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J Neurosurg Volume 124 • April 2016 1100 CLINICAL ARTICLE J Neurosurg 124:1100–1106, 2016 B EYOND typical cerebrovascular malformations in- cluding arteriovenous malformations (AVMs), de- velopmental venous anomalies (DVAs), cavernous malformations, and capillary telangiectasia, the disease concept and the proper treatment plan for some rare vas- cular lesions have not yet been well established. A poorly defined arterial blush and an early venous drainage with a dilated medullary vein from the arterial or capillary phase is one of the rare vascular anomalies. Such a vascular le- sion can be distinguished from an AVM by the absence of a discrete nidus and poorly identifiable, enlarged feeding arteries, and distinguished from DVAs by the early ve- nous drainage from the arterial or capillary phase. Various terminologies including arterialized DVA, 15 mixed-type vascular malformation, 6 venous angioma with arteriove- nous (AV) shunt, 10 atypical DVA with early venous filling, mixed venous and arteriovenous malformations, medul- lary venous malformation with an arterial component, or venous-predominant AVM (vp-AVM), 8 which is referred to here, have been used to interpret the rare vascular anomalies, but still no definite terminology has been es- tablished. ABBREVIATIONS AV = arteriovenous; AVM = arteriovenous malformation; DVA = developmental venous anomaly; GKS = Gamma Knife surgery; ICH = intracranial hemor- rhage; vp-AVM = venous-predominant AVM. SUBMITTED October 29, 2014. ACCEPTED April 2, 2015. INCLUDE WHEN CITING Published online September 25, 2015; DOI: 10.3171/2015.4.JNS142475. Long-term treatment outcome of venous-predominant arteriovenous malformation Jin Pyeong Jeon, MD, 1 Jeong Eun Kim, MD, 2 Jun Hyong Ahn, MD, 1 Won-Sang Cho, MD, 2 Young Dae Cho, MD, 3 Young-Je Son, MD, 2 Jae Seung Bang, MD, 2 Hyun-Seung Kang, MD, 2 Chul-Ho Sohn, MD, 3 Hyun-Tai Chung, PhD, 2 Chang Wan Oh, MD, 2 and Dong Gyu Kim, MD 2 1 Department of Neurosurgery, Hallym University College of Medicine, Chuncheon; and Departments of 2 Neurosurgery and 3 Radiology, Seoul National University College of Medicine, Seoul, Korea OBJECTIVE Treatment strategies for venous-predominant arteriovenous malformation (vp-AVM) remain unclear due to the limited number of cases and a lack of long-term outcomes. The purpose of this study was to report the authors’ expe- rience with treatment outcomes with a review of the pertinent literature in patients with vp-AVM. METHODS Medical and radiological data from 1998 to 2011 were retrospectively evaluated. The degree of the arterio- venous (AV) shunt was categorized into 2 groups, a high- and low-flow AV shunt based on the angiographic findings. RESULTS Sixteen patients with a mean age of 45.3 years (range 16–78 years) and a mean follow-up of 79.9 months (range 25–264 months) were examined. Symptomatic lesions were noted in 13 patients: intracranial hemorrhage (ICH) in 9, seizure in 1, and headache in 3. A high-flow shunt was observed on angiography in 13 patients. Among these 13 patients, 12 patients were symptomatic. Nine patients presenting with ICH underwent hematoma removal with additional Gamma Knife surgery (GKS; n = 4), GKS only (n = 2), or conservative treatment (n = 3). The 3 asymptomatic patients received conservative treatment, and 1 rebleeding episode was observed. Seven of 8 patients who underwent GKS as an initial or secondary treatment modality experienced a marked reduction in the AV shunt on follow-up angiography, but complete obliteration was not observed. CONCLUSIONS Poor lesion localization makes a vp-AVM challenging to treat. Symptomatic patients with a high-flow shunt are supposedly best treated with GKS, despite the fact that only 87.5% of the vp-AVMs treated this way showed a reduction in the malformation volume, and none were cured. http://thejns.org/doi/abs/10.3171/2015.4.JNS142475 KEY WORDS arteriovenous malformation; developmental venous anomaly; intracranial hemorrhage; venous malformation; vascular disorders ©AANS, 2016 Unauthenticated | Downloaded 07/01/21 05:10 PM UTC

