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TRACHEOESOPHAGEAL FISTULA

NWAFORNSO, CHIAZOR I.NELSON CLUB PRESENTATION.DEPARTMENT OF PAEDIATRICS.

FEDERAL MEDICAL CENTRE, KEFFI.14th SEPTEMBER 2015

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OUTLINE• INTRODUCTION• EMBRYOLGY• CLASSIFICATION• EPIDEMIOLOGY• EMRYOLOGY• AETIOLOGY/ RISK FACTORS• CLINICAL FEATURES• DIFFERNTIAL DIAGNOSIS• WORKUP• MANAGEMENT• COMPLICATION• PROGNOSIS• PREVENTION• CONCLUSION.

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INTRODUCTION

• Tracheoesophageal fistula(TEF) is a congenital or acquired communication between the trachea and esophagus.

• Esophageal atresia(EA) occurs in association with TEF in about 90% of instances.

• TEF most likely leads to fatal pulmonary complications.

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Introduction

• Most congenital TEFs are diagnosed immediately following birth or in infancy.

• They are commonly associated with other congenital anomalies.

• Acquired TEF occur secondary to malignant disease, infection,, trauma, etc.

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EMBRYOLOGY

• The esophagus and trachea both develop from the primitive foregut.

• During 4 – 6 weeks of life the caudal part of the foregut forms the ventral diverticular which gives rise to the trachea.

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Embryology - 1

• The longitudinal tracheoesophageal fold fuses to form a septum, dividing the foregut into a ventral laryngotracheal tube and a dorsal esophagus.

• Posterior deviation of the septum causes incomplete separation of the esophagus from the laryngo tracheal tube resulting in TEF.

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Embryology - 2

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Embryology - 3

• Isolated esophageal atresia can occur when the esophagus fails to recannalize by the 8th week.

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Pathogenesis

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Classification of Tracheoesophageal fistula

Anatomic characteristics Percentage of cases Esophageal atresia with distal TEF 87%

Isolated esophageal atresia without TEF

8%

Isolated TEF (H type). 4%

Esophageal atresia with proximal TEF 1%

Esophageal atresia with proximal and distal TEF

1%

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Classification - 1

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Classification – 2

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Epidemiology

• Common congenital anomaly with an incidence of 1/2000- 4000 live births.

• Acquired TEF is rare.• No racial predilection• Commonly observed in neonates and

during the first year of life.• No data recorded in FMC Keffi from 2013

till date.

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AETIOLOGY

• Exact aetiology of TEF is unclear.• Embryologically believed to be due to

incomplete closure of laryngotracheal groove.

• Observed in mothers that used imidazole containing decongestants in the first trimester of pregnancy.

• Association with trisomies 18, 21, 13.

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Aetiology - 1

• Associated risk factors• Advanced maternal age• European ethnicity• Obesity• Low socioeconomic status• Tobacco smoking• Infants < 1500g ( highest mortality).

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Aetiology - 2

• Approximately 50% affected infants have associated anomalies.

• Syndromes include VATER/VACTERL( vertebral, anorectal, cardiac, tracheal, esophageal, renal, radial).

• Some genetic factors have been implicated with discrete mutations in syndromic cases.

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Aetiology - 3

• Examples : Feingold syndrome(N –MYC), CHARGE syndrome (chd7), anorectal- esophageal –genital syndrome(SOX2).

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CLINICAL FEATURES

• Approximately 80 -90% of affected infants have respiratory decompensation within the first hour of life.

• Neonates present with frothing, bubbling at the mouth and nose after birth.

• Episodes of coughing, cyanosis and respiratory distress.

• Feeding exacerbates symptoms.

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Clinical features - 2

• About 10-20% of older children present late in life with chronic respiratory problems – refractory bronchospasm, recurrent pneumonia.

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Clinical features - 3

• Clinically they have the following features.• Funnel shaped chest• Mediastinal shift• Absent breath sounds• Presence of peristaltic sound on affected

side.• Displaced heart sounds• Scaphoid abdomen

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DIAGNOSIS

• Prenatal diagnosis• Maternal ultrasound after 14 weeks G.A-

polyhydramnios, absence of fluid filled stomach, small abdomen, distended esophageal pouch.

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Diagnosis - 2

• Post natal diagnosis.• Plain chest radiograph tracheal compression,Absence of gastric bubble.(EA + proximal

TEF, isolated EA).Aspiration pneumonia opacity in the

posterior segment of upper lung zone.Insertion of NG tube-coiling in the

mediastinum(associated TEF).

