mucormycosis case presentation

1. CASE PRESENTATION
DR. ANVESH NARIMETI
1st yr postgraduate
INTERNAL MEDICINE
GANDHI HOSPITAL

2. IDENTIFICATION DATA
NAME-PVK PRASAD
AGE-49 Yrs
SEX-MALE
OCCUPATION-TEACHER
ADDRESS-GUNTUR
RELIGION-HINDU

3. CHIEF COMPLAINT
Fever -45 days back
Swelling of right side of face -40 days back
Loss of vision in rt eye 35 days back

4. HISTORY OF PRESENT ILLNESS
Patient was apparently normal and doing all his work
normally before 45 days and developed one episode of
fever
• sudden onset
• high grade associated with chills and rigors
• continuous fever
• associated with generalised body pains
• subsided with medication
After 2 days of fever patient noticed swelling of right side
of cheek not associated with pain.
Gradually in two days patient developed swelling in rt
eye,,, not associated with pain,,, associated with
redness of eye,, and protrusion of rt eye

5. • Followed by that patient had difficulty in
moving the eye,,, and decreased vision in the
rt eye,,, and patient unable to open the eye lid
and with in span of 5 days patient completely
lost vision in the rt eye
• Patient developed deviation of angle of
mouth towards left side and patient presented
to gandhi hospital after 10 days of presenting
symptom

6. • h/o of loss of vision in rt eye
• h/o of decreased sensation on the fore head and malar
prominence
• No h/o difficulty in mastication
• h/o of drooling of saliva from rt side of mouth, and
unable to fill the air in rt side of mouth
• Deviation of angle of mouth towards lt side
• No h/o of difficulty in hearing
• Able to perceieve all the smells
• No h/o of nasal regurgitation
• No h/o difficulty in swallowing

7. • No h/o weakness of limbs and trunk
• No h/o paresthesias and numbness
• No h/o of trauma
• No h/o of LOC
• No h/o of ear discharge,
• No h/o of halitosis,
• No h/o of ear pain
• No h/o of seizures,
• No h/o of Headache
• No h/o of neck pain,
• No h/o of Vomtings
• No h/o of jaw claudication

8. PAST HISTORY
• h/o of diabetes from last 15 yrs on irregular
medication
• h/o of hypertension from last 3 monts
irregular treatment
• No h/o of TB ,CAD ,CVA,EPILEPSY ,THYROID
DISORDERS

9. • Personal h/o-strict vegetarian,non smoker,not
an alcoholic,no other addictions,sleep and
bowel and bladder habits are normal
• Family history-born out of consanguinous
marriage 4th in birth order out of 5,,,family h/o
of diabetes in father and 3 other siblings.,,no
other significant history
• Drug h/o- occasionally using OHA and anti
hypertensives,

10. SUMMARY
• 49 yrs old patient,,,TYPE 2 DM and HTN on
irregular treatment,,with fever and followed
with H/O Suggestive of right sided 2nd , 3rd , 4th
, and 6th ,,and ophthalmic and maxillary
division of 5th nerve and 7th nerve involvement
with proptosis of rt eye ball with painless loss
of vision.

11. DIFFERENTIAL DIAGNOSIS
• Orbital apex syndrome
• Cavernous sinus thrombosis
• Superior orbital fissure syndrome
• Fungal infection in uncontrolled diabetes-
Mucormycosis,aspergillosis
• Tolasa hunt syndrome
• Orbital tumours
• Isoloated sphenoid sinus tumours

12. GENERAL EXAMINATION
• Patient ill looking ,,,toxic and no pallor
• no icterus,
• no cyanosis ,
• no clubbing,
• no lymphadenopathy
• no pedal edema

15. RIGHT EYE
• Blackish discolouration of peri orbital area and
lids
• Edema of both lids
• Conjuctival chemosis +
• Proptosis - non pulsatile, non compressible,non
tender,not variying with change in position.
• Perception of light absent
• Cornea hazy
• Pupils completely blocked by exudates
LEFT EYE -NORMAL

16. • Ear examination is –normal
• Nose examination –black discolaration of rt
side of nose,,, nasal cavity is normal
• Broadening of nasal bridge
• Oral cavity –normal
• Oro pharynx-normal
• Rt side maxillary sinus tenderness present