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  • J Neurosurg  Volume 124 • April 20161100

    cliNical articleJ Neurosurg 124:1100–1106, 2016

    Beyond typical cerebrovascular malformations in-cluding arteriovenous malformations (AVMs), de-velopmental venous anomalies (DVAs), cavernous malformations, and capillary telangiectasia, the disease concept and the proper treatment plan for some rare vas-cular lesions have not yet been well established. A poorly defined arterial blush and an early venous drainage with a dilated medullary vein from the arterial or capillary phase is one of the rare vascular anomalies. Such a vascular le-sion can be distinguished from an AVM by the absence of a discrete nidus and poorly identifiable, enlarged feeding

    arteries, and distinguished from DVAs by the early ve-nous drainage from the arterial or capillary phase. Various terminologies including arterialized DVA,15 mixed-type vascular malformation,6 venous angioma with arteriove-nous (AV) shunt,10 atypical DVA with early venous filling, mixed venous and arteriovenous malformations, medul-lary venous malformation with an arterial component, or venous-predominant AVM (vp-AVM),8 which is referred to here, have been used to interpret the rare vascular anomalies, but still no definite terminology has been es-tablished.

    abbreviatioNs AV = arteriovenous; AVM = arteriovenous malformation; DVA = developmental venous anomaly; GKS = Gamma Knife surgery; ICH = intracranial hemor-rhage; vp-AVM = venous-predominant AVM.submitted October 29, 2014.  accepted April 2, 2015.iNclude wheN citiNg Published online September 25, 2015; DOI: 10.3171/2015.4.JNS142475.

    Long-term treatment outcome of venous-predominant arteriovenous malformationJin pyeong Jeon, md,1 Jeong eun Kim, md,2 Jun hyong ahn, md,1 won-sang cho, md,2 Young dae cho, md,3 Young-Je son, md,2 Jae seung bang, md,2 hyun-seung Kang, md,2 chul-ho sohn, md,3 hyun-tai chung, phd,2 chang wan oh, md,2 and dong gyu Kim, md2

    1Department of Neurosurgery, Hallym University College of Medicine, Chuncheon; and Departments of 2Neurosurgery and 3Radiology, Seoul National University College of Medicine, Seoul, Korea

    obJective Treatment strategies for venous-predominant arteriovenous malformation (vp-AVM) remain unclear due to the limited number of cases and a lack of long-term outcomes. The purpose of this study was to report the authors’ expe-rience with treatment outcomes with a review of the pertinent literature in patients with vp-AVM.methods Medical and radiological data from 1998 to 2011 were retrospectively evaluated. The degree of the arterio-venous (AV) shunt was categorized into 2 groups, a high- and low-flow AV shunt based on the angiographic findings.results Sixteen patients with a mean age of 45.3 years (range 16–78 years) and a mean follow-up of 79.9 months (range 25–264 months) were examined. Symptomatic lesions were noted in 13 patients: intracranial hemorrhage (ICH) in 9, seizure in 1, and headache in 3. A high-flow shunt was observed on angiography in 13 patients. Among these 13 patients, 12 patients were symptomatic. Nine patients presenting with ICH underwent hematoma removal with additional Gamma Knife surgery (GKS; n = 4), GKS only (n = 2), or conservative treatment (n = 3). The 3 asymptomatic patients received conservative treatment, and 1 rebleeding episode was observed. Seven of 8 patients who underwent GKS as an initial or secondary treatment modality experienced a marked reduction in the AV shunt on follow-up angiography, but complete obliteration was not observed.coNclusioNs Poor lesion localization makes a vp-AVM challenging to treat. Symptomatic patients with a high-flow shunt are supposedly best treated with GKS, despite the fact that only 87.5% of the vp-AVMs treated this way showed a reduction in the malformation volume, and none were cured.http://thejns.org/doi/abs/10.3171/2015.4.JNS142475KeY words arteriovenous malformation; developmental venous anomaly; intracranial hemorrhage; venous malformation; vascular disorders

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  • venous-predominant arteriovenous malformation

    Consensus on the treatment of vp-AVM remains con-troversial due to the different disease concepts as well as the limited number of cases and a lack of information on long-term outcomes. Pereira et al.16 believed that increased medullary blush represents a rapid transit time, not an AV shunt. Accordingly, an atypical DVA with early venous drainage did not become symptomatic. On the contrary, Im et al.8 reported that a vp-AVM more closely resembles an AVM because of its histological and clinical similar-ity. They believed that more aggressive treatment modali-ties such as resection or Gamma Knife surgery (GKS) can be used to prevent symptomatic vp-AVM or to treat in-tracranial hemorrhage (ICH) associated with a vp-AVM. Nevertheless, poorly localized abnormal lesions due to the absence of a definite nidus and enlarged feeding ar-teries as well as limited information on long-term treat-ment outcomes after GKS can make it difficult to select the appropriate treatment modality. The aim of this study was to report our experience with the long-term treatment outcomes of vp-AVMs as well as review the pertinent lit-erature.