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Plain radiograph – coiled NG tube, absent gastric bubble.

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Plain abdominal radiograpgh – gaseous stomach distention in

EA + distal fistula.

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Diagnosis - 3

• Contrast studies. 1 – 2 mls of barium instilled in the esophagus, and a plain lateral decubitus chest radiograph shows spilling of content in lungs.

• N.B rarely done due to risk of aspiration and pulmonary injury.

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Diagnosis - 4

• Multisector CT scans• Flexible esophagoscopy• Flexible bronchoscopy.

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DIFFERENTIAL DIAGNOSIS

DIAGNOSIS CHARACTERISTICSLaryngotracheoesophageal cleft

Absent stomach bubble, aspiration after feeds, associated with TEF.

Esophageal web/rings Dysphagia, recurrent vomiting, aspiration in later life.

Esophageal stricture. Feature similar to esophageal rings.

Esophageal diverticulum Dysphagia, chest pain, aspiration pneumonia, present in later life.

Tracheal atresia Severe respiratory distress, cyanosis, failure to ventilate despite tracheal intubation.

Congenital short esophagus Abnormally short esophagus, intrathoracic part of stomach. Vomitting, gastroesophageal reflux.

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MANAGEMENT

• A multidisciplinary approach involving the paediatric gastroenterologist, pulmonologist, and cardiothoracic surgeon.

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Management - 1

• Stabilization of the patient.• Maintain a patent airway• Prevent aspiration of secretions• Prone positioning of patient to prevent

secretion from a distal fistula.• Esophageal suctioning to minimize

aspiration from a blind pouch.

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Management - 2

• In healthy infants without pulmonary complications, primary repair done in first few days of life.

• Delay is done in low birth weight infants, presence of pulmonary infection, or other anomalies.

• Conservative management- parenteral nutrition, gastrostomy,upper pouch suctioning.

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Management - 3

• Broad spectrum antibiotics are also commenced for lower respiratory tract infection.

• Forgarty balloon catheter may be used in patients with RDS to obliterate the TEF while awaiting surgery.

• Tracheostomy is considered only if a staged repair is planned.

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Management - 4

• Surgical repair involves surgical ligation of TEF and end to end anastomosis of esophagus.

• If the gap between the atretic ends is> 3-4 cm, a neoesophagus is created.

• Thoracoscopic surgical repair is now feasible with good outcome.

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Management - 5

• Feeding • After surgery baby is placed on I.v fluids

for 48 hours.• Feeding through gastrotomy tube

commenced on 3rd day post operatively.• Oral feeding commenced on the 10th day if

general condition is stable.

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COMPLICATIONS

• Recurrent pneumonia• Acute lung injury• Acute RDS• Lung abscess• Poor nutrition• Bronchiectasis• Respiratory failure• Death .

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Complications - 2

• Post op complications:• Tracheal stenosis• Recurrent fistula.o Others • Abnormal esophageal motility• Hiatal hernia• Barret esophagus• Gastro esophageal reflux disease

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Follow up

• Very essential• Evaluation by barium studies.

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OUTCOME

• Survival rate in healthy infants undergoing surgery may be >100%, and 80 – 95% in unfit patients.

• In a study done at UNTH Enugu by Nwosu and Onyekwulu, a total of 10 cases were seen over a 12 year period with only one survival after surgery.

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Outcome

• Delay in arriving at diagnosis, late presentation, associated congenital anomalies, and co morbidities contribute to poor outcome.

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Waterston prognostic classification.

GROUP SURVIVAL(%) WATERSTON CLASSIFICATION A 100% Birth weight > 2500g, well child

B1 85% Birth weight 1800 - <2500g, well child

B2 Higher weight with moderate associated anomalies.

C1 65% Birth weight < 1800g

C2 Birth weight < 1800g, severe anomalies.

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Conclusion

• Tracheoesophageal fistula is a common congenital anomaly with fatal lung complications if not managed on time.

• It is a surgical emergency.• Early presentation and diagnosis helps

improve survival rate and outcome

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Refernces

• Langfield embryology, by Sadler, 5th edition. Chapter 13, pages 290 – 293.

• Nelson textbook of paediatrics, by R. Behrman et al, 19th edition, pages 4648 – 4650.

• Nigerian journal of medicine. ISSN 1115- 2613. volume 22. Number 4, October – December 2013.page 295-297.

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References .

• The short textbook of paediatrics, by Suraj Gupte, 11th edition. Chapter 40, page 695 – 696.

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• THANK YOU FOR YOUR TIME.