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

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

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

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

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

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

Classification

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

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

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

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

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

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

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

Gap-o-g ram

Gap-o-g ram

Gap-o-g ram

Bronchoscopy/Esophagoscopy

Timing of Intervention Waterston - 1962 Category Birth Wt Pneumonia Anomalies Timing A >2.5kg - Immediate B 1.8-2.5kg + Delayed C <1.8kg ++ Staged

Timing of Intervention Poeneru - 1993 Class On Vent Anomalies Timing I N none/minor Immediate II Y major life-threatening Delayed

Timing of Intervention Spitz - 1994 Group Birth Wt Cardiac dz Timing I >1.5kg - Immediate II <1.5kg + Delayed III Staged

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%

Operative Planning Primary Anastomosis Delayed/Staged Primary Anastomosis Esophageal replacement

Operative Planning Primary Anastomosis Delayed/Staged Primary Anastomosis Esophageal replacement

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

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

Operative Planning Primary Anastomosis Delayed/Staged Primary Anastomosis Esophageal replacement

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

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.”

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.”

Foker Technique

Foker Technique

Operative Planning Primary Anastomosis Delayed/Staged Primary Anastomosis Esophageal replacement

Colonic Interposition

Gastric Tube Isoperistaltic Retroperistaltic

Gastric Tube

Gastric Transposition

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

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

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

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)

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)

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

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

?

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):373-83. 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):148-53. 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 541-3. 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):1830-2. 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 276-81.