41•4° Acta Med Kinki Univ 0 •

Vol. 37, No. 1 45-48, 2012

A case of allerg is fungal rhinosinusitis

Naoki Shogaki, Daisuke Muramoto, Akihide Yamamoto, Kazunori Mori and Katsumi Doi

Department of Otolaryngology Kinki Osakasayama, Osaka

University Faculty of Medicine, 589-8511, Japan

Abstract

Allergic fungal rhinosinusitis (AFRS) is a

type of paranasal sinusitis, the cause of which is

related to an allergic reaction to fungal infec-

tion. This condition has been called "allergic mucin", characterized by the presence of eosino-

phils concentrated in the mucosal fluid. AFRS in Japan is still rare or might be un-/mis-

diagnosed by the lack of proper recognition of this specific condition. In this report, we

describe a case of AFRS caused by Alternaria

fungi infection. The case is 40-year-old woman.

The first recommendations in the treatment for

AFRS are surgery. However, when postoper-

ative treatment is not appropriate, the recur-

rence rate may increase. In this case, we did

operation for two times. The nature of AFRS is

intractable, and careful follow-up and additional

treatments may be necessary even after surgery.

Key words : Alternaria, allergic fungal

rhinosinusitis, AFRS

Introduction

Allergic fungal rhinosinusitis (AFRS) is a type of paranasal sinusitis, the cause of which is related to an allergic reaction to the fungal infec-tion. Fungi grow in the mucous membrane lining the nasal cavity, remain there, and never cause systematic invasion. This condition has been called "allergic mucin" characterized by the

presence of eosinophils concentrated in the mucosal fluid. Dematiaceous fungi and Asper-

gillus have often been reported as the causative agents. The number of reports concerning AFRS has been increasing worldwide after

Millar's' first report in 1981, but AFRS in Japan

is still rare or might be un-/misdiagnosed by the lack of proper recognition of this specific condi-tion. In this report, we describe a case of AFRS caused by Alternaria fungi infection, along with a literature review.

Case report

Case : 40-year-old woman

Chief complaint : Stuffy nose Medical history : The patient visited our ear,

nose, and throat (ENT) department of Kinki

University Hospital since she had been aware of

a stuffy nose for 1 year. Nasal endoscopic inspec-

tion revealed polyp formation occupying the

Fig. 1 CT images before the first operation

Received May 10, 2011 ; Accepted July 29, 2011

45

N. Shogaki et al.

entire left nasal cavity. Plain Computed Tomo-

graphy (CT) imaging confirmed the presence of soft tissue densities located in the left frontal, ethmoidal, and maxillary sinuses, and bone destruction in part of the left maxillary sinus

(Figure 1). Bone defects were present in some parts of the maxillary sinus cavity, which suggest-ed the possibility of malignant disease. Past history : Aspirin asthma Serologic examination : Leukocytes, 7,200/Ju1

(eosinophils : 5.9%) ; total serum IgE, 684 IU/ ml (standard level : under 295 IU/ml) ; specific IgE value (standard level : under 0.34 IU/ml) to

Aspergillus : 1.51, Alternaria : 10.0, Candida : 1.23. Surgical findings : Endoscopic sinus surgery was conducted under general anesthesia. Polyps were first removed with a micro-debrider, and then the opening of the left maxillary, ethmoidal, and frontal sinuses was completed, which made it possible to discern that the fungal body looked like a peanut-butter lump in those sinuses. Clinical course after surgery : The ethmoidal sinus became re-congested within 2 months after surgery, but no additional therapy was conduct-ed. When the patient visited again two years after surgery, the recurrence of AFRS was in-dicated on the opposite side (Figure 2). She decided to receive a second surgery under general anesthesia. Almost the same procedure was

performed to complete the opening of the right maxillary, ethmoidal, and frontal sinuses.

Prognosis : Although the patient experienced bilateral ethmoidal sinusitis within one month after the second surgery, conservative treatment with Montelukast sodium (nasal steroid drop) and daily Sinus washing with saline at home

gradually reduced mucosal swelling. Postoper-ative CT imaging indicated some mucosal swell-ing in the bilateral frontal sinuses, while other sinuses remained clear (Figure 3). The patient is now free from any subjective complaints. Pathological examination : HE staining demon-strated an accumulation of infiltrated eosino-

phils and Charcot-Leyden crystals in the allergic mucin collected during the surgery (Figure 4), while Grocott stain detected a conidial chain and hyphal wall (Figure 5). Finally, Alternaria fungi were separated from the submitted speci-men culture.

Fig. 2 CT image before the second operation

Fig. 4

(Bar : 100,am)

Allergic mucin in a frozen section Accumulation of eosinophils embedded in mucin is

seen (black arrow). Charct-Leyden crystals are eosinophilic and characterized by a spindle shape with sharply pointed ends (white arrows).

Fig. 3 CT images after the second operation

46

A case of allergic fungal rhinosinusitis

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t's methenamine silver-stained paraffin sec-Grocott's methenamine silver-stained paraffin se tions

Septate fungal hyphae are clearly visible (arrows).

