ICD-10 Code for Mucopolysaccharidosis

The default ICD-10-CM 2027 code for mucopolysaccharidosis is E76.3 – Mucopolysaccharidosis, unspecified. More specific codes depend on the details documented, as listed in the official index (11 codes, 11 billable).

Main code: E76.3 – Mucopolysaccharidosis, unspecified
Billable: Yes

Index entries for Mucopolysaccharidosis

beta-gluduronidase deficiency – E76.29
cardiopathy – E76.3 – I52 [manifestation]
Hunter's syndrome – E76.1
Hurler's syndrome – E76.01
Hurler-Scheie syndrome – E76.02
Maroteaux-Lamy syndrome – E76.29
Morquio syndrome – E76.219
- A – E76.210
- B – E76.211
- classic – E76.210
Sanfilippo syndrome – E76.22
Scheie's syndrome – E76.03
specified NEC – E76.29
type
- I
  - Hurler's syndrome – E76.01
  - Hurler-Scheie syndrome – E76.02
  - Scheie's syndrome – E76.03
- II – E76.1
- III – E76.22
- IV – E76.219
- IVA – E76.210
- IVB – E76.211
- VI – E76.29
- VII – E76.29

How to choose the right code

Start with the main term, then pick the indented subterm that matches the documentation (type, cause, site, laterality, episode of care). Always confirm the code in the tabular list: follow its Excludes1/Excludes2, “code first” and “use additional code” notes, and add a 7th character where required.

Source: CDC/NCHS ICD-10-CM FY2027 alphabetic index.