LA75.4

ICD-11 MMS 2026-01

Congenital pulmonary airway malformations

Description

A disease caused by failure of the bronchial structure to correctly develop during the antenatal period. This disease may present with severe respiratory distress in the newborn period, acute respiratory distress or infection later in life, or may be asymptomatic. This disease can be distinguished from other lesions and normal lung by polypoid projections of the mucosa, an increase in smooth muscle and elastic tissue within the cyst walls, an absence of cartilage in the cystic parenchyma, mucous secreting cells, and the absence of inflammation.

Index terms

  • Congenital pulmonary airway malformations
  • CCAM - [Congenital cystic adenomatoid malformation]
  • Congenital cystic adenomatoid malformation
Show allHide
  • Congenital cystic disease of lung
  • Congenital cystic lung
  • Congenital honeycomb lung
  • Congenital lung cyst
  • Congenital polycystic disease of lung
  • Congenital polycystic lung
  • Multiple congenital bronchogenic cysts
  • Congenital pulmonary airway malformation type 0
  • CPAM type 0 - [Congenital pulmonary airway malformation type 0]
  • Congenital pulmonary airway malformation type 1
  • CPAM type 1 - [Congenital pulmonary airway malformation type 1]
  • Congenital pulmonary airway malformation type 2
  • CPAM type 2 - [Congenital pulmonary airway malformation type 2]
  • Congenital pulmonary airway malformation type 3
  • CPAM type 3 - [Congenital pulmonary airway malformation type 3]
  • Congenital pulmonary airway malformation type 4
  • CPAM type 4 - [Congenital pulmonary airway malformation type 4]

Inclusions

  • Congenital honeycomb lung
  • Congenital polycystic disease of lung

Excludes

  • CB40.Y – Cystic lung disease, acquired or unspecified

Exclusions from above levels

  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Sibling codes

  • LA75.0 – Accessory lobe of lung
  • LA75.1 – Agenesis of lung
  • LA75.2 – Congenital hypoplasia of lung
  • LA75.3 – Congenital hyperplasia of lung
  • LA75.5 – Congenital lobar emphysema
  • LA75.6 – Congenital sequestration of lung
  • LA75.Y – Other specified structural developmental anomalies of lungs
  • LA75.Z – Structural developmental anomalies of lungs, unspecified

Foundation entity

Congenital pulmonary airway malformations – http://id.who.int/icd/entity/2091138945

Allowed postcoordination axes

Part of: LA75: Structural developmental anomalies of lungs › 20: Developmental anomalies