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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5776_Библиотеки_им_академика_М_И_Перельмана

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279Discussion
e x t r a c r a n i a l I C A s t e n o s i s >80% or an occlusion was found in 83% versus 40% (Baumgartner et al 2001). Compared with DSA and/or MRA, the combined use of extracranial and intracranial hemodynamically ultrasound param­eters in patients with presumed ICA dissection yielded a diagnostic sensitivity of 96%, specifi city of 94%, and positive and negative predictive values of 92%, and 97%, respectively (Benninger et al 2006).
Therefore, duplex ultrasound of the brain-supplying arteries plays an important part in the initial investiga­tion as well as follow-up of patients with ICA dissection revealing abnormalities in more than 90% of cases, mostly of hemodynamic character. A combination of unilateral high-grade stenosis, absence of atherosclerosis (seen in 80–90% of dissections), and the young age of the aff ect- ed patient make the diagnosis of an ICA dissection very likely. However, if atherosclerosis is present, the use of ultrasound carries the risk of overlooking a dissection and presuming an atherosclerotic stenosis instead.
In young patients with clinically suspected dissection who did not have ischemic stroke, the incidence of sten­oses or occlusions is lower. This implies that further radi­ologic diagnostics must be performed even if ultrasound ndings are normal.
Our case of unilateral distal extracranial ICA stenosis illustrates the diagnostic diffi culties in evaluating the craniocervical region. Ultrasonography is a well-estab­lished method for the evaluation of the extracranial ICA at the level of the carotid bifurcation as these vessel seg­ments can be visualized directly. The combination with hemodynamic parameters permits excellent determina­tion of grades of stenosis. However, analysis of the distal extracranial ICA, which lies deep to the mandibular angle, may cause considerable problems. In addition, the ICA often follows an elongated vessel course. Evaluation has therefore often to rely on the assessment of indirect he­modynamic parameters. In our case, these were the high pulsatility of the CCA and proximal ICA with well-pre­served diastolic fl ow, which indicated a distal fl ow ob- struction. Direct imaging of the more distal ICA can be attempted using the linear transducer in an axial plane pointing toward the base of the skull, or a 2-MHz TCCS probe using the same approach, neither of which was done in our case. Another technique that can be used to visualize the distal ICA is the transoral approach, with a 5–9-MHz convex array transducer (Kishikawa et al 2002, Yasaka et al 1998).
Conventional angiography has long been the gold stand­ard in the diagnosis of arterial dissections, since it can show the arterial lumen and allows extensive characterization of carotid and vertebral arteries. The most common fi nding in ICA dissection is the smooth or irregularly tapered midcer­vical stenosis (string sign) or occlusion. A dissection may be assumed if a rat-tail or fl ame-shaped occlusion is present. Dissecting aneurysm is a further typical sign. Pathogno­monic features such as an intima fl ap or a double lumen are rarely detected. Pelkonen and coworkers found that most of their patients had irregular stenoses (47%), followed by occlusions (29%), dissecting aneurysms (17%), or irregular
(Pelkonen et al 2003). The main problem of catheter angiog­raphy is its invasiveness. In high-risk populations it carries
a 4% risk of causing a permanent neurologic defi cit (for fur- ther discussion, see Case 24). Today, DSA has to be regarded as a second-line method, not only because of the above lim­itations but also because alternative and less invasive meth­ods yielding similar or even greater diagnostic accuracy are available.
MRI in combination with vascular ultrasound is now mostly replacing conventional angiography in the di­agnosis and follow-up of dissections of the carotid and vertebral arteries. In particular, the option of directly visualizing the intramural hematoma on cross-sectional images renders MRI a very useful technique in presump­tive vessel dissection. The size and shape of the intramu­ral hematoma depends on the surrounding structures, e.g., bone, fat, or venous plexus. The signal intensity of the wall hematoma depends on its maturity and MRI sequences. This crescent hematoma sign succeeds in a large number of patients using blood-sensitive MRI, especially T1-weighted (with or without fat suppres­sion) and T2-weighted sequences. Rarely, the hemat­oma may appear oval or circumferential. Over time, it shows a typical evolution of signal intensity related to the paramagnetic impact of the components of hemo­globin breakdown (Kitanaka et al 1994b). Importantly, the wall hematoma may be missed in the hyperacute stage in the fi rst days because the isointense hematoma may be obscured when surrounded by isointense tissues and the imaging might need to be repeated. In the sub­acute phase, the hematoma appears characteristically as a crescent-shaped hyperintense area around an ec­centric fl ow void corresponding to the vessel lumen in dissecting stenosis. In occlusive dissection, the whole cross-sectional area may present a more full moon ap­pearance. In our patient, the wall hematoma was already present on the imaging on initial presentation.
