E76.219
BillableMorquio mucopolysaccharidoses, unspecified
HCC Category Mapping
V28HCC 49 — Lysosomal Storage Disorders
0.226V24HCC 23 — Other Significant Endocrine and Metabolic Disorders
0.230ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders
0.000RxHCCHCC 41 — Lysosomal Storage Disorders
0.000What This Code Means
Morquio mucopolysaccharidosis, unspecified type, is a rare inherited metabolic disorder causing skeletal deformities and progressive physical problems when the specific type (A or B) is not documented.
Coding Tips
- •Use this code only when the specific Morquio type (A or B) cannot be determined from the medical record
- •Query the provider to determine the specific type if possible, as this affects treatment options and genetic counseling
Clinical Significance
Morquio mucopolysaccharidoses, unspecified is used when MPS IV is diagnosed but the subtype (A vs. B) has not been determined or documented. This code indicates a need for further diagnostic workup since treatment options differ between subtypes, particularly with elosulfase alfa being specific to Morquio A.
Documentation Requirements
- ✓Provider diagnosis of Morquio syndrome or MPS IV without specification of A or B subtype
- ✓Clinical findings consistent with MPS IV: skeletal dysplasia, short trunk dwarfism, joint laxity, corneal clouding
- ✓Reason subtype is not specified (testing pending, unavailable, or not yet ordered)
- ✓Current symptom management and care plan
- ✓Referral for enzyme assay or genetic testing if not yet performed
Commonly Confused Codes
E76.210 — Morquio A: use when GALNS deficiency is confirmedE76.211 — Morquio B: use when beta-galactosidase deficiency is confirmedE76.3 — Mucopolysaccharidosis, unspecified: even broader; use only when the MPS type is entirely unknownE76.29 — Other mucopolysaccharidoses: for non-Morquio MPS types that lack specific codes
Code Hierarchy
└E76Disorders of glycosaminoglycan metabolism└E76.2Other mucopolysaccharidoses└E76.21Morquio mucopolysaccharidoses└E76.219Morquio mucopolysaccharidoses, unspecified
└E76.219Morquio mucopolysaccharidoses, unspecified