1
Introduction
Rhinosporidiosis is a chronic granulomatous disease endemic to Sri Lanka, the Indian subcontinent and certain areas in Africa. Isolated cases have also been reported worldwide, but a number of these are felt to represent host migration from an endemic country. Infection most commonly affects mucus membranes of the nasal cavity, conjunctiva, nasopharynx, oral cavity, and external genitalia. Reports have also described cutaneous and upper airway involvement. Laryngotracheal involvement is more likely due to direct spillage of spores from the nasopharynx into the larynx during episodes of bleeding or surgical manipulation. Systemic involvement has been reported, but appears to be extremely rare . The life cycle and taxonomy of the causative agent, Rhinosporidium seeberi , remain incompletely understood and controversial . This is largely due to limitations in growing the organism in culture media. Medical therapy options have also been largely unsuccessful to date and the primary treatment modality involves surgical excision with medical therapy used selectively as an adjunct to surgery. Anatomic and surgical related considerations are largely based on the location and extent of the lesion. The primary goal of surgery is to clear all involved mucosal surfaces. A number of clinical challenges exist in the diagnosis and management of this disorder including the potential for delayed presentation, disease recurrence and local tissue destruction. We present a case of a patient who had both a delayed initial presentation and a subsequent delayed recurrence. The implications of the current case on treatment and surveillance for this lesion are discussed.
2
Case report
A 45 year old Burmese female presented with a history of endoscopic sinus surgery 4 years previously for symptoms of left sided nasal congestion, mucoid rhinorrhea and intermittent epistaxis. She had improved symptoms after the surgery. She reports subsequent recurrence of intermittent left sided epistaxis and rhinorrhea over the past 1 year. She denied other symptoms including facial pain, pressure, post-nasal drip, hyposmia, right sided symptoms, or systemic symptoms. Review of social history was notable for prior travel to Burma including swimming in natural waters in the past, most recently 8 years ago. On nasal endoscopy, a bulky friable, polypoid appearing lesion was noted along the left lateral nasal wall obstructing the middle meatus. Non-contrast CT scan of the paranasal sinuses demonstrated post-endoscopic sinus surgery changes and a 1.6 × 1.5 × 2.1 cm soft tissue lesion located on the left lateral nasal wall anterior to the maxillary antrostomy site ( Fig. 1 ). Office based biopsy and subsequent surgical pathology of the lesion revealed a submucosal lymphoplasmacytic infiltrate with sporangia containing numerous endospores consistent with rhinosporidiosis ( Fig. 2 ). The thick walled sporangia were highlighted by digested Periodic acid–Schiff (PAS) and Grocott’s methenamine silver (GMS) stains. Given the anatomic location of the lesion, endoscopic, transnasal surgery was performed. Wide surgical clearance of the involved tissue and a margin of healthy appearing tissue necessitated a medial maxillectomy encompassing resection of the left inferior turbinate and lateral nasal wall. This brought the maxillary sinus cavity into continuity with the nasal cavity. The superior-posterior aspect of the lesion abutted the inferior-medial orbit. The sinonasal mucosal surface was cleared surgically and cauterized. However, the inferior and medial orbital bony barriers were preserved to minimize the risk of intraorbital spread. Infectious disease consult was obtained and dapsone was recommended. However, the patient was unable to tolerate the medication secondary to blurred vision after three days of usage. The vision symptoms resolved after cessation of the medication. The patient has had serial office evaluations including nasal endoscopy and has had no evidence of recurrence at 9 month follow-up. Continued long term follow-up has been recommended.
2
Case report
A 45 year old Burmese female presented with a history of endoscopic sinus surgery 4 years previously for symptoms of left sided nasal congestion, mucoid rhinorrhea and intermittent epistaxis. She had improved symptoms after the surgery. She reports subsequent recurrence of intermittent left sided epistaxis and rhinorrhea over the past 1 year. She denied other symptoms including facial pain, pressure, post-nasal drip, hyposmia, right sided symptoms, or systemic symptoms. Review of social history was notable for prior travel to Burma including swimming in natural waters in the past, most recently 8 years ago. On nasal endoscopy, a bulky friable, polypoid appearing lesion was noted along the left lateral nasal wall obstructing the middle meatus. Non-contrast CT scan of the paranasal sinuses demonstrated post-endoscopic sinus surgery changes and a 1.6 × 1.5 × 2.1 cm soft tissue lesion located on the left lateral nasal wall anterior to the maxillary antrostomy site ( Fig. 1 ). Office based biopsy and subsequent surgical pathology of the lesion revealed a submucosal lymphoplasmacytic infiltrate with sporangia containing numerous endospores consistent with rhinosporidiosis ( Fig. 2 ). The thick walled sporangia were highlighted by digested Periodic acid–Schiff (PAS) and Grocott’s methenamine silver (GMS) stains. Given the anatomic location of the lesion, endoscopic, transnasal surgery was performed. Wide surgical clearance of the involved tissue and a margin of healthy appearing tissue necessitated a medial maxillectomy encompassing resection of the left inferior turbinate and lateral nasal wall. This brought the maxillary sinus cavity into continuity with the nasal cavity. The superior-posterior aspect of the lesion abutted the inferior-medial orbit. The sinonasal mucosal surface was cleared surgically and cauterized. However, the inferior and medial orbital bony barriers were preserved to minimize the risk of intraorbital spread. Infectious disease consult was obtained and dapsone was recommended. However, the patient was unable to tolerate the medication secondary to blurred vision after three days of usage. The vision symptoms resolved after cessation of the medication. The patient has had serial office evaluations including nasal endoscopy and has had no evidence of recurrence at 9 month follow-up. Continued long term follow-up has been recommended.
