3. CASE PRESENTATION
• 9 year old boy
• Presenting complaints:
– Painful + swollen left eye X 1 week
– Associated with fevers and headaches.
– No history of trauma or any prior systemic illness
– HIV test was found to be negative.
4. CASE PRESENTATION
On Examination
• Right eye: Normal exam
• Left Eye:
– Vision 6/36 with normal fundoscopy
– Warm and very tender periorbital swelling
– Restricted EOM (all directions), no ptosis
– Chemosis and a proptosis of 22 mm (versus right
eye which measured 17mm from lateral orbital
margin to the cornea).
5. CASE PRESENTATION
• Admission Dx: Orbital cellulitis (?Bacterial
Etiology)
• FBC: WBC 15.8 x 103/ uL (Netrophils: 79%)
• Initial treatment: IV ceftriaxone 1g BD (B/C
not available)
• Urgent Imaging: NOT POSSIBLE
6. CASE PRESENTATION
• Admission day 2:
– No clinical improvement
– Thus exploratory I and D performed (no pus drained)
• EUA: No additional findings
• MRI head (done on admission day 7)
– Proptosis
– Isointense mass in the medial aspect of the left orbit
measuring 3cm x 2.1cm x 2.9cm. Normal paranasal
sinuses
– DDx: Rhabdomyosarcoma VS Burkitt’s lymphoma.
8. CASE PRESENTATION
• 2nd week: admitted to the paediatric oncology ward
• Trial Chemotherapy for Burkitt’s lymphoma
(cyclophosphamide, Doxorubicin, Prednisolone and
Vincristine)
– 4 cycles over 4 weeks.
– Peripheral blood film, CSF analysis, abdominal ultrasound
and chest X-ray were all normal.
– 4th week: Clinical condition stable over the 4 weeks
– On re-examination: No significant improvement in
proptosis and there was a lateral deviation of the globe
with a tender rubbery mass palpable medially through the
lower lid.
9. CASE PRESENTATION
• Incisional biopsy of the mass was performed
through the lower lid, removing most of the
palpable superficial part:
– Histology result: Necrotising granulomatous
inflammation with fungal hyphae, consistent with
zygomycosis (Rhizopus VS Mucor)
11. • Treatment (Paediatric Medical team)
– Fluconazole P.O. 300mg od X 6 weeks
– Then: 150mg od X 4 weeks
• The clinical condition remained stable, the
proptosis gradually resolved
– MRI done 4 weeks into treatment: 27% reduction in
volume of the left orbital mass (compared to the first
MRI) with no spread beyond the orbit.
• Currently (1 Year after the 10 week treatment)
– No proptosis, normal vision and normal extraocular
muscle movements.
CASE PRESENTATION
13. MUCORMYCOSIS
• Mucormycosis is a severe infection caused by fungi of
the order Mucorales (Rhizopus, Lichtheimia, Mucor &
Rhizomucor are commonest) .
• Organisms are normally found in dust, soil and also in
upper respiratory mucosa of healthy individuals.
• Risk Factors (immunosuppression):
– Uncontrolled Diabetes (DKA)
– hematological malignancies (most common reason in
paediatric patients)
– Aplastic anemia ; Chronic renal insufficiency
– Haemochromatosis; Aluminium overload
– AIDS (rarely a risk factor)
14. • Pathogenesis:
– In immunocompromised patients, inhaled spores
germinate to form hyphae, spread to the
paranasal sinuses and subsequently to the orbit,
meninges, and the brain by direct extension.
– Infection is angioinvasive: results in thrombosis
and subsequent infarction and necrosis of affected
tissues
– Severe morbidity and high mortality
MUCORMYCOSIS
15. MUCORMYCOSIS
• Clinical Presentation
– Rhino-orbital-cerebral mucormycosis (RCM),
– Pulmonary
– Disseminated
– Gastrointestinal
– Cutaneous
• Initially, nonspecific symptoms:
– fever, facial pain, rhinorrhea and nasal congestion.
– With intracranial and orbital involvement:
ophthalmoplegia, proptosis, orbital cellulitis, vision
failures and changes in mental behavior
16.
17. • Diagnosis:
– Requires Histopathological demonstration of
hyphae.
– Mycological culture may be helpful.
MUCORMYCOSIS
18. DISCUSSION
• Mucormycosis was first described as a Human
pathogen in 18851
– It is rare and although known for a long time,
epidemiology is still unclear.
• Spain: incidence 0.4 cases per million/year
• France: 1.2 cases/million/year
• USA: 1.7 cases per million per year
– is rapidly progressive with severe morbidity and a
high mortality
19. • Immunosuppression is a known risk factor
– But the current patient did not have an
established co-morbidity
• However, there are increasing reports of
infection occurring in immunocompetent
individuals 2,3.
DISCUSSION
20. DISCUSSION
• Clinical picture depends on the anatomical
area involved
– Most common site: Paranasal sinuses4
– Concomitant organ involvement influences
success of therapy
• Although MRI showed no sinus involvement,
– Presenting symptoms were consistent with
literature on common presentation patterns
– i.e. fever, headache, peri-ocular swelling , ↓ EOMs
21. • Thus a fungal etiology should be considered in
the setting of orbital cellulitis with the above
clinical features with:
– No response to conventional systemic antibiotics
(Particularly in Immunosuppressed patients)
• This may aid in early diagnosis
– Which has been shown to be life-saving
DISCUSSION
22. DISCUSSION
• MRI findings were not classical for mucormycosis
(typically shows oedema and thickening of sinus
mucosa ± other findings).
