Presentation: A 97-year-old man presented with one week of worsening dysphagia, odynophagia and a mild hoarseness. The patient was able to tolerate a normal diet. Flexible fiberoptic laryngoscopy revealed a small ulcer of the right aryepiglottic fold, with normal vocal fold movement. Over the next two days, his dysphagia worsened, and he was no longer able to tolerate liquids.
Esophagogastroduodenoscopy was performed, and the only abnormality was a Schatzski ring at the gastroesophageal junction. A modified barium swallow study showed aspiration and hypopharyngeal pooling of contrast. Contrast enhanced computed tomography of the neck did not reveal any abnormality. The following day, a flexible fiberoptic laryngoscopy revealed ulcerations of the right hemilarynx, pooling of secretions and a complete right vocal fold paralysis (Figure 1). The left hemilarynx was unaffected.
—John M. Carter, MD, David Cai, MD and Brian A. Moore, MD, Tulane University School of Medicine and Ochsner Clinic Foundation, New Orleans.
What is your diagnosis, and how would you manage this patient? Go to the next page for the answer.
Laboratory testing was significant for varicella zoster IgG antibody titers that were five times normal limits, 5.06 (NL = 0.0–0.9). Varicella IgM antibody testing was negative. The patient was started on intravenous valacyclovir and prednisone. The following day, his dysphagia began to improve. Laryngoscopy revealed a reduction in the quality of the erythema and ulcerations. The patient had a complete resolution of the vesicles following two weeks of treatment. Right vocal fold movement was improved but did not return to normal.
Varicella zoster is a common systemic disease that rarely affects the larynx.1-4 The virus is known to establish a latent infection in the spinal cord or cranial nerve ganglia that reactivates secondary to various stressors. Ramsay Hunt syndrome type II is an infection involving the ear that results from a reactivation of the varicella virus located in the geniculate ganglion. Patients suffering from herpes zoster present with vesicular eruptions, acute pain and nerve paralysis in the distribution of the affected nerves. Herpes zoster of the larynx is uncommon and often presents with multiple cranial neuropathies.1-3 Furthermore, its vesicular appearance may be confused with a laryngeal neoplasm.
Herpes zoster of the larynx may present in a variety of ways. The patient may have one or more cranial nerve palsies, painful mucosal vesicles or pain without visible herpetic lesions. Cranial nerves IX, X and XI are most often involved but CN VII and VIII may be affected in more extensive cases.1 Patients may or may not have concurrent skin involvement.
Mucosal lesions of zoster of the head and neck are not typically limited to one nerve distribution and do not necessarily stem from one ganglion.1,2 In our case, disease was confined to the distribution of the superior and recurrent branches of cranial nerve X. Lin and colleagues described the only 11 previously reported cases of varicella zoster infection that presented as cranial nerve palsies with mucosal lesions that lacked skin manifestations or evidence of herpes zoster oticus. Four of these cases involved only the hemilarynx and cranial nerve X, as in our presentation.1
The diagnosis of this disease process is largely clinical and may be supplemented by viral antibody assays and polymerase chain reaction (PCR).1,3,5,6 Treatment with anti-viral medications and steroids lacks convincing evidence but may be used to hasten recovery.
- Lin YY, Kao CH, Wang CH. Varicella zoster virus infection of the pharynx and larynx with multiple cranial neuropathies. Laryngoscope. 2011;121:1627-1630.
- Pinto JA, Pinto HC, Ramalho Jda R. Laryngeal herpes: a case report. J Voice. 2002;16:560-563.
- Chitose SI, Umeno H, Hamakawa S, Nakashima T, Shoji H. Unilateral associated laryngeal paralysis due to varicella-zoster virus: virus antibody testing and videofluoroscopic findings. J Laryngol Otol. 2008;122:170-176.
- Watelet JB, Evrard AS, Lawson G, et al. Herpes zoster laryngitis: case report and serological profile. Eur Arch Otorhinolaryngol. 2007;264:505-507.
- Beards G, Graham C, Pillay D. Investigation of vesicular rashes for HSV and VZV by PCR. J Med Virol. 1998;54:155-157.
- Van Loon AM, van der Logt JT, Heessen FW, Heeren MC, Zoll J. Antibody-capture enzyme-linked immunosorbent assays that use enzyme-labelled antigen for detection of virus-specific immunoglobulin M, A and G in patients with varicella or herpes zoster. Epidemiol Infect. 1992;108:165-174.