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Lacrimal sac rhinosporidiosis: case report and review of literature with a new grading system to optimize treatment

Rhinosporidiosis is a chronic granulomatous disease affecting the mucous membrane primarily and is caused by Rhinosporidium seeberi, an aquatic protistan parasite. The nose is the most common site of involvement and is seen in 83.3% cases, followed by ocular involvement in 11.2% cases and other sites like larynx, trachea and bronchus in 5.5% cases. In various oculosporidiosis case series, lacrimal drainage system involvement was seen to vary from 14.3% to 59.6% cases. Isolated lacrimal sac involvement in rhinosporidiosis was found in 45.8% (72 out of 157) cases of the lacrimal drainage system in a review of 31 studies. A variety of surgical procedures have been used to treat rhinosporidiosis of lacrimal sac like dacryocystorhinostomy, Dacryocystectomy, lateral rhinotomy and local lesion excision with a success rate varying from 28.5% to 92.3%. This wide variation in the success rate was due to the fact that a uniform surgical procedure was performed in all the cases of a particular series irrespective of the extent of disease. Grading the lacrimal sac rhinosporidiosis to decide the extent of surgical excision may help achieve better results. We present a grading system based on our own experience in a case of extensive rhinospodiosis of lacrimal sac and review of 31 studies published in the literature. A 24-year-old male from Nepal presented with the complaints of watering from his right eye of 13 years duration, swelling in the right medial canthal area with an extension to the inferior part of the orbit for 12 years and nasal blockage for 1.5 years. The patient had a history of previous intervention in which biopsy was taken from the nose and sent for histopathology that confirmed rhinosporidiosis. An extended intranasal endoscopic dacryocystectomy was done along with debridement and coblation of the lesion over the septum and nasopharynx. Intraoperatively a large rhinosporidiosis mass was seen filling the sac and was removed in toto along with the sac and nasolacrimal duct. Recurrence of a tiny lesion after 6 months in our case despite wide excision with the drilling of bony nasolacrimal duct and coblation, made us review the literature.


Introduction
Rhinosporidiosis is a chronic granulomatous disease caused by Rhinosporidium seeberi. It is a rare aquatic protistan parasite. 1 It commonly affects the mucous membrane but can also affect other structures including larynx, trachea, skin, genitalia, lungs and rectum. Isolated lacrimal sac rhinosporidiosis is very rare. 1 Kuriakose in 1963 coined the term oculosporidiosis for rhinosporidiosis of the eye. 2 A PubMed search of all the articles published in English on rhinosporidiosis of lacrimal sac and oculosporidiosis was performed. The relevant cross-referral  of all these articles was also reviewed. The first description of this parasite Rhinosporidium seeberi was given by Malbran from Buenos Aires, in 1892, who described it as a sporozoan parasite in nasal polypus followed by Guillermo seeber in 1986, who referred it as sporozoan belonging to subdivision coccidia. 3 It is now considered an aquatic protistan parasite belonging to the class mesomycetozoa. 1,4,5,6 Kirkpatric published the first case of lacrimal sac (LS) rhinosporidiosis in 1916. 1,7,8 Though oculosporidiosis is worldwide in distribution, it is relatively more prevalent in Southern India, Srilanka and Southeast Asia 9 and accounts for 15% of the cases of rhinosporidiosis. 4,6,10 The most common age group affected is 15-40 years with a predominance in males. 1,2,3,7,[10][11][12] In various oculosporidiosis case series, lacrimal drainage system involvement was seen to vary from 14.3% to 59.6 % cases. 2,4,[11][12][13][14][15][16] Frequency of isolated lacrimal sac involvement was seen in 45.8% (72/157) cases of the lacrimal drainage system in total of 31 studies reviewed.
History of treatment of lacrimal sac rhinosporidiosis dates back to 1949 when Rambo did incision and curetting of the LS region and packed it with sulphonamide without much relief. 2,17 Kuriakose quoted that recurrence is always the rule and often a LS fistula results which are very resistant to further treatment. 2 Excision of LS polyp has been reported to be unsatisfactory as complete removal is difficult due to excessive bleeding 11 and the recurrence is inevitable. 10 Advancement in technology with the introduction of high definition camera and endoscopes along with coblator have made it possible to perform meticulous excision of the lesion but the subepithelial extension cannot be eradicated completely. 7,11,12 10 and Dacryocystectomy (DCT) 3,11,20,21,22,23 with a success rate varying from 28.5% to 92.3%.
A review of published literature on LS rhinosporidiosis was conducted (Table 1) and it was observed that there were no set criteria used for choosing a particular procedure based on the extent of disease.
As a result, when a uniform procedure was applied to all the cases irrespective of the spread, those in grade 1 as per our grading, showed good results while those falling in grade 2&3 recurred. 10-12 A grading system has been devised to understand the disease extension, surgical plan and recurrence pattern ( Table 2).

