Mpox-associated supraglottitis in an immunocompetent, reportedly sexually abstinent child

Abstract

Mpox is a double-stranded DNA virus of the Orthopoxvirus genus related to smallpox virus endemic to Africa with more than 16,000 cases reported in nonendemic countries in 2022. Classically associated with adult men who have sex with men (MSM), Mpox was once labeled a public health emergency by the World Health Organization as concerning to the general population. Supraglottitis is a rare complication of Mpox that is underreported in the literature and presents a potential airway emergency. Prompt identification is necessary for preventing airway decompensation.

CASE SUMMARY

A 15-year-old African American male with a past medical history of type 1 diabetes mellitus (T1DM) presented with a 5-day history of intermittent fever to 39.4°C (103°F), myalgias, dysphagia, bilateral ear pain, hoarse voice, decreased oral intake, nausea, and diarrhea. He denied neck stiffness, cough, dyspnea, vomiting, abdominal pain, or dysuria. When interrogated alone, the patient denied any sexual activity but reported participating in close-contact activities including cheerleading. He denied recent travel, handling or consuming wild game, or any sick contacts. He was found to be negative for SARS-CoV-2, streptococcal antigen, influenza, and respiratory syncytial virus at an outside facility 2 days prior to presentation. The patient record showed vaccination against Haemophilus influenzae type B. Physical exam was notable for dry mucous membranes with three nontender, nodular white lesions on the right central upper lip and right dorsal tongue (Figure 1). He was Mallampati class 3 with uvular enlargement and mild anterior nontender cervical lymphadenopathy, with the right worse than the left. Leukocyte count and other laboratory studies were normal. Computed tomography of the neck with contrast revealed moderate thickening of the epiglottis and aryepiglottic folds. The otolaryngology service was consulted, and flexible laryngoscopy revealed moderate thickening of the epiglottis (Figure 2). He was admitted to the pediatric intensive care unit for airway monitoring and was started on empiric intravenous clindamycin 600 mg every 6 hours, intravenous ceftriaxone 2 g every 24 hours, and intravenous dexamethasone 6 mg every 6 hours. Polymerase chain reaction tests were negative for methicillin-sensitive Staphylococcus aureus, methicillin-resistant Staphylococcus aureus, herpes simplex virus types 1 and 2 IgG, and COVID-19. Group A Streptococcus rapid antigen detection test and respiratory viral panel were also negative.

Figure 1.

Lesions on (a) upper lip and (b) dorsal tongue.

Figure 2.

Flexible laryngoscopy findings of (a) edematous epiglottis and (b) arytenoid cartilages with patent glottis, (c) right piriform sinus lesion, and (d) posterior pharynx lesions.

Serum studies for mycoplasma IgM and IgG, Haemophilus influenzae type B IgG, cytomegalovirus IgM, and Epstein-Barr virus IgM and IgG were negative. Testing was not sent for Bordetella pertussis, human immunodeficiency virus, Chlamydia trachomatis serovars D-K, or Neisseria gonorrhoeae.

On hospital day 3, he was transferred from the pediatric intensive care unit to the medicine floor and dexamethasone was stopped given clinical improvement. On hospital day 4, he developed multiple 1 to 5 mm tender vesicular perianal lesions and nontender lesions of similar size in all extremities, chest, abdomen, and face (Figure 3). Orthopoxvirus polymerase chain reaction was sent and returned positive. On hospital day 5, he was discharged on oral cephalexin 500 mg every 6 hours and oral clindamycin 600 mg every 8 hours to complete 10 days of antibiotic therapy. On outpatient follow-up visit 2 weeks later, all lesions had crusted (Figure 4). At that time, further patient questioning was not concerning for sexual abuse or other sexual exposure.

Figure 3.

Vesicles appearing during hospital day 4 on (a) right buttock, (b) gluteal fold, (c) left forearm, and (d) right neck.

Figure 4.

Crusted lesions inferior to the (a) left eye, (b) posterior scalp, and (c) left forearm during outpatient follow-up 13 days after vesicular appearance.

