2027 ICD-10-CM Diagnosis Code Q87.86Kleefstra syndrome

ICD-10-CM Codes›Q00-Q99›Q80-Q89›Q87

ICD-10-CM Q87.86
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q87.86 is a billable ICD-10-CM diagnosis code for kleefstra syndrome. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 564 through 566. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 40 closely related codes. The code is exempt from POA reporting. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Chromosomal abnormalities.

Q87.86 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model. It still risk-adjusts in the RxHCC Part D (V08) category 148 for payment year 2027.

Code Identity

ICD-10-CM Code
Q87.86
Billable Status
Yes — Valid for Submission
Code Describes
Kleefstra syndrome
Short Description
Kleefstra syndrome
Same as the full description in the CMS dataset.
Parent Code
Q87.8
Other specified congenital malformation syndromes, not elsewhere classified

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ80-Q89Other congenital malformations
CategoryQ87Other specified congenital malformation syndromes affecting multiple systems
This CodeQ87.86Kleefstra syndrome

Present on Admission (POA)Billing

Q87.86 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Other congenital malformations (Q80-Q89).

Medicare Risk Adjustment (HCC)Billing

Q87.86 does not risk-adjust for Medicare Advantage under the CMS-HCC V28 model, but it maps to payment categories in the other CMS models shown below.

CMS-HCC V28 (Medicare Advantage Payment Model)
Not mapped
no payment category in the model that pays 100% of MA risk scores
Prior Model (CMS-HCC V24)
Not mapped under V24
this diagnosis newly risk-adjusts under the V28 model
Part D (RxHCC)
RxHCC 148 — Mild or Unspecified Intellectual Disability/Developmental Disorder
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Kleefstra syndrome

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q87.86: its own notes plus those printed at Q87.8, Q87, and Chapter 17. A note printed at a category, block or chapter applies to every code under it.

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From Q87.8 Other specified congenital malformation syndromes, not elsewhere classified applies to 9 codes

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • inborn errors of metabolism (E70-E88)

Use Additional Code

Add a second code, after this one, to fully describe the condition.

From Q87 Other specified congenital malformation syndromes affecting multiple systems applies to 21 codes
  • code(s) to identify all associated manifestations

Notes

General instructions on how these codes are used.

From Chapter 17 (Q00-QA1) Congenital malformations, deformations and chromosomal abnormalities applies to 900 codes
  • Codes from this chapter are not for use on maternal records

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name Q87.86, its category, or a range that includes it.

Excludes1 3

These codes carry an Excludes1 note naming Q87.86: they are not reported together with it, unless the two conditions are unrelated.

  • Q67 Congenital musculoskeletal deformities of head, face, spine and chest
    congenital malformation syndromes classified to Q87.- names Q87.-, which includes this code
  • Q75 Other congenital malformations of skull and face bones
    congenital malformation syndromes classified to Q87.- names Q87.-, which includes this code
  • Q89.7 Multiple congenital malformations, not elsewhere classified
    congenital malformation syndromes affecting multiple systems (Q87.-) names Q87.-, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

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

CCSR MAL009
Chromosomal abnormalities
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Kleefstra Syndrome

    a condition caused by mutation or deletion of the ehmt1 gene, encoding histone-lysine n-methyltransferase ehmt1. it is characterized by severe intellectual disability, hypotonia, cardiac defects, and characteristic facial features.

Code History & ChangesHistory

Replacement Q87.86 replaces the following previously assigned code(s):

  • Q87.89 - Oth congenital malformation syndromes, NEC
FY 2025AddedAdded to the ICD-10-CM code setEffective October 1, 2024.
FY 2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q87.86Overview

What is the ICD-10 code for kleefstra syndrome?

The ICD-10-CM code for kleefstra syndrome is Q87.86 (sometimes written as Q8786). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is Q87.86 a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report kleefstra syndrome on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does Q87.86 group to?

When kleefstra syndrome is the principal diagnosis on an inpatient stay, it groups to MS-DRG 564, 566, 565, with relative weights from 0.7374 to 1.4909 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q87.86 a CC or MCC?

CMS lists Q87.86 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 40 closely related codes in its exclusion list.

Is Q87.86 exempt from POA reporting?

Yes. CMS lists this code among those exempt from present on admission reporting, so hospitals do not assign a POA indicator for kleefstra syndrome on inpatient claims. The code appears in the Other congenital malformations (Q80-Q89) range of the CMS exempt list.

Does Q87.86 risk-adjust for Medicare Advantage payment?

Not for Medicare Advantage: Q87.86 maps to no category in the CMS-HCC V28 model. It still risk-adjusts in the RxHCC Part D (V08) category 148 (Mild or Unspecified Intellectual Disability/Developmental Disorder).