1. Dr. Sam Chippington Martin Churchill-Coleman
OESOPHAGEAL ATRESIA
Dr. Sam Chippington
Martin Churchill-Coleman

2. Atresia - is the absence of a usual opening in a tubular structure
Embryology – the exact cause of oesophageal atresia is not known but both the oesophagus and trachea originate from the primitive foregut and this is thought to be a separation defect.
Occurrence – Approximately 1:4000 live births

3. PRENATAL DIAGNOSIS
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.

4. 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.

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

6. Small foetal stomach bubble
Umbilical vein
small gastric bubble
“collapsed”
Spine

7. FOETAL MRI
Some centres carry out 3rd trimester MRI – using single shot rapid acquisition T2 weighted images – the diagnosis is considered positive if the upper oesophagus is dilated and the lower oesophagus is not demonstrated

8. CLINICAL DIAGNOSIS
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 a replogle tube is passed – typically this will reach 10-12cm before reaching the blind end of the upper pouch.

11. TYPES OF OESOPAGEAL ATRESIA, TRACHEAL FISTULA OR COMBINATION OF THE TWO
5 TYPES OF MALFORMATION
87% have oesophageal atresia with distal tracheo-oesophageal fistula
8% Isolated atresia with no fistula
4% Isolated tracheo-oesophageal a ‘H’ Type fistula with no atresia
1% Atresia with upper pouch fistula
1% Atresia with upper and lower pouch fistula

12. NEONATAL ABDOMEN CHEST RADIOGRAPHS
CXR – will normally demonstrate a dilated upper pouch containing a replogle tube
ABDOMEN – a normal bowel gas pattern will be visible where there is connection via fistula to the lower oesophagus
Therefore there are only two types of malformation where the abdomen will be gasless – the isolated atresia without fistula and the fistula to the upper pouch of the oesophagus

13. Demonstrating an H type Fistula
SPINE
HEAD
FEET

14. 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 ‘H’ Type fistula present with recurrent chest infections or more dramatically when diving into a swimming pool
‘H’ Type fistulae are demonstrated by oesophagogram – taking a series of images with the patient prone and injecting water soluble contrast through a naso-gastric tube which is withdrawn from the stomach to the mouth so that the whole length of the oesophagus is interrogated.

15. HISTORY OF TREATMENT
Oesophageal 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.

16. ASSOCIATED DEFECTS
Associated congenital abnormalities are discovered in approximately one half of patients with oesophageal atresia
The acronym VACTERL has been used to describe the condition of multiple anomalies in these infants

17. LIMB defects – radial agenesis most common
VERTEBRAL – Hemivertebrae and scoliosis
ANORECTAL MALFORMATION
CARDIAC DEFECTS – VSD, Patent Ductus Arteriosus and Tretralogy of Fallot
TRACHEO
ESOPHAGEAL (American esophageal)
RENAL TRACT – Ectopic kidneys, horseshoe, duplex systems, renal agenesis, urethral malformations and hypospadias
LIMB defects – radial agenesis most common

18. In addition to the vacterl anomolies there is increased incidence of Duodenal atresia, malrotation, intestinal malformations, Meckel’s diverticulum and annular pancreas.

19. Oesophageal Atresia and Tracheo-Oesophageal Fistula

21. Air filled upper
oesophagus
Hemivertebra
Replogle tube

22. Surgical Repair Oesophageal Atresia First described 1703 22
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 22

23. Surgical Repair Oesophageal Atresia 1936 – First surgical repair
First described 1703
1936 – First surgical repair
1939 – First successful surgical repair
Gastrostomy inserted, delayed surgical repair
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. Surgical Repair Oesophageal Atresia 1936 – First surgical repair
First described 1703
1936 – First surgical repair
1939 – First successful surgical repair
Gastrostomy inserted, 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 24

25. Further Developments 1945-1965 By 1965 1965- 1990
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

27. Surgical
Anastomosis

28. Laparoscopic Repair Newest approach Fewer musculoskeletal sequelae
Winging of the scapula
Asymmetrical chest wall
Thoracic scoliosis
Breast maldevelopment
Asymmetrical chest wall – atrophy of serratus anterior 28

