Esophageal Achalasia

Aka: Esophageal Achalasia, Achalasia, Cardiospasm

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II. Epidemiology

  1. Rare condition with Incidence <5% of cases presenting to specialty centers
  2. More common in older adults than younger patients

III. Pathophysiology

  1. Chronic progressive degenerative disorder often with delayed diagnosis
    1. Delayed diagnosis results in esophageal dilitation, resulting in cases refractory to surgical management
  2. Chronic esophageal Smooth Muscle denervation
    1. Deficient distal esophagus Cholinergic innervation due to loss of myenteric plexus (Auerbach's plexus)
    2. Decreased tissue nitric oxide
    3. Inhibitory Neuron loss
  3. Results
    1. Loss of normal esophageal peristalsis
    2. Lower Esophageal Sphincter (LES) dysfunction, preventing LES relaxation
      1. LES Incoordinate contraction
      2. LES tonic contractions
      3. Constriction of LES in response to Swallowing

IV. Types

  1. Achalasia Type 1 (Classic Achalasia)
    1. No contractility or peristalsis
    2. Lower esophageal sphincter fails to relax (all Achalasia types)
    3. Responds to Laparoscopic Heller Myotomy
  2. Achalasia Type 2 (with esophageal compression)
    1. No normal peristalsis (but some pressurizations)
    2. Lower esophageal sphincter fails to relax (all Achalasia types)
    3. Responds to all treatment options
  3. Achalasia Type 3 (Spastic Achalasia)
    1. No normal peristalsis
    2. Spastic contractions in distal Esophagus (>20% of swallows)
    3. Lower esophageal sphincter fails to relax (all Achalasia types)
    4. Responds poorly to treatment

V. Symptoms

  1. Dysphagia
  2. Chest Pain
  3. Regurgitation of food, Saliva, esophageal secretions
  4. Weight loss

VI. Diagnostics

  1. Upper Endoscopy Achalasia findings
    1. Food retained in Esophagus
    2. Increased resistance across esophagogastric junction
  2. High-Resolution Esophageal Manometry
    1. Required for Achalasia diagnosis
    2. Performed with Nasogastric Tube with closely positioned pressure sensors measure intraluminal pressure
    3. Esophageal pressures are measured as the patient swallows various foods and liquids
    4. Lower esophageal sphincter pressure is also measured before Swallowing and during relaxation
  3. Upper GI (Barium Esophagram)
    1. Proximal esophageal dilatation
    2. Beadlike or "bird beak" narrowing of distal segment at LES
    3. Spasm of lower esophageal sphincter

VII. Differential Diagnosis

  1. See Esophageal Dysphagia

VIII. Management

  1. See Esophageal Dysmotility for general measures
  2. Myotomy (definitive therapy)
    1. Laparoscopic Heller Myotomy
      1. Incises Muscles of the distal Esophagus, lower esophageal sphincter and gastric cardia
    2. Peroral Endoscopic Myotomy
      1. Newer, more technically challenging, but less invasive procedure than the laparoscopic Heller myotomy
      2. Incises the same Muscles as the Heller procedure
  3. Pneumatic dilation (by endoscopy)
    1. Disrupts lower esophageal sphincter
    2. Not as effective or longlasting as myotomy (dilation may need to be repeated)
  4. Onabotulinumtoxin A
    1. Endoscopic injection into lower esophageal sphincter
    2. Unknown efficacy, but may be used in patients at too high risk for surgery

IX. References

  1. Wilkinson (2020) Am Fam Physician 102(5):291-6 [PubMed]

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Related Studies

Ontology: Esophageal Achalasia (C0014848)

