Q42.2

Billable

Congenital absence, atresia and stenosis of anus with fistula

HCC Category Mapping

V28HCC 78 — Intestinal Obstruction/Perforation

0.000

What This Code Means

A birth defect where the anus is completely absent or abnormally closed, with an abnormal opening (fistula) connecting to another structure, preventing normal bowel elimination.

Coding Tips

•Document the specific location of the fistula (perineal, urethral, vaginal, etc.) as this impacts surgical planning.
•This condition is often identified at birth; ensure documentation reflects the fistula location for complete coding.

Clinical Significance

Congenital anal atresia with fistula is a complex anorectal malformation requiring careful surgical planning to achieve both anatomic reconstruction and functional continence. The fistula location significantly influences surgical approach and long-term outcomes.

Documentation Requirements

✓Anatomic description of anal atresia and fistula type
✓Fistula location (perineal, vestibular, rectourethral, rectovesical)
✓Associated anomalies evaluation (VACTERL sequence)
✓Surgical reconstruction plan and staging
✓Colostomy details and management
✓Post-operative continence assessment protocols
✓Dietary and bowel management programs
✓Long-term multidisciplinary follow-up plans

Commonly Confused Codes

Q42.0 — Congenital rectal atresia with fistula (rectal vs anal level)Q42.1 — Congenital rectal atresia without fistula (rectal level, no fistula)Q42.3 — Congenital anal atresia without fistula (no fistula component)Q52.0 — Congenital absence of vagina (different anatomic structure)

Code Hierarchy

└Q42Congenital absence, atresia and stenosis of large intestine └Q42.2Congenital absence, atresia and stenosis of anus with fistula

└Q42.2Congenital absence, atresia and stenosis of anus with fistula