Chief Complain62 year old Caucasian female presents

with
4 week history of left eye blurriness

HPI Presented to OSH 2 weeks ago with

headache and red left eye.

Headache x 1 month,

Redness getting worse for 2 weeks

Sinusitis was suspected and was sent

home on Z-pack.
Headache started getting worse.
Photophobia x 10 days
Nausea and Vomiting x 10 days

HPI Continued Worsening of photophobia

- Started seeing Flashes and Floaters.

- Dropping of left eye lid x 2-3 days

Complete loss of vision in left eye x 1 day

Went to see PCP couple of days ago

- Sent to OSH ER

- ? Surgery was planned

- Discharged/ left AMA on ABx

Differentials????

PMHHypertension
Diabetes mellitus
Heart abnormality
Hepatitis C

PSHTonsillectomy
Lap cholecystectomy
Fractured tibia

Medications

Aspirin
Tramadol
Losartan
Amlodipine
Metformin
Glipizide
Zofran

Review of Systems:
General: weight loss, fatigue, weakness
HEENT: Denies rashes, itching, headache, acute visual changes, hearing loss,
tinnitus, rhinorrhea, hoarseness, sore throat
Cardiac: Denies chest pain, palpitations, dyspnea on exertion, edema
Respiratory: Denies shortness of breath, wheezing, sputum, hemoptysis
Gastrointestinal: Denies abdominal pain, nausea, vomiting, change in bowel habits,
diarrhea, constipation, hematochezia, or melena
Genitourinary: Denies dysuria, hematuria hesitancy, frequency
Musculoskeletal: Denies muscle weakness, joint pain or stiffness
Neurologic: Denies numbness or tingling in extremities, dizziness, lightheadedness
Hematologic: Denies easy bruising/bleeding
Endocrine: Denies thyroid problems, diabetes

Physical Exam

VITALS:
BP 147/65
Pulse 76
Temp 97.6 F (36.4 C)
Resp 18 BMI 25.60 kg/m2 | SpO2 97%

GEN: alert, awake, in acute distress

HEENT:NCAT, MMM, left eye ptosis, left eye dilated and unreactive, right
eye with mydriasis with some light accommodation
CHEST: Clear to auscultation bilaterally
HEART: Regular rate and rhythm, Nl S1 and S2 No gallops.
ABD: Soft and non tender, non-distended
EXT: No edema.

Neuro exam

Cranial Nerves:
Pupils-right 4mm reactive to light, left-8mm non reactive to light; Complete ptosis of left eye,
EOM- right-intact except some limitation of right lateral movement (LR muscle), left eye- EOM impaired in all
directions
Facial sensation intact bilaterally to light touch but complains about tingling bilaterally in V1 distribution; no facial
palsy
Hearing intact to finger rub bilaterally
Palate symmetric; normal tongue protrusion
Shoulder shrug symmetric
Motor:
Abnormal Movements: None
Bulk: Atrophy of interossei muscles of hands, also tibialis anterior muscle atrophy
Tone: Normal
Strength: RUE 5/5, LUE 5/5, RLE 5/5, LLE 5/5
DTR: Bi Tri BR Pat Ach Toes
R 2 1 2 2+ 1 Down
L 2 1 2 2+ 1 Down
Hammer toes and high arched feet (pes cavus) noted
Sensory:
Intact to light touch bilaterally
Cerebellar:
FNF intact bilaterally
Gait:
Normal stride and stance, but difficult to do tandem walk, toe walking and single leg standing not possible

Ophthal Exam

DIFFERENTIALS??????

Differential Infectious process(Bacterial

endophthalmitis, Keratitis, Aspergillosis,
Mucor, Brain Abscess)
Glaucoma( Angle closure)
Giant Cell arteritis, Optic Neuritis, CRAO,
CRVO
Neoplastic
Trauma, Vitreous Hemorrhage

Labs BMP:
NA 127 L
K 3..9

CL

94 L

CO2 25
BUN 23
CREA
0.8
GLU 177 H

CBCWBC
9.6
HGB 13.3
Neutro 68%
Lymho 22%
HCT 35.2
PLT 136 L
UDS positive for
opiates

Hepatic Function

Total Protein
Albumin
Tbil
ALP
ALT
AST

5.0
2.6
0.6
89
45
38

Others RPR , HIV negative

HepC Ab reactive
Other hepatitis panel nonreactive
B12 442
Folate 6.7 (low)
MMA 269
TSH 0.259, FT4 1.5
A1c
7.1
ESR 35, CRP <0.5

Serum Protein Electrophoresis

Total Protein
Albumin
Alpha-1 Globulins
Alpha-2 Globulins
Beta Globulins
Gamma Globulins

Serum Immunofixation Electrophoresis-

5.0 (L) 6.0-8.3 g/dLFinal

3.0 (L) 3.3-5.6 g/dLFinal
0.2 0.1-0.3 g/dLFinal
0.6 0.5-1.0 g/dLFinal
0.5 (L) 0.6-1.1 g/dLFinal
0.7 0.6-1.6 g/dLFinal

Polyclonal IgG, IgA, and IgM immunoglobulins found.

No monoclonal immunoglobulins detected. Non-secretory myeloma (NSM) cannot be excluded
on the basis of this result.

CT Head

CT ORBIT

MRI BRAIN

MRI BRAIN-

MRI ORBIT

CT head "An ill-defined soft tissue density mass in the posterior

left ethmoid sinus measuring approximately 1.8 x 1.6 cm
extending to the left sphenoid sinus with bone
destruction of the left lateral wall of the ethmoid sinus /
left sphenoid bone.
The mass is seen invading the left optic canal and
superior orbital fissure likely representing a malignancy
and less likely representing infection."

