Your lungs > Children's Lung Diseases > Congenital lung abnormalities
Congenital lung abnormalities
- Congenital lobar emphysema; this is an over-expansion of one section (lobe) of the lung. It is due to a ball-valve type of obstruction blocking off the airway leading to that lobe. If small, there may be no symptoms at all but obstructions of larger airways may result in the over-distended lobe squashing surrounding normal lung. Breathing difficulties may then occur in the newborn
- Bronchogenic cysts; these are due to abnormal development of the airways. Since larger cysts may squash surrounding normal lung, surgical removal is generally recommended.
- Other lung cysts may be single or multiple (e.g. congenital cystic adenomatous malformations). Large cysts may present with breathing difficulties at birth. Otherwise there may be repeated chest infections or infections which are slow to clear. The decision to remove them surgically depends on their size and the symptoms which they produce
- Pulmonary Sequestration is a rare congenital lung abnormality when a lung lobe receives its blood supply from an unusual blood vessel. Since the airways of this sequestered lobe do not connect with the rest of the lung airways, sputum collects there; the result is frequent chest infections. Pulmonary Sequestration may be associated with other congenital lung abnormalities. Again, surgical removal of that lobe is usually recommended
- Lung hypoplasia (reduced lung development); very rarely a whole lung fails to develop during pregnancy (pulmonary aplasia or pulmonary agenesis). Depending on the severity, lung hypoplasia may present with breathing difficulties at or soon after birth with repeated chest infections. However, some affected children have surprisingly few problems. Pulmonary hypoplasia may develop secondary to a diaphragmatic hernia, cystic adenomatous malformations (see later for explanations) or other abnormalities.
- Congenital Diaphragmatic hernia; the diaphragm is the main breathing muscle. It separates the abdomen from the lungs. Congenital diaphragmatic hernia is a serious birth defect resulting in an abnormal hole through the diaphragm. It allows abdominal organs to move into the chest cavity, squashing the lung and contributing to its poor development (lung hypoplasia). The hernia is often on the left side and is seen in about 1 in 5000 live births. The cause is unknown. It can present with severe breathing difficulties at birth.
- Congenital causes of upper airway obstruction; a number of rare birth defects cause obstruction of the upper breathing tubes in the first few weeks of life. These can result in breathlessness, stridor (see definitions), snoring, obstructive sleep apnoea (see below for explanation), day-time sleepiness, feeding and swallowing problems and failure to thrive. Conditions include:
- Laryngomalacia; this is an abnormal softening of the tissues of the voice box. It accounts for 90-95% cases of upper airways obstruction. Fortunately, it often improves with time.
- Choanal atresia and stenosis; a congenital disorder in which the back of the nasal passage (choana) is blocked, usually by abnormal bony or soft tissue formed during the unborn baby’s development. Sometimes other abnormalities are associated, such as coloboma (malformation of part of the eye), retarded development, abnormalities of the genitals in males, heart defects and ear cartilage deformities (known as CHARGE)
- Nasal mass obstructions (cysts or solid masses)
- Pierre-Robin syndrome (abnormally small jaw and cleft palate)
- Other syndromes with abnormalities of head and face include Crouzon, Apert, Treacher-Collins and Goldenhar syndromes
- Vocal cord paralysis: The vocal cords are folds of tissue located in the voice box that vibrate when air passes over them to produce the sound waves associated with talking and singing. If one of them is paralysed, the upper airways may obstruct.