This distinct signal increase subsequently fades within 2 months after acute dissection and a dissect­ing lesion may then be diffi cult to prove (Paciaroni et al
2005). A fresh intraluminal thrombus may mimic wall hematoma but often reveals varying signal intensities, caused by its specifi c components (Schwaighofer et al
1990). Therefore, a crescent-shaped formation with ho­mogeneously increased signal intensity is highly sugges­tive for dissection but not specifi c, while an additional widening of the external vessel lumen is confi rmatory of dissection and should be searched for if a dissection is suspected. If additionally performed, the fl ow-sensitive TOF-MRA is able to show fl ow reduction, due for exam- ple to a sub-basal extracranial stenosis, in the form of a reduced intracranial ICA signal intensity. In-plane fl ow due to the tortuous course of the ICA, as well as turbu­lent fl ow, may impair the image quality and may lead to an overestimation of the stenosis or to a false diagnosis of occlusion. In the subacute stage a high-intensity sig­nal of the intraluminal clot may mimic intact blood fl ow. The diagnostic yield to detect dissecting stenoses and aneurysms is improved if contrast-enhanced (ce)-MRA is used (Touzé et al 2001). Because of its better spatial resolution and better visualization of lumen narrow­ing, vessel occlusion, and dissecting aneurysm, ce-MRA should be performed whenever MRI is used for diagno­sis of presumed dissection.
280 Case 11 Secondary Occlusion in Left-sided Extracranial Internal Carotid Artery Dissection
CT angiography (CTA) sensitively depicts the char­acteristic imaging appearance of a tapering vessel fol­lowing dissection. However, it cannot directly visualize the intramural hematoma, due to limited soft tissue contrast. It needs careful interpretation within the base of the skull because of the interference with the bone structures surrounding the ICA. A critical evalua­tion of the source images and careful postprocessing of the images aiming to remove or reduce the bone signal might help to evaluate the vessel continuity and in­tegrity. Results similar to those of MR techniques have been reported for the detection and follow-up of ICA dissections (Leclerc et al 1996). Because of the short investigation time, multislice CTA is currently the fi rst- line modality in presumed cervicocerebral vascular pathology, especially in stroke patients. This technique provides comprehensive and high-resolution vessel as­sessment, superior to current MRA modes. Like MRI,
the CTA technique allows visualization of the frequently seen enlarged external vessel diameter of the dissected vessels. An initial small study comparing CTA and MRI/ TOF-MRA demonstrated CTA superiority as it depicted all seven dissections, of which two were missed by the MRI technique. In the same series, CTA identifi ed a dis- secting aneurysm missed by MRI (Elijovich et al 2006). Diff erent bone subtraction techniques have been used, e.g., threshold-based bone removal in single-energy CTA as well as dual-energy postprocessing, all aiming to achieve an image quality similar to conventional DSA. Currently the most promising approach is dual-source CTA, which makes it possible to separate bone, calcifi ed plaques, hemorrhage, and contrast medium by their dif­ferent absorption spectra (Postma et al 2015). Yet, even dual-source CTA suff ers from pitfalls such as overesti- mation of vessel stenosis (Watanabe et al 2009), thus failing to attain conventional DSA image quality.
Case 12
Extracranial Bilateral Internal Carotid Artery and Right Vertebral Artery Occlusion, and Left Vertebral Artery Stenosis
281
Clinical Presentation
A 79-year-old man, who had never sought medical at­tention before, was admitted with unusual dizziness that had occurred intermittently over the past 3 days. The patient had no vascular risk factors apart from a history of heavy smoking and alcohol abuse. The neurologic examination on admission revealed mild gait ataxia (National Institutes of Health Stroke Scale [NIHSS] score: 1).
