Vascular Malformations
Arteriovenous Malformations
1. True or False. Which of the following statements accurately describes an arteriovenous (AV) fistula? |
| G7 p.1098:70mm |
a. They are low flow, high pressure lesions with a low incidence of hemorrhage. | false |
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b. They are high flow, high pressure lesions with a high incidence of hemorrhage. | false |
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c. They are high flow, high pressure lesions with a low incidence of hemorrhage. | true (Think: counterintuitive: low bleeding rates even though they are high flow and high pressure lesions.) |
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d. They are low flow, low pressure lesions with a high incidence of hemorrhage. | false |
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2. Complete the following about vascular malformations: |
| G7 p.1098:75mm |
a. True or False? A vein of Galen aneurysm is actually: |
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i. an arteriovenous malformation (AVM) | false |
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ii. a cavernous malformation | false |
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iii. an AV fistula | true |
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iv. a venous malformation | false |
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b. Name the other AV fistulas: |
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i. d_____ _____ | dural AVM |
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ii. c_____ c_____ f_____ | carotid cavernous fistula |
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3. True or False. Regarding arteriovenous malformations: |
| G7 p.1098:100mm |
a. AVMs are characterized by dilated arteries and veins with dysplastic vessels, no capillary bed, and no intervening neural parenchyma. | true |
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b. In adulthood, AVMs are medium to high pressure and high flow. | true | |
c. AVMs usually present with seizures, less often with hemorrhage. | false (They usually present with hemorrhage and less often with seizures.) |
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d. These are congenital lesions with a lifelong risk of bleeding of ˜2 to 4% per year. | true |
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4. True or False. The average age of patients diagnosed with AVMs is |
| G7 p.199:18mm |
a. 11 years | false |
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b. 21 years | false |
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c. 33 years | true |
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d. 45 years | false |
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5. True or False. AVMs can |
| G7 p.1099:35mm |
a. cause bleeding | true |
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b. cause seizures | true |
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c. steal blood from surrounding parenchyma | true |
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d. cause heart failure | true |
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e. cause headache | true |
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6. Complete the following about AVMs: |
| G7 p.1099:85mm |
a. What is the peak age for hemorrhage? | 15 to 20 years |
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b. What is the mortality for each bleed? | 10% |
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c. What is the morbidity for each bleed? | 30 to 50% |
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d. What is the average risk of rebleeding per year? | 4% (2 to 4%) |
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e. What is the risk of mortality per year? | 1% |
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f. What is the combined mortality and morbidity per year? | 2.7% |
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7. True or False. Regarding AVMs: |
| G7 p.1099:140mm |
a. Small AVMs tend to present more often as hemorrhages than do large ones. | true (Hint: The little ones bleed more.) |
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b. Small AVMs are less lethal than large ones. | false | G7 p.1099:160mm |
8. True or False. As with aneurysms there is an increased rate of rebleeding with AVMs that have ruptured. | false (Although some say rebleeding rate increases to 6% for every year after bleed, most agree the rates stay the same at 4%/yr.) | G7 p.1100:88mm |
9. True or False. Regarding AVM bleeding rates: |
| G7 p.1100:117mm |
a. Studies suggest a higher risk of bleeding depending on whether the initial presentation was hemorrhage (3.7%/yr) vs seizure (1 to 2%/yr). | true |
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b. The hemorrhage risk may be higher in pediatric or with posterior-fossa AVMs. | true | |
c. The younger the patient at diagnosis, the higher the risk of developing convulsions. | true |
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d. The accepted risk of major bleeding is 6% per year. | false (The accepted risk of major bleeding is 4% per year. A study of 166 symptomatic AVMs with long average follow-up found the risk of major bleeding was constant at 4% per year.) |
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10. What is the risk of bleeding (at least once) from an AVM during the lifetime of a 35-year-old healthy male, assuming a 3% annual bleeding risk? | 73% | G7 p.1100:90mm |
11. Complete the following about AVMs: |
| G7 p.1100:165mm |
a. True or False. 11% of patients with AVM have aneurysms. | false (7% of patients with AVMs have aneurysms.) |
