Volume 4, Issue 4 , Pages 143-146, December 2009
Facial paralysis due to invasive Aspergillus of the temporal bone in an immunocompetent child
Article Outline
Summary
Fungal mastoiditis caused by Aspergillus fumigatus predominantly occurs in immunocompromised patients. However, few cases were reported in the immunocompetent patients. These cases are usually challenging to cope with in terms of diagnosis and treatment. Facial nerve dysfunction is one of the accompanying complications of fungal mastoiditis. We present a case of facial paralysis due to fungal mastoiditis in a 5-year-old immunocompetent child. She had a history of prolonged use of broad spectrum antibiotics due to meningitis and ear surgery.
Keywords: Facial nerve paralysis, Aspergillus, Immunocompetent, Temporal bone, Child
1. Introduction
Invasive fungal infection has been widely reported in the rhinologic literature, but sparsely reported in the otologic literature. Fungal infection of the ear is almost exclusively restricted to the external auditory canal. In the case of chronic otitis media, fungi can spread to the tympanic cavity, but it usually remains harmless, which does not require intensive clinical intervention [1]. Common fungal pathogens isolated are Aspergillus and Candida species.
Invasive temporal bone fungal infection, fungal mastoiditis, is a rare entity, but a serious infection that is most commonly seen in immunocompromised patients. Aspergillus spp., particularly Aspergillus fumigatus, are the most frequently isolated fungi. Other rarely reported fungi are Mucor [2], Blastomyces dermatitidis [3], Scedosporium apiospermum [4], Lecythophora hoffmannii [5].
One of the most common etiology of the facial nerve paralysis in childhood is infectious causes, particularly bacteria. Fungal etiology is rare and is usually due to fungal mastoiditis that is associated with immunocompromised state.
We present a case with facial paralysis due to invasive A. fumigatus of the temporal bone in an immunocompetent child.
2. Case report
A 5-year-old girl, who was operated from both ears due to chronic otitis media (COM) with cholesteatoma, was referred to our clinic with pain, otorrhea at the right ear and signs of right peripheric facial paralysis. One year ago, a canal-wall down mastoidectomy operation was performed to the left ear and the follow-up period was uneventful. Meningitis, a complication of COM with cholesteatoma, developed during the preoperative period of the right ear. She was operated after the treatment of meningitis in the intensive care unit with broad spectrum antibiotics. She underwent radical mastoidectomy due to diffuse cholesteatoma in the mastoid and middle ear cavities. Facial nerve canal was reported normal in the operation and postoperative facial nerve functions were also normal. She was discharged from the clinic with antibiotics and ear drops. Facial paralysis, dizziness and otorrhea developed 2 months after the operation. The microscopic examination revealed insufficient epithelization of the cavity, necrosis of the obliterative local muscle flap and foul-smelling otorrhea. House-Brackman Grade IV facial paralysis was determined clinically. The cavity was cleaned and culture was obtained. Local and broad spectrum antibiotics and steroids were administered empirically until the culture result was obtained. As the culture was negative and the complaints of the patient continue, mastoidectomy cavity débridement was performed under general anesthesia. During the débridement, a severe bone destruction was observed and tissue culture was obtained again. The tympanic segment of the facial nerve canal was totally and the labyrinthine segment was partially dehiscent. They were previously intact. The facial nerve seems to be suspiciously necrotic and dense necrosis was observed around the fallopian canal. Lateral and superior semicircular canals were destructed and bone destruction was observed in the whole mastoidectomy cavity and sparse bone erosion was seen at the dural plate of sigmoid sinus and anterior and posterior cranial fossa.
The culture of mastoidectomy cavity revealed A. fumigatus sensitive to Voriconazole. No immunocompromising factor was determined in the clinical and laboratory work-up of the patient. Treatment of Voriconazole (Vfend, Pfizer, Germany), 8
mg/kg/day intravenously, was given for 2 weeks. Then, Voriconazole was administered 100mg/day per oral for 6 weeks. Voriconazole has three important side effects, such as liver abnormalities and hepatitis, skin abnormalities and transient visual disturbances [6]. Liver function tests (alanine transferase and aspartate transferase), ophthalmologic and dermatologic consultations of the patient were performed once a week. No side effect of voriconazole was observed in our patient during the treatment and it was well tolerated. It is available in both oral and intravenous preparations, and the bioavailability of both is excellent. Dizziness, pain and otorrhea complaints were recovered and the cavity was almost epithelized and facial paralysis was regressed to House-Brackmann IV for the mouth and II for the eye clinically at the end of a follow-up period of 2 months (Fig. 1). At the end of a follow-up period of 6 months, the temporal bone computerized tomography of the patient revealed destruction and new bone formation of the lateral semicircular canal and destruction of tympanic segment of the fallopian canal (Fig. 2) and the electromyography study revealed a partial facial nerve injury and on going regeneration. No additional surgical intervention was performed. In the follow-up period for 8 months up to now, facial paralysis was regressed to House-Brackmann III for the mouth and II for the eye clinically and she had no vestibular complaints. Informed consent of the patient was obtained for the figures.
