ICD-10-CM 2027
ICD-10 code for Penta X syndrome
From the official ICD-10-CM alphabetic index entry “Syndrome › penta X”. Page updated September 29, 2026.
About coding Penta X syndrome
The ICD-10-CM code for Penta X syndrome is Q97.1 (Female with more than three X chromosomes).
Within Q97 (other sex chromosome abnormalities, female phenotype, not elsewhere classified), Q97.1 is specifically for female with more than three X chromosomes. Related codes cover karyotype 47, XXX (Q97.0), mosaicism, lines with various numbers of X chromosomes (Q97.2), female with 46, XY karyotype (Q97.3), other specified sex chromosome abnormalities, female phenotype (Q97.8) and sex chromosome abnormality, female phenotype, unspecified (Q97.9).
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 742 (Uterine and Adnexa Procedures for Non-Malignancy with CC/MCC, relative weight 1.8456), DRG 743 (Uterine and Adnexa Procedures for Non-Malignancy without CC/MCC, relative weight 1.2974), DRG 760 (Menstrual and Other Female Reproductive System Disorders with CC/MCC, relative weight 1.0129) and DRG 761 (Menstrual and Other Female Reproductive System Disorders without CC/MCC, relative weight 0.7041), in MDC 13 (Diseases and Disorders of the Female Reproductive 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 Penta X syndrome ICD-10 codes
What is the ICD-10 code for Penta X syndrome?
- Q97.1 — Female with more than three X chromosomes.
Is Q97.1 billable?
- Yes. Q97.1 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can Q97.1 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict Q97.1 as a principal diagnosis.
Is Q97.1 a CC or MCC?
- No. Q97.1 is neither a CC nor an MCC under MS-DRG v44.0.