Case report
A 24-year-old boy from Nepal presented with the complaints of watering from his right eye for 13 years, right medial canthal swelling extending to the inferior part of the orbit for 12 years and nasal blockage for 1.5 years. External local examination revealed a large diffuse boggy swelling in right infraorbital area extending superiorly along the nasal bridge reaching up-to-the eyebrow and inferiorly along the infraorbital margin. The overlying skin was inflamed, tender with no regurgitation through the punctum on gentle pressure over the swelling though, the nasolacrimal duct was blocked on syringing. There was a history of biopsy from the nose that showed rhinosporidiosis. On endoscopic examination, there was a granular, lobulated, reddish pink growth over the posterior part of the septum at the bony cartilage junction with oedema and inflammation in the middle meatus area and another 2 Â 2 mm lesion in the nasopharynx.
CT scan showed a hyperdense mass filling the right lacrimal sac area with subcutaneous extension and bony destruction. Right inferior turbinate was absent with just a stump seen with the air-filled nasal cavity on the right side. There was a bony erosion of the lacrimal fossa with soft tissue extension into the nose (Fig. 1A and B).
Written informed consent was obtained from the patient. Institutional review board approval was obtained. Intraoperatively a large rhinosporidiosis mass was seen in the LS lumen ( Fig. 1C and D) that was removed along with the walls of the sac and NLD followed by coblation of the base and surrounding area to avoid seeding of the spores. This was achieved by an extended endonasal endoscopic DCT involving complete extirpation of the sac with nasolacrimal duct (NLD) up-to its distal end with uncinectomy and clearance of ethmoids. Bone overlying the lacrimal fossa and the frontal process of the maxilla was drilled. The lateral wall of the nose in and around the bony NLD was removed, septal and nasopharyngeal lesions were debrided and coblated followed by irrigation with 5% povidone-iodine for 2 minutes. Histopathology revealed the sporangia of rhinosporidiosis at various stages of the life cycle. There was dense chronic inflammatory cell infiltrate mainly plasma cells and lymphocytes ( Fig. 1E and F).
Regular check endoscopies were done at 1 week, 2 week, 4 weeks. Though the patient remained asymptomatic for 6 months. At 6 months follow up, a check endoscopy showed a small granular lesion on the lateral nasal wall. The patient was happy and satisfied as he was asymptomatic. His swelling in the medial canthal area had completely disappeared with well-healed skin and no epiphora (Fig G and H). However, in view of the tiny recurrent lesion, he was advised endoscopic excision and electrocautery but he refused any further intervention.

