Congenital absence, atresia and stenosis of anus w/o fistula

This code signifies a birth defect characterized by the complete or partial absence, or abnormal narrowing, of the anal opening. Importantly, this condition occurs without the presence of an abnormal connection (fistula) between the rectum and another organ or the skin. It is a form of anorectal malformation.

Assign this code for congenital imperforate anus or anal stenosis where surgical correction is indicated and no fistula is documented. Documentation should clearly state the absence of a fistula, often noted during physical examination, imaging studies, or surgical exploration.

• Differentiate carefully from other anorectal malformations that do involve a fistula, as these are coded elsewhere.
• Ensure documentation explicitly states "without fistula" to support this specific code.
• Consider sequencing this code as the primary diagnosis when it is the reason for the encounter, especially for initial surgical repair.