Transcript Orbital Apex Syndrome

Ophthalmic Life and Death
A Case Presentation
Dr Phillip Hayes
Central Coast day Hospital
2012
Clinical history
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34 year old woman
IDDM
Presented initially to GP June 2007
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Presumed migraine
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Nearly constant Headache 6 months around L
temporal
Only partial response to NSAID
Referred Neurology outpatients
Clinical History
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Seen at Neurology OP
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July 2007
Continuous unilateral headache
? Hemicrania
Admitted to Gosford Hosp for further
investigation
MRI / MRV to exclude cortical vein thrombosis
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What is a good way to screen for
Thrombosis Intracranial Venous sinuses
causing headache.
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What is a good way to screen for
Thrombosis Intracranial Venous sinuses
causing headache?
Ophthalmoscopy to exclude Increased ICP
by excluding Papilloedema
Background History
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Type 1 Diabetes Mellitus
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Endometriosis
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Diagnosed at aged 22
Actrapid / Protaphane
Poorly controlled
No documented complications
Microgynon (OCP)
Smoker
Prior history of Migraines
Clinical History
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Admitted Neurology 19/7/07
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? Migraine, ? Venous thrombosis
OP CT scan Brain – no acute intracranial pathology
Treated with paracetamol, NSAID, amitriptyline with
significant improvement
Anxious for discharge
MRI arranged as outpatient with follow-up
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Subsequently did not attend this appointment
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Clinical History
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Re-admitted via ED 24/09/07 (2 months
later)
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Severe L temporal headaches over 2/52
Associated with gradual loss of vision L eye
Extremely unwell
Vomiting
Missed several insulin doses
Examination
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Vomiting, distressed, afebrile,
L temporal tenderness & hyperaesthesia
Impression of L proptosis
L RAPD noted
Acuity R 6/6 and L 6/36
No ophthalmoplegia
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Painful on prolonged L lateral gaze
No nystagmus or diplopia
Examination
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Vomiting, distressed, afebrile,
L temporal tenderness & hyperaesthesia
Impression of L proptosis
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RAPD
noted
L 6/36
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Acuity R 6/6 and
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No ophthalmoplegia
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Painful on prolonged L lateral gaze
No nystagmus or diplopia
RAPD
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An objective sign of vision loss
Localises pathology to between the
Retina and the Optic Chiasm
It compares the quantitative neural signal
between each eye
Media opacity doesn’t produce RAPD
Can occasionally occur with normal VA
Examination
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Vomiting, distressed, afebrile,
L temporal tenderness & hyperaesthesia
Impression of L proptosis
L RAPD noted
Acuity 6/6 R and 6/36 L
No ophthalmoplegia
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Painful on prolonged L lateral gaze
No nystagmus or diplopia
Summary
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Anxious, young, sick, poorly controlled Diabetic
Severe Left Headache
Left eye: reduce vision with RAPD
Do we do LP, MRI, look at the retina or call an
Ophthalmologist?
Further Examination
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Ophthalmology review
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Confirmed RAPD
Possible Left Proptosis
No ophthalmoplegia
Normal optic discs
Narrow arterioles, no diabetic retinopathy
No retinal pathology
Remainder of Cranial Nerves and PN exam
normal
What next?
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What is needed for diagnosis.
What next?
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What is needed for diagnosis.
Urgent Neuro imaging and basic blood
work.
Should we start treatment??
