Associations: Found in 50% of cases, most frequently DWM and cardiac defects, and chromosomal abnormalities, most frequently trisomy 18, are found in 20%. ACC may be part of several syndromes.
Management: In continuing pregnancies, a careful search for associated anomalies and postnatal follow-up is indicated. The delivery should occur in a tertiary center with full capabilities of diagnosis and management.
Prognosis: Depends on associated anomalies and the extent of agenesis; relatively good in isolated ACC, with an 85% chance of a normal developmental outcome and a 15% risk of handicap. Complete ACC has a worse prognosis than partial ACC.
Recurrence risk: Depends on the underlying specific disorder. Most isolated defects are sporadic.