bringing excellence to life oesophageal atresia dr. sam chippington martin churchill-coleman
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Bringing excellence to life
OESOPHAGEAL ATRESIA
Dr. Sam ChippingtonMartin Churchill-Coleman
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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
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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.
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Normal foetal stomach
Umbilical cord
Gastric bubble
Spine
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.
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Absence of foetal stomach bubble
Umbilical cord
Absence of gastric bubble
Spine
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Small foetal stomach bubble
Umbilical vein
small gastric bubble
“collapsed”Spine
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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
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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.
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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
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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
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Demonstrating an H type Fistula
HEAD FEET
SPINE
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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.
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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.
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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
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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
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In addition to the vacterl anomolies there is increased incidence of Duodenal atresia, malrotation, intestinal malformations, Meckel’s diverticulum and annular pancreas.
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Air filled upper oesophagus
Replogle tube
Hemivertebra
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Oesophageal Atresia First described 1703
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Oesophageal Atresia First described 1703
1936 – First surgical repair 1939 – First successful surgical
repair Gastrostomy inserted, delayed
surgical repair
Bringing excellence to life
Oesophageal Atresia 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
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1945-1965 Focus on successful repair in
otherwise healthy neonates (and birth weight > 2.5kg)
By 1965 Success rate 80-90%
1965- 1990 Refinement of the procedure
Low birth weight Co-existing morbidity
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Surgical Anastomosis
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Newest approach
Fewer musculoskeletal sequelae Winging of the scapula Asymmetrical chest wall Thoracic scoliosis Breast maldevelopment
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Primary oesophageal anastomosis Within 48 hours of birth
Delayed primary oesophageal anastomosis Unfit for surgery Other associated anomalies
e.g.cardiac
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Staged repair Long gap oesophageal atresia
‘Stretch’ upper pouch Mechanical Electromagnetic
Wait for growth Colonic Interposition
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Acute -Anastomotic leak
Pathogenesis Use silk sutures at the anastomosis Excessive anastomotic tension Excessive distal oesophageal
mobilisation Colonic interposition – graft necrosis
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Late
Missed TOF Present with recurrent chest infection
Recurrent TOF Erosion through site of previous
repair Anastomotic suture line leak
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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
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Anastomotic stricture
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Long Term Complications
Oesophageal function Disordered oesophageal motility
Barium swallow Oesophageal function tests
Manometry pH studies
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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
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Oesophageal Function
Moderate- severe gastro-oesophageal reflux
Incompetent gastro-oesophageal sphincter
? Iatrogenic hiatus hernia
Oesophagitis Symptoms persist into adult life
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Oesophageal Function
Abnormal oesophageal motor function No progressive peristalsis
Moderate- severe gastro-oesophageal reflux
Reflux and disordered motility Oesophagitis Pulmonary symptoms
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Long Term Complications
Respiratory
Recurrent bronchitis Persistent cough - ‘TOF’ cough Pneumonia
Commonest first 8 years of life
Tracheomalacia (reported in up to 10%)
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Respiratory Symptoms
Recurrent inhalation of food Consequence of dysmotility and
reflux Fat seen in tracheal secretion
aspirates
Association between oesophageal stricture and recurrent bronchitis
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In Summary
Survival for patients with OA and TOF good
Symptoms relating to dysmotility and reflux have a significant impact Frequently persist into adulthood