Esophageal Motility Disorders


Zenker’s Diverticulum

  1. Pathophysiology
    • false diverticulum
    • results from the herniation of the pharyngeal mucosa and submucosa at the point of transition (Killian’s triangle) between the oblique fibers of the inferior pharyngeal constrictor (thyropharyngeus) muscle and the horizontal fibers of the cricopharyngeus muscle
    • manometric studies implicate high pressures or incomplete relaxation of the upper esophageal sphincter during swallowing
    • esophageal dysmotility or shortening from reflux may also play an etiologic role

    Zenkers Diverticulum
  2. Clinical Manifestations
    • majority of patients are older males
    • earliest symptom is a sensation of food sticking in the throat
    • later symptoms include spontaneous regurgitation of foul-smelling, undigested food and bad breath
    • respiratory symptoms (from aspiration) include hoarseness, bronchospasm, pneumonia

  3. Diagnosis
    • barium swallow will demonstrate the sac

      • Barium Swallow Demonstrating a Zenker's Diverticulum
    • endoscopy and manometry are not necessary to make a diagnosis

  4. Treatment
    1. Surgery
      • patient is placed supine with the neck extended and head turned to the right
      • incision is made along the left sternocleidomastoid muscle
      • the diverticulum is identified and mobilized (a bougie may facilitate this)
      • a myotomy is performed, beginning at the neck of the diverticulum
      • the myotomy is extended cephalad by dividing 1 to 2 cm of the inferior constrictor muscle of the pharynx and caudad by dividing the cricopharyngeus muscle and 4 to 5 cm of cervical esophagus
      • most small (< 2 cm) diverticulum will disappear after this myotomy
      • larger diverticulum (> 2 cm) may be transected with a stapling device
      • some surgeons perform a diverticulopexy instead of a diverticulectomy in high-risk patients in order to avoid a staple line leak
      • recurrence is much more frequent if the myotomy is omitted
      • complications include leak or perforation with possible mediastinitis and recurrent nerve injury

      Diverticulectomy and Diverticulopexy for Zenker's Diverticulum
      Diverticulectomy (L) and Diverticulopexy (R)

    2. Endoscopy
      • becoming more common
      • involves endoscopic division of the common wall between the diverticulum and the esophagus
      • may be done with a rigid or flexible endoscope
      • the main advantage of flexible endoscopy is that it avoids a general anesthetic in poor-risk surgical candidates
      • the most serious complication is esophageal perforation
      • surgery is the better option for symptomatic diverticula < 3 cm in size

      Endoscopic Management of Zenker's Diverticulum

Achalasia

  1. Pathophysiology
    • ‘lack of relaxation’
    • results from degeneration of ganglion cells in the myenteric plexuses of the esophageal wall, which may be idiopathic (most common) or infectious (Chagas’ disease)
    • the neurons that are primarily affected are inhibitory, resulting in unopposed smooth muscle stimulation and, ultimately, failure of relaxation of the LES and aperistalsis
    • hypertension of the LES leads secondarily to elevated esophageal pressure and esophageal dilatation
    • some achalasia patients also have a defect in UES relaxation, leading to loss of the belch reflex
    • premalignant condition (8% chance of developing cancer at 20 years)

  2. Presentation
    • dysphagia for both solids and liquids that develops over many years
    • regurgitation of undigested food
    • use of large quantities of water to wash food down
    • GERD-like retrosternal burning pain
    • aspiration pneumonia common

  3. Diagnosis
    1. Barium Esophagram
      • mild dilatation early, massive dilatation (megaesophagus) late
      • peristalsis disordered early, nonexistent late
      • retained intraesophageal contents typical
      • classic finding is the ‘bird’s beak’ taper

      Barium Swallow Demonstrating Classic Achalasia
    2. Chest X-ray
      • widened mediastinum
      • posterior mediastinal air-fluid level
      • absence of gastric air bubble

      Chest X-ray findings in Achalasia
    3. Manometry
      • gold standard test for diagnosis
      • incomplete LES relaxation with swallowing
      • elevated LES pressure (> 35 mm Hg)
      • increased intraesophageal baseline pressure
      • aperistalsis in the esophageal body
      • low-amplitude waveforms indicating a lack of muscular tone

    4. Esophagoscopy
      • useful to evaluate for esophagitis or the presence of carcinoma

  4. Therapy
    • palliative, not curative
    • treatment directed towards relieving the obstruction caused by a nonrelaxing LES
    • no treatment addresses the decreased motility in the esophageal body

    1. Medical Therapy
      • sublingual nitroglycerin, nitrates and calcium-channel blockers may help alleviate symptoms very early in the disease, or in patients who are not candidates for more invasive treatments
      • least effective treatment option

    2. Botulinum Toxin Therapy (Botox)
      • injected directly into the LES during endoscopy
      • most patients have early improvement in symptoms but require retreatment within 6 – 12 month

    3. Esophageal Dilatation
      • hydrostatic or pneumatic balloons are used to dilate and rupture the fibers of the LES
      • multiple endoscopies are required per treatment (graded approach)
      • initial success rates are high (85%), but efficacy wanes over time
      • main complication is esophageal rupture (4%) which has a 20% mortality rate
      • procedure may be repeated when symptoms recur, but each subsequent dilation is progressively less likely to result in a sustained remission
      • often is the first treatment chosen

