ICD-10 Code E71.0 – Maple-syrup-urine disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.0 – Maple-syrup-urine disease
What it is
E71.0 identifies maple-syrup-urine disease, an inherited disorder of branched-chain amino acid metabolism. It causes accumulation of leucine, isoleucine, and valine because the body cannot break them down normally.
Clinical signs
Clinical features vary; refer to documentation. Typical findings include poor feeding, vomiting, lethargy, neurologic decline, and the characteristic sweet odor of urine or cerumen in affected patients.
When to use this code
Use this code when the record clearly documents maple-syrup-urine disease, including congenital or longstanding disease. It may also support encounters for evaluation, monitoring, or management of the disorder when the diagnosis is established. Check documentation if the note only mentions abnormal amino acids without naming the condition.
Do not use for
Do not use E71.0 for nonspecific metabolic abnormalities, transient neonatal feeding problems, or suspected disease without confirmation. Do not assign it when the chart only lists family history or carrier status.
Coding tip
Use the confirmed diagnosis from the provider’s assessment, not just screening or lab language, before assigning E71.0.