Presentation on esophageal atresia and tracheoesophageal fistula

Presentation on esophageal atresia and tracheoesophageal fistula

1. Presentation on “Care of child with esophageal Atresia and Tracheoesophagial fistula” by Syanthika Dutta B.Sc Nursing 3 rd year CON, MCH

2. Overview- A fistula is an abnormal communication between two epithelial-lined surfaces. An atresia refers to complete obliteration or absence of an orifice of a hollow organ. A stenosis is change in diameter of a hollow organ to become narrower. It can also be called stricture.

3. Atresia Fistula Stenosis

4. Introduction- Esophageal atresia (EA) and tracheoesophageal fistula(TEF) are the most common congenital anomalies of oesophagus . It is a acquired communication between the trachea and esophagus . Most of the patient with TEF are diagnosed immediately following after birth. TEF are often associated with life threatening complications

5. Definition- Esophageal Atresia (EA)- defined as the complete absence of continuity of oesophagus to stomach. Tracheoesophageal Fistula (TEF)- defined as the congenital abnormal connection between proximal or distal oesophagus and the trachea. A tracheoesophageal fistula, as the name implies, is an abnormal communication between the trachea and esophagus .

6. Normal Anatomy

7. Incidence- EA with or without TEF is the most common oesophageal malformation, occurring in approximately 1 in 3500 live births. The vulnerable groups are- premature and low birth weight (LBW) infants and mothers with polyhydramnios . Often associated with other GI and cardiac anomalies.

8. Embryology- The median pharyngeal groove (primitive digestive tube) develops in the ventral aspect of foregut at day 22 of gestation, which develops into the respiratory and digestive tubes. These two tubes are separated by the mesenchyme . The development of an abnormal epithelial-lined connection between the two tubes results in the creation of the TEF. Excess mesenchymal growth can stretch and disrupt the oesophagus, creating EA.

9. Lung buds

10. Classification- (Gross classification)

11. Classification- ( contd ). Type C Type D Type B Type A Type E

12. Classification- (Gross classification)-5 types- EA with or without TEF :-   Type A- Isolated EA without TEF - both distal and proximal part of esophagus is blind and no fistula is developed between esophagus and trachea. Atresia / Blind pouch

13. Continuation. Type B- Proximal TEF with distal EA - the proximal and distal part of esophagus is connected to trachea by a fistula and the distal part of esophagus is blind. Fistula Atresia

14. Type C- Distal TEF with proximal EA – the proximal part of esophagus is blind and distal part is connected by a fistula to trachea. Atresia Fistula

15. Type D-EA with proximal and distal TEF - both proximal and distal parts of esophagus are connected to trachea by two separate fistula.

16. Type E- TEF without EA or H type TEF - there is no presence of atresia in the esophagus but is attached with trachea by a single fistula.

17. Etiology - The exact cause is unknown . Some factors- Heritable genetic factors- A 2% risk is present when one of the sibling is affected; occasional association is with trisomies of 21, 13 and 18 chromosomes. Maternal alcohol and smoking Exogenous sex hormones Exposure to methimazole Prolonged mechanical ventilation via endoctracheal tube Maternal polyhydamnios Diabetes Mellitus during the first trimester of pregnancy.

18. Pathophysiology -

19. Clinical Manifestations- 3C’s- Coughing Chocking Cyanosis Excessive oral secretions- the infants drools and has substantial mucus along with oral secretions . Respiratory Distress Gagging Abdominal Distension Regurgitation Aspiration pneumonia

20. Intermittent unexplained cyanosis and laryngospasm . Poor feeding Seal- bark cough Violent response after first or second swallow of feeding such as infant cough and chokes fluid through nose and mouth.

21. Diagnosis- Ultrasound scanning - prenatal diagnosis may reveal the size of gastric bubble , polyhydramnios and other associated anomalies Plain X-ray of abdomen Chest X-ray using air contrast in the proximal pouch. If distal TEF is present, air in the stomach will be seen on X-ray. Passing of radiopaque catheter through oesophagus- the catheter does not reach to the desired length. Tracheo-brochoscopy - to examine the tracheobronchiolar tree . It helps in detection of associated tracheobronchial anomalies. ECG- for infants with clinical signs of cardiovascular disease .

