Oesophageal atresia

My presentation in General Surgery course which was in September 2012.

1. esophageal atresia & tracheo-esophafeal fistula By Fahad H. Al Hulaibi 208004222 Kingdom of Saudi Arabia Ministry of Higher Education King Faisal University College of Medicine

2. Objectives: Definition. Epidemiology. Embryology of esophagus & Trachea. Types & classifications. Associated anomalies. Pathophysiology . Diagnosis & Treatment.

3. Definition Esophageal atresia : is a disorder of the digestive system in which the esophagus does not develop properly. ؟!!

4. Atrasia :  Congenital absence or closure of a normal body opening. Normal Atrasia

5. Fisula :  Is a permanent abnormal passageway between two organs in the body or between an organ and the exterior of the body. Normal F istual

6. Epidemiology 1 case in 3000-4500 births. the highest incidence of this disorder is in Finland, where it is 1 case in 2500 births.

7.  2% risk of recurrence is present when a sibling is affected. Increase in advanced maternal age.

8. Embryology of esophagus & Trachea

9. At week 4 , the Tracheabroncheal diverticulum developed to Tracheabroncheal sputum. There is a failure to separation in the sputum leading to fistula . During week 8, the primitive gut failure to recanalization . That lead to atrasia .

10. Types & classifications

11. Type A - Esophageal atresia without fistula or so-called 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 and distal TEFs (< 1%) Type E - TEF without esophageal atresia or so-called H-type fistula (4%) Type F - Congenital esophageal stenosis (< 1%) (This is not discussed in this article)

12. Etiology Currently, most authorities believe that the development of esophageal atresia has a nongenetic basis . In a 1987 Kluth eschews has the concept that esophageal vascular events , ischemic events, or both may be causes in cases of esophageal atresia without fistula. In 2003, Spilde et al reported esophageal atresia -TEF formations   Adriamycin induced teratogenesis .

13. Associated anomalies (VACTERL) Vertebral defects. Anorectal malformations. Cardiovascular defects. TrachoEsophageal deffect . Renal anomalies. Limb deformities. 25% of all patients with esophageal atresia

14. CHARGE Coloboma . Heart defects. Atresia choanae . Developmental retardation. Genital hypoplasia . Ear deformities .

15. Neurologic defects -Neural tube defects, hydrocephalus, tethered cord, holoprosencephaly GI defects -Duodenal atresia , ileal atresia , hypertrophic pyloric stenosis , omphalocele , malrotation , Meckel diverticulum Pulmonary defects - Unilateral pulmonary agenesis, diaphragmatic hernia Genitalia defects - Undescended testicles, ambiguous genitalia, hypospadias

16. Pathophysiology A fetus with EA cannot effectively swallow amniotic fluid. A fetus with esophageal atresia and a distal TEF, amniotic fluid presumably flows through the trachea and down the fistula to the gut  Polyhydramnios    premature labor

17. The neonate with EA cannot swallow and there is copious amounts of saliva. Aspiration of saliva or milk, if the baby is allowed to suckle, can lead to an aspiration pneumonitis .

18. 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 an acute gastric perforation, which is often lethal.

19. Diagnosis Prenatal: 1. polyhydramnios

20. 2. Prenatal Ultrasonography sensitivity  40 % Abscenc of stomach bubbles, with fliud filled loops of bowels

21. Post-natal: 1. White, frothy bubbles of mucus in the mouth and, sometimes, the nose. 2. Episodes of coughing, choking and cyanosis. 3. These episodes may be exaggerated during feeding.

22. Diagnosis Laboratory Studies: CBC count Electrolyte levels Venous gas concentrations BUN and serum creatinine levels Blood glucose level Serum calcium level ABG concentrations, as necessary

23. Diagnosis Genetic testing: chromosome analysis microarray genomic hybridization (array GH)

24. Chest radiography NG tube arrested Air in stomach  AP view Lateral view

25. CT scan

26.  Renal UltraSonography is used to evaluate associated kidney anomalies, ureteral anomalies, or both.

27. Echocardiography.   who have clinical signs of cardiovascular disease.  

28. Limb radiography   if the limbs appear abnormal. 

29. treatment Preoperative management: 1. The oral pharynx should be cleared. 2.  The infant's head should be elevated. 3. IV fluids (10% dextrose in water). 4. Oxygen therapy is used. 5. In infants with respiratory failure, endotracheal intubation should be performed. 6. broad-spectrum antibiotics(such as ampicillin plus gentamicin )

30. Surgical techniques vary according to surgeons' preferences and variations in pathologic anatomy. The best esophagus is the patient's esophagus.

31. Infants born with esophageal atresia without fistula  early gastrostomy

32. Kimura, Livaditis , Scharli , or Foker procedures

33. Infants born with esophageal atresia with fistula  early gastrostomy

34. referances Roberta A Pagon , Editor-in-chief, Thomas D Bird, Cynthia R Dolan, Karen Stephens, and Margaret P Adam, GeneReviews ™, Seattle (WA): University of Washington, Seattle; 1993. Amulya K Saxena , Esophageal Atresia With or Without Tracheoesophageal Fistula, Medscape . eMedicine , Updated: Apr 25, 2012, link: ( http://emedicine.medscape.com/article/935858-overview#showall ) DWAYNE C. CLARK, Esophageal Atresia and Tracheoesophageal Fistula, 1999 Feb 15;59(4):910-916. Kronemer KA, Snyder-Warwick A. Esophageal atresia / tracheoesophageal fistula. 2008. Available online at  eMedicine . Accessed 4-1-11.