However, theories have been raised regarding the vascular, iatrogenic, viral and genetic causes of pulmonary agenesis in an attempt to explain the pathogenesis of the disorder.
[5] In most cases of pulmonary agenesis, surgical resection is performed to remove the malformed lobe or the entire defected lung of the patient depending on the severity of the respiratory impairment.
[7] The estimated prevalence of pulmonary agenesis is 34/1,000,000 live births, with a slightly higher possibility in the female population.
[9] The severity of unilateral pulmonary agenesis varies depending on the area of tissue affected, being either a single lobe or a whole lung.
[4] Frequently seen clinical features includes dyspnea, respiratory distress, recurrent pulmonary infections, and limited exercise tolerance.
[4] The retention of bronchial secretions often leads to recurrent pulmonary infections, adding to damage in lung function, hence causing respiratory stress.
[11] Although the exact cause of the disorder remains obscure, theories have been advanced throughout history to explain the pathogenesis of lung agenesis.
[16] Based on an in vitro experiment done in rats, researchers observed lung aplasia in animals that were fed with a diet deficient in vitamin A.
[2] Prenatal diagnosis of pulmonary agenesis is yet to be reached satisfaction, due to the technical difficulties in differentiating this disorder with other malformation.
[2] Congenital diaphragmatic hernia (CDH), in this case, the upward displacement of the diaphragm and abdominal organs, is a possible clinical outcome detectable before birth.
[18] Plombage, also known as extraperiosteal or extrapleural pneumonolysis, is a historical treatment procedure for cavity tuberculosis of upper lobes of lungs used between the 1930s and 1950s.
[13] Recent approach involves the implantation of tissue expander either via open thoracotomy or thoracoscopically in an attempt to shift the mediastinum back to its anatomical location.
[13] If the defect is extensive but there is a chance for the fetus to live, an exo-utero intrapartum treatment (EXIT) may be performed to salvage the potential life.
[18] EXIT technique involves partial delivery of a baby through an incision in the uterus while remaining attached to their mother's placenta.
Most causes of death are because of the presence of associated anomalies and malformations, which are common for pulmonary agenesis especially involving right-sided defects.
[18] In fact, it has been suggested that right-sided defects produce a more severe mediastinal shift, distorting the trachea and great vessels.