AHNS Abstract: B156

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Program Number: B156
Session Name: Poster Session

Breaking the Mold: Treating Laryngeal Mucormycosis While Sparing the Larynx

Salim C Lutfallah, BS; Christian Lemoine, MD; Vilija Vaitaitis, MD; Louisiana State University Health Sciences Center Department of Otolaryngology, New Orleans, LA

Introduction: We present a case of isolated laryngeal mucormycosis, successfully managed with tracheostomy, and systemic and inhaled antifungal therapy. To our knowledge, this is the second reported case of isolated laryngeal mucormycosis and the only case in which the larynx was spared. Mucormycosis is a rare and life-threatening fungal infection, caused by Mucorales species, that primarily affects immunosuppressed and diabetic individuals and typically involves the nasal cavity and paranasal sinuses. Effective management requires early diagnosis, aggressive antifungal therapy, surgical debridement, and control of underlying risk factors. The aggressive nature of this infection can lead to rapid progression and significant mortality if not treated early. 

Case Presentation: A 36-year-old male with uncontrolled type I diabetes mellitus and a recent hospitalization for diabetic ketoacidosis presented with hoarseness, odynophagia, and shortness of breath. He had leukocytosis, and neck imaging showed subcutaneous and laryngeal emphysema (Image 1). Flexible laryngoscopy revealed right vocal fold paralysis and areas of leukoplakia throughout the oropharynx and larynx (Image 2). He was admitted to the intensive care unit and started on broad-spectrum antibiotics and fluconazole with tight glycemic control. His respiratory status worsened, requiring emergent intubation. He was taken for a direct laryngoscopy (DL), which showed purulence in the larynx and granulation tissue in the right subglottis with healing of the perforation (Image 3). Pathology was initially read as necrotic tissue but was later addended to report fungal elements (Image 4). Liposomal amphotericin B (LAMB) was started, and he was taken for a repeat DL, which showed worsening areas of necrosis (Image 5). These areas were debrided, biopsies were obtained, and a tracheostomy was performed. Biopsy results showed angioinvasion and confirmed invasive fungal disease (Image 6). He subsequently underwent multiple endoscopic debridements to remove necrotic tissue and ultimately cleared the disease without sacrificing major anatomical structures. During his recovery, he developed complete subglottic stenosis, which was addressed with tracheal dilation (Image 7). Posaconazole was initiated long-term, and he was discharged home. He has since undergone further dilations for subglottic stenosis and remains tracheostomy-dependent. 

Discussion: Although laryngeal mucormycosis is rare, its aggressive nature requires prompt, multimodal treatment. Antifungal therapy with LAMB is the first-line treatment, and long-term maintenance with posaconazole is critical to prevent recurrence. Monitoring antifungal drug levels, especially of posaconazole, is essential due to its variable absorption and potential for drug interactions, which can lead to subtherapeutic levels and breakthrough infections.

Conclusion: Laryngeal mucormycosis is an uncommon infection that requires prompt and aggressive management due to its severity. Antifungal therapy with LAMB and long-term posaconazole, combined with surgical debridement, forms the foundation of treatment.

Image 1: Computed tomography of the neck showing air surrounding the inferior larynx

Image 2: Flexible fiberoptic laryngoscopy

Image 3: (A) glottic and (B) subglottic view from initial DL

Image 4: Fungal elements seen on biopsy from initial DL

Image 5: Worsening subglottic necrosis noted on repeat DL in (A) glottis and (B) subglottis

Image 6: Fungal elements with angioinvasion

Image 7: Repeat DL with (A) resolution of necrosis and (B) complete subglottic stenosis

 

 

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