• Failure of Lower Esophageal Sphincter to relax during swallowing and absence of esophageal peristalsis.
• Affects both LES & smooth muscles of esophagus.
Pathogenesis -
• Due to progressive inflammatory destruction of inhibitory myenteric neurons in Auerback's plexus.
Clinical features -
• Triad - Dysphagia, Regurgitation, and Weight loss.
• Chest pain
• Nocturnal coughing.
• Allgrove syndrome - Achalasia, Alacrima & Addison's disease
Diagnosis -
• Barium swallow - Bird beak, Pencil tip or Rat tail sign.
• Mecholyl test +ve.
• CCK test +ve.
• Manometry - Gold standard.
Findings - Incomplete LES relaxation, increased resting LES tone, and lack of peristalsis of the esophagus.
• Pressure of LES <26 mm Hg is normal, >100 is considered achalasia, > 200 is nutcracker achalasia.
• Prolonged esophageal pH monitoring to rule out GERD.
Treatment -
• Initial treatment should be pneumatic dilation or laparoscopic surgical myotomy with a partial fundoplication.
• Botox - Botulinum toxin therapy is recommended for patients not suited to PD or surgery.
Limited success rate & high reoccurence.
• Medical therapy - Subligual Nitroglycerine, Nitrates, CCB. (used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery or in case of failed botox therapy)
• Lap Heller Myotomy with partial anterior fundoplication - Surgery of choice.
• Peroral endoscopic myotomy (POEM) - 50% show gerd
• Toupet or Dor fundoplication (to prevent GERD)
Important - In current practice, a partial anterior fundoplication is more frequently performed since it is simpler to perform and covers the exposed esophageal mucosa.
• Affects both LES & smooth muscles of esophagus.
Pathogenesis -
• Due to progressive inflammatory destruction of inhibitory myenteric neurons in Auerback's plexus.
Clinical features -
• Triad - Dysphagia, Regurgitation, and Weight loss.
• Chest pain
• Nocturnal coughing.
• Allgrove syndrome - Achalasia, Alacrima & Addison's disease
Diagnosis -
• Barium swallow - Bird beak, Pencil tip or Rat tail sign.
• Mecholyl test +ve.
• CCK test +ve.
• Manometry - Gold standard.
Findings - Incomplete LES relaxation, increased resting LES tone, and lack of peristalsis of the esophagus.
• Pressure of LES <26 mm Hg is normal, >100 is considered achalasia, > 200 is nutcracker achalasia.
• Prolonged esophageal pH monitoring to rule out GERD.
Treatment -
• Initial treatment should be pneumatic dilation or laparoscopic surgical myotomy with a partial fundoplication.
• Botox - Botulinum toxin therapy is recommended for patients not suited to PD or surgery.
Limited success rate & high reoccurence.
• Medical therapy - Subligual Nitroglycerine, Nitrates, CCB. (used primarily in elderly patients who have contraindications to either pneumatic dilatation or surgery or in case of failed botox therapy)
• Lap Heller Myotomy with partial anterior fundoplication - Surgery of choice.
• Peroral endoscopic myotomy (POEM) - 50% show gerd
• Toupet or Dor fundoplication (to prevent GERD)
Important - In current practice, a partial anterior fundoplication is more frequently performed since it is simpler to perform and covers the exposed esophageal mucosa.
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