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. 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.

Kuriakose in 1963 coined the term oculosporidiosis for rhinosporidiosis of the eye. 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. 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 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. Excision of LS polyp has been reported to be unsatisfactory as complete removal is difficult due to excessive bleeding and the recurrence is inevitable. 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]

Various authors have published their results using surgical procedures like dacryocystorhinostomy (DCR) involving endoscopic DCR[18], [19] external DCR, modified DCR 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.

Table 1

Review details of 31 studies on Lacrimal sac rhinosporidiosis published in literature.

SN	Author	Year	n	Presentation	ROPLAS	NLD/syringing	Epiphora	Site/spread	CT scan findings	Treatment	Recurrence	Follow up	Medical therapy	Pond bath	Location
1	Rajesh Raju and Sandeep19	2018	13	Swelling over LS area, Blood stained nasal discharge	Doughy swelling medial to medial canthus	Partial or complete block (Numbers NM)	Present in 2/13 cases	LS, NLD& Nose	Not done	Endoscopic DCR with NLD excision	1/13	16 months	Not given	Present	Kerela( South India)
2	Prabhu et al.36	2018	4	No details available (Radiological study)	NM	NM	NM	LS, NLD & nose	LS pyocele & NLD mucocele seen as a result of NLD block. Enhancing soft tissue mass in NLD and nose with same attenuation showing an upward extension.	Sac was excised, it was full of pink vascularised polypoidal growth	NM	NM	NM	NM	Tamilnadu (India)
3	Suneer and Sivasnkari33	2018	2	NM	NM	NM	NM	LS & NLD	Not done	Excision of lesion no details	NM	NM	NM	Present	Kanyakumari (South India)
4	Chakraborti et al.24	2017	1	Gradually increasing, soft, painless swelling of left lower eyelid	Serosanguinous	Patent	Nil	LS	Soft tissue enhancing mass near medial canthus of Left orbit. DCG showing diverticula	Via Ant orbitotomy through sub ciliary approach diverticula was removed leaving sac behind. It recurred with fistula with discharging spores	1st postop day	1 year	Iodine + Amoxyclav	Present	West Bengal (India)
5	Girish and Prathima27	2017	1	Diffuse nontender infra orbital swelling of the left eye	Mucopurulent discharge	NM	Intermittent	LS + NLD	Hyperdense lesion in subcutaneous plane in left infraorbital region	DCT with enbloc resection of NLD.	NM	NM	100 mg x3 months	Present	North Eastern part of India
6	Jamison et al.34	2016	1	Swelling at nasal aspect of left lower lid.	NM	Patent	Absent	LS, NLD & nose	CT-DCG- donut distribution i.e. contrast passed through NLD in circumferential manner till it drained in IM, with asymmetry in lateral wall of nose	Gelatinous lesion attached to superior wall of lacrimal sac extending into NLD. Details of excision not mentioned.	NM	5 months	NM	NM	Pakistan
7	Basu et al.20	2016	1	Pinkish swelling over left lower orbital area for 3 years with a deep T scar from the previous intervention	NM	Patent	Nil	LS & NLD	Enhancing soft tissue mass in lacrimal sac region extending to superior aspect of right maxillary antrum, disruption of medial wall of the left orbit and blurred adjacent fat planes.	DCT, excision of mass with sac	NIL	6 months	100 mg OD X6 days for 6 months	NM	Kolkata (India)
8	Mishra et al.1	2015	1	Soft, non-tender swelling at the medial canthus of left eye, 4cmx2cm in size	NM	Blocked	Present	LS + NLD	Hyperdense lesion with mean CT attenuation of 48 hounsfield unit lesion is extending to upper part of NLD	An elliptical incision was made over the medial canthus of left eye. Mass with sac removed. Silastic tube placed from punctum to nose	NIL	1 month	100 MG OD X6 months	NM	Bhuvneshwar (India)
