2024 ICD-10-CM Diagnosis Code Q98.3

Other male with 46, XX karyotype

ICD-10-CM Code:
Short Description:
Other male with 46, XX karyotype
Is Billable?
Yes - Valid for Submission
Chronic Condition Indicator: [1]
Code Navigator:

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities
    • Chromosomal abnormalities, not elsewhere classified
      • Oth sex chromosome abnormalities, male phenotype, NEC

Q98.3 is a billable diagnosis code used to specify a medical diagnosis of other male with 46, xx karyotype. The code is valid during the current fiscal year for the submission of HIPAA-covered transactions from October 01, 2023 through September 30, 2024. The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

This code is applicable to male patients only. It is clinically and virtually impossible to use this code on a non-male patient.

Approximate Synonyms

The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • 46,XX ovotesticular disorder of sex development
  • 46,XX testicular disorder of sex development
  • Ovotesticular disorder of sex development
  • Palmoplantar keratoderma, 46,XX sex reversal, predisposition to squamous cell carcinoma syndrome
  • Sex phenotype-karyotype dissociation syndrome
  • XX males

Clinical Classification

Clinical Category:
Chromosomal abnormalities
CCSR Category Code:
Inpatient Default CCSR:
Y - Yes, default inpatient assignment for principal diagnosis or first-listed diagnosis.
Outpatient Default CCSR:
Y - Yes, default outpatient assignment for principal diagnosis or first-listed diagnosis.

Index to Diseases and Injuries References

The following annotation back-references for this diagnosis code are found in the injuries and diseases index. The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10-CM code(s).

Code Edits

The Medicare Code Editor (MCE) detects and reports errors in the coding of claims data. The following ICD-10-CM Code Edits are applicable to this code:

  • Diagnoses for males only - The Medicare Code Editor detects inconsistencies between a patient’s sex and any diagnosis on the patient’s record, these edits apply to MALES only .

Present on Admission (POA)

Q98.3 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here.

CMS POA Indicator Options and Definitions

POA IndicatorReason for CodeCMS will pay the CC/MCC DRG?
YDiagnosis was present at time of inpatient admission.YES
NDiagnosis was not present at time of inpatient admission.NO
UDocumentation insufficient to determine if the condition was present at the time of inpatient admission.NO
WClinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.YES
1Unreported/Not used - Exempt from POA reporting. NO

Convert Q98.3 to ICD-9-CM

  • ICD-9-CM Code: 758.7 - Klinefelter's syndrome
    Approximate Flag - The approximate mapping means there is not an exact match between the ICD-10 and ICD-9 codes and the mapped code is not a precise representation of the original code.

Patient Education

Klinefelter Syndrome

What is Klinefelter syndrome (KS)?

Klinefelter syndrome (KS) is a genetic condition that happens when a male is born with an extra copy of the X chromosome. KS can affect different stages of physical, language, and social development. It also usually causes infertility.

What causes Klinefelter syndrome (KS)?

KS is not inherited. It's caused by a random error that happens when a sperm or egg is formed. This error causes a male to be born with an extra X chromosome.

Chromosomes are tiny "packages" in your cells that contain your genes. Genes carry information, called DNA, that controls what you look like and how your body works. Normally, you're born with a set of 46 chromosomes in each cell, two of which are the sex chromosomes. Females usually have two X chromosomes (XX), and males usually have one X and one Y (XY). In KS, the male usually has two X chromosomes and one Y (XXY).

In rare cases, a male could have XY in some cells and XXY in other cells. This is called mosaic Klinefelter syndrome. Males with mosaic Klinefelter syndrome may have fewer symptoms, depending on the number of XY cells they have in their bodies and where those cells are located.

What are the symptoms of Klinefelter syndrome (KS)?

Some males with KS may have no symptoms or very mild symptoms. So they might not know that they have KS, or they might not get diagnosed with it right away. In other cases, the symptoms can be more severe.

Boys with KS may be taller than other boys their age, with more fat around the belly. After puberty, they may have:

  • Smaller testes and penis
  • Breast growth (called gynecomastia)
  • Less facial and body hair
  • Reduced muscle tone
  • Narrower shoulders and wider hips
  • Weaker bones
  • Decreased sexual interest
  • Lower energy

Many of these symptoms happen because of low testosterone in the body. Testosterone is the main male sex hormone.

Boys with KS may also have learning or language problems. These problems may affect them socially, so they may be shy and quiet and can have trouble fitting in.

Most males with KS are infertile because they make little or no sperm.

How is Klinefelter syndrome (KS) diagnosed?

A genetic test called a karyotype test can diagnose KS. This test can show if there are abnormal chromosomes, including if there is an extra X chromosome.

What are the treatments for Klinefelter syndrome (KS)?

There is no cure for KS, but treatments are available. The sooner the treatment is started, the better. If treatment is started by early puberty, it will likely help in reducing the symptoms.

Treatments for KS may include:

  • Testosterone replacement therapy
  • Surgery to remove or reduce breasts
  • Physical, speech, behavioral, and occupational therapy

In some cases, fertility treatments may help men with KS father children.

NIH: National Institute of Child Health and Human Development

[Learn More in MedlinePlus]

Code History

  • FY 2024 - No Change, effective from 10/1/2023 through 9/30/2024
  • FY 2023 - No Change, effective from 10/1/2022 through 9/30/2023
  • FY 2022 - No Change, effective from 10/1/2021 through 9/30/2022
  • FY 2021 - No Change, effective from 10/1/2020 through 9/30/2021
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016. This was the first year ICD-10-CM was implemented into the HIPAA code set.


[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.