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Year: 2007  Vol. 11   Num. 2  - Apr/June Print:
Case Report
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Nasal Rhinosporidiosis - Four Cases Relate and Literature Review
Rinosporidiose Nasal - Relato de Quatro Casos e Revisão de Literatura
Author(s):
Lauro do Nascimento Abud1, José Clemente Pereira2
Key words:
Rhinosporidiosis. Etiology. Diagnosis.
Abstract:

Introduction: Rhinosporidiosis is a disease resulting from infection by Rinosporidium seeberi. There is a relation with patients from the countryside and with baths in lakes. It is endemic in Indian and Sri Lanka but relatively rare in Brazil. Clinically it is presented with nasal obstruction, epistaxis and pedunculated polypoid mass with septal implantation. Its treatment is surgical extirpation and eletrocauterization of the polyp base. Objective: To relate four cases of nasal rhinosporidiosis within a literature review and discuss its diagnosis, treatment and follow-up. Cases Report: The present article discribes four cases of nasal rhinosporidiosis and age range between 8 and 22 years old, all patients from the countryside and symptons of chronic unilateral nasal disease. Three of them were submitted to surgical treatment and one follows with espontaneus remission. Conclusion: Nasal rhinosporidiosis is a disease resulting from infection by Rinosporidium seeberi and presents a benign follow-up, its diagnosis and treatment are simple but we need to be aware of it. It is presented a case of espontaneous remission not found in world literature.

INTRODUCTION

Rhinosporidiosis is a chronic infection caused by Rinosporidium seeberi that mainly affects the nasal and conjunctive mucosas , being able to affect other regions as: vagina, penis, oral cavity, lachrymal sac, urethra, tracheobronchial tree, larynx, nasal bone and cavity (9,14). In only 6% of the cases it is presented as multiple injuries, and cutaneous and visceral affection is rarely described.

Described for the first time by Malbram (1892), Argentina, it was only published in 1900 by Guilhermo Seeber in his doctoring thesis, also in Argentina, classifying it as protozoon (12). O'Kinealy (1903), India, being unaware of Seeber's description, isolates the microorganism and baptizes it as Rinosporidium kinealy. In 1912, Seeber claims the priority of the discovery, thus being called Rinosporidium seeberi. Ashworth (1923) describes the agent at great length classifying it as fungus (1,3,6,10).

Geographically, most of the described cases proceed from India and Sri Lanka (9). In Brazil it is relatively rare (10).

It is clinically presented as friable polyp, of soft consistency, with moriform aspect, being its most frequent symptoms nasal blockage and epistaxis (3,10). It can affect, besides human beings, other animals as horses, bovines and geese (3). Its treatment is eminently surgical with resection of the injury and electrocoagulation of its insertion (3,6,10).

The present work aims at reporting 04 (four) c cases of nasal Rhinosporidiosis in the Otorhinolaryngology unit of Hospital de Base of the Federal District (HBDF) between May 2002 and July 2004, standing out important points for its diagnosis and treatment, as well as carrying out literature revision.


CASE REPORTS

Case 1


A 12-year-old patient, black, living in Barreiras-BA, proceeding from agricultural zone. History of baths in lakes and wells with backwater. He went to the Otorhinolaryngology clinic of HBDF with complaint of left nasal blockage for eight months associated to recurrent epistaxe of small size for which nasal packing was not necessary. He denied other complaints. At the physical examination he presented pinky mass in left nasal fossa with small yellowish points, polyploidy aspect, of soft and peduncle consistency, with insertion in nasal base and bleeding at touching. Incisional biopsy of the injury with histopathology result of RHINOSPORIDIOSIS was carried through. Medication was not used.

Patient returned one month after his first assistance with spontaneous remission from of the nasal injury and improvement of the clinical symptoms. Nasal video-endoscopy was carried out, confirming remission. He has been on semestrial assistance for two years without return signals.

Case 2

22-year-old patient, male, black, living in Brasilia for one year, proceeding from agricultural area of the Bahia. Antecedent of bath in wells with backwater. Complaint of right nasal blockage for one year with recurrent and right nasal mass. Without other complaints or antecedents of clinical interest. At the physical examination he presented tumor obstructing all right nasal fossa, peduncle with insertion in head of inferior corneto, polyploidy, pink aspect with small yellow points over the mass (strawberry aspect), bleeding at touching. He had been forwarded from another clinic with CT scan of facial sinus which evidenced mass in right fossa, inferior insertion in corneto of 2.5 cm Histopathologic of RHINOSPORIDIOSIS (Figures 1, 2).


