3. •INCIDENCE :
o Incidence of TEF and EA ranges between
1 : 3000 and 1 : 4500 births .
o Most cases are sporadic, incidence is 2.4-fold
higher in twins .
o 20% to 40% of infants with TEF/EA are born
prematurely and/or are SGA.
4. • Mortality and morbidity increases with
decreasing GA.
premature infant (14%)
term infant (<5%).
• Approximately 20% to 25%, but as many as
50%, TEF/EA have additional congenital
defects.
5. The incidence of associated defects is
particularly high (50% to 70%) in the group
with isolated EA but least common (10%) in
infants with the H-type fistula (Type E).
10. • The most common anomalies include
Congenital heart disease (23% to 35%,VSD
,ASD , TOF , atrioventricular canal, and
coarctation of the aorta),
Genitourinary disorders (15% to 24%),
Gastrointestinal disorders (16% to 24%,
duodenal or ileal atresia , malrotation , and
imperforate anus),
11. Skeletal anomalies (13% to 18%),
Central nervous system disorders (10%),
Mediastinal abnormalities (8%),
Chromosomal disorders (5.5.%)
(Dave et al. 1999, Pinheiro et al. 2012).
15. Classification of TEF
• Several classifications have been proposed to
stratify preoperative risk.
16. • Waterston (1964) preoperative risk based on
birth weight and the presence of pneumonia
and other congenital anomalies :
• Group A: Birth weight >2500 g, no other
complications
• Group B: Birth weight 1800 g to 2500 g with no
other complications, or birth weight >2500 g
with moderate pneumonia/congenital anomaly
• Group C: Birth weight <1800 g with no other
complications, or birth weight >2500 g with
severe pneumonia/severe congenital anomaly
17. The Montreal classification congenital
anomalies and the need for MV :
• Group 1: Isolated major anomaly, isolated
dependence on mechanical ventilation, or
presence of non significant anomalies
• Group 2: Presence of severe congenital
anomalies or dependence on mechanical
ventilation associated with one major
anomaly
18. Spitz et al. 1994
• Group 1: Birth weight more than 1500 g without
a major cardiac anomaly (97% survival).
• Group 2: Birth weight less than 1500 g or major
cardiac anomaly (59% survival).
• Group 3: Birth weight less than 1500 g and
major cardiac anomaly (22% survival).
More recently, survival rates improved to 98%,
82%, and 50%, respectively (Lopez et al. 2006).
20. Anatomical classification
• Vogt initially classified these lesions, Gross
modified the anatomic variations of TEF
Both systems establishing types depending on
the presence of esophageal atresia and, if
present, the location of the fistula.
24. VOGT classification
• Type I- esophageal agenesis, very rare
• Type II- pure esophageal atresia
• Type IIIA- EA with proximal TEF
• TYPEIIIB- EA with distal TEF
• TYPEIIIC- dual TEF with both segments
communicating with trachea
25. • In most cases, TEF and EA co-occur
(type B 2%, type C 86%, type D <1%, and type E
4%)
• Isolated EA, type A, is reported in
approximately 7% of cases.
26. • Most common version, Type C , a fistula exists
between the trachea and the lower
esophageal segment at a point slightly above
the carina, and the upper esophageal segment
ends blindly in the mediastinum at the level of
the second or third thoracic vertebra.
28. Embryology
• Embryogenesis of TEF/EA incompletely
defined, several theories proposed .
• The trachea & esophagus develop from the
foregut in the first 4 to 5 weeks of gestation.
• One theory suggests respiratory system results
from a rapid outgrowth from the foregut.
• Tracheal primordium buds off the foregut and
remains a separate structure during subsequent
development.
29. • Another theory active growth of
mesenchymal septum that divides the
foregut into ventral (respiratory) and dorsal
(gastrointestinal) structures.
• Other theories seem to combine segments of
each of these two theories, proposing that
the trachea and esophagus separate as a
result of foregut folds.
30. • Both respiratory and esophageal
components seem to display disorders of
differentiation in the setting of TEF/EA, but
the pathogenesis remains incompletely
understood.
• Many have concluded that both multigenic
and epigenetic/environmental factors
contribute (Smigiel et al. 2015, Pinheiro et al.
2012).
33. Prenatal Diagnosis
• Polyhydramnios and small or absent fetal
stomach bubble are MC ultrasound findings.
• Low positive predictive value, and false
positives are common.
• A dilated blind-ending upper pouch of the
esophagus (upper pouch sign) on ultrasound,
but not an accurate prenatal method for
diagnosing TEF/EA
34. MRI imaging, nonvisualization of the
intrathoracic portion of the esophagus; lead
to a high false-positive rate for the diagnosis
of TEF.
• Overall, the prenatal detection rate for TEF
ranges from 40% to 50% .
35. Anesthetic management of TEF
• Preoperative Management :
• The ligation of a TEF is urgent, but not
emergent, except in the setting of respiratory
insufficiency severe enough to require
ventilatory support, especially in the
premature infant.
