TRACHEOESOPHAGEAL FISTULA hbmjgffddddffg

TEF

1. TRACHEOESOPHAGEAL FISTULA (TEF)

2. DEFINITION TRACHEO-OESOPHAGEAL FISTULA Abnormal connection between the trachea and the esophagus OESOPHAGEAL ATRESIA Failure of esophagus to form continuous passage from the pharynx to the stomach

3. INCIDENCE & ETIOLOGY 1 in 3500 births, with slight male dominance Premature infants with Birth weight less than 2500 grams Presence of maternal polyhydramnios and single umbilical artery can result in atresia Children with TEF have other anomalies that are represented by acronym ‘VACTERL’ V – Vertebral Column Defects A – Anorectal Malformation C – Cardiac Defects TE – Tracheo -esophageal Fistula R – Renal Anomalies L – Limb Anomalies

4. TYPES Vogt classified Oesophageal atresia and Fistula into 5 types: Type I (Type A): proximal and distal segments of esophagus are blind; there is no connection to trachea; accounts for 3.7% to 7% of cases; second most common

5. Contd …… Type II (Type B): proximal segment of esophagus opens into trachea by a fistula; distal segment is blind; rare, 0.8% of cases . Type III (Type C): proximal segment of esophagus has blind end; distal segment of esophagus connects into trachea by a fistula; most common, with 86% of cases.

6. Contd …….. Type IV (Type D): esophageal atresia with fistula between proximal and distal ends of trachea and esophagus; (rare, 0.7% to 6% of cases). Type V (Type E): proximal and distal segments of esophagus open into trachea by a fistula; no esophageal atresia but sometimes referred to as an H-type fistula ; occurs in 4.4% to 7% of cases, not usually diagnosed at birth.

7. PATHOPHYSIOLOGY The upper part of esophagus is developed from the retropharyngeal segment and lower part from the pregastric segment of foregut At about 4 weeks of gestation, a laryngo -tracheal groove is formed which divides the foregut into two longitudinal tubes , which further develop into respiratory tract and digestive tract. Defective separation due to deviated or incomplete septum or incomplete fusion of tracheal folds results in malformation of trachea and esophagus.

8. CLINICAL MANIFESTATION Excessive secretions coming out of nose and constant drooling of saliva. Saliva is frothy Intermittent unexplained cyanosis and laryngospasm caused by aspiration of accumulated saliva in blind pouch Abdominal distention occurs in type III, IV, V Violent response after first or second swallow of feeding The infant coughs and chokes. Fluid returns through nose and mouth. Cyanosis occurs. The infant struggles .

9. DIAGNOSTIC EVALUATION Antenatal Ultrasound scan Failure to pass a 10F catheter (smaller catheters may coil) into the stomach through nose or mouth. Catheter is left in situ while an X-ray confirms the diagnosis. X-ray of abdomen and chest - reveal air filled oesophageal pouch and air in stomach and catheter coiled in the blind pouch. Barium X-ray may be used in some cases . USG enables identification of the type of TEF

10. MANAGEMENT IMMEDIATE TREATMENT Propping infant at 30-degree angle to prevent reflux of gastric contents. 10F NG tube remains in the esophagus and is aspirated frequently to prevent aspiration until continuous low suction is applied. Pouch is washed out with normal saline to prevent thick mucus from blocking the tube. Gastrostomy to decompress stomach and prevent aspiration; later used for feedings. Nothing by mouth (NPO); I.V. fluids.

11. Contd ……. TREATMENT OF EXISTING PATHOLOGIC PROCESSES Existing pathologic processes are either acquired complications, such as pneumonitis , or complications from concomitant lesions such as heart failure . SUPPORTIVE THERAPY Supportive therapy includes meeting nutritional requirements, I.V. fluids, antibiotics, respiratory support, maintaining thermally neutral environment.

12. SURGICAL MANAGEMENT Prompt primary repair: fistula found by bronchoscopy is divided, followed by esophageal anastomosis of proximal and distal segments if infant weighs more than 2000 g and is without pneumonia. Short-term delay: subsequent primary repair is used to stabilize infant and prevent deterioration when the patient's condition contraindicates immediate surgery.