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b. Aneurysms associated with AVMs usually arise from a _____artery. | feeding (75%) |
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c. If it is not clear which bled, the AVM or the aneurysm, it is usually the _____. | aneurysm |
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d. Do aneurysms regress after AVM removal? | yes (66%) |
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12. Matching. Match the pathology and the numbered magnetic resonance imaging (MRI) criterion. |
| G7 p.1101:70mm |
tumor; AVM |
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a. flow void on T1-weighted imaging (T1WI) or T2-weighted imaging (T2WI) |
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b. feeding arteries |
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c. edema |
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d. draining veins |
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e. complete ring of low density surrounding the lesion |
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13. Which magnetic resonance (MR) sequence best shows hemosiderin? | gradient echo | G7 p.1101:95mm |
14. Complete the following about AVMs: |
| G7 p.1101:105mm |
a. Presence of edema can help differentiate AVM from _____. | tumor (Edema is more likely in tumors.) |
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b. True or False. A hemosiderin ring may suggest an AVM rather than a neoplasm. | true (The AVM may have bled in the past, whereas hemosiderin ring in tumors is rare.) |
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15. True or False. Criteria for the Spetzler-Martin grading of AVMs include the following: |
| G7 p.1101:100mm |
Hint: SED size eloquence drainage |
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a. presence of associated aneurysm | false |
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b. size | true |
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c. pattern of venous drainage | true |
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d. eloquence of adjacent brain | true | |
16. Complete the following about AVMs: |
| G7 p.1101:161mm |
a. True or False. The Spetzler-Martin grade of a 4 cm AVM that drains into the vein of Galen and is located in the visual cortex is |
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i. grade 1 | false |
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ii. grade 2 | false |
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iii. grade 3 | false |
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iv. grade 4 | true (size 4 cm = 2, eloquence = 1 drainage, deep = 1) |
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v. grade 5 | true |
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b. and has a morbidity rate of _____% | 27% |
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i. of which _____% is minor | 20% |
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ii. and _____% is major | 7% |
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17. Complete the following about AVMs: |
| G7 p.1101:102mm |
a. Using the Spetzler-Martin AVM grading system, what grade is an AVM located in the visual cortex of a 38-year-old man that has a nidus measuring 2.5 cm in diameter and shows on angiogram high flow and drainage into a cortical vein? | size: <3.0 cm size 1 point, eloquent brain 1 point, superficial venous drainage – 0 points = 2 points = grade 2 |
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b. We expect a minor deficit of _____% | 5% |
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c. and a major deficit of _____%. | 0% |
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18. Complete the following about AVMs: |
| G7 p.1101:103mm |
a. True or False. An AVM that lies over the left motor cortex, is 5.9 cm, and drains superficially is a Spetzler-Martin grade: |
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i. 6 | false |
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ii. 3 | true (size: 3 to 6 cm 2 points, for eloquent area 1 point, for superficial drainage 0 points; 3 = 3 points) |
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iii. 4 | false |
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iv. 1 | false |
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b. and has a morbidity of _____% | 16% |
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i. minor being _____% | 12% |
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ii. major being _____% | 4% |
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19. True or False. Regarding AVMs: |
| G7 p.1102:54mm |
a. Conventional radiation is effective in less than 20% of cases. | true |
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b. Stereotactic radiosurgery eliminates the risk of bleeding almost immediately. | false (Stereotactic radiosurgery takes 1 to 3 years to work, during which the patient is still at risk of bleeding from the AVM.) |
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c. Surgery eliminates the risk of bleeding almost immediately. | true |
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d. Stereotactic radiosurgery should be considered for small AVMs in eloquent cortex. | true | |
20. Complete the following about embolization of AVM: |
| G7 p.1102:110mm |
a. Does not permanently _____ AVMs | obliterate |
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b. Does _____ surgery | facilitate |
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c. Induces acute _____ changes | hemodynamic |
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d. May require _____ procedures | multiple |
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e. Embolization prior to stereotactic |
