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Histopathologically confirmed ocular rhinosporidiosis in two Canadians
Histopathologically confirmed ocular rhinosporidiosis in two Canadians Mona Harissi-Dagher,* MD; Nicole Robillard,* MD; Christine Corriveau,* MD; Michèle Mabon,* MD; Guy S. Allaire,† MD ABSTRACT • RÉSUMÉ Case report: In India and Southeast Asia, rhinosporidiosis is a common infectious disease, but it has rarely been reported in western countries. Infrequently, isolated ocular rhinosporidial infections have been reported, but to our knowledge, there are no reported cases in Canada.Two cases of rhinosporidiosis have been recently diagnosed and managed at our university-based hospital. Comments: Rhinosporidiosis presents with certain characteristic clinical features; however, the diagnosis is confirmed histopathologically. The presence of typical sporangia and spores in a fibrovascular stroma infiltrated by acute and chronic inflammatory cells including granulomas is diagnostic. Surgical excision is the treatment of choice, and recurrence is possible but rare. Observation : La rhinosporidiose est une maladie infectieuse courante en Inde et en Asie du Sud-est, mais rarement rapportée dans les pays occidentaux. Il est peu fréquent qu’on fasse état d’infections oculaires de cette nature et, à notre connaissance, on n’en a signalé aucun cas au Canada. Deux cas de rhinosporidiose ont été diagnostiqués et traités à notre hôpital universitaire. Commentaires : La rhinosporidiose présente certaines caractéristique cliniques particulières; le diagnostic est cependant confirmé par un examen histopathologique. La présence de sporangia et de spores typiques dans un stroma fibrovasculaire infiltré de cellules inflammatoires aiguës et chroniques comprenant des granulomes en permet le diagnostic. L’excision chirurgicale est le traitement de préférence; la récurrence est possible mais rare.
From the departments of *Ophthalmology and †Pathology, Le Centre hospitalier de l’Université de Montréal (CHUM), Montreal, Que. Originally received Mar. 17, 2005 Accepted for publication Dec. 5, 2005 Correspondence to: Dr. Mona Harissi-Dagher, Department of Ophthalmology, CHUM–Notre Dame Hospital, 1560 Sherbrooke East, Montreal QC H2L 4M1; fax (514) 412-7663; [email protected] This article has been peer-reviewed. Can J Ophthalmol 2006;41:226–9
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Rhinosporidiosis—Harissi-Dagher et al
I
n India and Southeast Asia, rhinosporidiosis is a common infectious disease, but it has rarely been reported in western countries and thus may pose a diagnostic challenge.1–3 The causative agent, Rhinosporidium seeberi, long considered a fungus, has now been definitively classified using molecular biological tools in a new clade, the Mesomycetozoea.4 The main ocular manifestation of rhinosporidiosis is a conjunctival or eyelid infection.1–3 Infrequently, isolated ocular rhinosporidial infections have been reported in other parts of the world, but to our knowledge, there are no reported cases in
Rhinosporidiosis—Harissi-Dagher et al
Canada. We have recently diagnosed and managed 2 cases of rhinosporidiosis presenting 3 months apart to our university-based ophthalmology clinic in Montreal. CASE
REPORT
1
A 48-year-old Canadian man presented with a right lower eyelid mass. The lesion, first noticed 2 months previously, began as small white spots on the conjunctiva and developed into a moderately large and irritating gelatinous mass. The history was negative for any ocular surgery or trauma. The patient was in good general health except for a remote nasal septoplasty. He was on chloroquine for malaria prophylaxis for a recent trip to Vietnam, 1 month previously, his first overseas trip. As a stoneworker, he handled ornamental stones derived from many regions of the world, including Asia. He complained of repeatedly rubbing his eyes at work because of a dusty environment. Examination of the patient revealed a visual acuity of 6/6 OU. The bulbar conjunctival lesion caused irritation and a mass effect, preventing proper apposition of the eyelids (Fig. 1). On the basis of the clinical appearance of the lesion, diagnoses of pyogenic granuloma or papilloma were suggested. Surgical excision of the lesion was performed. Histopathologic examination of the conjunctival biopsy showed, in the stroma, multiple sporangia at various stages of maturation containing numerous endospores. The sporangia were often surrounded by epithelioid histiocytes. In the intervening stroma, there was a heavy lymphoplasmacytic infiltrate with occasional neutrophils and eosinophils. Because of the unusual aspect of the lesion, the case was sent in consultation to Dr Ahmed Hidayat (Armed Forces Institute of Pathology (AFIP), Washington, DC). The final diagnosis was conjunctival rhinosporidiosis. (Fig. 2) An infectious disease consultation confirmed the localized nature of the infection and systemic medication was deemed unnecessary. The patient was followed up in the ophthalmology clinic 2 months later with no recurrence noted at that time. CASE
REPORT
2
A 51-year-old Canadian man presented with a persistent right upper eyelid mass. The mass had progressively increased in volume over the past 3 months and had become irritating. There was no history of
Fig. 1—Ocular rhinosporidiosis of the bulbar conjunctiva. Note the vascular appearance as well as the tiny white spots corresponding to sporangia on the surface of this polypoid lesion.
