ICD-10 Code G71.220 – X-linked myotubular myopathy (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code G71.220 – X-linked myotubular myopathy
What it is
G71.220 identifies X-linked myotubular myopathy, an inherited muscle disorder that causes weakness because muscle fibers do not develop normally. Use it for documented X-linked myotubular myopathy, not for nonspecific muscle weakness.
Clinical signs
Typical findings include severe hypotonia, generalized weakness, and delayed motor development, often beginning at birth or in early infancy. Clinical features vary; refer to documentation.
When to use this code
Use this code when the provider documents X-linked myotubular myopathy, centronuclear myopathy due to the X-linked form, or a confirmed MTM1-related diagnosis. It also fits when the record clearly states the condition is congenital or inherited and specifically X-linked. Check documentation if the note only says myopathy.
Do not use for
Do not use this code for acquired myopathy, unspecified muscular dystrophy, or generalized weakness without a confirmed diagnosis. Do not assume the X-linked form if the genetic or clinical documentation is absent.
Coding tip
Code only when the chart explicitly links the myopathy to the X-linked form; otherwise, query for clarification.