Discussion

AFRS is a type of paranasal sinusitis, the

cause of which is related to an allergic reaction

to fungal infection. Millar's report, entitled : "Allergic aspergillosis of the maxillary sinuses"

, in 1981, is the first document in the literature.'

In 1989, the condition was defined as "Allergic

fungal sinusitis", since non-A spergillus fungi also

produce the same pathology.2 Since then, a number of reports on AFRS have been publi-

shed in Europe and the United States,3'4 while

this specific condition has not well recognized in Japan. In 2006, the criterion for AFRS was

proposed based on the guidelines of 5 medical associations including AAAAI5 (American Academy of Allergy Asthma and Immunology), in which "rhinosinusitis" was classified into the following four types : 1) Acute presumed bacterial rhino sinusitis

(ABRS) 2) Chronic rhinosinusitis without nasal polyps

(CRSsNP) 3) Chronic rhinosinusitis with nasal polyps

(CRSwNP) 4) Allergic fungal rhinosinusitis (AFRS) Detailed criteria for AFRS diagnosis including symptoms and objective documentations are

provided as well (Table 1). The present case satisfies most symptoms and documentations and can be easily diagnosed according to these criter-ia.

It was reported that the prevalence of AFRS was 4-10% among the cases of chronic rhinosinusitis requiring surgery in Europe and the United States.6-8 There are a few domestic

reports : the prevalence was 3.9% in Matsuwaki's and 8.3% in Nakayama's report, respectively.9,1° According to Cody's report," the AFRS causa-tive agents are rated as follows : Bipolaris : 22. 5%, Curvularia : 19.6%, Aspergillus : 14.7%, Dre-chslera : 4.9%, Alternaria : 3.9%, Cladosporium :

Table 1 Criteria for AFRS (AAAAI, 2006)

Pattern of symptoms

Symptoms present For> 12 wk

Symptoms for diagnosis

Requires > 1 of the following symptoms :

1. Anterior and/or posterior drainage 2. Nasal obstruction

3. Decreased sense of smell

4. Facial pain/pressure/fullness

Objective documentation Requires :

1. Endoscopy to document presence of allergic mucin (pathology showing sparse fungal hyphae with

degranulating eosinophils) and inflammation, such as edema of middle meatus or ethmoid area or

NPs 2. Evidence of rhinosinusitis by CT or MRI

3. Evidence of fungal specific IgE (skin test or in vitro blood test)

4. No histologic evidence of fungal invasion when risk factors for invasive fungal disease are present

Other possible documentation(Not required) : 1. Fungal culture

2. Total serum IgE level

3. Imaging by more than one technique (CT or MRI) highly suggestive of AFRS

47

N. Shogaki et al.

1.0%. Although AFRS caused by Alternaria was not common in the USA, further analyses is necessary to clarify the causative fungi in Japan. In the diagnosis of AFRS, identifications of allergic mucin, viral existence, and Charcot-Leyden crystals are critical.3-5 In our case, HE staining demonstrated the accumulation of eosinophils and Charcot-Leyden crystals in aller-

gic mucin (Figure 4), and Grocott staining demonstrated the fungi in the specimen (Figure 5). The first recommendations in the treatment for

AFRS are surgery and systemic steroid adminis-tration.'2 Conservative therapies such as applica-tions of topical steroid, antifungal agents, and anti-leukotriene agents are also recommended." Surgical treatment is most effective to remove allergic mucin and the causative fungi, while

preserving the normal mucous membrane and promoting drainage/ventilation of the paranasal

sinuses."'" However, when postoperative treat-ment is not appropriate, the recurrence rate may

increase.'243 In this case, the treatment after the first surgery was just observation without any medication. As a result, recurrence of the disease occurred not only on the affected side, but also on the opposite side within two years. There-fore, the openings of the parasinuses were fully

dilated in the second surgery to prepare for

postoperative treatment. Mucosal swellings in bilateral ethmoidal sinuses remained after one month. With applications of topical steroid drop, Montelukast sodium (anti-leukotriene), and daily washing of the sinuses with saline, mucosal swelling gradually improved. The

patient has continued the same conservative treatments, and is still free from any objective symptoms, although CT scanning revealed some soft tissue densities in the frontal sinuses (Figure 3). There have been a few reports on AFRS in

Japan, indicating the possibility that a number of undiagnosed and misdiagnosed cases exist. It is unfortunate to say that this disease is not well recognized by pathologists and ENT doctors. Therefore, it is important for ENT doctors to ask

pathologists whether or not the pathologies of AFRS and fungi are present in specimens col-lected during surgery. The nature of AFRS is intractable, and careful follow-up and additional treatments may be necessary even after surgery.

Conclusion

A case of allergic fungal rhinosinusitis

(AFRS) caused by Alternaria fungi was report-ed. There is only one report except our report in

Japan which caused by Alternaria fungi, as long

as we researched. After two surgeries and subse-

quent conservative therapies, her AFRS became well controlled. The nature of AFRS is intrac-

table, and careful follow-up and additional treat-ments may be necessary even after surgery.

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