Fig. 1.
(a) Right facial paralysis, before the treatment, (b) Right facial paralysis, after the treatment.
Fig. 2.
(a) Destruction of the fallopian canal (thin arrow) and destruction of the lateral semicircular canal (thick arrow) (Axial CT scan), (b) Destruction of tegmen tympani (thin arrow) and tegmen mastoidea (thick arrow) (Coronal CT scan).
3. Discussion
Aspergillus is a common saprophyte that occurs worldwide and the most common isolate is A. fumigatus. Although fungal mastoiditis caused by A. fumigatus predominantly occurs in immunocompromised patients [7], few cases were reported in the immunocompetent patients [1], [4], [8], [9], [10], [11]. Risk factors for invasive fungal disease include cytotoxic drugs, irradiation, prolonged neutropenia, graft-vs-host disease, T-cell immunodeficiency, poorly controlled diabetes mellitus, broad spectrum antibiotics, systemic corticosteroids, prolonged hospitalization, status and type of underlying malignancy, and acquired immunodeficiency syndrome [7], [12]. We think that fungal mastoiditis was developed due to the use of prolonged local and systemic antibacterial agents and prolonged hospital stay in our patient. No immunocompromising factor was determined in the clinical and laboratory investigation of the patient.
The spectrum of fungal infections has become increasingly broad in immunocompetent patients, besides immunosuppressive diseases. In immunocompetent hosts, spread of fungal spores is usually controlled by innate immune factors such as phagocytic activity. When innate immunity fails, hyphal formation in tissues may occur. Infection then becomes established to produce disease, as leukocytes are less effective in removing hyphae. Cases in which spontaneous invasive fungal infections arise without any apparent evidence of an immunocompromised state is still of particular interest [5].
Diagnosis of invasive fungal temporal bone infection requires a high index of suspicion and it is difficult to diagnose. In spite of an antibacterial treatment, patients who have prolonged otorrhea and rapidly progressing audiovestibular symptoms and signs should be considered in terms of a fungal infection. Also, postoperatively, insufficient and delayed epithelization of the mastoid cavity can demonstrate such a disease. In diagnosis, bone scanning and PET-scan can be performed in addition to culture and temporal bone computerized tomography. Because, bone scanning can demonstrate the osteoblastic reaction secondary to osteitis by the high uptake of the isotope in the involved mastoid [13]. PET-scan can accurately demonstrate the phase of inflammation in healing bones and the progress of infections in osteomyelitic bones [14]. But, it was not available to perform bone scanning and PET-scan in our hospital. This disease is often aggressive and can extend through soft tissue and vascular planes into the adjacent structures. Without any intervention, death is likely, and even after the recommended therapy, mortality and morbidity related to facial nerve palsy, partially and totally sensorineural deafness, perilymph fistula and balance problems are common [1], [7], [15]. Our patient was also presented with hearing loss, dizziness and facial paralysis. Once the diagnosis is confirmed, treatment of fungal mastoiditis consists of aggressive antifungal chemotherapy, surgical débridement and attempts to control the underlying immunological condition.
Previous reports have described kinds of drugs, such as Amphotericin B, itraconazole, voriconazole and fluconazole, effective against the invasive Aspergillus [1], [7], [10]. We also treated our patient with voriconazole for 2 months and the disease was controlled. Voriconazole is a new, broad spectrum triazole antifungal agent. Structurally, it is related to fluconazole, but as a second-generation drug its potency and spectrum of activity are improved. It is primarily used to treat acute invasive aspergillosis and voriconazole is more effective than amphotericin B for the treatment of invasive aspergillosis [16].
In conclusion, fungal mastoiditis in childhood should be considered not only in immunocompromised patients, but also in the immunocompetent patients that have insufficient and delayed epithelization of the mastoid cavity and have prolonged otorrhea which does not respond to antibacterial agents. Although it is rarely seen, fungal agents should be considered in the etiology of the facial paralysis in childhood.
Acknowledgement
Presented as a poster presentation at the 8th International Cholesteatoma and Ear Surgery on June 15-20, 2008, in Antalya, Turkey.
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PII: S1871-4048(08)00072-5
doi:10.1016/j.pedex.2008.10.002
© 2008 Elsevier Ireland Ltd. All rights reserved.
Volume 4, Issue 4 , Pages 143-146, December 2009
