E71.0 — Maple-syrup-urine disease
ICD-10-CM
Prior Authorization
No Prior Auth Required
Code is covered without prior authorization (high confidence)
Documentation Required
- Note in policy: 'MRS is considered medically necessary for disease monitoring of these diagnoses when recent MRI findings are inconclusive and a change in therapy is being considered.'
- Implied / inferred requirement: documentation of recent MRI findings that are inconclusive (i.e., copies of MRI reports/images) and documentation that the MRS findings will be used to determine management (e.g., to determine whether biopsy/resection can be safely postponed or to distinguish recurrent tumor from radiation necrosis).
- Use of appropriate diagnosis code(s) consistent with the policy's 'ICD-10 codes covered if selection criteria are met'—the claim should reflect a covered indication listed in the policy when selection criteria are met.
- Use of the covered CPT code for MRS when performed for a listed medically necessary indication: 76390 (Magnetic resonance spectroscopy).
Key Coverage Criteria
- Diagnosis of the following disorders: Metachromatic leukodystrophy (MCL);
- Myocardial disease — (referenced as a clinical application of MRS)
- Distinguishing recurrent brain tumor from radiation-induced tumor necrosis;
- Diagnosis and monitoring of the following metabolic disorders: Canavan disease;
- Diagnosis and monitoring of the following metabolic disorders: Creatine deficiency;
- Diagnosis and monitoring of the following metabolic disorders: Nonketotic hyperglycinemia;
2 Active Policies
CIGNA-0136 — Infant Nutritional Formula - (0136)
AETNA-CPB-0202 — Magnetic Resonance Spectroscopy (MRS)
Related Codes
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