‫بسم اهلل الرحمن الرحيم‬

OA/TEF... ALHADA HOSP. NICU

SENIOR REGISTRAR LECTURE
EOSOPHAGEAL ATRESIA &
TRACHEO- EOSOPHAGEAL
FISTULA
BY

Dr. AbdelFattah Mujahed

SENIOR REGISTRAR in NICU

1/1/1432 - 7/12/2010

OA/TEF... ALHADA HOSP. NICU

 Disease Name and Synonyms

• Oesophageal Atresia with or without

Tracheoe-oesophageal fistula,
• OA, TOF (English)
• TEF (American)

OA/TEF... ALHADA HOSP. NICU

 Epidemiology
• Relatively common.
• One in 2500–3000 live births/ slight male
predominance
• majority of cases of are sporadic/non-syndromic
• a small number with chromosomal abnormalities.
• Familial/syndromic cases are extremely rare,
˂ 1% of the total.
• OA is 2 to 3 times more common in twins

Orford J, Glasson M, Beasley S, Shi E, Myers N, Cass D: Oesophageal atresia in twins. Pediatr Surg Int 2000,16(8):541-
545

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 HISTORICAL BACKGROUND
• The first documented case of OA in conjoined
twin girls was written by William Durston in
1670
• The first account of OA with a TEF was written
by Thomas Gibson in 1697.

Myers, N.A. 1991 The early history of oesophageal atresia and tracheoesophageal fistula. In: Oesophageal Atresia. S.W.
Bea- sley, N.A.Myers, and A.W. Auldist, eds. Chapman & HallMedical, London, pp. 1–16.

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"About November 1696, I was sent for to
an infant that would not swallow. The
baby seemed very desirous of food, and
took what was offered in a spoon with
greediness; but when it went to swallow
it, it was liked to be choked, and what
should have gone down returned by the
mouth and nose, and it fell into a
struggling convulsive sort of fit upon it
but the next day died“
Gibson T: The anatomy of humane bodies epitomized 5th edition. Edited by: Awnsham , Churchill J. London ; 1697.

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• The next recorded case was almost 150 years
later by Thomas Hill in 1840

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• “ was called, in the night,. "The newborn
infant made no effort to swallow but
immediately convulsed and the drink
which had been given returned by mouth
and nose, mixed with bloody mucus".
• He recommended that "gently stimulating
the rectum would remove the difficulty",
however, when an attempt was made to do
so, there was "no vestige of an anus".
• Hill was the first to document an associated anomaly with OA.
Hill TP: Congenital malformation. Boston Med Surg J 1840,21:320-321.

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• In 1939 and 1940, Ladd of Boston and
Leven of Minnesota first achieved surgical
success in stages.

Leven NL: Congenital atresia of the esophagus with tracheoesophageal fistula. J Thorac Cardiovasc Surg 1941, 10:648-
657.
Ladd WE: The surgical treatment of esophageal atresia andtracheoesophageal fistulas. N Engl J Med 1944, 230:625-637.

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Dr. Leven adopts his patient

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• In 1941 Cameron Haight (THE PIONEER)
performed the first successful primary repair.
His patient was an infant girl, 12 days old on
admission who had been transported over a
distance of 500 miles to Ann Arbor, Michigan.

Haight C, Towsley H: Congenital atresia of the esophagus with tracheoesophageal fistula: extrapleural ligation
of fistula and end-to end anastomosis of esophageal segments. Surg Gynecol Obstet 1943, 76:672-688.

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 Aetiology
.
The aetiology of
oesophageal atresia is
likely to be multifactorial
and remains unknown
OA/TEF... ALHADA HOSP. NICU
 Aetiology
• Multifactoral
• Absent Sonic Hedgehog (Shh) Signaling Pathway
• Defective FGF Signaling pathway
• Environmental:
-Contraceptives ?
-Methimazole ?
Spilde, T, et al. Fibroblast Growth Factor Signaling in the Developing Tracheoesophageal Fistula.
Journal of Pediatric Surgery, Vol 38, No 3 (March), 2003: pp 474-477
Spilde, T., et al. A Role for Sonic Hedgehog Signaling in the Pathogenesis of
HumanTracheoesophageal Fistula. Journal of Pediatric Surgery, Vol 38, No 3 (March), 2003:
pp 465-468

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fold2.mov

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OA/TEF... ALHADA HOSP. NICU
 classification
• Vogt in 1929
• Ladd (1944) and Gross (1953) modified the
classification
• Kluth (1976) published an extensive "Atlas of
Esophageal Atresia" which comprised 10
major types, each with numerous subtypes
which is based on the original Vogt
classification

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TEF? EA? Description Vogt Gross

Esophageal agenesis. Very rare, and not included in the classification

No Yes Type 1 -
by Gross.

