Giant Cervicothoracic Madelung Disease Presenting with Airway Compromise: A Case Report from Ethiopia

Abstract

INTRODUCTION: To describe a rare (airway-threatening) cervicothoracic presentation of Madelung disease (multiple symmetric lipomatosis) with upper mediastinal extension and to introduce a reproducible peri-operative strategy in a resource-limited setting. CASE PRESENTATION: We report a case of a 40-year-old Oromo Ethiopian male who presented with progressive cervicothoracic adipose masses resulting in dyspnea and venous congestion. Awake fiberoptic intubation, staged resection, early extubation, and close airway management were the hallmarks of peri-operative management. Contrast-enhanced CT showed non-encapsulated, diffuse adipose infiltration from the neck into the upper mediastinum with effacement of the trachea. Because extensive bilateral cervical dissection posed a high risk of postoperative airway compromise (edema/hematoma) and prolonged anesthesia, a pre-planned two-stage surgical strategy was chosen. Stage 1 anterior debulking removed 3.5 kg of fatty tissue, and Stage 2 posterolateral debulking removed an additional 2.0 kg (total 5.5 kg). There were no major complications. The patient was discharged one week after the second procedure. At 1 month follow-up, there was significant improvement in breathing and daily functioning with no evidence of early recurrence. Clinical and ultrasound follow-up at 3, 6, and 9 months and 1 year showed no recurrence or complications. CONCLUSIONS: Giant cervicothoracic Madelung disease can lead to critical airway compromise. Planned awake fiberoptic intubation and staged debulking resulted in safe care and good outcomes. Reporting detailed airway and surgical strategies for this uncommon presentation may be helpful to teams working in resource-constrained settings. Long-term surveillance remains essential because postoperative recurrence has been reported.