II. Epidemiology

  1. Rare conditions, even in specialty centers
  2. Peak age of onset >60 years old

III. Pathophysiology

  1. Loss of inhibitory Neuron innervation
  2. Peristalsis preserved to some extent but is disordered and incoordinated
    1. Waves of peristalsis occur simultaneously
    2. Distal Esophageal Spasm is characterized by premature, forceful contractions
    3. Jackhammer Esophagus has properly timed contractions, but increased forceful contractions

IV. Differential Diagnosis

V. Evaluation

VI. Management

  1. See Esophageal Dysmotility for general measures and overall approach
  2. General Measures
    1. Offer reassurance
      1. Functional disorders and Hypercontractile Esophagus improve or resolve spontaneously in a majority of patients
    2. Optimize GERD Management
      1. Antisecretory therapy (e.g. Proton Pump Inhibitor)
    3. Discontinue Opioids
    4. Functional Disorders (Hypercontractile Esophagus has significant overlap with functional disorders)
      1. Stress management
      2. Consider Cognitive Behavioral Therapy
      3. Consider Antidepressants
        1. Selective Serotonin Reuptake Inhibitors (SSRI)
        2. Tricyclic Antidepressants
    5. Mindful eating
      1. Eat smaller, more frequent meals
      2. Eat slowly
      3. Choose softer foods
      4. Avoid foods and situations that trigger symptoms
  3. Medications
    1. Precautions
      1. These medications decrease lower esophageal sphincter pressure and may worsen GERD
      2. Smooth Muscle relaxants are best limited to hypermotility confirmed by manometry
    2. Agents
      1. Calcium Channel Blockers
      2. Nitrates (Nitroglycerin)
      3. Phosphodiesterase-5 Inhibitors (release nitric oxide)
    3. Other agents which are safe and may be effective
      1. Peppermint Oil (2 mints sublingual before each meal)

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Related Studies

Ontology: Esophageal spasm (C0014863)

