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Esophageal Atresia
Long Island College HospitalDaniel H. Hunt, M.D.
May 5th, 2006
History8 y.o. boy admitted on 2/17/06 with a diagnosis of severe gastroesophageal reflux disease. He was scheduled to undergo an elective revision of his colonic interposition graft performed 7 years earlier for esophageal atresia. He had multiple prior admissions for aspiration pneumonia and received tube feedings for failure to thrive.PMH: Asthma, GERDAllergies: latexBirth Hx: 32 ½ weeks, polyhydramniosPSH: esophagostomy and gastrostomy ’97, colonic interposition ‘98Medications: Advair, Singular, Prevacid, Albuterol
Physical
Wt 64lbs 75%Cardiac: no murmursChest: CTA b/lAbd: well healed upper midline scar, RUQ feeding tube, NT/ND, no massesGenitalia: normal, testes descended b/lRectal: normal toneExt: no c/c/e
Laboratory Values
1354.0
9825
100.6
129 2936.613.839
13.328.1
1.1
Intraop Events
Procedure: thoracoabdominal exploration(median sternotomy), tapering coloplasty, partial resection colon interposition, Nissen fundoplication, Stamm gastrostomyEBL: 50ccFluids: 1700ccOR time: 5 hours
Postop Course
POD#1 - Extubated in PICUPOD#6 – Esophogram and return of bowel functionPOD#7 - Tube feeding startedPOD#8 - Full liquids startedPOD#15 – Discharged home tolerating soft diet
Management of Esophageal Atresia
Historical Perspective
1670 – Durston - First reported case in conjoined twins 1888 – Steele - First attempted surgical correction of pure EA by gastrostomy 1939 – Levin and Ladd - First survivors of staged repair of EA-TEF
Historical Perspective
1941 – First successful primary repair - Haight
Left extrapleural approachPrimary single layer anastomosisFistula ligation
“In recent years there has been no more dramatic advance in surgery than that which has taken place in the treatment of congenital atresia of the esophagus.” Gross R.
Historical Perspective
Gradual increase in survival rates from 1941 to present 95-99% Advances in neonatal intensive care, nutritional support, surgical management, antibiotic therapy
Epidemiology
Incidence 1 in 3,570 to 4,000Slightly higher frequency in males85% cases with tracheoesophageal fistulaHigher rate low birth weight and preterm birth
Epidemiology
57.3% with atresia only, 36.4% with other major malformations, 6.3% chromosomal anomalies*1 in 10,000 white births 0.55 in 10,000 non white birthsHigher rate in monozygotic twins *
* Robert E, et al. Reproductive Toxicology, Vol 7, pp 405-421, 1993
Embryology
Derived from the primitive foregut4th week of gestation trachesophageal diverticulum forms from the laryngotracheal grooveTracheoesophageal septum develops during the 4th to 5th weeksElongates with descent of heart and lungs7th week reaches final relative length
Moore KL: The Respiratory System. The Developing Human Being. 5th ed. Philadelphia, WB Saunders, 1993
Clark DC. American Family Physician, 59(4), 1999
Etiology
Aberration at 26-32 days gestationDifferential growth rateCellular differentiationApoptosis
Notochord abnormalities – sonic hedgehog-Gli signaling pathwayNeural crest abnormalities
Vleesch Dubois VN, et al., Eur J Pediatr Surg 2002;12(2):83-89
Presentation/Diagnosis
Prenatal ultrasoundPolyhydramnios (1 in 12)Small or absent stomachDistended blind esophageal pouch
Prenatal MRI Blind esophageal pouch
Presentation/Diagnosis
PostnatallyExcessive droolingFine frothy bubbles in mouth and noseChoking, coughing, cyanotic episodes, and regurgitation with feedingInability to pass 10Fr OGT
Imaging
Prenatal ultrasound Good screening test 42% sensitivePolyhydramnios in combination with small or absent stomachNo significant role postnatally
Imaging
Prenatal MRIFollowing positive US findings100% sensitivity and 80% specificPositive if esophagus absent in mid chestNo significant role postnatally
Imaging
Plain RadiographyConfirmatory testOGT in esophageal pouchPresence or absence of gas in the abdomenAssess gap lengthAnomalies
PneumonitisAtelectasisCardiacVertebralAortic arch
Imaging
Gasless AbdomenCoiled OGT
Imaging
Contrast studiesRarely neededIdentification of proximal fistulaMeasuring gap lengthShould use water soluble contrastPerform under fluoroscopy
Imaging/Diagnostics
BronchoscopyEsophagoscopyCTMRI
Classification
Clark DC. American Family Physician, 59(4), 1999
Associated Anomalies
VACTERL – vertebral, anorectal, tracheoesophageal, radial, renalTrisomy 18 and 21 (7%)Congenital heart disease (25%)Urinary tract (22%)Orthopedic – vertebral and radial (15%)GI – i.e. duodenal atresia, imperforate anus (22%)
Management
Minimal handling to minimize gastric distention and regurgitationNPO!!Avoid bag-mask ventilationMaintain in partial upright position 45°Repeated upper esophageal pouch suctioning minimum q10min or low continuousTransfer to tertiary pediatric institution for management and definitive care
Management
IV Tx - 10% dextrose with hypotonic NaCl solutionVitamin K analog preoperativelyBroad spectrum antibioticsTPN if surgery delayed for several days
Preoperative Investigations
Plain radiograph of torso ± contrastEchocardiographyRenal ultrasonographyBronchoscopy – selectivelyEsophagoscopy - selectively
Management of Anomalies
Congenital heart diseaseNon-duct-dependent conditions – early EA repairDuct dependent disease – PGE1 infusion –EA repair when stable followed by cardiac surgeryRight aortic arch – EA through left thoracotomy
