PowerPoint on tracheosopageal fistula

1. TRACHEOESOPHAGEAL
FISTULA
NWAFORNSO, CHIAZOR I.
NELSON CLUB PRESENTATION.
DEPARTMENT OF PAEDIATRICS.
FEDERAL MEDICAL CENTRE, KEFFI.
14th SEPTEMBER 2015
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2. OUTLINE
• INTRODUCTION
• EMBRYOLGY
• CLASSIFICATION
• EPIDEMIOLOGY
• EMRYOLOGY
• AETIOLOGY/ RISK FACTORS
• CLINICAL FEATURES
• DIFFERNTIAL DIAGNOSIS
• WORKUP
• MANAGEMENT
• COMPLICATION
• PROGNOSIS
• PREVENTION
• CONCLUSION.
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3. 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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4. 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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5. 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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6. 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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7. Embryology - 2
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8. Embryology - 3
• Isolated esophageal atresia can occur
when the esophagus fails to recannalize
by the 8th week.
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9. Pathogenesis
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10. 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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11. Classification - 1
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12. Classification – 2
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13. 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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14. 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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15. Aetiology - 1
• Associated risk factors
• Advanced maternal age
• European ethnicity
• Obesity
• Low socioeconomic status
• Tobacco smoking
• Infants < 1500g ( highest mortality).
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16. 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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17. Aetiology - 3
• Examples : Feingold syndrome(N –MYC),
CHARGE syndrome (chd7), anorectal-
esophageal –genital syndrome(SOX2).
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18. 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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19. 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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20. 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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21. 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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22. 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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23. Plain radiograph – coiled NG
tube, absent gastric bubble.
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24. Plain abdominal radiograpgh –
gaseous stomach distention in
EA + distal fistula.
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25. 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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26. Diagnosis - 4
• Multisector CT scans
• Flexible esophagoscopy
• Flexible bronchoscopy.
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27. DIFFERENTIAL
DIAGNOSIS
DIAGNOSIS CHARACTERISTICS
Laryngotracheoesophageal
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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28. MANAGEMENT
• A multidisciplinary approach involving the
paediatric gastroenterologist,
pulmonologist, and cardiothoracic
surgeon.
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29. 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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30. 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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31. 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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32. 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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33. 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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34. COMPLICATIONS
• Recurrent pneumonia
• Acute lung injury
• Acute RDS
• Lung abscess
• Poor nutrition
• Bronchiectasis
• Respiratory failure
• Death .
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35. 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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36. Follow up
• Very essential
• Evaluation by barium studies.
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37. 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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38. Outcome
• Delay in arriving at diagnosis, late
presentation, associated congenital
anomalies, and co morbidities contribute
to poor outcome.
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39. 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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40. 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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41. 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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42. References .
• The short textbook of paediatrics, by Suraj
Gupte, 11th edition. Chapter 40, page 695
– 696.
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43. • THANK YOU FOR YOUR TIME.
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