Pediatric Ramsay Hunt Syndrome

Say it ain’t so, Bieber! We all just want you to sing… but I guess that is challenging to do when half of your face does not move. (Ok… full disclosure… I am that Dad that sings songs loudly to embarrass my children… it is my right! But, in exercising this right, I have come to enjoy Bieber’s lyrical verses… oh what an ironic twist of fate.) It is, honestly, kind of creepy to know that a virus is just living in our nerves… waiting… for that most inopportune time to re-emerge and cause distress. We have previously discussed Varicella Zoster Virus (VZV) and Chickenpox as well as Facial Paralysis. Now let us see how the two may be related – Ramsay Hunt Syndrome:

Ramsay Hunt Syndrome: Basics

  • Ramsay Hunt Syndrome is characterized by: [Aydogdu, 2015; Derin, 2014]
    • Auricular Skin Rash
  • Due to reactivation of Varicella Zoster Virus (VZV). [Aydogdu, 2015; Derin, 2014]
    • VZV is latent within the geniculate ganglion.
    • Reactivation affects CN VII and CN VIII.
    • Also known as “Herpes Zoster Oticus.”
  • Ramsay Hunt Syndrome is rare in children. [Aydogdu, 2015; Derin, 2014]
    • Incidence estimated at 2.7/100,000 Children.
    • Develops in 1% of patients with HSV infection.
    • ~10 – 15% of Facial Paralysis is due to Ramsay Hunt Syndrome.
    • However, it is still the 2nd most common cause of Facial Paralysis in children (Bell’s Palsy is #1).
  • Symptoms and Prognosis are worse than those for Bell’s Palsy. [Aydogdu, 2015; Derin, 2014]

Ramsay Hunt Syndrome: Presentation

  • The syndrome is characterized by 1) ear rash, 2) peripheral facial paralysis, and 3) cochleovesicular symptoms… but…
    • Initial presentation can be subtle at first and…
    • Symptoms evolve over time (so initially you may not see all 3 characteristics).
  • Nonspecific findings occur initially: [Aydogdu, 2015]
    • Often starts with a “Viral Prodrome” (like all things in pediatrics).
    • Fever and fatigue.
    • Often lasts for 1-3 days.
  • Skin Rash develops next: [Aydogdu, 2015; Derin, 2014]
    • May start with Ear Pain (think about the pain felt prior to the rash of zoster being seen).
    • Develop erythematous vesicular lesions.
    • May be on the pinna or in the external auditory canal or on the Tympanic Membrane.
    • May ALSO involve the oral mucosa!
      • Can see vesicles on anterior tonsillar pillars.
      • May also see lesions on anterior 2/3rds of the tongue.
  • Cranial Nerve Involvement then develops. [Aydogdu, 2015; Derin, 2014]
    • CN VII
      • Facial Paralysis develops within 1-2 weeks after rash develops.
    • CN VIII
      • May present with:
        • Nausea, Vomiting, Vertigo, Nystagmus
        • Tinnitus, Hearing Loss (occurs in ~24% of children with Ramsay Hunt Syndrome (RHS)).
        • Can be more prominent than the facial paralysis.
      • Less commonly seen in children than adults with RHS.
      • Presence of these findings is associated with worse prognosis.

Ramsay Hunt Syndrome: Management

  • Diagnosis is made clinically. [Aydogdu, 2015; Derin, 2014]
    • VZV IgG and IgM antibody titers can be measured to confirm diagnosis.
    • Lab values do not, however, determine management.
  • Therapies to consider: [Aydogdu, 2015; Derin, 2014]
    • Antiviral Rx (ex, Acyclovir)
      • Inhibit viral replication, reduce formation of new lesions, and allow for earlier healing.
      • Ideally started within the first 72 hours.
    • High-Dose Steroids (ex, Methylprednisolone)
      • Reduce the inflammation of the peripheral nerves.
      • Combined steroid with antiviral therapy is more effective than steroids alone.
    • Eye Care
      • Due to the facial paralysis, the eye will be at risk for not being protected.
      • Eye lubricants are recommended.
    • Nausea/Vertigo Management
      • Antiemetics
      • Sedatives

Moral of the Morsel

  • Once again… Viruses are not benign! Whether from a Pandemic or from your own nervous system, viruses can do a lot of damage!
  • Fever and Ear Pain without AOM. Hmmm… maybe this is early RHS. The otalgia may be the only symptom in the first 3 days.
  • Bell’s Palsy? Are you certain? Really evaluate for ear pain / rash or other vertigo symptoms.
  • Steroids and Acyclovir. Simple and effective. Try to start them early!

References

Sean M. Fox
Sean M. Fox

I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same. I trained at the Combined Emergency Medicine and Pediatrics residency program at University of Maryland, where I had the tremendous fortune of learning from world renowned educators and clinicians. Now I have the unbelievable honor of working with an unbelievably gifted group of practitioners at Carolinas Medical Center. I strive every day to inspire my residents as much as they inspire me.

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