Initial Management
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Commenced on Insulin dextrose infusion
Opiate analgesia to no effect
Several IV anti-emetics
Investigations
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pH 7.41 (Normal), trace ketonuria
Biochemistry – unremarkable
WCC 12.8
CRP 37, ESR 25
TFT normal, CK 22
HbA1C 16%
t
Imaging
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CT Brain & orbits
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Diffuse swelling of muscles of L orbit
Inflammatory changes at orbital apex
Probable compression of optic nerve
MRI
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Inflammation of extra-ocular muscles and soft
tissue of L orbital apex
Orbital Inflammation
Graves
50%
Infection
45%
Idiopathic
(Pseudotumour)
• Thyroid Eye Disease (Throid
Orbitopathy)
• Orbital Cellulitis
• Non Specific Orbital inflammation
• Systemic
Idiopathic Orbital inflammation
(pseudotumour)
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Pain + proptosis, injection, chemosis,
ophthalmoplegia
Individual muscles, trochlea, or lacrimal gland
Makes up 5% of orbital conditions
Tendons usually involved( vs thyroid eye disese)
Idiopathic or associated condition with systemic
Idiopathic Orbital inflammation
(pseudotumour)
Idiopathic Orbital inflammation
(pseudotumour)
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May need biopsy to exclude other
pathology but findings usually non-specific
Trial of steroids with rapid resolution is
supportive
Other immunosuppressive therapies
Idiopathic Orbital inflammation
(pseudotumour)
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A Subgroup of these diverse group of
conditions is localised to the orbital apex
Termed “ The Orbital Apex Syndrome”
The orbital apex
Orbital Apex Syndrome
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Headache
Peri-orbital / facial pain
Minimal Proptosis
Reduced visual acuity
RAPD
Diplopia, Field defect
Ophthalmoplegia
Optic atrophy (not seen early)
Orbital Apex Syndrome
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Orbital apex
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Entry portal for all nerves & vessels to eye &
origin of all extraocular muscles except
inferior oblique
Syndrome involving dysfunction of
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Optic nerve
III, IV, VI, V1
Radiological evidence of inflammation in
posterior orbit
Orbital Apex Syndrome
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This is the most likely diagnosis in this
diabetic woman.
However, it is not really a diagnosis but a
description of a clinical syndrome.
Orbital Apex Syndrome
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Inflammatory
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Infectious
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Fungi – Mucormycosis, Aspergillosis
Strep, staph, actinomyces, anaerobes, TB
Neoplastic
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Sarcoid, SLE, Wegner’s, Churg-straus, GCA
Tolosa-hunt syndrome
Idiopathic Orbital inflammation
Dysthyroid eye disease
Lymphoma, nasopharyngeal ca
Iatrogenic / Trauma
Vascular
Orbital Apex Syndrome
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Infection
Something that responds to Systemic
Steriods
Something that can kill you rapidly like
Murcomycosis
Something else like a tumour
Mucormycosis
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Zygomycetes
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Rhinocerebral mucormycosis
Pulmonary, GI, Renal, Cutaneous, CNS
Broad, irregularly branched, with few septa
Thrive in acidic high glucose media
Predisposed hosts
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Diabetes
Immunosuppressed
IVDU
Iron overload
Mucormycosis
Zygomycetes fungus
Mucormycosis
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Zygomycetes
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Rhinocerebral mucormycosis
Pulmonary, GI, Renal, Cutaneous, CNS
Broad, irregularly branched, with few septa
Thrive in acidic high glucose media
Predisposed hosts
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Diabetes
Immunosuppressed
IVDU
Iron overload
Mucormycosis
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Inhalation of spores to paranasal sinuses of
susceptible host
Infarction and necrosis with vascular invasion
Usually aggressive - very fast pace
Typically in a diabetic with DKA (70%)
Mucormycosis
Mucormycosis
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Enter the orbit via ethmoid or maxillary sinus
Often febrile
Orbital cellulitis picture
Mucormycosis
Orbital Mucormycosis
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Vision loss
Headache
Neurological symptoms
Infection progresses to the orbital apex,
cavernous sinus and brain
Mucormycosis
Mucormycosis
Mucormycosis
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Diagnosis
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Sinus inspection and biopsy of sinus or orbit along
with washings
Direct microscopy or histopath or culture
Imaging to identify degree of adjacent tissue
involvement
Treatment
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Aggressive surgical debridement (orbital exenteration)
Amphotericin IV
Optimize metabolic factors
Mucormycosis
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Prognosis
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Overall mortality of 25-50% in rhinocerebral
mucormycosis
Delayed diagnosis and advanced or extensive
disease lead to increased mortality
Pulmonary involvement - 80% mortality
Further Progress
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After urgent CT and MRI she was
reviewed by Ophthalmologist.
