PRIMARY RHINOSPORIDIOSIS OF NASOPHARYNX.

                             PRIMARY  RHINOSPORIDIOSIS  OF NASOPHARYNX.

                                  DR.K.K.DESARDA. DR. SHEETAL.DR.NEELIMA.

                                                       KEM HOSPITAL PUNE.

                  

Abstract.

A rare case of primary Nasopharyngeal rhinosporidiosis with extension to nasal cavity,maxillary sinus anteriorly and posteriorly extending to oropharynx is reported in a young male patient. The pathophysiology, clinical feature, diagnosis and management of this condition are reviewed.
Key words: Rhinosporidiosis, Rhinosporidium Seeberi

Indroduction:

Rhinospordiosis is a chronic granulomatous disease characterized by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces, the etiologic agent being Rhinosporidium seeberi. The disease was First described  by seeber (1900) in Argentina. This noval pathogen commonly affects mucosa of nose, eye and upper aero-digestive tract of men and animals. Isolated deep seated Rhinosporidiosis is rare. Diagnosis is mainly based on clinical suspicion and histopathological confirmation. At present, no existing medical treatment cures the disease and endoscopic excision of the mass with cauterization of the base is considered as treatment of choice.

Case Report

A 45 year old male patient came to us with history of left nasal obstruction,epistaxis, distorted speech with nasal twang and intermittent dysphagia and headache for over 6 yrs He was treated at the local district hospital but without great relief.
The patient did not give any history of TB,DM and any majot illness in past apart from nasal obstruction , intermittent nose bleeds,and nasal speech with poor intake of food.. patient was chchectic with mild pallor, with a pulse rate of 84/minute, regular, BP-100/80 mmHg, CVS-NAD, Chest:-NAD.

Investigations:

Hb%-8.0gm% ,TLC 8000/mm ,DC-N80%,L-18%,E-2%,ESR-5mm.

Serum urea 30mg%,serum creatinine-1.42%,Urine –NAD,

AbsAg –negative,Anti HCV-negative,Elisa for HIV-negative,serum Bilirubin0.6mg%

SGPT and SGOT within normal limits

DC:-N-82%, L-17%, E-01%
FBS:-106 mg%
ESR:- 5mm in 1st hr,
Sr.

Radiological investigations: x-ray chest NAD,CT coronal PNS revealed Hughe mass occupying left maxilla,OMU blocked with mass. The mass was occupied in the nasal cavity, nasopharynx with extension to oropharynx.FNAC done for HP. Examination.which revealed rhinospordiosis.

Nasal endoscopy revealed pinkish mass occupying left nasal cavity,nasopharynx and left maxilla. and mass was,protruding in to oropharynx ,pushing the palate anteriorly.The septum was pushed to right   causing  nasal obstruction.. Clinical diagnosis of Rhinospordiosis.was made.

Patient was advised surgery and subsequently undergone endoscopic excision.
Nasal endoscopy revealed pinkish mass occupying left  nasal cavity,nasopharynx and protruding in to oropharynx,pushing the palate anteriorly. The mass bled during the clearance. Complete endoscopic clearance of the naso-maxillary-and nasopharynx was done. The complete haemostasis secured.by cauterization.The anterior and post nasal packing with BIPP was done .The  enblock specimen was sent  for histopathological examination. Post op recovery was uneventful.

Patient was given broad spectrum antibiotic,anti inflammatory agents and Dapsone theray . He was advised to continue Dapsone theray 100mg OD for six months and attend follow up clinic every 3 months for any recurrence.

    Rhinosporidosis  post surgery specimen.

   

  Enblock  resection of  Rhinosporidium of Naso-oro pharynx.

    Histopathlogy:

                                             

 H&E stain of nasal polyp showing two mature sporangia and

several immature ones with a single centrally placed spore.

.
DISCUSSION:

Rhinosporidiosis is a chronic granulomatous disease characterized by production of polyps or other manifestations of hyperplasia on mucous membrane surfaces. The etiologic agent is Rhinosporidium seeberi.Most of the early studies of rhinosporidiosis were made in India and Ceylon where the disease occurs frequently. Sporadic case have been detected and studied in many parts of the world. The systematic position of R. seeberi is still uncertain. Most investigatrs consider it has not been isolated in culture.

 Friable, highly vascular, sessile or pedunculated polyps may appear on almost any mucosal surface, and rarely secondary lesions are found on skin, probably as aresult of autoinoculation by scratching. Lesions of the mucosae often spread by extension beyond the mucocutaneous border.
Primary lesions appear most often on the nasal mucosa and are accompanied by painless itching and a profuse mucoid discharge. The lesion is at first flat and sessile. Later hyperplastic growth greatly exceeds lateral extension of the lesion so that a polypoid mass much larger than the peduncle develops. The polyp may extend from the neres into the pharynx or externally over the lip and may reach weight of 20grams. It is friable and bleeds freely after trauma. Its surface is mucoid and papillate or so lobulate that its surface suggests that of a cauliflower. The color varies from pink to purplish red, and close examination of the surface mayh reveal minute white sports which are the mature sporangia of the fungus.
Lesions of the eye may cause symptoms similar to those produced by a foreign body, lacrimation or photophobia. Growth of the polyp may cause eversion of the lid. Lesions on th eskin being as papillomas and become warty with inclusions of myxomatous material. They are relatively painless except when on the sole of the foot and when they become so large as to be uncomfortably heavy. Dissemination to visceral organs is rare.

