BOLADE MIDE Nov 09

Disorders of esophageal motility are functional disorders that interfere with the normal act of swallowing or produce dysphagia without any associated intraluminal organic obstruction or extrinsic compression. They have abnormal manometric patterns.

Primary Esophageal Motility Disorders Achalasia, "vigorous" achalasia Diffuse and segmental esophageal spasm Nutcracker esophagus Hypertensive lower esophageal sphincter Nonspecific esophageal motility disorders Secondary Esophageal Motility Disorders Collagen vascular diseases: progressive systemic
sclerosis, polymyositis and dermatomyositis, mixed connective tissue disease, systemic lupus erythematosus, et al Neuromuscular diseases: multiple sclerosis, Chagas dx Endocrine and metastatic disorders: DM, myxoedema,

LES pressure 1525 mm Hg (never > 45 mm Hg) with normal relaxation with swallowing Mean amplitude of distal esophageal peristaltic wave 30100 mm Hg (never > 180 mm Hg) Simultaneous contractions occurring after < 10% of wet swallows Monophasic waveforms (with no more than two peaks) Duration of distal esophageal peristaltic wave: 26 sec No repetitive contractions

The term achalasia is of Greek derivation and literally means failure or lack of relaxation. Achalasia is primarily a disorder of motility of the lower oesophageal or cardiac sphincter. Achalasia is the most common functional disorder of the esophageal body and LES, There is increased pressure of LES $ absent or reduced peristalsis of the oesophageal body

The tone and the activity of the sphincter is controlled by a balance of excitatory transmitters such as acetylcholine and substance P and inhibitory transmitters such as nitric oxide and vasoactive intestinal peptide (VIP).

Achalasia tends to present in adult life between about 25 and 40 years old with less than 5% occurring in children. A study of achalasia in Cardiff between 1926 and 1977 found an incidence of 4 to 6 per million.

Aetiology is unknown it may be secondary to ganglionic dysfunction of the myenteric plexus. Likewise, injury to the esophageal myenteric plexus by cold, heat, chemicals, or excision Chagas disease (American trypanosomiasis)

loss of ganglion cells in Auerbachs plexus, increased resting LES tone lower esophageal sphincter fails to relax dilatation of esophageal body & muscular hypertrophy of esophageal wall chronic functional obstruction with intraluminal stasis.

The myenteric plexus innervates the longitudinal and circular smooth muscle layers and is concerned primarily with motor control The tone of the LES is under neural control. Release of acetylcholine from vagal endings causes the sphincter to contract, and release of NO and VIP causes it to relax.

The main symptom is increasing dysphagia, which may at first be intermittent, but soon becomes progressive. Regurgitation of food is often a prominent symptom. It usually occurs at night but at times after every meal and made worse by bending or lying flat. Halitosis is a disturbing problem.

Retrosternal pain which may radiate to the neck and interscapular and subcostal regions.
Weight loss

Achalasia often results in recurrent respiratory symptoms related to aspiration, such as pneumonia. Marked distention of the dilated esophagus may produce dyspnea from compression of the mainstem bronchi and hilum.

In the early stages of achalasia, the patient notes a sticking sensation, usually at the level of the xiphoid, after ingestion of liquids, and later solids. Patients eat slowly, use large volumes of water to wash food into the stomach, and may twist the upper torso, elevate the chin and extend the neck, or walk in an effort to aid esophageal emptying. As more water is swallowed, the weight of the fluid column in the esophagus increases until the LES is forced open, with sudden relief as the esophagus empties. Dysphagia progresses slowly and is well tolerated for many years. Therefore, patients with achalasia often do not seek medical attention until progressive dysphagia interferes with their lifestyle.

Chest x-ray:
May possibly show signs of aspiration. The classical picture of a CXR in achalasia shows a

dilated oesophagus behind the heart. There is usually a retrocardiac air-fluid level in the esophagus from the retained food and saliva The gastric air bubble may be small or absent.

