1. Esophageal Motility Disorders Iskander Al-Githmi, MD, FRCSC, FRCSC (Ts & CDS), FACS, FCCP Consultant & Asst. Professor of Cardiothoracic Surgery Consultant & Asst. Professor of Cardiothoracic Surgery King Abdulaziz University College of Medicine King Abdulaziz University College of Medicine

2. Anatomy of The Esophagus The esophagus is a hollow muscular organ, approximately 25cm in length that extend from the pharynx to the stomach The pharynx is a muscular tube, approximately 12cm in length that serve as entry to the esophagus and respiratory tract.

3. Anatomy of The Esophagus Cervical Esophagus: Just lies to the left of midline behind the larynx and the trachea. The entry to esophagus called upper esophageal sphincter (UES). Thoracic Esophagus: The upper part passes behind the carina & Lt. main stem bronchus. The lower part passes behind the left atrium. Abdominal Esophagus: Is the smallest portion of the esophagus (2-4cm length). It has lower esophageal sphincter (LES)- non anatomical with normal resting pressure 10-20mmHg.

4. Anatomy of The Esophagus Normal esophageal narrowing: UES at the level of cricoid cartilage 14mm in diameter. UES at the level of cricoid cartilage 14mm in diameter. Broncho-aortic constriction 17mm in diameter. Broncho-aortic constriction 17mm in diameter. LES (19mm) as it travels the diaphragm & located 3-5cm at distal part of the esophagus. LES (19mm) as it travels the diaphragm & located 3-5cm at distal part of the esophagus. Clinical Importance of normal esoph. narrowing: Clinical Importance of normal esoph. narrowing: Potential for development of diverticulum's (Zenker) in the neck. Potential for development of diverticulum's (Zenker) in the neck. Potential for perforation during esophagoscopy Potential for perforation during esophagoscopy Pills-induced stricture. Pills-induced stricture.

5. Anatomy of The Esophagus The esophageal wall: The proximal esophagus is predominantly striated muscle. The proximal esophagus is predominantly striated muscle. The distal esophagus is predominantly smooth muscle. The distal esophagus is predominantly smooth muscle. The mid esophagus contained a graded transition of striated and smooth muscle. The mid esophagus contained a graded transition of striated and smooth muscle.

6. Anatomy of The Esophagus The esophageal wall: The muscle oriented in two perpendicular opposing layers an inner circular layer and outer longitudinal layers both called muscularis propria. The muscle oriented in two perpendicular opposing layers an inner circular layer and outer longitudinal layers both called muscularis propria. The outermost layer of the esophagus called adventitia (fibro-areolar layer), but no serosa. This may contribute for cancer spread. The outermost layer of the esophagus called adventitia (fibro-areolar layer), but no serosa. This may contribute for cancer spread. Underneath the adventitia there is a longitudinal muscle layer and beneath there is circular layer. Underneath the adventitia there is a longitudinal muscle layer and beneath there is circular layer. Between the two muscle layers there are network of sympathetic and parasympathetic fibers (myentric plexus) Between the two muscle layers there are network of sympathetic and parasympathetic fibers (myentric plexus)

7. Anatomy of The Esophagus The esophageal wall: Beneath the muscle layers lies the submucosa which contain mucus gland, blood and lymphatic vessels and network works of nerve fibers (meissners). Beneath the muscle layers lies the submucosa which contain mucus gland, blood and lymphatic vessels and network works of nerve fibers (meissners). Beneath the submucosa is the mucosa which consist of squamous epithelium except the distal 2cm at G-E junction (Z-line) or transition to columnar epithelium. Beneath the submucosa is the mucosa which consist of squamous epithelium except the distal 2cm at G-E junction (Z-line) or transition to columnar epithelium.

8. Anatomy of The Esophagus Blood supply & venous drainage Cervical esophagus received its arterial blood from inferior thyroid artery. Cervical esophagus received its arterial blood from inferior thyroid artery. Thoracic esophagus received its arterial blood from bronchial, aorta, left gastric artery and from inferior phrenic artery. Thoracic esophagus received its arterial blood from bronchial, aorta, left gastric artery and from inferior phrenic artery. The esophageal veins drain to periesophageal venous network & to inferior thyroid vein in the neck and to azygos and hemiazygos veins in the thorax. The esophageal veins drain to periesophageal venous network & to inferior thyroid vein in the neck and to azygos and hemiazygos veins in the thorax.