17. VITALS
• BLOOD PRESSURE – 150/90 mm of hg rt arm
supine position
• PULSE RATE-90 bpm regular in rythem,normal
volume,,, thickened vessel wall, no radio radial
delay ,no radio femoral delay, all peripheral
pulses are equally felt.
• RESPIRATORY RATE-18 per min abdomino
thoracic

18. CENTRAL NERVOUS SYSTEM
• HIGHER MENTAL FUNCTIONS – patient is
conscious, coherent,oriented with time place
and person,,
• Speech-dysarthria +,
• Memory intact

19. Cranial nerves
• 1ST NERVE-normal
• 2ND NERVE- rt side absent perception of light
Fundus- cannot be examined on rt side. left
side proliferative diabetic retinopathy
• 3rd ,4th ,6th cranial nerve rt side total
ophthalmoplegia,, direct and indirect pupilary reflex
absent, left side direct reflex is present indirect
pupilary reflex is absent
• 5th nerve decreased sensation in ophthalmic and
maxillary division on rt side,over mandibular region
sensation is good and mastication is good.corneal
reflex couldn’t be examined.

20. RIGHT 7TH NERVE
Deviation of angle of mouth to left side
loss of naso labial fold
Loss of frowning
Unable to hold air
Taste sensation lost
• Left 7th nerve normal
• 8TH NERVE-Patient is not cooperating for
tuning fork tests
• 9th , 10th , 11th ,& 12th , cranial nerves are
normal

21. • Motor system normal
• Sensory system- pinprick sensation reduced
upto mid leg. Vibration and joint sensation
normal
• CEREBELLAR SYSTEM NORMAL
• Gait normal
• Autonomic nervous system normal
• No signs of meningeal irritation

22. • Cardiovascular system- normal
• Respiratory system -normal
• Gastrointestinal system- normal

23. INVESTIGATIONS
• HB-9.1, PCV 28%,TLC-17,200,PLT-4.2
• DC-N88 L8 M3 E1
• PT-12.9 APTT-22.6
• BL00D UREA-66
• SERUM CREATININE-2.5
• NA 127 K 4.2
• SGOT-24,SGPT-44
• RBS-500
• ALP-330,SERUM PROTIENS-6.7GM
• ALBUMIN-3.0
• SERUM BILURUBIN-0.4
• HIV NR HBSAG NR
• ECG –NORMAL
• CXR PA VIEW NORMAL
• ESR –INCREASED
• Usg abdomen –normal
• 2d echo -normal

24. • CT BRAIN-HYPODENSITY IN RT TEMPORAL
REGION-? CEREBRITIS
MUCOSAL THICKENING OF B/L ETHMOID AND
SPHENOIDAL SINUS
• CT PNS- ORBITAL CELULITIS,soft tissue
attenuation in rt pre septal,extra coronal fat
with soft tissue swelling in rt periorbital region
s/o inflamatory changes
• Pan sinusitis with blockage of rt osteomeatal
unit,mild dns to rt

25. • MRI BRAIN- Enhancing altered signal intensity
foci in right nasal cavity with extensivemucosal
thickening of all paranasal sinuses with extension
of lesion into the sphenoid bone and destroying
the greater wing of rt sphenoidwith edema of rt
temporal lobe-s/o fungal sinusitis
• Proptosis of rt eye ball
• Edema in subcutaneous tissue of rt cheek
• Infarcts in b/l frontal parietal,left temporal lobe
white matter ,corona radiata&centrum semiovale

28. • Patient was subjected to FESS and intra nasal
sinus biopsy taken from rt maxillary sinus and
sent for lab
• KOH PREPARATION –showing plenty of branching
septate hyphae
• Calcoflour white mount shows broad widely
branching sparesly septate hyphae,resmbling
zygomycetes
• Fungal culture showing growth of rhizopus
species

29. Final diagnosis
• Uncontrolled Type 2 DM
• Uncontrolled Hypertension
• Rhinocerebral mucormycosis
• Rt orbital cellulitis
• Multiple cranial nerve lesions(2,3,4,6,7,v1 v2)
• Proliferative Diabetic retinopathy
• Diabetic neuropathy

