1. Ophthalmic Life and Death A Case Presentation
Dr Phillip Hayes
Central Coast day Hospital
2012

2. Clinical history 34 year old woman IDDM
Presented initially to GP June 2007
Nearly constant Headache 6 months around L temporal
Presumed migraine
Only partial response to NSAID
Referred Neurology outpatients

3. Clinical History Seen at Neurology OP July 2007
Continuous unilateral headache
? Hemicrania
Admitted to Gosford Hosp for further investigation
MRI / MRV to exclude cortical vein thrombosis

4. What is a good way to screen for Thrombosis Intracranial Venous sinuses causing headache.

5. What is a good way to screen for Thrombosis Intracranial Venous sinuses causing headache?
Ophthalmoscopy to exclude Increased ICP by excluding Papilloedema

6. Background History Type 1 Diabetes Mellitus Endometriosis Smoker
Diagnosed at aged 22
Actrapid / Protaphane
Poorly controlled
No documented complications
Endometriosis
Microgynon (OCP)
Smoker
Prior history of Migraines

7. Clinical History Admitted Neurology 19/7/07
? 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
Subsequently did not attend this appointment

8. Clinical History Re-admitted via ED 24/09/07 (2 months later)
Severe L temporal headaches over 2/52
Associated with gradual loss of vision L eye
Extremely unwell
Vomiting
Missed several insulin doses

9. Examination Vomiting, distressed, afebrile,
L temporal tenderness & hyperaesthesia
Impression of L proptosis
L RAPD noted
Acuity R 6/6 and L 6/36
No ophthalmoplegia
Painful on prolonged L lateral gaze
No nystagmus or diplopia

10. Examination L RAPD noted Vomiting, distressed, afebrile,
L temporal tenderness & hyperaesthesia
Impression of L proptosis
L RAPD noted
Acuity R 6/6 and L 6/36
No ophthalmoplegia
Painful on prolonged L lateral gaze
No nystagmus or diplopia

12. RAPD 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

13. Examination Vomiting, distressed, afebrile,
L temporal tenderness & hyperaesthesia
Impression of L proptosis
L RAPD noted
Acuity 6/6 R and 6/36 L
No ophthalmoplegia
Painful on prolonged L lateral gaze
No nystagmus or diplopia

14. Summary 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?

15. Further Examination Ophthalmology review
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

16. What next?
What is needed for diagnosis.

17. What next? What is needed for diagnosis.
Urgent Neuro imaging and basic blood work.
Should we start treatment??

18. Initial Management Commenced on Insulin dextrose infusion
Opiate analgesia to no effect
Several IV anti-emetics

19. Investigations pH 7.41 (Normal), trace ketonuria
Biochemistry – unremarkable
WCC 12.8
CRP 37, ESR 25
TFT normal, CK 22
HbA1C 16%

21. t

23. Imaging CT Brain & orbits MRI Diffuse swelling of muscles of L orbit
Inflammatory changes at orbital apex
Probable compression of optic nerve
MRI
Inflammation of extra-ocular muscles and soft tissue of L orbital apex

25. Orbital Inflammation Graves 50%
Thyroid Eye Disease (Throid Orbitopathy)
Infection
45%
Orbital Cellulitis
(Pseudotumour)
Idiopathic
Non Specific Orbital inflammation
Systemic

26. Idiopathic Orbital inflammation (pseudotumour)
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

27. Idiopathic Orbital inflammation (pseudotumour)

28. Idiopathic Orbital inflammation (pseudotumour)
May need biopsy to exclude other pathology but findings usually non-specific
Trial of steroids with rapid resolution is supportive
Other immunosuppressive therapies

29. Idiopathic Orbital inflammation (pseudotumour)
A Subgroup of these diverse group of conditions is localised to the orbital apex
Termed “ The Orbital Apex Syndrome”

30. The orbital apex

31. Orbital Apex Syndrome Headache Peri-orbital / facial pain
Minimal Proptosis
Reduced visual acuity
RAPD
Diplopia, Field defect
Ophthalmoplegia
Optic atrophy (not seen early)

32. Orbital Apex Syndrome Orbital apex Syndrome involving dysfunction of
Entry portal for all nerves & vessels to eye & origin of all extraocular muscles except inferior oblique
Syndrome involving dysfunction of
Optic nerve
III, IV, VI, V1
Radiological evidence of inflammation in posterior orbit

33. Orbital Apex Syndrome
This is the most likely diagnosis in this diabetic woman.
However, it is not really a diagnosis but a description of a clinical syndrome.