Initial Neuroradiologic Findings
Unenhanced cranial CT was unremarkable. Diff u- sion-weighted (DW) MRI showed a small subacute right posterior inferior cerebellar arter y (PICA) infarction at the cortical–subcortical border (Fig. B12.1). Intracrani­al time-of-fl ight MR angiography (TOF-MRA) showed bilateral internal carotid artery (ICA) and right-sided vertebral arter y (VA) occlusion. No further intracra­nial pathology was observed. Contrast-enhanced (ce) MRA of the brain-supplying arteries revealed bilateral proximal ICA occlusions, a moderate distal stenosis of the left common carotid artery (CCA), and a high-grade stenosis of the left VA origin. The right VA signal was missing over the entire length of the artery (Fig. B12.2 and Fig. B12.3).
Suspected Diagnosis
Right-sided cerebellar ischemic infarction likely caused by a periocclusional artery-to-artery embolism in right distal VA occlusion. Clinically asymptomatic bilateral ICA occlusion and high-grade left proximal VA stenosis, likely caused by severe generalized extracranial atherosclerosis.
Questions to Answer by Ultrasound Techniques
• Was the burden of extracranial steno-occlusive lesions confi rmed by ultrasound?
• Could ultrasound distinguish between a proximal and distal left VA occlusion?
• If a distal VA occlusion was present, could ultrasound be used to diff erentiate occlusions located proximally versus distally to the PICA origin?
• What was the pattern of collateral blood fl ow?
Initial Neurosonologic Findings
Extracranial Duplex Sonography
B-mode sonography revealed generalized atherosclerosis in both carotid arteries. Both CCAs had a high-resistance ow signal with increased pulsatility. The left distal CCA showed a 40% local lumen reduction in the cross-sectional plane caused by a largely hypoechoic plaque. Despite this lumen reduction, no fl ow disturbances or increased fl ow velocities were observed. Flow was absent in both ICAs. The left ICA had a roughly 5 mm residual small-vessel lumen (“blind sack”). No residual lumen was observed in the right ICA. Both external carotid arteries (ECAs) had mildly internalized fl ow signals. The dominant left V2-VA (diameter 5.4 mm) had a mild poststenotic fl ow pattern. Nevertheless, fl ow velocities were high and the blood volume fl ow measured 580 mL/min. At the V1-VA segment, the fl ow was turbulent and had a fl ow velocity similar to the V2-VA. The origin was not detect­ed. Only minimal systolic spikes were registered in the right V1- and V2-VA segments, which otherwise had a normal diameter (3.9 mm). No cervical spinal collater­als were observed throughout the entire V2-VA length (Figs. B12.4–B12.9).
Transcranial Duplex Sonography
All intracranial vessels had mild poststenotic fl ow pat- terns. Blood fl ow in both anterior cerebral arteries (ACAs) was antegrade. Increased and turbulent fl ow velocities were seen in both P1-PCAs and posterior communicating arteries (PCoAs). This, in combination with the normal ow velocities in both P2-PCAs, was considered indicative of collateral fl ow function from the posterior toward the anterior circulation. Transforaminal insonation re­vealed a marked fl ow signal in the left V4-VA and basi- lar artery. On the right side, no proximal or distal V4-VA signals were observed. Transorbital insonation revealed a retrograde fl ow in both ophthalmic arteries (OAs) (Figs. B12.10–B12.18).
Conclusion
Bilateral proximal extracranial ICA occlusion and dis­tal right VA occlusion proximal to the PICA origin. Left proximal VA stenosis sonographically assumed to be of beginning hemodynamic relevance or a collateral fl ow due to the mild poststenotic downstream fl ow pattern.
282 Case 12 Extracranial Bilateral Internal Carotid Artery and Right Vertebral Artery Occlusion, and Left Vertebral Artery Stenosis
Considering the ce-MRA fi ndings, the fi nal diagnosis was determined to be a high-grade VA stenosis of in­cipient hemodynamic relevance. Intracranial collateral blood fl ow to both the middle cerebral artery (MCA) and ACA territories occurred primarily via both PCoAs, and to a lesser extent via both OAs.
Fig. B12.19 shows a schematic of the patient’s extra-
and intracranial brain-supplying arteries.