CT ANGIO HEAD & NECK

Complete opacification of the left sphenoid sinus
with erosions in the anterior, lateral and superior
walls of the sinus.
Erosions of the medial wall of the left superior
orbital fissure, inferior wall of the left optic canal,
and anterior wall of the distal left carotid canal
Soft tissue extension into the left orbital apex
likely producing mass effect upon the optic
nerve.

MRI Head
Acute left sphenoid sinusitis superimposed upon chronic
sinusitis causing dehiscence or erosion of the anterior
and lateral walls of the left sphenoid sinus including the
medial wall of the left supraorbital fissure with extension
of disease into the left orbital apex.

Inflammation of the posterior left optic nerve sheath

complex and posterior myotendinous junctions of the left
rectus muscles at the annulus of Zinn consistent with
orbital apicitis.

HOSPITAL COURSE ENT, Ophthal, Neuro consulted.

ENT recommended biopsy of mass.
Ophthal recommended MRI and biopsy.
Neurosurgery consulted for optic nerve
decompression.
Neuro recommended MRI orbits and MRI
brain and spine.

Hospital Course ContinuedENT- Endoscopic Sinus Surgery

- Bilateral maxillary antrostomies, total
ethmoidectomies and sphenoidotomies.
- Bilateral optic nerve decompression.
- Image-guided endoscopic removal of left
sphenoid sinus lesion
- Lesion was thought to be a large fungal
ball.

Hospital Course Continued Mass was removed and sent for micro and
pathology.
Micro revealed segmented hyphae.
ID was consulted , Amphotericin B and decadron
started.
Vision acutely worsened and now pt reported
complete loss of vision in R eye as well .
Neurosurgery consulted for further assistance in
treatment

Hospital Course Neurosurgery said no interventions.

Flexible Nasal-NasopharyngealLaryngoscopy was performed that showed
normal nasal mucosa but purulent fluid
from L sphenoethmoid outflow tract.
All cultures so far being negative.

Culture from Sinus BiopsyAerobic cx: GNB, growth in thioglycolate Achromobacter

Denitrificans, stain - no organism seen
Anaerobic cx: light growth mold, aerobic cx compatible
with routine culture
Tissue cx: GNB (3 colonies after 48 hrs); stain - no
organism seen
Fungal cx: NGTD; smear - many segmented hyphae

Microscopic Read The sphenoid mass is a large fungus ball.

Hyphae have acute angle branching, have relatively

uniform diameters, and have septae, compatible with
an Aspergillus-like species.

Hospital Course Antimicrobial coverage was switched from

Flagyl and ceftriaxone to amphotericin B
and Zosyn. Steroid was continued for few
more days.
In the mean time she developed ?visual
hallucinations likely due to steroids.
Opthalmology suggested Visual hallucinations are
consistent with Charles Bonnet syndrome (non-threatening
hallucinations that the patient is aware is not real). This is due to
loss of vision and likely not related to the steroid therapy.

Hospital Course Steroids was discontinued.

Patient started developing hypomagnesemia and hypokalemia.
No improvement in vision in either of the
eyes after almost a week of presentation.
Now what????

Hospital Course Wait.

Finally.Identification of Mold was made
and it was Scedosporium.
Amphotericin was stopped.
Started on Voriconazole.
Zosyn was continued (which covers Achromobacter)
for a week and stopped.

Hospital CourseRepeat ENT procedure unrevealing for

infiltrate.
Discharged on Voriconazole to rehab with
plan to f/u ENT, EYE and ID.

Final Diagnosis Invasive Fungal Sinusitis with

compression of optic nerve first on left
and then onto the right optic nerve
caused by a mold, Scedosporium.

Scedosporium
found ubiquitously in the environment,
including in soil and polluted water.
Two most common ones are
- Scedosporium apiospermum

- Scedosporium prolificans
Human infection = from inhalation of
spores or through direct inoculation, as in
a skin puncture

Scedosporium-

Microscopy-

 Interestingly, Scedosporium is the second

most commonly detected filamentous
mold in clinical samples, after Aspergillus
spp.
S. apiospermum is the second most
common filamentous fungus isolated from
the airways of cystic fibrosis patients

Clinical Manifestations Respiratory tract-

frequently involves in patients with chronic

granulomatous disease , chronic glucocorticoid use , hematopoietic cell
transplantation ,hematologic malignancy, or solid organ transplantation .

The initial presentation typically includes fever, cough, sputum production,

pleuritic chest pain, tachypnea, and malaise.

Central nervous system- brain abscesses in both

immunocompetent and immunocompromised patients .

Skin, soft tissue, and bone- nodules or as

erythematous to purple papules or bullae, osteomyelitis has also
been reported due to S. apiospermum

Clinical Manifestations Keratitis and endophthalmitis

- eye pain,
photophobia, foreign body sensation, conjunctival or corneal
erythema, tearing, and changes in visual acuity.
Scedosporium corneal infections can lead to frank corneal
ulceration, abrasion, perforation, infiltrate, and anterior chamber
hypopion

Diagnosis

Histopathology
Cultures
Molecular techniques
Antigen detection

Treatment

Antifungal therapy should be initiated as soon as possible

for Scedosporium infections.
The antifungal agent with the greatest efficacy against S. apiospermum is
voriconazole
For the empiric treatmentof S.apiospermum infections, voriconazole
monotherapy hsould be started.
For empiric therapy of S. prolificans infection, voriconazole plus terbinafine
with or without an echinocandin can be tried.

Surgical debridement should be performed when feasible (ie, when infection

is localized), as it has been associated with improved outcomes.

In neutropenic patients, granulocyte-colony stimulating factor to hasten

recovery from neutropenia can be considered, although further study is
needed to establish its efficacy.

Another case http://

cid.oxfordjournals.org/content/31/6/1499.fu
ll