1. Prognostic Predictors of Esophageal Atresia Cases Two-Years Experience at Queen Rania Hospital for Children, King Hussein Medical Center.
Majed Sarayrah, MD, FACS.
Ahmad Abuqura, MD. Ola Haddad, RN. Ebtisam Sarayrah, RN
Yanal Abaza, MD Baha’ Abdullah, MD.
Queen Rania Al-Abdulla Hospital for Children.
King Hussein Medical Center, Amman-Jordan.

3. Objective:
To evaluate our experience in managing esophageal atresia cases highlighting the main factors influencing the outcome.
Methods:
Retrospectively, we reviewed the medical records of 52 cases of esophageal atresia over a period of two years, (2013 and 2014) at Queen Rania Al-Abdullah Hospital.
Follow up period was all through 2015.

4. Results:
Patients’ age of presentation ranged from one to eight days with a mean age of 3.5 days.
Most cases were type C variant.
20 cases (38%) were premature with a mean GA 33weeks and LBW with a mean birth weight of 1.5kg.
38 cases (73%) had associated congenital anomalies; the most common of them was heart defects, two cases had single ventricle.
VACTERL association was noticed in 5 cases (11%).
All cases had the same work up pre-operatively; cardiac echo and renal ultrasound in addition to routine labs.
Total mortality cases were 9 (17%), 7 of them were premature with major cardiac defects while the other 2 died due to leak and sepsis.

5. Postoperative gross leak was noted in 4 cases, 2 of them were reopened and the esophagus was excluded. Symptomatic anastomotic stricture was noted in 14 cases of the survivors (73%) treated by dilatation program. 2 cases had anti-reflux surgery due to refractory GERD after the age of 1 year. The follow up period ranged from 10-21months.

6. Conclusion:
Prematurity and major cardiac defects were the main bad prognostic predictors of outcome in treating esophageal atresia.

8. Definition:
Congenital anatomical discontinuity of the esophageus with or without communication to the trachea.
The exact cause of esophageal atresia is not known but both the oesophagus and trachea originate from the primitive foregut and this is thought to be a separation defect.

9. Epidemiology:
Finland 1 in 2440; US & Australia 1 in 4500 live birth.
62% male.
Black people have lower prevalence than white population.
Increased risk with first pregnancy, mother <20 yr.
Multiple birth: (TEF-3.7%; EA-TEF 4.9% EA 8.8% ).
Chromosomal anomalies 6%-10% (trisomy 18 & 21)
Empirical risk: 0.5%-2% recurrent risk in of one affected child; 20% if more than one sibling affected, 3%-4% in an affected child born to affected parents.

10. Ultrasound diagnosis is indicative rather than absolute
Antenatally:
Ultrasound diagnosis is indicative rather than absolute
Obstetric Ultrasound may show unexplained polyhydramnios
Absent stomach or small stomach
Early diagnosis allows time and place of delivery to be planned so that early paediatric surgical correction and neonatal care is available. This also gives time for preparation and education of the parents.

11. Normal foetal stomach Gastric bubble Spine Umbilical cord
Cross sectional view of fetal abdomen with vertebral column to left of the image, the gastric bubble above and the umbilical cord to the right.

12. Absence of foetal stomach bubble
Absence of gastric bubble
Umbilical cord
Spine

13. After birth these babies often appear to blow bubbles when attempting to swallow mucous and saliva
If these signs go unnoticed the first feed is almost always accompanied by coughing, choking and cyanosis.
In these cases N/G tube is passed – typically this will reach 10-12cm before reaching the blind end of the upper pouch.

14. CXR – will normally demonstrate a dilated upper pouch containing a coiled N/G tube.
ABDOMEN – a normal bowel gas pattern will be visible where there is connection via fistula to the lower oesophagus

15. The chest radiograph
A plain radiograph will confirm the tube has not reached the stomach

16. Therefore there are only two types of malformation where the abdomen will be gasless
– the isolated atresia without fistula.
the fistula to the upper pouch of the oesophagus.

17. The Gasless Abdomen
Absence of gas in the abdomen suggests that the patient has either atresia without a fistula or atresia with a proximal fistula only

18. DELAYED DIAGNOSIS
Diagnosis of four of the types is easily made after attempting to feed a new baby
However the ‘H’ Type Fistula because there is no atresia may not be identified for many years.
Often ‘N’ Type fistula present with recurrent chest infections or more dramatically when diving into a swimming pool.
‘N’ Type fistulae are demonstrated by esophagogram – taking a series of images with the patient prone and injecting water soluble contrast through N/G tube which is withdrawn from the stomach to the mouth so that the whole length of the oesophagus is interrogated.