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i. Radiosurgery _____ obliteration rate | reduces |
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ii. From _____ to _____% | 70 to 47% |
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21. True or False. Endovascular embolization is usually adequate by itself to treat |
| G7 p.1102:155mm |
a. conventional AVMs | false (Embolization alone is inadequate to treat AVMs.) |
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b. direct fistulas | true (It is usually adequate to primarily treat direct fistulas without the use of other methods such as surgery and stereotactic radiosurgery.) |
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22. What can be predicted about the 2-year result from the 6-month angiographic assessment after embolization? |
| G7 p.1103:110mm |
a. If no residual is seen, it will _____. | also not be seen at 2 years |
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b. If residual is seen, it will _____. | not progress to obliteration; that is, the AVM will not progress on its own to obliteration in 2 years |
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23. What pretreatment can be used to reduce the incidence of perfusion pressure breakthrough? | propranolol 20 mg four times a day for 3 days | G7 p.1103:125mm |
24. True or False. Propranolol used for 3 days prior to AVM resection can minimize the incidence of postop normal perfusion pressure breakthrough. | true | G7 p.1103:125mm |
Venous Angiomas
25. True or False. Regarding venous angiomas: |
| G7 p.1104:133mm |
a. They are usually demonstrable on angiography as a starburst pattern. | true |
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b. Typically seizures are rare. | true | |
c. Typically hemorrhage is rare. | true |
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d. Surgery is usually indicated to prevent bleeding. | false (Surgery is not indicated to prevent bleeding. Surgery is very rarely indicated. Surgery may be considered for documented bleeding or for intractable seizures definitely attributed to the lesion.) |
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26. True or False. Neural parenchyma is not found between the vessels of a venous angioma. | false | G7 p.1104:130mm |
27. True or False. Venous angiomas require prompt surgical attention. | false (Venous angiomas require no treatment.) | G7 p.1104:130mm |
28. True or False. Venous angiomas are low flow, low pressure lesions. | true | G7 p.1104:130mm |
Angiographically Occult Vascular Malformations (AOVMs)
29. True or False. The incidence of angiographically occult vascular malformations (AOVMs) among all cerebrovascular malformations is |
| G7 p.1105:120mm |
a. 2% | false |
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b. 5% | false |
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c. 10% | true |
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d. 4% | false |
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30. True or False. Angiographically occult vascular malformations most often present with hemorrhage. | false (seizures or headache) | G7 p.1105:130mm |
31. True or False. The following cerebrovascular malformations are the most common angiographically occult vascular malformations: |
| G7 p. 1105:170mm |
a. venous angioma | false |
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b. capillary telangiectasia | false |
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c. cavernous angioma | false |
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d. arteriovenous malformation | true (Arteriovenous malformation is the most common angiographically occult vascular malformations [AOVM]. AVM 44 to 60%; cavernous angioma 19 to 31%; venous angioma 9 to 10%; telangiectasias 4 to 12%; mixed or unclassified 11%.) | |
32. True or False. The following vascular malformations contain intervening brain tissue: |
| G7 p.1105:170mm |
a. AVM | false |
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b. venous angioma | true |
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c. cavernous angioma | false |
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d. capillary telangiectasia | true |
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33. True or False. Each of the following syndromes is associated with capillary telangiectasias except: |
| G7 p.1106:30mm |
a. Sturge-Weber | true |
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b. Osler-Weber-Rendu | true |
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c. Louis-Barr | true |
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d. Myburn-Mason | true |
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e. Waardenburg | false |
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Cavernous Malformations
34. True or False. The following is true regarding cavernous malformation: |
| G7 p.1106:140mm |
a. They most often present with seizures. | true |
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b. They are angiographically occult. | true |
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c. They occur more commonly in the brain stem vs supratentorially. | false |
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d. They can occur sporadically or in a hereditary form. | true |
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35. True or False. The following are characteristics of cavernous malformations (CMs): |
| G7 p.1106:140mm |
a. high flow malformation | false |
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b. no intervening brain parenchyma | true |
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c. usually not demonstrable on angiogram | true |
