Achalasia and hypercontractile esophagus
These two conditions belong to specific motility disorders with distinct clinical and instrumental presentations.
Achalasia (achalasia) manifests with dysphagia to both solids and liquids, as well as chest pain and regurgitation.
- High-resolution manometry: reveals absent lower esophageal sphincter relaxation (failed LOS relaxation) during swallowing and increased pressure throughout the esophagus (panoesophageal pressurisation). The upper sphincter functions normally.
- Fluoroscopy (fluoroscopy): the classic sign is marked esophageal dilatation with tapering at the esophagogastric junction (GOJ). In early stages, the caliber of the organ may remain normal, but peristalsis is already absent in the distal segment. Contrast (barium) retention for more than 5 minutes confirms the pathology.
- Additional methods: CT (CT) often shows a dilated esophagus containing solid debris and fluid. Endoscopy is mandatory to rule out carcinoma. A hot water swallow test helps differentiate achalasia from cancer: in achalasia, it causes rapid and pronounced relaxation of the lower sphincter.
Hypercontractile esophagus (jackhammer oesophagus) is approximately five times less common. In these patients, chest pain is more prominent than dysphagia. On fluoroscopy, the organ takes on a "corkscrew" appearance (corkscrew appearance). Manometry shows alternating normal peristaltic waves with simultaneous non-peristaltic contractions in the lower segment.
Impact of systemic and neuromuscular diseases
Motility disorders are frequently secondary and develop against the background of systemic conditions such as diabetes mellitus or hypothyroidism.
Scleroderma (progressive systemic sclerosis) affects the esophagus more often than other parts of the gastrointestinal tract. Due to atrophy of the smooth muscle of the muscularis propria (muscularis propria), peristalsis decreases, and a patulous esophagogastric junction (patulous GOJ) develops. This leads to severe gastroesophageal reflux. If a scleroderma patient has pulmonary involvement, a patulous esophagus is detected in 80% of cases, often alongside enlarged mediastinal lymph nodes.
Neuromuscular disorders such as Parkinson's disease, myasthenia gravis, multiple sclerosis, and stroke sequelae typically provoke oropharyngeal dysphagia. A special place is occupied by inclusion body myositis (inclusion body myositis). This is the most common myopathy in individuals over 40, with an incidence comparable to achalasia. The disease is frequently missed due to vague symptoms. Because it affects skeletal muscle, the pharynx and upper third of the esophagus are involved.
Structural changes and diverticula
Motility disorders or external factors can lead to anatomical defects of the organ wall.
Esophageal diverticula (oesophageal diverticula) are divided into two groups by their mechanism of formation:
- Traction (tractional): form due to scarring and shrinkage of an adjacent lymph node (e.g., after past tuberculosis). The node adherent to the esophagus literally pulls the wall outward.
- Pulsion (pulsion): form due to high intraluminal pressure arising from impaired peristalsis.
Intramural esophageal pseudodiverticulosis on a barium swallow radiograph appears as multiple flask-shaped outpouchings (flask-shaped pits). Physiologically, these are dilated mucous glands. The condition is often accompanied by the formation of a benign stricture at the level of the aortic arch (aortic knuckle).
It is also worth mentioning esophageal varices (oesophageal varices). In the vast majority of cases in the lower part of the organ, they develop in an "uphill" (uphill) fashion as a consequence of portal hypertension.
Briefly: answers to topical questions
What types of primary esophageal motility disorders are distinguished according to the Chicago Classification?
The Chicago Classification divides primary esophageal motility disorders into three groups.
- Achalasia — types I, II, III and esophagogastric junction outflow obstruction
- Disorders of esophagogastric junction and motility — diffuse esophageal spasm, hypercontractile contractions (DCI >8000, jackhammer esophagus), hypocontractility / absent contractility
- Peristaltic abnormalities — ineffective esophageal motility with large and small breaks, fragmented peristalsis, rapid contractions, hyperkinesia of the thoracic esophagus (nutcracker esophagus)
What systemic diseases can cause secondary esophageal motility disorders?
Systemic diseases that can cause esophageal motility disorders:
- Scleroderma (progressive systemic sclerosis) — atrophy of esophageal smooth muscle and decreased peristalsis
- Diabetes mellitus
- Hypothyroidism
For the classification of diverticula: Traction — "Pull" the wall from the outside (due to adhesions and lymph nodes), Pulsion — "Push" from the inside (due to increased pressure from motility disorders).
Class and exam questions
Which method is considered the most accurate for diagnosing esophageal motility disorders?
High-resolution manometry is recognized as the gold standard, allowing a detailed assessment of esophageal pressure and sphincter function during swallowing.
Why does inclusion body myositis affect only part of the esophagus?
This disease affects the skeletal muscle that makes up the upper third of the esophagus and pharynx. The lower segments, consisting of smooth muscle, remain intact.
What is the difference in symptoms between achalasia and hypercontractile esophagus?
In achalasia, dysphagia to solids and liquids as well as regurgitation come to the forefront. In hypercontractile esophagus (corkscrew esophagus), severe chest pain is the dominant symptom.
What else to study on this topic
- High-resolution manometry: detailed analysis of graphs and parameters.
- Diffuse esophageal spasm: diagnostic criteria and differentiation from other pathologies.
- The role of endoscopy in assessing complications of gastroesophageal reflux in scleroderma.
- Radiological signs of intramural pseudodiverticulosis and benign strictures.
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