Sandbox:Madhu

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Madhu Sigdel

Historical Perspective

Discovery

• DES was first described by Osgood, in 1889 in 6 patients who presented with chest pain and dysphagia.

• Creamer (1954) made the first manometric descriptions of DES.

References

Classification of DES

• There is no established system for the classification of DES although it is categorized as one of the major disorders of peristalsis according to The Chicago Classification v.3.0.

Risk Factors

• Common risk factors in the development of Diffuse Esophageal Spasm include Age (60-80 years), presence of GERD, Hypertension, anxiety or depression, and drinks (eg. red wine, very hot or cold liquid or fluid).

Pathophysiology

Pathogenesis

• The exact pathogenesis of DES is not fully understood. However, current high-resolution manometric studies suggest impairment of inhibitory myenteric plexus neuron. These neurons use nitric oxide (NO) as neurotransmitter. Hence, these patients may also have dysregulation of endogenous NO synthesis or/and degradation. The final result is premature and rapidly propagated or simultaneous contraction of smooth muscles of distal esophagus.

References

Natural History, Complications, and Prognosis

Natural History

• The symptoms of DES usually develop in the first/ second/ third decade of life, and start with symptoms such as ___.
• The symptoms of (disease name) typically develop ___ years after exposure to ___.
• If left untreated, [#]% of patients with [disease name] may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].

Complications

• Common complications of [disease name] include:
  • [Complication 1]
  • [Complication 2]
  • [Complication 3]

Prognosis

• Prognosis is generally excellent/good/poor, and the 1/5/10-year mortality/survival rate of patients with [disease name] is approximately [#]%.
• Depending on the extent of the [tumor/disease progression/etc.] at the time of diagnosis, the prognosis may vary. However, the prognosis is generally regarded as poor/good/excellent.
• The presence of [characteristic of disease] is associated with a particularly [good/poor] prognosis among patients with [disease/malignancy].
• [Subtype of disease/malignancy] is associated with the most favorable prognosis.
• The prognosis varies with the [characteristic] of tumor; [subtype of disease/malignancy] have the most favorable prognosis.

Diagnostic Criteria

• The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met:
  • [Criterion 1]
  • [Criterion 2]
  • [Criterion 3]
  • [Criterion 4]
• The diagnosis of DES is based on The Chicago Classification criteria using high resolution esophageal manometry
  • [Criterion 1]
  • [Criterion 2]
  • [