The oesophagus - Congenital anomalies of the oesophagus.

An overview of the various congenital anomalies of the esophagus

1. Oesophagus BRIEF INTRODUCTION -Also food pipe -Transports food from mouth to the stomach

2. CONGENITAL ANOMALIES - Has been known to occur in 1 child per 5000 births -Develop prenatally or may be identified in later stages of infancy or life . OCCURENCE

3. CONGENITAL ANOMALIES Oesophageal atresia(EA) Atresia - A condition in which an orifice or passage in the body is closed or absent Tracheoesophageal fistula(TEF ) Fistula – An abnormal connection between organs TYPES Pathology of Gastrointestinal Tract

4. A n oesophageal disorder where a baby's oesophagus is not fully developed; therefore an infant cannot pass food from the mouth to the stomach . C an occur singularly, but mainly occurs with tracheoesophageal fistula or other defects in the kidney, heart, ribs, digestive system or spinal column. C ause is unknown. H as 4 types; Type A, B, C and D . F actors that may increase the chances are other anal or intestinal defects, defective genes, paternal age ( in some cases) and use of reproductive technologies. Esophageal Atresia

5. Outcomes as per the type of esophageal atresia

6. Types of EA - Upper and lower parts of oesophagus have closed ends . -No attachment to the trachea. Type A - Upper part of the oesophagus attached to the trachea and lower part closed end. Type B Pathology of the Gastrointestinal tract Type C Type D - Most common type -Upper part closed ends, lower part attached to trachea. - Most rare and severe -Upper and lower parts not connected; but each separately connected to trachea.

7. VISUAL REPRESENTATION Fig 1.1 Drawing of an infant with esophageal atresia Type B. Figure 1.2 Representations of the Types of esophageal atresia versus normal anatomy

8. SYMPTOMS Pathology of the Gastrointestinal tract D ifficulty in breathing B lue skin colour when baby is feeding C oughing and choking when feeding F rothy white bubbles in baby's mouth

9. Pathology of the Gastrointestinal tract M ainly detected after birth during feeding X-ray to show tube inserted through the mouth cannot pass to the stomach I n rare cases, can be diagnosed through prenatal ultrasound DIAGNOSIS

10. Surgery that connects the oesophageal gap. PRIMARY ANAMOSTOSIS JEJUNAL INTERPOSITION Using part of the middle part of the small intestine(jejunum) to replace the missing part of the oesophagus . Technique that stimulates upper and lower ends to grow. Offered only in one hospital worldwide. FOKER PROCESS TREATMENT Pathology of the Gastrointestinal tract Oesophageal growth that reduces time under anaesthesia STATIC AND DYNAMIC INTERNAL TRACTION

11. Tracheoesophageal fistula C ongenital or acquired connection of the trachea and oesophagus C an cause food or liquids to pass into the trachea causing other conditions O ccurs in one in 5000 births and alongside other birth defects C auses include: iatrogenic injury, neck trauma or prolonged ventilation through a tracheostomy tube . Types of TEF: most common are five

12. Types of TEF Type 3A- oesophagus ends on lower trachea Type 3B- oesophagus arising from lower trachea Type 3C- oesophagus is both arising and terminated on lower trachea

13. Type H/E A variant of Type 3C; where the proximal oesophagus termination in the lower trachea and distal oesophagus arising from the lower trachea. Pathology of the Gastrointestinal tract Fig 1.3 showing TEF Type H(also known as Type E)

14. Pathology of the gastrointestinal tract

15. Pathology of the Gastrointestinal tract Diagnosed via MRI scan where the atretic oesophagus , its position and anomaly are clarified . M ostly detected after birth when baby fails to swallow food I n some cases, failure of the tracheoesophageal ridges to fuse can cause TEF . DIAGNOSIS

16. SYMPTOMS Pathology of the Gastrointestinal tract Dysphagia C oughing and wheezing G astro-intestinal reflux disease. L eak of contents at point of anastomosis. Tracheo-malacia . S tricture as a result of gastric acid erosion. R ecurrent chest infections.

