2026 ICD-10-CM Diagnosis Code K22.0Achalasia of cardia

ICD-10-CM CodesK00–K95K20-K31K22

ICD-10-CM K22.0
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

K22.0 is a billable ICD-10-CM diagnosis code for achalasia of cardia. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026) and groups to MS-DRG 391 through 392. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Esophageal disorders.

Code Identity

ICD-10-CM Code
K22.0
Billable Status
Yes — Valid for Submission
Code Describes
Achalasia of cardia
Short Description
Achalasia of cardia
Same as the full description in the CMS dataset.
Parent Code
Other diseases of esophagus

Code Classification

ChapterK00–K95Diseases of the digestive system
SectionK20-K31Diseases of esophagus, stomach and duodenum
CategoryK22Other diseases of esophagus
This CodeK22.0Achalasia of cardia

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Absent peristalsis
  • Achalasia
  • Achalasia due to Chagas disease
  • Achalasia of esophagus
  • Acquired achalasia of esophagus
  • Adrenocorticotropic hormone resistance syndrome
  • Aperistalsis of esophagus
  • Congenital achalasia of esophagus
  • Dilatation of esophagus
  • Disorder of esophageal peristalsis
  • Glucocorticoid deficiency with achalasia
  • Hypertensive spasm of cardiac sphincter
  • Idiopathic achalasia of esophagus
  • Idiopathic megaesophagus
  • Megaesophagus
  • Moyamoya disease
  • Moyamoya disease with early onset achalasia
  • Pituitary thyroid hormone resistance
  • Primary adrenocortical insufficiency
  • Resistance to thyroid hormone syndrome

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Achalasia NOS
  • Cardiospasm

Type 1 Excludes

  • congenital cardiospasm Q39.5

Index to Diseases and InjuriesGuidance

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Achalasia(cardia) (esophagus)
    • Aperistalsis, esophagus
    • Cardiospasm(esophagus) (reflex) (stomach)
    • Dilatation
      • esophagus
        • due to achalasia
    • Incompetency, incompetent, incompetence
      • esophagogastric (junction) (sphincter)
    • Megaesophagus(functional)
    • Megaloesophagus(functional)
    • Spasm(s), spastic, spasticity
      • cardia
    • Syndrome
      • gastroesophageal
        • junction

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR DIG004
Esophageal disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Moyamoya Disease

    a noninflammatory, progressive occlusion of the intracranial carotid arteries and the formation of netlike collateral arteries arising from the circle of willis. cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. it is characterized by endothelial hyperplasia and fibrosis with thickening of arterial walls. this disease primarily affects children but can also occur in adults.
  • AAAS wt Allele|AAA|AAASb|ADRACALA|ADRACALIN|ALADIN|Achalasia, Adrenocortical Insufficiency, Alacrimia (Allgrove, Triple-A) Gene|Achalasia, Adrenocortical Insufficiency, Alacrimia Gene|Aladin WD Repeat Nucleoporin wt Allele|Allgrove, Triple-A Gene|GL003

    human aaas wild-type allele is located in the vicinity of 12q13.13 and is approximately 17 kb in length. this allele, which encodes aladin protein, is involved in peripheral and central nervous system development and mitotic spindle formation. mutations in the gene are associated with achalasia-addisonianism-alacrima syndrome (allgrove or triple-a syndrome).
  • Achalasia

    failure of the smooth muscle fibers of the gastrointestinal tract to relax at any one point of junction of one part with another.
  • Esophageal Achalasia|Achalasia Cardia|Achalasia of Cardia

    a finding indicating the lack of adequate relaxation of the lower esophageal sphincter resulting in difficulty swallowing food.
  • Triple A Syndrome|3A syndrome|Achalasia-Addisonianism-Alacrima Syndrome|Allgrove Syndrome|Allgrove Syndrome|Triple-A syndrome

    an autosomal recessive condition caused by mutation(s) in the aaas gene encoding the protein aladin, and characterized by primary adrenal insufficiency, esophageal dysmotility, and absence of tear production.
  • Moyamoya Disease

    a rare inherited vascular disorder characterized by constriction of arteries at the base of the brain, resulting in the formation of collateral circulation in order to compensate for the constriction. the name "moyamoya" in japanese means "puff of smoke" and derives from the characteristic radiographic appearance of the collateral vessels.
  • Moyamoya Disease 2|MYMY2

    an autosomally inherited subtype of moyamoya disease often presenting in childhood caused by mutation(s) in the rnf213 gene, encoding e3 ubiquitin-protein ligase rnf213.
  • RNF213 wt Allele|ALK Lymphoma Oligomerization Partner on Chromosome 17 Gene|ALO17|C17orf27|Chromosome 17 Open Reading Frame 27 Gene|DKFZp762N1115|FLJ13051|KIAA1554|KIAA1618|MGC46622|MGC9929|MYMY2|MYSTR|Moyamoya Disease 2 Gene|NET57|Ring Finger Protein 213 wt Allele|hCG_1812857

    human rnf213 wild-type allele is located in the vicinity of 17q25.3 and is approximately 135 kb in length. this allele, which encodes e3 ubiquitin-protein ligase rnf213 protein, may play a role in the regulation of protein ubiquitination. a chromosomal translocation t(2;17)(p23;q25) of this gene with the alk gene is associated with anaplastic large cell lymphoma.
  • Megaesophagus

    an abnormal dilation of the esophagus not due to obstruction.
  • Alacrima, Achalasia, and Impaired Intellectual Development Syndrome|AAMR

    an autosomal recessive condition caused by mutation(s) in the gmppa gene, encoding mannose-1-phosphate guanyltransferase alpha. it is characterized by alacrima, achalasia, and impaired intellectual development.

Patient EducationClinical

Esophagus Disorders

The esophagus is the muscular tube that carries food and liquids from your mouth to the stomach. You may not be aware of your esophagus until you swallow something too large, too hot, or too cold. You may also notice it when something is wrong. You may feel pain or have trouble swallowing.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert K22.0 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
530.0 Achalasia & cardiospasm
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About K22.0Overview

Is K22.0 (Other diseases of esophagus) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report achalasia of cardia on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What MS-DRG does K22.0 group to?

When achalasia of cardia is the principal diagnosis on an inpatient stay, it groups to MS-DRG 391, 392, with relative weights from 0.7796 to 1.2683 depending on complications. Higher weights mean higher Medicare reimbursement.

What is the ICD-9 equivalent of K22.0?

Under the General Equivalence Mappings, achalasia of cardia converts to ICD-9-CM 530.0 (achalasia & cardiospasm). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.