    4. Surgical Therapy
      • esophageal myotomy is needed to relieve LES hypertension
      • classic approach is the transthoracic left-sided open myotomy from the gastric cardia to the level of the aortic arch
      • procedure can also be done from a transabdominal approach
      • laparoscopic and thoracoscopic approaches are now becoming standard
      • an area of controversy is whether an anti-reflux procedure is necessary and which procedure should be done
      • a complete 360-degree Nissen fundoplication has the potential to increase LES pressure and worsen symptoms of esophageal obstruction - for this reason, a partial wrap (Toupet) is often chosen
      • esophagectomy (transhiatal) should be considered for end-stage disease or failure of more than one myotomy

      Myotomy and Partial Fundoplication for Achalasia
    5. Per-Oral Endoscopic Myotomy
      • replicates the surgical procedure endoscopically
      • no long-term follow up data yet

      Per Oral Endoscopic Myotomy

Distal (Diffuse) Esophageal Spasm

  1. Pathophysiology
    • poorly understood hypermotility disorder
    • motor abnormality of the lower two-thirds of the esophagus that may be related to impaired inhibitory innervation
    • muscular hypertrophy and degeneration of branches of the vagus nerve have been observed
    • esophageal contractions are repetitive, simultaneous, high-amplitude, tertiary

  2. Presentation
    • chest pain, often mimicking angina pectoris by radiating into the neck or down the arm
    • dysphagia for solids and liquids is common
    • regurgitation of food is unusual
    • may have other functional GI disorders (irritable bowel syndrome, pyloric spasm)

  3. Diagnosis
    • initial evaluation usually is cardiac in nature (chest x-ray, EKG)
    • esophageal etiology is often considered only after a negative cardiac workup
    • need also to rule out intraabdominal pathology (gallstones, peptic ulcer)
    • esophagoscopy is necessary to rule out a distal obstructing lesion that produces tertiary contractions
    • barium esophagram may show a thickened esophageal wall (>5 mm), pulsion diverticulum, the classic ‘corkscrew’ esophagus, or it may be normal

      • Barium Swallow Demonstrating a Corkscrew Esophagus
    • manometry is the critical diagnostic test
    • repetitive, simultaneous, high-amplitude or long duration tertiary contractions define spasm
    • if standard or ambulatory manometry fails to demonstrate DES, provocative maneuvers (bethanecol) may induce the motility disorder
    • diagnostic goal is the correlation of subjective symptoms with objective evidence of spasm on manometry

  4. Treatment
    1. Medical Therapy
      • avoid ‘trigger’ foods
      • control GERD symptoms with PPIs
      • antispasmodics are occasionally helpful (peppermint oil – Altoid mints)
      • calcium channel blockers or nitroglycerine are occasionally helpful
      • tricyclic antidepressants are helpful for some patients

    2. Endoscopic Therapy
      • bougie dilation for severe dysphagia provides relief in 70% - 80%
      • Botox injections may provide short-term relief

    3. Surgical Therapy
      • results are much less favorable than in achalasia
      • esophagomyotomy should be used only in the most severe and refractory cases of dysphagia, or if a pulsion diverticulum is present
      • a long transthoracic myotomy is necessary, extending from the aortic arch onto the proximal stomach
      • since the myotomy will make the LES incompetent, an anti-reflux procedure is necessary
      • a 360-degree fundoplication is contraindicated, just as in the case in achalasia

Hypercontractile (Nutcracker) Esophagus

  1. Presentation
    • symptoms are similar to DES: chest pain, dysphagia, odynophagia
    • most painful of all esophageal hypermotility disorders

  2. Diagnosis
    • barium swallow may show segmental spasm, or be normal
    • manometry shows extremely high-amplitude (225 to 430 mm Hg) normally progressive peristaltic contractions, often of prolonged duration (> 6 seconds)
    • LES pressure is normal and relaxes with each wet swallow
    • manometry findings need to coincide with subjective complaint of chest pain
    • ambulatory monitoring may be necessary to distinguish this disorder from DES

  3. Therapy
    • medical management is the treatment of choice and is similar to the management of DES
    • avoid trigger foods
    • surgery rarely is indicated or helpful

Ineffective Esophageal Motility

  1. Pathophysiology
    • contraction abnormality of the distal esophagus
    • usually associated with GERD
    • may be secondary to inflammatory injury to the esophageal body
    • decreased motility of the esophageal body leads to poor acid clearance in the lower esophagus

  2. Symptoms
    • reflux symptoms and dysphagia

  3. Diagnosis
    • esophagram shows nonspecific abnormalities of esophageal contraction
    • diagnosis made by manometry: nontransmitted or low-amplitude peristaltic contractions in more than 30% of wet swallows, decreased LES tone

  4. Treatment
    • altered motility is irreversible
    • best treatment is prevention: effective treatment of GERD







References

  1. Sabiston, 19th ed., pgs 1023 – 1032
  2. Cameron, 11th ed., pgs 39 – 43, 44 – 47
  3. UpToDate. Zenker’s Diverticulum. Schiff MD, Bradley. May 28, 2019. Pgs 1 – 22
  4. UpToDate. Achalasia: Pathogenesis, Clinical Manifestations, and Diagnosis. Spechler MD, Stuart. Feb 09, 2018. Pgs 1 – 22
  5. UpToDate. Overview of the Treatment of Achalasia. Spechler MD, Stuart. March 15, 2019. Pgs 1 – 17
  6. UpToDate. Major Disorders of Esophageal Hyperperistalsis: Clinical Features, Diagnosis, and Management. Castell MD, Donald. July 17, 2019. Pgs 1 – 21