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

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Presentation on theme: "Ophthalmic Life and Death A Case Presentation Dr Phillip Hayes Central Coast day Hospital 2012."— Presentation transcript:

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

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

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

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

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

7 Clinical History Admitted Neurology 19/7/07 Admitted Neurology 19/7/07 ? Migraine, ? Venous thrombosis ? Migraine, ? Venous thrombosis OP CT scan Brain – no acute intracranial pathology OP CT scan Brain – no acute intracranial pathology Treated with paracetamol, NSAID, amitriptyline with significant improvement Treated with paracetamol, NSAID, amitriptyline with significant improvement Anxious for discharge Anxious for discharge MRI arranged as outpatient with follow-up MRI arranged as outpatient with follow-up Subsequently did not attend this appointment Subsequently did not attend this appointment

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

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

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

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12 RAPD An objective sign of vision loss An objective sign of vision loss Localises pathology to between the Retina and the Optic Chiasm Localises pathology to between the Retina and the Optic Chiasm It compares the quantitative neural signal between each eye It compares the quantitative neural signal between each eye Media opacity doesn’t produce RAPD Media opacity doesn’t produce RAPD Can occasionally occur with normal VA Can occasionally occur with normal VA

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

14 Summary Anxious, young, sick, poorly controlled Diabetic Anxious, young, sick, poorly controlled Diabetic Severe Left Headache Severe Left Headache Left eye: reduce vision with RAPD Left eye: reduce vision with RAPD Do we do LP, MRI, look at the retina or call an Ophthalmologist? Do we do LP, MRI, look at the retina or call an Ophthalmologist?

15 Further Examination Ophthalmology review Ophthalmology review Confirmed RAPD Confirmed RAPD Possible Left Proptosis Possible Left Proptosis No ophthalmoplegia No ophthalmoplegia Normal optic discs Normal optic discs Narrow arterioles, no diabetic retinopathy Narrow arterioles, no diabetic retinopathy No retinal pathology No retinal pathology Remainder of Cranial Nerves and PN exam normal Remainder of Cranial Nerves and PN exam normal

16 What next? What is needed for diagnosis. What is needed for diagnosis.

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

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

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

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21 t

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

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

26 Idiopathic Orbital inflammation (pseudotumour) Pain + proptosis, injection, chemosis, ophthalmoplegia Pain + proptosis, injection, chemosis, ophthalmoplegia Individual muscles, trochlea, or lacrimal gland Individual muscles, trochlea, or lacrimal gland Makes up 5% of orbital conditions Makes up 5% of orbital conditions Tendons usually involved( vs thyroid eye disese) Tendons usually involved( vs thyroid eye disese) Idiopathic or associated condition with systemic Idiopathic or associated condition with systemic

27 Idiopathic Orbital inflammation (pseudotumour)

28 May need biopsy to exclude other pathology but findings usually non-specific May need biopsy to exclude other pathology but findings usually non-specific Trial of steroids with rapid resolution is supportive Trial of steroids with rapid resolution is supportive Other immunosuppressive therapies Other immunosuppressive therapies

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

30 The orbital apex

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

32 Orbital Apex Syndrome Orbital apex Orbital apex Entry portal for all nerves & vessels to eye & origin of all extraocular muscles except inferior oblique Entry portal for all nerves & vessels to eye & origin of all extraocular muscles except inferior oblique Syndrome involving dysfunction of Syndrome involving dysfunction of Optic nerve Optic nerve III, IV, VI, V 1 III, IV, VI, V 1 Radiological evidence of inflammation in posterior orbit Radiological evidence of inflammation in posterior orbit

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

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

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

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

38 Mucormycosis Zygomycetes fungus

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

40 Mucormycosis Inhalation of spores to paranasal sinuses of susceptible host Inhalation of spores to paranasal sinuses of susceptible host Infarction and necrosis with vascular invasion Infarction and necrosis with vascular invasion Usually aggressive - very fast pace Usually aggressive - very fast pace Typically in a diabetic with DKA (70%) Typically in a diabetic with DKA (70%)

41 Mucormycosis

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

43 Mucormycosis

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

45 Mucormycosis

46 Mucormycosis

47 Mucormycosis Diagnosis Diagnosis Sinus inspection and biopsy of sinus or orbit along with washings Sinus inspection and biopsy of sinus or orbit along with washings Direct microscopy or histopath or culture Direct microscopy or histopath or culture Imaging to identify degree of adjacent tissue involvement Imaging to identify degree of adjacent tissue involvement Treatment Treatment Aggressive surgical debridement (orbital exenteration) Aggressive surgical debridement (orbital exenteration) Amphotericin IV Amphotericin IV Optimize metabolic factors Optimize metabolic factors

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

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

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

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

53 Westmead Biopsy of L posterior ethmoidal sinus & L orbital soft tissue Biopsy of L posterior ethmoidal sinus & L orbital soft tissue Commenced steroids and anti-fungals Commenced steroids and anti-fungals Biopsy Biopsy Non-specific inflammation, Non-specific inflammation, No granulomata No granulomata No fungal hyphae. No positive culture No fungal hyphae. No positive culture Anti-fungals ceased Anti-fungals ceased Discharged on tapering regime high dose steriods Discharged on tapering regime high dose steriods Acuity 6/6 L eye Acuity 6/6 L eye F/U with ophthalmology & endocrine F/U with ophthalmology & endocrine

54 Non Specific Orbital Apex Syndrome Diabetes was not related to her vision loss. Diabetes was not related to her vision loss. She responded well to Systemic steriods. She responded well to Systemic steriods. The optic neuropathy was probably compressive but may have been peri-neuritic. 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. 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. Diabetes was not related to her vision loss. She responded well to Systemic steriods. She responded well to Systemic steriods. The optic neuropathy was probably compressive but may have been peri-neuritic. 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. 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? a) Dense unilateral Cataract b) Dense Vitreous Haemorrhage c) Temporal Retinal Detachment d) All the above

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

58 In orbital apex syndrome a common finding is a) Vision loss b) Proptosis c) Ptosis d) Lid retraction

59 In orbital apex syndrome a common finding is a) Vision loss b) Proptosis c) Ptosis d) Lid retraction

60 The most common Orbital inflammation is a) Orbital Cellulitis b) Orbital Pseudotumour c) Tolosa Hunt Syndrome d) Thyroid Orbitopathy

61 The most common Orbital inflammation is a) Orbital Cellulitis b) Orbital Pseudotumour c) Tolosa Hunt Syndrome d) Thyroid Orbitopathy

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

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

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

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

66 Thank you

67 Tolosa-Hunt syndrome Idiopathic inflammatory granulomatous process of cavernous sinus Idiopathic inflammatory granulomatous process of cavernous sinus Unilateral orbital pain Unilateral orbital pain Opthalmoplegia (III,IV,VI,) Opthalmoplegia (III,IV,VI,) Rare 1 per million per year Rare 1 per million per year Inflammation may extend beyond CS Inflammation may extend beyond CS Optic disc oedema or pallor reported but loss of visual acuity rare 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 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 Low index of suspicion for misdiagnosis even if response to steroids Lymphoma, vasculitis, and some infections will respond Lymphoma, vasculitis, and some infections will respond Diagnosis of exclusion Diagnosis of exclusion


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