1) Esophageal atresia and tracheoesophageal fistula is a congenital anomaly caused by incomplete separation of the esophagus and trachea during embryonic development. 2) Patients typically present with excessive salivation, choking or coughing during feeding. Investigations include radiographs and endoscopy to classify the anomaly and assess for associated conditions. 3) Treatment involves surgical closure of any fistula and reanastomosis of the esophageal segments. Patients are risk stratified based on birth weight and cardiac anomalies to predict outcomes, with higher risk groups having staged or palliative procedures.

1. OESOPHAGEAL ATRESIA AND
TRACHEOESOPHAGEAL FISTULA
PEADIATRIC SURGERY UNIT,
DEPT OF SURGERY.
UMTH

2. Learning objectives
• Discuss predictors of poor outcome and long
term morbidity of EA

3. Outline
• Introduction
• Case summary
• Embryology/aetiology
• Classification
• Clinical Features
• Investigations
• Risk categorisation
• Treatment
• Complications
• Outcome
• Recent advances
• Summary

4. Introduction
• Epitome of Paediatric Surgery
“To anastomose the ends of an infant’s esophagus the
Surgeon must be as delicate and precise as a skilled
watchmaker. No operation offers a greater
opportunity for pure technical artistry.” Willis Pott, 1950
• Overall Improvement in survival with
focus on complications

5. Introduction
• Significant challenge in modern paediatric
surgery (urgency to make timely and appropriate
diagnosis)
• Survival is likely, unless NB belong to a specific
risk group
• Improvement in neonatal care, have contributed
to the survival of these high risk patients

6. History
• 1935 pre-survival period
• Donovan 1935- first survivor of isolated EA
– Initial gastrostomy, esophageal replacement 15yr by Humpries
• Haight and Towley 1943
• First successful primary anastomosis

7. History
• Watersone 1964
– Risk groups based on weight, pneumonia &
congenital anomaly
• Spitz 1994
– Revised at risk groups for the 1990s
currently been used

8. Epidemiology
• Incidence – 1:3500-4500 live birth
• Varies geographically
• Incidence high
– Finland 1:2440, Europe 2.82/10,000, whites >60%, 1st
pregnancy, ↑maternal age 35-45yrs, in vitro fertilization,
twin gestation
• Slight male preponderance

9. CASE PRESENTATION
• BHH
• 2hr old term male neonate.

10. PRESENTING COMPLAINT
• Excessive salivation since birth
• Absent anal opening since birth

11. HxPC
• Product of a term pregnancy, delivered to a
28yr old P8 +0 A7
• Noticed to have excessive salivation since
birth.
• Associated hx of choking on feeding
• Cough and difficulty in breathing,
• No hx suggestive of cyanotic spell
• No vomiting following feeding

12. • Noticed absent anal opening since birth
• No associated hx of abdominal distention, passage of
feacal material in urine, or through other opening
around the perineum.

13. • Pregnancy was booked at four month
gestation.
• Regular with follow up visit and antenatal
medications
• No hx suggestive of illicit drug use during
pregnancy

14. • There was positive hx of polyhydramnious
from mother, which was also picked on abd
uss.
• No hx of maternal febrile illness, rashes,
jaundice or foul smell vaginal discharge
• No hx of exposure to radiation

15. • Mother not a known diabetic or hypertensive
pt
• Delivery was via SVD,
• Child cried immediately after birth

16. SOCIAL HX
• 7th child ,in a monogamous non sanguineous
setting,
• Lost elder sibling in her 4 months, said to be
due to congenital hydronephrosis. Nil family
hx of similar disease.
• Mother is a FTHW with no formal education.
Father is a civil servant.

17. O/E
• A calm neonate, pink, with excessive
salivation, no dysmorphic facae, afebrile, not
pale, acyanosed, anicteric, not dehydrated, no
peripheral lymph node enlargement or pedal
oedema.
• Birth wt: 2.9kg OFC: 34cm
• Lt: 49cm HR: 152bpm
• RR:40cpm

18. Abdomen
• Full, moves with resp. with visible peristalsis.
Liver enlarged by 2cm, Spleen not enlarged,
Kidneys no ballotable, BS present and hyper
active.
• Normal male external genitalia
• Absent anal opening
• Well developed gluteal fold

19. • Resp: clear lung fields, BVBS
• CVS: HS 1 and 2 no murmur
• CNS: conscious, normal skull with
hyperpigmented patch on the sacral area, no
defect on the spine, Complete moro, good
grasp, normal tone.
• MSS: Upper and lower limbs appeared grossly
normal.

