ICD-10 Code E71.118 – Other branched-chain organic acidurias (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.118 – Other branched-chain organic acidurias
What it is
E71.118 identifies a branched-chain organic aciduria that does not fit a more specific ICD-10-CM code. It is used for inherited metabolic disorders involving abnormal breakdown of branched-chain amino acids.
Clinical signs
Clinical features vary; refer to documentation. Patients may present with metabolic decompensation, poor feeding, vomiting, lethargy, or developmental concerns, depending on the specific disorder.
When to use this code
Use this code when the record documents an other or unspecified branched-chain organic aciduria and no more specific diagnosis is named. It may also apply when genetics or metabolic testing confirms the disorder, but the provider documents it broadly rather than by subtype.
Do not select it for a suspected condition without confirmation unless the documentation supports a confirmed diagnosis. Check documentation for the exact metabolic disorder name before coding.
Do not use for
Do not use this code for a specific branched-chain organic aciduria that has its own ICD-10-CM code. Do not use it when the chart only describes abnormal screening results or a family history.
Coding tip
Match the provider’s exact metabolic diagnosis wording, and default to the more specific code when one is documented.