Proximal and distal esophageal bud--a normal esophagus with a

No Yes Type 2 Type A
missing mid-segment.

Proximal esophageal termination on the lower trachea with distal

Yes Yes Type 3A Type B
esophageal bud.

Proximal esophageal atresia (esophagus continuous with the mouth

ending in a blind loop superior to the sternal angle) with a distal
Yes Yes Type 3B Type C
esophagus arising from the lower trachea or carina. (Most common,
up to 90% of cases.)

Proximal esophageal termination on the lower trachea or carina with

Yes Yes Type 3C Type D
distal esophagus arising from the carina.

A variant of type D: if the two segments of esophagus communicate,

Type E (or
Yes No this is sometimes termed an H-type fistula due to its resemblance to -
Type H)
the letter H. TEF without EA.
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OA/TEF... ALHADA HOSP. NICU
interactive3.swf

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OA/TEF... ALHADA HOSP. NICU
 Associations
• Over 50% of infants with OA have one or more
additional anomalies:
(incidence of associated anomalies 65% with pure Artesia
and only 10 % with H-type Fistula)
• Cardiovascular 29%
• Anorectal anomalies 14%
• Genitourinary 14%
• Gastrointestinal 13%
• Vertebral/skeletal 10%
• Respiratory 6%
• Genetic 4%
• Other 11%
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 VATER association
• The first described by Quan and Smith in 1973
consists of a combination of anomalies including
• Vertebral
• Anorectal
• Tracheooesophageal
• Renal or Radial abnormalities.
• This association was later expanded as the
VACTERL association to include Cardiac and Limb
defects
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OA/TEF... ALHADA HOSP. NICU
 CHARGE association
• Coloboma
• Heart defects
• Atresia choanae
• Retarded growth and development
• Genital hypoplasia
• Ear deformities

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 POTTER'S Sequence

• Renal agenesis,
• Pulmonary hypoplasia
• Typical dysmorphic facies

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 SCHISIS association
• Omphalocoele
• cleft lip and/or palate
• genital hypoplasia

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 cardiac anomalies

• VSD,PDA, ASD, single umbilical artery, Rt sided

Aortic arch
• Tetralogy of Fallot.
• Major cardiac malformations are one of the
main causes of mortality in infants with OA

OA/TEF... ALHADA HOSP. NICU

 The vertebral anomalies
• Mainly confined to the thoracic region and are
responsible for later development of scoliosis.
• Hemivertebrae, radial dysplasia, polydactyly,
syndactyly, rib malformations, scoliosis, lower
limb defects
• The claim that the presence of 13 ribs is
associated with long-gap atresia has not been
substantiated.

OA/TEF... ALHADA HOSP. NICU

 gastrointestinal anomalies

frequently encountered are:

• Duodenal atresia
• Malrotation
• Pyloric stenosis.

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 Miscellaneous anomalies

• cleft lip and palate

• omphalocoele
• lung abnormalities
• choanal atresia
• hypospadias.

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 Clinical description and diagnosis
• Delay in diagnosis until the infant presents
with coughing and choking during the first
feed is NO LONGER ACCEPTED in modern
neonatal practice.
• The only exception is type E (H or N fistula)

Lewis Spitz: Oesophageal atresia, Orphanet Journal of Rare Diseases 2007, 2:24

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• Prenatal diagnosis
• High index of suspicion
• Early intervention ?

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 Prenatal diagnosis
US
The first sign of esophageal atresia in
the fetus may be polyhydramnios
• small or absent fetal stomach after the 18th week
of gestation.
• Overall the sensitivity of US is 42%
• But in combination with polyhydramnios the
positive predictive value is 56%
Stringer MD, McKenna KM, Goldstein RB, Filly RA, Adzick NS, Harrison MR: Prenatal diagnosis of esophageal atresia. J
Pediatr Surg 1995, 30(9):1258-1263

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 Methods to improve the Prenatal
Diagnostic Rate
• US examination of the fetal neck to view the
blind-ending upper pouch and to observe fetal
swallowing
• MRI: 100% sensitivity

Shulman A, Mazkereth R, Zalel Y, Kuint J, Lipitz S, Avigad I, Achiron R: Prenatal identification of esophageal atresia: the
role of ultrasonography for evaluation of functional anatomy. Prenat Diagn 2002, 22(8):669-674.
Langer JC, Hussain H, Khan A, Minkes RK, Gray D, Siegel M, Ryan G: Prenatal diagnosis of esophageal atresia using
sonography and magnetic resonance imaging. J Pediatr Surg 2001, 36(5):804-807.

