© Dr.H.G.Schulz - Version 1.0 from 05.08.2011

Disturbances of the motor function of the Esophagus

(Achalasia - Diffuse Esophageal Spasm - Nutcracker-Esophagus - Diverticel of the Esophagus)

Disturbances in transport function of the esophagus can first present with problems in swallowing (Dysphagia), upper stomach pain, heartburn and unspecific chest pain. Frequently patients with such symptoms are first seen by cardiologists whose investigations often do not lead to specific heart related diagnosis. In such situations functional assessment of esophagus functioning via low-invasive measurements can sometimes be beneficial in order to detect the underlying reasons for the abovementioned symptoms and to start adequate treatment.

Achalasie (Cardiaspasm):

Different types of achalasia :

A - Hypermotile achalasia = Vigorous Achalasie (pain, dysphagia, regurgitations)

B - Hypomotile achalasia (Dysphagia, pain, regurgitations)

C - Amotile achalasia (Dysphagia, regurgitations)

Achalasia is an ambiguous disorder of the esophagus with a disturbed passage of food. Symptoms usually become servere with increasing duration of the disease. Dysphagia (Problems in swallowing) is usually the main symtom, but other symptoms such as thoracic spasms (Heartburn) can occur. In some patients these spasms occur before all other symptoms, which in turn can falsely be interpreted as cardiac problems.

The underlying reason for the swallowing problems can be a dysfunction of the lower esophagus sphincter muscle responsible for opening and closing the esophagus (detailed information here). Moreover, the transport of the food from the mouth to the stomach is not sufficiant. The muscles are relaxed, and they contract insufficiently while the overall pressure of the muscles is low. As a result, the food remains in the esophagus. This can lead to vomiting and passive movement of the food back towards the mouth (so-called regurgitations). Moreover, particularly at night, food can get into the lung creating serious aspiration pneumonia.

The problems in swallowing lead to a significant weight loss in almost all affected patients over the time.

The course of the disease can be quite different. In some patients the disease is rapidly progressing, were as in others the progress can be rather slowly. There is one thing all patients have in common: Without sufficient treatment their condition is getting worse.

The diagnosis can be verified by doing a pressure measurement in the oesophagus (so-called manometry). Another investigation involving the swallowing of contrast agents while doing a chest x-ray can show a rather classical picture (it looks a little bit like a wine-glass). However, this particular measurement alone may not be sufficiently accurate to diagnose achalasia in early stages of the disease. The pressure measurement/manometry and the contrast x-ray cannot only be relied on. It is required that an endoscopy is performed in order to rule out other reasons for the outlined problems.

Contrast X-ray images of the different forms of achalasia:

Classic type of achalasia	Megaesophagus	Vigorous Achalasia

Therapeutic options:

The following options are available:

1) Pharmacologic treatment: i.e. calcium-antagonists, etc.

2) Interventional strategies: pneumatic balloon dilatation of the sphincter muscle, injection of Botulinum-toxine in the sphincter muscle

3) Operative strategies: Laparoscopic myotomy of the sphincter muscle (Hellers Myotomy)

Pharmacologic agents used to treat achalasia decrease the tonus of the muscles of the oesophagus. These drugs were mostly originally designed to treat other disorders (i.e. high blood pressure, etc.), which is the reason why some side-effects can occur. For example, calciumantagonists (i.e. nifedipin) and nitrates are used for pharmacological treatment of achalasia and can lead to lowered blood pressure, nausea and above all discontinued treatment because of such side effects.

The substance sildenafil (Viagra) was used in patients with achalasia because of its muscle-relaxing properties. The therapeutic effect was better as regards the problems with swallowing (36.4%) when compared to the classical pharmacologic treatments. However, the observed side effects were also more severe (nausea, headache, low blood pressure).

Above all, the clinical benefit of pharmacologic agents used to treat achalasia so far must be considered as rather limited. Because of this, the use of pharmacologic treatment is rather limited to the beginning of the disease and for patients who did not respond to interventional or surgical treatment.

The pneumatic dilatation of the sphincter is an approved procedure using a balloon. The advantage is that no operation is needed as this can be done during endoscopy. However, there is a 5% risk of a perforation of all layers of the mucosa with a following mediastinitis, a life-threatening infection of the mediastinum (= a group of structures in the thorax that are surrounded by loose connective tissue, including the heart). Moreover, sometimes there is the need of a further dilatation, which can be a disadvantage for later surgical treatment.

Literatur: Veröffentlichte Ergebnisse in der Medizinliteratur

Die Technik der Ballondilatation ...

Endoskopische Applikation des BTX durch die Schleimhaut in den Schließmuskel (Katheter mit Nadel weiß im Bild links)

The injection of botulinum toxin is also an approved procedure which can also be done during endoscopy. Botulinum toxin, a substance leading to a relaxation of the sphincter muscle, is injected into the muscle. The responder rate (65-100%) is initially good. However, long-term results are rather disappointing.

A continuous symptom relief cannot be achieved using this technique, and head-to-head comparisons with pneumatic dilatation were indicative for pneumatic dilatation being superior.

Zusatzinformationen zu Botulinustoxin

Hellers Myotomy	Operative myotomy of the sphincter muscle is also a standard procedure (Heller myotomy). It was described in 1903 by Heller and has been used a lot since its introduction as a surgical technique. Until the mergence of endoscopic techniques it was the standard procedure to treat achalasia. Open surgery using Heller myotomy were shown to have good long term results (Info).

Because of the availability of laparoscopic techniques (info) one can now offer rather low invasive interventional strategies. This combines the good long-term results of open Heller myotomy (info) with the advantages of laparoscopic surgery (info). The muscles layers of the oesophagus sphincter are cut lengthwise a few centimetres while the mucosa layer will not be affected. In combination with an antireflux plasty (info) in order to prevent the reflux of liquids and food from the stomach to enter the oesophagus this way of treatment

This procedure is from a surgical viewpoint quite demanding, but in the hands of an experienced surgeon it should not cause major problems. The main advantage of this technique ist he good long-term outcome.

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