Other disorders of fatty-acid metabolism

This code signifies a group of rare genetic conditions affecting the body's ability to break down fatty acids for energy, excluding specific, more common fatty acid oxidation disorders. These metabolic errors can lead to a buildup of toxic fatty acid intermediates, impacting various organ systems.

Apply this code when documentation indicates a diagnosis of a fatty-acid metabolism disorder that is not otherwise specified or does not have a more precise ICD-10-CM code. This typically includes diagnoses such as multiple acyl-CoA dehydrogenase deficiency (MADD) or other unclassified fatty acid oxidation defects. Supporting documentation should clearly state the diagnosis of an "other" or "unspecified" fatty acid metabolism disorder.

• Ensure documentation explicitly rules out more specific fatty acid oxidation disorders (e.g., E71.310 for long-chain acyl-CoA dehydrogenase deficiency).
• Review the medical record for any genetic testing results or enzyme assays that might provide a more precise diagnosis.
• Always sequence this code as a primary diagnosis when it is the reason for the encounter or admission.