OA/TEF... ALHADA HOSP. NICU

 US
• polyhydramnios
• Absent or small stomach
• Blind dilated eosophageal pouch
• Defective swallowing movement
+

MRI
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 Pathophysiology
MUSCULAR↓ DYSMOTILITY

ESOPHAGUS
INNERVATION↓
GERD

C →U

I TRACHEA
TRANSVERSE↑
TRACHEOMALACEA

MS

Cheng W, Bishop AE, Spitz L, Polak JM: Abnormal enteric nerve morphology in atretic esophagus of fetal rats with adriamycin-induced
esophageal atresia. Pediatr Surg Int 1999, 15(1):8-10.
Cheng W, Bishop AE, Spitz L, Polak JM: Abnormalities of neuropeptides and neural markers in the esophagus of fetal rats with
adriamycin-induced esophageal atresia. J Pediatr Surg 1997, 32(10):1420-1423.
Wailoo MP, Emery JL: The trachea in children with tracheoesophageal fistula. Histopathology 1979, 3(4):329-338.

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 Presentation in neonatal period
• Scaphoid abdomen
• Excessive frothy secretions
• Failure to pass naso gastric tube more
than 10-12 cm (6-10 cm)
• Milk regurgitation, shocking &cyanosis
• Gasless abdomen in x ray

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 High index of suspicion
• History of polyhydramnios:
33% with TEF and 100% with OA
Pass a nasogastric tube and request Xray
• Excessive frothy secretion: to exclude OA
• Aspiration pneumonia in newborn or recurrent:
TEF should be excluded
• Presence of an association
Syndromes: esp. Trismy 21 ,18, 13q del,Digeorge
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OA/TEF... ALHADA HOSP. NICU
OA/TEF... ALHADA HOSP. NICU
 Plain Radiography
Confirmatory test
OGT in esophageal pouch
Presence or absence of gas in the abdomen
Atelectasis: Pneumonitis
Anomalies:
• „ Cardiac
• GIT
• „ Vertebral
• „ Aortic arch

OA/TEF... ALHADA HOSP. NICU

 Does gasless
abdomen exclude
distal TEF
?
OA/TEF... ALHADA HOSP. NICU
The Internet Journal of Pediatrics and Neonatology 2009 : Volume 9 Number 2

Esophageal atresia with distal

tracheoesophageal fistula
with gasless abdomen: a
diagnostic dilemma
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OA/TEF... ALHADA HOSP. NICU
The Internet Journal of Pediatrics and Neonatology 2009 : Volume 9 Number 2

14% of babies with a gasless

abdomen were found to
have a distal TEF
OA/TEF... ALHADA HOSP. NICU
The Internet Journal of Pediatrics and Neonatology 2009 : Volume 9 Number 2

the absence of gas in the

stomach on x-ray does not
necessarily mean the absence
of a distal fistula, because some
time fistula is very narrow and
may be blocked by mucous plug
OA/TEF... ALHADA HOSP. NICU
The Internet Journal of Pediatrics and Neonatology 2009 : Volume 9 Number 2

We suggest that a repeat x ray chest

should be done in all cases of pure
esophageal atresia after proper
resuscitation to rule out patent
distal tracheoesophageal fistula.
OA/TEF... ALHADA HOSP. NICU
• OA + abdominal air in X-ray conclusive for the
presence of distal TEF
• BUT
• THE REVERSE IS NOT TRUE:
• MUCUS PLUG OBLITERATING THE FISTULA
• VERY NARROW FISTULE
• MREMBRANE OCCLUDING FISTULA

OA/TEF... ALHADA HOSP. NICU

• Goh et al recommended that all children with
TEF and a “gasless abdomen” should have a
bronchoscopy to look for a distal fistula and to
diagnose associated proximal fistulas

• L. Spitz, Esophageal atresia and tracheoesophageal malformations.

In: K.W. Ashcraft, G.W. Holcomb III and J.P. Murphy, Editors,
Pediatric surgery (4th ed), Elsevier Saunders, Philadelphia; 2005:
pp. 352-370.

