used because it may curl up in the upper esophageal segment, giving a false impression of esophageal continuity.

18. Investigations 1 st line Investigations :- Plain radiograph Esophagogram Endoscopy Others:- Renal Ultrasonography Echocardiography Bronchoscopy Laboratory investigations.

19. A plain radiograph Fig (A) Orogastric catheter in the blind esophageal pouch & presence of air in the stomach. Fig (B)The radiopaque tube in the blind esophageal pouch & absence of air in the stomach

20. esophagogram (Contrast X-Ray) Plain x-ray with contrast in the upper esophagus above the atresia.

21. Renal ultrasonography To evaluate associated kidney anomalies, ureteral anomalies, or both.

22. Echocardiography Who have clinical signs of Cardiovascular disease.

23. Management Aims of management:- Prevention of Aspiration Pneumonia & its consequences. Establishment of communication between proximal & distal end of esophagus. Separation of esophagus from trachea.

24. Preoperative management The oropharynx should be cleared. The infant's head should be elevated. IV fluids & Nutrition. Oxygen therapy. In infants with respiratory failure, endotracheal intubation should be performed. Broad Spectrum Antibiotics.

25. Timing of surgical correction (A) Immediate Primary Repair No pulmonary complications, viz. severe pneumonia. An arterial PaO2> 60mm Hg in room air. Not associated with major congenital malformations which are compatible with life. Gap between proximal and distal esophageal pouch less than 2.5 cm.

26. (B) Delayed surgical intervention Associated with pneumonia, sepsis or cardiac malformation. Severe prematurity The gap between proximal and distal pouch is more than 2.5cm.

27. (C) Staged operation:- The indications are: If primary repair can not be done due to reasons mentioned in delayed surgical repair Failed primary repair. Long gap between two esophageal pouches viz.in type 1 esophageal atresia, where gap is usually more than 3-4 cm.

28. Stages of surgical correction Left cervical esophagostomy and feeding gastrostomy immediately after birth. Thoracotomy & Ligation of tracheoesophageal fistula. Replacement of the gap between proximal and distal esophagus by isolated vascularized segment of colon or by gastric tube.

29. Complications (A) Early :- Pneumothorax Anastomotic leakage Recurrence of fistula Pulmonary atelectasis or infection (B) Late:- Anastomatic Stricture Gastroesophageal reflux(GER) Tracheomalacia Disordered esophageal Peristalsis(Dysphagia)

30. Prognosis

31. Take Home Message Early diagnosis allows time and place of delivery to be planned so that early pediatrics surgical correction and neonatal care is available. This also gives time for preparation and education of the parents.

32. Than k You!