Clinical Course
The patient was send to rehabilitation with aspirin and statin medication. There, his unsteadiness remitted but the dizziness remained. The etiology of the three and a half-vessel disease was severe atherosclerosis caused by severe, long-standing, combined heavy smoking and alco­hol misuse. Stenting of the left VA stenosis was discussed but refused by the patient. Regular 6-month follow-ups revealed no further clinical events, and the neurosono­logic fi ndings remained unchanged over an observational period of 2 years.
AB
Fig. B12.1 Diff usion-weighted MRI, axial plane, revealing a small fresh infarct in the right PICA territory (arrow).
AB C
Fig. B12.2 3D MRA, coronal maximal intensity projection (MIP), TOF­MRA (A) and contrast-enhanced (ce) MRA (B). Missing bilateral ICA signals. Prominent PCoA on both sides (arrowheads). Small right dis­tal V4-VA fi lling, indicating a possible retrograde fl ow toward the PICA or to relevant V4-VA perforator arteries (arrow). Note that ce-MRA is superior to TOF-MRA in visualizing the right distal V4-VA lumen.
Fig. B12.3 Extracranial 3D ce-MRA, coronal MIP, diff er- ent views (A–C). Bilateral ICA occlusion with large residual lumen on the left ICA (A, ar­rowhead). Residual lumen of the distal right V4-VA (A, small arrow). Moderate CCA steno­sis, best identifi ed in B (large arrow). (C) Proximal left VA stenosis (arrow) and right ICA occlusion (arrowhead).
283Clinical Course
CCA-L
Fig. B12.4 Extracranial duplex, longitudinal plane (left), and cross­sectional plane (right). Left CCA with 40% lumen reduction (ar­rows).
ICA-R
ICA-L
Fig. B12.5 Extracranial duplex, longitudinal plane. Left ICA with a large “blind sack” several centimeters long and a weak, almost r e t r o g r a d e fl ow signal.
V2-VA-L
Fig. B12.6 Extracranial duplex, longitudinal plane. Right ICA fi lled with homogeneous hypoechoic material (arrow).
V1-VA-L
Fig. B12.8 Extracranial duplex, longitudinal plane. V1-VA fl ow sig- nal similar to the V2-VA segment. The direct VA origin could not be visualized (fl ow velocity 95/33 cm/s).
Fig. B12.7 Extracranial duplex, longitudinal plane. Mild poststen­otic fl ow pattern in the dominant left V2-VA with marked increased blood volume fl ow (580 mL/min, diameter 5.4 mm).
V2-VA-R
Fig. B12.9 Extracranial duplex, longitudinal plane. Missing fl ow sig- nal in the right V2-VA (Diameter 3.9 mm).
284 Case 12 Extracranial Bilateral Internal Carotid Artery and Right Vertebral Artery Occlusion, and Left Vertebral Artery Stenosis
M1-MCA-L
Fig. B12.10 TCCS (trans temp oral ap proa ch), lef t-sid ed i nson ation , midbrain plane. Mild poststenotic fl ow pattern in the left M1-MCA (fl ow velocity 74/30 cm/s).
P1-PCA- / PCoA-L
M1-MCA-R
Fig. B12.11 TCCS (transtemporal approach), right-sided inson­ation, midbrain plane. Mild poststenotic fl ow pattern in the right M1-MCA (fl ow velocity 57/24 cm/s).
P2-PCA-L
Fig. B12.12 TCCS (trans temp oral ap proa ch), lef t-sid ed i nson ation , midbrain plane. Raised fl ow velocity in the junction of left PCoA and P1-PCA indicating collateral fl ow (fl ow velocity 140/40 cm/s).
PCoA-R
Fig. B12.14 TCCS (transtemporal approach), right-sided insona­tion, midbrain plane. Turbulent fl ow in the right PCoA.
Fig. B12.13 TCCS (trans temp oral ap proa ch), lef t-sid ed i nson ation , midbrain plane. Normal fl ow velocity and mild poststenotic fl ow pattern in the left distal P2-PCA (fl ow velocity 55/24 cm/s).
P2-PCA-R
Fig. B12.15 TCCS (transtemporal approach), right-sided insona­tion, midbrain plane. Normal fl ow velocity and mild poststenotic ow pattern in the right distal P2-PCA (fl ow velocity 61/27 cm/s).
