ICD-10 Code E71.311 – Medium chain acyl CoA dehydrogenase deficiency (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.311 – Medium chain acyl CoA dehydrogenase deficiency
What it is
E71.311 identifies medium chain acyl CoA dehydrogenase deficiency, an inherited disorder of fatty acid metabolism. It means the body cannot properly break down medium-chain fats for energy.
Clinical signs
Clinical features vary; refer to documentation. Commonly documented findings include recurrent hypoglycemia, vomiting, lethargy, and metabolic decompensation, especially during fasting or illness.
When to use this code
Use E71.311 when the clinician documents confirmed medium chain acyl CoA dehydrogenase deficiency. You may also use it when the record clearly states MCAD deficiency, medium-chain acyl-CoA dehydrogenase deficiency, or a related inherited fatty acid oxidation disorder.
Do not infer the diagnosis from nonspecific metabolic symptoms alone. If the chart only notes abnormal screening, suspected disease, or family history, check documentation before assigning this code.
Do not use for
Do not use this code for general hypoglycemia, vomiting, or poor feeding without a documented MCAD deficiency diagnosis. It is also not for carrier status or screening results alone.
Coding tip
Code only when the provider documents the disorder explicitly; if the wording is unclear, check documentation.