Other glycogen storage disease

This code represents a group of rare, inherited metabolic disorders characterized by defects in enzymes involved in glycogen synthesis or breakdown, leading to abnormal accumulation or structure of glycogen in various tissues. These disorders can manifest with a wide range of symptoms affecting the liver, muscles, heart, and other organs.

Use this code when documentation specifies a glycogen storage disease that does not have a more specific ICD-10-CM code (e.g., Type I, II, III, IV, V, VI, VII, IX, X, or XI). This code is appropriate for atypical presentations or newly identified forms of glycogen storage disease where the specific enzyme defect or clinical subtype is not yet classified elsewhere.

• Always strive for the most specific glycogen storage disease code available if the type is documented.
• Review physician documentation carefully for specific enzyme deficiencies or named subtypes of GSD to ensure accurate coding.
• Consider sequencing this code after any acute manifestations or complications directly related to the glycogen storage disease.