1. Long Gap Esophageal Atresia
Andrew Ludwig, M.D.
Resident, Department of Surgery
University of Washington

2. Historical Context
1670 – William Durston describes EA in a conjoined twin
1697 – Thomas Gibson writes “An Anatomy
of Humane Bodies Epitomized, a Clinical
Account of Esophageal Atresia with Distal
Tracheo-oesophageal Fistula”
1898 – W. Hoffman attempts first primary repair

3. Historical Context
1939 – William Ladd successfully completes pharyngogastric conduit in Boston
Within 24hrs, N.H. Levin completes a similar operation in St. Paul
Levin subsequently adopted the boy as his only child

4. Historical Context
1941 – Cameron Haight performs first successful primary repair of EA in a 12-day-old infant
Penicillin had not yet been discovered
The only antimicrobial agent used was sulfathiazole administered rectally

5. Embryology
4th-7th weeks of gestation
Failure/deviation of septation

6. Epidemiology Incidence of EA is 1 in 3000-4500 births
M:F ratio of 1.26
Recurrence risk of 0.5-2% with affected sibling
Low concordance rate in twins

7. Classification

8. Associated Anomalies Associated anomalies are found in ~50% of cases
VACTERL (25%)
CHARGE
Trisomies (5-10%)
Cardiac (35%)
Gastrointestinal (20%)
Gentitourinary (20%)

9. Clinical Features & Diagnosis
Prenatal Ultrasound (40% sensitive)
Polyhydramnios (66%)
Small/absent gastric bubble
Anechoic area in neck
“Pouch sign” >26wks
Prenatal MRI

10. Clinical Features & Diagnosis
Prenatal Ultrasound (40% sensitive)
Polyhydramnios (66%)
Small/absent gastric bubble
Anechoic area in neck
“Pouch sign” >26wks
Prenatal MRI

11. Clinical Features & Diagnosis
Postnatal: Excessive secretions that cause drooling, choking, respiratory distress, and feeding intolerance
Failure to pass NGT into the stomach
A-P chest x-ray

12. Chest X-Ray
Lack of bowel gas = lack of TEF

13. Workup Chest x-ray Distended proximal pouch containing Replogle
Heart shadow and size
Vertebral and rib anomalies
Aspiration pneumonitis
Diaphragmatic hernia

14. Workup Renal ultrasound Echo/Arteriogram Gap-o-gram
Left vs. Right aortic arch
Vascular ring
Gap-o-gram
Estimate gap length
Bronchoscopy/Esophagoscopy
Evaluate for proximal and distal fistulae

15. Gap-o-g ram

16. Gap-o-g ram

17. Gap-o-g ram

18. Bronchoscopy/Esophagoscopy

19. Timing of Intervention
Waterston
Category
Birth Wt
Pneumonia
Anomalies
Timing A
>2.5kg -
Immediate B kg +
Delayed C
<1.8kg ++
Staged

20. Timing of Intervention
Poeneru
Class
On Vent
Anomalies
Timing I N
none/minor
Immediate II Y
major
life-threatening
Delayed

21. Timing of Intervention
Spitz
Group
Birth Wt
Cardiac dz
Timing I
>1.5kg -
Immediate II
<1.5kg +
Delayed
III
Staged

22. Prognosis Depends on the presence and severity of associated anomalies
Waterston
Category
Mortality A 1% B
25% C
29%
Spitz
Group
Mortality I 3% II
41%
III
78%
Poeneru
Class
Mortality I
7.3% II
69.2%

23. Operative Planning Primary Anastomosis
Delayed/Staged Primary Anastomosis
Esophageal replacement

24. Operative Planning Primary Anastomosis
Delayed/Staged Primary Anastomosis
Esophageal replacement

25. Primary Anastomosis A. B. C. D. E.

27. Techniques for Repair of LGEA
Native Esophagus
Esophageal Replacement
Bougienage
Colonic Interposition
Spiral Myotomy (Kimura)
Circular Myotomy (Levaditis)
Gastric Tube
Greater or Lesser Curvature
Iso- or Retroperistaltic
Extrathoracic Elongation
Gastric Transposition (Pull-up)
Traction Suture Elongation (Foker)
Jejunal or Ileal Interposition

28. Operative Planning Primary Anastomosis
Delayed/Staged Primary Anastomosis
Esophageal replacement

29. Delayed Primary Repair
Continuous Replogle suction of proximal pouch
45° sitting position
Prophylactic abx (amp & gent)
G-tube Feeds
Gastrostomy, TEF ligation, cervical esophogostomy (spit fistula)
Sham feeding to prevent oral aversion
Bougienage

30. Bougienage
“The relative importance of the delay and growth of the infant versus the elongation of the pouch(es) is uncertain…However…when the elongation regimen was ended, in every instance the upper segment had gained relatively more length than the corresponding distal segment and in all patients the upper pouch was relatively voluminous, thick-walled, and took sutures well.”

