Esophageal Atresia with Esophageal Atresia with Tracheo-Esophageal Tracheo-Esophageal

FistulaFistula

AnatomyAnatomyIn full term neonate esophagus is 9 – 10 cm In full term neonate esophagus is 9 – 10 cm in length, lumen 3 – 4 mmin length, lumen 3 – 4 mm

Arterial supply:Arterial supply: Upper 1/3 – Inferior thyroid artery – Upper 1/3 – Inferior thyroid artery –

vertically orientedvertically oriented Middle 1/3 – Bronchial arteries, direct Middle 1/3 – Bronchial arteries, direct

branches from aorta – transversely oriented.branches from aorta – transversely oriented. Lower 1/3 – Left Gastric & Phrenic arteriesLower 1/3 – Left Gastric & Phrenic arteries..

Embryology:Embryology:Normal development begins at 4rth wk.Normal development begins at 4rth wk.

Median laryngotracheal groove Median laryngotracheal groove

As foregut elongates ridges appear on the As foregut elongates ridges appear on the lateral wall which fuse, starting caudally, to lateral wall which fuse, starting caudally, to separate laryngotracheal tube from separate laryngotracheal tube from oesophagus. oesophagus.

Separation is not complete till 5th wk when Separation is not complete till 5th wk when bifurcation of trachea lies at T4 level. bifurcation of trachea lies at T4 level.

Incomplete separation of foregut from Incomplete separation of foregut from laryngotracheal groove along with laryngotracheal groove along with unbalanced distribution of foregut cell unbalanced distribution of foregut cell material results in TEF.material results in TEF.

The lung buds develop at the caudal end The lung buds develop at the caudal end of primordial trachea. of primordial trachea.

Stomach appears as dilatation of foregut Stomach appears as dilatation of foregut at 5th wk.at 5th wk.

Pathological anatomy Pathological anatomy

Two pouches: Upper & lower.Two pouches: Upper & lower.

Upper pouch varies in length but usually Upper pouch varies in length but usually reaches the arch of the Azygous vein. It is reaches the arch of the Azygous vein. It is thick & hypertrophied.thick & hypertrophied.

Distance between the two pouches could Distance between the two pouches could vary from 0.5 – 6 cms , average 1 cm.vary from 0.5 – 6 cms , average 1 cm.

Classification Classification Vogt & Gross classification:Vogt & Gross classification:

A. A. EA without fistula 6 – 8%EA without fistula 6 – 8% BB.. Upper pouch fistula with lowerUpper pouch fistula with lower pouch atresia 1%pouch atresia 1%

CC.Upper pouch atresia with lower pouch fistula 85%. .Upper pouch atresia with lower pouch fistula 85%. The lower pouch commences from the posterior The lower pouch commences from the posterior Wall of the trachea, 0.5 – 1 cm from the carina. Wall of the trachea, 0.5 – 1 cm from the carina.

DD.. Double esophageal fistulae Double esophageal fistulae E.E. H type esophageal fistula H type esophageal fistula

2 -32 -3%% 3 -5% 3 -5%

FF. Esophageal stenosis. Esophageal stenosis

GG.. Membranous atresia Membranous atresia

Pathophysiology Pathophysiology

Pooling of saliva in the blind upper pouch results Pooling of saliva in the blind upper pouch results in aspiration pneumonia.in aspiration pneumonia.

Escape of air down the fistula into the stomach Escape of air down the fistula into the stomach causes distension & splinting of the diaphragm.causes distension & splinting of the diaphragm.

Gastro esophageal reflux occurs in more than Gastro esophageal reflux occurs in more than 70% of TEF. Results in acid reflux into lungs 70% of TEF. Results in acid reflux into lungs through fistula & chemical pneumonitis.through fistula & chemical pneumonitis.

