Achalasia and diffuse oesophageal spasm (motility disorders)

Define: Esophageal achalasia

Failure of the LES to relax!STIFF - HYPERTONIC →→ food accumulation/dysfunctional peristalsis → progressive dysphagia

Pathogenesis: Achalasia

Degeneration of inhibitory neurons within the myenteric plexi → lack of relaxation

Epidemiology: Achalasia

Most commonly occurs in YOUNGER patients (<60 years)

Clinical features: Achalasia

- Progressive dysphagia (months to years) to BOTH solids/liquids- Regurgitation/vomiting- Coughing (esp. at night)- Retrosternal pain+/- weight loss

Complications: Achalasia

- Aspiration pneumonia- Higher risk of esophageal cancer

Diagnostics: Achalasia

In ANY patient with dysphagia - oesophageal cancer needs to be excluded! - Barium swallow - "bird beak sign" - narrowing of GE junction, proximal dilation - Manometry (esophageal motility study) = CONFIRMATORY (high pressure in lower 1/3, lack of peristalsis)- Endoscopy - EXCLUDE MALIGNANCY!

DDx: Achalsia

Mechanical obstruction - presentation/manometric findings can mimic achalasia- Esophageal/stomach cancer- Chagas disease- Amyloidosis- Sarcoidosis

Treatment: Achalasia

Conservative- Small meals, chew thoroughly- Eat slowly- Sleeping with many pillows (prevent regurg)Low surgical risk?- Endoscopically guided balloon dilation - Heller myotomy High surgical risk?- Botulinum toxin injection

Procedure: Heller myotomy

Laparoscopic divisions of the lower esophageal circular muscle fibres

Complication: Heller myotomy

GERD! (excessive cutting → loss of tone → GERD) ∴ a simultaneous fundoplication surgery may be done!

Procedure: Nissen fundoplication

Fundus is wrapped around the lower esophagus - forms a BELT → tightens and strengthens the LES! Laparoscopically or open

Diffuse esophageal spasms

- Repetitive- Uncoordinated- Non-progressive contraction waves of the esophagus.Typically presents as dysphagia, regurgitation, and squeezing retrosternal chest pain during meals.Causes: Mostly unknown--Very hot or cold drinks, caffeine may be "triggers"Diagnosis:- Barium swallow (corkscrew esophagus - non specific)- Manometry (simultaneous multi-peak contractions)Treatment: 1) CCB, nitrates, PPIs2) Endoscopic dilation, botox injections3) Surgery (rare cases) - long esophagomyotomy