31. Bougienage
“When an ultra-long-gap EA was suspected preoperatively we have allowed the infant to grow and delayed the primary repair until 62 to 173 days of age. Despite growth of the infant, no diminution in gap length was seen.”

32. Foker Technique

33. Foker Technique

35. Operative Planning Primary Anastomosis
Delayed/Staged Primary Anastomosis
Esophageal replacement

36. Colonic Interposition

37. Gastric Tube
Isoperistaltic
Retroperistaltic

38. Gastric Tube

39. Gastric Transposition

40. What Are The Consequences?
Delayed Primary Repair
Foker Technique
Colonic Interposition
Gastric Tube & Transposition
Long time on tube feeds
Multiple surgeries
Reflux
No guarantee of primary repair
Need for paralysis/ventilator
Lack of peristalsis
Barrett’s esophagus
Cumulative risk of aspiration
Suture breakage and repositioning
Dilation of graft
Dumping
Oral aversion
Decreased lung volumes
Three anastomoses required
Decreased absorption

41. 15 studies published in the last 5 years
264 Long-gap patients (73% CI, 21% GPU, 6% JI)
Measured postoperative survival, gastrointestinal complications, and respiratory complications
Review of the English-language literature published in the past 5 years
Postoperative survival Rate
Respiratory complications: (pneumothorax, pneumonia, atelecta-
sis, mediastinitis, pleural effusion, and temporary diaphragm/vocal cord paresis)
Gastrointestinal complications: dysphagia, reflux, dumping, esophagus ulceration, anastomotic diverticulum, cervical fistula, graft redundancy, graft ulceration, intestinal obstruction, short bowel syndrome, dumping syndrome, delayed gastric emptying, cyclical vomiting, pyloric stenosis, diarrhea, colitis, peritonitis, small intestinal ischemia, and stomach perforation

42. GPU associated with higher rate of respiratory morbidity
CI associated with higher rate of gastrointestinal complications
Overall anastomotic leak rate = 20.6%

43. Total of 15 children (gaps 5-14cm) Three Groups:
Gap length >5cm
Total of 15 children (gaps 5-14cm)
Three Groups:
FT alone (mean gap length – 6.5cm)
KA + FT (mean gap length – 9.5cm)
Spit Fistula + FT (mean gap length – 6.5cm)

44. FT alone allowed primary repair of all patients in Group A with the lowest complication rate
In Group B, Kimura advancement compensated for the longer mean gap length
But complication rate was higher
Group C experienced the most serious complications (leakage, mediastinitis, perforation, inability to primarily repair)

45. Meta-analysis comparison of Foker to delayed primary anastomosis
Foker – 71 patients from 6 studies
Mean gap length – 5.4
DPA – 451 patients from 44 studies
Mean gap length – 3.6

46. Foker technique Lower risk of complications (leak, stricture, GRED)
Shorter time to definitive anastomosis

47. ?

48. References
Mahour GH, Woolley MM, Gwinn JL. Elongation of the upper pouch and delayed anatomic reconstruction in esophageal atresia. J Pediatr Surg 1974 Jun;9(3):
Woolley MM, Leix F, Johnston PW, Hays DM. Esophageal atresia types A and B: upper pouch elongation and delayed anatomicreconstruction. J Pediatr Surg 1969 Feb;4(1):
Gallo G, et al. Long-gap esophageal atresia: a meta-analysis of jejunal interposition, colon interposition, and gastric pull-up. Eur J Pediatr Surg 2012 Dec;22(6):420-5.
Sroka M, et al. The Foker technique (FT) and Kimura advancement (KA) for the treatment of children with long-gap esophageal atresia (LGEA): lessons learned at two European centers. Eur J Pediatr Surg 2013 Feb;23(1):3-7.
Loukogeorgakis SP, Pierro A. Replacement surgery for esophageal atresia. Eur J Pediatr Surg 2013 Jun;23(3):182-90
Nasr A, Langer JC. Mechanical traction techniques for long-gap esophageal atresia: a critical appraisal. Eur J Pediatr Surg. 2013 Jun;23(3):191-7.
Foker JE, et al. Development of a true primary repair for the full spectrum of esophageal atresia. Ann Surg. 1997 Oct;226(4):533-41; discussion
Foker JE, et al. A flexible approach to achieve a true primary repair for all infants with esophageal atresia. Semin Pediatr Surg. 2005 Feb;14(1):8-15.
Varjavandi V, Shi E. Early primary repair of long gap esophageal atresia: the VATER operation. J Pediatr Surg. 2000 Dec;35(12):
Kimura K, Soper RT. Multistaged extrathoracic esophageal elongation for long gap esophageal atresia. J Pediatr Surg. 1994 Apr;29(4):566-8.
Spitz L, Kiely E, Pierro A. Gastric transposition in children--a 21-year experience. J Pediatr Surg. 2004 Mar;39(3):276-81; discussion