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ophageal atre
.flv

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Pediatric Anesthesia Volume 16 Issue 4, Pages 479 - 483 Published Online:
14 Mar 2006

An unknown second
tracheoesophageal fistula: a rare
cause of postoperative
respiratory failure
OA/TEF... ALHADA HOSP. NICU
 Pre operative care
• NPO
• Head up
• Replogle tube
• Continuous suction 20-30 mmHg
• ETT distal to fistula & bevel anterior
• If ventilated: low pip

NB.
• Confirm diagnosis
• Echo to exclude association (aortic arch position)
• Tracheobronchoscopy to exclude any missed fistula

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Replogle tube

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OA/TEF... ALHADA HOSP. NICU
EMERGENCY ligation of distal TEF

IS CORRECTION OF OA/
TEF AN EMERGENCY
PROCEDURE
???
OA/TEF... ALHADA HOSP. NICU
 EMERGENCY ligation of distal TEF
• Generally, the operative correction
of an OA is not regarded as an
emergency procedure
• The only exception is preterm baby
with RDS and on mechanical
ventilation
Jones TB, Kirchner SG, Lee FA, Heller RM: Stomach rupture associated with esophageal atresia, tracheoesophageal
fistula, and ventilatory assistance. AJR Am J Roentgenol 1980, 134(4):675-677.

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 SUGGESTED PROCEDURES
• EMERGENCY GASTROSTOMY →deterioration
• ETT placement distal to the fistula→ carina?
• FOGERTY CATHETER→ difficult

Filston HC, Chitwood WR Jr., Schkolne B, Blackmon LR: The Fogarty balloon catheter as an aid to management
of the infant with esophageal atresia and tracheoesophageal fistula complicated by severe RDS or
pneumonia. J Pediatr Surg 1982, 17(2):149-151
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 Emergency ligation
• emergency transpleural ligation of the fistula
as the procedure of choice.
• The aim should be to re-operate in 8–10 days
to divide the fistula and repair the OA

Holmes SJK, Kiely EM, Spitz L: Tracheo-oesophaeal fistula and the respiratory distress syndrome. Pediatr surg int 1987,
2:16-18.
Malone PS, Kiely EM, Brain AJ, Spitz L, Brereton RJ: Tracheo-oesophageal fistula and pre-operative mechanical
ventilation. Aust N Z J Surg 1990, 60(7):525-527.

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 Post operative
• IV Fluids: D 10% ¼NS or D 10% ½NS
• Replacement: ml/ml
• S.Electrolytes monitoring
• Low PIP if ventilated
• Management of early complications if any.

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 Complications
:Early
• Anastomotic leakage: 15-20%
• Anastomotic stricture:30–40%
• Recurrent fistula: 5–14%
:Late
• Gastrooesophageal reflux.
• Tracheomalacia.
• Dysmotility .
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 Recurrent Fistula
Should be suspected if the infant manifests
• coughing during feeds, apnoeic or cyanotic
episodes
• recurrent respiratory infections after
"successful“ repair of the OA.

• Urgent investigations to exclude the

possibility of a recurrent fistula.
Ghandour KE, Spitz L, Brereton RJ, Kiely EM: Recurrent tracheo-oesophageal fistula: experience with 24 patients. J
Paediatr Child Health 1990, 26(2):89-91.

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 Risk
categorization
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 Prognosis scores
Waterston 1962
Group A: ˃2500 g birth weight and well
Group B:
• 1. 1800–2500 g and well
• 2. Higher birthweight, moderate pneumonia and congenital
anomaly.
Group C:
• 1. Birth weight ˂1800 g
• 2. Higher birth weight and severe pneumonia and severe congenital
anomaly.
• Group A: 99% survived
• Group B: 95% survived
• Group C: 71% survived

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 Prognosis scores
Spitz 1992
Group I: Birth weight ˃ 1500 g with no major
cardiac anomaly.
Group II: Birth weight ˂1500 g or major cardiac
anomaly
Group III: Birth weight ˂ 1500 g PLUS major
cardiac anomaly.
• Group I: 98% survived
• Group II: 82% survived
• Group III: 50% survived
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 Selection for non-treatment:
• Potter's syndrome (bilateral renal agenesis)
• trisomy 18
• infants with totally uncorrectable major
cardiac defects
• with Grade IV intraventricular haemorrhage

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 Genetic counseling

• majority of cases are sporadic/non-syndromic

• The overall risk of OA recurrence in a sibling of
an affected child is about 1%.

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OA/TEF... ALHADA HOSP. NICU