17. Resection of any fistula Anamostosis of any discontinued sections NEONATAL SURGERY INTERVENTIONAL THERAPY Not surgical Done by local injection of glue either biological or chemical. Insertion of a metallic or plastic tube to keep body pathways open ESOPHAGEAL STENTING TREATMENT Pathology of the Gastrointestinal tract

18. Hiatal Hernia Anatomic disorder where the upper part of the stomach bulges through the diaphragm. Two anatomic patterns: 1. Axial (or sliding) hernia and 2. Nonaxial (or paraesophageal ) hernia. 95% cases are sliding hernia and 5% paraesophageal . Sliding hernia – protrusion of the stomach above the diaphragm creates a bell-shaped dilation. Paraesophageal hernia – a separate portion of the stomach, enters the thorax. Occurrence – reported in 1 to 20% of adults.

19. Causes & Risk Factors Causes A hiatal hernia might be caused by: Age-related changes in your diaphragm Injury to the area, for example, after trauma or certain types of surgery Being born with an unusually large hiatus Persistent and intense pressure on the surrounding muscles, such as while coughing, vomiting, straining during a bowel movement, exercising or lifting heavy objects Risk Factors: Over 50 years of age Obese

20. SYMPTOMS Pathology of the Gastrointestinal tract Mucosal ulceration Bleeding Perforation in the oesophagus Heartburn Regurgitation of gastric juices Reflux esophagitis ( in sliding hiatal hernia) Obstructed oesophagus (in paraesophageal hiatal hernia)

21. DIAGNOSIS X-Ray of the upper digestive system- after drinking a chalky liquid to coat digestive tract Upper endoscopy- using a camera to examine for inflammation or obstruction Esophageal manometry - measures the rhythmic muscle contraction in oesophagus when one swallows.

22. TREATMENT Surgery Antacids that neutralize stomach acid. Antacids, such as Mylanta, Rolaids and Tums. Medications to reduce acid production e.g C imetidine, F amotidine. Medications that block acid production and heal  the oesophagus e.g Lansoprazole and Omeprazole.

23. ACHALASIA Achalasia – means failure to relax Incomplete relaxation of the lower esophageal in response to swallowing. Produces functional obstruction of the oesophagus. Major abnormalities in achalasia: Aperistalsis Incomplete relaxation of esophageal sphincter with swallowing Increased resting tone of the lower esophageal sphincter There is primary and secondary achalasia

24. CAUSES May be hereditary or autoimmune Can be caused by degeneration of the esophageal nerves. Is a common disease in South America. Risk individuals: Older people Individuals with autoimmune disorders

25. SYMPTOMS Dysphagia or Trouble swallowing Pain or discomfort in your chest Weight loss Coughing Heartburn I ntense pain or discomfort after eating

26. Pathology of the Gastrointestinal tract Esophageal manometry  to diagnose achalasia. X-ray or similar exam of your oesophagus . Endoscopy Barium swallow. If you have this test, you’ll swallow barium prepared in liquid form. Your doctor will then track the barium’s movement down your esophagus through X-rays . DIAGNOSIS

27. TREATMENT Esophagomyotomy Pneumatic dilation to widen the sphincter or alter it. Botox to relax the sphincter. It is injected using an endoscope. Nitrates or Calcium channel blockers to relax the sphincter.

28. Lacerations (Mallory-Weiss Syndrome) A tear of the mucuous membrane at the point where oesophagus and stomach meet. Often caused by violent vomiting and coughing Most tears heal within 7 to 10 days without treatment.

29. SYMPTOMS Abdominal pain Hematemesis – vomiting blood Involuntary retching Black or bloody stools

30. ENDOSCOPIC THERAPY are two: Injection therapy & Coagulation therapy SURGERY FAMOTIDINE OR LANSOPRAZOLE Medication

31. Varices Abnormal, enlarged veins in the oesophagus . Occurs most often in people with liver diseases. Develop when normal blood flow to the liver is blocked by a clot or scar in the tissue Usually no symptoms; unless the veins bleed. Types of TEF: most common are five

32. Risk Factors High portal blood pressure – bigger portal blood pressures, Large varices – risk of bleeding increases varices. Severe liver disease – advanced cirrhosis or liver disease. Ongoing alcohol consumption – in patients with varices, drinking more increases risk of bleeding.

33. SYMPTOMS Pale, clammy skin Hematemesis – vomiting blood Black or bloody stools Irregular breathing

34. Diagnosis Regular screening Esophagastroduoendoscopy CT scan MRI scan

35. TREATMENT Surgery Variceal ligation - tying the varices with bands to stop blood flow. Transjugular intrahepatic portal-systemic shunting (TIPS) - using a catheter to stop portal blood pressure in oesophagus