rhinosporidiosis-etiology-cycle-pathology-treatment-complications.

1. Orissa journal of otorhinolaryngology & head &neck surgery(vol.2;no.1;jan-jun 2008
)

2. Definition
 Classification
 History
 Incidence
 Mode of spread
 Life cycle
 Cardinal features
 Clinical classification
 Histopathology
 Clinical manifestations
 Journal proper
 Investigations
 treatment.


3. Rhinosporidiosis has been defined as a
chronic granulomatous disease
characterised by production of polyps
and other manifestations of hyperplasia
of nasal mucosa(predominantly).
 The etiological agent is Rhinosporidium
seeberi.


4. Rhinosporidium seeberi:
 Initially believed to be a sporozoan, but
later considered to be a fungus and has
been provisionally placed under the
 Family -Olipidiaceae,
 Order -chytridiales of phycomyetes by
ASHWORTH.


5. More recent
classification puts it
under DRIP’S
clade(Mesomycetozo
ea/Ichthyosporea)
 It has not been
possible to
demonstrate fungal
proteins in
Rhinosporidium even
after performing
sensitive tests like
Polymerase chain
reactions.


6. 1892 - Malbran observed the organism in
nasal polyp
 1900 - Seeber described the organism
 1923 - Ashworth described its life cycle
 1953 - Demellow described the mode of
its transmission


7. Rhinosporidiosis has been
reported from about 70
countries with diverse
geographical features
although the highest
incidence has been from
India and Sri Lanka.
 Males are effected 4times
more frequently.
 Occurs in age group of 1540.


8. Theories::
 Demellow's theory of direct transmission.
 Autoinoculation theory of Karunarathnae
(responsible for satellite lesions).
 Haematogenous spread - to distant sites
 Lymphatic spread - causing
lymphadenitis (rare).


9. Demellow's theory of direct transmission::
 He postulated that infection always
occured as a result of direct transmission
of the organsim.
 When nasal mucosa comes into contact
with infected material while bathing in
common ponds, infection found its way
into the nasal mucosa.


10. Karunarathnae accounted for satellite
lesions in skin and conjunctival mucosa
as a result of auto inoculation.
 Karunarathnae also postulated that
Rhinosporidium existed in a dimorphic
state.
 It existed as a saprophyte in soil and
water and it took a yeast form when it
reached inside the tissues.
 This dimorphic capability helped it to
survive hostile environments for a long
period of time.


11. Host factors responsible for endemicity:
 Eventhough quite a large number of
people living in the endemic areas take
bath in common ponds only a few
develop the disease.
 This indicates a predisposing factor in
the host.
 Blood group studies indicate that
rhinosporidiosis is common in patient's
with group O (70%), the next high
incidence was in group AB.


12. 





Spore is the ultimate infecting
unit.
It measures about 7 microns,
about the size of a red cell.
It is also known as a spherule.
It has a clear cytoplasm with 15
- 20 vacuoles.
It is enclosed in a chitinous
membrane which protects the
spore from hostile environment.
It is found only in connective
tissue spaces and is rarely
intracellular.

13. 1)mature
sporangium.
 2)spores(sporan
giospora/endos
pores.)
 3)Immature
sporangium.


14. 





The spore increases in size,
and when it reaches 50 - 60
microns in size granules
starts to appear, its nucleus
prepares for cell division
Mitosis occurs.
By the time 7th division
occurs it becomes 100
microns in size.
A fully mature sporangia
measures 150 - 250
microns.
Mature spores are found at
the centre and immature
spores are found in the
periphery.
The full cycle is completed
within the human body.

15. 
Trophozoite / Juvenile
sporangium - It is 6 100 microns in
diameter, unilamellar,
stains positive with
PAS, it has a single
large nucleus,
(6micron stage), or
multiple nuclei (100
microns stage), lipid
granules are present.

16. 




Intermediate sporangium
- 100 - 150 microns in
diameter
It has a bilamellar wall,
outer chitinous and inner
cellulose.
It contains mucin.
There is no organised
nucleus, lipid globules are
seen.
Immature spores are seen
within the cytoplasm.
There are no mature
spores.

17. 








Mature sporangium - 100 - 400
microns in diameter, with a thin
bilamellar cell wall.
Inside the cytoplasm immature and
mature spores are seen. They are
found embedded in a mucoid
matrix.
Electron dense bodies are seen in
the cytoplasm.
The bilamellar cell wall has one
weak spot known as the
operculum.
This spot does not have chitinous
lining, but is lined only by a
cellulose wall.
Maturation of spores occur in both
centrifugal and centripetal fashion.
The mature spores find their way out
through this operculum on rupture.
The mature spores on rupture are
surrounded by mucoid matrix
giving it a comet appearance.
It is hence known as the comet of
Beattee.