Fig. 2—Photomicrograph. Sporangia of different sizes in the conjunctival stroma, confirming the diagnosis of rhinosporidiosis. Sporangia range in diameter from 200 to 300 μm (haematoxylin–eosin; original magnification ×480).
trauma or foreign body to the eye. The patient had never travelled outside Canada. The patient was known for Bean syndrome, a disease consisting of multiple hemangiomas, for which he had undergone numerous surgeries. He also was positive for hepatitis C virus (HCV) and human immunodeficiency virus (HIV), with a CD4 count of 342 cells/μL of blood and a viral load of 4800. He was not receiving antiretroviral therapy because of intolerance to these drugs.
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His ocular exam revealed a corrected visual acuity of 6/7.5 OD and 6/6 OS. A right upper eyelid lesion involving the palpebral conjunctiva in proximity to the superior punctum was noted. The lesion was a vascular polypoid mass with tiny white spots covering its surface. Right macular pigmentary changes were attributed to a previous history of central serous chorioretinopathy. Considering the diagnosis of Bean syndrome, a palpebral hemangioma was suspected. Surgical excision was carried out under local anesthesia. The conjunctival biopsy showed large sporangia containing numerous endospores. Some of the sporangia showed a ruptured capsule with extrusion of endospores beneath the surface epithelium. There were numerous neutrophils forming microabscesses, which surrounded the sporangia. A diagnosis of conjunctival rhinosporidiosis was made. The lesion recurred one month later and surgical excision with cautery was undertaken (Figs. 3 and 4). Follow-up is pending.
Fig. 3—Infection of the upper eyelid and palpebral conjunctiva by Rhinosporidium seeberi. Elevated vascularized nodule adjacent to upper punctum.
COMMENTS
Rhinosporidiosis is endemic in south Asia, notably India and Sri Lanka. Migration of people who acquired rhinosporidiosis in their native countries has resulted in the increasing occurrence of this disease in the west. To our knowledge, no cases of ocular rhinosporidiosis have yet been reported from Canada. There have been cases reported in the United States, mostly from the southern states with one case in western New York state.1,5 This latter occurrence raised the question of the organism’s existence in temperate North American climates. Both our patients were Canadian, had always resided in Quebec, had no history of travel to endemic areas before the development of the lesions, and had no contact with infected patients. Patient 1, a good historian, had already noted the growth on his inferior conjunctiva before his trip to Vietnam. Rhinosporidiosis is an infective disease where the pathogen is always found in the tissue lesion. There is no evidence, however, that this disease is infectious or contagious, since no transmission has been documented.1 The majority of cases are sporadic. The presumed mode of transmission is from the natural aquatic habitat of Rhinosporidium seeberi through traumatized epithelium, most commonly in nasal sites. The occurrence of rhinosporidiosis in river-sand workers in India and in Sri Lanka is particularly relevant to this mode of infection, through abrasions
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Fig. 4—Photomicrograph. A large sporangium containing endospores extends into the epithelium and is about to rupture (haematoxylin–eosin; original magnification ×1200).
