Neuroophth emergencies mds 2-new

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Neuroophth emergencies mds 2-new

  1. 1. Neuro-Ophthalmic Emergencies Emergencies Raed Behbehani , MD FRCSC
  2. 2. What is an emergency ? • Vision threatening ? • Life threatening ? • Recognition. • Proper investigations/imaging study. • Appropriate referral.
  3. 3. Painful Diplopia
  4. 4. Case • A 53 year old patient with acute diplopia. • Previous episodes few years ago , which lasted two months and recovered. • Diabetes, and hyprlipedemia. • Visual acuity : 20/20 OU. • Pupils : Equally reactive , pupils equal in size.
  5. 5. Case
  6. 6. Pupil-Sparing Third Nerve Palsy • Diabetes, hypertension, hyperlipedemia, smoking, high hematocrit. • Pupils is spared. • Pupil involvement reported only in 14%-32% , but anisocoria (difference in pupil size) is less than 1 mm (relative-sparing). • Improve within 4-12 weeks (defer neuro-imaging).
  7. 7. Case • 78 year old man with acute diplopia, and headache. • Diabetes, hypertension, atrial tachycardia. • Prior history of tight feeling around the eye with 20 seconds of diplopia. • No history of jaw claudication or transient visual loss.
  8. 8. Pupil-involving 3rd Nerve Palsy • Pupil involvement indicates compression of the pupillary fibers. • Posterior communicating artery aneurysm, or mass. • Appropriate neuro-imaging is (MRI/MRA, MRI/CTA,Angiogram is the gold standard for aneurysm detection).
  9. 9. Risk of Aneurysm and “Rule of Pupil” Ophthalmoplegia Pupil Aneurysm Risk Complete/Partial Complete 86%-100% Partial Spared 30% Complete Spared very low If signs of sub-arachnoid hemorrhage present (headache, photophobia, nausea) “rule
  10. 10. Painful Ptosis and Anisocoria
  11. 11. Case • A 67 year old man presents with pain in his right eye for 5 days. • Hypertension and ischemic heart disease on treatment. • No double vision. • VA : 20/30 OU. • Mild nuclear sclerosis cataracts. • Fundus: normal.
  12. 12. Case
  13. 13. Evaluation of Horner’s • Misois, and ptosis (upper and lower lid). • Dilatation lag, anisocoria worse in dark. • Cocaine test. • Hydroxyamphetamine (not used much). • Iopidine. • MRI/MRA of the head/neck/upper chest CT.
  14. 14. Oculo-sympathetic Pathway
  15. 15. Acute Horner’s Syndrome • Painful Horner’s syndrome is a neurologic emergency. • Although can be seen in many types of headaches (Cluster, Migraine etc). • Rule out ICA dissection. • MRI/MRA of the head/neck/upper mediastinum is indicated.
  16. 16. Horner’s Syndrome (MRI)
  17. 17. ICA dissection • Goal is to prevent secondary neurologic deficit (stroke). • Obsevation ,Anti-coagulation, or stent implantation. • Referral for a neurovasculr specialist.
  18. 18. Acute vision loss in an elderly patient
  19. 19. Case • A 68 year old patient with sudden loss of vision in the right eye. • History of episodes transient loss of vision. • Diabetes for 30 years. • Feeling unwell lately with, and loss of appetite, malaise and myalgias. • Visual acuity: Count finger right , 20/30 left. • Right RAPD.
  20. 20. Case
  21. 21. Case Cord-like STA
  22. 22. Case Investigations • ESR = 86 • CRP positive. • Platelets elevated ( 560). • Mildly anemic.
  23. 23. Arteritic Ischemic Optic Neuropathy • New onset of headache (temporal) , acute or transient loss of vision, jaw claudication, weight loss, fever, and myalgias. • Age usually over 60. • Occult GCA ( No systemic symptoms, transient diplopia or transient visual loss). • A true neuro-ophthalmic emergency (54-95% second eye involvement if untreated) ! • Giant cell arteritis (systemic vasculitis, Aortitis in 20% consider PET/MRA).
  24. 24. GCA Central retinal artery occlusion Branch-retinal artery occlusion
  25. 25. Posterior Ischemic Optic Neuropathy (PION) • Both the retina and optic nerve look normal. • PION is relatively common in Giant Cell arteritis. • Flourescin angiogram can show choroidal hypoperfusion. • Involvement of 2 circulations (systemic vasculitis), retinal artery occlusion and AION indicate giant cell arteritis.
  26. 26. GCA Diplopia (transient, constant)Ophthalmo plegia
