TEF/EA: The less talked about issues

TEF/EA: The less talked about issuesAlyssa Brzenski MDMay 2, 2012OverviewBackgroundPre-repair bronchoscopyThorascopic repairTo extubate or not?Esophageal atresia treatment of long-gap esophageal atresia Complications following TEF/EA repairCase 1Called 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.Case 25 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.BackgroundTEF/EA associated with1:2,500-4,000 live births30% of which the neonate is prematureFew cases diagnosed prenatallyMay present with inability to pass an OGT

Background

Co-morbidities

Waterson Classification

Spitz Classification

Pre-repair Bronchoscopy

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

Thorascopic vs. Open Repair

Thorascopic vs. Open RepairReduces Musculocutaneous sequelae32% of patients have significant musculocutaeous sequelae24% with winged scapula20% asymmetry of chest wall 2/2 atrophic serratus anterior18% developed thoracic scoliosisBetter visualizationReduced Pain Post-operatively

Patient Position

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.

Anesthetic ConsiderationsRoutine ASA monitors +/- A-lineMaintence of spontaneous ventilation during inductionClassic teaching that paralysis can be given after fistula ligatedBalanced anesthetic +/- epidural for post-op pain managementMay have difficulty with hypercapnia or difficulty ventilating

Fistula Management

Extubate or Not?Must consider pre-op lung disease and other comorbiditiesSpontaneous ventilation decreases the stress placed on the suture lineRisk of injury to the repaired fistula with re-intubationLong-gap Esophageal AtresiaDefined as Greater than 3cm between the esophageal endsIdeal to use the patients own esophagus Excess tension on the esophageal anastamosis is associated with increased complications and worse outcomeSurgical OptionsPrimary anastamosis at time of initial repairSerial staged dilation with bougie followed by esophageal anastamosisExternal tension with sutures, magnets, etc to lengthen esophagus following by esophageal anastamosisEsophageal replacement with gastric pullthrough, colonic graft or jejunal graft

Gastric Pullthrough

Gastric PullthroughFree up the stomach via laparoscopyCervical approach to bring down the cervical esophagoscopy (spit fistula), followed by creating a track in the mediastinum to approach the two ends of the esophagusAnesthetic Concerns of Gastric PullthroughLengthy procedureCapnothorax or Capnomediastinum when surgeon taking down the stomachCan have difficulty ventilating during the esophagoscopy take down and esophageal mediastinum due to large dilators compressing a small airwayBleeding Need adequate IV accessComplications following TEF/EA RepairAnastomotic leakRecurrent esophageal fistulaEsophageal stricturesGERD/Esophageal dismotilityTracheomalacia/ Pulmonary IssuesMusculocutaneous disturbancesAnastomotic leakEarly complication occurring in 17% of patientsTypically will resolve spontaneously without oral feeds or with pleural drainageCase reports of glycopyrolate and atropine used to minimize secretionsMajor leaks may require cervical esophagostomy and gastrostomy with delayed definitive repairEsophageal strictures and recurrent fistula are more likely to follow

Recurrent TEF

Recurrent Esophageal FistulaSerious complication affecting 5-20% of patientsOpen thoracotomy associated with morbidity and mortality rates of 10-22%Endoscopic Closure preferredPresents with cough, choking, or cyanosis with feeding, or recurrent pneumoniaEndoscopic Closure of RTEFClosure can be obtained with de-epitheliazation of the fistula, application of tissue adhesivesDe-epitheliazation of the fistulaApplication of tissue adhesives(Tissel, dermabond, etc)Combination of bothHighest 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-epitheliazationDe-epithelithalization

Fibrin Glue

Endoscopic Closure of RTEFPerformed with Rigid BronchPossibility of inability to ventilate if aspiration of a Fibrin Plug Occlusion of the trachea with the glue Esophageal StricturesOccurs in 6-40% of patientsMore common with Gap >2.5cmEA/TEF type A, C, DNon-absorbable suturesPresents with dysphagia, poor feeding, and emesisTreated with Esophageal dilationImproves with timeEsophageal DysmotilityEsophageal peristalsis is abnormal in 75-100% of patients with EA/TEFSmall discoordinate contractions lead to increased risk for esophageal obstructionsImproves 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 ageGERDOccurs in 35-58% of TEF/EA childrenDue to intrinsic motor dysfunction of the esophagus as well as possible anastomotic tension56% of patients with GERD respond to medical therapy13-25% of patients will require a Nissen fundoplicationHowever, attempts are made to avoid fundoplication due to risk of severe dysphagia following given dyskinetic esophagusRespiratory ComplicationsPresent in 46% of patients following EA/TEF repair74% GERD13% with tracheomalacia13% with recurrent TEF

TracheomalaciaPresent in 75% of pathologic specimens in patients with EA/TEFClinically significant in 10-20%Usually found at or just above the level of the original EA/TEFPresents with brassy cough, stridor, and dyspnea with feedsTreatment usually medicalBibliographyBroemling 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: 422430.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.