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Background <p>Madelung disease (multiple symmetric lipomatosis) is a rare metabolic disorder characterized by progressive accumulation of non-encapsulated lipomatous tissue in the cervical and parapharyngeal regions, which can critically distort upper airway anatomy and substantially increase the risk of failed intubation. When a difficult airway is anticipated, awake tracheal intubation (ATI) is recommended. Videolaryngoscopy (VL) is increasingly recognized as an evidence-based alternative to fiberoptic bronchoscopy (FOB) in selected patients with supraglottic soft-tissue crowding; however, its use in combination with pharmacologic sedation in patients with concurrent hepatic dysfunction has not been previously described.</p> Case presentation <p>A 70-year-old man with Madelung disease and compensated liver cirrhosis (Child-Pugh class A) was scheduled for posterior lumbar decompression and fusion at the L4–5 level in the prone position. Preoperative flexible nasopharyngolaryngoscopy confirmed significant supraglottic crowding with partial obscuration of the epiglottic inlet caused by perilaryngeal lipomatous infiltration, without intrinsic glottic pathology. In the operating room, multimodal topical anesthesia with 10% lidocaine spray was combined with cooperative sedation using dexmedetomidine (loading dose 1.0&#xa0;µg/kg over 10&#xa0;min, maintenance infusion 0.5&#xa0;µg/kg/h) and remifentanil (0.05&#xa0;µg/kg/min), titrated to Patient State Index (PSI) values of 70–85 with preserved spontaneous ventilation. Orotracheal intubation with a McGrath™ MAC videolaryngoscope (size 3 blade) was achieved on the first attempt without hypoxemia, coughing, or hemodynamic instability. General anesthesia, the intraoperative course, and extubation were completed without airway-related complications. The patient was discharged on postoperative day 16.</p> Conclusions <p>This case demonstrates that awake VL combined with dexmedetomidine–remifentanil sedation and topical anesthesia is a safe and effective approach in Madelung disease when airway compromise is caused by supraglottic soft-tissue crowding. Remifentanil is the preferred opioid adjunct in patients with hepatic dysfunction owing to its hepatic-independent elimination.</p>

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Dexmedetomidine–remifentanil-assisted awake videolaryngoscopic intubation in a patient with Madelung disease and compensated liver cirrhosis undergoing prone lumbar spine surgery: a case report

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  • 发表日期：

    2026年5月26日

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  • Jin-Wook Park,
  • Jung Hee Choi

摘要

Background

Madelung disease (multiple symmetric lipomatosis) is a rare metabolic disorder characterized by progressive accumulation of non-encapsulated lipomatous tissue in the cervical and parapharyngeal regions, which can critically distort upper airway anatomy and substantially increase the risk of failed intubation. When a difficult airway is anticipated, awake tracheal intubation (ATI) is recommended. Videolaryngoscopy (VL) is increasingly recognized as an evidence-based alternative to fiberoptic bronchoscopy (FOB) in selected patients with supraglottic soft-tissue crowding; however, its use in combination with pharmacologic sedation in patients with concurrent hepatic dysfunction has not been previously described.

Case presentation

A 70-year-old man with Madelung disease and compensated liver cirrhosis (Child-Pugh class A) was scheduled for posterior lumbar decompression and fusion at the L4–5 level in the prone position. Preoperative flexible nasopharyngolaryngoscopy confirmed significant supraglottic crowding with partial obscuration of the epiglottic inlet caused by perilaryngeal lipomatous infiltration, without intrinsic glottic pathology. In the operating room, multimodal topical anesthesia with 10% lidocaine spray was combined with cooperative sedation using dexmedetomidine (loading dose 1.0 µg/kg over 10 min, maintenance infusion 0.5 µg/kg/h) and remifentanil (0.05 µg/kg/min), titrated to Patient State Index (PSI) values of 70–85 with preserved spontaneous ventilation. Orotracheal intubation with a McGrath™ MAC videolaryngoscope (size 3 blade) was achieved on the first attempt without hypoxemia, coughing, or hemodynamic instability. General anesthesia, the intraoperative course, and extubation were completed without airway-related complications. The patient was discharged on postoperative day 16.

Conclusions

This case demonstrates that awake VL combined with dexmedetomidine–remifentanil sedation and topical anesthesia is a safe and effective approach in Madelung disease when airway compromise is caused by supraglottic soft-tissue crowding. Remifentanil is the preferred opioid adjunct in patients with hepatic dysfunction owing to its hepatic-independent elimination.