20. DIAGNOSIS
EOSOPHAGEAL ATRESIA +
TRACHEOESOPHAGEAL FISTULA and ANORECTAL
AGENESIS WITHOUT FISTULA.

21. INVESTIGATIONS
• Babygram confirm arrest of nasogastric tube
about 10cm from the lower incisor.
• Plain Abd X-ray result showed gas in the
stomach.

22. • PCV: 0.68
• RBC: 6.6mmol/l
• E/U/C : azotemia of 8.0. Creatinine not
retrieved other parameters essentially within
normal limit
• Total bilirubin: 20.3mg/dl
• Conj. bilirubin: 11.2mg/dl, 56% conjugated.

23. • Operations:
– Closure of fistula
– Esophagoesophagostomy
• Intra op findings were:
– Proximal blind pouch
– Distal tracheoesophageal fistula

24. EMBRYOLOGY
• The embryology of the foregut is still subject
to controversy.
• Development of the digestive system begins at
4th week during folding
• Endoderm gives rise to most of its epithelium
& glands
• The cranial end is ectodermal,namely the
stomodeum.

26. • During the fourth week of gestation the
foregut starts to differentiate into a ventral
respiratory part and a dorsal esophageal part.
• The laryngotracheal diverticulum then
evaginates ventrally into the mesenchyme.

27. • The ventral respiratory system separates from
the esophagus by the formation of lateral
tracheoesophageal folds that fuse in the midline
and create the tracheoesophageal septum.
• At 6 to 7 weeks of gestation, the separation is
complete.
• Incomplete fusion of the folds would result in a
defective tracheoesophageal septum and
abnormal connection between the trachea and
esophagus.

28. • This theory has been challenged.
• In chicken embryo studies, these folds could
not be demonstrated.
• Instead, cranial and caudal folds were found
in the region of tracheoesophageal
separation.
• According to this theory, EA/TEF would then
be due to an imbalance in the growth of these
folds

29. The Adriamycin (doxorubicin) rat model
• It has greatly helped with understanding the
development of EA/TEF.
• From this model, it appears the EA develops first,
with the lung bud arising from the atretic foregut
dividing into three rather than two branches.
• The middle branch would be the distal
esophagus eventually connecting with the
stomach.

30. AETIOLOGY
• The pathogenesis of Eosophageal atresial
malformations remains unknown.
• Many theories proposed to explain EA, EA-TEF,
TEF, no single unifying theory has been
proposed that addresses all the variations
seen with this group of anomalies.

31. • Most EA occur sporadically, highly unlikely
that simple, inheritable genetic mechanism is
responsible.
• Most likely heterogenous and multifactorial,
and involves multiple genes and complex
gene-enviroment interactions

32. • Environmental factors implicated:
– use of methimazole in early pregnancy
– prolonged use of contraceptive pills
– maternal diabetes and thalidomide exposure.
– EA is occasionally seen in the fetal alcohol
syndrome and
– in maternal phenylketonuria

33. • Also associated with of trisomy 18 and trisomy 21.
• Trisomy 18 carries is a greater risk for EA development.
• Three separate genes have been associated with EA/TEF:
– MYCN haploinsufficiency in Feingold syndrome,
– CHD7 in CHARGE syndrome, and
– SOX2 in the anophthalmia-esophageal-genital (AEG) syndrome.
• EA may occasionally be part of the
– Opitz G/BB syndrome,
– Fanconi anemia,
– oculo-auriculo-vertebral syndrome

34. CLASSIFICATION
• E.C. Vogt a radiologist in 1932 classified the
anomalies anatomically into:
• Type 1: absent eosophagus
• Type 2: EA without TEF
• Type 3: EA with TEF
– a: EA with Proximal TEF
– b; EA with Distal TEF
– c: EA with proximal and distal TEF
• Type 4: isolated TEF with intact eosophagus.

35. TYPE % GROSS TYPE
1. EA with distal TEF 85.8 C
2. EA without TEF 7.8 A
3. TEF without EA 4.2 E
4. EA with TEF to both pouches 1.4 D
5. EA with proximal TEF 0.8 B

36. CLASSICATION: PROGNOSTIC
GROUP SURVIVAL WATERSON CLASSIFICATION
A 100% Birth weight >2.5kg and otherwise healthy
B 85% Birth weight 2-2.5kg and well or higher weight with
moderate associated anomalies (non-cardiac plus
PDA, VSD or ASD).
C 65% Birth weight <2kg or higher with severe assocaited
cardiac anomalies.
Waterston’s 1962 classification seperated patients into
groups based on birth weight, pneumonia and congenital
anomalies.
Group A were treated with immediate repair
Group B “ “ “ delayed repair
Group C “ “ “ staged repair

37. CLASSIFICATION: PROGNOSTIC
GROUP SURVIVAL
I. Birth weight >1.5kg without major CHD 97%
II. Birthweight <1.5kg or major CHD 59%
III. Birth weight <1.5kg and major CHD 22%
SPITZ Classification – Most commonly used
currently.