  27. 27. AAION Management • Stat ESR , CRP and CBC (platelets). • ESR can be normal in 15-20% of cases. • CRP is more sensitive and specific. • CRP and CBC have 97% sensitivity and specificity. • Start high dose systemic steroids (IV or Oral) immediately upon suspicioun ( AAION can develop in fellow eye within days if untreated !) • Arrange for temporal artery biopsy within 2 weeks , while patient is on steroids.
  28. 28. QuickTime™ and a decompressor are needed to see this picture. Video
  29. 29. TAB
  30. 30. GCA Treatment • Systemic steroids for a at least 1-2 years. • Titrate dose according to laboratory indices (CRP,ESR) and symptoms. • Manage diabetes and osetoporosis. • Collaboration with rheumatologist.
  31. 31. “Worst Headache”
  32. 32. Case • 52-year-old, morbidly obese man presents with severe headache (worst in his life). • Ischemic cardiac disease and angioplasty, COPD, hypertension, and NIDDM. • On examination: complete right ptosiswith unreactive mid-dilated right pupil، left partial ophthalmoplegia with V1 hypesthesia.
  33. 33. Case
  34. 34. Visual Fields
  35. 35. Visual Field Defects in Chiasmal Syndrome
  36. 36. MRI Pituitary mass with high signal on T1
  37. 37. Pituitary Apoplexy • “Worst headache in my life”. • Visual loss, and/or ophthalmoplegia ( uni- or bilateral). • Patients usually present 2 weeks after ictus. • > 80% did not have history of pituitary tumor • Ophthalmoplegia (extension to cavernous sinus with cranial nerve involvement). • Life threatening (hypotension, shock) because of hypo-pituitarism, and low cortisol levels, and diabetes insipidus.
  38. 38. Case • A 50 year old with blurred vision and headache for the last 2 weeks. • Medical History : Diabetes for 5 years. • Smoker 15 years. • No prior Surgeries
  39. 39. Case • Conscious and oriented. • Visual acuity : 20/20 OU • Pupils : PERL no RAPD. • Normal anterior Segment . • Normal ocular motility.
  40. 40. Case
  41. 41. Case • CT and MRI/MRV - normal. • Blood pressure 220/150 !
  42. 42. Malignant Hypertesnion • Accelerated hypertension with target organ damage. • Papilledema must be present for diagnosis ! • Dysfunction of cerebral blood flow autoregultaion causing cerebral edema. • Pre-eclampsia . • Encephalopathy can be present.
  43. 43. Proptosis in immunocompromised or diabetic patient
  44. 44. Case • 60 year old man with myelodysplastic disorder on chemotherapy. • Proptosis, fever, and dyspnea . • Periorbital swelling and erythema, which got worse over 3 days. • Visual acuity : 20/20 Both eyes. • Normal pupils, ocular motility and fundus examination.
  45. 45. Case
  46. 46. CT
  47. 47. Mucormycosis • Vascular thrombosis, tissue necrosis, and fungal dissemination. • The mortality rate is as high as 90%. • Diabetic ketoacidosis , immunosuppressed, organ transplant patients, steroid use, and desferrioxamine. • Other fungal organisms: Aspergillus. • Pain and ophthalmoplegia. • CT of the orbit/paranasal sinuses/cavernous sinus or MRI of the orbit with fat suppression.
  48. 48. Mucor • Immediate biopsy (ENT/Orbit) , with debridement. • Orbital exenteration is not always needed. • Correct any metabolic acidosis to reduce unbound iron (critical for the proliferation of mucor) • Local delivery of amphotericin B with indwelling catheters. • Systemic antifungal (IV liposomal encapsulated Amph B less nephrotoxic +- posaconazole). • Boost immunity (correct neutropenia).
  49. 49. Mucor • Medical therapy and surgical debridement increase the survival rate (78%) compared to medical management alone (57.5%).
  50. 50. Mucormycosis
  51. 51. Mucor Non-septate hyphae with branching at 90 degrees.
  52. 52. Summary • Pupil involvement in 3rd nerve palsy suggests compressive lesion (aneuurysm), get and MRI/MRA or MRI/CTA. • Always rule out ICA dissection in acute Horner’s syndrome. • Always rule out GCA as the etiology for ophthalmoplegia or visual loss in >60 year patients. • In acute severe headache with ophthalmoplegia with multiple CN involvement think of pituitary apoplexy. • Proptosis and eye redness in diabetic/immunospressed patients can be due to life-threatening fungal infection.

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