E76.29

HCC Category Mapping

What This Code Means

Other mucopolysaccharidoses include rare inherited metabolic disorders such as Sly syndrome and Maroteaux-Lamy syndrome, where the body cannot properly break down complex sugars.

Coding Tips

• •Use this code for MPS types IV (other than Morquio), VI, VII, and IX when specifically documented
• •Clearly document which specific mucopolysaccharidosis type is being coded to ensure accurate classification

Clinical Significance

Other mucopolysaccharidoses captures MPS types that do not have individual ICD-10-CM codes, including MPS VI (Maroteaux-Lamy), MPS VII (Sly syndrome), and MPS IX (Natowicz syndrome). These are rare but clinically significant lysosomal storage disorders requiring enzyme replacement therapy and multisystem monitoring.

Documentation Requirements

• ✓Specific MPS type documented (e.g., Maroteaux-Lamy/MPS VI, Sly syndrome/MPS VII, Natowicz/MPS IX)
• ✓Confirmatory enzyme assay or genetic testing identifying the specific enzyme deficiency
• ✓Current disease manifestations by organ system
• ✓Treatment status: enzyme replacement therapy (galsulfase for MPS VI), supportive interventions
• ✓Provider statement confirming this is a mucopolysaccharidosis type other than I, II, III, or IV

Commonly Confused Codes