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