Membranous part of trachea is wider Membranous part of trachea is wider than normal, hence c shaped cartilage than normal, hence c shaped cartilage which keeps trachea open during which keeps trachea open during expiration is less in circumference. This expiration is less in circumference. This results in partial collapse of tracheal results in partial collapse of tracheal lumen during expiration – lumen during expiration – TracheomalaciaTracheomalacia

Associated anomalies Associated anomalies

VACTERL / VATER associationVACTERL / VATER association : Vertebral : Vertebral AnalAnal CardiacCardiac Tracheo –EsophagealTracheo –Esophageal RenalRenal LimbLimb

CHARGECHARGE : Coloboma : Coloboma Heart disease Heart disease Atresia choanaeAtresia choanae Retarded growthRetarded growth Genital hypoplasiaGenital hypoplasia Ear (deafness)Ear (deafness)

Waterston’s Prognostic Waterston’s Prognostic classification:classification:

GroupGroup Birth Birth weightweight

Pulmonary Pulmonary statusstatus

CongenitalCongenitalanomaliesanomalies

SurvivalSurvival

AA > 2500g> 2500g NoNopneumoniapneumonia

NoNoanomaliesanomalies

100%100%

BB 1800 – 1800 – 2500g2500g

ModerateModeratepneumoniapneumonia

ModerateModerateanomaliesanomalies

85%85%

CC < 1800g< 1800g SevereSeverepneumoniapneumonia

SevereSevereCardiacCardiacanomaliesanomalies

65%65%

Clinical features Clinical features Incidence: 1 per 3000-3500 live birthsIncidence: 1 per 3000-3500 live births

Antenatal USG: Maternal PolyhydramniosAntenatal USG: Maternal Polyhydramnios

Classically newborn with frothing at the mouth inspite Classically newborn with frothing at the mouth inspite of oropharyngeal suction, drooling of saliva, of oropharyngeal suction, drooling of saliva, choking, coughing, dyspnea, cyanosis especially if choking, coughing, dyspnea, cyanosis especially if baby has been fed.baby has been fed.

Firm red rubber tube passed into oropharynx gets Firm red rubber tube passed into oropharynx gets arrested about 10cms from the alveolar ridge arrested about 10cms from the alveolar ridge

DiagnosisDiagnosisX ray with stiff red rubber tube in the esophagus – AP & X ray with stiff red rubber tube in the esophagus – AP & Lateral views. Lateral views.

Soft tubes like NG tubes will be found to coil in the upper Soft tubes like NG tubes will be found to coil in the upper pouch.pouch.

Confirm diagnosisConfirm diagnosis

Presence of air in the abdomen s/o lower pouch fistula. Presence of air in the abdomen s/o lower pouch fistula. Absence s/o pure EA.Absence s/o pure EA.

Level of upper pouch in terms of thoracic vertebrae.Level of upper pouch in terms of thoracic vertebrae.

Assess pulmonary & cardiac status.Assess pulmonary & cardiac status.

Vertebral anomalies.Vertebral anomalies.

Management Management

Routine investigationsRoutine investigations

ECHO: To look for cardiac anomalies & ECHO: To look for cardiac anomalies & side of arch of aortaside of arch of aorta

USG abdomenUSG abdomen

SurgerySurgery

Pre operative management Pre operative management Surgical repair is not an emergency. Necessary to Surgical repair is not an emergency. Necessary to stabilise & evaluate completely.stabilise & evaluate completely.

Continuous or frequent upper pouch suction with Continuous or frequent upper pouch suction with low pressure.low pressure.

Prone or lateral head up positionProne or lateral head up position

Warmer careWarmer care

Supplemental O2Supplemental O2

IV antibiotics.IV antibiotics.

Operative managementOperative managementAim: To achieve complete primary repairAim: To achieve complete primary repair

Anaesthesia: ETGA. Positioning of ET distal to Anaesthesia: ETGA. Positioning of ET distal to the fistula is desirable but is often not possible.the fistula is desirable but is often not possible.

Position: Left lateral positionPosition: Left lateral position

Incision: Transverse incision just below the Incision: Transverse incision just below the angle of the scapula. Divide the Latissimus angle of the scapula. Divide the Latissimus dorsi & Serratus anterior. Fourth intercostal dorsi & Serratus anterior. Fourth intercostal space is identified & opened.space is identified & opened.

Procedure: Extrapleural dissection.Procedure: Extrapleural dissection.

Azygous vein identified & divided.Azygous vein identified & divided.

