Background:
Congenital lung disorders encompass a spectrum of several conditions, one of which is lung hypoplasia. While hypoplasia is typically identified and intervened upon in the neonatal period, some patients can remain asymptomatic or even be diagnosed as adults. Given the rarity of the condition persisting in adults though, it is not clear what medical or surgical interventions may be helpful if respiratory status declines.
Case description:
In this report, we describe an elderly man with a history of right lung hypoplasia, pulmonary artery agenesis, and bronchial atresia who developed progressive dyspnea and worsening cough. His condition was complicated by multiple Aspergillus infections for which he received prolonged courses of anti-fungal therapy. He was also treated for bacterial pneumonia many times over a 10-year period. However, as his symptoms remained refractory to medical management, he underwent pneumonectomy, which revealed diffuse cystic changes in the right lung. He is currently doing well post-operatively with resolution of his dyspnea.
Conclusions:
Although hypoplastic lung disorders have been described in asymptomatic adults, this report highlights the successful utilization of pneumonectomy in an individual with refractory symptoms much later in adulthood. This case additionally describes possible complications of cystic lung disease in this patient population, serving as further rationale for aggressive intervention.