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Ophthalmic Life and Death A Case Presentation

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

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Ophthalmic Life and Death A Case Presentation

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  1. Ophthalmic Life and DeathA 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 • 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 • 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

  11. 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

  12. 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

  13. 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?

  14. 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

  15. What next? • What is needed for diagnosis.

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

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

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

  19. t

  20. Imaging • CT Brain & orbits • 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

  21. Orbital Inflammation

  22. 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

  23. Idiopathic Orbital inflammation (pseudotumour)

  24. 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

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

  26. The orbital apex

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

  28. Orbital Apex Syndrome • Orbital apex • 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

  29. 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.

  30. Orbital Apex Syndrome • Inflammatory • 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

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

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

  33. Mucormycosis Zygomycetes fungus

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

  35. 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%)

  36. Mucormycosis

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

  38. Mucormycosis

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

  40. Mucormycosis

  41. Mucormycosis

  42. Mucormycosis • Diagnosis • 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

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

  44. 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

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