caused by sand particles with the pathogen in ground water. Isolated granulomas can be produced by this microorganism and by infected foreign matter deposited in the conjunctiva during injuries sustained in an agricultural setting.1 Our first patient, because of the nature of his occupation as a stoneworker, was at risk of contracting this infection. Case 2, however, notwithstanding his immune deficiency as a result of AIDS, did not have a typical history for acquiring this infection. In fact, rhinosporidiosis has not been related to immunodeficiency in the literature. However, this case represents the second instance of
Rhinosporidiosis—Harissi-Dagher et al
ocular rhinosporidiosis occurring in an HIV-positive patient in North America, after a first report from Texas.6 The majority of cases occur in upper respiratory sites, notably the anterior nares, nasal cavity, nasopharynx, larynx, soft palate, and buccal cavity. About 15% of cases present in an ocular location, either the bulbar or palpebral conjunctiva, the lacrimal sac, or nasolacrimal duct. Scleral melting has been documented as a complication of conjunctival infection, and it may even be a finding at initial presentation.7,8 The disease can also manifest itself as a cutaneous granuloma.9 Systemic disease is rare. In both our patients, the disease was localized to the ocular surface, the first case affecting the bulbar conjunctiva and the second the upper eyelid and palpebral conjunctiva. Clinically, ocular rhinosporidiosis presents as a friable, richly vascularized polyp that may be pedunculated or sessile. The surface is covered with tiny white spots consistent with underlying mature sporangia beneath the epithelium.1 The gross appearance, though distinctive, is not diagnostic. In case 1, an initial diagnosis of conjunctival papilloma or pyogenic granuloma was made because of the wellvascularized and papillomatous aspect of the lesion. The lesion in case 2 was mistaken for a hemangioma linked to Bean syndrome that he had had since childhood. Diagnosis was confirmed in both cases by histopathology, which showed the typical sporangia and spores. The organism, in all stages of its life cycle, is present in a fibrovascular stroma infiltrated by acute and chronic inflammatory cells including granulomas. Relatively large sacs or sporangia (200–300 μm diameter) may be empty or filled with spores (6–7 μm diameter).1,3 One characteristic phenomenon found on histopathology is the transepithelial elimination of rhinosporidial sporangia at the surface of the mucosa, a dispersal mechanism for rhinosporidial endospores (Fig. 4). Definitive diagnosis requires histopathologic confirmation. Corroboration is made by identification of the pathogen on biopsied specimens rather than by stromal and cellular response. Meticulous excision is the treatment of choice, although rare cases of spontaneous regression have
been reported.1–3 The potential for recurrence is due to spillage of endospores on adjacent mucosa. Recurrences are rare and can be prevented by cauterization of the base of the lesion or alternatively by cryotherapy.2,3 Medical therapy is still controversial since cultures of R. seeberi have been unsuccessful in all artificial media thus making sensitivity determination impossible. Dapsone is the only drug that has had some success in treating R. seeberi. It acts by arresting maturation of sporangia and accelerating their degenerative changes.9 In summary, Rhinosporidiosis is an uncommon fungal infection in the western hemisphere. It presents with certain characteristic clinical features, but the diagnosis is confirmed histopathologically. We have presented 2 cases of ocular rhinosporidiosis, both acquired in Canada. This entity should be included in the differential diagnosis of well-vascularized polypoid conjunctival lesions. REFERENCES 1. Arseculeratne SN. Recent advances in rhinosporidiosis and rhinosporidium seeberi. Indian J of Med Micro 2002;20: 119–31. 2. Arora R, Ramachandran V, Raina U, Mehta DK. Oculosporidiosis in Northern India. Indian Pediatr 2001;38: 540–3. 3. Shrestha SP, Hennig A, Parija SC. Prevalence of rhinosporidiosis of the eye and its adnexa in Nepal. Am J Trop Med Hyg 1998;59:231–4. 4. Arseculeratne SN. Rhinosporidiosis: what is the cause? Curr Opin Infect Dis 2005;18:113–8. 