1. Alyssa Brzenski MD May 2, 2012

2. Overview Background Pre-repair bronchoscopy Thorascopic repair To extubate or not? Esophageal atresia – treatment of long-gap esophageal atresia Complications following TEF/EA repair

3. Case 1 Called to do a case in the NICU. The patient is a 2 day old 26 week neonate with a distended abdomen. He under went an ex-lap yesterday for NEC with free-air and resection of part of the small bowel and primary anastamosis. Over the last few hours, progressive abdominal distention with free air seen again on X-ray. The surgeon gains adequate exposure of the abdomen and can not find any area of bowel perforation, but notes that the stomach is enlarged and seems to be increasing in a rhythmic cycle, perhaps with the ventilator.

4. Case 2 5 month old term infant presenting for definitive repair of EA. Initially, taken to the operating room at an OSH on DOL 1 for repair of TEF. On exposure, the gap was noted to be 4cm and thought to be too lengthy for closure. Fistula was ligated, cervical esophagoscopy was created and g-tube placed. Plan today to perform esophageal anastamosis with lap assisted gastric pull-through via a cervical approach.

5. Background TEF/EA associated with 1:2,500-4,000 live births 30% of which the neonate is premature Few cases diagnosed prenatally May present with inability to pass an OGT

6. Background

7. Co-morbidities

9. Waterson Classification

10. Spitz Classification

11. Pre-repair Bronchoscopy

12. The Evidence behind the pre-repair Bronch May change the operative management (changed operative approach in 57% with 31% being crucial changes) Bronchoscopy can Define the fistula location Determine unusual characteristics of the fistula(double fistula or trifurcation) Determine presence of tracheobronchitis (surgery contraindicated) Locate the aortic arch Influence anesthetic management

13. Thorascopic vs. Open Repair

14. Reduces Musculocutaneous sequelae 32% of patients have significant musculocutaeous sequelae 24% with winged scapula 20% asymmetry of chest wall 2/2 atrophic serratus anterior 18% developed thoracic scoliosis Better visualization Reduced Pain Post-operatively

15. Patient Position

16. Anesthesia for Thorascopic Rarely need lung isolation as operative lung compressed by CO2 insufflation (5mmHg) Can be associated with mild desaturation requiring 100% O2 or mild hand ventilation. Some centers using HFOV for these repairs to minimize the movement of the operative side (MAP 14-24, Hz=10-14, delta P=20-27, FiO2 adjusted to Sat of 92%) EtCO2 will be falsely low due to compression of the lung and CO2 insufflation.

17. Anesthetic Considerations Routine ASA monitors +/- A-line Maintence of spontaneous ventilation during induction Classic teaching that paralysis can be given after fistula ligated Balanced anesthetic +/- epidural for post-op pain management May have difficulty with hypercapnia or difficulty ventilating

18. Fistula Management

19. Extubate or Not? Must consider pre-op lung disease and other comorbidities Spontaneous ventilation decreases the stress placed on the suture line Risk of injury to the repaired fistula with re-intubation

20. Long-gap Esophageal Atresia Defined as Greater than 3cm between the esophageal ends Ideal to use the patient’s own esophagus Excess tension on the esophageal anastamosis is associated with increased complications and worse outcome

21. Surgical Options Primary anastamosis at time of initial repair Serial staged dilation with bougie followed by esophageal anastamosis External tension with sutures, magnets, etc to lengthen esophagus following by esophageal anastamosis Esophageal replacement with gastric pullthrough, colonic graft or jejunal graft

22. Gastric Pullthrough

23. Free up the stomach via laparoscopy Cervical approach to bring down the cervical esophagoscopy (spit fistula), followed by creating a track in the mediastinum to approach the two ends of the esophagus

24. Anesthetic Concerns of Gastric Pullthrough Lengthy procedure Capnothorax or Capnomediastinum when surgeon taking down the stomach Can have difficulty ventilating during the esophagoscopy take down and esophageal mediastinum due to large dilators compressing a small airway Bleeding– Need adequate IV access

25. Complications following TEF/EA Repair Anastomotic leak Recurrent esophageal fistula Esophageal strictures GERD/Esophageal dismotility Tracheomalacia/ Pulmonary Issues Musculocutaneous disturbances

