ICD-10-CM 2027
ICD-10 code for pylorus achalasia
From the official ICD-10-CM alphabetic index entry “Achalasia (cardia) (esophagus) › pylorus”. Page updated September 29, 2026.
About coding pylorus achalasia
The ICD-10-CM code for pylorus achalasia is Q40.0 (Congenital hypertrophic pyloric stenosis).
Within Q40 (other congenital malformations of upper alimentary tract), Q40.0 is specifically for congenital hypertrophic pyloric stenosis. Related codes cover congenital hiatus hernia (Q40.1), other specified congenital malformations of stomach (Q40.2), congenital malformation of stomach, unspecified (Q40.3), other specified congenital malformations of upper alimentary tract (Q40.8) and congenital malformation of upper alimentary tract, unspecified (Q40.9).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 393 (Other Digestive System Diagnoses with MCC, relative weight 1.5881), DRG 394 (Other Digestive System Diagnoses with CC, relative weight 0.9228) and DRG 395 (Other Digestive System Diagnoses without CC/MCC, relative weight 0.6382), in MDC 06 (Diseases and Disorders of the Digestive System), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about pylorus achalasia ICD-10 codes
What is the ICD-10 code for pylorus achalasia?
- Q40.0 — Congenital hypertrophic pyloric stenosis.
Is Q40.0 billable?
- Yes. Q40.0 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can Q40.0 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict Q40.0 as a principal diagnosis.
Is Q40.0 a CC or MCC?
- No. Q40.0 is neither a CC nor an MCC under MS-DRG v44.0.