Nerrs neuro 2013 answers

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Nerrs neuro 2013 answers

  1. 1. Unknown cases Amy F Juliano, MDMassachusetts Eye and Ear Infirmary Harvard Medical School
  2. 2. 41 year-old woman with 5-yr history ofright ear blockage and intermittent pain
  3. 3. Diagnosis?
  4. 4. Sympathetic chain schwannoma
  5. 5. Sympathetic chain• Travels up the neck in the carotid sheath• Continues up through the carotid canal• Forms plexus on ICA
  6. 6. Schwannomas in the head and neck• Most common – Vagus >> sympathetic chain• Vagus – dysphagia, hoarseness Sympathetic chain – Horner’s• In most cases, patients present with a palpable mass or have no symptoms
  7. 7. • Vagus - Lateral to ICA • Sympathetic chain, cervical sympathetic ganglion - Posteromedial to ICA
  8. 8. Treatment• Observation• Surgery – Post-operative Horner’s – First bite syndrome• First bite syndrome – Pain in the parotid area on the first bite of food – Intense ear pain increased with strong sialogogues – Due to loss of sympathetic input to parotid after severing the chain – Denervation hypersensitivity
  9. 9. 43 year-old man with seizures
  10. 10. Diagnosis?
  11. 11. Multichannel dural AVF of left sigmoid-transverse sinus Venous infarction with vasogenic edema
  12. 12. Dural AVF• Abnormal connection between dural arteries or pachymeningeal branches of cerebral arteries and dural veins• Arteries can be recruited from branches of both the ECA and ICA• Venous drainage can occur through large dural venous sinuses, and retrogradely through cortical cerebral veins• If cortical cerebral veins are involved, there is a higher risk of rupture and hemorrhage from the dAVF• Frequently located in the transverse and sigmoid sinuses
  13. 13. Dural AVF• Believed to result from thrombosis of a dural venous sinus, with subsequent collateral revascularization• Leads to venous hypertension, an initiating factor opening up microscopic vascular connections within the dura• Results in abnormal fistulous connection between arteries and veins in the walls of a dural sinus or involving an adjacent cortical vein
  14. 14. Dural AVF• Venous hypertension may be related to thrombosed dural venous sinus or arterialized veins – Pulse synchronous bruit – Pulsatile tinnitus – Headache – Visual impairment – Papilledema – Venous infarct – Cerebral hemorrhage (SAH, SDH, parenchymal)
  15. 15. Dural AVF• Borden classification system – Type I: antegrade drainage through a dural venous sinus or meningeal vein (usually benign clinical behavior) – Type II: antegrade drainage into dural venous sinus and retrograde flow into cortical veins (may present with hemorrhage) – Type III: direct retrograde flow into cortical veins (causes venous hypertension with risk of hemorrhage)
  16. 16. Dural AVF• Cognard system – Type I: normal antegrade flow into a dural venous sinus – Type IIa: drainage into a sinus with retrograde flow within the sinus – Type IIb: drainage into a sinus with retrograde flow into cortical vein(s) – Type II a+b: drainage into a sinus with retrograde flow within the sinus and cortical vein(s) – Type III: direct drainage into a cortical vein without venous ectasia – Type IV: direct drainage into a cortical vein with ectasia > 5 mm and 3x larger than the diameter of the draining vein – Type V: direct drainage into spinal perimedullary veins
  17. 17. Dural AVF• Treatment is indicated in aggressive cases, typically those showing cortical venous reflux on angiography• Treatment options include surgical and endovascular approaches, or occasionally radiation
  18. 18. 15 year-old boy with neck mass
  19. 19. Follow-up US 3 weeks later
  20. 20. Follow-up CT 2 months after initial CT
  21. 21. Diagnosis?
  22. 22. Pyriform sinus fistulae
  23. 23. • Acute suppurative thyroiditis and thyroid abscess are extremely rare.• Acute suppurative neck infections are frequently recurrent when associated with branchial fistulas (3rd or 4th)• When an inflammatory infiltration or abscess is present between the pyriform fossa and the thyroid bed in the lower neck, esp on L, an infected 3rd or 4th branchial fistula must be suspected
  24. 24. Pyriform sinus fistula
  25. 25. Pyriform sinus fistula• The 3rd & 4th branchial pouches form the pyriform sinus• Persistent ducts from either of these pouch sinuses may drain into the pyriform sinus• There are authors suggesting that there is wide discrepancy between clinical/radiologic presentations and the theoretical course of the 3rd and 4th branchial arch anomalies• They propose that persistence of the thymopharyngeal duct of the 3rd pouch, frequently passing through or adjacent to the thyroid gland, most often on the L side, is the more suitable explanation embryologically
  26. 26. Pyriform sinus fistula• >80% of cases are left-sided• >60% of cases occurred after acute URI• High rate of recurrence• Children or young adults• Pathway: pyriform sinus apex, anteroinferiorly through the strap muscle layer, beside or through the thyroid gland, into perithyroidal space
  27. 27. Pyriform sinus fistula• CT is preferred imaging modality – can show air in sinus or fistulous tract, thyroid gland involvement by loss of normal high density, subtle infiltration or stranding• Barium esophagram during active infection often do not show the sinus or fistulous tract, perhaps because of closure of the tract due to regional inflammation and edema• Perform barium swallow after infection has cleared
  28. 28. 46 year-old man with acute onset of vertigo
  29. 29. Diagnosis?
  30. 30. Endolymphatic sac tumor
  31. 31. Endolymphatic Sac Tumor• Papillary epithelial neoplasm involving the endolymphatic sac or duct• Also been termed CPA ceruminoma, adenocarcinoma, papillary adenomatous tumor, etc• Most occur sporadically; association with von Hippel Lindau• Papillary adenomatous architecture, areas of hemorrhage, hemosiderin, cholesterol clefts, giant cell reactions• Slow-growing, may recur locally• Involves posterior edge of petrous bone, frequently involve dura
  32. 32. Endolymphatic Sac Tumor• Hypervascular, locally-invasive, bone- destroying; can have reactive new bone formation• CT: geographic, moth-eaten, intratumoral bone reticular or spiculated, thin rim of calcification• MR: heterogeneous signal, areas of high signal on T1-weighted sequence• Late presentation: unilateral hearing loss, vestibular dysfunction; facial nerve palsy when tumor becomes large• Duration of hearing loss 6 months to 18 years

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