5. Reidy JJ, Sudesh S, Klafter AB, Olivia C. Infection of the conjunctiva by Rhinosporidium Seeberi. Surv Ophthalmol 1997; 41:409–13. 6. Stricker JB, Hurley DL, Slovacek KJ, McCombs WB 3rd. An unusual infection in a human immunodeficiency viruspositive man. Arch Pathol Lab Med 1999;123:1121–2. 7. De Doncker RM, de Keizer RJ, Oosterhuis JA, Maes A. Scleral melting in a patient with conjunctival rhinosporidiosis. Br J Ophthalmol 1990;74:635–7. 8. Sunderraj P. Scleral melting and conjunctival rhinosporidiosis. Br J Ophthalmol 1992;76:191. 9. Vijaikumar M, Thappa DM, Karthikeyan K, Jayanthi S. A verrucous lesion of the palm. Postgrad Med J 2002;78:302, 305–6. Key words: ocular rhinosporidiosis, Rhinosporidium seeberi
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From the departments of *Ophthalmology and †Pathology, Le Centre hospitalier de l’Université de Montréal (CHUM), Montreal, Que. Originally received Mar. 17, 2005 Accepted for publication Dec. 5, 2005 Correspondence to: Dr. Mona Harissi-Dagher, Department of Ophthalmology, CHUM–Notre Dame Hospital, 1560 Sherbrooke East, Montreal QC H2L 4M1; fax (514) 412-7663; [email protected] This article has been peer-reviewed. Can J Ophthalmol 2006;41:226–9
226
Rhinosporidiosis—Harissi-Dagher et al
I
n India and Southeast Asia, rhinosporidiosis is a common infectious disease, but it has rarely been reported in western countries and thus may pose a diagnostic challenge.1–3 The causative agent, Rhinosporidium seeberi, long considered a fungus, has now been definitively classified using molecular biological tools in a new clade, the Mesomycetozoea.4 The main ocular manifestation of rhinosporidiosis is a conjunctival or eyelid infection.1–3 Infrequently, isolated ocular rhinosporidial infections have been reported in other parts of the world, but to our knowledge, there are no reported cases in
Rhinosporidiosis—Harissi-Dagher et al
Canada. We have recently diagnosed and managed 2 cases of rhinosporidiosis presenting 3 months apart to our university-based ophthalmology clinic in Montreal. CASE
REPORT
1
A 48-year-old Canadian man presented with a right lower eyelid mass. The lesion, first noticed 2 months previously, began as small white spots on the conjunctiva and developed into a moderately large and irritating gelatinous mass. The history was negative for any ocular surgery or trauma. The patient was in good general health except for a remote nasal septoplasty. He was on chloroquine for malaria prophylaxis for a recent trip to Vietnam, 1 month previously, his first overseas trip. As a stoneworker, he handled ornamental stones derived from many regions of the world, including Asia. He complained of repeatedly rubbing his eyes at work because of a dusty environment. Examination of the patient revealed a visual acuity of 6/6 OU. The bulbar conjunctival lesion caused irritation and a mass effect, preventing proper apposition of the eyelids (Fig. 1). On the basis of the clinical appearance of the lesion, diagnoses of pyogenic granuloma or papilloma were suggested. Surgical excision of the lesion was performed. Histopathologic examination of the conjunctival biopsy showed, in the stroma, multiple sporangia at various stages of maturation containing numerous endospores. The sporangia were often surrounded by epithelioid histiocytes. In the intervening stroma, there was a heavy lymphoplasmacytic infiltrate with occasional neutrophils and eosinophils. Because of the unusual aspect of the lesion, the case was sent in consultation to Dr Ahmed Hidayat (Armed Forces Institute of Pathology (AFIP), Washington, DC). The final diagnosis was conjunctival rhinosporidiosis. (Fig. 2) An infectious disease consultation confirmed the localized nature of the infection and systemic medication was deemed unnecessary. The patient was followed up in the ophthalmology clinic 2 months later with no recurrence noted at that time. CASE
REPORT
2
A 51-year-old Canadian man presented with a persistent right upper eyelid mass. The mass had progressively increased in volume over the past 3 months and had become irritating. There was no history of
Fig. 1—Ocular rhinosporidiosis of the bulbar conjunctiva. Note the vascular appearance as well as the tiny white spots corresponding to sporangia on the surface of this polypoid lesion.