285Discussion
BA
Fig. B12.16 TCCS (t rans foramin al app roac h). Str ong fl ow signal with increased fl ow velocity in the basilar artery (BA) (fl ow velocity 137/53 cm/s).
OA-R
OA-L
Fig. B12.17 TCCS ( tran sorbital a pproach ), lef t-si ded in sona tion. Reversed left OA fl ow (fl ow velocity 30/11 cm/s). Note the elon- gated course of the left OA revealing a bidirectional color signal. When in doubt, the fl ow pattern has to be considered to decide if an antegrade or retrograde fl ow is present.
Fig. B12.18 TCCS ( tran sorbital appr oach ), righ t-si ded insonat ion. Reversed right OA fl ow (fl ow velocity 37/11 cm/s).
Final Diagnosis
Periocclusional embolic right-sided PICA infarct in right distal VA occlusion and asymptomatic bilateral ICA occlu­sion. High-grade left proximal VA stenosis and moderate left CCA stenosis based on a severe generalized extrac­ranial atherosclerosis Acceptable collateral fl ow via both PCoAs and OAs.
Discussion
Clinical Aspects
Here we discuss a 79-year-old patient with a rare severe atherosclerotic vascular disease—namely, occlusion of three out of four extracranial brain-supplying arteries
RL
Fig. B12.19 Schematic of the patient’s extra- and intracranial brain-supplying arteries. Bilateral ICA and right distal VA occlusion. Proximal left VA stenosis as well as left CCA stenosis (circles). Col­lateral blood fl ow toward the anterior circulation mainly via both PCoAs assisted by both OAs.
and stenosis of the remaining VA. Bilateral extracrani­al ICA occlusion of atherosclerotic origin alone is rare, and there are no precise epidemiologic data regarding incidence or prevalence (for further reading on unilat­eral ICA occlusion, see Case 28). A duplex sonographic study reported a bilateral ICA occlusion in 15 of 3,200 unselected patients (0.47%) (Lazarides et al 1991). These data are in line with another study, which found that 8 of 2,228 patients had transient ischemic attacks (TIA)/ stroke and bilateral ICA occlusion (0.37%) (Mead et al
2006). Except rare cases of bilateral dissecting ICA oc­clusions, most bilateral occlusions are, as with our pa­tient, of atherosclerotic origin. The combined results of two studies (total of 95 patients with bilateral occlu­sion) revealed that 93–100% of patients with bilateral occlusion reported heavy smoking and usually also had at least one additional risk factor (e.g., hypertension,
286 Case 12 Extracranial Bilateral Internal Carotid Artery and Right Vertebral Artery Occlusion, and Left Vertebral Artery Stenosis
ischemic heart disease, hyperlipidemia, or diabetes) (AbuRahma and Copeland 1998). Moreover, bilateral oc­clusion seems to predominantly aff ect males, who make up 81–91% of the reported cases (Persoon et al 2009). Vertebrobasilar TIAs, which manifested clinically as syncope, vertigo, and drop attacks, were also observed in 14% of cases (Wade et al 1987). As these symptoms sometimes occurred in association with hyperextension of the neck or with orthostatic maneuvers (rising from a sitting or lying position, exercise, postprandial hypoten­sion, or transition from cold to warm environment), a steal phenomenon in the posterior circulation as a result of the collateral function was suspected. The “shaking limb sign”—a rare but prototypic sign of hemodynamic TIA, caused by ICA occlusion and other orthostatically induced TIA—has been seen in up to 18% of patients with bilateral ICA occlusion (Persoon et al 2009). There is no clear prognostic data. One large study in medically treat­ed patients reported an annual combined TIA and stroke risk of 15%, a stroke risk of 13%, and a mortality rate of 8% over a mean observational period of 42 months (Wade et al 1987). Higher overall mortality was reported in a smaller case series in which six of eight (75%) medically treated patients died during a mean follow-up of 6 years (AbuRahma and Copeland 1998). A better outcome with a calculated annual stroke rate of 1.2% was reported in a diff erent study covering a mean observational period of 5.9 years. The relatively low stroke rate might be re­lated to the high percentage of patients included on the basis of retinal ischemia (Persoon et al 2009). Unilateral ICA occlusion combined with isolated retinal ischemia is known to be associated with lower stroke recurrence rates than occlusion combined with cerebral ischemia symptoms (Grubb et al 1998, Klijn et al 2000). Other possible explanations include both improved medical secondary prevention therapy and more eff ective man- agement of cardiovascular risk factors (compared, e.g., to the 1980s).
