Q40.0Congenital hypertrophic pyloric stenosis

Q40.0 is the ICD-10-CM diagnosis code for congenital hypertrophic pyloric stenosis. It belongs to category Q40 (other congenital malformations of upper alimentary tract), block Q38-Q45 (other congenital malformations of the digestive system) and chapter 17 (congenital malformations, deformations, chromosomal abnormalities, and genetic disorders). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.

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.

It is exempt from present-on-admission (POA) reporting, so no POA indicator is required.

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Before ICD-10, this condition was coded in ICD-9-CM as 750.5 (congenital hypertrophic pyloric stenosis).