5. Types of TEF
• Type A = Atresia
• Type B = Below/ Blind end
• Type C = Commonest type
• Type D = Double fistula
• Type E = ‘H’ (Edge)
• Type C is the commonest
• Type A and E pose fewer challenges
• Type E has better prognosis
6. How to diagnose …..????
• Prenatal
• Polyhydramnios
• Absent / small stomach bubbles
• X-ray thorax and abdomen
• Echocardiogram
• ECG
• ABG and serum electrolytes
• Lumbar ultrasound: Sacral dimple
7. Is it a surgical emergency….?
• Aspiration can cause pneumonia
• Rule out associated comorbidities
• Stabilization required
8. Associated comorbidities
Organ system Incidenc
e
Example
Cardiac 29 % VSD, PDA, TOF, ASD, HLHS, Right Aortic Arch
Gastrointestinal 14 % Duodenal atresia, imperforate anus, PS
Genitourinary 14 % Renal agenesis, hypospadiasis, horse shoe kidney
Musculoskeletal 10 % Vertebral anomalies, Limb defects
VACTERL 10 % Vertebral, anal, CHD, renal and limb
Respiratory 6 % Tracheo-bronchomalacia, pulmonary hypoplasia
Genetics 4 % Trsisomies 21, 18, deletion 13
• Cardiac anomalies are the commonest with an
incidence of 29 %
10. High risk patients
• Weight < 2 Kg and pre-term baby
• Coexisting complex congenital heart disease
• Pericarinal fistulas
• Poor pulmonary compliance
• Thoracoscopic repairs
11. Waterston Classification(1962)
Category Weight/ Co-morbidities Surgical timing
Category A > 2500 grams Can undergo surgery
Category B 1800 – 2500 peumonia or
congenital anomally
Short term delay, needs
stabilizing treatment
prior to surgery
Category C < 1800 grams or severe
peumonia or congenital
anomally
Required staged repair
12. Spitz Classification(1994)
Group Features Survival (%)
I
>1500 grams , no
major cardiac
anomally
98.5
II
< 1500 grams or
major cardiac
anomally
82
III
< 1500 grams and
major cardiac
anomally
50
13. Okamoto classification(2009)
Class Birth weight Anomally Survival rate
I ≥ 2 Kg Nil 100
II < 2 Kg Nil 81
III ≥ 2 Kg Major cardiac
anomaly
72
IV > 2 Kg Major cardiac
anomaly
27
14. Anesthesia concerns
• Position of fistula
• Small vs large fistula
• Pulmonary compliance
• Spontaneous ventilation Vs controlled
ventilation
• Pre-repair Bronchoscopy..??
15. The Evidence behind the pre-repair
Bronchoscoy
• Bronchoscopy can
• Define the fistula location
• Determine unusual characteristics of the
fistula(double fistula or trifurcation)
• Determine presence of tracheobronchitis (surgery
contraindicated)
• Locate the aortic arch
• Influence anesthetic management
• May change the operative management
(changed operative approach in 57% with 31%
being crucial changes)
18. Anesthesia technique
• Inhalational induction
• Intravenous induction and maintenance(bronchoscopy)
• Opiod: fentanyl 1 mic/kg
• Muscle relaxant if mask ventilation satisfactory
• Caudal/ thoracic epidural
• Standard ASA monitoring
• No single best anesthesia technique is defined
• Anesthesia technique has to be individualised based
on patient characteristics
19. Intraoperative period
• Arterial line: complex heart lesions and
pulmonary disease, thoracoscopic surgery
• Muscle relaxant
• Morphine 50-100 mics/ kg
• Cuadal anesthesia
22. Surgery
Primary repair
• Right thoracotomy
• Thoracoscopic repair
• Left thoracotomy(2.5%)
Staged repair
• Long gap atresia
• Bronchoscopic
application of adhesive
or fibrin glue
23. The surgical debate……
Thoracoscopy Thoracotomy
•Transpleural • Extrapleural/Transpleural
•Longer operative time • Shorter operative time
•Better visualization • Adequate visualization
24. 89 patients/16 yrs
• Shoulder elevation: 24%
• Chest deformity: 20%
• Abduction limited: 100%
• Spine deformities: 18%
• Breast deformities: 27%
(3/11)
Why Thoracoscopy ?
Jaureguizar E, et al: Morbid musculoskeletal sequelae of
thoracotomy for tracheo-esophageal fistula. J Pediatr Surg 20: 511-
514,
25. American Surgical Association, 2005
Ann Surg 242:422-430, 2005
• Retrospective study
• Six international centers
• 2000 – 2004
• 104 Pts
26. Advantages of Thoracoscopy
• Reduce musculoskeletal
sequelae
• Superior visualization of
anatomy
• Easy to identify fistula for
ligation
• Reduced post-operative
pain
27.
28. 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.
29. 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
30. • 2007 – 2010
• 17 neonates
- Mean age - 4 days
- Mean wt - 2.9 ±1.0 kg
- Vent changes – 3 patients
J Laparoendosc Surg 21:877-879, 2011
31. 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)
32. 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
33. 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 pullthrough,
colonic graft or jejunal graft
35. 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
36. 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
41. Recurrent Esophageal Fistula
• Serious complication affecting 5-20% of patients
• Open thoracotomy associated with morbidity and
mortality rates of 10-22%
• Endoscopic Closure preferred
• Presents with cough, choking, or cyanosis with
feeding, or recurrent pneumonia
42. Tracheomalacia
• Present in 75% of pathologic specimens in patients
with EA/TEF
• Clinically significant in 10-20%
• Usually found at or just above the level of the original
EA/TEF
• Presents with brassy cough, stridor, and dyspnea with
feeds
• Treatment usually medical
43. Esophageal Dysmotility
• Esophageal peristalsis is abnormal in 75-100% of
patients with EA/TEF
• Small discoordinate contractions lead to
increased risk for esophageal obstructions
• Improves with time as 65% of kids will be
admitted with GI sx in the first 10 years of their
life, but only 3% of patients will be admitted after
18 years of age
44. GERD
• Occurs in 35-58% of TEF/EA children
• Due to intrinsic motor dysfunction of the esophagus as
well as possible anastomotic tension
• 56% of patients with GERD respond to medical therapy
• 13-25% of patients will require a Nissen fundoplication
• However, attempts are made to avoid fundoplication due
to risk of severe dysphagia following given dyskinetic
esophagus