Congenital cirrhosis (of liver)

This code signifies a rare, chronic liver disease present at birth or manifesting shortly thereafter, characterized by irreversible scarring and impaired liver function. It represents a primary congenital etiology of cirrhosis, distinct from acquired forms.

Assign this code when documentation explicitly states "congenital cirrhosis" or indicates cirrhosis diagnosed in infancy or early childhood with a confirmed congenital origin. Supporting documentation would include genetic testing results, imaging studies, and liver biopsy findings confirming the congenital nature.

• Do not use for cirrhosis acquired later in life, even if the underlying cause is congenital (e.g., alpha-1 antitrypsin deficiency leading to cirrhosis).
• Always code the underlying genetic or metabolic disorder, if known, as a primary diagnosis when applicable, with this code as a secondary diagnosis.
• Distinguish from other forms of neonatal liver disease or cholestasis that may not have progressed to cirrhosis.