Physical Exam. Vitals stable on room air Abdomen soft, non-distented Normal external genitalia Patent anus No limb anomalies

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Barry Carpenter
5 years ago
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1 Case Presentation 1 day-old full-term baby girl noted to have drooling of saliva and increased secretions at birth Fetal had shown polyhydramnios Birth weight 3515g Apgar 7@1min and 8@5min Unable to pass NGT Normal meconium stools and urinary output

2 Physical Exam Vitals stable on room air Abdomen soft, non-distented Normal external genitalia Patent anus No limb anomalies

3 XR DOL#1

4 Further Studies Echocardiogram normal Renal US normal Vertebral XRs normal

5 Initial Management Replogle catheter in upper esophageal pouch Upright sitting position Antibiotic coverage and IV fluids Stamm gastrostomy DOL#5

6 Operation DOL#8 Right retro-pleural thoracotomy in 5 th intercostal space Distal TEF identified at carina with gap 3-4cm Fistula divided and tracheal defect repaired with 5-0 prolene Distal and proximal pouches mobilized End-to-end esophagoseal anastomosis with 3-0 silk 10F chest tube and penrose drain placed

7 Esophogram POD#12

8 Hospital Course POD#14 oral diet advanced POD#20 Discharged in stable condition

9 Esophageal Atresia Christopher Turner Richmond University Medical Center

10 History 1670: First description, William Durston 1888: First attempt to correct EA, Charles Steele 1941: First successful primary repair EA/TEF, Cameron Haight 270 years from first description to first survivor

11 History Holmes 1869: The attempt ought not, I think, be made. Richter 1913: The physician who, after making his diagnosis of congenital atresia of the esophagus, decides to let his little patient die undisturbed can amply justify his course. Gage 1936: The operation would always be finished as a postmortem procedure. Lanman 1940: In spite of the fatal outcome in all the 30 operative cases, it is felt that considerable progress along rational lines is being made.

12 1940

13 1941

14 Embryology (His 1887) Formation of tracheoesophageal septum begins caudally and ends cranially

15 Embryology 2010 Normal Abnormal

16 Epidemiology Incidence 1:3500 live-born infants Higher prevalence in white mothers Increased risk with first pregnancy, older maternal age, and in vitro fertilization

17 Associated Anomalies 50-70% of infants have at least one associated congenital malformation

18 VACTERL Idiopathic occurrence of multiple anomalies Generally three components to qualify Vertebral 17% Anal 12% Cardiac 20% Renal 16% Limb 10%

19 Gross Classification 6% 5% 84% 1% 4%

20 Watterson Risk Groups (1962)

21 Spitz Risk Groups (1994)

22 Prenatal Diagnosis Prenatal EA is not commonly diagnosed US shows small or absent stomach bubble and maternal polyhydramnios Predictive value only 20-40%

23 Clinical Findings Usually symptoms in the first hours of life Earliest sign is excessive salivation First feeding is followed by regurgitation, choking and coughing Other features are cyanosis, inability to swallow, inability to pass a feeding catheter

24 Catheter curled in the upper pouch suggests the diagnosis

25 Dilute barium can be used to confirm the diagnosis

26 Air in the abdomen confirms the presence of distal TEF

27 Absence of air in the abdomen typically represents isolated EA without distal TEF

28 Diagnosis of TEF without EA can be made by barium esophagography Bronchoscopy is often required for confirmation

29 Additional Testing Physical exam focused on associated defects Echocardiography Renal ultrasonography Chromosomal analysis

30 Preoperative Treatment Immediate management to prevent aspiration and treat pneumonitis Sump catheter in the upper pouch Upright sitting position Broad spectrum antibiotics IV fluids with 10% dextrose and hypotonic saline Routine intubation should be avoided

31 Operative Repair Positioning

32 Operative Repair Extrapleural Dissection

33 Operative Initial Exposure Repair EA with Distal TEF

34 Division Operative of Azygous Repair Vein EA with Distal TEF

35 Mobilization Operative of Repair Distal Pouch EA with Distal TEF

36 Operative Repair EA with Distal TEF

37 Operative Repair Anastomosis

38 Mobilization of Distal Pouch

39 Interrupted Closure of TEF

40 Dissection of Upper Pouch

41 End-to-End Anastomosis

42 Outcomes 85-95% survival rates Risk factors for death and long-term morbidity Lower birth weight Prematurity Major congenital heart disease Severe associated anomalies Ventilator dependence Long gap length

43 Complications 50% have complications in the first year Early Anastomotic Leak 13-16% Esophageal Stricture 80% Recurrent Fistula 3-14% Late GERD 30-70% Tracheomalacia 10-25%

44 Anastomotic Leak Most managed with adequate drainage and nutritional support 95% close spontaneously 3-5% require reoperation Cervical esophagostomy and delayed esophageal replacement may be required

45 Esophageal Stricture Treated by dilation performed by bougienage Most respond to 1-3 dilations in the first months Some require resection and reanastomosis Routine H2 blockers or PPI

46 GERD 30-70% Management thickening of feeds, prone or upright positioning, acid reduction agents Results in failure to thrive, chronic pulmonary infections, refractory strictures 45-75% of these infants require anti-reflux operation

47 Tracheomalacia 10-25% Weakness that allows anterior and posterior tracheal walls to come together during expiration Results in barking cough, recurrent pneumonias, apneic spells Treatment for severe symptoms is aortopexy

48 Long Gap EA

49 Operative Repair Long Gap EA Gastric Transposition

50 Spontaneous Esophageal Growth

51 Operative Repair Long Gap EA Foker Procedure

52 27 short gaps (<1cm) 18 moderate gaps (1-2.4cm) 25 long gaps (>2.5cm)

55 Esophageal anastomosis can withstand considerable tension and allows a reliable true primary repair for the full EA spectrum Growth is rapid and traction sutures will produce significant esophageal lengthening within days With increasing tension, GER and strictures are more common

56 Summary EA with distal TEF most common 50-70% have associated anomaly CHD account for most deaths Immediate management: sump catheter, upright sitting position, antibiotics, IVFs Operation: extrapleural dissection with single layer anastomosis 50% have complications in the first year

57 Question #1 SESAP What is the most common anatomic configuration of EA and TEF? A. Pure esophageal atresia B. EA with proximal TEF C. EA with distal TEF D. EA with proximal and distal TEF E. H-type TEF

58 Question #2 SESAP What is the most effective treatment for lifethreatening tracheomalacia in a 2-month old infant following repair of EA with distal TEF? A. Tracheostomy B. Thoraotomy and revision of tracheal repair C. Nasal continuous positive airway pressure D. Aortopexy E. A radially expandable tracheal stent

59 Question #3 Rush Review A 3000g infant is born with EA and distal TEF. If the infant does not exhibit respiratory distress and associated anomalies are not present, which of the following is the preferred treatment? A. Gastrostomy, cervical esophagostomy, and delayed repair B. Gastrostomy, sump tube drainage of the proximal pouch and delayed repair C. Fistula ligation and delayed esophageal repair D. Division of the fistula with primary esophageal anastomosis E. Primary repair with colonic interposition

Tracheoesophageal Fistula and Esophageal Atresia

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