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Tracheoesophageal Fistula
Dr Neisevilie Nisa
AIIMS New Delhi
22.2.2016
Overview
• Background
• Diagnosis
• Pre-repair bronchoscopy
• Anesthesia technique
• Surgery
• Complications following TEF/EA repair
Background
• 1:2,500-4,000 live births
• Sporadic, non-syndromal
• Associated congenital anomalies: 50 %
• 30% premature neonate
• Prenatal diagnosis: 40 % predictive value
Clinical presentation
• Inability to pass suction catheter
• Excessive salivation
• Choking with feeds
• Cyanosis with feeds
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
How to diagnose …..????
• Prenatal
• Polyhydramnios
• Absent / small stomach bubbles
• X-ray thorax and abdomen
• Echocardiogram
• ECG
• ABG and serum electrolytes
• Lumbar ultrasound: Sacral dimple
Is it a surgical emergency….?
• Aspiration can cause pneumonia
• Rule out associated comorbidities
• Stabilization required
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 %
Associated syndromes
• Coexisting congenital abnormalities: 50 %
• Isolated EA: 65 %
High risk patients
• Weight < 2 Kg and pre-term baby
• Coexisting complex congenital heart disease
• Pericarinal fistulas
• Poor pulmonary compliance
• Thoracoscopic repairs
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
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
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
Anesthesia concerns
• Position of fistula
• Small vs large fistula
• Pulmonary compliance
• Spontaneous ventilation Vs controlled
ventilation
• Pre-repair Bronchoscopy..??
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)
Pre-repair Bronchoscopy
Airway management
• Tracheal intubation distal to fistula
• One lung ventilation
• Salems’ technique
• Fiberoptic tracheoscopy
• Fogarty catheter occlusion
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
Intraoperative period
• Arterial line: complex heart lesions and
pulmonary disease, thoracoscopic surgery
• Muscle relaxant
• Morphine 50-100 mics/ kg
• Cuadal anesthesia
Tsui technique
for threading caudal catheters
Patient Position
Surgery
Primary repair
• Right thoracotomy
• Thoracoscopic repair
• Left thoracotomy(2.5%)
Staged repair
• Long gap atresia
• Bronchoscopic
application of adhesive
or fibrin glue
The surgical debate……
Thoracoscopy Thoracotomy
•Transpleural • Extrapleural/Transpleural
•Longer operative time • Shorter operative time
•Better visualization • Adequate visualization
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,
American Surgical Association, 2005
Ann Surg 242:422-430, 2005
• Retrospective study
• Six international centers
• 2000 – 2004
• 104 Pts
Advantages of Thoracoscopy
• Reduce musculoskeletal
sequelae
• Superior visualization of
anatomy
• Easy to identify fistula for
ligation
• Reduced post-operative
pain
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.
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
• 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
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)
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
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
Complications following TEF/EA Repair
• Anastomotic leak
• Recurrent esophageal fistula
• Esophageal strictures
• GERD/Esophageal dismotility
• Tracheomalacia/ Pulmonary Issues
• Musculocutaneous disturbances
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
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
Anesthesia concerns after TEF repair
• Dysphagia
• GERD
• Tracheomalacia
• Bronchial hyperactivity
• Scoliosis
Any queries…???
•
Thank you
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
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
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
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

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Anesthesia for Tracheoesophageal fistula

  • 1. Tracheoesophageal Fistula Dr Neisevilie Nisa AIIMS New Delhi 22.2.2016
  • 2. Overview • Background • Diagnosis • Pre-repair bronchoscopy • Anesthesia technique • Surgery • Complications following TEF/EA repair
  • 3. Background • 1:2,500-4,000 live births • Sporadic, non-syndromal • Associated congenital anomalies: 50 % • 30% premature neonate • Prenatal diagnosis: 40 % predictive value
  • 4. Clinical presentation • Inability to pass suction catheter • Excessive salivation • Choking with feeds • Cyanosis with feeds
  • 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 %
  • 9. Associated syndromes • Coexisting congenital abnormalities: 50 % • Isolated EA: 65 %
  • 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)
  • 17. Airway management • Tracheal intubation distal to fistula • One lung ventilation • Salems’ technique • Fiberoptic tracheoscopy • Fogarty catheter occlusion
  • 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
  • 20. Tsui technique for threading caudal catheters
  • 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
  • 34. Complications following TEF/EA Repair • Anastomotic leak • Recurrent esophageal fistula • Esophageal strictures • GERD/Esophageal dismotility • Tracheomalacia/ Pulmonary Issues • Musculocutaneous disturbances
  • 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
  • 37. Anesthesia concerns after TEF repair • Dysphagia • GERD • Tracheomalacia • Bronchial hyperactivity • Scoliosis
  • 40.
  • 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