Extracranial–intracranial (EC–IC) bypass surgery has been suggested because of the high stroke recurrence rates estimated for patients with bilateral ICA occlusion (el-Fiki et al 1985, Friedman et al 1987). Interestingly, however, the prognosis in bilateral ICA occlusion seems in fact to be better than unilateral ICA occlusion (Klijn et al
2001). Compensation of a bilateral ICA occlusion requires the presence or development of stable collaterals either before occurrence of the fi rst symptoms or after fi rst is- chemia. Interestingly, a subgroup analysis of the ACAS study demonstrated that patients with an asymptomatic ICA stenosis 60% and a contralateral ICA occlusion had a lower stroke recurrence risk compared to those without contralateral occlusion (Baker et al 2000).
Within this context, the question arises as to how these patients should be managed in terms of blood pressure. Carotid endarterectomy trials have shown that conservatively treated patients with bilateral oc­clusive processes (bilateral high-grade stenosis or high-grade stenosis and contralateral occlusion) have a higher stroke risks if blood pressure is reduced below 140 mm Hg (Rothwell et al 2003a).
In our patient, the diagnosis was made after cerebellar stroke occurrence, which manifested clinically as gait ataxia.
The only symptom suggestive of persistent brainstem malp­erfusion was chronic dizziness. Transient visual complaints were not reported. Because of his age, bypass surgery was not recommended. He did not have increased blood pres­sure and therefore no antihypertensive medications could be discontinued to improve his cerebral perfusion.
Our patient additionally had an asymptomatic left VA stenosis at its origin (V0-VA segment). A contralateral steno-occlusive lesion or a PICA-ending VA can lead to diz­ziness, vertigo, blurred vision, and ataxia—i.e., symptoms resulting from hypoperfusion in the posterior circulation. Here, the clinical prognosis also depends on the effi ciency of the collaterals. For instance, anastomoses to deep cer­vical arteries usually starting at the V2-VA segment may, in part, compensate for reduced fl ow. These collaterals can be found in up to 31% of patients with proximal VA occlusions and in 9% of patients with proximal VA steno­sis (Wityk et al 1998). In our case, the above anastomoses were not detected. (For further reading on V0-VA stenosis and collateral pathways, see Chapter 5, “VA Occlusion” under “Extracranial Pathology,” and Case 45.)
Angiologic and Anatomic Aspects
ICA occlusions are easily diagnosed using duplex ultra­sound. The characteristic fi ndings are both absent color and absent Doppler fl ow signals along the extracranial ICA course. If the occlusion is located further distally, a proximal stump signal with alternating fl ow and a miss- ing diastolic fl ow component might be found. A com- parative study between duplex ultrasound and digital subtraction angiography (DSA) in 91 patients with ICA oc­clusion, reported that duplex ultrasound had a sensitivity of 91%, specifi city of 99%, and positive and negative pre- dictive values of 96%, and 98%, respectively (AbuRahma et al 1997). The relatively high rate of false-positive results is best explained by the pitfalls in detecting a nearly oc­cluded ICA. The use of echo contrast agents may improve (1) the detection of minimal fl ow within a severe steno- sis, (2) the evaluation of fl ow in the presence of severely calcifi ed plaque, and therefore (3) the diff erentiation be- tween true and near occlusion (Fürst et al 1999, Ohm et al 2005) (for further discussion on ICA near occlusion, see also Case 15). Of note, ICA occlusions may reopen over time. In dissecting ICA disorders, reopening has been ob­served in up to 90% of cases (Steinke et al 1994). In cardiac or aortic-to artery embolism, vessel reopening should be expected in all cases. If no recanalization occurs, reevalu­ation of its etiology may be necessary. Even in atheroscle­rosis-related proximal ICA occlusions, a reopening may rarely occur and has been observed in 16 of 696 patients (2.3%). As duplex ultrasound and DSA have shown, this occurred after a mean time interval of 38 months from occlusion diagnosis (Camporese et al 2011).