20. Preoperative Investigations
A plain radiograph
Renal ultrasonography and echocardiography are routine preoperative investigations
Endoscopy or a careful contrast study performed in a tertiary center. In some centers, bronchoscopy is performed routinely in all infants with esophageal atresia.

21. HISTORY OF TREATMENT
Esophageal atresia is not compatible with life unless it is surgically repaired.
Before 1939 when the first successful repair took place this diagnosis was fatal.
Since that time surgical techniques and anaesthetics have improved and the great majority of these patients survive to lead a normal life.
Most patients nowadays undergo a primary anastomosis in the first few days of life.
Large gaps between the upper and lower pouches are problematic and used to be bridged by colon transplants and J tubes fashioned from the lesser curvature of the stomach.

22. Surgery
•1869 Timothy Holmes first suggested surgical anastomosis.
•First operative attempt:1888 Charles Steelle performed a gastrostomy, push a steel probe through the gastrostomy, simultaneously push down a bugie.

23. Surgical Repair Oesophageal Atresia First described 1703
1936 – First surgical repair
1939 – First successful surgical repair.Gastrostomy , delayed surgical repair.
1941 – First primary oesophageal anastomosis and ligation of tracheo-oesophageal fistula
1936 Thomas Lanman (American Surgeon Boston) . Patient Survived 3 hours.
1939 WilliaM Ladd - Boston(best known for the designing the operation still used for malrotation)dividing the tracheoesophageal fistula, marsupializing the upper esophagus to the neck, and creating a feeding gastrostomy
1941 -Dr. Cameron Haight of Ann Arbor, Michigan, was the first to perform anastomosis with repair in Patient spent 18 months in hospital, but was still alive in 2005 23

24. Further Developments 1945-1965
Focus on successful repair in otherwise healthy neonates (and birth weight > 2.5kg)
By 1965
Success rate 80-90%
Refinement of the procedure
Low birth weight
Co-existing morbidity
Mortality highest in low birth weight <1.5kg and associated major congenital heart disease
Improvements in survival largely attributable to improvements in
Neonatal intensive care
Anaesthetic management
Ventilatory support
Surgical techniques

25. Associated Abnormalities
Incidence of Associated Anomalies in Esophageal Atresia.
Anomaly Frequency(%)
Congenital heart disease
Urinary tract
Orthopaedic (mostly vertebral and radial)
Gastrointestinal (e.g., duodenal
atresia,imperforate anus)
Chromosomal (usually trisomy 18 or 21)
Total with one or more associated
anomalies

26. System affected
Musculoskeletal
Gastrointestinal
Cardiac
Genitourinary.
Potential anomalies
Hemivertebrae, radial dysplasia or amelia, polydactyly, syndactyly, rib malformations, scoliosis, lower limb defects
Imperforate anus, duodenal atresia, malrotation, intestinal malformations, Meckel's diverticulum, annular pancreas
VSD, PDA, tetralogy of Fallot, ASD, single umbilical artery, right-sided aortic arch
Renal agenesis or dysplasia, horseshoe kidney, polycystic kidney, ureteral and urethral malformations, hypospadias

27. DiGeorge Sequence.
Polysplenia sequence.
Holt-Oram Syndrome.
Peirre Robin Sequence.
Feingold Syndrome.
Fanconi Syndrome.
Townes-Brock Syndrome.
Bartsocas-Papas Syndrome.
McKusic

28. Surgical Options Primary Esophageal anastomosis
Within 48 hours of birth
Delayed primary Esophageal anastomosis
Unfit for surgery
Other associated anomalies e.g. cardiac

29. Type of Surgery:
Thoracotomy:
Extra-pleural
Trans pleural

30. Comparison of the techniques:
Thoracotomy:
Scoliosis.
Winged scapula.
Chronic pain.
Shoulder weakness.
Chest wall deformity.
Maldevelopment.