18. 
Mature spores give rise to
electron dense bodies which are
the ultimate infective unit

1 - Trophozoite (juvenile
sporangium)
2 & 3 - Immature bilamellar
sporangia
4a & 4b - intermediate sporangia
with centrifugal and centripetal
maturation of endospores
5 - Mature sporangium with
spores exiting through the
operculum
6 - Free endospore with residual
mucoid material giving it a
comet like apperance (comet of
Beattie)
7a - Free electorn body (ultimate
infective unit)
7b - Free electron dense body
surrounded by other electron
dense bodies which are nutritive
granules.







19. Features::
The cardinal features of rhinosporidiosis
are
 1. chronicity
 2. recurrence &
 3. dissemination.


20. The reasons for chronicity are
 1. Antigen sequestration - The chitinous
wall and thick cellulose inner wall
surrounding the endospores is
impervious to the exit of endosporal
antigens from inside, and is also
impermeable to immune destruction.
 2. Antigenic variation - Rhinosporidial
spores express varying antigens thereby
confusing the whole immune system of
the body.


21. 3. Immune suppression - possible release
of immuno suppressor agents .
 4. Immune distraction.
 5. Immune deviation.
 6. Binding of host immunoglobins.


22. Nasal
 Nasopharyngeal
 Mixed
 Bizzarre (ocular and
genital)
 Malignant rhinosporidiosis
(cutaneous
rhinosporidiosis)


23. Common sites affected:
 Nose - 78%
 Nasopharynx
 Tonsil
 Eye - 1%
 Skin - very rare
 Also affects the lips, palate, uvula,
maxillary antrum, epiglottis, larynx,
trachea, bronchus, ear, scalp, vulva,
vagina, penis, rectum, and the skin.


24. 





Lesions in the nose can be
polypoidal, reddish and
granular masses.
They could be multiple
pedunculated and friable.
They are highly vascular
and bleed easily.
Their surface is studded with
whitish dots (sporangia)
They can be clearly seen
with a hand lens.
The whole mass is covered
by mucoid secretion.

25. 



Histopathology::
There is granulation tissue
containing plasma cells,
lymphocytes, focal
collection of histiocytes
and neutrophils.
The overlying epithelium
is hyperplastic with focal
thinning and occasional
ulceration.
R.seeberi has a
distinctive morphology in
the tissue section.
The sporangia are
located predomiantly in
the stroma of the
mucosal polyp.

26. The largest sporangia are usually in a
subepithelial location.
 The size of the globular sporangia
depend on the stage of maturation.
 Young trophic forms (immature
sporangia) are spherical ,10-100
micrometer in diameter and have a
central basophilic nucleus.
 These develop into mature sporangia by
a process of progressive enlargement
and endosporulation.


27. Endospores represent asexual spores of
Rhinosporidium seeberi.
 After nuclear division in the juvenile
sporangia, endospores are formed by
condensation of cytoplasm around the
nuclei with the formation of cell walls.
This process is known as
endosporulation.


28. Mature sporangia are 100
to 350 micrometer in
diametre.
 The spores are 8-10
micrometer in diameter
and contain globular
eosinophilic inclusions.
 The released spores incite
a neutrophilic response in
the tissue.


29. These spores are also
passed in the nasal
discharge.
 The spores in the tissue
develop into small
trophic forms thus
enlarging the lesion.
 Special stains: R. Seeberi
is visualized by fungal
stains such as PAS,
Gomori's methenimine
silver and mucicarmine.


30. Increased vascularity is due to the
release of angiognenesis factor from the
rhinosporidial mass.
 Rhinosporidial spores stain with sudan
black, Bromphenol blue etc.


31. 
symptoms:: (nasal)
› Unilateral nasal obstruction
› Epistaxis
› Local pruritus
› Rhinorrhea
› Post nasal discharge with cough
› Foreign body sensation
› History of exposure to contaminant water

32. 
On examination
› Pink to deep red polyps.
› Strawberry like appearance.
› Bleeds easily upon manipulation.

33. In cutaneous manifestations::
 3 types of skin lesions are seen
 a)satellite lesions- in which skin
adjacent to the nasal rhinosporidiosis is
involved secondarily.
 b)generalised cutaneous lesionsoccurring through hematogenous
dissemination of the organism.
 c)primary cutaneous lesionsassociated with direct inoculation of
organisms on to the skin.


34. 
Cutaneous rhinosporidiosis may also
present as warty papules and nodules
with whitish spots, crusting, and bleeding
on the surface.