Discussion
Rhinosporidiosis is presumably a waterborne disease and generally, occurs after swimming in stagnant freshwater ponds, lakes, or rivers, but is also suspected to occur from dust or air. 2 The natural hosts of the aquatic parasite are fish and amphibians. 22 Patient presents with swelling in the medial canthal area with or without epiphora. Blood stained discharge from the eye or nose may be present. The nose is the most common site of involvement in rhinosporidiosis and is seen in 83.3% cases, followed by ocular adnexa in 11.2% cases, while other sites are involved in 5.4% cases. 15 The other sites involved are the mucous membrane of larynx, trachea, bronchus and genitalia. 15 In various oculosporidiosis case series, lacrimal drainage system involvement was seen in 14.3% to 59.6% cases. 2,4,[11][12][13] The current study presents a review of 31 studies published in the literature ( Table 1). Most of the cases presented with swelling in the medial canthal area extending along the infraorbital margin. 1,2,3,7,[9][10][11]17,14,21,[24][25][26][27][28][29][30][31][32][33] The swelling was fluctuant 1,16 and boggy 7,9,33 and felt like a bag of worms. 24 It was painless in most of the studies, 1,2,[4][5][6][7]9,10,15,16,18,26,3 while some of them reported tenderness over the swelling. 21,22,31 An associated symptom of epistaxis or blood-stained discharge from nose or eye 2,10,13,22,33 with bloody tears 21,30 should raise a high index of suspicion of rhinosporidiosis, especially in endemic areas. The nature of discharge on regurgitation test varied from serosanguinous 17,24,30 to purulent 4,22,27 and slimy sprinkled with white granular particles 11 to reddish discharge with granular material. 15 Epiphora may be absent in some cases of nasolacrimal rhinosporidiosis 32,34 because the spread of infection is pericanalicular and perisaccal. 27 However, epiphora when present, may be continuous 3,9,11 intermittent 9,14,18 or blood stained. 10,21,23 NLD was fully patent in some studies, 11,20,24,32,34 partially patent in others 14 and completely blocked in the rest. 1,3,27,30,35 Our patient presented with the characteristic features of large boggy swelling in the medial canthal area with continuous epiphora. The skin over the swelling was inflamed with puckering and impending fistula thus, firm pressure was not applied as ulceration of skin over the swelling on pressure has been reported. 1 This indicates subcutaneous spread with skin infiltration classifying it into grade 3.
There are various schools of thoughts on the possible route of spread in lacrimal sac involvement in rhinosporidiosis. While some felt the sac is involved by upward extension of the mass from the nose 13,23,35 the others commented that the 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. 7 LS could get involved by the spread of infection from lacrimal canaliculi to the sac 3,27 through permeation along the subepithelial connective tissue 1 or via the subepithelial lymphatic channels. 7 In our case, the LS was the primary site of involvement with spread to nose through NLD as epiphora and swelling remained the only symptoms for 12 years and nasal symptoms appeared after more than a decade.
Isolated rhinosporidiosis of the LS is very rare 11,20 and it becomes difficult to suspect a LS swelling to be due to infestation by rhinosporidium 5 and thus imaging modalities are helpful. On computed tomography, rhinosporidiosis is commonly seen as a homogenously enhancing lobulated lesion in the inferior nasal cavity with adjacent bone erosion. 36 Bone involvement is seen as irregularity, rarefaction, partial or complete erosion of inferior turbinate, thinning of the medial maxillary wall and septal erosion. 36 Contrast enhanced CT has an important role in delineating the site and extent of disease as well as bone and NLD involvement. NLD extension was defined as the extension of soft tissue mass within the NLD, having similar attenuation as the mass in nasal cavity. 36 Characteristic findings of ''donut distribution'' i.e. contrast passes through NLD in a circumferential manner till it drained in the inferior meatus on computed tomographic dacryocystography (CT DCG) with normal sac wash out. 34 The swelling in the medial canthal area with a patent NLD on CT DCG in an endemic area should raise the suspicion of LS rhinosporidiosis. Dilated sac and dilated NLD were also seen in other studies. 3,12 Computed tomography helped us to judge the extension of the lesion in our case to the nose through a bony breach with the subcutaneous spread.
The aim of grading is to facilitate the planning of a particular surgical procedure e.g. DCR, modified DCR, DCT, Extended DCT and the need for multi-disciplinary approach wherever needed.
Grade 1: Lesion limited to LS lumen as a pedunculated or sessile mass or polyp ± NLD but no lesion in nose or eye. Grade 2: Lesion involving LS, NLD and nose or eye. Grade 3: Lesion involving LS, NLD and nose or eye and spread to skin ± Lacrimo-cutaneous fistula.
Grading of the cases in various studies has been listed ( Table 1). In the same series, cases were in different grades but a uniform surgical procedure was applied to all leading to recurrence in the advanced cases.
The proposed strategy for surgical management of LS rhinosporidiosis is that the cases in Grade 1 with just a pedunculated or sessile mass limited to LS alone can be treated with DCR or modified DCR may be preferred modality. 10 In cases of grade 1 with mass completely filling the sac & reaching NLD, it is preferred to plan DCT with complete NLD resection and cauterization of the base. In grade 2, DCT with en bloc resection of NLD with thorough excision and cauterization of nasal or eye lesion is recommended. It might require a multidisciplinary approach based on the extent of the lesion. In grade 3 extended DCT involving uncinectomy, ethmoidectomy, complete extirpation of the sac with en bloc removal of NLD along with fistulectomy in cases with presence of fistula should be done. Extensive drilling of the bone of lacrimal fossa and nasolacrimal duct i.e. frontal process of maxilla, lacrimal bone and lateral wall of the inferior meatus is done additionally. Bipolar cauterization of the periorbita if infiltrated and removal of orbital fat if the lesion involves the orbit should be done. The patient needs to be explained the need for rigorous follow-up and regular endoscopic checks.
Due to the availability of the high definition camera and best endoscopes, it has become possible to address the extensive lacrimal sac, Naso-orbital and even skull base lesions endoscopically through the nose. Some studies advocated, an endonasal endoscopic approach citing the risk of seeding of the spore at the incision site in external DCR. 18 We, however, feel that the route of surgical intervention does not make much difference provided the removal is complete. This understanding is based on the assumption that a multidisciplinary approach will always be used in handling lesions extending extension beyond the sac into the nose or eye.
Grading the disease as a part of preoperative assessment not only helps in deciding the extent of excision but also helps in predicting the outcome and explaining the prognosis to the patient.
These observations suggest that limited disease has a better prognosis while the involvement of more than one site, subcutaneous spread and lacrimo-cutaneous fistula have guarded prognosis. This could also explain recurrence in our case as it presented in grade three with associated nasal lesion with the subcutaneous spread.
Role of Dapsone (Diamino-diphenyl-sulfone) in reducing recurrence rate is attributed to an arrest of maturation of spores and an accentuated granulomatous response. 9,15 Topical application of 5% Povidone-iodine for 2 minutes was performed as it causes metabolic inactivation of endospores. 10 However, in view of the molecular characterization of the aetiological agent, further research to discover effective treatment options is urgently needed. 37

Declaration of Competing Interest
The authors declare no conflicts of interest.