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

31. Surgical Options Staged repair Long gap oesophageal atresia
‘Stretch’ upper pouch
Mechanical
Electromagnetic
Wait for growth
Colonic Interposition
Defined as > 2 vertebral bodies or gap of > 3cm
Colonic interposition – usually ascending colon including the ileo-caecal valve
Ongoing risk of aspiration until repair performed 31

33. Colonic Interposition

34. Complications of Surgery
Acute -Anastomotic leak
Pathogenesis
Use silk sutures at the anastomosis
Excessive anastomotic tension
Excessive distal oesophageal mobilisation
Colonic interposition – graft necrosis
Sutures usually Vicryl
Not surprisingly more common in long gap – 54% compared with 11% in primary
Leak documented in up to 75% 34

35. Image anastomotic leak

36. 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

37. TOF image

38. 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-oesophageal reflux
Narrowing requiring at least 2 dilatations
Occurs in 75% of patients following anastomotic leak 38

39. Anastomotic stricture

40. Surgical Dilatation

41. Radiological Dilatation

43. Stenting

44. Long Term Complications
Oesophageal function
Disordered oesophageal motility
Barium swallow
Oesophageal function tests
Manometry
pH studies
Surgery restores gastro-oesophageal continuity but does not ensure normal oesophageal function

45. Abnormal oesophageal motor function
Oesophageal Function
Abnormal oesophageal motor function
No progressive peristalsis
Lack of co-ordinated peristaltic stripping wave
Oesophageal contractions simultaneous
Can involve whole oesophagus or distal two thirds
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
Oesophageal symptoms persist into adult life and sometimes severity can only be elicited by direct questioning

46. Moderate- severe gastro-oesophageal reflux
Oesophageal Function
Moderate- severe gastro-oesophageal reflux
Incompetent gastro-oesophageal sphincter
? Iatrogenic hiatus hernia
Oesophagitis
Symptoms persist into adult life
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

47. Abnormal oesophageal motor function
Oesophageal Function
Abnormal oesophageal motor function
No progressive peristalsis
Moderate- severe gastro-oesophageal reflux
Reflux and disordered motility
Oesophagitis
Pulmonary symptoms
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
Oesophageal symptoms persist into adult life and sometimes severity can only be elicited by direct questioning

48. Long Term Complications
Respiratory
Recurrent bronchitis
Persistent cough - ‘TOF’ cough
Pneumonia
Commonest first 8 years of life
Tracheomalacia (reported in up to 10%)
TOF cough 16-40% of adults
Tracheomalacia reported in 75% post mortem but clinically significant in about 10%
tracheomalacia is usually found at or just above level of the original EA/TEF, the site of collapse is most prominent in the distal one-third trachea in 60% of cases, and in the middle one third in 30%.1 The soft trachea is easily compressed between the aorta (or innominate artery) and the frequently dilated former esophageal pouch
Treatment for tracheomalacia is generally reserved for patients with dying spells or recurrent pneumonia. In one series,51 the indications for surgery were episodes of apnea and cyanosis in 65%, recurrent pneumonia in 25%, and worsening stridor in 46%. Aortopexy involves suturing the aorta to the posterior surface of the sternum. As the posterior aspect of the aorta adheres to the anterior aspect of the trachea, this draws the front wall of the trachea anteriorly, maintaining the tracheal lumen. This has been reported to have initial success rates of 35 to 88%

49. Recurrent inhalation of food
Respiratory Symptoms
Recurrent inhalation of food
Consequence of dysmotility and reflux
Fat seen in tracheal secretion aspirates
Association between oesophageal stricture and recurrent bronchitis
Defective motility causes delayed emptying, regurgitation and pooling of food in the oro-pharynx
In children dying in early post-op period, non-ciliated epithelium in major airways ?congenital ?due to reflux

50. Survival for patients with OA and TOF good
In Summary
Survival for patients with OA and TOF good
Symptoms relating to dysmotility and reflux have a significant impact
Frequently persist into adulthood