9	Sah et al.14	2014	1	Left medial infraorbital diffuse nontender swelling	Reddish mucopurulent discharge	Partial patent	Intermittent	LS + NLD	Isodense lesion with mild enhancement in preseptal compartment	Multiple tiny vascularized growth seen. Sac was sutured and removed enbloc with NLD	Nil	2 Yrs	100 mg x3 months	NM	Terai (Nepal)
10	Nuruddin10	2014	18	Soft doughy swelling in LS area with epistaxis and blood-stained discharge from puncta.	NM	Blocked only in 4 cases, patent in rest 14	Blood stained discharge only in 4 patients	LS n = 16, lacrimo-cutaneous fistula n = 2	Not done	Modified DCR done. All sac content along with medial and lateral wall was excised, a small portion of the sac around common canaliculi was left, DCR tube placed	2 out of 18	1 year	Dapsone not used Povidone iodine for 2 minutes	12 out of 18	Bangladesh
11	Guru and Pradhan 23	2014	10	Blood- tinged discharge from eye	Blood tinged discharge	Blood tinged discharge on irrigation	Blood tinged discharge	LS and nose n = 7, NLD and nose n = 3	Not done	DCT, cauterisation of base. Debridement of mucous membrane of NLD	NM	NM	Dapsone	NM	Burla Sambalpur (India)
12	Mukherjee et al.22	2013	1	Recurrent painful swelling below right lower lid	Negative	NM	Patent	LS	small oval swelling in the medial canthal area, deviated nasal septum maxillary sinus polyp with concha bullosa	DCT with wide excision with cauterization	NM	Not mentioned	Povidone iodine + 100 mg Dapsone OD × 6mnths	NM	Chennai (India)
13	Belliveau et al.21	2012	1	Bloody tear with mild tender swelling in the medial canthal area of the left eye.	Mucopurulent discharge	Patent	Bloody tear Present	LS and NLD	Left Lacrimal sac mass, no bony destruction.	Open excision biopsy done, frozen section followed by external DCR	NIL	5 years	No Dapsone	NM	Canada (Migrated from Bangladesh)
14	Mithal et al.25	2012	13	Soft fluctuating swelling in medial canthus area	NM		Blood tinged discharge	LS + NLD	Not done	DCT with en bloc resection of growth in NLD was done, pink vascularised growth with finger like extension was seen.	1	6 months	not given	NM	South India
15	Pushkar et al.4	2012	1	Large painless ill defined, boggy swelling 5x5cm in size below medial canthus,	Mucopurulent discharge	Blocked with mucopurulent disease	Present	LS with extension into NLD	DCG showed a dilated LS with intraluminal filling defect. Soft tissue mass lesion within LS and NLD extending up to IM. Expanded NLD had displaced the IT medially.	Sac wall was incised, pink vascularised, polypoidal growth was seen, sac was sutured, DCT done. Extension of growth in nasopharynx removed en bloc with the sac.	NIL	6 months	dapsone given	NM	Delhi (patient's native place not mentioned (India)
16	Rogers et al.26	2012	1	Swelling left inner canthus.	Watery discharge from nose	NM	Present	LS	Dacryocystogram showed passage of dye but significant constriction of the nasolacrimal duct.	External DCR done	Recurrence noted	10yrs	Not given	NM	Sydney (Patient origin (Bangladesh)
17	Satya narayana3	2009	3	Non-compressible boggy swelling of the lacrimal sac	NM	NM	NM	LS with skin infiltration as was evident from skin ulceration on pressure with discharge of thick mucous secretion containing spores.	Not done	Excision cautery, details not known	not mentioned	NM	NM	NM	Madras (India)
18	Ghosh et al.28	2008	1	Fluctuant swelling in medial canthus area with epiphora and purulent discharge from eye.	Mucoid secretion from medial canthus	NM	Purulent discharge from the left eye	LS	Not done	DCT	NM	NM	NM	NM	Kolkata (India)
19	Varshney et al.35	2008	1	Right facial swelling, nasal obstruction and intermittent epiphora.	NM	Blocked	watering	LS, Nose& oropharynx	Cystic fluid air filled lacrimal sac with irregular filling defect, no bony erosion. LS diverticulum	Lateral rhinotomy with sac excision	Nil	3 months	NM	NM	Dehradun (India)