The OA is usually a second-line collateral pathway in ICA steno-occlusive lesions. It may, however, become a relevant collateral vessel with retrograde fl ow and a fl ow pattern similar to that of an intracranial artery if hypo­plasia of the communicating arteries, or as in our case, severe contralateral hemodynamic restrictions are pres­ent. In our case, the only remaining brain-supplying ar­tery, the left VA, revealed a blood volume fl ow (BVF) of
287Discussion
580 mL/min. This is remarkably high, but remains lower than the global BVF of healthy subjects—reported to be 733 ± 54 mL/min (Schreiber et al 2005b). The physiolog­ic centrifugal BVF of the OA is 10 - 11 mL/min (Ambarki et al 2013). Collateral activation results in centripetal, increased OA fl ow. Its fl ow should at least reach values know from bypass surgery when the superfi cial tempo- ral artery is used to supply the brain. Here, BVF of 84 ± 32 mL/min (range 14–177 mL/min) have been reported (Neff et al 2004). To date, no data regarding BVF in ICA occlusion has been reported, but it seems plausible to assume that both OAs may compensate the missing fl ow volume in our presented case.
Compared with the proximal ICA, VA origin assessment
is usually more diffi cult (for further reading on proximal VA stenosis, see Case 45). The detectable V1- and V2-VA segments revealed a mild poststenotic fl ow pattern, indi- rectly suggestive of a hemodynamically relevant proximal stenosis. When in doubt, extracranial continuous-wave Doppler sonography may depict the VA off shoot signal as the small Doppler probe facilitates signal detection be­hind the clavicle. However, our patient’s concomitant bi­lateral ICA and contralateral VA occlusion made it unclear whether part of the poststenotic appearance was related to hyperperfusion in the single remaining brain-supplying artery. Finally, MRA clearly disclosed a high-grade stenosis, leading to readjusted evaluation of ultrasound fi ndings.
The exact defi nition of VA occlusion is equally prob- lematic (not only using the ultrasound technique) and requires specifi c anatomic knowledge to avoid diagnostic errors. As with ICA occlusion, an occluded vessel might be depicted by color-mode sonography with an absent color signal (see Fig. A5.75). Within the V2-VA segment, the blood fl ow in the concomitant vertebral vein is usu- ally preserved and might be of diagnostic aid (see Fig. A5.76). In our case, only systolic spikes were seen in the examined V1- and V2-VA segments. VA hypoplasia was ruled out as the diameter was 3.9 mm. Distinguishing short systolic spikes from vessel wall movements may be diffi cult and may also be interpreted as a proximal VA occlusion. However, proximal occlusion usually leads to
distal collateral activation. The VA has numerous extrac­ranial anastomoses at all levels of its extracranial course which can potentially serve as collaterals and prevent occlusion over its entire length. These are anastomoses from the thyrocervical trunk and muscular rami of ECA branches, especially from the occipital artery. Spinal rami of the contralateral VA can also participate in collat­eral blood supply. During a proximal VA occlusion, these collaterals, depending on their quantity and quality, may cause a secondary VA fi lling with “postocclusional” VA ow of varying magnitude detectable in the distal VA segments (see Fig. A5.77, Fig. A5.78, A5.79). Complete occlusion of the entire extracranial VA is rare. In the pres­ence of two equivalent VAs, the above-mentioned collat­eral pathways are rarely of importance as the contralat­eral VA will provide the blood supply to the posterior circulation and also retrograde via a vertebro-vertebral overfl ow toward the PICA of the aff ected VA. However, if contralateral VA hypoplasia or PICA termination is pres­ent, the extracranial anastomoses become relevant. In these instances, the distal VA fl ow is usually antegrade with a typical poststenotic fl ow pattern. Although the term “poststenotic” seems slightly inaccurate in a vessel segment distal to an occlusion, we would suggest its use nevertheless, as it clearly illustrates the common prob­lem of hemodynamic impairment in stenoses and oc­clusions. A distal extracranial VA occlusion, in contrast, may cause a stump signal (as seen in our patient), a high pulsatile fl ow signal with absent end-diastolic fl ow, or a biphasic or triphasic waveform comparable to an ECA branch, as its communication to these vessels usually re­mains open (see Fig. A5.80). For example, one study that included 10 distal VA occlusions (proximal to the origin of the PICA), reported that all subjects had zero diastolic ow (Saito et al 2004). From both radiologic and clin­ical viewpoints, it has to be emphasized that the term “VA occlusion” alone is not suffi cient. Ideally, it should be complemented by the exact location of the occlusion and information regarding possible secondary VA fi lling by collaterals distal to the occlusion. For further reading, see also Case 19 and Case 45.