Fig. 2—Photomicrograph. Sporangia of different sizes in the conjunctival stroma, confirming the diagnosis of rhinosporidiosis. Sporangia range in diameter from 200 to 300 μm (haematoxylin–eosin; original magnification ×480).
trauma or foreign body to the eye. The patient had never travelled outside Canada. The patient was known for Bean syndrome, a disease consisting of multiple hemangiomas, for which he had undergone numerous surgeries. He also was positive for hepatitis C virus (HCV) and human immunodeficiency virus (HIV), with a CD4 count of 342 cells/μL of blood and a viral load of 4800. He was not receiving antiretroviral therapy because of intolerance to these drugs.
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Rhinosporidiosis—Harissi-Dagher et al
His ocular exam revealed a corrected visual acuity of 6/7.5 OD and 6/6 OS. A right upper eyelid lesion involving the palpebral conjunctiva in proximity to the superior punctum was noted. The lesion was a vascular polypoid mass with tiny white spots covering its surface. Right macular pigmentary changes were attributed to a previous history of central serous chorioretinopathy. Considering the diagnosis of Bean syndrome, a palpebral hemangioma was suspected. Surgical excision was carried out under local anesthesia. The conjunctival biopsy showed large sporangia containing numerous endospores. Some of the sporangia showed a ruptured capsule with extrusion of endospores beneath the surface epithelium. There were numerous neutrophils forming microabscesses, which surrounded the sporangia. A diagnosis of conjunctival rhinosporidiosis was made. The lesion recurred one month later and surgical excision with cautery was undertaken (Figs. 3 and 4). Follow-up is pending.
Fig. 3—Infection of the upper eyelid and palpebral conjunctiva by Rhinosporidium seeberi. Elevated vascularized nodule adjacent to upper punctum.
COMMENTS
Rhinosporidiosis is endemic in south Asia, notably India and Sri Lanka. Migration of people who acquired rhinosporidiosis in their native countries has resulted in the increasing occurrence of this disease in the west. To our knowledge, no cases of ocular rhinosporidiosis have yet been reported from Canada. There have been cases reported in the United States, mostly from the southern states with one case in western New York state.1,5 This latter occurrence raised the question of the organism’s existence in temperate North American climates. Both our patients were Canadian, had always resided in Quebec, had no history of travel to endemic areas before the development of the lesions, and had no contact with infected patients. Patient 1, a good historian, had already noted the growth on his inferior conjunctiva before his trip to Vietnam. Rhinosporidiosis is an infective disease where the pathogen is always found in the tissue lesion. There is no evidence, however, that this disease is infectious or contagious, since no transmission has been documented.1 The majority of cases are sporadic. The presumed mode of transmission is from the natural aquatic habitat of Rhinosporidium seeberi through traumatized epithelium, most commonly in nasal sites. The occurrence of rhinosporidiosis in river-sand workers in India and in Sri Lanka is particularly relevant to this mode of infection, through abrasions
228
CAN J OPHTHALMOL—VOL. 41, NO. 2, 2006
Fig. 4—Photomicrograph. A large sporangium containing endospores extends into the epithelium and is about to rupture (haematoxylin–eosin; original magnification ×1200).