20	Ghorpade et al.29	2007	1	Gradually progressive, painful swelling under right eye for 8 months with scanty bloody nasal discharge.	NM	NM	NM	Small nasal mass with extension in LS & NLD	Not done	The mass was reached through an incision in the right naso-optic sulcus. A pinkish mass with whitish small dots on its surface was found to occupy the lacrimal sac and projecting into the lacrimal duct. It was extracted from the upper opening of the duct and the part in the inferior meatus was excised separately from the right nasal chamber followed by electrocautery.	Nil	8 months	Dapsone 100 mg daily orally, and has not had a recurrence since last 8 months.	Present	Bhilai (India)
21	Chowdhury RK et al.1	2007	3	Epistaxis	NM	NM	NM	LS n = 1, LS with subcutaneous spread n = 2	Not done	DCT done, pink vascularized finger like extension was seen in all the cases	Two out of three that had subcutaneous spread	NM	NM	Present	Western Orrisa(India)
22	Watve et al.9	2006	1	Nontender swelling Rt medial canthal area	Fluid	NM	Continuous epiphora purulent at times	LS	Not done	Endoscopic DCR done, a mass popped out of sac during surgery	NIL	NM	100 mg alternate day × 1 yr	NM	Western Maharashtra (India)
23	Nerurkar et al.18	2004	1	Diffuse, soft, non-tender Rt infraorbital swelling. diffuse	NM	Blocked	Intermittent	LS	Isodense lesion with mild enhancement in preseptal compartment. There was enhancement within extraconal compartment of right orbit medially suggestive of a lesion in NLD & inferior aspect of right orbit	Endoscopic DCR	Present	1 Week	50 mg daily	NM	Migrant from Orrisa (India)
24	Thakur et al.15	2002	3	Watering eye, no swelling, fistula in one case	Regurgitation produced a reddish discharge from the upper punctum	NM	watering present	LS n = 2, LS with fistula/previous history of DCR with silastic tube n = 1	Not done	DCT	Not mentioned	NM	NM	Present	Nepal
25	Shreshtha et al.11	1998	6	Soft, fluctuating swelling around LS with bleeding from eye & nose	Regurgitation was soft slimy and sprinkled with fine white granular particles	Patent	Purulent discharge	LS in 3, LS with spread in surrounding area n = 3	Not done	DCT done sac was opened, vascularised growth with finger like extension seen. Typical white spots on the surface of growth gave an appearance resembling that of bunches of fish eggs. Sac was sutured and removed.	1	2.5 years	Topical betadine	Present	Nepal (Lahan & Dharan)
26	Krishnan et al.30	1986	1	Swelling at the inner canthus of the right eye with epiphora and occasional blood- stained discharge	Serosanguinous discharge	Blocked	NM	LS	Dacrycystogrraphy showed diverticulum of LS	DCT (sac + diverticula)	NM	NM	NM	NM	South India (Pondicherry
27	Mukherjee et al.31	1982	48	Diffuse swelling over the sac n = 45 Widening of the nose bridge in n = 42, swelling extending under the lower lid n = 30, localised swelling over the sac n = 3	Nil	Blocked in 75% ( 36) cases, partial block in rest( 12 cases)	Nil	LS n = 42, LS & nose n = 6	Not done	DCT	NIL	NM	NM	NM	Raipur (MP India)
28	Suseela and Subramaniam13	1975	7	Epistaxis main symptom as the lesions involved nose,	Negative	Patent	NM	LS & nose	Dacryocystography showed dilated sac and duct	Excision biopsy	5 out of 7	NM	NM	Present	Kerala (India)
29	Jain and Sahai32	1974	1	Gradually increasing painless swelling underneath, the skin on rt lateral side of the bridge of nose	NM	patent	Absent	LS	Not done	DCT (sac with growth excised)	NIL	NM	NM	Agriculturist	Udaipur (India)
30	David and Sivaramasubrahmanyan7	1973	4	Swelling lower lid	Regurgitation of blood in nose	NM	NM	LS + Nose & Limbus	Not done	DCT	1	NM	NM	1 case had injury by fall + diving in tank	Tamilnadu (India)
31	Kuriakose et al.2	1963	6	Soft fluctuant, non-tender swelling of sac extending to lower lid with epistaxis.	Present	Patent	NM	NM	Not done	Details not mentioned, complete excision was difficult because of severe bleeding. Silver nitrate was applied after excision	Present	NM	Silver nitrate cautery	NM	Kerala (India)