13. Contd …….. Staging: initially, fistula division and gastrostomy are performed with later secondary esophageal anastomosis or colonic transplant performed approximately 1 year later to effect total repair. Approach may be used with a very small, premature infant or a very sick neonate or when severe congenital anomalies exist. Circular esophagomyotomy may be performed on proximal pouch to gain length and allow for primary anastomosis at initial surgery. Cervical esophagostomy : when ends of esophagus are too widely separated, esophageal replacement with segment of intestine is done at ages 18 to 24 months.

14. NURSING DIAGNOSIS PREOPERATIVE Risk for Aspiration related to structural abnormality Risk for Deficient Fluid Volume related to inability to take oral fluids Impaired breathing related to frequent laryngospasm and excessive secretions in the trachea Anxiety of parents related to critical situation of neonate

15. Contd ……… POSTOPERATIVE Ineffective Airway Clearance related to surgical intervention Ineffective Infant Feeding Pattern related to defect Acute Pain related to surgical procedure Impaired Tissue Integrity related to postoperative drainage Impaired nutrition related to surgery Altered comfort related to chest tube drainage and surgery Risk for Impaired Parent/Infant Attachment related to prolonged hospitalization

16. NURSING INTERVENTIONS PREVENTING ASPIRATION Position the infant with head and chest elevated 20 to 30 degrees to prevent or decrease reflux of gastric juices into the tracheobronchial tree. Turn frequently to prevent atelectasis and pneumonia. Perform intermittent nasopharyngeal suctioning or maintain indwelling Replogle tube (double-lumen tube) or sump tube with constant suction to remove secretions from esophageal blind pouch. Place the infant in an Isolette or under a radiant warmer with high humidity to aid in liquefying secretions and thick mucus.

17. Contd …… Maintain the infant's temperature in thermoneutral zone, and ensure environmental isolation to prevent infection by using Isolette . Administer oxygen as needed. Suction mouth to keep clear of secretions and prevent aspiration. Provide mouth care. Be alert for indications of respiratory distress.

18. C ontd …. PREVENTING DEHYDRATION Administer parenteral fluids and electrolytes as prescribed. Monitor vital signs frequently for changes in blood pressure (BP) and pulse, which may indicate dehydration or fluid volume overload. Record intake and output, including gastric drainage (if gastrostomy tube for decompression is present) and weight of diapers .

19. Contd ….. Reducing Parental Anxiety Explain procedures and necessary events to parents as soon as possible. Orient parents to hospital and intensive care nursery environment. Allow family to hold and assist in caring for infant. Offer reassurance and encouragement to family frequently. Provide for additional support by social worker, clergy, and counselor as needed.

20. Contd …… PROVIDE ADEQUATE NUTRITION Administer I.V. solutions until gastrostomy feedings can be started . Feed the infant orally or by gastrostomy , depending upon the type of surgery done. Begin gastrostomy feedings as soon as ordered because adequate nutrition is an important factor in healing. The gastrostomy is generally attached to gravity drainage for 3 days postoperatively, then elevated and left open to allow for air to escape and gastric secretions to pass into the duodenum before feedings are begun . Use care to prevent air from entering the stomach, thereby causing gastric distention and possible reflux.

21. Contd ……. PROVIDE COMFORT MEASURES Position comfortably. Avoid restraints when possible. Administer mouth care frequently. Offer pacifier frequently. Administer analgesics as ordered. Wean as soon as possible so ventilator can be discontinued without risk. Handling should be kept to a gentle minimum.

22. COMPLICATIONS Death from asphyxia Pneumonitis secondary to: Salivary aspiration Gastric acid reflux Concomitant lesions (approximately 40% to 50%): Congenital heart disease GI anomalies, particularly imperforate anus Skeletal and muscular deformities Renal anomalies Vertebral defects (possibility of VACTERL syndrome) Prematurity Dehydration and electrolyte imbalance