2027 ICD-10-CM Diagnosis Code Q04.5Megalencephaly

ICD-10-CM Codes›Q00-Q99›Q00-Q07›Q04

ICD-10-CM Q04.5
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

Q04.5 is a billable ICD-10-CM diagnosis code for megalencephaly. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 91 through 93, 791, 793. 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 4 closely related codes. The code is exempt from POA reporting. Coders also document this condition as bilateral megalencephaly. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Nervous system congenital anomalies.

For Medicare Advantage risk adjustment, Q04.5 maps to CMS-HCC Category 182 (Spinal Cord Disorders/Injuries) under the V28 model, adding a risk factor of about 0.478 for a community, non-dual, aged beneficiary in payment year 2027.

Code Identity

ICD-10-CM Code
Q04.5
Billable Status
Yes — Valid for Submission
Code Describes
Megalencephaly
Short Description
Megalencephaly
Same as the full description in the CMS dataset.
Parent Code
Other congenital malformations of brain

Code Classification

ChapterQ00-Q99Congenital malformations, deformations and chromosomal abnormalities
SectionQ00-Q07Congenital malformations of the nervous system
CategoryQ04Other congenital malformations of brain
This CodeQ04.5Megalencephaly

Present on Admission (POA)Billing

Q04.5 is exempt from POA reporting on inpatient claims to general acute care hospitals. Review the other POA exempt codes in Congenital malformations of the nervous system (Q00-Q07).

Medicare Risk Adjustment (HCC)Billing

Q04.5 maps to a payment category in the CMS-HCC model used to risk-adjust Medicare Advantage payments. Weights are the published community factors for payment year 2027.

CMS-HCC V28 Category (Payment Model)
HCC 182— Spinal Cord Disorders/Injuries
Payment HCC · PY 2027 one of 251 ICD-10-CM codes in this category
Risk Adjustment Factor (RAF) Weight
+0.478
community, non-dual, aged · ranges 0.270–0.478 across segments
Hierarchy
Superseded by HCC 180, HCC 181, HCC 191, and HCC 192
a more severe related category takes the payment when both are reported
Prior Model (CMS-HCC V24)
HCC 72
V24 retired V28 pays 100% of MA risk scores since PY 2026
Other CMS Models
PACE (CMS-HCC V22): HCC 72 · ESRD (V21): HCC 72 · ESRD (V24): HCC 72
ESRD V21 weights: 0.102 dialysis, 0.256–0.456 functioning graft · ESRD V24 weights: 0.099 dialysis, 0.222–0.523 functioning graft
Part D (RxHCC)
RxHCC 155 — Spinal Cord Disorders
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.

  • Bilateral megalencephaly
  • Familial megalencephaly
  • Hemimegalencephaly
  • Macroencephaly
  • Megalencephalic leukoencephalopathy with subcortical cysts
  • Megalencephaly, polymicrogyria, postaxial polydactyly, hydrocephalus syndrome
  • Megalencephaly, severe kyphoscoliosis, overgrowth syndrome
  • Polyhydramnios, megalencephaly, symptomatic epilepsy syndrome
  • Sporadic megalencephaly

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to Q04.5: its own notes plus those printed at Q04 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 Q04 Other congenital malformations of brain 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)

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.

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 MAL004
Nervous system congenital anomalies
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Megalencephaly

    a congenital abnormality in which the occipitofrontal circumference is greater than two standard deviations above the mean for a given age. it is associated with hydrocephalus; subdural effusion; arachnoid cysts; or is part of a genetic condition (e.g., alexander disease; sotos syndrome).

Patient EducationClinical

Brain Malformations

Most brain malformations begin long before a baby is born. Something damages the developing nervous system or causes it to develop abnormally. Sometimes it's a genetic problem. In other cases, exposure to certain medicines, infections, or radiation during pregnancy interferes with brain development.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert Q04.5 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
742.4 Brain anomaly NEC
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About Q04.5Overview

What is the ICD-10 code for megalencephaly?

The ICD-10-CM code for megalencephaly is Q04.5 (sometimes written as Q045). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is Q04.5 (Other congenital malformations of brain) a billable code?

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

What MS-DRG does Q04.5 group to?

When megalencephaly is the principal diagnosis on an inpatient stay, it groups to MS-DRG 793, 791, 91, 92, 93, with relative weights from 0.7783 to 4.2192 depending on complications. Higher weights mean higher Medicare reimbursement.

Is Q04.5 a CC or MCC?

CMS lists Q04.5 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 4 closely related codes in its exclusion list.

Is Q04.5 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 megalencephaly on inpatient claims. The code appears in the Congenital malformations of the nervous system (Q00-Q07) range of the CMS exempt list.

What HCC is Q04.5?

Q04.5 (megalencephaly) maps to CMS-HCC Category 182 (Spinal Cord Disorders/Injuries), commonly written as HCC 182, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 72 under the retired V24 model. It also maps in the PACE (CMS-HCC V22), ESRD (V21), and ESRD (V24) models. In the Part D prescription drug model it maps to RxHCC 155.

Does Q04.5 risk-adjust for Medicare Advantage payment?

Yes. When documented and reported on a Medicare Advantage encounter, Q04.5 adds a risk adjustment factor of about 0.478 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 0.270 to 0.478 depending on the payment segment). A more severe related category (HCC 180, HCC 181, HCC 191, and HCC 192) supersedes it when both are reported. See the full factor table on the HCC 182 category page.