Differential diagnosis:

Typical lesions of rhinospordiosis can be recognized usually by the pink to purple colour, friable consistency and the presence of barely visible white sporangia within the polyp. Atypical lesions or those in unusual anatomical sites must be differential from warts, condylomata and hemorrhoids.

Immunology:

Little is known about the immunology of rhinosporidiosis.

Pathology:

H&E stain of nasal polyp showing two mature sporangia and

several immature ones with a single centrally placed spore

On the examination of the gross tissue, unless rhinosporidiosis has been suggested by the clinician, or by the history of the patient's geographic residence, the pathologist may consider the specimen an ordinary nasal polyp. The correct diagnosis can usually be made without difficulty on examination of routine H and E stained slide. Under the scanning lens of the microscope, although the polypoid structure may be evident, the histopathologic pattern differs greatly from tht of the common nasal polyp. The most striking feature is the presence in the stroma or epidermis of numerous sharply defined globular cysts which usually vary from 10 to 200.. Some of the cysts may be partly collapsed, assuming a semilunar shape. In contrast to the loose, edematous, myxomatous stroma of the ordinary nasal polyp, the stroma in rhinospordiosis is rather dense. There is a chronic inflammatory reaction in which neutrophils, plasma cells and lymphocytes are prominent. In contrast to the usual nasal polyp. Eosinophils are inconspicuous. Occasionally purulent microabscesses occur.
The cysts of all size have a sharply defined chitinous appearing wall. In a large maturing cyst the wall alone may be 5  thick. Histologically, rhinosporidiosis should be differentiated, specially in immuno-suppressed persons with other fungal infection like Coccidioides immitis.

Epidemiology:

Although rhinosporidiosis is seen most often in children and young adults, it occurs at any age. No racial difference in susceptibility are recognized. The disease is seen much more frequently in men than in women, but the extent to which this difference is related to greater frequency or severity of exposure is difficult to evaluate. Infections are seen most often in labourers and in those with frequent exposure to water of streams and pools.

Geographic distribution:

Rhinosporidiosis is found must often in India and Ceylon, but it is reported also from the East Indies, the Malay, States, the Philippines, Iran, South Africa, Italy, England, Scotland, Southern United States, Mexico, Cuba, Argentina, Brazil and Paraguay.
Source of infection:
The disease is not contagious, and sources of infection are exogenous. The frequent history of prior extended to water of pools and rivers and the occurance of multiple cases among those members of a group of workmen most intimately and repeatedly exposed to water source suggest the R. seeberi has a natural habitat in water. Rhinosporidiosis was observed in workmen who dived under water to bring up san din buckets, but not in their associates who carried the sand from the water's edge. It has been suggested that water insects or fish may be hosts of the fungus.

Laboratory diagnosis:

Direct examination of the surface of the polyp may reveal the subsurface position of sporangia which are white and so large (up to 350  in diameter) that thery can be seen with naked eye. Dissection of sporangia or excision and microscopic examination of tissue confirms the diagnosis. Culture is not successful, and the inability of R. seeberi to grow on artificial media, as well as some peculiarties about its reproductive cycle in tissues, have raised the question whether it is actually a fungus.
It is resembles in general appearance and in manner of sporulation some species of synchytrium, which are obligate parasites of plants, and which produce characteristic galls on the host plant. Animal inocultion is not helpful in diagnosis. Although R.seeberi is found in natural infections of horses, mules and cows, experimental infections usually do not succeed. Recently molecular methods like polymerase chain reaction are being developed for diagnosis.

Conclusion:

 Rhinosporidiosis, a fungal infection due to Rhinosporidium seeberi, frequently produces polypoidal lesions in the nose. Sites like the conjunctiva, larynx, trachea, nasopharynx, skin and genitourinary tract are less frequently involved. Generalized rhinosporidiosis with skin and visceral involvement is extremely rare... Smears revealed numerous sporangia and spores of R seeberi. There were no mucocutaneous lesions. Histologic examination confirmed the  diagnosis of Rhinospordiosis.  The FNAC diagnosis of rhinosporidiosis is specific. Preoperative diagnosis is possible even in cases with unusual clinical presentations.

Rhinosporidiosis should be suspected or considered in all cases of swellings of nose,

 nasopharynx and skin. Although disseminated Rhinosporidiosis is very rare, still

remains a possibility and requires a different mode of treatment. Presently the medical

treatment of Rhinosporidiosis is not satisfactory and requires further study and

research.Meanwhile patients should receive Dapsone therapy for over six months.

References:
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2. Karunaratne W.A.E.: Rhinosporidiosis in Man, London, Athlone Press, 1964.
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5. Baron, E.J., Peterson, L.R. Finegold, S.M. New, Controversial difficult-to-cultivate or non-cultivate etiological agents of disease in Bailey and Scott's Diagnostic Microbiology, 9th Edition, Mosby, st.Louis, Baltimore, Boston, 1994; p-585.