    methodspatient population

    This study was approved by the institutional review board (1309–063–520) of Seoul National University Col-lege of Medicine. This retrospective analysis was per-formed in patients with a vp-AVM from 1998 to 2011 at a single center. A venous anomaly that had the following features on cerebral angiography was defined as a vp-AVM. First, a poorly defined arterial blush and an early venous drainage combined with dilated medullary veins noted during the arterial or capillary phase. Second, no definite nidus was noted.2,8 Vp-AVM was assessed by 2 factors, the presence of clinical symptoms relevant to vp-AVMs and the degree of the AV shunt at angiography. Symptomatic lesions included ICH, seizure, neurological deficits, and relevant clinical symptoms such as headache or dizziness. The degree of the AV shunt was categorized into 2 groups, a high- or low-flow AV shunt, based on the angiographic findings. A high-flow AV shunt was charac-terized by an abnormal prominent arterial blush and an early venous drainage from the arterial phase. A low-flow AV shunt was characterized by an arterial blush and an early venous drainage with a dilated medullary vein dur-ing the capillary phase (Fig. 1).

    data collectionMedical records including age, sex, hypertension, dia-

    betes mellitus, smoking, presenting symptoms, treatment methods, and follow-up duration were reviewed. Radio-logical data such as location, presence of hemorrhage, ar-terial component dominance, and rebleeding events were examined. Follow-up radiological tests were performed with MRI or digital subtraction angiography according to parameters that were reported previously.8 Hematoma re-moval, GKS, or conservative treatments were performed. Hematoma removal was indicated for the management of increased intracranial pressure (ICP) refractory to medical treatment. GKS was considered for symptomatic patients

    primarily or secondarily. GKS was performed with the Leksell Gamma Knife (Elekta Instrument AB).4 The basic procedure for GKS was as follows. First, the abnormal vas-cular staining lesions were covered as much as possible, but not the main draining veins. Then, low-dose GKS was per-formed with a marginal dose of ≤ 20 Gy (50% isodose).8,9

    MRI was performed at 6, 12, 24, and 36 months after diagnosis. Thereafter, continued MRI every 2 or 3 years was advised to determine the progression of the disease. If patients experienced new or worsened symptoms includ-ing seizure, headache, or neurological change, the MRI was checked. Specifically, for patients who presented with hemorrhage and underwent GKS, angiography was added to the MRI examination according to the clinical status of the patients. Repeated GKS was indicated for patients presenting with hemorrhage who did not show a reduction in vascularity or for patients who had worsened symptoms during the follow-up period.

    The annual hemorrhage rate of vp-AVM before in-tervention was estimated as the number of hemorrhages divided by the patient-years at risk, assuming that the vp-AVM was present at birth. Concerning the annual hem-orrhage rate after the intervention, none of our patients experienced hemorrhage after the intervention, therefore the postinterventional hemorrhage rate was estimated as the number of hemorrhage events divided by the patient risk-years from the diagnosis to the dates of last follow-up.

    literature review of pertinent clinical studiesThe MEDLINE database was searched from 1970 to

    2012 using the search terms “atypical venous anomaly,” “arterialized DVA,” “vp-AVM,” and “AV shunt,” exclud-ing case reports that had less than 2 cases. Clinical and ra-diological data, including the number of enrolled patients, age, sex, terminology for diagnosis, treatment methods, and complications, were investigated for comparison in our study.

    resultsclinical and angiographic Features

    The demographic characteristics of the 16 patients are presented in Table 1. There were 9 male patients (56.3%). The mean age of all patients in the study was 45.3 years (range 16–78 years) with a mean follow-up of 79.9 months (range 25–264 months). Symptomatic lesions were noted in 13 patients: ICH in 9, seizure in 1, and headache in 3. The presence of a high-flow shunt at angiography was ob-served in 13 patients. Among these 13 patients, 12 were symptomatic. The remaining 3 cases had low-flow shunts, and 2 cases were asymptomatic.

    long-term treatment outcomesThe annual hemorrhage rate of the patients with vp-

    AVMs before and after the intervention was 1.24% and 0.94%, respectively. A total of 13 symptomatic patients, including 9 patients with ICH presentation, underwent the following treatments: hematoma removal with additional GKS (n = 4), GKS only (n = 2), and conservative treatment (n = 3). Resection of the venous anomaly was performed due to a poorly demarcated lesion in the operative field.

