Non-invasive ventilation in severe acute respiratory failure complicating Madelung's disease: a case series and management review

Abstract

Madelung's disease (Launois-Bensaude syndrome) presents a critical challenge when managing acute respiratory failure (ARF) due to the high risk of difficult airway and failed intubation posed by symmetric cervical lipomatosis. This case series investigates the feasibility of non-invasive ventilation (NIV) as a primary strategy for ARF in these high-risk patients. We present two cases of severe ARF triggered by COVID-19 pneumonia in patients with Madelung's disease. Both patients were successfully managed with NIV using a total face mask, which avoided the need for hazardous endotracheal intubation. Key challenges included managing bilateral pneumothoraxes as a complication of NIV in one patient, and identifying and treating severe obstructive sleep apnea (OSA), a prevalent comorbidity as a crucial factor for successful weaning in the other. Our findings, integrated with a review of existing literature, demonstrate that a proactive NIV-first approach is a viable and potentially life-saving strategy in this population. This report provides a practical management framework, emphasizing careful interface selection, vigilance for complications, and systematic screening for underlying OSA to guide clinicians in optimizing outcomes for these complex patients.