32. MUCORMYCOSIS
• Mucormycosis represents a group of life-threatening infections
caused by fungi of the order Mucorales.
• Rhizopus oryzae (in the family Mucoraceae) is by far the most
common cause of infection
• These fungi cause infection primarily in patients with diabetes or
defects in phagocytic function (e.g., associated with neutropenia or
glucocorticoid treatment).
• Patients with elevated levels of free iron, which supports fungal
growth in serum and tissues, are likewise at increased risk for
mucormycosis.
• In iron-overloaded patients with end-stage renal failure, treatment
with deferoxamine predisposes to the development of rapidly fatal
disseminated mucormycosis; this agent, an iron chelator for the
human host, serves as a fungal siderophore, directly delivering iron
to the Mucorales.
• Mucormycosis typically occurs in patients with diabetes mellitus,
solid organ or hematopoietic stem cell transplantation (HSCT),
prolonged neutropenia, or malignancy

33. Mucormycosis can be divided into at least six
clinical categories based on clinical presentation
and the involvement of a particular anatomic
site
1. Rhinocerebral(most common in diabetics)
2. Pulmonary(in patients undergoing HSCT)
3. Cutaneous
4. Gastrointestinal
5. Disseminated
6. Miscellaneous

34. RHINOCEREBRAL MUCORMYCOSIS
• The initial symptoms of rhinocerebral mucormycosis are nonspecific
and include eye or facial pain and facial numbness followed by the
onset of conjunctival suffusion, blurry vision, and soft tissue
swelling. Fever may be absent in up to half of cases, while white
blood cell counts are typically elevated as long as the patient has
functioning bone marrow
• If untreated, infection usually spreads from the ethmoid sinus to
the orbit, resulting in compromise of extraocular muscle function
and proptosis, typically with chemosis. Onset of signs and
symptoms in the contralateral eye, with resulting bilateral
proptosis, chemosis, vision loss, and ophthalmoplegia, is ominous
and suggests the development of cavernous sinus thrombosis
• Upon visual inspection, infected tissue may appear to be normal
during the earliest stages of fungal spread and then progresses
through an erythematous phase, with or without edema, before
the onset of a violaceous appearance and finally the development
of a black necrotic eschar.

35. DIAGNOSIS
• Definitive diagnosis requires a positive culture from a
sterile site (e.g., a needle aspirate, a tissue biopsy
specimen, or pleural fluid)
• Histopathologic evidence of invasive mucormycosis.
• A probable diagnosis of mucormycosis can be established
by culture from a nonsterile site (e.g., sputum or
bronchoalveolar lavage)
• Biopsy with histopathologic examination remains the most
sensitive and specific modality for definitive diagnosis .
• Biopsy reveals characteristic wide (6- to 30-m), thick-walled,
ribbon-like, aseptate hyphal elements that branch
at right angles

36. TREATMENT
• AmB deoxycholate -1 mg/kg per day
• LAmB (LAmB) - 5–10 mg/kg per day
• amphotericin B lipid complex (ABLC) -5-7.5mg/kg
• However, dose escalation of LAmB to 10 mg/kg
per day for CNS mucormycosis may be considered
in light of the limited penetration of polyenes into
the brain
• ABLC dose escalation above 7.5 mg/kg per day is
not advisable
• Echinocandin–lipid polyene combinations
inprimarily diabetic patients with rhino-orbital-cerebral
mucormycosis

37. TAKE HOME MESSAGE
• Multiple cranial nerves palsy should not be attributed to spontaneous
microvascular events affecting more than one cranial nerve at a time. This
remarkable coincidence does occur, especially in diabetic patients, but the
diagnosis is made only in retrospect after all other diagnostic alternatives
have been exhausted
• Neuroimaging should focus on the cavernous sinus, superior orbital
fissure, and orbital apex, where all three ocular motor nerves are in close
proximity.
• In a diabetic or immunocompromised host, fungal infection (Aspergillus,
Mucorales, Cryptococcus) is a common cause of multiple nerve palsies.
• In a patient with systemic malignancy, carcinomatous meningitis is a likely
diagnosis

38. •THANK YOU