Figure 1. Lamina of second patient evidencing nasal tissue with several cysts in different phases maturation with important lymphocyst infiltration.


Figure 2. Lamina of second patient demonstrating mature cyst (sporangium) full of endospores initiating its rupture.



He was then undertaken to resection of the injury under local anesthesia in the Surgical Center with electrocoagulation of the implantation base. Nasal packing was not necessary. Medication after the surgery was not used, with exception of analgesics and antibiotic prophylaxis before the surgery.

The patient was evaluated with 2, 4, 6 months and 01 year after the surgery without return signals. Total improvement of the symptomatology. He is under semestrial assistance.

Case 3

A 15-year-old patient, male, black, proceeding from Maracaçumé - MA (agricultural zone). He went to our clinic of Otorhinolaryngology with complaint of gradual right nasal blockage for 08 months and sensation of strange body in the nose. He denied epistaxis. Without antecedents of clinical interest, also denied baths in lakes. At the right nasal examination he presented mass of polyploidy aspect in right nasal fossa, of moriform aspect, soft consistency, peduncle with insertion in anterior nasal sept, bleeding at touching (Figures 3, 4). Incisional biopsy with histopathologic of RHINOSPORIDIOSIS was carried out.


Figure 3. Previous Rhinoscopy of the third patient.


Figure 4. Surgical part of the third patient.



Patient was then submitted to resection of the injury under local anesthesia in the Surgical Center with electrocoagulation of its insertion. A small nasal drain was placed, which remained for 24 hours. Medication after the surgery was not used, with exception of analgesics and antibiotic prophylaxis before the surgery. Complete remission of the symptomatology. Follow-up of 01 year without return signals. The patient is under semestrial assistance.

Case 4

A 8-year-old female patient, proceeding from agricultural zone of Nova Conquista/MA, black race. Complaint of left nasal blockage for 01 year associated to recurrent epistaxis, nasal mass and sporadically bloody pyorrhea. Antecedent of baths in lakes with backwater.

He presented, at the examination, in left nasal fossa, polyploidy, granulomatous tumor, with bleeding aspect at touching, peduncled with previous insertion in nasal sept (Figures 5, 6, 7). Histopathologic after incisional biopsy: RHINOSPORIDIOSIS.


Figure 5. Previous Rhinoscopia of fourth patient - moriforme aspect.


Figure 6. Previous Rhinoscopy of the fourthpatient evidencing bleeding of the injury after simple cleanness.


Figure 7. Surgical part of the fourth patient.



She was undertaken to resection of the injury under general anesthesia in the Surgical Center with electrocoagulation of the insertion base. Complete remission of the symptomatology. Follow-up of 06 months without return signals. Medication after the surgery was not used, with exception of analgesics and antibiotic prophylaxis before the surgery.


DISCUSSION

Rhinosporidiosis is an illness of low incidence being the endemic zones dispersed for all over the world, predominating in tropical and subtropical regions where backwater and marshy zones exist. Most of the described cases proceed from India and Sri Lanka, east of Africa and America, being the description rare in the Europe (3). In Brazil few described cases exist, which is a statistics that can be imperfect once not all the cases are reported and because not all the nasal polyps which are surgically removed are submitted to the histopathologic examination (4,7).

The etiologic agent of Rhinosporidiosis, Rinosporidium seeberi, initially described as a protozoon by Guilhermo Seeber, started to be classified as one fungus from the accurate description by Ashworth (1923) (1). Useless attempts so far of culture of the pathogen carried through by Vanbreuuseghem (1976) defy the fungi hypothesis (13,14). Since then many microbiologists have considered it as one fungus although its uncertain taxonomy (3,5,6,9,11,14).

Fredricks and cols in recent study (2000), through genetic research, classify the Rinosporidium seeberi as the first human pathogen of t DRIP class, which is a class of aquatic parasites, Ichthyosporea(5).

Little is known about the habitat and mechanism of transmission of such pathogen. Some ways of transmission are postulated but none has been confirmed. The currently most accepted hypotheses are still part of the accurate description carried out by Ashworth (1923), pointing a relation between contagion and patients who had contact with backwater (1,5).