38. Diagnosis
• Prenatal
• Not common-18/52
• USS- 20%-40% yield
– Small stomach
– Absent stomach bubble
– ployhydramnios

39. Diagnosis
• Excessive
salivation

40. Diagnosis
• Regurgitation at feed
• Choking, coughing
• Cyanosis with or without feeding
• Abdominal distension-distal fistula
– Scaphoid abdomen-isolated EA

41. • Inability to pass a NGT
• Look for associated anomalies –(20%-70%)
• Incidence is high with isolated EA
– VACTERL/CHARGE associations
– SCHISIS malformations

42. Associated malformations
• VACTERL—H
• V vertebra
• A anorectal
• C cardiac
• T tracheo-
• E esophageal
• R renal
• L limb
• H hydrocephalus

43. Associated malformations
• CHARGE associations
• C coloboma of eye
• H heart
• A anorectal
• R renal
• G hypoGonadism
• E deafness (Ear)

44. Associated Malformations
• Systems affected are as follows:
• Cardiovascular – 29%
• Anorectal – 14%
• GUS – 14%
• GIT – 13%
• Vertebral / Skeletal – 10%
• Respiratory – 6%
• Genetic – 4%
• Others - 11%
• Ein et al 1989

45. Investigations
• Oropharyngeal tube met resistance ≈10cm
from the lower incisor
• Limited contrast study -0.5-1ml of diluted
barium into the upper pouch

46. Investigations
• Air in the stomach or
bowel suggestive of
distal TEF

47. Investigations
• Absent gas in the
abdomen reflects
isolated EA

48. Investigations
• EA and TEF with
duodenal atresia. NGT
coiled in upper pouch.

49. Investigations
• Upper pouch outlined
by barium

50. Investigations
• Broncoscopy-fistula
proximal to the carina

51. Investigations
• Echocardiography-
– roll out 1.8-2.5% right sided aortic arch
– Cardiac anomalies
• Renal USS
• Chromosomal analysis

52. Investigations
• TEF without EA
– High index of suspicion
– Delayed
– Coughing/ choking during feeding
– Recurrent aspiration
– Use video esophagography
– broncoscopy

53. Risk Categorisation
Waterstone - 1962
Gp A
bwt ≥ 2.5 kg and well (95%)
Gp B
Bwt - 1.8 – 2.5 Kg and well or
- ≥2.5 Kg with moderate
pneumonia and birth defects (85%)
Group C
Bwt ≤ 1.5 Kg or
Any weight with severe pneumonia
and associated malformations (65%)
Spitz - 1994
• Gp I -Bwt ≤ 1.5 Kg no major
cardiac anomaly (97%)
• Gp II - ‹ 1.5kg or major
cardiac anomaly (59%)
• Gp III - ≤ 1.5Kg and major
cardiac anomaly (22%)

54. Okamoto modification of the Spitz Classification: Predictors
of Survival in Cases of Esophageal Atresia
Class Description Risk Survival
Class I No major cardiac anomaly, BW ≥2000 g Low 100%
Class II No major cardiac anomaly, BW < 2000 g Moderate 81%
Class III Major cardiac anomaly,BW ≤2000 g Relatively high 72%
Class IV Major cardiac anomaly, BW < 2000 g High 27%

55. Pre-operative care
• 24-48hrs pre-op stabilization
• Pneumonitis
– Aspiration from upper pouch secretions
– Reflux of gastric acid
• Broad spectrum antibiotics
• Pulmonary physiotherapy
• IVF
• Vit k
• Parenteral nutrition

56. • nurse 30 degrees head
up/lateral position
• Suction under low
pressure with Replogle
catheter
Pre-op care

57. Pre-op care
• Avoid routine endotracheal intubation
– Risk gastric perforation
– Worsening resp.distress as the abdomen becomes
distended from ventilation through the TEF

58. No Treatment
• Potters Syndrome
• Severe uncorrectable CHD

59. Treatment
• Primary
• Delayed
• Stage

60. Treatment
• Open thoracotomy
– Division of fistula, primary anastomosis of the esophagus
• Preliminary broncoscopy

61. Treatment
• Position
– Left lateral decubitus

62. Treatment
• Access
– Curved infrascapula skin incision extending from
anterior axillary line to paravertebral region
– Right posterolateral thoracotomy
– Pleural space entered b/w 4th intercostal space
– Extra pleural vs Transpleural