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    Four symptomatic patients who had a seizure or headache received GKS (n = 2) or conservative treatment (n = 2). Three asymptomatic patients received conservative treat-ment. Seven of the 8 patients who underwent GKS pri-marily or secondarily experienced a marked reduction in the AV shunt on follow-up angiography. One remaining patient showed no change in the extent of the lesion for abnormal arterial and early venous drainage with a dilated medullary vein. A rebleeding event was observed in 1 of 3 conservatively treated patients initially presenting with ICH. Patients who underwent hematoma removal with ad-ditional GKS or GKS only did not experience any rebleed-ing events. Radiation-related complications such as radio-necrosis and brain edema were not noted in our study.

    review of the pertinent literatureFive previous studies were reviewed (Table 2). The

    number of patients in each study ranged from 3 to 15. The terminologies to describe a poorly defined arterial blush and an early venous drainage with a dilated medullary vein without a discrete nidus included mixed venous and arte-riovenous malformation,2 venous malformation with arte-rial fistulization,14 venous angioma with AV shunt,10 arte-rialized DVA,15 and vp-AVM. Two rebleeding events were reported after conservative treatment.8,15 The time interval between the first and second bleeding ranged from 1 to 85 months. Fatal complications due to incomplete resection of the venous anomaly and radiation necrosis were reported.

    illustrative casescase 5

    A 55-year-old man presented with ICH in the right frontal area. Right carotid angiography showed an abnor-mal vascular staining lesion in the insular area during the

    Fig. 1. The vp-AVMs were categorized into 2 groups at angiography: high- and low-flow AV shunts.  a and b: An example of a high-flow AV shunt, defined as an abnormal prominent arterial blush (arrow) and early venous drainage with a dilated medullary vein, without a discrete nidus during the arterial phase.  c and d: Example of a low-flow AV shunt, characterized by the presence of early venous drainage (arrow) during the capillary phase without prominent arterial blush.

    table 1. summary of the enrolled patients in the study

    Case No.

    Age (yrs), Sex  Presentation Location

    Shunt Flow Treatment

    vp-AVM Vol (cm3)/GKS Dose (Gy)*

    KPS Score

    Angiographic Outcome Rebleeding

    1 16, F ICH  BG, frontal High HR & GKS 22.4/12 100 Reduction No2 61, M Incidental Cerebellum High Conservative 100 No3 22, F ICH Frontal High GKS 3/20 100 Reduction No4 56, M Seizure Temporal High Conservative 100 No5 55, M ICH BG High  HR & GKS 7.4/18 70 Reduction No6 36, F Headache Parietal High Conservative 100 No7 48, M Incidental BG Low Conservative 100 No8 23, M ICH Cerebellum High GKS 2.1/20 100 No change No9 78, M ICH BG High Conservative 70 Yes10 68, F ICH Temporal, occipital High Conservative 100 No11 63, M ICH BG High HR & GKS 4.8/15 70 Reduction No12 31, F ICH Frontal, thalamus High HR & GKS 8.4/16 90 Reduction No13 27, M Headache Temporal High GKS 5.2/18 100 Reduction No14 17, M Headache Temporal, occipital High GKS 10.5/15 100 Reduction No15 67, F Incidental  Frontal Low Conservative 100 No16 56, F ICH w/ IVH Cerebellum Low Conservative 100 No

    BG = basal ganglia; HR = hematoma removal; IVH = intraventricular hemorrhage; KPS = Karnofsky Performance Scale. *  Dose = marginal radiation dose of ≤ 20 Gy (50% isodose).

