Congenital malformations of the respiratory system
Sylva Hotárková
Introduction
The development of the respiratory system, especially of the pulmonary tissue,
is very important for a child to survive in case of premature birth.
These problems are described in the chapter
Patology of the newborn and infancy.
The development of the respiratory system starts in the 4th week of intrauterine
development and continues throughout the whole intrauterine development
and postnatally till the 8th year of life.
The nubmber and size of alveoli increases during late gravidity
and during the childhood.
Congenital malformations of the respiratory tract are rather
rare, the overall incidence is about 1 : 10 000.
Etiology
Etiology of congenital malformations of the respiratory tract
is variable and in most cases unknown.
Congenital malformations of the larynx, trachea, lung
and diaphragm will be described.
Classification
Larynx:
- atresia
- rare fatal condition
- associated with pulmonary hyperplasia
- laryngeal atresia is often a part of the VACTERL
Trachea:
- agenesis
- rare fatal malformation
- classification: 7 types
- tracheo or bronchoesophageal connection in most cases
- in 75% of cases other congenital malformations are present, including
annular pancreas, duodenal atresia or VACTERL
- tracheoesophageal fistulae are described
in the chapter Patology of the GIT
and in detail in the chapter Congenital malformations of the esophagus
(not yet available in English)
The lungs, congenital malformations:
- variability of lobation are common and are not associated with any other pathology
- Agenesis
- unilateral (more common)
- bilateral (rare)
- Pulmonary isomerism
- Condition, where the number of lobar bronchi is the same in both sides.
If 3 bronchi on both sides are present, it is right-sided isomerism, which
is more common and occurrs more often in males.
In left-sided isomerism 2 bronchi are present; this condition is more common in females.
- Isomerism is often associated with congenital heart malformations
- Bronchogenic cysts
- located from the hypopharynx to the diaphragm, within the mediastinum or the lungs
- the cysts are lined by pseudostratified ciliated epithelium, their wall may
contain cartilage, connective tissue, smooth mucle and submucosal glands
- clinical presentation in pulmonary localization:
- respiratory distress syndrome
- lobar emphysema
Pictures
Left sided isomerism; symmetrical bronchial tree, bilobar right lung:
Levoisomerism, symmetric bronchial tree, Macro, autopsy (72146)
Levoisomerism, bilobar right lung, Macro, autopsy (72139)
Lung, four lobes:
Child, lung with four lobes, Macro, autopsy (72543)
Child, lung with four lobes, Macro, autopsy (72544)
Child, lung with four lobes, Macro, autopsy (72545)
