Cloacal exstrophy of urinary bladder

This code describes a severe congenital anomaly where the urinary bladder is everted and exposed, often accompanied by a defect in the abdominal wall, a malformed gastrointestinal tract (e.g., omphalocele, imperforate anus), and genital abnormalities. It represents a complex malformation resulting from a failure of the infraumbilical mesoderm to close during embryonic development.

Use this code for patients diagnosed with cloacal exstrophy of the urinary bladder, a rare and complex birth defect. Documentation should clearly indicate the presence of bladder exstrophy in conjunction with other cloacal malformations, such as omphalocele, imperforate anus, and widely separated pubic bones.

• Ensure documentation specifies "cloacal exstrophy" to differentiate from isolated bladder exstrophy (Q64.11).
• Code all associated congenital anomalies, such as omphalocele (Q79.2), imperforate anus (Q42.3), and genital malformations, in addition to this code.
• This code is typically a primary diagnosis for initial hospitalizations and surgical corrections of this complex defect.