36. • How to protect the lung from
aspiration pneumonia ???
37. • Preoperatively, to protect the lungs from
aspiration pneumonia:
Avoiding feeding
Upright, semiprone positioning of the infant
to minimize gastroesophageal reflux
Intermittent suctioning of the upper pouch
Administration of antibiotics
42. • Vascular anomalies (e.g., right aortic arch,
2.5% to 5%) that can alter the surgical
approach , therefore echocardiography
should be done preoperatively.
44. • ECG for rate and rhythm.
• BP-invasive for complicated cardiac lesion,
otherwise a non-invasive technique.
• Intra arterial cannula for ABG
• A precordial stethoscope to monitor both
resp & cardiac activity.
45. • Temperature monitoring.
• Blood loos by weighing sponges and
measuring suction bottles.
• Do not put an esophageal stethoscope for
fear of perforating the blind pouch.
47. Transport the infant in a heated isolette.
Warm the operating room to more than 27°
C (80° F).
Use a warming mattress (water temperature
of 40° C).
Heat and humidify gases to 36° C (at the
trachea).
48. Use a radiant heat warmer with a
servocontrol mechanism.
Wrap noninvolved areas with plastic.
Warm intravenous fluids and blood.
Warm scrubbing and irrigation solutions.
Monitor the temperature in the operating
room.
50. Holliday and Segar( 4-2-1 rule):
Daily fluid requirements directly depend on
metabolic demand-specially that 100 ml of water
required for 100 calories of EE.
4-2-1 Rule ---- this amount does not include:
fluid deficits, third-space losses, modifications
b/c of hypo or hyperthemia or requirements by
unusual demands
51. • Original Holliday & Segar problematic for
acutely ill or with significant cardiac or renal
dysfunction.
• So, now more accurate method of fluid
management:
Administer 20-40 ml/kg of BSS during
course of anesthesia
Postoperative fluid management should be
2-1- 0.5 rule =2ml/kg for first 10kg, 1ml/kg
for next 10kg, & 0.5ml/kg for each kg above
20kg.
52. • If after 12hrs the child unable to
convert to oral intake ,then standard
hypotonic solution (D5 0.45% saline)
is initiated at 4-2-1 rule rate to avoid
hypernatremia
53. • What is daily fluid
requirement for term
newborn in the days after
birth???
54. • 70ml/kg on day 1
• 80ml/kg on day 3
• 90 ml/kg on day 5
• 120ml/kg on day 7
• These requirements slightly higher for
preterm infants.
• Newborns generally started on 10% glucose
solution to prevent hypoglycemia.
55. • What is estimated blood volume
(EBV) in children????
58. • MABL=
EBV × ( Starting Hct- Target Hct ) /Starting Hct
• If blood loss is less than or equal to MABL
and no further significant blood loss occurs or
is anticipated in postoperative period, ---then
no need for transfusion of PRBCs.
59. • How to calculate volume of
PRBCs to be transfused???
60. • Volume of PRBCs transfused( in ml)=
(Desired Hct - Present Hct) × EBV / Hct of
PRBCs(~60%)
61. Intraoperative Management
Airway management:
• “Awake intubation” is safest approach to
secure the airway in an infant with TEF .
• Theoretically, this technique allows
appropriate positioning of the ETT without
PPV and; minimizes the risk for gastric
distention.
62. • Mild sedation by small doses of fentanyl (0.2
to 0.5 mcg/kg) or morphine (0.02 to 0.05 mg/
kg), intubation of the trachea is accomplished
without excessive hemodynamic stimulation
or depression.
• In reality, in some patients, especially the
premature infant, apnea accompanies even
seemingly minimal sedation.
63. • Prefer an alternative technique that includes
an inhaled anesthetic without muscle
relaxation accompanied by cautious, gentle
PPV .
• Hemodynamic instability in response to
either narcotics or an inhalation agent, a
NMB may be delivered, for support of PPV.
64. • With normal lung compliance, gentle positive
pressure can be delivered with minimal
delivery of inspired gases into the stomach
via the fistula.
• Optimally, the endotracheal tube can be
advanced beyond the fistula, but, with the
fistula often close to the carina-single-lung
ventilation.
65. • In all cases, the surgeon should be present
during induction of anesthesia e.g on rare
occasions, the distended stomach may need
to be decompressed emergently (e.g.,
transcutaneous /ultrasound-guided insertion
of a catheter or needle).
67. • Evaluating the upper airway via rigid or
fiberoptic bronchoscopy an integral part of
the presurgical / intraoperative course of
TEF/EA repair.
• Bronchoscopy can aid:
In detecting the number & location of fistula
Assess for tracheomalacia
Other anomalies relevant
Assist in precisely placing an ETT below the
fistula.
68. • Rigid bronchoscopy is performed prior to the
start of surgery, the choice of anesthetic
induction technique involving spontaneous
ventilation and controlled ventilation with muscle
relaxation is very much dependent on the surgical
skill of the operator performing the
bronchoscopy.
