Abstract
Rhinosporidiosis is a chronic granulomatous disease involving the mucous membranes of nose and nasopharynx caused by Rhinosporidium seeberi. Rhinosporidiosis involving the eye is known as Oculosporidiosis. Usually affected patients present with nasal obstruction with an associated mass, epistaxis and watery discharge. In our case, there was involvement of the nasolacrimal duct which resulted in bloody discharge from the punctum.
Keywords: Rhinosporidiosis, Epistaxis, Nasolacrimal duct, Lacrimal sac, Dapsone
Introduction
Rhinosporidiosis caused by Rhinosporidium seeberi is a chronic granulomatous disease involving the nasal mucous membranes and ocular conjunctivae of humans and animals, producing slowly growing masses that degenerate into polyps. On rare occasions it presents as disseminated disease with subcutaneous and pulmonary involvement. Extranasal lesions are believed to be secondary to direct infection or finger-borne autoinoculation. Isolated nasolacrimal duct involvement is very rare. The mainstay of treatment is surgical excision. The authors present a case of isolated nasolacrimal duct rhinosporidiosis with its surgical management.
Case Report
This 22 year old male presented with complaints of profuse unilateral bleeding from left nostril which was sudden in onset associated with bleeding from left eye punctum. There was no history of trauma/bleeding disorders. Local examination showed active bleeding from left nostril; Post nasal bleed present; Active bleeding from left eye punctum. Vision and the extraocular movements were normal. Diagnostic Nasal Endoscopy showed Soft, fleshy, friable mass visualised in the left inferior meatus and was seen encroaching valve of Haasner (Fig. 1). There was partial patency of the left nasolacrimal duct which was ascertained by some regurgitation of fluid on performing sac syringing. CECT PNS showed heterogeneous faint hypo-hyperdensity seen in left inferior meatus extending upto the intra osseous and membranous parts of Left nasolacrimal duct. On intravenous contrast administration, focal linear intense enhancing area seen at the above mentioned regions (Fig. 2). Differential diagnosis of hemangioma, adenocarcinoma, inverted papilloma and invasive fungal sinusitis were made. Following this, biopsy was taken from the lesion at the site of left inferior meatus and sent for examination. Multiple large thick walled sporangia with endospores within those sporangia were visualized. Surrounding those sporangia were areas of lymphoplasmocytic infiltration. Final diagnosis of Rhinosporidiosis was made. After obtaining informed and written consent from patient, Endoscopic Dacrocystorhinostomy with Denkers procedure was done. Inferior turbinate was excised. The entire nasolacrimal duct with the disease was removed in toto and sent for histopathological which confirmed Lacrimo Nasal Rhinosporidiosis.
Fig. 1.
Diagnostic nasal endoscopy showing a fleshy mass in the inferior meatus
Fig. 2.
CECT showing heterogeneous faint hypo-hyperdensity seen in the left inferior meatus extending upto the intra osseous and membranous parts of left nasolacrimal duct
Discussion
Rhinosporidiosis caused by Rhinosporidium seeberi is a chronic granulomatous disease predominately affecting the nose and nasopharynx. In his 1921 doctoral thesis, Argentinean Guillermo Seeber first described the organism isolated from a nasal polyp in an agricultural worker [1]. Ashworth coined the term Rhinosporidium seeberi in 1923. The taxonomical classification of Rhinosporidium seeberi is controversial. The organism was traditionally thought to be fungal but recent molecular studies suggest it to be either a cyanobacterium (bacteria capable of photosynthesis) or a protozoan (parasite that infests fish and amphibians). The disease is most common in southern India and Sri Lanka, but sporadic cases have been reported from East Africa, Central and South America, South East Asia and other parts of the world. The natural habitat of the organism is unknown, but it is believed, based on the epidemiologic data, that stagnant pools of fresh water are an important source. The disease is most prevalent in rural districts, among persons bathing in public ponds or working in stagnant water, such as rice fields. Rhinosporidiosis is most common in persons in the age range of 15–40; males are more commonly affected than females.