CLINICAL QUESTIONS

1. Which of the following modes of transmission has been linked to transmission of Mpox?

   1. Sexual transmission

   2. Handling rodents and/or fomites

   3. Respiratory droplet exposure

   4. All of the above

2. The characteristic mucocutaneous lesion associated with Mpox initially presents in which form?

   1. Macule

   2. Papule

   3. Vesicle

   4. Pustule

DISCUSSION

Mpox commonly presents with a prodromal stage fever, localized lymphadenopathy, and myalgias commonly followed by a spreading, pruritic mucocutaneous lesion originating on the genitals, perianal area, or oral mucosa that progresses to the palms and soles and classically evolves from macule, papule, vesicle, pustule, to scab.^1,2 Supraglottitis is not a common symptom. Supraglottitis is a particularly concerning finding because continued swelling of supraglottic structures can rapidly develop into airway compromise and respiratory arrest. Though the etiology of Mpox-associated supraglottitis is uncertain, it may be similar in origin to the penile edema noted in many cases of Mpox.^1,3 A literature search revealed one case of Mpox-associated epiglottitis, but did not comment whether the patient was a child or an adult.² Others have reported cases of Mpox-associated peritonsillar abscess.^1,4 The Centers for Disease Control and Prevention (CDC) revealed only 16 reported Mpox cases in persons 11 to 15 years of age—11 boys and 5 girls. The most common age group reported to the CDC was 31 to 35 years. Interestingly, CDC data on race and ethnicity shows that a plurality of Mpox cases were among African Americans at 32.27%.⁵

The latest Mpox epidemic has been characterized as primarily transmitting via skin-to-skin contact, commonly among MSM and in those with immunocompromised states. Oropharyngeal manifestations of Mpox are particularly tied to oral sex.^2–4 With our patient’s reported sexual naïveté, less common modes of transmission must be considered such as handling rodents or fomites, ingesting wild game, or what is most likely in our patient, respiratory droplet exposure.¹ Sexual abuse must be considered as a mode of transmission especially in pediatric populations as a cause for supraglottitis. Though not technically listed among the CDC list of immunocompromising conditions, T1DM has been linked to disturbance of chemotaxis and phagocytosis in cells of the innate immunity.⁶ T1DM must thus be considered as a possible contributing factor to this atypical presentation of Mpox.

There are currently no treatment guidelines for Mpox. Supportive therapy and administration of vaccinia immunoglobulin extracted from sera of patients vaccinated against smallpox are considered reasonable. Smallpox vaccination has been shown to have a protective effect against Mpox, though this patient has no record of smallpox vaccination, given it has not been routinely given since the 1970s. Tecovirimat (used in the reported case of Mpox-associated epiglottitis) is a smallpox antiviral recently approved by the US Food and Drug Administration for use against severe cases of Mpox in adults and children weighing ≥13 kg.^1,2 Though our patient received no antivirals, his clinical status improved with 10 days of oral cephalexin and clindamycin, along with intravenous dexamethasone.

In conclusion, given the potentially severe complications of supraglottitis, clinicians should maintain a high index of suspicion for Mpox as an etiology in pediatric patients presenting with fever, localized lymphadenopathy, and multiple, nontender oropharyngeal lesions coupled with respiratory distress.

ANSWERS TO CLINICAL QUESTIONS

Question 1, d. Mpox has been most commonly associated with sexual transmission (typically in the setting of MSM). Less commonly, Mpox has been associated with respiratory droplet exposure. The first reported cases of Mpox exposure in the Western hemisphere were in a group of people who had transported infected prairie dogs that were transported alongside a giant Gambian rat.¹

Question 2, a. Mpox has been described classically as progressing through macular, papular, vesicular, and then pustular phases. Clinically, lesions are most commonly identified during a vesicopustular phase, first either in the perioral or perianal regions before spreading to the palms and soles. Unlike chickenpox, where lesions may be in several phases of maturation simultaneously, Mpox lesions have been noted to progress uniformly.

Disclosure statement/Funding

The authors report no funding or conflicts of interest. The patient gave permission to publish this case report.