34. Orbital Apex Syndrome Inflammatory Infectious Neoplastic
Sarcoid, SLE, Wegner’s, Churg-straus, GCA
Tolosa-hunt syndrome
Idiopathic Orbital inflammation
Dysthyroid eye disease
Infectious
Fungi – Mucormycosis, Aspergillosis
Strep, staph, actinomyces, anaerobes, TB
Neoplastic
Lymphoma, nasopharyngeal ca
Iatrogenic / Trauma
Vascular

36. Orbital Apex Syndrome Infection
Something that responds to Systemic Steriods
Something that can kill you rapidly like Murcomycosis
Something else like a tumour

37. Mucormycosis Zygomycetes Broad, irregularly branched, with few septa
Rhinocerebral mucormycosis
Pulmonary, GI, Renal, Cutaneous, CNS
Broad, irregularly branched, with few septa
Thrive in acidic high glucose media
Predisposed hosts
Diabetes
Immunosuppressed
IVDU
Iron overload

38. Mucormycosis
Zygomycetes fungus

39. Mucormycosis Zygomycetes Broad, irregularly branched, with few septa
Rhinocerebral mucormycosis
Pulmonary, GI, Renal, Cutaneous, CNS
Broad, irregularly branched, with few septa
Thrive in acidic high glucose media
Predisposed hosts
Diabetes
Immunosuppressed
IVDU
Iron overload

40. Mucormycosis
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%)

41. Mucormycosis

42. Mucormycosis Enter the orbit via ethmoid or maxillary sinus
Often febrile
Orbital cellulitis picture

43. Mucormycosis

44. Orbital Mucormycosis Vision loss Headache Neurological symptoms
Infection progresses to the orbital apex, cavernous sinus and brain

45. Mucormycosis

46. Mucormycosis

47. Mucormycosis Diagnosis Treatment
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
Aggressive surgical debridement (orbital exenteration)
Amphotericin IV
Optimize metabolic factors

48. Mucormycosis Prognosis
Overall mortality of 25-50% in rhinocerebral mucormycosis
Delayed diagnosis and advanced or extensive disease lead to increased mortality
Pulmonary involvement - 80% mortality

50. Further Progress
After urgent CT and MRI she was reviewed by Ophthalmologist.
Dx “orbital apex syndrome” with need to exclude mucormycosis in view of Diabetic status.
ENT review same night
Sinuses grossly normal, no evidence mucor in naso-pharynx
Transferred to Westmead Hospital for orbital and sinus biopsy

51. Westmead Biopsy of L posterior ethmoidal sinus & L orbital soft tissue
Commenced steroids and anti-fungals

52. Westmead Biopsy of L posterior ethmoidal sinus & L orbital soft tissue
Commenced steroids and anti-fungals
Biopsy
Non-specific inflammation,
No granulomata
No fungal hyphae. No positive culture

53. Westmead Biopsy of L posterior ethmoidal sinus & L orbital soft tissue
Commenced steroids and anti-fungals
Biopsy
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

54. Non Specific Orbital Apex Syndrome
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.

55. Non Specific Orbital Apex Syndrome
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.

56. Which of the folllowing can cause a RAPD?
Dense unilateral Cataract
Dense Vitreous Haemorrhage
Temporal Retinal Detachment
All the above

57. Which of the folllowing can cause a RAPD?
Dense unilateral Cataract
Dense Vitreous Haemorrhage
Temporal Retinal Detachment
All the above

58. In orbital apex syndrome a common finding is
Vision loss
Proptosis
Ptosis
Lid retraction

59. In orbital apex syndrome a common finding is
Vision loss
Proptosis
Ptosis
Lid retraction

60. The most common Orbital inflammation is
Orbital Cellulitis
Orbital Pseudotumour
Tolosa Hunt Syndrome
Thyroid Orbitopathy

61. The most common Orbital inflammation is
Orbital Cellulitis
Orbital Pseudotumour
Tolosa Hunt Syndrome
Thyroid Orbitopathy

62. A useful test to screen for raised intracranial pressure is
Consensual pupillary light reflex
MRI/ MRV
Fundus Fluorescein Angiogram
Bimicroscopy of optic nerve head

63. A useful test to screen for raised intracranial pressure is
Consensual pupillary light reflex
MRI/ MRV
Fundus Fluorescein Angiogram
Bimicroscopy of optic nerve head

64. A diabetic patient that is unwell with eye pain and swelling and sudden vision loss could have
Mucormycosis
Dense Vitreous haemorrage
Hypogylcaemic crisis
All of the above

65. A diabetic patient that is unwell with eye pain and swelling and sudden vision loss could have
Mucormycosis
Dense Vitreous haemorrage
Hypogylcaemic crisis
All of the above

66. Thank you

67. Tolosa-Hunt syndrome
Idiopathic inflammatory granulomatous process of cavernous sinus
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

68. Tolosa-Hunt syndrome
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