It is suggested that the ground with dry excrement particles of animals and the water can be contaminated with spores of the pathogen, thus the dust inhalation and/or the contact of the nasal mucosa with contaminated water inoculated through digital-ungual microtraumatisms would be the possible ways of transmission (4), explaining the predominance in the nasal and ocular conjunctive cavities.

Although there is a great amount of spores in the nasal cavity of the sick person, the infectivity is low, since long-term carriers rarely have other infected relatives at home (11,14,15).

The populations of low economic level are more frequently attacked with a great part of the sick people who come from agricultural zone, mainly agriculturists and swimmers of lagoons (8).

Some cases are described in animals such as horses, fish and cattle in general. However, the transmission animal-man is not proven (14). In the endemic regions of India and Sri Lanka the infection in animals is frequent and it is believed that man is an accidental host of the illness (2).

Rhinosporidiosis is more frequent in men than in women (3:1). According to some authors this happens due to the highest exposition of the masculine sex to jobs related to land, adobe and backwater (3,4,6). We do not observe predominance in relation to the sex (1:1), however great part of the described cases in literature is proceeding from India and Sri Lanka where the cultural limitations to which women are submitted hinder them to perform activities exposed to the contagion. The two aforementioned described patients of the feminine sex had been exposed to baths in backwater and manual activity, although they were under 18.

Most of the cases is observed between the 10 and 40 years of age being able to be found in younger and older patients, according to our casuistry. Difference of susceptibility for races is not known (6). Despite our casuistry evidences affection in the black race, we believe, however, that this predominance is consequence of the biggest incidence of the black race in northeast agricultural zone.

The most frequent localization is the nasal (70%) although it is found in other localizations as conjunctive (15%), oral cavity, lachrymal sac, urethra, paranasal genitalia, larynx, skin, bone (10). It can affect more than one localization per patient. The most common nasal sites are, in decreasing order, nasal sept mucosa, inferior cornet and nasal fossa base (6).

The clinical treatment is slow and the generally unilateral complaints are characterized as: nasal blockage, epistaxis, pyorrhea, nasal mass, and sensation of strange body (4,6). In the clinical examination it is presented as tumor of polyploidy aspect, friable, bleeding at touching, painless, being able to be sessile or peduncle and finely lobate with small yellow points over the mass (strawberry aspect), corresponding to mature sporangia (4,6).

There is description of systemic dissemination through hematogenic via in a patient with primary injury in the skin and that during 1 year presented systemic extension of his injuries (2).

Due to the macroscopic similarity with other pathologies it is necessary to carry through differential diagnosis with: angiofibroma, inverted papilloma, nasal polyps, among others (6).

The diagnosis is based on clinical history, with important epidemiologic approach, detailed otorhinolaryngologic examination and histopathologic study for confirmation (6).

The histologic diagnosis is easy, once the presence of the sporangia in different periods of training of maturation is unmistakable (6). The corresponding epithelium to the affected zone is recognized (generally nasal mucosa) underneath which there is one stroma with edema in which some cysts are found in different periods of maturation. These cysts vary between 10 and 350mm of diameter and contain several spores (endospores) as big as a red blood cell (approximately 16,000). When under development , these cysts are called trophocyst and sporangia when they are mature. Stroma shows a chronic inflammatory reaction with predominance of plasmatic cells, lymphocyte and scarce neutrophils. They differ from the allergic polyps for the little amount of eosinophils. Some of these sporangia can be ragged and spores are observed in the exudates of stroma. These ragged sporangia can provoke granulomatous reaction, strange body type(6).

The treatment of Rhinosporidiosis is eminently surgical with resection of the injury with electrocoagulation of the implantation base (3,4,5,6,9,10,14), to reduce returns or residual tumor, being the hemorrhage its most common complication (3).

The medicament treatment is described, however without important result when used separately. Diaminodiphenyl sulphone (dapsone) is used, by some authors, adjuvant to the surgical treatment to reduce the return index (3,7).

The return index is very changeable in literature, 10 to 70%. However, most of the reports show incidence in average of 10% related to incomplete exeresis of the injury (8). Delayed returns are described, being long-term assistance necessary (6). In the present study we do not observe any return until the present moment, however all the patients keep semester assistance.

