E76.1

Billable

Mucopolysaccharidosis, type II

HCC Category Mapping

V28HCC 49 — Lysosomal Storage Disorders

0.226

V24HCC 23 — Other Significant Endocrine and Metabolic Disorders

0.230

ESRDHCC 23 — Other Significant Endocrine and Metabolic Disorders

0.000

RxHCCHCC 41 — Lysosomal Storage Disorders

0.000

What This Code Means

Mucopolysaccharidosis type II, also known as Hunter syndrome, is an inherited disorder where the body accumulates complex sugars in cells, causing progressive damage to organs, bones, and the nervous system.

Coding Tips

•MPS II is X-linked recessive; document whether the patient is a carrier, affected male, or affected female
•Specify the severity (mild, moderate, or severe) if documented, as this affects management and prognosis

Clinical Significance

Mucopolysaccharidosis type II (Hunter syndrome) is an X-linked recessive lysosomal storage disorder caused by deficiency of iduronate-2-sulfatase, leading to accumulation of dermatan and heparan sulfate. It primarily affects males and ranges from severe (with neurodegeneration) to attenuated forms, requiring enzyme replacement therapy and multidisciplinary care.

Documentation Requirements

✓Specific diagnosis of MPS II (Hunter syndrome) documented by provider
✓Confirmatory enzyme assay showing iduronate-2-sulfatase deficiency or IDS gene mutation analysis
✓Disease severity classification (severe/neuronopathic vs. attenuated/non-neuronopathic)
✓Current manifestations: coarse facial features, hepatosplenomegaly, cardiac involvement, skeletal abnormalities, airway obstruction
✓Treatment status: enzyme replacement therapy (idursulfase/Elaprase), intrathecal therapy, or supportive care

Commonly Confused Codes

E76.01 — Hurler syndrome (MPS I-H): autosomal recessive, different enzyme (alpha-L-iduronidase), includes corneal clouding (absent in Hunter)E76.22 — Sanfilippo syndrome (MPS III): primarily neurological with behavioral changes, different enzyme deficiencies E76.210 — Morquio A (MPS IVA): predominantly skeletal with normal intelligence E76.29 — Other mucopolysaccharidoses: for MPS types without specific codes (VI, VII, IX)

Code Hierarchy

└E76Disorders of glycosaminoglycan metabolism └E76.1Mucopolysaccharidosis, type II

└E76.1Mucopolysaccharidosis, type II