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OESOPHAGEAL ATRESIA

OESOPHAGEAL ATRESIA. Dr. Sam Chippington Martin Churchill-Coleman. Atresia - is the absence of a usual opening in a tubular structure

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OESOPHAGEAL ATRESIA

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  1. 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.

  9. 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

  10. 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

  11. Demonstrating an H type Fistula SPINE HEAD FEET

  12. 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.

  13. 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.

  14. 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

  15. 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 • LIMBdefects – radial agenesis most common

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

  17. Oesophageal Atresia and Tracheo-Oesophageal Fistula

  18. Air filled upper oesophagus Hemivertebra Replogle tube

  19. Surgical Repair Oesophageal Atresia First described 1703

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

  21. Surgical Repair 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

  22. Further Developments 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

  23. Surgical Anastomosis

  24. Laparoscopic Repair Newest approach Fewer musculoskeletal sequelae Winging of the scapula Asymmetrical chest wall Thoracic scoliosis Breast maldevelopment

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

  26. Surgical Options Staged repair Long gap oesophageal atresia ‘Stretch’ upper pouch Mechanical Electromagnetic Wait for growth Colonic Interposition

  27. Colonic Interposition

  28. Complications of Surgery Acute -Anastomotic leak Pathogenesis Use silk sutures at the anastomosis Excessive anastomotic tension Excessive distal oesophageal mobilisation Colonic interposition – graft necrosis

  29. Image anastomotic leak

  30. Complications of Surgery Late Missed TOF Present with recurrent chest infection Recurrent TOF Erosion through site of previous repair Anastomotic suture line leak

  31. TOF image

  32. 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

  33. Anastomotic stricture

  34. Surgical Dilatation

  35. Radiological Dilatation

  36. Stenting

  37. Long Term Complications • Oesophageal function • Disordered oesophageal motility • Barium swallow • Oesophageal function tests • Manometry • pH studies

  38. 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

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

  40. Oesophageal Function • Abnormal oesophageal motor function • No progressive peristalsis • Moderate- severe gastro-oesophageal reflux • Reflux and disordered motility • Oesophagitis • Pulmonary symptoms

  41. Long Term Complications • Respiratory • Recurrent bronchitis • Persistent cough - ‘TOF’ cough • Pneumonia • Commonest first 8 years of life • Tracheomalacia (reported in up to 10%)

  42. Respiratory Symptoms • Recurrent inhalation of food • Consequence of dysmotility and reflux • Fat seen in tracheal secretion aspirates • Association between oesophageal stricture and recurrent bronchitis

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

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