Lower pouch identified. Fistula divided Lower pouch identified. Fistula divided flush with trachea & tracheal end closed.flush with trachea & tracheal end closed.

Another ET is passes into the oral cavity & Another ET is passes into the oral cavity & oropharynx to identify upper pouch. oropharynx to identify upper pouch.

Single layer end-end anastamosis after Single layer end-end anastamosis after passing NG tube into the stomach across passing NG tube into the stomach across the anastamosis.the anastamosis.

Procedure: Extrapleural dissection.

Post-op Post-op

Electively paralyse & ventilate the child for Electively paralyse & ventilate the child for 24 – 48 hours in slightly flexed position.24 – 48 hours in slightly flexed position.

Tube feeds started after 72 hrs.Tube feeds started after 72 hrs.

Dye study done on day 5 to look for leak.Dye study done on day 5 to look for leak.

Complications Complications

Early:Early:

Anastamotic leakAnastamotic leak

Anastamotic strictureAnastamotic stricture

Recurrent TEFRecurrent TEF

Swallowing incoordinationSwallowing incoordination

AspirationAspiration

Late:Late:

TracheomalaciaTracheomalacia

Gastro esophageal reflux, Barrett's esophagusGastro esophageal reflux, Barrett's esophagus

Motility disorders, dysphagia, bolus impactionMotility disorders, dysphagia, bolus impaction

Asthma, bronchitisAsthma, bronchitis

Scoliosis, chest wall deformitiesScoliosis, chest wall deformities..

Long gap TEFLong gap TEF

If the distance between the two pouches is If the distance between the two pouches is greater than 2 vertebral bodies.greater than 2 vertebral bodies.

At thoracotomy, it is difficult to bring the At thoracotomy, it is difficult to bring the pouches together after fistula ligation pouches together after fistula ligation inspite of extensive mobilisation.inspite of extensive mobilisation.

Options:Options:Primary anastamosis under tensionPrimary anastamosis under tensionFlap / myotomyFlap / myotomy

Cervical esophagostomy & tube Cervical esophagostomy & tube gastrostomy followed by esophageal gastrostomy followed by esophageal replacement.replacement.

Esophageal replacement at 1 year of ageEsophageal replacement at 1 year of age::

Colonic interposition Colonic interposition Gastric tubeGastric tubeGastric pull upGastric pull upJejunal interpositionJejunal interposition

Pure esophgeal atresiaPure esophgeal atresia

All pure atresia are long gap.All pure atresia are long gap.

No need of thoracotomy as there is no No need of thoracotomy as there is no fistulafistula

Proceed with cervical esophagostomy & Proceed with cervical esophagostomy & tube gastrostomy followed by esophageal tube gastrostomy followed by esophageal replacement at 1yr of age.replacement at 1yr of age.

H type fistulaH type fistula

Esophageal continuity is maintained, there is no Esophageal continuity is maintained, there is no atresia. Hence may present in infancy or later.atresia. Hence may present in infancy or later.

70% of fistulae occur at T2 level.70% of fistulae occur at T2 level.

Classical triad of symptoms:Classical triad of symptoms:Coughing/choking/cyanosis after feeds.Coughing/choking/cyanosis after feeds.Recurrent lower respiratory tract infectionsRecurrent lower respiratory tract infectionsGaseous abdominal distensionGaseous abdominal distension

DiagnosisDiagnosis

Plain X ray chest : Air esophagogramPlain X ray chest : Air esophagogram

Catheter test to look for bubbling of air.Catheter test to look for bubbling of air.

Cine-esophagogram: Infant in prone position. Cine-esophagogram: Infant in prone position. NG tube placed in the distal esophagus. NG tube placed in the distal esophagus. Water soluble contrast injected as catheter is Water soluble contrast injected as catheter is

withdrawn slowly under fluoroscopy. withdrawn slowly under fluoroscopy. Dye will be seen to leak into trachea at the site of Dye will be seen to leak into trachea at the site of

fistula.fistula.

Bronchoscopy Bronchoscopy

Management: Management:

Excision of fistula with repair of trachea & Excision of fistula with repair of trachea & esophagus through an oblique right esophagus through an oblique right cervical incision just above the clavicle.cervical incision just above the clavicle.