Hereditary lymphedema

This code signifies a chronic, inherited condition characterized by abnormal swelling, typically in the limbs, due to impaired lymphatic drainage. It results from congenital malformations or dysfunction of the lymphatic system, leading to the accumulation of protein-rich fluid in the interstitial tissue.

Assign this code when documentation confirms a diagnosis of lymphedema that is explicitly stated as hereditary or congenital in origin. This includes conditions like Milroy's disease or Meige's disease. Supporting documentation should clearly indicate the genetic or familial nature of the lymphedema.

• Differentiate from secondary lymphedema (I89.0), which is acquired due to trauma, surgery, infection, or malignancy.
• Do not use for unspecified lymphedema; seek clarification if the etiology is not documented as hereditary.
• Code any associated complications, such as cellulitis or lymphangitis, in addition to this code.