Pulmonary malformation

Classification according to ICD-10
Q33	Congenital malformations of the lungs
Q33.0	Congenital cyst lung
Q33.1	Accessory lung lobe
Q33.2	Pulmonary sequestration (congenital)
Q33.3	Agenesis of the lungs
Q33.4	Congenital bronchiectasis
Q33.5	Ectopic tissue in the lungs (congenital)
Q33.6	Hypoplasia and dysplasia of the lungs
Q33.8	Other congenital malformations of the lungs
Q33.9	Congenital malformation of the lungs, unspecified
ICD-10 online (WHO version 2019)

A pulmonary malformation is a malformation ( malformation ) of the lungs that occurred or was created before birth ( prenatal ) .

Pulmonary malformations can be differentiated as follows:

Missing or underdeveloped lung system

A lack of application or development of lung tissue can occur bilaterally, unilaterally, even in only one flap. Depending on the extent of the missing lung tissue, there are no to severe breathing disorders. Forms that affect only one side or individual parts of the lung result in compensatory overinflation.

• Agenesis with the absence of bronchi , parenchyma and pulmonary vessels
• Aplasia with only recently landscaped Bronchialstamm. With agenesis and aplasia, concomitant malformations of the trachea (hypoplasia), the vertebral bodies and congenital heart defects are to be expected. The x-ray shows a ventral herniation of the healthy lung to the aplastic side, later often scoliosis of the thoracic spine develops .
• Hypoplasia with a small bronchial trunk and partially created lung structure. With this form, pneumothorax is common .

Pulmonary sequester

Details can be found under lung sequester .

Congenital lobar emphysema

A Congenital lobar emphysema consists of a solid hyperinflation of lung lobes by a valve mechanism due to faulty equipment or of the bronchi of the cartilage rings, of lumen-traversing masses or for unknown reasons.

Cystic pulmonary malformation

Cystic lung malformations are to be understood as:

• Bronchogenic cysts

Bronchogenic cysts consist of a chamber lined with ciliated epithelium . Often they are asymptomatic. According to the localization, two forms can be distinguished:

• • Mediastinal bronchogenic cysts , the most common form with around 85%, arise during the early embryonic phase, lie around the carina tracheae and rarely have a connection to the tracheobronchial system
  • Intrapulmonary bronchogenic cysts , less common at 15%, arise later in embryonic development, lie in the lung tissue and communicate so that they are filled with air and / or fluid
• CCAM ( cystic adenomatoid malformation of the lungs )

In the context of syndromes

Lung malformations also occur in the context of syndromes. So when Ellis-Yale-Winter syndrome , a rare autosomal - recessive hereditary disease with a combination of microcephaly , heart defects and lung malformation.

literature

• CONGENITAL LUNG MALFORMATIONS A REVIEW
• M. Nadeem, B. Elnazir, P. Greally: Congenital pulmonary malformation in children. In: Scientifica. Volume 2012, 2012, p. 209896, doi : 10.6064 / 2012/209896 , PMID 24278678 , PMC 3820530 (free full text) (review).
• Congenital Pulmonary Malformations in Pediatric Patients: Review and Update on Etiology, Classification, and Imaging Findings

Individual evidence

1. ↑ W. Schuster, D. Färber (editor): Children's radiology. Imaging diagnostics. Springer 1996, ISBN 3-540-60224-0 , p. 63 ff.
2. ↑ Emphysema, congenital lobaric. In: Orphanet (Rare Disease Database).
3. ↑ Emedicine
4. ↑ Microcephaly - heart defect - lung malformation. In: Orphanet (Rare Disease Database).

This article is about a health issue. It is not used for self-diagnosis and does not replace a diagnosis by a doctor. Please note the information on health issues !