Lacrimal sac rhinosporidiosis: A case report

Case Reports Lacrimal Sac Rhinosporidiosis: A Case Report Nupur K. Nerurkar, MS (ENT), DORL, Renuka A. Bradoo, MS (ENT), DORL, Anagha A. Joshi, MS (ENT), DNB, DORL, Jayesh Shah, MBBS, and Shantanu Tandon, MBBS Rhinosporidiosis is a disease caused by Rhinosporidium seeberi. It usually affects the nasal mucosa and rarely the conjunctiva, lacrimal sac, tonsils, and skin. We present a case study of an isolated lacrimal sac rhinosporidiosis in an 8-year-old girl who was a migrant from Orissa, an Eastern coastal state of India. The mode of presentation and management of this case with a review of literature is discussed in brief. (Am J Otolaryngol 2004;25:423-425. © 2004 Elsevier Inc. All rights reserved.)

CASE REPORT An 8-year-old female child presented with a diffuse right infraorbital swelling that had developed 10 days after an accidental fall on a muddy surface. The swelling had been present for a period of 8 months and had remained unchanged during this time. The patient also complained of intermittent epiphora. External examination of the right eye revealed a diffuse, soft, and nontender swelling in the infraorbital region. A pea-sized, firm nodule was also palpable at the medial canthus. The overlying skin was normal. The vision and the extraocular movements were normal. There was no patency of the right nasolacrimal passage at the time of presentation, which was ascertained by regurgitation of fluid through the upper canaliculus on performing sac syringing. The left eye and ear, nose, and throat examination was unremarkable.

From the Department of ENT, Lokmanya-Tilak Municipal General Hospital and Lokmanya Tilak Municipal Medical College, Sion, Mumbai, India. Address correspondence to Nupur K. Nerurkar, MS (ENT), DORL, D 603 Simla House, Napean Sea Road, Mumbai 400 026, India. E-mail: nupurkapoor@yahoo. com © 2004 Elsevier Inc. All rights reserved. 0196-0709/$ - see front matter doi:10.1016/j.amjoto.2004.04.012

A high-resolution computed tomography scan of the paranasal sinuses revealed an isodense lesion with mild enhancement within the preseptal compartment along the inferior aspect of the right orbit. There was also a subcentimeter-sized small component with peripheral enhancement within the extraconal compartment of the right orbit medially (Fig 1), which was suggestive of a lesion in the right nasolacrimal sac. An endoscopic dacrocystorhinostomy (DCR) with biopsy was performed. An incision was made on the medial wall of the lacrimal sac, after which white caseous material exuded out giving an impression of tuberculosis of the lacrimal sac. All material and granulations within the sac were sent for histopathology, bacterial, and fungal studies. Patency of the nasolacrimal system was confirmed by intraoperative sac syringing. The histopathology report was suggestive of rhinosporidiosis (Fig 2), and the patient was then started on diaminodiphenylsulfone (Dapsone; Burroughs Wellcome, Brentford, United Kingdom) on a daily dosage of 50 mg. A check nasal endoscopy 1 week postoperatively revealed a well-defined mass with a mulberry-like appearance on the lateral wall of the lacrimal sac, which was excised completely. The repeat histopathology report reconfirmed rhinosporidiosis. At the time of sac syringing, care was taken to prevent fluid from spilling into the nasopharynx so as to avoid seeding of Rhinosporidium seeberi. The exter-

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nal infraorbital swelling has subsided considerably. The lacrimal sac has remained patent and disease free 1 year postoperatively. DISCUSSION Rhinosporidiosis is presumably a waterborne disease, and 88% of cases are reported from India and Sri Lanka.1 It is a disease caused by Rhinosporidium seeberi placed under the family Oplidiaceae, order Chytridiales of Phycomycetes.2 It usually involves the nasal mucosa and rarely the conjunctiva, lacrimal sac, tonsils, and skin. Primary ocular rhinosporidiosis occurs in 10% of cases.3 Of these, 7.32% were reported to involve the lacrimal sac in a study performed by Shreshta et al.4 The mode of transmission is controversial, but the spread probably occurs through water and dust. Trauma is a necessary prerequisite for infection5 because spores are transferred through traumatized mucosal surfaces. There seems to be a relationship between the disease and agriculture, suggesting that rhinosporidiosis in all probability inhabits the soil.6 In our case, the infection was probably caused by a fall on a muddy surface contaminated by Rhinosporidium, which caused the spores to be transferred from the soil to the lacrimal passage.

Fig 1. An axial computed tomography scan of paranasal sinuses with right orbit showing isodense lesion within the inferior aspect of the preseptal compartment and a small component with peripheral enhancement within the extraconal compartment medially.

Fig 2. Histopathology of the lesion at 50ⴛ magnification with hematoxylin and eosin staining.

The lacrimal sac is usually affected by infection ascending from the nose. The infection then has a tendency to spread diffusely underneath the skin.7 However, in this patient, there was isolated involvement of the nasolacrimal sac without any nasal or conjunctival components. Epiphora is unusual in nasolacrimal rhinosporidiosis because the spread of infection is pericanalicular and perisaccular.7 However, our patient had complaints of intermittent epiphora with a blocked nasolacrimal apparatus at the time of presentation. Although dacryocystectomy has been described in the literature4 for lacrimal rhinosporidiosis, this case could be managed by an endoscopic DCR with excisional biopsy of the lesion. The role of surgery in this case was to provide a histopathological diagnosis and achieve nasolacrimal patency, thus relieving the patient’s epiphora. The advantages of an endoscopic DCR as opposed to an external one are avoidance of seeding at external incision site, preservation of the normal physiological action of the nasolacrimal apparatus, and early detection of any recurrence on regular check endoscopies. It is essential to administer Dapsone postoperatively for a year to tackle the local subepithelial and subcutaneous spread and to prevent recurrence.8 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.9

LACRIMAL SAC RHINOSPORIDIOSIS

CONCLUSION An endoscopic approach to lacrimal sac pathology is preferable to an external approach to achieve both a biopsy and sac patency. This avoids an external scar and seeding of spores in the skin during surgery. Regular check endoscopies can monitor any recurrence of the disease. Dapsone is required postoperatively to prevent recurrence caused by subepithelial and subcutaneous tissue spread. REFERENCES 1. Karunaratne WAE: Rhinosporidiosis in Man. Athlone, University of London, 1964

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2. Ashworth JH. Transactions of the Royal Society of Tropical Medicine and Hygiene, Edinburgh. 53:301, 1923 3. Mukherhee PK: Rhinosporidiosis (oculosporidiosis), in Fraufelder FT, Roy FH (eds): Current Ocular therapy. Philadelphia, PA, Saunders, 1995, pp 77-79 4. Shrestha SP, Hennig A, Parija SC: Prevalence of rhinosporidiosis of the eye and its adnexa in Nepal. Am J Trop Med Hygiene 59:231-234, 1998 5. Blitzer A, Lawson W: Fungal infections of the nose and paranasal sinuses. Otolaryngol Clin North Am 26: 1007-1035, 1993 6. Ashworth JH, Logan Turner A: A case of rhinosporidiosis. J Laryngol Otol 38:285-299, 1923 7. Kameshwaran S: ENT Diseases in a Tropical Environment (ed 2). Chennai, MERF Pvt Ltd, 1999 8. Nair K: Clinical trial of diaminodiphenylsulphone (DDS) in nasal and nasopharyngeal rhinosporidiosis. Laryngoscope 89:291-295, 1979 9. Job A, Venkateswaran S, Mathan M, et al: Medical therapy of rhinosporidiosis with Dapsone. J Laryngol Otol 107:809-812, 1993