– MRI is more sensitive than CT scan in imaging orbital soft
tissues.
– But both CT scan and MRI show variable imaging patterns
and may not be sensitive enough for fungal lesions
involving bone or subcutaneous tissue8
• One case series of 28 Patients (Turkey): Images
consistent with mucormycosis were detected in 57% of
the cases that had cranial MRI and in 69% of the cases
that had orbital MRI4.
23. • Definitive Diagnosis:
– Histopathological demonstration of hyphae (gold
standard)
– And Mycological culture (but culture is positive in 50 –
75 % of cases)4
• Treatment:
– Control of underlying condition (e.g. controlling DM,
Stopping immunosuppressive RX)
– Debridement of necrotic tissue (penetration of
antifungals into infected area is weak)…..may need
exenteration
– Antifungal treatment
DISCUSSION
24. • In current case, the extensively taken incisional
biopsy could have played a therapeutic role.
• Some case reports of successful treatment
without exenteration12
• First line systemic treatment is Amphotericin B
– Posaconazole is occasionally given as maintenance
therapy or with intolerance to first line Rx.
– A number of case reports have reported success with
treatment using fluconazole in pansinusitis,
pulmonary mucormycosis6,9.
DISCUSSION
25. • Our Patient was treated with fluconazole
(Amphotericin B was not available)
• Response to therapy was good.
– However: not much clinical data to support use of
fluconazole in treating mucormycosis
– With most sources stating it is not effective for
mucormycosis13
DISCUSSION
26. CONCLUSION
• Although the case was atypical; having no clear
risk factors and no rapid progression before
antifungal treatment… (because
immunocompetent?)
• It highlights the importance of considering
mucormycosis as a possible differential in orbital
cellulitis, particularly in
– cases with poor response to conventional therapy
– Immunosuppressed individuals (where early diagnosis
may be life saving)
27. REFERENCES
1. Bala K, Chander J, Handa U, Punia R, Attri A. A prospective study of
mucormycosis in north India: Experience from a tertiary care hospital.
Medical Mycology. 2015;53(3):248-257.
2. Hussain S, Salahuddin N, Ahmad I, Salahuddin I, Jooma R. Rhinocerebral
invasive mycosis: occurence in immunocompetent individuals. European
Journal of Radiology. 1995;20(2):151-155.
3. Rao Sridhara S, Paragache G, Panda N, Chakrabarti A. Mucormycosis in
Immunocompetent Individuals: An Increasing Trend. J of Otol.
2005;34(06):402.
4. Kursun E, Turunc T, Demiroglu Y, , Alışkan H, Arslan A. Evaluation of 28
cases of mucormycosis. Mycoses. 2015;58(2):82-87.
5. Sheybani F, Naderi H, Sarvghad M, Ghabouli M, Arian M. How should we
manage a patient with invasive mucoromycosis who develops life-
threatening reaction to amphotericin B? Report of two cases and
literature review. Medical Mycology Case Reports. 2015;8:29-31.
28. 6. FUNADA H, MIYAKE Y, KANAMORI K, OKAFUJI K, MACHI T, MATSUDA T. Fluconazole therapy for
pulmonary mucormycosis complicating. Japanese Journal of Medicine. 1989;28(2):228-231.
7. Farooq A, Patel R, Lin A, Setabutr P, Sartori J, Aakalu V. Fungal Orbital Cellulitis: Presenting
Features, Management and Outcomes at a Referral Center. Orbit. 2015;34(3):152-159.
8. Altini C, Niccoli A, Ferrari C, Rubini D, Dicuonzo F, RubinI G. 18F-FDG PET/CT contribution to
diagnosis and treatment response of rhino-orbital-cerebral mucormycosis. Hell J Nucl Med.
2015;18(1):68-70.
9. Koçak R, Tetiker T, Koçak M, Başlamişli F, Zorludemir S, Gönlüşen G. Fluconazole in the treatment
of three cases of mucormycosis. European Journal of Clinical Microbiology & Infectious Diseases.
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10. Petrikkos G, Skiada A, Lortholary O, Roilides E, Walsh T, Kontoyiannis D. Epidemiology and Clinical
Manifestations of Mucormycosis. Clin Infect Dis. 2012;54(1).
11. Nithyanandam S, Jacob M, Battu R, Thomas R, Correa M, D'Souza O. Rhino-orbito-cerebral
mucormycosis. A retrospective analysis of clinical features and treatment outcomes. Indian J
Ophthalmol. 2003;51(3):231-6.
12. Pelton R, Peterson E, Patel B, Davis K. Successful Treatment of Rhino-orbital Mucormycosis
Without Exenteration. Ophthalmic Plastic and Reconstructive Surgery. 2001;17(1):62-66.
13. Spellberg B, Walsh T, Kontoyiannis D, Edwards, Jr., J, Ibrahim A. Recent Advances in the
Management of Mucormycosis: From Bench to Bedside. Clinical Infectious Diseases.
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