Lung agenesis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Lung agenesis is defined as partial or complete failure of the lung to form with absence of the lung parenchyma, the bronchus and the pulmonary vessels.
Classification
The condition can be unilateral (compatible with life) or bilateral (fatal).
Pathophysiology
Embryogenesis
The trachea begins to develop at about 4 weeks of gestation as an outpouching from the foregut. Lung agenesis develops as a result of the bronchial analogue to develop into tow lung buds.
On the affected side, the pleural cavity is absent, and the healthy lung occupies the entire thorax. If a left lung develops, it is often trilobed.
Symptoms are due to due to tracheal compression and kinking of the trachea, shift of the mediastinal contents, posterior dislocation of the trachea as a result of a dislocated aortic arch and somtimes an associated truncus arteriosus.
Associated Conditions
- Truncus arteriosus [1][2][3]
- Dextrocardia with situs solitus may be present among patients with agenesis or hypoplasia of the right lung [4]
Epidemiology and Demographics
Lung agenesis is extremely rare and is observed in 1:15,000 autopsies.
Gender
There is a slight female preponderance.
Natural History, Complications, Prognosis
Natural History
The natural history is variable.
Complications
There can be kinking of the trachea and compression of the trachea by the aorta.
Prognosis
Although 50% of patients may die before the age of 5, the remainder of the individuals may be asymptomatic throughout their lives. Bilateral lung agenesis is a lethal anomaly.
Diagnosis
Symptoms
Treatment
Symptomatic Patients
Symptoms are due to due to tracheal compression and kinking of the trachea, shift of the mediastinal contents, posterior dislocation of the trachea as a result of a dislocated aortic arch and somtimes an associated truncus arteriosus.
Indications for surgery include:
The surgical procedure varies and includes aortopexy and diaphragmatic translocation which improve mediastinal shift and reduce heart rotation. Relief of tracheal compression is critical as is reduction in hyperinflation of the remaining lung.
Asymptomatic Patients
Prophylactic surgery is not necessary.
References
- ↑ Krivchenya DU, Dubrovin AG, Krivchenya TD, Khursin VN, Lysak CV (2000). "Aplasia of the right lung in a 4-year-old child: surgical stabilization of the mediastinum by diaphragm translocation leading to complete recovery from respiratory distress syndrome". Journal of Pediatric Surgery. 35 (10): 1499–502. doi:10.1053/jpsu.2000.16424. PMID 11051161. Retrieved 2012-09-16. Unknown parameter
|month=ignored (help) - ↑ Skandalakis JE, Gray SW, Symbas P: The trachea and the lungs. In: Skandalakis JE, Gray SW, editors. Embryology for Surgeons. 2nd ed. Baltimore, MD: Williams and Wilkins; 1994. p 429-32.
- ↑ Dohlemann C, Mantel K, Schneider K, Guntner M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: Airway obstruction and its release by aortopexy. J Pediatr Surg 1990;25:290-3.
- ↑ Dohlemann C, Mantel K, Schneider K, Guntner M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: Airway obstruction and its release by aortopexy. J Pediatr Surg 1990;25:290-3.