The risks of rejection vs. infection: Ramsay Hunt syndrome, Gradenigo syndrome, and varicella meningoencephalitis in a heart transplant patient

Background Orthotopic heart transplant (OHT) recipients are at increased risk for varicella zoster reactivation, and severe complications may arise due to their immunosuppressive regimens. Managing immunosuppression in acute infection is difficult, and specific guideline recommendations or evidence from the literature are lacking. However, patient care must involve weighing the risk of transplant rejection with the consequences of worsening infection. Case summary An OHT patient with a history of multiple episodes of acute rejection, latent varicella zoster virus (VZV) infection, and recent completion of anti-viral prophylaxis presented with unilateral facial droop and pain, abducens nerve palsy, crusting facial rash, and ear swelling. Imaging revealed necrotizing otitis externa, with associated otitis media, and petrous apicitis concerning for Gradenigo syndrome. A VZV-positive viral panel confirmed our suspicion for Ramsay Hunt syndrome (RHS). The patient's mentation continued to decline, and subsequent lumbar puncture also revealed VZV meningoencephalitis. The patient's mycophenolate mofetil (MMF) was suspended, with continuation of tacrolimus, and initiation of intravenous acyclovir. The patient demonstrated gradual resolution of his infection, without developing any signs of acute rejection. Discussion Varicella zoster virus reactivation is common in OHT patients, particularly when viral prophylaxis is discontinued; however, cardiologists should be aware of the rarer manifestations that can manifest in these immunocompromised patients. This is the first documented case of simultaneous RHS, Gradenigo syndrome, and VZV meningoencephalitis in any patient, regardless of transplant status. We demonstrate that even in patients at very high risk of rejection, MMF can be safely discontinued and host immunity maintained with temporary tacrolimus monotherapy.