In 2011, Hacken et al. conducted a systematic review, aiming to answer what are the effects of treatments in people with bronchiectasis but without cystic fibrosis. There are not enough studies to prove if bronchopulmonary hygiene, physical therapy, mucolytics, inhaled hyperosmolar agents, oral corticosteroids, leukotriene receptor antagonists, short-acting beta 2 agonists, long-acting beta 2 agonists, or anticholinergic therapy is beneficial. However, there are some promising results in other approaches. Lung transplantation This was previously attempted in a patient with end-stage lung disease secondary to WCS. Although the patient did not have proximal airway collapse prior to transplantation, his posttransplant course was complicated by the development of bronchomalacia of the right and left mainstem bronchi. The patient experienced recurrent pulmonary infections and died of bacterial pneumonia one year after transplantation. In 2012, the first WCS patient lung transplant with prolonged survival (approaching 10 years) was reported. Lobectomy Surgery to remove part of the lungs is often considered for people with extreme damage to one or two lobes of the lung who are at risk of severe infection or bleeding. However, surgery in two patients, one given a triple lobectomy and the other a right upper lobectomy, resulted in severe respiratory failure. Medicine WCS is an obstructive disorder that shares some similarities with chronic obstructive pulmonary disorder (COPD). Non-invasive positive pressure ventilation (NPPV) has been reported to improve chronic respiratory failure in patients with bronchiectasis. NPPV combined with long-term home oxygen therapy decreases carbon dioxide retention and improves dyspnea in hypercapnic COPD. Moreover, long-term NPPV may decrease acute exacerbation and recurrent hospitalization. There are case reports that NPPV may have an advantage in adult patients with WCS who have severe respiratory failure and recurrent pulmonary infections.
Williams–Campbell syndrome
Keppen–Lubinsky syndrome
Avellis syndrome
DeSanto-Shinawi syndrome
Hecht Scott syndrome
Williams syndrome
Pelger–Huët anomaly
Shprintzen–Goldberg syndrome