E71.318

HCC Category Mapping

What This Code Means

A group of rare genetic disorders affecting the body's ability to break down fatty acids for energy, not classified under the more specific fatty acid oxidation disorders.

Coding Tips

• •This is a catch-all code; use only when the specific type of fatty acid oxidation disorder is not documented or does not fit other categories
• •Document the specific enzyme deficiency or metabolic pathway affected if known to support medical necessity

Clinical Significance

This code captures fatty acid oxidation disorders not classified in more specific E71.31x subcategories, including rare enzyme deficiencies such as 2,4-dienoyl-CoA reductase deficiency or mitochondrial trifunctional protein deficiency. These disorders impair the body's ability to derive energy from fats during fasting, creating risk of metabolic crises. Clinical presentations vary but may include hypoglycemia, cardiomyopathy, liver dysfunction, and skeletal myopathy.

Documentation Requirements

• ✓Document the specific fatty acid oxidation disorder identified, enzyme activity or genetic testing results, acylcarnitine profile abnormalities, and clinical manifestations.
• ✓Record treatment approach including dietary modifications and carnitine or riboflavin supplementation if applicable.

Commonly Confused Codes