| Abstract|| |
Rhinosporidiosis is a chronic granulomatous disease caused by Rhinosporidium seeberi. It usually affects the mucocutaneous tissue of the nose. Bone involvement is rare. We report a case of Rhinosporidiosis of the nasopharynx which later involved the right little finger where ray amputation was performed.
Keywords: Rhinosporidiosis, osseous involvement, little finger
|How to cite this article:|
Kundu AK, Phuljhele S, Jain M, Srivastava RK. Osseous involvement in rhinosporidiosis. Indian J Orthop 2013;47:523-5
| Introduction|| |
Rhinosporidiosis is a chronic granulomatous disease caused by Rhinosporidium seeberi. It is found both in man and animals and is mostly transmitted by direct contact with spores through dust, infected clothing or fingers and bathing in stagnant water.  The usual site of affection is the nasopharyngeal mucous membrane followed by ocular involvement; other sites like bones are rare.  We report a case of osseous spread of rhinosporidiosis involving the hand in a patient with a previous surgery for nasal polyp.
| Case Report|| |
A 23 year male, farmer by profession of Raipur District, Chhattisgarh, India presented with 1 year history of progressive swelling over ulnar aspect of the right hand. He could not recollect any history of trauma. The mass caused occasional discomfort. It had ulcerated with bleeding 1 month ago. He had since earlier been operated thrice for nasal polyp in past 5 years, which was however confirmed as Rhinosporidiosis on biopsy.
Clinical examination revealed the swelling to be warm, circumscribed, firm, measuring 5 cm × 4 cm and largely confined to ulnar aspect of hand [Figure 1]a and b. The swelling had multiple ulcerations with friable tissue and bleeding points. Movements of hand were almost full with slight restriction of movement of 4 th and 5 th fingers. Examination of the nose revealed a small polyp in right antrum [Figure 2]. He also had a swelling of the right lacrimal gland. Radiograph of the hand revealed soft-tissue swelling with destruction of the distal part of 5 th metacarpal and whole of proximal phalanx. There was no periosteal reaction or soft tissue calcification [Figure 3]. Magnetic resonance imaging scan of the hand confirmed the roentogarphic findings [Figure 4]a and b. Hematological examinations revealed a total white cell count of 8,400/mm  ; the differential count was: 64% Neutrophils, 4% eosinophils, 24% lymphocytes and 8% monocytes. The erythrocyte sedimentation rate was 40 mm/1 st hour. A clinicoradiological diagnosis of osseous rhinosporidiosis was made. A biopsy was taken to confirm the diagnosis.
|Figure 1: Clinical photograph of hand (a) volar view (b) ulnar oblique view showing the swelling with ulceration|
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|Figure 2: Flaring of the nose and swelling of right lacrimal gland partly obscured under black band|
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|Figure 3: X-ray of the hand (anteroposterior view) showing osteolytic lesion involving the proximal phalanx and part of 5th metacarpal. There is no periosteal reaction|
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|Figure 4: MRI (Rt) hand (a) coronal T2W image of the hand showing the leison; (b) axial T1W image at the site of leison|
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|Figure 5: (a) Photomicrographs of cytology smear showing ruptured sporangium with dispersed endospores (H and E, ×400); (b) Photomicrographs of histopathological section showing the multiple sporangia with endospores (H and E, ×100)|
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Cytology showed sporangia with ruptured walls containing spores of varying sizes [Figure 5]a. Histopathological examination revealed multiple sporangia harboring numerous endospores, with the intervening stroma containing moderate infiltrates of lymphocytes, plasma cells and histiocytes. The diagnosis of rhinosporidiosis was established [Figure 5]b.
The patient was counselled for ray amputation and prognosis discussed at length. A racket shaped incision was made and wide margin excision was carried out. The patient had an uneventful recovery postoperatively and received tab Dapsone (100 mg bid) for 6 months. The patient had subsequently polypectomy and dacrocystectomy carried out for nasal and eye problems respectively. Biopsy reports of both were consistent with rhinosporidiosis. Patient was doing well without any signs of recurrence at one year followup [Figure 6].
|Figure 6: (a) Followup clinical photograph of hand showing ray amputation of little finger (b) X-ray of the hand (anteroposterior and oblique views) at one year followup showing that there are no signs of recurrence|
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| Discussion|| |
The disease rhinosporidiosis, caused by R. seeberi, has been described in humans and animals since 1892. The organism has never been isolated in vitro and its taxonomic position remains debatable with recent inclusion into a new class the Mesomycetozoea.  Rhinosporidiosis has been reported from about 70 countries with diverse geographical features although the highest incidence has been from South Asia namely Srilanka and India. In India, the distribution is largely subtropical with Chhattisgarh being an endemic area. 
It is more common in adult men and is possibly transmitted to humans by direct contact with spores through dust, infected clothing or fingers and bathing in stagnant waters.  Other modes of transmission include autoinoculation and hematogenous.  Though rhinosporidiosis is an infective disease; it is not infectious as no transmission has ever been documented of cross-infection between members of the same family or between animals and humans. 
Rhinosporidiosis frequently involves the nasopharynx (70%)  presenting as a painless, friable, polypoidal growth that are pink or purple-red and studded with minute white dots, which are sporangia containing the spores. Extra nasal involvement of the conjunctiva and lacrimal sac are common. Occasionally, rhinosporidiosis affects the lips, palate, uvula, maxillary antrum, epiglottis, larynx, trachea, bronchus, ear, scalp, vulva, vagina, penis, rectum and the skin.  Bony involvement is rare. Isolated osteolytic lesions have been reported involving the talus,  tibia, , femoral condyle, , hand and feet,  calcaneum,  and clavicle.  Dissemination to anatomically unrelated sites is mainly attributed to hematogenous spread.  Direct implantation by contaminated nasal secretions either during trauma or surgery can also implant the spores and cause secondary lesions. 
Differential diagnosis includes tumorous conditions such as giant cell tumor,  chondrosarcoma,  other sporoidal infections and granulomatous conditions, which have to be excluded by histopathology. The sporangium of R. seeberi is larger and has thicker walls (diameter of 50-100 mm vs. 20-80 mm) as compared with Coccidioides immitis. The size and number of endospores on sporangia of R. seeberi is also greater than those of C. immitis.  C. immitis is cultivable and stains with mucicarmine. Further, paucity of eosinophils or eosinophic precipitates (Splendorr-Hoeppli phenonmenon) contrasts rhinosporidiosis with opportunistic, mycelial or deep mycosis. ,
Surgical excision of the mass with wide margins by diathermy is the treatment of choice whenever possible to prevent recurrence. Dapsone has been the gold standard antimicrobial agent to prevent recurrence, but its effectiveness has been debatable owing to impenetrability of the sporangial wall.  In osseous lesions with pathological breech of the cortex and soft tissue involvement, salvage is difficult. Therefore, in the present case 5 th ray amputation was performed.
Osseous dissemination of rhinosporidiosis is rare and can mimic primary bone tumor and it requires a high degree of clinical suspicion with proper pathological support owing to paucity of radiological clues. Team of surgeons is often required as in present case with appropriate antimicrobial drugs to prevent recurrence.
| Acknowledgment|| |
The Author would like to thank the patient for providing consent to use his photograph in this article.
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347/J, Janta Colony, Gudiyari, Raipur 492 011, Chhattisgarh
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]