• The surgeon and anesthesiologist must decide if
single-lung ventilation would facilitate the
surgical procedure, and if the patient can tolerate
the technique
69. • In the newborn, single-lung ventilation implies
advancing the endotracheal tube into the
mainstem bronchus of the lung contralateral to
the surgical site.
• If tolerated, collapse of the lung may improve
surgical exposure, but an unacceptable decrease
in oxygen saturation can occur in some instances,
even with a high FiO2 and continuous positive
airway pressure (CPAP) to the nonventilated lung.
70. • Salem et al (1973) suggested distal
positioning of the ETT , with the bevel facing
anteriorly and the posterior wall of the ETT
occluding the fistula, but this maneuver is
challenging to achieve and maintain
• Precordial stethoscopes positioned in the left
axilla (i.e., during a right thoracotomy) can
allow breath-to-breath monitoring to
determine ventilation to the dependent lung.
72. Surgery
Primary repair
• Right thoracotomy
• Thoracoscopic repair
• Left thoracotomy(2.5%)
Staged repair
• Long gap atresia
• Bronchoscopic
application of adhesive
or fibrin glue
74. 89 patients/16 yrs
• Shoulder elevation: 24%
• Chest deformity: 20%
• Abduction limited: 100%
• Spine deformities: 18%
• Breast deformities: 27%
Jaureguizar E, et al: Morbid musculoskeletal sequelae of
thoracotomy for tracheo-esophageal fistula. J Pediatr Surg 20:
511-514,
75. Advantages of Thoracoscopy• Reduce
musculoskeletal
sequelae
• Superior visualization
of anatomy
• Easy to identify fistula
for ligation
• Reduced post-
operative pain
76. Anesthesia for Thoracoscopy
• Operative lung compressed by CO2 insufflation
(5mmHg)
• Associated with mild desaturation requiring 100% O2
or hand ventilation.
• HFOV minimize the movement of the operative side
• EtCO2 will be falsely low due to compression of the
lung and CO2 insufflation.
77. Anesthesia for Thoracoscopy
• Routine ASA monitors +/- A-line
• Maintenance of spontaneous ventilation during
induction
• Muscle paralysis can be given after fistula ligated
• Balanced anesthetic +/- epidural for post-op
pain management
• May have difficulty with hypercapnia or
difficulty ventilating
78. Extubate or Not?
• Must consider pre-op lung disease and other
comorbidities
• Spontaneous ventilation decreases the stress
placed on the suture line
• Risk of injury to the repaired fistula with re-
intubation
• Tracheomalacia: 10-20 %
• Recurrent laryngeal nerve palsy (diaz et al)
79. Long-gap Esophageal Atresia
• Defined as Greater than 3cm between the
esophageal ends or 2 vertebra bodies
• Excess tension on the esophageal
anastamosis is associated with increased
complications and worse outcome
• Ideal to use the patient’s own esophagus
80. Surgical Options
• Primary anastamosis at time of initial repair
• Serial staged dilation with bougie followed by
esophageal anastamosis
• External tension with sutures, magnets, etc to
lengthen esophagus following by esophageal
anastamosis
• Esophageal replacement with gastric pull
through, colonic graft or jejunal graft
83. Anastomotic leak
• Early complication occurring in 17% of patients
• Typically will resolve spontaneously without oral feeds or
with pleural drainage
• Case reports of glycopyrolate and atropine used to
minimize secretions
• Major leaks may require cervical esophagostomy and
gastrostomy with delayed definitive repair
• Esophageal strictures and recurrent fistula are more
likely to follow
84. Esophageal Strictures
• Occurs in 6-40% of patients
• More common with
– Gap >2.5cm
– EA/TEF type A, C, D
– Non-absorbable sutures
• Presents with dysphagia, poor feeding, and
emesis
• Treated with Esophageal dilation
• Improves with time
85. • What are anesthetic concerns after
TEF/EA repair?????
87. SUMMARY
Anesthestic Management for TEF
Monitors :
Standard monitoring
Upper- and lower-extremity pulse oximeter
Arterial access if hemodynamically unstable,
congenital heart disease, or
significant lung disease preoperatively
88. •Anesthestic :
• Inhaled induction with sevoflurane
• Establish intravenous access
• Maintain spontaneous ventilation
• If bronchoscopy is performed prior to surgical
repair:
• Glycopyrrolate
• Topicalize vocal cords and trachea with
lidocaine
• Titrate inspired anesthetic concentration
• Proporol or ketamine PRN
89. If fistula is 1 cm or more above carina, intubate
(bevel ETT anterior) (inflate cuff if cuffed tube)
If fistula below carina, then left mainstream
intubation
Neuromuscular blockade after lung isolation
Position left lateral decubitus for right
thoracotomy (no right aortic arch)
Fentanyl for analgesia
Consider regional anesthesia
Temperature: forced-air warmer