Tracheoesophageal Fistula PEDIATRIC.pptx

Tracheoesophageal FistulA

1. TRACHEOESOPHAGEAL FISTULA Dr . Poonam Trivedi Neonatology Fellow , B. J. WADIA HOSPITAL

2. INTRODUCTION Tracheoesophageal fistula with esophageal atresia is one of the common congenital anomaly in neonates. Esophageal atresia and tracheoesophageal fistula may occur as separate congenital defects but more frequently they are seen as compound defects. At least 85% of infants with esophageal atresia also have TEF.

3. DEFINITIONS Tracheoesophageal fistula: It is an abnormal connection between trachea and oesophagus Oesophageal Atresia : It is failure of oesophagus to form continuous passage from the pharynx to the stomach

4. EPIDEMIOLOGY Incidence of TEF with EA is 1:3000 to 1:4000 live births Male predominance Increased incidence in premature infants Although this anomaly seen in siblings and identical twins, role of genetic factors in unclear.

5. EMBRYOLOGY

6. Embryology Stages in development: A) Laryngeotracheal diverticulum forms as ventral out pouching from the caudal part from the primitive pharynx. B) Longitudinal Tracheopharyngeal folds begin to fuse towards midline to eventually form tracheosophagal septum at 4 weeks of gestation . Defective septum due to deviation or incomplete fusion of these longitudinal folds leads to formation of TEF. C) During 8 th week, primitive gut failure of recanalisation leads to atresia .

7. Classification According to Gross classification , TEFs are classified as:- 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 and distal TEF (<1%) Type E : TEF without EA or H type fistula (4%)

8. Pathophysiology A fetus with esophageal atresia cannot effectively swallow amniotic fluid . Polyhydramnios  Premature labour Aspiration of saliva or milk leads to aspiration pnemonitis In babies with esophageal atresia and distal TEF lungs may expose to gastric secretion Also air from trachea can passed down to distal fistula which can lead to abdominal distention and in severe cases gastric perforation.

9. Clinical Presentation Infants often have vomiting and excess salivation soon after feeding. Respiratory distress due to following reasons 1 ) A irway obstruction by excess salivation. 2 ) Aspiration of saliva and milk. 3 ) Compromised pulmonary capacity due to diaphragmatic elevation because of abdominal distension. 4 ) Reflux of gastric contents through fistula. Coughing & Choking C yanosis Abdominal distension Poor feeding P neumonia

10. ASSOCIATED ISSUES & ANOMALIES 1) Babie s with TEF and EA often have: LBW 20% - SGA 20% - Premature 2) Other Anomalies: Chromosomal Abormalities VACTERL Association: Vertebral defects(17%) Imperforate Anus(12%) Cardiac defects(20%) TEF with EA Renal Defects(16%) Limb anomalies(10%)

11. DIAGNOSIS A) PRENATALY: 1)ULTRASOUND EXAMINATION :- Polyhydramnios is present in approximately 1/3 rd of pregnancy with TEF/EA because fetus is unable to swallow amniotic fluid. Absence of fluid filled stomach,small abdomen,lower than expected fetal weight & distended esophageal pouch. 2)FETAL MRI :- Confirm the diagnosis of TEF/EA.

12. B)POSTNATALY: 1) Inability to pass catheter in to the stomach completely. 2) X-ray: Catheter coiled in upper esophageal pouch seen. A distended blind upper esophageal pouch with air that is unable to progress in to stomach. Cardiac and vertebral anomalies.

13. 3) Bronchoscopy : Visualises fistula between trachea and oesophagus .

14. 4) Echocardiogram and ultrasonography : To look for cardiac and renal anomalies .

15. Management The definitive treatment for TEF/EA is Surgical. Pre-operative management: AIM: Focuses on minimising risk of aspiration and avoiding gaseous abdominal distention. SUCTION: A sump multiple end hole Replogle catheter should be positioned in the upper esophageal pouch to continously aspirate saliva. POSITION: Head of the bed should be elevated 30 degrees RESPIRATORY SUPPORT: Oxygen therapy.Mechanical ventilation should be avoided . NPO, IV Fluids & B road spectrum antibiotics Appropriate timings of surgery Nasogastric aspirations C hest physiotherapy & P ostural drainage

16. DEFINITIVE TREATMENT: SURGERY Surgical intervention depends on the type of TEF, distance between proximal and distal pouch of esophagus, general condition of baby and associated anomalies. If infant has cardiac defects that requires surgery it is best to repair fistula first, if not post operative management becomes difficult If distance between upper and lower esophageal segment is ( <2.5cm) and general condition is good primary repair i.e E sophago-esophagostomy (Right dorsolateral thoracotomy ) is done.

17. STAGING SURGERY: When the distance between two esophageal segments is large and condition of the infant is poor and associated with other anomalies, a two stage procedure is required. First stage: Tracheoesophageal fistula is ligated and gastrostomy done to reduce risk of reflux and to provide feeding. Second stage: Proximal and distal esophageal segments are anastomosed . If gap is too large, a segment of colon is used for reconstruction of esophagus. This is done about 18-24 months. A Fogarty catheter may be passed with a bronchoscope to occlude th fistula in extreme cases in which infant cannot tolerate thoracotomy and definitive procedure.

18. Post operative management IV Nutritional support Respiratory support Enteral feeding through gastrostomy or O G tube Antibiotics & Analgesics

19. Complications Anastomotic leak (10-15%) Diagnosed by presence of saliva in chest tube and confirmed by contrast swallow study. Most leaks close spontaneously and management is expectant. Esophageal stricture (5-10%) Diagnosis confirmed by barium swallow examination. Treatment: serial esophageal dilatation. Gastroesophageal reflux (40-70%) Treatment: Antacids and intestinal motility agents Recurrent fistula (10%) Manifestation: Aspiration with GER Treatment: small fistula close spontaneously, large fistula surgical repair Tracheal obstruction (25%) Diagnosis of tracheal obstruction due to tracheomalacia is made by bronchoscopy Most cases subside over 1-2 years of life.

20. PROGNOSIS TEF/EA with/without respiratory complications Overall survival rate FT infants without respiratory complications 95% Premature infants or those with moderate to severe respiratory distress 85% Infants with multiple anomalies/ severe respiratory distress 75% Infants with BW <1500gms and major cardiac anomaly 50%

21. TAKE HOME MESSAGE TEF with EA is a fairly common condition encountered by neonatologists & pediatric surgeons. Despite increased experience and advancement in management, etiology still remains a mystery. Early prenatal diagnosis allows time and place of delivery so that expertise care and early surgical correction is available. The definitive treatment for TEF/EA is surgical and timely management increases the overall survival rate.