Patients commonly present with complaint of nasal obstruction with an associated mass, epistaxis, watery discharge that becomes purulent with secondary infection, or mass projecting from the nose [1, 2]. Nasal examination shows a polypoid, obstructive, fleshy, hemorrhagic (strawberry) mass and hypertrophic mucosa. The characteristic “strawberry” appearance is secondary to increased vascularity punctuated by gray or yellow spots which represent bulging sporangia through the attenuated epithelium [1, 3]. Common points of attachment of the polypoid lesions (in descending order) are the nasal septum, nasal floor, anterior end of the inferior turbinate, middle turbinate, and skin at the junction of the nasal vestibule and upper lip.
About 15% of cases of rhinosporidiosis are ocular in location, seen principally in the bulbar and palpebral conjunctiva [4]. Rhinosporidiosis of the lacrimal sac (LS) and naso-lacrimal duct (NLD) is very rare. Some authorities regard the lacrimal sac and lacrimal duct as the primary site of infection, followed by distal spread into the nasal passages, via the naso-lacrimal duct. This view is controversial, as nasal rhinosporidiosis occurs as a primary lesion, in the absence of lacrimal sac/ nasolacrimal duct involvement. [5, 6]
Isolated lacrimal duct Rhinosporidiosisis very rare. Rhinosporidiosis of the eye is known as oculosporidiosis. In various oculosporidiosis case series, lacrimal drainage system involvement was seen to vary from 14.3% to 59.6% cases. [7]
All reported cases of Oculosporidiosis presented with painful or painless, soft, fluctuant swelling in the medial canthus area due to the involvement of lacrimal sac. Whereas in our case, there was no swelling in the medial canthal area [7].
Epiphora is unusual in nasolacrimal rhinosporidiosis because the spread of infection is pericanalicular and perisaccular. However, our patient had complaints of bleeding from left punctum with blocked nasolacrimal apparatus.
There are various schools of thoughts on the possible route of spread in lacrimal system involvement in rhinosporidiosis. Few authors reported that lacrimal sac could get involved by the spread of infection from lacrimal canaliculi to the sac [8, 9] through permeation along the subepithelial connective tissue [10] or via the subepithelial lymphatic channels [11]. A study by David SS et al. [11] reported that LS cannot be involved through NLD because the lacrimal folds act as a valve to prevent the secretion of the nose from being driven up into the duct. In our case, a fleshy mass was visualised in the left inferior meatus and was seen encroaching valve of Haasner and involving the nasolacrimal duct. This supports the theory of retrograde spread of Rhinosporidiosis.
Management of Ocular rhinosporidiosis is primarily surgical excision, which can be done using an open or nasal endoscopic approach. The advantages of the nasal endoscopic approach are avoidance of an unsightly facial scar, reduces chances of seeding at incision site, and recurrence can be detected early using nasal endoscopy during follow-up. In our case, Endoscopic Dacrocystorhinostomy with Denkers procedure was done. Inferior turbinate was excised. Medial wall of maxilla was drilled to expose nasolacrimal duct and with further dissection, lacrimal bone was removed to expose the lacrimal sac. Intra operatively, the lacrimal sac was free of disease. DCR was done and the duct-sac junction was ligated. The entire nasolacrimal duct with the disease was removed in toto and sent for histopathological examination which confirmed Lacrimo Nasal Rhinosporidiosis. Patient was treated with Dapsone after biopsy result to tackle the local subepithelial and subcutaneous spread and to prevent recurrence. The role of Dapsone in reducing the rate of postoperative recurrence is attributed to an arrest of maturation of the spores and an accentuated granulomatous response with fibrosis after Dapsone therapy.
Conclusion
After extensive search through available medical literature published world-wide, to the best of our knowledge, we conclude that this is the first reported case of Rhinosporidiosis with isolated involvement of nasolacrimal duct and without involvement of lacrimal sac, the common canaliculi or conjunctiva. Thereby, we support the theory that retrograde spread from the nasal cavity is the possible route of spread of ocular rhinosporidiosis.
Funding
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Compliance with Ethical Standards
Conflict of interest
The authors declare that they have no conflict of interest.
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Footnotes
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