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Primary Esophageal Motility Disorders

      Esophageal motility disorders often manifest with chest pain and dysphagia. Achalasia is a disorder of the lower esophageal sphincter and the smooth musculature of the esophageal body. In achalasia the lower esophageal sphincter typically fails to relax with swallowing, and the esophageal body fails to undergo peristalsis. In contrast to spastic disorders of the esophagus, achalasia can be progressive and cause pronounced morbidity. Pseudoachalasia mimics achalasia in terms of symptoms but can be caused by infectious disorders or malignancy. Treatment for achalasia is nonstandardized and includes medical, endoscopic, and surgical options. Spastic disorders of the esophagus, such as diffuse esophageal spasm and nutcracker esophagus, and nonspecific esophageal motility disorder are benign and nonprogressive, with similar findings on esophageal manometry. Although the exact cause remains unknown, these disorders may represent a manifestation of gastroesophageal reflux disease. Treatment of spastic disorders includes medical and surgical approaches and is aimed at symptomatic relief.
      DES ( diffuse esophageal spasm), EUS ( endoscopic ultrasonography), GERD ( gastroesophageal reflux disease), LES ( lower esophageal sphincter), NE ( nutcracker esophagus), NSMD ( nonspecific esophageal motility disorder)
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        • Kahrilas PJ
        Esophageal motility disorders: current concepts of pathogenesis and treatment.
        Can J Gastroenterol. 2000; 14: 221-231
        • Koshy SS
        • Nostrant TT
        Pathophysiology and endoscopic/balloon treatment of esophageal motility disorders.
        Surg Clin North Am. 1997; 77: 971-992
        • Katz PO
        in: Castell DO Richter JE Dalton CB Esophageal Motility Testing. Elsevier, New York, NY1987: 107-117
        • Ott DJ
        Motility disorders of the esophagus.
        Radiol Clin North Am. 1994; 32: 1117-1134
        • Van Dam J
        Endosonographic evaluation of the patient with achalasia.
        Endoscopy. 1998; 30: A48-A50
        • Sandler RS
        • Nyren O
        • Ekbom A
        • Eisen GM
        • Yuen J
        • Josefsson S
        The risk of esophageal cancer in patients with achalasia: a population-based study.
        JAMA. 1995; 274: 1359-1362
        • Gelfond M
        • Rozen P
        • Gilat T
        Isosorbide dinitrate and nifedipine treatment of achalasia: clinical, manometric and radionuclide evaluation.
        Gastroenterology. 1982; 83: 963-969
        • Pasricha PJ
        • Ravich WJ
        • Hendrix TR
        • Sostre S
        • Jones B
        • Kalloo AN
        Intrasphincteric botulinum toxin for the treatment of achalasia [published correction appears in N Engl J Med. 1995;333:75].
        N Engl J Med. 1995; 332: 774-778
        • Patti MG
        • Way LW
        Evaluation and treatment of primary esophageal motility disorders.
        West J Med. 1997; 166: 263-269
        • Heller E
        Extramuköse cardioplastik beim chronischen cardiospasmus mit dilatation des oesophagus.
        Mitt Grenzeg Med Chir. 1913-1914; 27: 141-149
        • Csendes A
        • Braghetto I
        • Henriquez A
        • Cortes C
        Late results of a prospective randomised study comparing forceful dilatation and oesophagomyotomy in patients with achalasia.
        Gut. 1989; 30: 299-304
        • Chen YM
        • Ott DJ
        • Hewson EG
        • et al.
        Diffuse esophageal spasm: radiographic and manometric correlation.
        Radiology. 1989; 170: 807-810
        • Valori RM
        Nutcracker, neurosis, or sampling bias?.
        Gut. 1990; 31: 736-737
        • Ott DJ
        Esophageal motility disorders.
        Semin Roentgenol. 1994; 29: 321-331
        • Melzer E
        • Tiomny A
        • Coret A
        • Bar-Meir S
        Nutcracker esophagus: severe muscular hypertrophy on endosonography.
        Gastrointest Endosc. 1995; 42: 366-367
        • Melzer E
        • Ron Y
        • Tiomni E
        • Avni Y
        • Bar-Meir S
        Assessment of the esophageal wall by endoscopic ultrasonography in patients with nutcracker esophagus.
        Gastrointest Endosc. 1997; 46: 223-225
        • Achem SR
        • Kolts BE
        • Wears R
        • Burton L
        • Richter JE
        Chest pain associated with nutcracker esophagus: a preliminary study of the role of gastroesophageal reflux.
        Am J Gastroenterol. 1993; 88: 187-192
        • Just RJ
        • Castell DO
        Chest pain of undetermined origin.
        Gastrointest Endosc Clin N Am. 1994; 4: 731-746
        • Richter JE
        • Spurlin TJ
        • Cordova CM
        • Castell DO
        Effects of oral calcium blocker, diltiazem, on esophageal contractions: studies in volunteers and patients with nutcracker esophagus.
        Dig Dis Sci. 1984; 29: 649-656
        • McBride PJ
        • Hinder RA
        • Filipi C
        • Raiser F
        • Katada N
        • Lund RJ
        Surgical treatment of spastic conditions of the esophagus.
        Int Surg. 1997; 82: 113-118
        • Eypasch EP
        • DeMeester TR
        • Klingman RR
        • Stein HJ
        Physiologic assessment and surgical management of diffuse esophageal spasm.
        J Thorac Cardiovasc Surg. 1992; 104: 859-868
        • Filipi CJ
        • Hinder RA
        Thoracoscopic esophageal myotomy—a surgical technique for achalasia diffuse esophageal spasm and “nutcracker” esophagus.
        Surg Endosc. 1994; 8: 921-925

      Questions About Primary Esophageal Motility Disorders

      • 1.
        Which one of the following is not a major or minor diagnostic criteron for NE?
        • a.
          Repetitive contractions
        • b.
          Esophageal peristaltic contraction amplitude greater than 180 mm Hg
        • c.
          LES pressure greater than 40 mm Hg
        • d.
          “Corkscrew” sign on barium swallow
        • e.
          Prolonged contractions
      • 2.
        Which one of the following investigations is not useful in the evaluation of a patient with suspected achalasia?
        • a.
          Upper endoscopy
        • b.
          Esophageal electromyography
        • c.
          Barium swallow
        • d.
        • e.
          Esophageal manometry
      • 3.
        Which one of the following illnesses would not result in radiographic changes that mimic primary achalasia?
        • a.
          Esophageal carcinoma
        • b.
          Benign esophageal strictures
        • c.
          Chagas disease
        • d.
        • e.
          Zenker diverticulum
      • 4.
        Which one of the following has been shown to improve symptoms in spastic disorders of the esophagus in a prospective, blinded, controlled study?
        • a.
        • b.
        • c.
          Isosorbide mononitrate
        • d.
          Botulinum toxin
        • e.
      • 5.
        Which one of the following is a complication of surgical esophageal myotomy?
        • a.
          Dysphagia with a hypocontractile esophagus
        • b.
          Gastric volvulus
        • c.
          High risk for aspiration pneumonia
        • d.
          Vocal cord atrophy
        • e.
          Cameron lesions

      Correct answers: 1. d, 2. b, 3. e, 4. a, 5. a