63. Treatment
• Azygous vein is divided
• Distal TEF mobilized and closed leaving 1-2mm
of the esophagus on the trachea
• Check air tightness of tracheal closure
– Instill warm saline in the pleural cavity, look for bubbles with positive pressure
ventilation

64. Treatment
• Trans anastomotic tube
– Aspirate air in distended stomach
– Patency of distal lumen
• Avoid excessive handling of distal esophagus
• Proximal pouch identification can be
facilitated by the anaesthesiologist

65. Treatment
• End –end esophagoesophagostomy
• Leave a drain-? Transpleural
• End-side anastomosis + single suture to ligate
the fistula Salama et al
– High rate of recurrent fistula
– Poenaru et al identified low rate of GER

66. Treatment
• Minimally invasive (thoracoscopic repair EA -
TEF)
• Thoracoscopy has been used since 2 decades
• Not yet gold standard for OA but widely used

67. Treatment – Operative
Minimally invasive surgery
Advantages
• Superior visualisation
• Improved cosmesis
• Eliminates morbidity
• Less chest wall complications-
scoliosis,wing scapula,chronic
pain,chest wall asymmetry,
maldevlopment
Disadvantages
• Not suitable in unstable pts
• Also in preterm and
• Pts with severe abdominal
distension

68. Emergency Treatment
• Preterm
• Large distal fistula with gross abdominal
distension
• Transpleural ligation of fistula

69. Emergency treatment
• Other options
• Gastric division
• Banding of gastroesophgeal jxn
• Positioning of the tip endotracheal tube below fistulous
orifice
• Fogarty balloon catheter to ablate the flow of ventilator air
• Emergency gastrostomy to decompress the air-filled
stomach

70. Emergency treatment
• Cervical esophagostomy
• advantages
• Early sham feeding
– Stimulates neural maturation
– Development of learning skills needed for feeding
– Speech acquisition
• disavantages
• Need for esophageal replacement later

71. Treatment
• Intraoperative maneuvers to decrease gap
– Circular (Livaditis) /spiral myotomy
– Full thickness anterior/posterior flap esophagoplasty
(Gough)
– Bagolan traction elongation technique
– Gastric transposition/hiatal mebolization

72. Treatment
myotomies
Proximal pouch flap
esophagoplasty

73. Post operative care
• Broad spectrum antibiotics
• ICU
• IVF
• Trans anastomotic tube feeding 48hrs
• Elective paralysis+ ventilator support 3-5days
• ?anastomosis under tension

74. Post operative care
• Ventilator care

75. Post operative care
• H2 antagonist prophylaxis
• ? Anastomotic stricture
• Esophagogram 5-7days
• Prophylactic dilatation at 3wks

76. Treatment (Long gap EA)
• Delayed primary repair
• Definition varies
– >6cm
– >6vertebral segments
– Cannot be anastomose primarily

77. • Prone to
– Prolonged hospitalization
– Associated significant morbidity
– Later swallowing difficulties

78. Pre operative delayed procedures
• Upper pouch bougienage
• Electromagnetic field pull together metallic
bullets
• Kimura’s multi stage, extrathoracic esophageal
elongation technique

79. Foker Method
• Foker’s elongation
traction technique

80. Stage procedures-Isolated EA
• 50% incidence of prematurity
• 11% incidence of Down’s syndrome
• 10% incidence of duodenal atresia

81. Stage procedure-isolated EA
• Gastrostomy, Oesophagostomy and replacement
– A gap length of 6 vertebral bodies
– Priliminary broncoscopy to eliminate presence of
occluded distal TEF
• Types of replacement
– Colonic
– Gastric transposition
– Gastric tube
– Jejunal interposition

82. Isolated (H-type) TEF
• Fistula run oblique (N-type) from trachea to esophagus
• 4% of esophageal anomalies
• Occur3-4 wks of life
• Xterized
– Choking on feeding
– Cyanotic spells
– Intermittent abdominal distention on crying or coughing

83. Isolated (H-type) TEF
• Olderchildren have recurrent bouts right
upper lobe of pneumonia
• Radiograph
– Aspiration pneumonia
– Gastric distention
– Tube video esophagography
– Broncoscopy/esophagoscopy confirm the
diagnosis

84. Isolated TEF - Treatment
• Identify fistula and divide
– Use pleural/inter costal muscle flaps to minimize fistula recurrence
• Patient should remain intubated
– Risk of tracheal oedema and recurrent laryngeal
nerve palsy
• Use Nd:Yag laser – coagulate fistula