Management of Anomalies
Urinary TractReflux associated nephropathyR/O bilateral renal agenesis or severely multicystic dysplastic kidneysRenal scan if absent on US
Management of Anomalies
GastrointestinalCareful anorectal exam – EA repair followed by anorectal anomalyDuodenal atresia – combined EA repair duodenoduodenostomyHigh imperforate anus – combined EA repair colostomy
Management of Anomalies
ChromosomalEA repair contraindicated in trisomy 1870% mortality with EA + major chromosomal anomaly
Orthopedic – no contraindication to EA repairVertebral i.e. Hemivertebrae – progressive scoliosisRadial club handAbsent thumbHip dislocationTalipes equinovarus
Surgical Therapy
DeterminantsType of esophageal anomalyCondition of infantOther congenital anomalies present
Surgical Therapy
Staged Approach Pure EA and select EA and TEFStamm gastrostomy Delayed fistula division and esophageal reconstruction
Surgical Therapy
EA and TEFFistula division with primary esophageal anastomosisRight thoracotomy via 4th ICSFistula divided close to trachea with air-tight ligationMobilization of proximal segment with circular myotomy if extra length requiredSingle layer closure with absorbable suture knots internalFeeding tube placed across anastomosis
Surgical Therapy
Pure esophageal atresiaDelayed repair gastrostomy then primary anastomosisEsophageal lengthening
BougienageCircumferential or spiral myotomyProximal and distal esophageal mobilizationMultistaged extrathoracic lengthening
Cervical esophagostomyFailed repairPrimary anastomosis impossible
Surgical Therapy
Esophageal replacementColon –
Right, left, or transverseRetrosternal or posterior mediastinalPassive conduit
Colon Interposition
ProsActs as a conduit antiperistaltically or isoperistalticallyGood vascular supply via marginal arteryCan be placed in esophageal bed of posterior mediastinumHas mucous shield, which protects against refluxResponds to acid with a peristaltic rush for clearanceMinimizes/eliminates tension on the upper and lower esophageal segments
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Colon Interposition
ConsRequires 3 anastomosesEmpties more slowly than the esophagusRequires preoperative bowel preparationLong surgical procedure with extensive mobilizationDilates and becomes redundant over timeSlows food transit
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Gastric Tube
Reversed (antiperistaltic) tube More commonSupplied by left gastroepiploic artery
Nonreversed (isoperistaltic) tubeSupplied by the right gastroepiploic artery
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Gastric TubePros
Does not become dilated, tortuous, or redundantLess risk of ischemia because of robust blood supplySimplified construction through stapler useHas a favorable anatomic location in the upper abdomenRequires fewer anastomosesHas comparable diameter and occupies less space in the thorax and neckHas only one suture lineRequires no bowel preparation and is a faster procedure
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Gastric Tube
ConsExtensive gastroesophageal refluxLeaves a small gastric reservoirCreates a long suture lineMay result in gastric outlet obstructionMay be unable to reach high in neckDifficult to place in posterior mediastinum
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Gastric Transposition
ProsReadily available and easily mobilized stomachInvolves single anastomosisAdequate length availableHas excellent blood supplyInvolves a technically easy procedureHas low incidence of leaks and strictures
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Gastric TranspositionCons
Large bulky possibly causing space problems intrathoracicallyRefluxPossible stricture or aspiration due to lack of gastroesophageal valvePoor gastric emptyingMay affect pulmonary functionResults in depleted iron stores causing anemiaMay not reach as high in neck as other methods because of blood supply
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Jejunum
ProsJejunal caliber similar to that of normal esophagusFunctions as reliable food transporterResults in low incidence of leaks and stricturesFunctions as an effective gastroesophageal barrierDoes no require a bowel preparation
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Jejunum
ConsLength of conduit limited by blood supplyInfarction commonly resulting from passage through chestProcedure more technically difficultRequires 3 anastomosesHas high peptic ulcer susceptibilityBlood supply lacking marginal arteryHas high failure rate
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
Complications
Anastomotic leakage 14-21%Fistula recurrence 3-14%Esophageal strictures 40%Gastroesophageal reflux 40-70%Tracheomalacia 10-20%Esophageal dysmotility
Minkes RK, Congenital Anomalies of the esophagus. Emedicine, May 2006
104 patients with EA and distal TEF onlyMean operative time 129.9 min, mean days of hospitalization 18.15 operations converted to open (4.8%)11.5% developed early leak31.7% required esophageal dilatation24% required required fundoplication later3 deaths
Annals of Surgery 2005;242:422-430
Summary
Once a death sentence EA and TEF has a survival rate approaching 100% with advances in neonatal care and pediatric surgeryPrimary esophageal repair is the accepted approach if possibleThe selection of esophageal conduits should be made on an individual basis