Respiratory Failure of Williams-Campbell Syndrome is Effectively Treated by Noninvasive Positive Pressure Ventilation

Hiroshi Wada; Ruriko Seto; Hideto Yamada; Taishi Nagao; Takashi Hajiro; Yasutaka Nakano

doi:10.2169/internalmedicine.50.4971

CASE REPORTS

Respiratory Failure of Williams-Campbell Syndrome is Effectively Treated by Noninvasive Positive Pressure Ventilation

Hiroshi Wada, Ruriko Seto, Hideto Yamada, Taishi Nagao, Takashi Hajiro, Yasutaka Nakano

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Keywords: noninvasive positive pressure ventilation (NPPV), Williams-Campbell syndrome, bronchiectasis

JOURNAL OPEN ACCESS

2011 Volume 50 Issue 16 Pages 1729-1732

DOI https://doi.org/10.2169/internalmedicine.50.4971

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Abstract

Williams-Campbell syndrome is a rare disease, characterized by a congenital deficiency of cartilage in the fourth to sixth order bronchi, leading to chronic respiratory failure with recurrent pulmonary infections. An effective and practical treatment has not yet been established. A 31-year-old man who was diagnosed as Williams-Campbell syndrome by inspiratory and expiratory computed tomography findings developed recurrent pulmonary infections and showed progressive deterioration of dyspnea. Domiciliary NPPV was administered, followed by a dramatic improvement of respiratory failure and a decrease in the episodes of pulmonary infections. NPPV may have an advantage in adults with Williams-Campbell syndrome who have severe respiratory failure and recurrent pulmonary infections.

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