achalasia (cardiospasm)
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Etiology
- idiopathic
- Chagas' disease (Latin America <travelers>)
Epidemiology
- affects men & women equally
- annual incidence of 1 per 100,000
- tends to occur in patients 30-60 years of age
Pathology
- absence of peristalsis in the body of the esophagus
- failure of the esophagus to relax in response to swallowing
- the lower esophageal sphincter may be tonically contracted & may undergo spasms
- loss of ganglion cells of the esophageal myenteric plexus
- unchecked cholinergic nerve activation, preventing relaxation of the lower esophageal sphincter
- degenerative changes in the dorsal motor nucleus of the vagus have been described
- loss of neurons releasing vasoactive intestinal polypeptide (VIP) which induces relaxation of the lower esophageal sphincter may play a role
- Trypanosoma cruzi destroys the ganglion cells of the
Clinical manifestations
- generally, insidious onset with long duration of symptoms, often years
- dysphagia generally localized to the middle to lower chest
- dysphagia when attempting to swallow both liquids & solids
- odynophagia
- non-acidic regurgitation of indigested food, choking, coughing
- weight loss
Diagnostic procedures
(GI)
- upper GI endoscopy to rule out cancer at the gastroesophageal junction (all patients, 2nd diagnostic test after barium swallow)[4]
- esophageal mannometry
- incomplete relaxation of the lower esphageal sphincter
- elevated lower esophageal sphincter pressure
- absence of peristalsis in the distal, smooth muscle segment of the esophagus
- normal peristalsis of the upper 1/3 of the esophagus
Radiology
- barium swallow fluoroscopy (image)[8]
- primary screening test for achalasia[4]
- esophageal dilatation with a beak-like narrowing of the distal segment (gastroesophageal junction)
- non peristaltic contractions of the lower 2/3 of the esophagus
- spasm of lower esophageal sphincter
- chest radiograph shows air-fluid level
- CT of abdomen & thorax, looking closely at the gastro-esophageal junction to rule out cancer in the elderly[5]
Complications
Differential diagnosis
- pseudoachalasia
- adenocarcinoma of the gastroesophageal junction
- dysphagia progressing from solids alone to both solids & liquids suggests malignancy
- rapid progression, weight loss
- amyloidosis
- sarcoidosis
- adenocarcinoma of the gastroesophageal junction
- Chagas' disease
- eosinophilic esophagitis
- dysphagia & food bolus obstruction
- most diagnosed between 2nd & 5th decades of life
- comobidities of atopy, such as asthma, rhinitis, dermatitis, seasonal or food allergies
- esophageal web
- dysphagia with solids alone
- disorders producing autonomic neuropathy
Management
- surgical myotomy of lower esophageal sphincter 1st line[4]
- upper GI endoscopy prior to myotomy (see Diagnostic procedures: above)[11]
- per-oral endoscopic myotomy (POEM)
- laparoscopic
- 80% remission at 5-10 years, long-term outcome unknown
- complications: symptomatic GERD 10%
- Nissen fundoplication routinely performed after surgical myotomy[4]
- open surgery
- 75% remission at 5 years
- thoracotomy required
- complications:
- pneumatic esophageal sphincter dilatation
- botulinum toxin, endoscopic intrasphincteric injection
- patient not surgical candidate
- repeated injections necessary
- 60% remission in 1 year
- complications:
- rash 20%
- transient chest pain
- calcium channel blockers or nitrates
- 3rd line if patient unable to tolerate surgery, dilation or botulinum toxin[4]
- ineffective, not recommended[4]
- surveillance endoscopy for esophageal cancer screening is not recommended[6]
Notes
- in 1/3 of patients, endoscopy results may be normal or barium swallow may be nondiagnostic[6]
More general terms
Additional terms
References
- ↑ Rubin & Farber, Pathology, 2nd ed. JB Lippincott Philadelphia, 1994, pg 622
- ↑ DeGowin & DeGowin's Diagnostic Examination, 6th edition, RL DeGowin (ed), McGraw Hill, NY 1994, pg 872
- ↑ Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 281
- ↑ 4.0 4.1 4.2 4.3 4.4 4.5 4.6 4.7 4.8 Medical Knowledge Self Assessment Program (MKSAP) 11, 14, 15, 16, 17, 18, 19. American College of Physicians, Philadelphia 1998, 2006, 2009, 2012, 2014, 2018, 2021.
Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022 - ↑ 5.0 5.1 5.2 UCLA Intensive Course in Geriatric Medicine & Board Review, Marina Del Ray, CA, Sept 12-15, 2001
- ↑ 6.0 6.1 6.2 Vaezi MF et al. ACG clinical guideline: Diagnosis and management of achalasia. Am J Gastroenterol 2013 Aug; 108:1238 PMID: https://www.ncbi.nlm.nih.gov/pubmed/23877351
- ↑ Francis DL, Katzka DA. Achalasia: update on the disease and its treatment. Gastroenterology. 2010 Aug;139(2):369-74 PMID: https://www.ncbi.nlm.nih.gov/pubmed/20600038
- ↑ 8.0 8.1 Allaix ME, Katz J (images) Medscape: Achalasia http://reference.medscape.com/article/169974-overview
- ↑ 9.0 9.1 Chen YI, Inoue H, Ujiki M et al. An international multicenter study evaluating the clinical efficacy and safety of per-oral endoscopic myotomy in octogenarians. Gastrointest Endosc 2017 Feb 21 <PubMed> PMID: https://www.ncbi.nlm.nih.gov/pubmed/28235595 <Internet> http://www.giejournal.org/article/S0016-5107(17)30109-8/abstract
- ↑ 10.0 10.1 Ponds FA, Fockens P, Lei A et al. Effect of peroral endoscopic myotomy vs pneumatic dilation on symptom severity and treatment outcomes among treatment- naive patients with achalasia: A randomized clinical trial. JAMA 2019 Jul 9; 322:134. PMID: https://www.ncbi.nlm.nih.gov/pubmed/31287522 https://jamanetwork.com/journals/jama/fullarticle/2737682
Jacobson BC, Lichtenstein DR. Peroral endoscopic myotomy (POEM) vs pneumatic dilation: Establishing a new therapeutic option for achalasia. JAMA 2019 Jul 9; 322:119. https://jamanetwork.com/journals/jama/fullarticle/2737663 - ↑ 11.0 11.1 NEJM Knowledge+ Gastroenterology
- ↑ Zaninotto G, Bennett C, Boeckxstaens G et al. The 2018 ISDE achalasia guidelines. Dis Esophagus. 2018;31. PMID: https://www.ncbi.nlm.nih.gov/pubmed/30169645