NM: Not mentioned, LS: Lacrimal sac, NLD: Nasolacrimal duct, DCR: Dacryocystorhinostomy, DCT: Dacryocystectomy, ROPLAS: Regurgitation on Pressure Over Lacrimal Sac Area, n: Number of cases, IT: Inferior turbinate, Rt: Right, Lt: Left, IM: Inferior meatus, DCG- Dacryocystography.

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], [11], [12] A grading system has been devised to understand the disease extension, surgical plan and recurrence pattern (Table 2).

Table 2

Grading of Lacrimal sac rhinosporidiosis based on the extent of the disease.

S.N.	Author	Year	n	Site of involvement	Treatment	Recurrence
Grade 1
1	Suneer and Sivasnkari33	2018	1	LS + NLD	DCT	NM
2	Chakraborti et al.24	2017	1	LS diverticula	Via Ant orbitotomy through sub ciliary approach diverticula was removed leaving sac behind.	1/1 with fistula discharging spores
3	Girish and Prathima27	2017	1	Ls + NLD	DCT with en bloc resection of NLD	NM
4	Basu et al.20	2016	1	LS + NLD	DCT	Nil
5	Mishra1	25	1	LS + NLD	DCT with intubation from punctum to nose	Nil
6	Sah14	2014	1	LS + NLD	DCT with en bloc resection of growth in NLD	Nil
7	Nuruddin et al.10	2014	16	LS	Modified DCR	Nil
8	Mithal etal25	2012	13	LS + NLD	DCT with enbloc resection of NLD	1/13
9	Rogers et al.26	2012	1	LS	External DCR	1/1
10	Ghosh et al.28	2008	1	LS	DCT	Nil
11	Chowdhury et al.12	2007	1	LS	DCT	Nil
12	Watve et al.9	2006	1	LS	Endoscopic DCR	Nil
13	Nerurkar et al.18	2004	1	LS	Endoscopic DCR	1/1
14	Thakur et al.15	2002	1	LS	DCT	NM
15	Shrestha et al.11	1998	3	LS	DCT	Nil
17	Krishnan et al.30	1986	1	LS diverticulum	DCT	NM
18	Mukherjee et al.31	1982	42	LS	DCT	Nil
16	Jain and Sahai32	1974	1	LS	DCT	Nil
Grade 2
1	Rajesh Raju and Sandeep19	2018	13	LS + NLD + Nose	Endoscopic DCR with NLD excision	1/13
2	Prabhu et al.36	2018	1	LS + NLD + Nose	DCT	NM
3	Suneer and Sivasnkari33	2018	1	LS + NLD + Nose	DCT + en bloc resection of NLD	NM (states high chance of recurrence)
4	Jamison et al.34	2016	1	LS + NLD + Lateral wall of inferior meatus	Lesion excision en mass	NM
5	Guru and Pradhan 23	2014	10	LS & nose n = 7, NLD & nose n = 3	DCT	NM
6	Pushkar et al.4	2012	1	LS + NLD + Nose	DCT + enbloc resection of sac	Nil
7	Varshney et al.35	2007	1	LS + Nose + Oropharynx	Lateral rhinotomy + DCT	Nil
8	Shrestha et al.11	1998	3	LS + surrounding areas	DCT	1/3
9	Mukherjee et al.31	1982	6	LS + Nose	Nil	Nil
10	Suseela13	1975	7	LS + NLD + Nose	Excision biopsy	5/7
11	David and Sivaramasubrahmanyan et al.7	1973	4	LS + nose + limbus	DCT	1/4
Grade 3
1	Nuruddin et al.10	2014	2	LS + Nose + canaliculi + Lacrimo-cutaneous fistula	Modified DCR	2/2(100%)
2	Satyanarayana3	2009	3	LS with skin infiltration as was evident from skin ulceration that occurred on pressure over the swelling	NM	NM
3	Chowdhury et al.12	2007	2	LS + subcutaneous spread	DCT	2/2(100%)
4	Thakur et al.15	2002	1	LS with skin fistula	Excision of growth	NM

LS: Lacrimal sac, NLD: Nasolacrimal duct, DCT: Dacryocystectomy, DCR: Dacryocystorhinostomy, NM: Not mentioned, n: Number of patients.

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 × 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).

Fig. 1

A and B: Computed tomography scan of orbit & paranasal sinuses, axial & coronal view revealed soft tissue mass in the lacrimal sac fossa with a bony breach (arrow head) and extension into the nose. C and D: Endoscopic view of the right nasal cavity showing rhinosporidiosis mass in the lacrimal sac with probe insitu. E and F: Microphotograph showing epithelium of lacrimal sac with multiple sporangia and sporocysts of rhinosporidiosis with chronic inflammation. G and H: Preoperative and postoperative clinical photograph of the patient.

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. The natural hosts of the aquatic parasite are fish and amphibians.

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. The other sites involved are the mucous membrane of larynx, trachea, bronchus and genitalia. 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], [17], [14], [21], [9], [10], [11], [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. It was painless in most of the studies,[1], [2], [9], [10], [15], [16], [18], [26], [34], [5], [6], [7] 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 to reddish discharge with granular material.

Epiphora may be absent in some cases of nasolacrimal rhinosporidiosis[32], [34] because the spread of infection is peri-canalicular and perisaccal. 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 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. 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.

LS could get involved by the spread of infection from lacrimal canaliculi to the sac[3], [27] through permeation along the subepithelial connective tissue or via the subepithelial lymphatic channels. 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 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. Bone involvement is seen as irregularity, rarefaction, partial or complete erosion of inferior turbinate, thinning of the medial maxillary wall and septal erosion.

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.

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.

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. 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. 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.

However, in view of the molecular characterization of the aetiological agent, further research to discover effective treatment options is urgently needed.

Declaration of Competing Interest

The authors declare no conflicts of interest.