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home - Oesophagus - Oesophageal Dysmotility - Achalasia Written by Dr Sebastian Zeki

Achalasia

Manometric sub-classification (use is unvalidated) Type I- achalasia with minimal esophageal pressureType II- achalasia with esophageal compression- more likely to respond to botulinum toxin, pneumatic dilation, or Heller myotomy than othersType III-achalasia with spasm. than type I or type II patients. Endoscopy —Needed to rule out malignancy AetiologyPrimary Achalasia may be autoimmune.Primary achalasia can be associated with HLA-DQw1 antibodies to enteric neurons.Primary achalasia may also be related to previous infection with HSV or measles.Secondary causes are also known as pseudoachalasia.Chagas disease can result in a loss of intramural ganglion cells.Malignancy is the most common cause of pseudoachalasia either by direct compression/ paraneoplastic. BURP Clinical Presentations:Dysphagia for solids (91 %) and liquids (85 %)- the latter is v. suggestive for achalasia.Difficulty belching-occurs in 85%.Hiccoughs.Heartburn (often preceedes dysphagia).Weight loss- this is usually in the range of 5 to 10 kg, although profound weight loss can be seen.Regurgitation and aspiration.Chest pain (usually younger patients and can diminish over years- doesnt improve with dilatation). Associations:Adrenal glucocorticoid deficiency and alacrima (triple A syndrome or Allgrove syndrome).SCC esophageal cancer (x16 risk).Cancer-this is diagnosed an average of 14 years after the diagnosis of achalasia. Achalasia HICCOUGH EpidemiologyThe incidence is 1 case per 100,000.M=F.Achalasia occurs at virtually any age, but in pre-adolescence it is unusual. a) Aperistalsis c) LES hypertension b) Incomplete relaxation (>45mmHg) AXR- Dilated oesophagus. No gastric bubbleRadiographic studies — Accuracy- 95 %. Beak-like narrowing of distal oesophagusIf progresses, the oesophagus becomes sigmoidSometimes get purposeless, spastic oesophageal contractions (=vigorous achalasia) Diagnosis Other causes of achalasia:Amyloidosis.Sarcoidosis.Neurofibromatosis.Eosinophilic gastroenteritis.MEN type 2B.Juvenile Sjögren's syndrome.Chronic idiopathic intestinal pseudo-obstruction.Fabry disease. Manometry —Can’t discriminate achalasia from pseudoachalasia.3 characteristic manometric features: Risk is too small to warrant surveillance. PathophysiologyOccasionally degenerative changes found in the ganglion cells of the vagal dorsal motor nucleus in the brainstem.Wallerian degeneration is seen in the vagal fibers that supply the oesophagus.Decreased numbers of inhibitory neurons (ganglion cells) are seen in the myenteric plexuses with the remainder being inflamed so the LES cant relax, and oesophageal body cant move.The cholinergic neurons (which cause contraction) are spared. Written by Dr Sebastian Zeki

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