22. Laboratory values- - Complete blood count (CBC) -Electrolyte levels -BUN -Blood glucose -Serum calcium - ABG analysis

23. Chest Xray showing Oesophageal Atresia

24. Chest X ray showing Tracheoesophageal Fistula

25. Management- Immediate management- - Infant is managed with head elevated at a position of 30degree to reduce the risk of gastric secretion entering into lungs . - Nasogastric aspiration of secretions for every 5-10mins. Blind pouch should be washed with normal saline to prevent blocking of tube with thick mucus . - IV fluid administration . - Airway clearance with oxygen therapy . - Nothing per mouth

26. Supportive management- -Maintenance of nutritional requirements and warmth. -Infection prevention -Antibiotic therapy -Respiratory support -Chest physiotherapy and postural drainage  

27. Medical management- In healthy infants without pulmonary complications, primary repair is done along with parenteral nutrition and upper pouch suction. Pre-0peratively, a cuffed endotracheal tube is place distal to the fistula site in order to prevent the reflux of gastric contents to the lungs.  

28. Surgical management- a. Corrective approach D one by end-to-end anastomosis with excision of the fistula by right postolateral thoracotomy with internal chest drainage. It is done with infants more than 2kg body weight with no pneumonia, thereby emphasising clinically stable conditions.  

29. Corrective approach of surgery

30. B.Correction by staging- Intially gastrostomy is performed, followed by esophageal anastomosis or colonic transplant after one year. This is done mainly for premature and very sick infants with congenital anomalies. Circular oesophagotomy - performed on proximal pouch to gain length and allow for primary anastomosis at initial surgery.  Cervical oesophagotomy / Esophagocoloplasty - When oesophagus ends are too widely separated oesophageal replacement with the segments of intestine (colonic reposition). It is done at the age of 18 to 24 months.  After surgical methods, IV fluid therapy is given for the first 48 hours followed by gastrostomy feeding.

31. Nursing management-   a. History- A mother who is carrying the foetus may have polyhydramnios , which occurs in approximately in 33% cases and virtually 100% of mothers with foetuses with oesophageal atresia without fistula.   B.Physical Examination- VACTERL (vertebral defects, anorectal malformations, cardiovascular defects, tracheoesophageal defects, renal anomalies, and limb deformities) refers a set of associated anomalies that should be readily apparent upon physical examination. T he presence of the others must be assessed.          

32. C . Nursing Diagnosis- a. Preoperative- Ineffective airway clearance related to abnormal communication between trachea and oesophagus as evidenced by excessive secretions, respiratory distress. Imbalanced nutrition- less than body requirements r elated to feeding difficulties as evidenced by regurgitation of feed. Impaired swallowing related to obstruction secondary to anatomical defect as evidenced by drooling of saliva. Risk of aspiration r elated to abnormal communication between oesophagus and trachea. Risk for fluid volume deficit related to inadequate oral intake. Parental anxiety r elated to neonatal surgical emergency. Altered family process related to physical defect and hospitalization.

33. b. Postoperative - Ineffective airway clearance related to surgical interventions as evidenced by assessment. Altered nutrition, less than body requirements related to inadequate oral intake as evidenced assessment and observation. Pain related to surgical interventions as evidenced by facial grimancing and cry of the infant. Impaired skin integrity related to surgical wound as evidenced by assessment of the surgical site incision. Risk of infection related to the hospital procedures. Risk of injury related to complex surgery. Risk of altered parenting related to prolonged illness. Knowledge deficit related to home- based long term care.    

34. D.Nursing Interventions- a. Preoperative- Preventions of aspirations by positioning, suctioning and NPO. Assessment of respiratory status. Administration of oxygen. Prevention of dehydration by IV fluid, intake and output chart, monitoring of vital signs and general health. Perform oropharyngeal suctioning to remove accumulations of secretions. Placement of suction catheter in proximal pouch for every 10-15mins. Watch for any choking and cyanosis during feeding. Prevention of infections by infection control measures. Teach family about gastrostomy feeding techniques. Reducing parental anxiety by explaining the outcomes of the impending surgery and by emotional support.

35. b. Postoperative - Maintaining of clear airway. Providing adequate feeding by IV fluid and gastrostomy feeding. Pain management- By tactile stimulations Administering of analgesics (if necessary) Provision of comfortable position with comfort devices. Periodic inspection and assessment of the surgical site. Maintaining of chest tube drainage with necessary precautions. Monitoring of child’s condition and detection of problems early. Prevention of infections by careful handling of the baby. Stimulating parent-child bondage by parental participation of child care. Improving knowledge by necessary health education, encouraging questions and explaining answers.

36.   Complications- Recurrent pneumonia Acute lung injury Acute respiratory distress syndrome, lung abscess Poor nutrition Bronchiectasis from recurrent aspiration Respiratory failure Hiatal hernia GERD Anastomotic leak Tracheomalacia The major postoperative complications are- tracheal stenosis and recurrent fistula.