AUTHORS: Hannah Fiske, MDand Harlan G. Rich, MD, FACP, AGAF
Achalasia is a motility disorder of the esophagus classically characterized by incomplete relaxation of the lower esophageal sphincter (LES) and aperistalsis of the esophageal smooth muscle resulting in functional obstruction of the esophagus.
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TABLE 1 Esophageal Motor Disorders
Achalasia | Scleroderma | Distal Esophageal Spasm | |
---|---|---|---|
Symptoms | Dysphagia Regurgitation of nonacidic material | Gastroesophageal reflux disease Dysphagia | Substernal chest pain (angina-like) Dysphagia with pain |
Radiographic appearance | Dilated, fluid-filled esophagus Distal bird-beak stricture | Aperistaltic esophagus Free reflux Peptic stricture | Simultaneous noncoordinated contractions |
Manometric findings | High resting pressure | Low resting pressure | Normal pressure |
Lower esophageal sphincter | Incomplete or abnormal relaxation with swallow | ||
Body | Low-amplitude, simultaneous contractions after swallowing | Low-amplitude peristaltic contractions or no peristalsis | Some peristalsis Diffuse and simultaneous nonperistaltic contractions, occasionally high amplitude |
From Andreoli TE et al: Andreoli and Carpenters Cecil essentials of medicine, ed 8, Philadelphia, 2010, Saunders.
TABLE 2 Chicago Classification v4.0 of Esophageal Motility Disorders5
Disorder | Diagnostic Criteria | ||
---|---|---|---|
Disorders of EGJ Outflow | |||
Type I achalasia (classic) | Abnormal median IRP and 100% failed peristalsis | ||
Type II achalasia | Abnormal median IRP, 100% failed peristalsis, and ≥20% of swallows with panesophageal pressurization | ||
Type III achalasia | Abnormal median IRP and ≥20% of swallows with premature/spastic contractions and no evidence of peristalsis | ||
EGJ outflow obstruction | Abnormal median IRP (supine and upright), ≥20% elevated intrabolus pressure (supine), and sufficient evidence of peristalsis such that criteria for achalasia are not met; diagnosis requires clinical symptoms in addition to manometric criteria | ||
Disorders of Peristalsis | |||
DES | Normal median IRP and ≥20% swallows with premature/spastic contractions with DCI >450 mm Hg•sec•cm; diagnosis requires clinical symptoms in addition to manometric criteria | ||
Hypercontractile (jackhammer) esophagus | Normal median IRP and ≥20% hypercontractile swallows with DCI >8000 mm Hg•sec•cm; diagnosis requires clinical symptoms in addition to manometric criteria | ||
Absent contractility | Normal median IRP and 100% failed peristalsis/absent contractility (DCI <100 mm Hg•sec•cm) | ||
Ineffective esophageal motility (includes the definition of fragmented peristalsis) | >70% ineffective swallows or with DCI <450 mm Hg•sec•cm or ≥50% failed peristalsis |
DES, Diffuse esophageal spasm; DCI, distal contractile interval; DL, distal latency; EGJ, esophagogastric junction; IRP, integrated relaxation pressure ULN, upper limits of normal.
FIG E1 Algorithm for applying the Chicago classification of esophageal motor disorders.
CFV, Contractile front velocity; DCI, distal contractile interval; DL, distal latency; EGJ, esophagogastric junction; IBC, isobar contour; IRP, integrated relaxation pressure; PEP, panesophageal pressurization; ULN, upper limits of normal (see Table 2 for more details).
From Yadlapati R et al: What is new in Chicago Classification version 4.0, Neurogastroenterol Motil 33(1):e14053, 2021. https://doi.org/10.1111/nmo.14053.
Barium swallow with fluoroscopy (particularly a timed barium esophagram (TBE)) may demonstrate:
Manometry (Fig. E4) is considered the gold standard test to confirm the diagnosis. High-resolution manometry (HRM) or high-resolution esophageal pressure topography (HREPT) has defined subsets of patients with achalasia who may have different responses to medical or surgical therapies and prognoses. This technique uses the integrated relaxation pressure (IRP) >15 mm Hg to define better the failure of esophagogastric junction relaxation. HRM also utilizes the distal contraction integral (DCI) to define hypercontractile vs. weak swallows vs. failed peristalsis. The distal latency (DL) defines premature contractions.
FLIP is a new technique that can measure compliance and distensibility across the esophagogastric junction and may demonstrate both impaired LES relaxation and response to achalasia therapy.6
FIG E2 Classic esophagram of a patient with achalasia.
The esophagus is dilated with a birds beak tapering of the distal esophagus. Retained secretions form the heterogeneous air-fluid level seen at the top of the barium column.
From Flint PW et al: Cummings otolaryngology, head and neck surgery, ed 7, Philadelphia 2021, Elsevier.
FIG E3 Esophagram of late-stage achalasia.
The esophagus now has a sigmoidlike tortuosity with a large amount of retained debris.
From Flint PW et al: Cummings otolaryngology, head and neck surgery, ed 7, Philadelphia 2021, Elsevier.
FIG E4 Manometric findings in achalasia.
Aperistalsis is manifested by isobaric contractions without propagation. The lower esophageal sphincter pressure, which is elevated, shows minimal relaxation with swallowing.
From Flint PW et al: Cummings otolaryngology, head and neck surgery, ed 7, Philadelphia 2021, Elsevier.