31. VAT: Superior visualization, Improved cosmesis.
Limited working space, lack of articulation of instruments, tying suture under tension.
2-4 H op time, 30-50% stenosis, 12-15% leak, 30-50% GER.
Feasibility

32. Thoracoscopic Repair:
Newest approach
Fewer musculoskeletal sequelae
Winging of the scapula
Asymmetrical chest wall
Thoracic scoliosis
Breast maldevelopment
Asymmetrical chest wall – atrophy of serratus anterior 32

33. Complications of Surgery
Acute -Anastomotic leak
Pathogenesis
Use silk sutures at the anastomosis
Excessive anastomotic tension
Excessive distal esophageal mobilisation
Colonic interposition – graft necrosis
Reintubation in the ICU.
Missed another fistula
Sutures usually Vicryl
Not surprisingly more common in long gap – 54% compared with 11% in primary
Leak documented in up to 75% 33

34. Anastomotic leak:
Mostly managed with adequate drainage & nutritional support, 95% close spontaneously.
Major disruption occur 3% to 5% of postoperative leak, typically recognized early
Factors for leak:
Poor surgical technique
Ischemia of esophageal ends
Use of myotomy
Excessive tension at anastomoticsite
Reoperation: control sepsis with drainage and repair by pleural or pericardial patch, with or with out intercostals muscle flap buttress.

35. Complications of Surgery
Late
Missed TOF
Present with recurrent chest infection
Recurrent TOF
Erosion through site of previous repair
Anastomotic suture line leak
2-18 months post repair. Seen in approx 10%
Ligation rather than complete division increases the likelihood of recurrent TOF
Instrumentation eg NG tubes can produce recurrent TOF

36. Complications of Surgery
Late
Anastomotic Stricture -40% of repairs.
Pathogenesis
Anastomotic leak
Two layer anastomosis
Anastomosis under tension
Silk sutures
Gap length at presentation
Associated gastro-esophageal reflux
Narrowing requiring at least 2 dilatations
Occurs in 75% of patients following anastomotic leak 36

37. Anastomotic stricture:
Spitz & hitchcock defined stricture as the presence of symptoms: dysphagia & recurrent respiratory probles from aspiration or forgine body obstruction & narrowing noted on endoscopy.
Stricture require dilatation occur up to 80% of patient.
Factors responsible: Poor anastomotic technique: excessive tension, two-layered anastomosis, silk suture, Ischeamia at ends, GER, Anastomotic leak.
Traditionally treated by dilatation: antegrade or retrograde bouginage using Savory dilator, Gruntzig balloon dilator
53% respond to single dilatation in the first month.

38. Long Term Complications
Esophageal function
Disordered Esophageal Motility
Barium swallow
Esophageal function tests
Manometry
PH Studies.
Surgery restores gastro-oesophageal continuity but does not ensure normal oesophageal function

39. Continued,,, Moderate- severe GERD, Reflux and disordered motility
Esophagitis
Pulmonary symptoms
Incompetent gastro-esophageal sphincter
? Iatrogenic hiatus hernia
Reflux due to incompetence of the gastro-oesophageal sphincter +/- ? Due to mobilisation of the distal oesophagus causing an iatrogenic hiatus hernia
Seen in up to 60% patients
Reflux + disordered motility = impaired acid clearing ability
Long term success rate of anti-reflux surgery is not good, if recurrent or life threatening respiratory symptoms or impaired nutrition then little choice other than operative intervention – required in up to 25% of patients 6-21/12 of age
Oesophageal symptoms persist into adult life and sometimes severity can only be elicited by direct questioning

40. 14% incidence of tracheobronchial abnormalities: ectopic or absent right upper lobe, congenital bronchial stenosis and a decreased ratio of circumferential cartilaginous trachea to membranous trachea.
EA associated with abnormalities of Vagal nerve innervations that are not related to surgical intervention.

41. Outcome:
Between 1939 to 1969 Dr. Haight did 284 infants with EA and report as 52% overall survival rate.
85-95% overall survival.
H-type fistula have less association.
VACTERL association have high mortality .

42. Risk factors & Survival Group
Waterston Classification
A 100% Birth wt >2500gm, otherwise healthy
B 85% Birth wt ,otherwisewell, or
Higher wt with moderate anomaly (non cardiac anomaly plus PDA, VSD, ASD)
C 65% Birth wt< 2000gm & otherwise well, or
Higher wt with sever associated cardiac anomaly

43. Predictor of survival group
Spit’s
I Birth weight>1500g with out major congenital heart disease 97%
II Birth weight <1500 g or major congenital heart disease 59%
III Birth weight < 1500 g and congenital heart disease 22%

44. Risk for death:
Inceased risk of death & long term morbidity include:
1.Lower birth wt (<1500 g) & prematurity.
2.Major CHD.
3.Sever associated anomaly & ventilator dependent
4.Long gap EA.
Delayed presentation, aspiration and pneumonia
Anastomotic leak and chest sepsis.

45. In Summary Survival for patients with EA and TOF is Excellent
Goal is 100% survival in a full term NN with B.W >2.5 Kg.
Postoperative care is as important as surgery
Symptoms relating to dysmotility and reflux have a significant impact thus, consider Fundoplication.