2027 ICD-10-CM Diagnosis Code E76.22Sanfilippo mucopolysaccharidoses
ICD-10-CM Codes›E00–E89›E70-E88›E76
- Billable — Valid for Submission
- CC — Complication or Comorbidity
- Risk Adjusts — HCC 49
- Chronic Condition
E76.22 is a billable ICD-10-CM diagnosis code for sanfilippo mucopolysaccharidoses. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 642. 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 37 closely related codes. Coders also document this condition as mucopolysaccharidosis, MPS-III-A. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.
For Medicare Advantage risk adjustment, E76.22 maps to CMS-HCC Category 49 (Specified Lysosomal Storage Disorders) under the V28 model, adding a risk factor of about 9.256 for a community, non-dual, aged beneficiary in payment year 2027.
Code Identity
Code Classification
Medicare Risk Adjustment (HCC)Billing
E76.22 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.
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.
- Mucopolysaccharidosis, MPS-III-A
- Mucopolysaccharidosis, MPS-III-B
- Mucopolysaccharidosis, MPS-III-C
- Mucopolysaccharidosis, MPS-III-D
- Sanfilippo syndrome
Instructional NotesGuidance
Instructions from the official ICD-10-CM Tabular List that apply to E76.22: its own notes plus those printed at block E70-E88 and Chapter 4. A note printed at a category, block or chapter applies to every code under it.
Applicable To
Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.
- Mucopolysaccharidosis, type III (A) (B) (C) (D)
- Sanfilippo A syndrome
- Sanfilippo B syndrome
- Sanfilippo C syndrome
- Sanfilippo D syndrome
Excludes1
Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.
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.
- Ehlers-Danlos syndromes (Q79.6-)
Notes
General instructions on how these codes are used.
- All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
Source: CMS ICD-10-CM Tabular List. How to read instructional notes.
Referenced in Other NotesGuidance
Instructional notes printed at other codes that name E76.22, its category, or a range that includes it.
Excludes1 2
These codes carry an Excludes1 note naming E76.22: they are not reported together with it, unless the two conditions are unrelated.
Index to Diseases and InjuriesGuidance
Alphabetical index entries that point to this code.
Sanfilippo syndrome E76.22
III E76.22
Clinical ClassificationClinical
AHRQ’s CCSR groups this code into broader clinical categories.
Clinical InformationClinical
Mucopolysaccharidosis III
mucopolysaccharidosis characterized by heparan sulfate in the urine, progressive mental retardation, mild dwarfism, and other skeletal disorders. there are four clinically indistinguishable but biochemically distinct forms, each due to a deficiency of a different enzyme.
Patient EducationClinical
Carbohydrate Metabolism Disorders
Metabolism is the process your body uses to make energy from the food you eat. Food is made up of proteins, carbohydrates, and fats. Chemicals in your digestive system (enzymes) break the food parts down into sugars and acids, your body's fuel. Your body can use this fuel right away, or it can store the energy in your body tissues.
Read the full article at MedlinePlus
Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.
Convert E76.22 to ICD-9-CMHistory
The closest ICD-9-CM equivalents under the General Equivalence Mappings.
Code HistoryHistory
Questions About E76.22Overview
What is the ICD-10 code for sanfilippo mucopolysaccharidoses?
The ICD-10-CM code for sanfilippo mucopolysaccharidoses is E76.22 (sometimes written as E7622). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
Is E76.22 (Other mucopolysaccharidoses) a billable code?
Yes. This is a billable ICD-10-CM code, specific enough to report sanfilippo mucopolysaccharidoses on HIPAA-covered claims from October 1, 2026 through September 30, 2027.
What MS-DRG does E76.22 group to?
When sanfilippo mucopolysaccharidoses is the principal diagnosis on an inpatient stay, it groups to MS-DRG 642 (inborn and Other Disorders of Metabolism), which carries a relative weight of 1.3226. Higher weights mean higher Medicare reimbursement.
Is E76.22 a CC or MCC?
CMS lists E76.22 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 37 closely related codes in its exclusion list.
Can E76.22 be reported with a code from E34.5?
Not as a rule. The Excludes1 note at block E70-E88, which applies to E76.22, lists "androgen insensitivity syndrome (E34.5-)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).
What HCC is E76.22?
E76.22 (sanfilippo mucopolysaccharidoses) maps to CMS-HCC Category 49 (Specified Lysosomal Storage Disorders), commonly written as HCC 49, in the CMS-HCC V28 model used for Medicare Advantage risk adjustment in payment year 2027. It mapped to HCC 23 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 41.
Does E76.22 risk-adjust for Medicare Advantage payment?
Yes. When documented and reported on a Medicare Advantage encounter, E76.22 adds a risk adjustment factor of about 9.256 to the beneficiary's RAF score for a community, non-dual, aged enrollee (published V28 weights range from 1.528 to 13.778 depending on the payment segment). HCC 49 sits at the top of its hierarchy, so no other condition category supersedes it. See the full factor table on the HCC 49 category page.