 The barium swallow in achalasia is characteristic. A

dilated esophagus with a tapering, beak-like narrowing of the distal end. Later stages demonstrate massive dilatation, tortuosity, and a sigmoid-shaped esophagus, often termed a megaesophagus. Retained intraesophageal food contents are typically seen. Weak, irregular, unco-ordinated or absent peristalsis on fluoroscopy.

 Manometry is the gold standard for diagnosis of

achalasia.

May show diagnostic features

- there is a high resting pressure in the cardiac sphincter, - incomplete relaxation on swallowing and - absent orreduced peristalsis in body

If manometry is normal but clinical symptoms or radiological evidence suggest achalasia, a condition called pseudoachalasia may be present. Causes include oesophageal and gastric malignancies and other tumours involving the distal oesophagus. Endoscopy with biopsy and CT can be helpful.
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Indications include to evaluate the severity of esophagitis, the possibility of associated carcinoma, a distal esophageal stricture from reflux esophagitis, or a tumor of the cardia mimicking achalasia (pseudoachalasia). The oesophagus contains food debris and is dilated. The instrument is passed through the cardia into the stomach without any difficulty. 5 - 10% of achalasia may have associated carcinoma.

 May also be required to exclude gastro-

oesophageal reflux disease (GORD) that often occurs with achalasia

The haemoglobin, serum electrolytes and blood urea may be normal or slightly abnormal. The white blood cell count may be elevated as a result of pulmonary infection.

1.

Pulmonary disease: Recurrent aspiration of food

particles and saliva into the tracheobronchial tree may cause pneumonitis, pneumonia, chronic bronchitis, emphysema, or lung abscess.

2. Carcinoma of the oesophagus. Long-standing

mucosal irritation from retention esophagitis appears to induce the metaplasia &may occur even after successful

operation. Achalasia is a premalignant esophageal lesion, with carcinoma developing as a late complication in 1 - 10% of patients who have this condition for an average of 15 - 25 years.

3. Nutritional deficiencies: Avitaminosis, anaemia,

The aim is to reduce the pressure of the L.O.S. so as to allow food to pass into the stomach unimpeded.

NON OPERATIVE TREATMENT Calcium channel blockers,BOTOX and nitrates are effective in only about 10% of patients and tend to be reserved for those who are unable to tolerate other forms of treatment.

 Forceful dilatation (pneumatic or hydrostatic). A balloon is inserted into the lower oesophagus via an endoscope and it is inflated to rupture the muscle of the oesophagus whilst leaving the mucosa intact. The success rate is 70 - 80% with 5% rate of perforation. Endoscopic injection of botulinum toxin (inhibits acetylcholine release). only 30% of patients have satisfactory relief at the end of 1 year.

Open or laparoscopic Trans-thoracic and trans-abdominal approaches. The goal of treatment is to incise anteriorly the longitudinal and circular muscle of the distal esophagus. Surgical intervention is indicated after failure of repeated dilatation, in mega-oesophagus, when associated carcinoma is suspected or as first line treatment.

A longitudinal incision is made in the serosal and muscular layers of the lower 10-l2cm of the oesophagus, just to the oesophagogastric junction. The myotomy should extend to include the L.O.S. but not into the gastric wall. The mucosa bulges through the incised muscle fibres. Care must be taken not to puncture the mucosa. The muscle fibres must be completely divided

Transhiatal oesophagectomy with cervical oesophago-gastric anastomosis should be strongly considered in symptomatic patients with tortuous megaesophagus, failure of prior myotomy, or undilatable reflux stricture. Esophageal resection provides definitive treatment of the esophageal abnormality and eliminates the late risk of carcinoma.

Other causes of dysphagia, including:

oesophagus Gastro-oesophageal reflux Plummer-Vinson (Patterson Brown-Kelly) syndrome

Rozycki syndrome is deafness, short stature, vitiligo, muscle wasting, and achalasia.