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d. no large draining veins or arteries | true |
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36. True or False. The percentage of central nervous system (CNS) vascular malformations that cavernous malformations represent is |
| G7 p. 1107:45mm |
a. 2% | false |
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b. 20% | false |
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c. 10% | true (10%, quoted prevalence is 5 to 13% of all CNS vascular malformations) |
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d. 1% | false | |
37. Complete the following regarding cavernous malformations (CMs): |
| G7 p.1107:65mm |
a. There are _____ genetic subtypes of CM. | three |
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b. They may present with |
| G7 p.1107:95mm |
i. s_____ in 60% | seizures |
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ii. p_____ n_____ deficit in 50% | progressive neurological |
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iii. h_____ in 20% | hemorrhage |
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iv. i_____ finding in 50% | incidental |
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c. Risk of significant bleeding is |
| G7 p.1107:106mm |
i. _____ to _____% per year. | 2 to 3% |
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ii. True or False. It is higher in females. | true |
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iii. Risk in females is _____%. | 4.2% |
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iv. Risk in males is _____%. | 0.9% |
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v. True or False. Risk is increased by |
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prior bleed | controversial |
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pregnancy | false |
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parturition | false |
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38. Complete the following regarding cavernous malformations: |
| G7 p.1107:160mm |
a. The risk of bleeding is _____. | low (and only rarely significant) |
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b. The best test is _____ _____. | T2WI MRI |
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c. Radiologic appearance is _____. | pathognomonic |
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d. New onset seizures |
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i. may be an indication for _____ _____ | surgical resection |
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ii. because removal before _____ may reduce future seizures. | kindling |
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e. Stereotactic radiosurgery |
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i. may have a limited place in cavernous malformation treatment (True or False.) | true (but very limited) |
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ii. except in r_____ h_____. | recurrent hemorrhage |
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39. True or False. Venous angiomas may be seen adjacent to |
| G7 p.1107:168mm |
a. solitary cavernous malformations | true |
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b. multiple cavernous malformations | false |
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Dural AVM
40. True or False. The most common location of dural AVM is |
| G7 p.1109:132mm |
a. superior sagittal sinus | false |
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b. tentorial | false |
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c. transverse sinus | true |
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d. torcula | false | |
41. True or False. Dural AVMs are most commonly found in |
| G7 p.1109:158mm |
a. men > 40 years of age | false |
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b. men < 40 years of age | false |
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c. women > 40 years of age | true |
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d. women < 40 years of age | false |
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42. Complete the following about dural AVMs: |
| G7 p.1109:170mm |
a. True or False. Etiology is thought to be related to |
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i. trauma | false |
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ii. congenital cause | false |
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iii. thrombosis and revascularization | true |
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iv. chronic infection | true |
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b. True or False. The sinus that is most commonly occluded is |
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i. superior sagittal | false |
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ii. straight | false |
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iii. transverse | false |
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iv. sigmoid | true |
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v. confluens | false |
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c. Which artery is the dominant feeder in most cases? | occipital | G7 p.1109:180mm |
43. True or False. Each of the following is a common presenting sign or symptom of dural AV fistula (AVF), also known as dural AV malformation: |
| G7 p.1110:15mm |
a. hydrocephalus | false |
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b. bruit | true |
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c. headache | true |
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d. tinnitus | true |