We have presented a case of spontaneous remission after incisional biopsy. Such patient did not make medication use for treatment, as well as the other patients, once the medicament treatment together with the surgical treatment is not part of our clinic's conduct. The patient denies any "homemade" treatment. Extensive similar bibliographical revision was carried through however we do not find similar report.


FINAL COMMENTS

- Rhinosporidiosis is a chronic infectious illness caused by the Rinosporidium seeberi, presenting slow and benign clinical treatment.

- Its diagnosis, when detailed clinical history is carried out, with approach in epidemiologist data, physical and histopathologic examination, is relatively easy.

- The surgical treatment is simple, with excellent results and excellent prognosis. However its follow-up must be long for the risk of delayed returns.

- Although there are not similar case described, we have observed case of spontaneous remission after incisional biopsy.


BIBLIOGRAPHY

1. Ashworth JH, Logan Turner A. A case of rhinosporidiosis. Journal of Laryngology and Otology. 1923, 38:285-299.

2. Connant NF, Smith DT, Baker RD, Callaway JL. Micologia, rinosporidiosis. Tercera edición. México:Nueva Editorial Interamericana. 1972, 374-382.

3. Dadá MS, Ismael M, Neves V, Neves JB. Presentación de Dos Casos de Rinosporidiosis Nasal. Acta Otorrinolaringol Esp. 2002, 53:611-614.

4. França Jr GV, Gomes CC, Sakano E, Altemani A, Shimizu LT. Rinosporidiose nasal na Infância. Jornal de Pediatria.1997, 70:299-301.

5. Fredriks DN, Jolley JA, Lepp PW, Kosek JC, Relman DA. Rhinosporidium seeberi: A Human Pathogen from a Novel Group of Aquatic Protistan Parasites. Emerging Infectious Diseases. 2000, 6:273-282.

6. González G, Viada J, Escalona A, Náquira N. Rhinosporidiosis. Rev. Otorrinolaring. 1985, 45:39-46.

7. Job A, Venkateswaran S, Mathan M, Krishnaswani Raman, R. Medical Therapy of Rhinosporidiosis with Dapsone. The Journal of Laryngology and Otology. 1993, 107:809-812.

8. Melo RRG, Boolinger S. Rinosporidiose Nasal. Revista Brasileira de Oto-Rino-Laringologia. 1975, 41:44-5.

9. Mohan H, Chander J, Dhir R, Singhal U. Rhinosporidiosis in Índia: a case report and review of literature. Mycoses. 1995, 38:223-225.

10. Ramos S, Ramos RF, Vargas PRM, Caetano RF, Figueiredo FA, Souza FS. Rinosporidiose: relato de um caso. Revista Brasileira de Otorrinolaringologia. 1998, 5:159-161.

11. Satyanarayana C. Rhinosporidiosis with a record of 255 cases. Acta Oto-Laryng. 1960, 51:348-366.

12. Seeber GR. Un Nuevo esporozoario parasito del hombre dos casos encontrados en polipos nasales. Thesis University Nac. de Buenos Aires. 1900.

13. Vanbreuseghem R. Rhinosporidiosis: Klinischer Aspeckt, Epidemiologie and ultrastructurelle Studien Von Rhinosporidiosis seeberi. Dermatologische Monatsschrift. 1976, 162:512-526.

14. Van Der Coer JMGL, Maires HAM, Wieling EWJ, Wong-Alcalá LSM. Rhinosporidiosis in Europe. The Journal of Laryngology and Otology. 1992, 106:440-443.

15. Van Haacke NP, Mugliston TAH. Rhinosporidiosis. The Journal of Laryngology and Otology. 1982, 96:743-750. Abud LN Arq. Int.









1. Evidence of Otolaryngology Specialist by the Brazilian Association of Otolaryngology and Cervico Facial Surgery. Otorhinolaryngologist and former Resident of Otolaryngology at the Hospital de Base of the Federal District.
2. Otorhinolaryngologist.

Institution: Department of Otolaryngology and Head and Neck Surgery of the Base Hospital of the Federal District.

Lauro Birth of Abud
Mailing address: Square Tobias Barreto, 510, Rooms 403/404 - Neighborhood San Jose - Aracaju/SE - CEP 49015-180 - Fax: (79) 3214-0831 - E-mail: labud@uol.com.br

This article was submitted in PMS (Management System Publications) in the R @ IO 28/7/2006 and approved in 1/10/2006 23:08:00.

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