Agenesis of lung

This code represents a rare congenital anomaly characterized by the complete absence of one or both lungs, along with the corresponding bronchus and pulmonary vasculature. It results from a failure of lung bud development during embryogenesis.

Use this code when documentation clearly indicates the congenital absence of a lung. This diagnosis is typically made prenatally via ultrasound or postnatally through imaging studies such as chest X-ray, CT scan, or MRI.

• Differentiate from lung hypoplasia (underdevelopment of the lung) or aplasia (absence of lung tissue but presence of a rudimentary bronchus).
• Always review imaging reports and physician notes for definitive statements regarding the complete absence of the lung.
• Consider sequencing with other congenital anomalies if present, as agenesis of the lung can be associated with other syndromes.