Achalasia affects the esophagus, the tube that carries swallowed food from the back of the throat down into the stomach. A ring of muscle called the lower esophageal sphincter encircles the esophagus just above the entrance to the stomach. This sphincter muscle is normally contracted to close the esophagus.
When the sphincter is closed, the contents of the stomach cannot flow back into the esophagus. Backward flow of stomach contents (reflux) can irritate and inflame the esophagus, causing symptoms such as heartburn. The act of swallowing causes a wave of esophageal contraction called peristalsis.
Peristalsis pushes food along the esophagus. Normally, peristalsis causes the esophageal sphincter to relax and allow food into the stomach. In achalasia, which means "failure to relax," the esophageal sphincter remains contracted. Normal peristalsis is interrupted and food cannot enter the stomach.
Causes of achalasia cardia or cardiospasm
Though thew actual cause of achalasia cardia is not known exactly, but there are some theories postulated for the aetiology of achalasia cardia of oesophagus.
The motor nerve supplying oesophagus is vagus nerve which control the movement. It is assumed that any vagal disturbance may be a cause of achalasia cardia or cardiospasm.
The pinch- cock action of diaphragmatic cruras may be responsible as it may lead to dilatation of lower end of esophagus.
Aurebach,s myenteric plexus is a bunch of nerves supplying the oesophagus and degeneration of this aurebach,s plexus my lead to dilatation of esophagus causing achalasia cardia.
Many persons having a habit of mouth breathing may suffer from achalasia thus it is assumed that air swallowing may have a role in causing cardiospasm or achalasia cardia.
Hirschprung's disease or megacolon is a congenital gastrointestinal disease and achalaisa cardia may be due to primary dilatation like the same disease.
Lack of integrated parasympathetic stimulation and non propulsive motility of the esophagus may lead to dilatation of the esophagus causing achalasia cardia.
Treatment
As the degenerative neural lesion of this disease cannot be corrected, treatment is directed at palliation of symptoms and prevention of complications. Effective peristalsis is rarely restored by successful treatment, but improved oesophageal emptying and a decrease in oesophageal diameter are generally expected.
Four palliative treatments are available: pharmacotherapy, botulinum toxin injection, pneumatic dilatation and myotomy. They all aim to decrease LOS pressure and improve emptying by gravity.
Pharmacotherapy : -
Smooth muscle relaxants alleviate symptoms and improve oesophageal emptying in up to 70% of patients. Nitrites, such as sublingual isosorbide dinitrite, and calcium channel blockers, such as diltiazem, nifedipine and verapamil, have this effect.
The role of pharmacological agents in the long-term management of achalasia is unclear.
It is not known whether their long-term use prevents dilatation and complications. This treatment option is suitable for patients with medical conditions that interfere with pneumatic dilatation or myotomy. Also, patients with severe weight loss can be treated pharmacologically until a healthy nutritional status can be re-established, making them better candidates for other forms of treatment.
Botulinum Toxin : -
Botulinum toxin type A is derived from the controlled fermentation of Clostridium botulinum. The toxin binds to presynaptic cholinergic neuronal receptors, is internalised, and irreversibly interferes with acetylcholine release, probably by preventing the neurotransmitter vesicle docking and fusing with the axonal membrane.
Pasricha and colleagues first demonstrated the similar ability of botulinum toxin to decrease LOS basal tone and improve symptoms in patients with achalasia.
An initial, beneficial response at the level of the LOS occurs in 90% of patients, but symptoms reappear within a year in many initial responders. Side effects of this treatment are rare, but include chest discomfort for a few days after the injection and an occasional rash.
The best use of botulinum toxin injection in achalasia is still being explored, but this seemingly safe approach with little apparent morbidity may be of great advantage when a short-term treatment response is desired.
Dilatation : -
Forceful dilatation of the gastroesophageal sphincter to a diameter of approximately 3 cm is necessary to tear the circular muscle and to ensure a lasting reduction in LOS pressure. Many types of dilators have been developed for this purpose, but it is pneumatic dilators which are conventionally used today.
The technique of dilatation, inflation pressure and duration of inflation varies. Water-soluble contrast material is used to detect distal oesophageal leaks. Surgical consultation is undertaken if perforation is evident. Small perforations are managed conservatively with broad-spectrum antibiotics.16 Clinical deterioration e.g. shock, sepsis, haemorrhage or a finding of free-flowing barium into the mediastinum, requires immediate thoracotomy and repair.
At least 60% of patients have a good response and success rates exceeding 95% have been reported.17 The response rate varies with patient age, (younger patients do not do as well as older patients), and duration of symptoms, (those with a shorter history do not respond as well), but it does not seem to be related to the degree of oesophageal dilatation or tortuosity. The efficacy of this procedure is decreased by as much as half with each subsequent dilatation.
Morbidity is mostly related to oesophageal perforation, a complication in approximately 5% of patients, but surgical repair is required in less than half of these cases. Perforation may be more likely in severely malnourished patients, which raises the possibility that re-establishment of a good nutritional status decreases the complication rate following dilatation.
Surgery
The goal of surgical therapy in achalasia is to decrease LOS resting pressure without completely compromising its competency against gastroesophageal reflux (GOR).
The Heller procedure was described in 1913 and now a modification of this procedure is used most commonly in the surgical management of achalasia. An anterior myotomy is performed by dividing the circular muscle of the oesophagus down to the level of the mucosa. The myotomy extends less than 1cm onto the stomach and to several centimetres above the palpable region of the lower sphincter.
The transthoracic approach is preferred, as it helps confirm the diagnosis, allows careful palpation and inspection of the oesophagus, and enables the surgeon to extend the myotomy proximally as far as is necessary. Open myotomies have good results in 80-90% of patients. They decrease the LOS pressure more reliably, and therefore have a greater efficacy than pneumatic dilatation.
Minimally invasive surgical procedures are becoming a preferable alternative to open myotomy, allowing the Heller myotomy to be performed thoracoscopically and laparoscopically .
Shorter hospitalisation, less pain and early resumption of activity are the benefits of the minimally invasive approach, which remains as effective as the open techniques in the relief of dysphagia. Complications of minimally invasive surgery include: anterior gastric perforation, mucosal perforation at the gastroesophageal (GO) junction and, most significantly, GOR.
Surgery is not necessary for a patient who has few symptoms and minimal oesophageal dilatation. It is, however, required for those with dilatation and food retention to prevent serious pulmonary complications and, of course, to provide symptomatic relief.
Pre-Operative Evaluation : -
Symptomatic Evaluation : - The severity of the symptoms is scored by the patient by questionnaire before and after surgery.
Barium Swallow : - This should be the first test performed in the evaluation of dysphagia. It usually shows a "bird's beak" narrowing at the GO junction and oesophageal dilatation proximal to the narrowing.
Endoscopy: - Endoscopy should follow a barium swallow to rule out pseudoachalasia. It excludes gastroduodenal abnormalities, peptic malignancy and stricture.
Oesophageal Manometry : - Typical manometrical findings are the absence of oesophageal peristalsis and a hypertensive LOS which fails to relax completely in response to swallowing.
Prolonged Ambulatory pH Monitoring : - Prolonged pH monitoring is performed to determine if abnormal reflux is present. In patients who have a positive test result, it is essential to distinguish between true reflux and increased acidity due to stasis and fermentation.
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