A complication of American trypanosomiasis (Chaga's disease).

Oesophageal spasm is a hypermotility disorder of oesophagus. The oesophagus normally propels food from the upper oesophageal sphincter towards the stomach through waves of coordinated muscle contraction, or peristalsis. When these waves do not progress normally, oesophageal spasm can result.

Oesophageal spasm can be divided into: Diffuse oesophageal spasm: There are uncoordinated oesophageal contractions. Several sections of the oesophagus can contract at once and food is unable to pass normally. Nutcracker oesophagus: The contractions are coordinated but with an excessive amplitude. Food can usually pass down the oesophagus but pain is common.1

Exact prevalence unknown as symptoms may be mild and go undiagnosed Can affect all age groups but more common in elderly Women > men

There is no clear aetiology. There may be a dysfunction of neural control, perhaps exacerbated by stress. Microvascular compression of the Vagus nerve in the brainstem has also been postulated.1

Dysphagia: Intermittent dysphagia is the predominant symptom. Chest pain: episodes of severe, crushing central retrosternal pain. Oesophageal spasm is often initially diagnosed as angina pectoris. Pain is due to bolus impaction and/or strong contraction of the oesophageal muscle. It is precipitated by eating, occasionally by anxiety and may be severe, crushing and radiate to the back, neck or even upper limbs. Globus: The sensation of something stuck in throat. Regurgitation of food Heartburn: Occurs in 20% of patients.

Myocardial infarction Oesophageal web, ring or stricture Oesophageal carcinoma Gastro-oesophageal reflux Oesophageal perforation/mediastinitis Achalasia

This can confirm the diagnosis of diffuse oesophageal spasm. There is a characteristic corkscrew oesophagus seen due to multiple simultaneous oesophageal contractions. Nutcracker oesophagus does not have such a characteristic appearance.

24 hour ambulatory manometry is preferable as spasm may be intermittent. DES shows intermittent, uncoordinated, simultaneous, prolonged, powerful oesophageal contractions induced by swallowing and interspersed with normal peristalsis. In nutcracker oesophagus there are cocoordinated peristaltic contractions of extremely high-amplitude (as high as 225 to 430 mm Hg), often of prolonged duration.

Oesophageal pH studies: Allow assessment of concurrent gastro-oesophageal reflux disease.

Ultrasound: High-frequency intraluminal ultrasound can assess the sensory and motor function of the oesophagus and help to differentiate diffuse oesophageal spasm and nutcracker oesophagus.1 Resting and exercise ECGs: These may be needed if the history contains features that could suggest a cardiac cause for the chest pain, especially if there are other cardiac risk factors.

Non-drug Reassurance that this is not heart disease is key. Dietary manipulation (including pured diets i.e blended) helps some but response is difficult to predict.

Drug Calcium-channel blockers: Reduce amplitude of contractions. Nitrates: glycerol trinitrate. Mechanism of action unclear. Tricyclic antidepressants (imipramine) and anxiolytics: Can reduce pain and associated anxiety. Benzodiazepines may be used Endoscopic botulinum toxin injection: Gives transient relief Proton pump inhibitors: May be needed if there is associated reflux.

Surgical Endoscopic balloon dilatation of the gastrooesophageal sphincter: Helps some patients Oesophagomyotomy: Used rarely if the condition is very disabling. Laparoscopic Heller myotomy is thought to be the surgical treatment of choice for diffuse oesophageal spasm. Oesophagectomy: Very rarely used if symptoms are intractable.

Mortality rate is virtually nil, but there can be much morbidity. Chest pain and dysphagia can lead to an inability to eat and subsequent malnutrition. There may be considerable associated psychological problems.

Motility disorders of the oesophagus can be very distressing to the patient cos of the dysphagia . Appopriate management can improve the quality of life of such and make

10/9/2012

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