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ENT review same night
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Dx “orbital apex syndrome” with need to
exclude mucormycosis in view of Diabetic
status.
Sinuses grossly normal, no evidence mucor in
naso-pharynx
Transferred to Westmead Hospital for
orbital and sinus biopsy
Westmead
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Biopsy of L posterior ethmoidal sinus & L orbital soft
tissue
Commenced steroids and anti-fungals
Westmead
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Biopsy of L posterior ethmoidal sinus & L orbital soft
tissue
Commenced steroids and anti-fungals
Biopsy
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Non-specific inflammation,
No granulomata
No fungal hyphae. No positive culture
Westmead
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Biopsy of L posterior ethmoidal sinus & L orbital soft
tissue
Commenced steroids and anti-fungals
Biopsy
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Non-specific inflammation,
No granulomata
No fungal hyphae. No positive culture
Anti-fungals ceased
Discharged on tapering regime high dose steriods
Acuity 6/6 L eye
F/U with ophthalmology & endocrine
Non Specific Orbital Apex
Syndrome
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Diabetes was not related to her vision loss.
She responded well to Systemic steriods.
The optic neuropathy was probably compressive but
may have been peri-neuritic.
Apart from her symptoms and radiology there was not
much clinical information except subjective and objective
vision loss.
Non Specific Orbital Apex
Syndrome
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Diabetes was not related to her vision loss.
She responded well to Systemic steriods.
The optic neuropathy was probably compressive but
may have been peri-neuritic.
Apart from her symptoms and radiology there was not
much clinical information except subjective and objective
vision loss.
Which of the folllowing can
cause a RAPD?
a)
b)
c)
d)
Dense unilateral Cataract
Dense Vitreous Haemorrhage
Temporal Retinal Detachment
All the above
Which of the folllowing can
cause a RAPD?
a)
b)
c)
d)
Dense unilateral Cataract
Dense Vitreous Haemorrhage
Temporal Retinal Detachment
All the above
In orbital apex syndrome a
common finding is
a)
b)
c)
d)
Vision loss
Proptosis
Ptosis
Lid retraction
In orbital apex syndrome a
common finding is
a)
b)
c)
d)
Vision loss
Proptosis
Ptosis
Lid retraction
The most common Orbital
inflammation is
a)
b)
c)
d)
Orbital Cellulitis
Orbital Pseudotumour
Tolosa Hunt Syndrome
Thyroid Orbitopathy
The most common Orbital
inflammation is
a)
b)
c)
d)
Orbital Cellulitis
Orbital Pseudotumour
Tolosa Hunt Syndrome
Thyroid Orbitopathy
A useful test to screen for raised
intracranial pressure is
a)
b)
c)
d)
Consensual pupillary light reflex
MRI/ MRV
Fundus Fluorescein Angiogram
Bimicroscopy of optic nerve head
A useful test to screen for raised
intracranial pressure is
a)
b)
c)
d)
Consensual pupillary light reflex
MRI/ MRV
Fundus Fluorescein Angiogram
Bimicroscopy of optic nerve head
A diabetic patient that is unwell
with eye pain and swelling and
sudden vision loss could have
a)
b)
c)
d)
Mucormycosis
Dense Vitreous haemorrage
Hypogylcaemic crisis
All of the above
A diabetic patient that is unwell
with eye pain and swelling and
sudden vision loss could have
a)
b)
c)
d)
Mucormycosis
Dense Vitreous haemorrage
Hypogylcaemic crisis
All of the above
Thank you
Tolosa-Hunt syndrome
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Idiopathic inflammatory granulomatous
process of cavernous sinus
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Unilateral orbital pain
Opthalmoplegia (III,IV,VI,)
Rare 1 per million per year
Inflammation may extend beyond CS
Optic disc oedema or pallor reported but
loss of visual acuity rare
Tolosa-Hunt syndrome
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Steroid responsive(usually in 72 hours for
pain) but permanent deficits can occur
and relapse common
Low index of suspicion for misdiagnosis
even if response to steroids
Lymphoma, vasculitis, and some infections
will respond
Diagnosis of exclusion