Congenital malformation syndromes involving early overgrowth

This code identifies a group of rare genetic disorders characterized by accelerated and disproportionate growth beginning in infancy or early childhood. These syndromes often involve multiple organ systems and can present with distinct facial features, developmental delays, and an increased risk of certain tumors.

Apply this code when documentation specifies a congenital syndrome primarily defined by early, generalized, or localized overgrowth. Examples include Sotos syndrome, Weaver syndrome, or Beckwith-Wiedemann syndrome, when the overgrowth is the predominant feature and the specific syndrome is not otherwise classified. Documentation should clearly state the diagnosis of an overgrowth syndrome.

• Do not use this code for isolated overgrowth not part of a recognized syndrome.
• Always sequence this code as the primary diagnosis when it is the reason for the encounter.
• Review documentation carefully to ensure the overgrowth is congenital and part of a defined syndrome.