E76.210
BillableMorquio A mucopolysaccharidoses
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 A mucopolysaccharidosis is a rare inherited metabolic disorder characterized by severe skeletal deformities, short stature, and progressive joint and spine problems due to accumulation of complex sugars.
Coding Tips
- •Morquio A is the classic form; ensure you are not confusing it with Morquio B, which has different enzyme deficiency
- •Document associated complications such as cardiac valve disease, hearing loss, or vision problems for comprehensive coding
Clinical Significance
Morquio A mucopolysaccharidosis (MPS IVA) is caused by deficiency of N-acetylgalactosamine-6-sulfatase (GALNS), leading to accumulation of keratan sulfate and chondroitin-6-sulfate. It primarily affects the skeleton with severe short stature, spinal cord compression risk, and joint laxity while preserving intelligence, making it distinct from many other MPS types.
Documentation Requirements
- ✓Specific diagnosis of Morquio A syndrome (MPS IVA) documented
- ✓Confirmatory enzyme assay showing GALNS deficiency or genetic testing with GALNS gene mutation
- ✓Skeletal manifestations: short trunk dwarfism, pectus carinatum, genu valgum, odontoid hypoplasia
- ✓Cervical spine stability assessment (risk of atlantoaxial subluxation/spinal cord compression)
- ✓Treatment plan including enzyme replacement therapy (elosulfase alfa/Vimizim), surgical interventions, or supportive care
Commonly Confused Codes
E76.211 — Morquio B (MPS IVB): different enzyme deficiency (beta-galactosidase), same skeletal phenotypeE76.219 — Morquio, unspecified: use only when A vs. B subtype is not documentedE76.1 — Hunter syndrome (MPS II): has skeletal features but also hepatosplenomegaly and potential neurodegenerationQ77.4 — Achondroplasia: skeletal dysplasia that may mimic Morquio radiographically but is a different entityE76.22 — Sanfilippo syndrome (MPS III): primarily neurological, not skeletal
Code Hierarchy
└E76Disorders of glycosaminoglycan metabolism└E76.2Other mucopolysaccharidoses└E76.21Morquio mucopolysaccharidoses└E76.210Morquio A mucopolysaccharidoses
└E76.210Morquio A mucopolysaccharidoses