9. Anatomy of The Esophagus Lymphatic drainage: The lymphatic plexus are located in the mucosa and the muscular layers drained to mediastinal lymph nodes. The lymphatic plexus are located in the mucosa and the muscular layers drained to mediastinal lymph nodes. Clinical facts about the esophagus Cervical esophagus is 5 cm in length and 15cm distance from upper incisors Cervical esophagus is 5 cm in length and 15cm distance from upper incisors Thoracic esophagus is 12cm in length and 25cm distance from upper incisors Thoracic esophagus is 12cm in length and 25cm distance from upper incisors Lower esophagus is 2cm in length & 38cm from upper incisors Lower esophagus is 2cm in length & 38cm from upper incisors

10. Physiology of The Esophagus The function of the esophagus is to transport the ingested material from the pharynx to the stomach by peristaltic waves. Primary peristalsis: Triggered by the swallowing center in the brain stem and the contraction wave travel at speed 2cm/s. Secondary peristalsis: Induced by esophageal distension from retained bolus, refluxed material. Its role is to clear the esophagus form retained bolus.

11. Physiology of The Esophagus Tertiary peristalsis: Are non peristaltic contraction and play no known physiological role. Frequently observed in elderly people called (presbyesophagus), also seen in motility disorders.

12. Physiology of The Esophagus Mechanism of swallowing During the pharyngeal phase of swallowing, a primary peristalsis is created, that relax the UES and forces the food bolus through it. The UES remain constricted and has resting pressure of 20-60 mmHg. The peristaltic waves travel at the speed 2cm/s and reach the stomach in 5-10 second During the pharyngeal phase of swallowing, a primary peristalsis is created, that relax the UES and forces the food bolus through it. The UES remain constricted and has resting pressure of 20-60 mmHg. The peristaltic waves travel at the speed 2cm/s and reach the stomach in 5-10 second

13. Physiology of The Esophagus Secondary peristalsis get initiated if the primary peristalsis failed to get food to the stomach and the esophagus became distended. Secondary peristalsis get initiated if the primary peristalsis failed to get food to the stomach and the esophagus became distended.

14. Esophageal Motility Disorders Achalasia Spastic esophageal motility disorders such as diffuse esophageal spasm, nutcracker esophagus and hypertensive LES Secondary esophageal motility disorders related to scleroderma, diabetes, alcohol consumption..

15. Esophageal Motility Disorders Achalasia (failure to relax) Is the only esophageal motility disorder with an established pathology. Is the only esophageal motility disorder with an established pathology. The predominant pathophysiology of achalasia is the loss of Auerbach ganglion cells from the wall of the esophagus,starting at LES and progress proximally. The predominant pathophysiology of achalasia is the loss of Auerbach ganglion cells from the wall of the esophagus,starting at LES and progress proximally. Incidence is 1-3 / 100,000 population / year. Incidence is 1-3 / 100,000 population / year.

16. Esophageal Motility Disorders Achalasia (failure to relax) Characterized by failure of LES to relax completely during swallowing Characterized by failure of LES to relax completely during swallowing The loss of nerve ganglion along the esophageal wall cause a peristalsis leading to stasis of food and subsequent dilatation. The loss of nerve ganglion along the esophageal wall cause a peristalsis leading to stasis of food and subsequent dilatation. Manometry may reveal elevated LES pressure > 40 mmHg in 60% of patients. Manometry may reveal elevated LES pressure > 40 mmHg in 60% of patients.