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e. visual impairment | true |
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f. papilledema | true |
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g. blindness | true |
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Vein of Galen Malformation
44. True or False. Vein of Galen malformations cause symptoms by |
| G7 p.1112:85mm |
a. causing obstructive hydrocephalus | true |
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b. hemorrhage | false |
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c. congestive heart failure | true |
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d. seizures | false |
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45. Complete the following regarding vein of Galen malformations: |
| G7 p.1112:125mm |
a. If untreated mortality is _____%. | 60 to 100% |
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b. Hydrocephalus usually presents at age _____ _____. | 1 year | G7 p.1112:147mm |
Carotid-Cavernous Fistula
46. Describe Barrow-Spector classification of spontaneous carotid-cavernous fistulas. |
| G7 p.1113:55mm |
a. type 1 _____ | traumatic |
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b. type 2 _____ | spontaneous (Type A = direct high flow shunt between cavernous ICA and cavernous sinus, frequently due to ruptured aneurysm. B = dural shunts between meningeal branches of ICA and cavernous sinus. C = dural shunts between meningeal branches of ECA and cavernous sinus [CS]. D = dural shunts between meningeal branches of ICA and ECA and cavernous sinus.) |
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i. type 2A _____ flow between _____ and _____ | high flow between ICA aneurysms and CS |
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ii. type 2B _____ flow between_____ and _____ | low flow between meningeal branches of ICA and CS |
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iii. type 2C _____ flow between _____ and _____ | low flow between meningeal branches of ECA and CS |
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iv. type 2D _____ flow between _____ and _____ and _____ | low flow between meningeal branches of ICA and ECA and CS |
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47. True or False. The following is an example of a low-flow carotid-cavernous fistula: |
| G7 p.1113:56mm |
a. internal carotid artery (ICA) → cavernous sinus type 2A | false (Direct ICA-cavernous fistulas occur from aneurysmal rupture and are high flow fistulas.) |
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b. ICA meningeal branch → cavernous sinus type 2B | true (Connections between meningeal branches of either ICA or ECA and cavernous sinus are low flow fistulas.) |
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c. external carotid artery (ECA) meningeal branch → cavernous sinus type 2C | true (Connections between meningeal branches of either ICA or ECA and cavernous sinus are low flow fistulas.) |
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48. Complete the following about carotid-cavernous fistulas (CCFs): |
| G7 p. 1113:60mm |
a. What is the frequency in the head trauma patient? | 0.2% |
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b. True or False. Low flow CCFs may thrombose spontaneously. | true (in ˜50% of patients) |
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c. What pain-relieving procedure may produce a CCF as a complication? | percutaneous trigeminal procedures | |
49. True or False. The percentage of patients with craniocerebral trauma that develop carotid-cavernous fistulas is: |
| G7 p.1113:60mm |
a. 0.02% | false |
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b. 0.2% | true |
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c. 2% | false |
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50. True or False. According to Barrow-Spector, a carotid-cavernous fistula that is a low flow shunt between meningeal branches of the external carotid artery and the cavernous sinus is a type |
| G7 p.1113:82mm |
a. 2A | false |
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b. 2C | true |
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c. 2B | false |
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d. 2D | false |
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51. True or False. The following is the most important factor in treating a carotid-cavernous fistula: |
| G7 p.1114:15mm |
a. progressive diplopia | false |
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b. progressive exophthalmos | false |
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c. worsening headaches | false |
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d. progressive visual loss | true (Progressive visual loss is overwhelmingly the most important factor influencing the decision to treat a carotid-cavernous fistula. Diplopia can be ameliorated with frosted glasses, whereas vision loss cannot be ameliorated.) | G7 p.1114:33mm |
52. True or False. Regarding carotidcavernous fistula: |
| G7 p.1114:22mm |
a. Surgery is the treatment of choice for carotid-cavernous fistulas. | false (Endovascular embolization is the treatment of choice.) |
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b. Low flow CCFs thrombose spontaneously |
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i. 80% of the time | false |
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ii. 50% of the time | true |
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iii. 20% of the time | false |
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iv. They don’t thrombose spontaneously. | false |
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