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  • venous-predominant arteriovenous malformation

    midarterial phase and an early venous drainage of the syl-vian vein with a dilated medullary vein during the capil-lary phase. The 3D reconstructed image revealed a caput medusa feature of the veins. GKS was performed to treat a vp-AVM. A follow-up angiogram obtained 24 months later showed a reduction of the vascular staining lesion and a decrease in early venous drainage (Fig. 2).

    case 13A 27-year-old man visited a hospital due to a migraine

    headache with aura in the left temporal area. The brain MRI revealed multiple signal voids in the left posterior temporal lobe. Left vertebral angiography showed poor-ly marginated lesions of arterial blush and early venous drainage with a dilated medullary vein during the mid-arterial phase. The vp-AVM volume measured by MRI based on projections drawn from the angiography images was 5.2 ml, and the marginal dose was 18 Gy. A follow-up angiogram obtained 24 months after GKS showed a reduction of the extent of the arterial blush in the lesion and nearly the disappearance of the early venous drainage. Moreover, the patient’s headache had improved (Fig. 3).

    discussionAtypical venous anomaly consisting of an abnormal ar-

    terial blush and early venous drainage with a dilated med-ullary vein from the arterial or capillary phase, without a definite nidus, has been noted using various terminologies, including vp-AVM. Treatment strategies for vp-AVM re-main unclear because of the limited number of cases and a lack of information on long-term outcomes. Accordingly, selection of treatment modalities is usually conducted ac-cording to the physician’s disease concept and experience. Pereira et al.16 suggested that an increased medullary blush concomitant with an early venous drainage implicates a rapid transit time by an enlarged collector vein. They believed that medullary blush was not related to true AV shunting and rarely was symptomatic. Komiyama et al.10 recommended initial conservative treatment for a venous angioma irrespective of the combination with an AV shunt, due to their benign prognosis. Lasjaunias et al.12 and Goulao et al.3 reported that a DVA is a normal variant of the venous drainage pattern that occurs as a compensatory drainage system. Accordingly, a dilated vein concomitant

    table 2. literature review of studies on abnormal arterial blush and early venous drainage with a dilated medullary vein from the arterial or capillary phase without a discrete nidus

    Authors & YearNo. of Pts (males)

    Mean Age (range)

    Terminology Used to  Describe Diagnosis Treatment Complications

    Awad et al., 1993 3 Mixed VM & AVM Gyrectomy (n = 2), HR + GKS (n = 1)Mullan et al., 1996 3 VM + arterial fistulization Not statedKomiyama et al.,    1999

    3 (2) 24 yrs (22–26) Venous angioma w/ AV shunt Conservative (n = 3) None 

    Im et al., 2008 15 (9) 27.4 yrs (8–71) vp-AVM (n = 15) Conservative (n = 6), GKS (n = 4), HR + GKS (n = 2), resection of venous anomaly (n = 3)

    Rebleeding (n = 1), fatal (n = 1) due to incomplete resection

    Oran et al., 2009 7 (4) 25.6 yrs (6–51) Arterialized DVA (n = 4), arterialized DVA + AVM (n = 3)

    Conservative (n = 2), GKS (n = 2), decompression (n = 1), EE + GKS (n = 1), EE + DVA resection (n = 1)

    Rebleeding (n = 1), radia-tion necrosis (n = 1), EE + DVA resection (n = 1)

    Present series,    2014

    16 (9) 45.3 yrs (16–78) vp-AVM (n = 16) Conservative (n = 8), HR + GKS (n = 4), GKS (n = 4)

    Rebleeding (n = 1) 

    EE = endovascular embolization; Pts = patients; VM = venous malformation. 

    Fig. 2. Case 5. A 55-year-old man presented with ICH in the right frontal area.  a and b: Abnormal arterial blush in the insular area (arrows) during the midarterial phase and an early venous drainage of the sylvian vein (arrow) with a dilated medullary vein during the capillary phase were observed.  c and d: A follow-up angiogram obtained 24 months later after GKS revealed a reduc-tion of the arterial blush and early venous drainage.

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  • J. p. Jeon et al.

    with a venous anomaly may represent fragile vessel walls that are susceptible to hemodynamic compromise by an inadequate response to hemodynamic stress. However, Hi-rata et al.6 and Im et al.8 believed that an atypical venous anomaly (described as mixed venous and arteriovenous malformation, and vp-AVM, respectively) resembles a true AVM based on histological and clinical similarity. In our study, we divided vp-AVMs into 2 groups: high- and low-flow AV shunts. Twelve of our 13 cases with vp-AVMs and a high-flow AV shunt were symptomatic, and 1 of 3 cases with a vp-AVM and low-flow shunt was symptom-atic. Accordingly, the presence of symptoms appears to be associated with the severity of the shunt flow.8