Definition (MSHCZE) Vzácné onemocnění, při němž dochází k poruše hybnosti jícnu (nedostatečnému uvolnění svalstva na přechodu jícnu a žaludku) a jeho neschopnosti přenášet sousto do žaludku. Projevuje se poruchou polykání (dysfagie). Příčina je neznámá, podstatou onemocnění je porucha nervových buněk ve stěně jícnu. Léčba dilatace (rozšiřování) jícnu, někdy je nutná operace. (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (NCI) A finding indicating the lack of adequate relaxation of the lower esophageal sphincter resulting in difficulty swallowing food.
Definition (CSP) failure of normal relaxation of the lower esophageal sphincter associated with uncoordinated contractions of the thoracic esophagus, resulting in functional obstruction and difficulty swallowing.
Definition (MSH) A motility disorder of the ESOPHAGUS in which the LOWER ESOPHAGEAL SPHINCTER (near the CARDIA) fails to relax resulting in functional obstruction of the esophagus, and DYSPHAGIA. Achalasia is characterized by a grossly contorted and dilated esophagus (megaesophagus).
Concepts Disease or Syndrome (T047)
MSH D004931
ICD9 530.0
ICD10 K22.0
SnomedCT 45564002, 155672003, 17460002
English Achalasia, Esophageal, Achalasias, Esophageal, Cardiospasm, Cardiospasms, Esophageal Achalasias, ACHALASIA CARDIAE, CARDIOSPASM, achalasia (diagnosis), achalasia, Achalasia cardiae, Achalasia esophageal, Esophagus achalasia of, Oesophageal achalasia, Oesophagus achalasia of, Hypertensive lower oesophageal sphincter, Achalasia & cardiospasm, Achalasia, Achalasias, Esophageal Achalasia, Esophageal Achalasia [Disease/Finding], Achalasia;oesophageal, achalasia esophagus, achalasia cardiospasm, achalasia cardia, achalasia of esophagus, cardiospasms, esophagus achalasia, Cardiospasm (disorder), Achalasia oesophageal, Achalasia of cardia (disorder), Esophageal achalasia, Achalasia of esophagus, Achalasia of cardia, Lack of reflex relaxation of lower esophageal sphincter, Megaoesophagus, Achalasia of oesophagus, Lack of reflex relaxation of lower oesophageal sphincter, Achalasia of esophagus (disorder), cardiospasm, Cardiospasm, NOS, Hypertensive lower esophageal sphincter, Achalasia and cardiospasm, Achalasia;esophageal, esophageal achalasia, oesophageal achalasia
French CARDIOSPASME, Achalasie et cardiospasme, ACHALASIE DU CARDIA, Spasme du cardia, Cardiospasme, Achalasie de l'oesophage, Achalasie du cardia, Achalasie oesophagienne
Dutch achalasia cardiae, slokdarm, achalasia van, achalasie van de slokdarm, achalasie en cardiospasmus, achalasie van slokdarm, Achalasie van cardia, cardiospasmus, oesofageale achalasie, Achalasie, slokdarm-, Cardiospasme, Oesofagusachalasie, Slokdarmachalasie
German Oesophagus Achalasie, oesophageale Achalasie, Achalasie oesophageal, Achalasie und Kardiospasmus, Achalasia cardiae, ACHALASIA CARDIAE, Achalasie der Kardia, KARDIOSPASMUS, Oesophagusachalasie, Kardiaspasmus, Kardiospasmus, Achalasie, Ösophagus-, Ösophagusachalasie
Italian Acalasia e spasmo del cardias, Acalasia del cardias, Cardiospasmo, Acalasia esofagea, Acalasia dell'esofago
Portuguese Acalasia do esófago, Acalasia do cárdia, Acalasia e espasmo cárdico, ACALASIA DO CARDIA, CARDIOSPASMO, Cardiospasmo, Acalasia esofágica, Acalasia Esofágica, Cardioespasmo
Spanish Acalasia de esófago, Acalasia y cardioespasmo, Achalasia cardiae, ACALASIA, ESPASMO DEL CARDIAS, acalasia del cardias, cardioespasmo (concepto no activo), esfínter esofágico inferior hipertensivo, ausencia del reflejo de relajación del esfínter esofágico inferior, cardioespasmo, acalasia del esófago (trastorno), acalasia del esófago, Acalasia esofágica, Cardioespasmo, Acalasia del Esófago
Japanese 噴門アカラシア, ﾌﾝﾓﾝｹｲﾚﾝ, ﾌﾝﾓﾝｱｶﾗｼｱ, ｼｮｸﾄﾞｳｱｶﾗｼｱ, 食道アカラジア, 食道無弛緩症, 食道無弛緩, 弛緩不能症-食道, 噴門痙攣症, アカラジア-食道, 食道無し緩症, 巨大食道, 食道弛緩不能症, 噴門けいれん, 噴門痙攣, 噴門けいれん症, 食道アカラシア, アカラシア-食道
Swedish Esofagusakalasi
Finnish Mahansuun kouristus
Russian KARDIOSPAZM, AKHALAZIIA PISHCHEVODA, PISHCHEVODA AKHALAZIIA, MEGAEZOFAGUS, АХАЛАЗИЯ ПИЩЕВОДА, КАРДИОСПАЗМ, МЕГАЭЗОФАГУС, ПИЩЕВОДА АХАЛАЗИЯ
Czech Kardiospasmus, Achalázie kardie, Achalázie a kardiospasmus, Jícnová achalázie, Achalázie jícnu, achalázie jícnu, kardiospasmus, ezofágus - achalázie, achalázie, neuromuskulární poruchy jícnu, achalázie kardie, ezofagokardiální achalázie
Korean 분문의 이완불능증
Polish Kurcz przełyku, Kurcz wpustu, Achalazja przełyku, Achalazja wpustu
Hungarian Oesophagealis achalasia, Achalasia cardiae, Achalasia oesophagealis, Nyelőcső achalasia, Achalasia és cardiospasmus, Nyelőcső achalasiája, Cardiospasmus, Oesophagus achalasia
Norwegian Øsofagusakalasi, Spiserørsakalasi
Derived from the NIH UMLS (Unified Medical Language System)

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