288
Case 13
Right Internal Carotid Artery Stenosis in Fibromuscular Dysplasia and Granulomatosis with Polyangiitis (formerly Wegener’s Granulomatosis)
Clinical Presentation
A 51-year-old woman presented with stepwise deterio­ration of a left hemiparesis that had started 3 days before admission. The medical history revealed chronic rhinitis, sinusitis, and bronchitis but no vascular risk factors. On neu­rologic examination, the patient had a severe left-sided bra­chiofacial hemiparesis (National Institutes of Health Stroke Scale [NIHSS] score: 8). In addition, she had nasal congestion.
Initial Neuroradiologic Findings
Cerebral CT (CCT) scan on the day of admission revealed ischemic infarction in the anterior and posterior territo­ries of the right middle cerebral artery (MCA). MRI was not performed (Fig. B13.1).
Suspected Diagnosis
Ischemic brain infarction in the right MCA territory of unknown origin.
Question to Answer by Ultrasound Techniques
• Was there evidence of a stenotic process, particularly in the right internal carotid artery (ICA) or MCA?
Initial Neurosonologic Findings (Day 1)
Extracranial Duplex Sonography
B-mode sonography revealed only mild atheroscle­rosis of the carotid arteries. Color imaging showed elongation and caliber variations of both distal ICAs, but predominantly aff ecting the right side. Blood fl ow velocity in the proximal right ICA was mildly reduced (fl ow velocity: 47/24 cm/s) and the pulsatility slightly increased. Within the distal segment of the right ICA, a non-angle-corrected peak systolic fl ow velocity of 250 cm/s was observed. Normal fl ow velocities were seen in the remaining extracranial vessels (Fig. B13.2 and Fig. B13.3).
Transcranial Duplex Sonography
The temporal window was inadequate for a complete insonation of the anterior circulation. Punctual signals of the proximal M1-MCA could be obtained, revealing an obvious fl ow velocity diff erence between the two sides (fl ow velocity: right M1-MCA, 57/25 cm/s; left M1-MCA, 111/55 cm/s) (not shown). The calculated Zanette asymmetry index for the systolic velocities was 64 and the right-to-left ratio (= velocity of the aff ected M1/normal M1 velocity) was 0.51. Assessment of the other intracranial vessels showed normal and symmet­ric fl ow signals.
Conclusion
Distal extracranial right ICA stenosis, ~70%. Suspected right distal M1-MCA occlusion, probably embolic in nature.
Clinical Course (1)
Thrombolysis was not indicated because of the time delay and the signs of infarction on CT. The above vas­cular changes in a relatively young patient without classic vascular risk factors did not favor an atheroscle­rotic etiology. In particular, no arterial hypertension was present. Normal 24-hour ECG and echocardiog­raphy made a cardioembolic source unlikely. There was no coagulopathy involving proteins C and S, anti­cardiolipin antibodies, activated protein C resistance, and lupus inhibitor. The cerebrospinal fl uid (CSF) was normal. In view of the history of chronic rhinitis, si­nusitis, and bronchitis, specifi c laboratory tests were performed which revealed an increased erythrocyte sedimentation rate (ESR) (70 mm/hour, Westergren), mild anemia, and thrombocytosis of 1,200/nL (normal range 130–340/nL), but normal white blood cell counts. In addition, the level of cytoplasmic antineutrophilic cytoplasmic antibody (cANCA) was increased to 98 E/ mL (normal value <15 E/mL), and urine proteins and erythrocytes were elevated. Finally, a nasal mucosa bi­opsy confi rmed granulomatosis with polyangiitis (GPA; formerly known as Wegener’s granulomatosis).