Definition (MSH) A hypermotility disorder of the ESOPHAGUS that is characterized by spastic non-peristaltic responses to SWALLOWING; CHEST PAIN; and DYSPHAGIA.
Concepts Disease or Syndrome (T047)
MSH D015155
ICD10 K22.4
SnomedCT 79962008, 196614005, 68681007
English Diffuse Esophageal Spasm, Diffuse Esophageal Spasms, Esophageal Spasm, Esophageal Spasm, Diffuse, Esophageal Spasms, Esophageal Spasms, Diffuse, Spasm, Diffuse Esophageal, Spasm, Esophageal, Spasms, Diffuse Esophageal, Spasms, Esophageal, ESOPHAGOSPASM, OESOPHAGOSPASM, DOS - Diffuse oeso spasm, ESOPHAGEAL SPASM DIFFUSE, OS - Esophageal spasm, diffuse esophageal spasm (diagnosis), diffuse esophageal spasm, ES - Oesophageal spasm, Spasm esophageal, Esophageal Spasm, Diffuse [Disease/Finding], dysmotility esophageal, esophageal spasm, corkscrew esophagus, diffuse esophageal spasms, esophageal motor disorder, spasm esophageal, dyskinesia esophagus, esophagospasms, esophagus spasm, oesophageal motility disorder, oesophageal spasms, esophageal motility disorder, Spasm;oesophageal, oesophagus spasm, diffuse oesophageal spasm, oesophagus spasms, disorders esophageal motor, dyskinesia of esophagus, esophageal dysmotility, esophageal spasms, spasm of oesophagus, esophagospasm, esophagus spasms, oesophageal dysmotility, spasm of esophagus, Spasm oesophageal, Esophagospasm (disorder), Esophagospasm, Diffuse spasm of esophagus, Esophagism, Spasm of esophagus, DOS - Diffuse esophageal spasm, DOS - Diffuse oesophageal spasm, Diffuse esophageal spasm, Diffuse oesophageal spasm, Oesophageal spasm, Oesophagospasm, Diffuse spasm of oesophagus, Oesophagism, Spasm of oesophagus, OS - Oesophageal spasm, Diffuse spasm of esophagus (disorder), ES - Esophageal spasm, Esophageal spasm, esophagismus, esophagus; spasm, spasm; esophagus, Spasm;esophageal, oesophageal spasm
French SPASME OESOPHAGIEN, Spasme oesophagien, Spasme de l'oesophage
Portuguese ESPASMO ESOFAGICO, Esofagospasmo, Espasmo esofágico, Espasmo Esofágico Difuso, Espasmo Esofágico
Dutch slokdarmspasme, oesofagusspasmen, oesofageale spasmen, slokdarmspasmen, spasmen oesofageaal, oesofagus; spasme, spasme; oesofagus, oesofagusspasme, Oesofagusspasme, Slokdarmspasme, Spasme, oesofagus-, Spasme, slokdarm-
German Spasmus des Oesophagus, OESOPHAGUSSPASMUS, Oesophagusspasmus, Krampf, ösophagealer, Krampf, Speiseröhren-, Ösophagospasmus, diffuser, Ösophagospasmus, Ösophagusspasmus, diffuser, Ösophagusspasmus, Spasmen, ösophageale, Speiseröhrenkrampf
Italian Esofagospasmo, Spasmo esofageo, Spasmo esofageo diffuso
Spanish Esofagoespasmo, Espsmo esofágico, ESOFAGOSPASMO, espasmo de esófago, espasmo esofágico, esofagoespasmo, espasmo de esófago (concepto no activo), esofagismo, espasmo difuso del esófago (trastorno), espasmo difuso del esófago, Espasmo esofágico, Espasmo Esofágico Difuso, Espasmo Esofágico
Swedish Esofaguskramp
Japanese ショクドウケイレン, けいれん-食道, 食道けいれん, びまん性食道痙攣, 汎発性食道痙攣, 瀰漫性食道痙攣, 食道痙攣-瀰漫性, 食道痙攣, びまん性食道けいれん, 汎発性食道けいれん, 痙攣-食道, 食道けいれん-びまん性, 食道痙攣-びまん性, 食道痙攣-び漫性, 食道痙攣-汎発性
Finnish Diffuusi ruokatorven spasmi
Russian SPAZM PISHCHEVODNYI, PISHCHEVODNYI SPAZM DIFFUZNYI, ПИЩЕВОДНЫЙ СПАЗМ ДИФФУЗНЫЙ, СПАЗМ ПИЩЕВОДНЫЙ
Czech Jícnový spasmus, Spasmus jícnu, ezofágus - spazmus difuzní, difúzní jícnový spazmus
Polish Skurcz przełyku rozlany
Hungarian Nyelőcső görcs, Oesophagospasmus, Oesophagealis görcs, Oesophagus görcse, Nyelőcsőgörcs
Norwegian Spiserørskramper, diffuse, Øsofaguskramper, Øsofaguskramper, diffuse, Spiserørskramper, Diffuse øsofaguskramper, Diffuse spiserørskramper

Ontology: Nutcracker Esophagus (C0028705)

Concepts Disease or Syndrome (T047)
MSH D015154
SnomedCT 235628006
English Esophagus, Nutcracker, Symptomatic oeso peristalsis, Nutcracker Esophagus, esophagus nutcrackers, nutcracker esophagus, nutcracker oesophagus, Nutcracker esophagus, Nutcracker oesophagus, Supersqueeze esophagus, Supersqueeze oesophagus, Symptomatic esophageal peristalsis, Symptomatic oesophageal peristalsis, NO - Nutcracker esophagus, NO - Nutcracker oesophagus, Nutcracker esophagus (disorder)
Dutch notenkrakerslokdarm, Notenkrakerslokdarm
French Diverticules de l'oesophage, Oesophage casse-noisette
German Nussknackeroesophagus, Nußknacker-Ösophagus
Portuguese Divertículos do esófago, Esôfago Quebra-Nozes
Spanish Esófago cascanueces, Esófago Cascanueces, esófago en cascanueces (trastorno), esófago en cascanueces, peristaltismo esofágico sintomático
Japanese くるみ割り食道, クルミワリショクドウ
Czech Vychlípenina jícnu, louskáčkovitý jícen
Hungarian Diótörő nyelőcső
Italian Esofago a schiaccianoci
Norwegian Nøtteknekker spiserør, Nøtteknekker øsofagus