26. Anastomotic leak Early complication occurring in 17% of patients Typically will resolve spontaneously without oral feeds or with pleural drainage Case reports of glycopyrolate and atropine used to minimize secretions Major leaks may require cervical esophagostomy and gastrostomy with delayed definitive repair Esophageal strictures and recurrent fistula are more likely to follow

27. Recurrent TEF

28. Recurrent Esophageal Fistula Serious complication affecting 5-20% of patients Open thoracotomy associated with morbidity and mortality rates of 10-22% Endoscopic Closure preferred Presents with cough, choking, or cyanosis with feeding, or recurrent pneumonia

29. Endoscopic Closure of RTEF Closure can be obtained with de-epitheliazation of the fistula, application of tissue adhesives De-epitheliazation of the fistula Application of tissue adhesives(Tissel, dermabond, etc) Combination of both Highest overall and first time success with combination treatment(93.3 and 66.7% respectively) Likely will need repeat procedures– first time success 28.6% with tissue adhesives and 50% for de- epitheliazation

30. De-epithelithalization

31. Fibrin Glue

32. Endoscopic Closure of RTEF Performed with Rigid Bronch Possibility of inability to ventilate if aspiration of a Fibrin Plug Occlusion of the trachea with the glue

33. Esophageal Strictures Occurs in 6-40% of patients More common with Gap >2.5cm EA/TEF type A, C, D Non-absorbable sutures Presents with dysphagia, poor feeding, and emesis Treated with Esophageal dilation Improves with time

34. Esophageal Dysmotility Esophageal peristalsis is abnormal in 75-100% of patients with EA/TEF Small discoordinate contractions lead to increased risk for esophageal obstructions Improves with time as 65% of kids will be admitted with GI sx in the first 10 years of their life, but only 3% of patients will be admitted after 18 years of age

35. GERD Occurs in 35-58% of TEF/EA children Due to intrinsic motor dysfunction of the esophagus as well as possible anastomotic tension 56% of patients with GERD respond to medical therapy 13-25% of patients will require a Nissen fundoplication However, attempts are made to avoid fundoplication due to risk of severe dysphagia following given dyskinetic esophagus

36. Respiratory Complications Present in 46% of patients following EA/TEF repair 74% GERD 13% with tracheomalacia 13% with recurrent TEF

37. Tracheomalacia Present in 75% of pathologic specimens in patients with EA/TEF Clinically significant in 10-20% Usually found at or just above the level of the original EA/TEF Presents with brassy cough, stridor, and dyspnea with feeds Treatment usually medical

38. Bibliography Broemling N, Campbell F. Anesthetic Management of Congenital Tracheoesophageal Fistula. Peds Anesth 21(2011): 1092-99. Holcomb GW et al. Thorascopic Repair of Esophageal Atresia and Tracheoesophageal Fistula: A Multi-Institutional Analysis. Ann Surg 2005;242: 422–430. Briganti V et al. Usefulness of dextranamer/hyaluronic acid copolymer in bronchoscopic treatment of recurrent tracheoesophageal fistula in children. International Journal of Pediatric Otolaryngology. 75(2011): 1191-94. Atzori P et al. Preoperative tracheobroncoscopy in newborns with esophageal atresia. Journal of Peds Sugery. 41(2006): 1054-57. Meier J et al. Endoscopic Management of Recurrent Congenital Tracheoesophageal Fistula: A Review of Techniques and Results. International Journal of Pediatric Otolaryngology. 71(2007): 691-97. Tovar JA, Fragoso AC. Current Controversies in the Surgical Treatment of Esophageal Atresia. Scandanavian Journal of Surgery. 100(2011): 273-8. Sung M et al. Endoscopic Management of Recurrent Tracheoesophageal Fistula with trichloroacetic Acid Chemocauterization: A Preliminary Report. Journal of Pediatric Surgery. 43(2008): 2124-7. Knottenbelt G et al. Tracheo-esophageal fistula and oesophageal atresia. Best practice and Research Clinical Anesthesiology. 24 (2010): 387-401.