85. EA and Right sided Aortic Arch
• Incidence – 1.8 – 2.5%
• Preop Echo very important
• Leak rate very high ≈42% with right thoracotomy
• If anastomosis difficult divide fistula and do a left
thoracotomy

86. Complications
Early
• Anastomotic leak
• Anastomotic stricture
• Recurrent TOF
Late
• Pulmonary dysfxn
• GOR
• Tracheomalacia
• Motility disorders
• Chest wall deformities
• “Anastomotic” stricture –usually 2o to
GORD
• Recurrent TOF

87. Anastomotic leak
• Occur in 13-16%
• most are less significant
• Major disruption rear
• Pt can deteriorate 24-48hrs from tension
pneumothorax, mediastinitis

88. Anastomotic leak
• Can be manage
– drainage, nutritional support, antibiotic cover
– Early exploration to secure anastomosis and
establish satisfactory drainage

89. Anastomotic leak
• Risk factors
– Poor surgical technique
– Ischaemia of the esophageal ends
– Use of myotomy
– Tension at anastonotic site

90. Esophageal stricture
• Incidence varies 37-55%
• In 80% of will require dilatation
• Due to
– poor anastomotic technique
– long gap EA
– ischaemia
– GERD
– anastomotic leak

91. • Symptoms
– Dysphagia
– Recurrent aspiration
– Foreign body obstruction
• Approach to Rx
– Balloon dilation
– Ante/retrograde bougienage
– Resection and anastomosis

92. Recurrent TEF
• 54% occur in the first year of repair
• 49% occur 15yrs later
• Approach to Rx
– Pleural flap
– Vascularised pericardial flap
– Azygous Vn flap
– Intercostal muscle flap

93. • Other options include:
– Diathermy fulguration
– Nd:YAG laser
– Injection of sclerosants
• Recurrence with above options high

94. GERD
• Occur in 30-70% of EA
• Due to
– shortening of intra abdominal portion of the
esophagus
– Esophageal motor dysfunction
• Risk of Barrett esophagus-9%

95. GERD
• Symptoms
• Vomiting, dysphagia, recurrent anastomotic
stenosis, stridor, cyanotic spells, recurrent
pneumonia
• Investigation
– UPI
– 24hr PH probe(multichannel esophageal
impidence+24hr PH probe) –promising
– Manometry-dysordered peristalsis
-↓LOS pressure

96. GERD
• Higher rates in
– Delayed Io repair
– Patients on gastrostomy
– Patients with anastomosis under tension
• Aggressive medical Rx or surgical fundoplication
(High failure rate – 15-38%)

97. Tracheomalacia
• Generalized or localized weakness of the
trachea that allow ant and post walls to come
together during expiration or coughing
• Reported in 75% of post mortem specimen
• symptomatic in 10-25%

98. Tracheomalacia
• Symptoms mimick- recurrent TEF, GERD,
anastomotic leak
• Brassing or barking cough
• Symptoms can improve with time
• Rx
– CPAP,aortopexy, tracheal stenting,
tracheostomy(severe)

99. Dysmotility disorders
• Dysphagia in the absence of anastomotic
stricture
• Usually bolus obstruction – esophageal
emptying by gravity
• Patients take excessive amounts of liquids to
push food down

100. Dysmotility disorders
• May be 2o to intrinsic innervation problems or
Vagal n damage at surgery
• Manometery

101. Risk for death & long term
complications
• Low birth weight<2500g/prematurity
• Twin birth
• Long gap EA
• Pre operative intubation
• Prolong post operative intubation >4days

102. • Inability to feed at 1month
• Anastomotic leak
• Major cardiac anomaly
• Severe associated anomalies

103. Outcomes
• Survival 85-95% compared to 50yrs ago
• Respiratory morbidity (pneumonia 15% at 5yrs, 5% at
15yr)
• Esophageal motility disorders 20% adolescent, 40%
adults
• GER 15-50%
• Quality of life (behavioral problems)

104. Outcomes (UMTH)
Year Age Type Other
anomalies
Rx Outcome
2012 9days C Nil Died
(Pneumonia)
2013 5days C ARM Primary repair Died (aspiration)
2013 2days C Nil Gastrostomy+banding survived
2014 14days C Nil Gastrostomy+banding Died(sepsis)
2014 3days C Nil Gastrostomy + banding survived

105. Future prospects – Role of Tissue
Engineering
• Recent advances in biomaterial science and
cellular biology
• Use of stem cells seeded on synthetic
biopolymers

106. Summary
• EA a challenging condition to manage, but
timely resuscitation and appropriate surgical
treatment underscore it’s outcome in absence
of associated anomalies.