Congenital Pulmonary Airway Malformation with Pulmonary Arteriovenous Malformation in Adulthood: A Case Report.
Background: Congenital pulmonary airway malformation (CPAM) is a congenital condition rarely detected in adults because most cases of CPAM are found through prenatal testing or through testing for recurrent pneumonia or lung abscesses in childhood. Pulmonary arteriovenous malformation (PAVM) is an abnormal vascular connection between the pulmonary arteries and veins, which is often related to hereditary hemorrhagic telangiectasia, but can also be induced by infections, trauma, or thoracic surgery. Herein, we report an adult case of coexisting CPAM and PAVM.
Methods: The patient was a 26-year-old woman. A medical checkup chest X-ray showed abnormalities. The patient had no past medical history, including of bleeding tendency or repeated pneumonia, and no familial history of CPAM and PAVM. A chest CT revealed multiple lung cysts (maximum diameter of 40 mm) in the left lower lobe of the lung, and congenital pulmonary cysts were suspected. The chest CT also showed two PAVMs (vessel diameters of 6 mm and 4 mm) in the same left lower lobe. Serum tests were positive for Aspergillus-specific antibodies and β-D-glucan, and pulmonary aspergillosis was diagnosed. An antifungal agent (itraconazole) was administered. However, consolidations had developed 9 months after, and the antifungal agent was changed to voriconazole. Then, the consolidations diminished slightly but nevertheless remained, and one of the PAVMs increased in diameter from 6 mm to 10 mm. A left lower lobectomy under thoracoscopy was performed owing to the uncontrolled infection and the risk of complications with PAVMs. The pathological diagnosis of the pulmonary cysts was CPAM type 1. The patient had no symptoms or complications after the surgery.
Conclusions: Cases of CPAM with PAVM are rare, especially in adults. CPAM often leads to pulmonary infection, and the pulmonary infection is known to be one of the causes of PAVM. In our case, Aspergillus might have infected the pulmonary cysts and affected the enlargement of the vascular diameter of PAVM. If CPAM and PAVM are present simultaneously, surgical treatment should be considered to prevent complications associated with CPAM and PAVM.