caused by sand particles with the pathogen in ground water. Isolated granulomas can be produced by this microorganism and by infected foreign matter deposited in the conjunctiva during injuries sustained in an agricultural setting.1 Our first patient, because of the nature of his occupation as a stoneworker, was at risk of contracting this infection. Case 2, however, notwithstanding his immune deficiency as a result of AIDS, did not have a typical history for acquiring this infection. In fact, rhinosporidiosis has not been related to immunodeficiency in the literature. However, this case represents the second instance of
Rhinosporidiosis—Harissi-Dagher et al
ocular rhinosporidiosis occurring in an HIV-positive patient in North America, after a first report from Texas.6 The majority of cases occur in upper respiratory sites, notably the anterior nares, nasal cavity, nasopharynx, larynx, soft palate, and buccal cavity. About 15% of cases present in an ocular location, either the bulbar or palpebral conjunctiva, the lacrimal sac, or nasolacrimal duct. Scleral melting has been documented as a complication of conjunctival infection, and it may even be a finding at initial presentation.7,8 The disease can also manifest itself as a cutaneous granuloma.9 Systemic disease is rare. In both our patients, the disease was localized to the ocular surface, the first case affecting the bulbar conjunctiva and the second the upper eyelid and palpebral conjunctiva. Clinically, ocular rhinosporidiosis presents as a friable, richly vascularized polyp that may be pedunculated or sessile. The surface is covered with tiny white spots consistent with underlying mature sporangia beneath the epithelium.1 The gross appearance, though distinctive, is not diagnostic. In case 1, an initial diagnosis of conjunctival papilloma or pyogenic granuloma was made because of the wellvascularized and papillomatous aspect of the lesion. The lesion in case 2 was mistaken for a hemangioma linked to Bean syndrome that he had had since childhood. Diagnosis was confirmed in both cases by histopathology, which showed the typical sporangia and spores. The organism, in all stages of its life cycle, is present in a fibrovascular stroma infiltrated by acute and chronic inflammatory cells including granulomas. Relatively large sacs or sporangia (200–300 μm diameter) may be empty or filled with spores (6–7 μm diameter).1,3 One characteristic phenomenon found on histopathology is the transepithelial elimination of rhinosporidial sporangia at the surface of the mucosa, a dispersal mechanism for rhinosporidial endospores (Fig. 4). Definitive diagnosis requires histopathologic confirmation. Corroboration is made by identification of the pathogen on biopsied specimens rather than by stromal and cellular response. Meticulous excision is the treatment of choice, although rare cases of spontaneous regression have
been reported.1–3 The potential for recurrence is due to spillage of endospores on adjacent mucosa. Recurrences are rare and can be prevented by cauterization of the base of the lesion or alternatively by cryotherapy.2,3 Medical therapy is still controversial since cultures of R. seeberi have been unsuccessful in all artificial media thus making sensitivity determination impossible. Dapsone is the only drug that has had some success in treating R. seeberi. It acts by arresting maturation of sporangia and accelerating their degenerative changes.9 In summary, Rhinosporidiosis is an uncommon fungal infection in the western hemisphere. It presents with certain characteristic clinical features, but the diagnosis is confirmed histopathologically. We have presented 2 cases of ocular rhinosporidiosis, both acquired in Canada. This entity should be included in the differential diagnosis of well-vascularized polypoid conjunctival lesions. REFERENCES 1. Arseculeratne SN. Recent advances in rhinosporidiosis and rhinosporidium seeberi. Indian J of Med Micro 2002;20: 119–31. 2. Arora R, Ramachandran V, Raina U, Mehta DK. Oculosporidiosis in Northern India. Indian Pediatr 2001;38: 540–3. 3. Shrestha SP, Hennig A, Parija SC. Prevalence of rhinosporidiosis of the eye and its adnexa in Nepal. Am J Trop Med Hyg 1998;59:231–4. 4. Arseculeratne SN. Rhinosporidiosis: what is the cause? Curr Opin Infect Dis 2005;18:113–8. 5. Reidy JJ, Sudesh S, Klafter AB, Olivia C. Infection of the conjunctiva by Rhinosporidium Seeberi. Surv Ophthalmol 1997; 41:409–13. 6. Stricker JB, Hurley DL, Slovacek KJ, McCombs WB 3rd. An unusual infection in a human immunodeficiency viruspositive man. Arch Pathol Lab Med 1999;123:1121–2. 7. De Doncker RM, de Keizer RJ, Oosterhuis JA, Maes A. Scleral melting in a patient with conjunctival rhinosporidiosis. Br J Ophthalmol 1990;74:635–7. 8. Sunderraj P. Scleral melting and conjunctival rhinosporidiosis. Br J Ophthalmol 1992;76:191. 9. Vijaikumar M, Thappa DM, Karthikeyan K, Jayanthi S. A verrucous lesion of the palm. Postgrad Med J 2002;78:302, 305–6. Key words: ocular rhinosporidiosis, Rhinosporidium seeberi
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