35. JOURNAL PROPER

36. Case report::
This study was done in J.N.M medical
college & Dr.B.R.A.M hospital, Dpt. Of
E.N.T Raipur.
 A case of recurrent rhinosporidiosis.
 A 42yr old farmer from low
socioeconomic status, who was a known
alcoholic & smoker attended OPD with
complaints of mass in nose & oral cavity,
chronic fungating mass in dorsum of lt
foot & antero-lateral aspect of lt lower
limb.


37. Pt had history of recurrent nasal &
pharyngeal rhinosporidiosis for which he
had been operated for 59 times since
the age of 10.
 In 1992 he got lateral rhinotomy, wide
base diathermy coagulation done for
nasal & pharyngeal rhinosporidiosis.
 In 1993 he had growth(rhinosporidiosishistology report) over left foot for which
excision of 1st metatarsal was done.
 In between 1994 to 2005 he had
recurring nasal & pharyngeal masses &
also palatal perforation.


38. In his last attendance pt had mass in
nasopharynx & oralcavity, large 8x7cms.
fungating mass involving 1st & 2nd toe &
dorsum of foot.
 Antero-lateral aspect of lower limb
showed 7x9cm fungating mass.
 Excision & diathermy cauterization of
nasopharyngeal mass was done.
 He was advised for excision of 1st & 2nd
toe & excision of fibular head but the pt
didnot follow the advice.


39. Investigations::
A diagnosis of the disease can be made
by simple aspiration cytology, the
examination of aspirated material with
Gomori methenamine silver and
periodic acid–Schiff reaction, and the
presence of the organism indifferent
stages of maturation even in the
absence of a histopathological study.
 It has to be differentiated from
coccidiomycosis.
 Endospores of coccidiomycosis have
sporangia of smaller size.


40. Other granulomatous diseases affecting
nose & sinuses
 Sarcoidosis
 Wegeners granulomatosis.
 Midline lethal granuloma.
 Tb,leprosy,syphilis.
 Coccidioidimycosis.
 Blastomycosis
 Rhinoscleroma.
 Sporotrichosis
 Leishmaniasis.


41. Treatment::

While several anti-bacterial
and anti-fungal drugs have
been tested clinically, the only
drug which was found to have
some anti-rhinosporidial effect
is dapsone (4,4diaminodiphenyl sulphone)
which appears to arrest the
maturation of the sporangia
and to promote fibrosis in the
stroma, when used as an
adjunct to surgery.

42. 
Dose of Dapsone- 100 mg once daily for
6 months to several years.Check LFT and
blood counts every 2 weeks.

43. The applicability of anti-rhinosporidial
therapy using medication can be
considered in two scenarios
 (a) presurgical or postsurgical and
 (b) solely medications.


44. Pre surgical use::
A serious complication of surgery in
rhinosporidiosis especially of the nasal
and nasopharyngeal sites, is theprofuse
intraoperative hemorrhage that results
from thehigh vascularity of the growths.
 Presurgical dapsone would minimize
hemorrhage by promoting resolution of
the infection, with promotion of fibrosis, as
well as preventing the colonization and
also prevents infection of new sites after
the release of endospores from the
surgically traumatized polyps.


45. Post surgical use::
Colonization of normal mucosae by the
endospores released from the site of
excision, could be controlled by post op
dapsone.
 In view of the danger of dissemination of
R.seeberi, especially after surgery,with
extensive histolysis of soft tissues
including bone and cartilage, it can be
considered advisable to commence
medications, however, small the original
lesion appears to be.


46. Surgical treatment::
Total excision of the polyp, preferably by
electro-cautery, is recommended.
 Pedunculated polypsradical removal
 Excision of sessile polyps with broad
bases of attachment to the underlying
tissues are sometimes followed by
recurrence due to spillage of
endospores on the adjacent mucosa.


47. Laser Surgical removal
Smaller lesions can easily be removed
by Co2 laser with minimal bleeding.
 But Larger polyps are difficult to remove
and the theoretical hazard of spreading
spores in the plume and need for
fumigation of the theatre later.


48. Conclusion::
Rhinosporidiosis shows both long
duration & tendency for recurrence.
 Recurrent seeding of circulation with
spores from nose & nasopharynx may
lead to involvement of nonmucosal sites.
 Trans epithelial infection is also important
for recurrence in sites & extension to
nearby sites.


49. Failure to remove all infected tissues at
the time of surgery & implantation of
spores in fresh areas of abrasions may
cause recurrence.
 Removal of growth by snare without
cauterisation was considered to result in
dissemination & recurrence.
 Good result obtained with diathermy was
explained on the basis that it avoids
implantation of spores & destruction is
deep.


50. Thank you!!!