it is an esophageal motility disorder in which the LES fails to relax with swallowing, aperistalsisin the distal two-thirds of esophagus and an increased resting tone of LES
Pathogenesis of achalasia
Results from degeneration of inhibitory neurons in the myenteric (Auerbach's) plexus
Causes of achalasia
Idiopathic
Chagas disease
Diabetic autonomic neuropathy
Amyloidosis
Sarcoidosis
Pseudo-achalasia = achalasia like symptoms due to cancer of gastro-esophageal junction
Clinicalpresentation of achalasia
Dysphagia for both solids and liquids
Age mostly < 50 years
Weight loss
Nocturnal cough, regurgitation
Heartburn does NOT occur because the closed esophageal sphincter prevents reflux
Diagnosis of achalasia
Barium Swallow: Bird's beak appearance (esophageal dilatation with uniform tapering of distal esophagus)
Esophageal Manometry: Inc resting pressure in LES, dec peristalsis in esophagus
Dysphagia caused by Scleroderma has decreased peristalsis and decreased resting LES pressure
Complications of achalasia
Squamous cell carcinoma 5% (most serious)
Candida esophagitis
Diverticulitis
Aspiration pneumonia
Airway obstruction
Clinical features of diffuse esophageal spasm
Episodic chest pain, mimicking an angina
Transient dysphagia
Nutcracker esophagus
Condition in which extremely forceful peristaltic activity leads to episodic chest pain and dysphagia
Diagnosis of diffuse esophageal spasm and nutcracker esophagus
Barium swallow: "Corkscrew" appearance due to dyscoordinated diffuse contractions
Manometry: Repetitive high-amplitude contraction (400 - 500 mmHg) for diffuse esophageal spasm, Very strong peristaltic waves of > 180 mmHg for nutcracker esophagus
Treatment of diffuse esophageal spasm and nutcracker esophagus
Calcium channel blockers
Nitrates
Pneumatic dilatation
Surgical myotomy
Zenker diverticulum
Also known as pharyngoesophageal diverticulum, it is the most common esophageal diverticulum defined as outpouching through the cricopharyngeus muscle, above the upper esophageal sphincter
Zenker diverticulum
Protrudes through the natural weak point i.e. Killian's Dehiscence between inferior pharyngeal constrictor and cricopharyngeus muscle
When diverticulum is small = pharyngeal dysphagia, When diverticulum is large = esophageal dysphagia
Clinical features of Zenker diverticulum
Dysphagia
Regurgitation
Mass in the neck
Halitosis - due to entrapped food
Gastroesophageal reflux disease (GERD)
Reflux of gastric contents into the lower esophagus, resulting in esophageal irritation and inflammation
Pathogenesis of GERD
Transient LES relaxation, OR Incompetent LES
Risk factors for GERD
Sliding hiatal hernia
Delayed gastric emptying
Reduction in reparative capacity of mucosa
Decreased LES tone due to: Hypothyroidism, CNS depressants, Pregnancy, Alcohol, Tobacco
Clinical features of GERD
Esophageal: Heartburn and regurgitation, Dysphagia, "Water brash" - salivation due to reflex salivary gland stimulation as acid enters the gullet
Based on history and empiric trial of proton pump inhibitors (PPI)
Endoscopy is the investigation of choice, especially when: Failure to respond to PPI, Alarm symptoms => 55 years, dysphagia, anemia, weight loss, positive fecal occult blood test
If diagnosis uncertain and endoscopy is normal, then: Manometry = decreased LES pressure, 24-hour pH monitoring is the most accurate investigation
Treatment of GERD
Lifestyle Modifications: Avoid precipitants, lose weight, elevate the head of bed, Avoid large and late-night meals
Herniation of stomach upward into the chest through esophageal hiatus of diaphragm
Types of hiatal hernia
Sliding Hernia (Axial): Herniation of proximal stomach through a widened diaphragmatic hiatus, The gastroesophageal junction is displaced above the diaphragm
Rolling Hernia (Non-axial): Also known as paraesophageal hernia, Herniation of portion of stomach (greater curvature) alongside the distal esophagus, The gastroesophageal junction remains at the level of diaphragm
Clinical features of hiatal hernia
Often asymptomatic, Commonly an incidental finding on CXR, Heartburn and regurgitation can occur, Para-esophageal hernia can cause gastric volvulus
Infectious esophagitis
Usually occurs in immunocompromised individuals, Presents with odynophagia i.e. painful swallowing
Causes of infectious esophagitis
Herpes Simplex Virus: Produces "punched-out" ulcers, Forms multi-nucleated giant cells with intra-nuclear inclusions
Cytomegalovirus: Produces "linear" ulcers, Forms both intra-nuclear and intra-cytoplasmic inclusions
Candida Albicans: Forms patchy gray-white pseudomembrane, Produces yeast and densely matted fungal hyphae
Barrett esophagus
Pre-malignant condition characterized by replacement of the normal squamous epithelium by the more resistant columnar epithelium containing areas of intestinal metaplasia, Occurs as a complication of long-standing GERD
Types of Barrett esophagus
Short Segment Barrett: < 3 cm of columnar epithelium extending cephalad from GE-junction
Long Segment Barrett: ≥ 3 cm of columnar epithelium extending cephalad from GE-junction (aka Classic Barrett)
Diagnosis of Barrett esophagus
Endoscopic biopsy - investigation of choice, Definitive diagnosis: when the columnar mucosa contains intestinal goblet cells, "intestinal metaplasia"
Management of Barrett esophagus
Barrett Metaplasia without dysplasia = PPIs and endoscopy every 2 - 3 years
Low-grade dysplasia = PPIs and endoscopy every 6 - 12 months