17. Esophageal Motility Disorders Spastic esophageal motility disorders Diffuse esoph.spasm (DES): This is probably related to fragmental degeneration of vagal nerve fibers. Diffuse esoph.spasm (DES): This is probably related to fragmental degeneration of vagal nerve fibers. Characterized by simultaneous, repetitive high pressure muscular contraction within the esophagus. Characterized by simultaneous, repetitive high pressure muscular contraction within the esophagus. The muscular wall is thickened, hypertrophied and is hypersensitive to stretching. The muscular wall is thickened, hypertrophied and is hypersensitive to stretching.

18. Esophageal Motility Disorders Scleroderma esophagus Collagen vascular disease. Collagen vascular disease. Characterized by smooth muscle hypertrophy and mainly involve the distal 2/3 of esophagus gradually lead to loss of peristalsis and weakening of LES causing GERD. Characterized by smooth muscle hypertrophy and mainly involve the distal 2/3 of esophagus gradually lead to loss of peristalsis and weakening of LES causing GERD. Involve the esophagus in 80% of patient with scleroderma. Involve the esophagus in 80% of patient with scleroderma.

19. Esophageal Motility Disorders Clinical History  Achalasia: The hall mark is dysphagia to both solid and liquid. The hall mark is dysphagia to both solid and liquid. Regurgitation commonly occur at night Regurgitation commonly occur at night Retrosternal chest pain. Retrosternal chest pain. Heartburn occur in 30% of patients which may be related to food fermentation and lactic acid. Heartburn occur in 30% of patients which may be related to food fermentation and lactic acid.

20. Esophageal Motility Disorders Clinical History  Spastic motility disorders Chest pain is the hall mark which may mimic angina due to esophageal distension. Chest pain is the hall mark which may mimic angina due to esophageal distension. Dysphagia to both solid and liquid. Dysphagia to both solid and liquid.  Scleroderma Involve the esophagus in 80% of patients. Involve the esophagus in 80% of patients. Symptoms are related to GERD [dysphagia, heartburn and regurgitation]. Symptoms are related to GERD [dysphagia, heartburn and regurgitation].

21. Esophageal Motility Disorders Problems to be considered  Coronary Artery Disease (CAD).  Mechanical obstruction (tumor).  Achalaisa and scleroderma increase risk of esophageal cancer.

22. Esophageal Motility Disorders Diagnosis  History  Physical examination-unremarkable  Barium Swallow Bird peak appearance- classic for achalasia Rosary beads or corkscrew- classic for DES

24. Esophageal Motility Disorders Diagnosis  Esophagoscopy to rule out tumor or inflammatory lesion but not to diagnose esophageal dysmotility.  Manometry study is to evaluate the esophageal motor pattern, contraction amplitude and LES pressure.

25. Flexible Gastro-Esophagoscope

27. Esophageal Manometry Cath.

28. Esophageal Manometry

30. Achalasia

31. Esophageal Motility Disorders Treatment  The primary goal is symptomatic relief directed at relieving the physiologic obstruction at the level of LES by surgical or balloon dilatation.  Nitrate and Ca channel & B blockers are currently used in all patients with esophageal motility disorders.  Antireflux therapy e.g proton pump inhibitors (esomeprazol) + prokinetic such as motilium or erythromycin.

32. Esophageal Motility Disorders Treatment  Botulinum toxin injection (Botox): Injected edoscopically in 4 quadrants into LES in treating patient with achalasia. Botox is a potential inhibitor of acetylcholine release from nerve terminals. It is indicated in those pt. not candidate for surgery or refuse surgery.  Endoscopic balloon dilatation: This is the standard therapy for patients with achalasia.  The mechanism based on disruption of circular muscle.  Balloon dilatation response rate is 70%

33. Esophageal Motility Disorders Treatment  Surgery (Heller Myotomy): surgical treatment targets to disrupt the LES.  This can be performed thoracoscopic or laparascopic.  Outcome is excellent 80-100% response rate.

34. Normal Esophagus

35. Barrett Esophagus Risk factors Age Male GERD Smoking Definition: Intestinal metaplasia Treatment: Antireflux therapy Medical: Pump inhibitors (esomeprazole) Prokinetic meds (Motilium) Annual Surveillance (esophagoscopy) Surgical: Fundoplication + Annual Surveillance Complications: Dysplasia Adenocarcinoma <1%/Yr