A 3-year-old girl is evaluated for hoarseness that has been getting worse over the past 2 months. The patient has had no fever, shortness of breath, or change in activity level. She is eating normally, gaining weight well, and does not seem to be in pain. Vital signs are within normal limits. On physical examination, the ears are normal appearing with no middle ear fluid. The nasal mucosa is clear with no rhinorrhea, and the turbinates are normal in size. There are no oral mucosal lesions. The tonsils are small and nonobstructive. There is no cervical lymphadenopathy. Aside from hoarseness, examination of cranial nerves is normal. Flexible laryngoscopy shows several finger-shaped lesions on both vocal cords. Which of the following is the most likely cause of this patient's current condition?

Question

A 3-year-old girl is evaluated for hoarseness that has been getting worse over the past 2 months.  The patient has had no fever, shortness of breath, or change in activity level.  She is eating normally, gaining weight well, and does not seem to be in pain.  Vital signs are within normal limits.  On physical examination, the ears are normal appearing with no middle ear fluid.  The nasal mucosa is clear with no rhinorrhea, and the turbinates are normal in size.  There are no oral mucosal lesions.  The tonsils are small and nonobstructive.  There is no cervical lymphadenopathy.  Aside from hoarseness, examination of cranial nerves is normal.  Flexible laryngoscopy shows several finger-shaped lesions on both vocal cords.  Which of the following is the most likely cause of this patient's current condition?

Quizplus · Accepted Answer

11ec25c8_d8c4_ddd0_bc81_755b896516d7_MD0003_00 Constant or progressive hoarseness is often related to a vocal cord lesion and should be evaluated by laryngoscopy. This patient with hoarseness and multiple finger-shaped lesions on her true vocal cords likely has laryngeal papillomas due to recurrent respiratory papillomatosis (RRP). These lesions can be finger-shaped, warty, or grapelike, and as with skin papillomas, have dark-red punctate areas corresponding to blood vessels. Laryngeal papillomas are caused by human papillomavirus (HPV) subtypes 6 and 11, which are also the subtypes most likely to cause genital warts (condyloma acuminatum), and therefore most likely acquired via vertical transmission prior to delivery (because neither vaginal nor cesarean delivery prevent transmission). Although benign, RRP is associated with significant morbidity (eg, voice problems, airway obstruction, repeated operative interventions). In addition, the clinical course is variable and unpredictable with fluctuations in severity. In rare instances, it can spread beyond the vocal cords to involve the lower respiratory tract. Medical therapy (eg, interferon, cidofovir) has limited efficacy; therefore, the mainstay of treatment remains surgical debridement, and patients often require many procedures. The incidence of RRP in children is decreasing, likely due to increased rates of maternal HPV vaccination. (Choice A) Patients with anomalous airway development can present with hoarseness (eg, laryngeal web); however, hoarseness would be present from birth rather than develop in childhood. (Choices B and C) Angioedema can be IgE-mediated (also with itching and urticaria) or inherited (deficiency of C1 esterase inhibitor) and is characterized by recurrent episodes of edema that may involve the larynx and cause hoarseness (although airway obstruction is the primary concern). However, it typically presents as discrete episodes (hours to a few days) rather than progressive hoarseness over weeks or months. (Choice E) Although maternal vaccination is decreasing the incidence of childhood laryngeal papillomas, RRP is caused by a virus (HPV) rather than a bacterium. Educational objective: Vertical transmission of human papillomavirus subtypes 6 and 11 can cause recurrent respiratory papillomatosis, which results in hoarseness due to finger-shaped growths on the true vocal cords. References: Update on recurrent respiratory papillomatosis. (http://www.ncbi.nlm.nih.gov/pubmed/31078306)

A 3-Year-Old Girl Is Evaluated for Hoarseness That Has Been