    Treatment modalities included conservative, open sur-gical, GKS, and endovascular intervention. Conservative treatment was performed under the rationale that patients with vp-AVMs experienced benign clinical behavior such as DVAs.10,16 However, vp-AVMs showed histological fea-tures similar to AVMs,6 and 2 cases of rebleeding have been reported previously.8,15 We also experienced 1 case of a rebleeding episode among the patients who underwent conservative treatment. Accordingly, physicians should be

    aware of the possibility of hemorrhagic presentation in pa-tients with vp-AVMs. Open surgery has been performed in vp-AVMs that manifested massive or repetitive bleed-ing. However, poor lesion localization due to the absence of a definite nidus and nonidentifiable enlarged feeding arteries can be challenging for complete resection. Incom-plete resection can lead to catastrophic hematoma, venous infarction, and severe brain swelling. Im et al.8 reported on a fatal complication after an incomplete resection of a vp-AVM. Moreover, lesions located in the eloquent area or deep in the brain are also challenging for resection. Ac-cordingly, resection should be performed only in patients who present with a recurrent hematoma or epilepsy.

    GKS has been used to treat vp-AVMs.2,8,14,15 A general principle of GKS is to cover the extent of the staining area during the arterial phase without harming the main drain-age vein to avoid possible venous infarction or hemor-rhage.13 Good treatment outcomes have been reported for venous anomalies,1,11,13 but complete obliteration has not yet been reported in patients with vp-AVMs. Because the absence of a discrete nidus makes dosimetry and defining the margin difficult, it can be hard to target the lesions. In

    Fig. 3. Case 13. A 27-year-old man presented with migraine headache with an aura in the left temporal area.  a and b: Poorly marginated lesions of arterial blush and an early venous drainage (arrows) with a dilated medullary vein were observed during the midarterial phase.  c and d: The volume measured by MRI based on projections drawn from the angiographic images (white outline) was 5.2 ml, and the marginal dose was 18 Gy.  e and F: Follow-up angiography obtained 24 months after GKS shows a reduction in the extent of the arterial blush and nearly the disappearance of the early venous drainage. The patient’s headache had improved.

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  • venous-predominant arteriovenous malformation

    our cohort, we had 7 cases (87.5%) with a marked reduc-tion in AV shunting and early venous drainage, but none were cured. Radiation-induced complications are a con-cern after GKS. Oran et al.15 reported a case of fatal radio-necrosis after GKS. Although no adverse events associated with GKS were observed in our case series, further study is required to better define the optimal parameters for the radiation dose, patient selection, and treatment strategy for radiation-resistant vp-AVM. Endovascular interventions can also be considered.16,17 In particular, a vp-AVM with an identifiable and prominently enlarged feeding artery could be feasible for selective embolization. However, most vp-AVM cases have shown a diffuse and undefined arterial feeding artery;18 thus, endovascular intervention appears to have limited use in patients with vp-AVMs. Based on our experience and review of the pertinent literature, we believed that symptomatic vp-AVMs with a high-flow shunt could be considered for GKS treatment. Except in those cases with massive or repetitive bleeding, GKS ap-pears to reduce hemodynamic stress in the main drainage system with a dilated vein by decreasing the shunt flow, although it did not reach complete obliteration. Asympto-matic vp-AVMs with or without a high-flow shunt can be treated conservatively.

    The differential diagnosis between a vp-AVM and a true AVM with medullary components is important be-cause a true AVM with medullary components resulted in a higher bleeding rate and poor neurological outcome. AVMs with medullary components are more likely to have enlarged feeding arteries with an edge shape and a stream-ing pattern for the medullary vein;7,10 moreover, a discrete nidus is observed.

    Two concerns remain regarding a GKS indication for patients with vp-AVMs presenting with headache and proper radiation dose. In our study, 3 patients complained of migraine headaches with auras. In addition, the head-aches developed ipsilateral to the vp-AVMs. Although cluster headaches, chronic paroxysmal hemicranias, and short-lasting unilateral neuralgiform headaches with conjunctival injection and tearing can also be associated with AVMs, those are atypical features.5 After consulta-tion with a neurologist, and according to the International Classification of Headache Disorder, 3rd edition criteria,5 we believed that headache was associated with vp-AVMs. During the follow-up period, 2 patients who had under-gone GKS showed reduction of the vascularity, and their migraine headaches were greatly lessened. In a review of the literature, detailed information on the GKS parameters for vp-AVMs has not been well described. We tried to in-clude all abnormal vascular staining lesions in the GKS planning; however, normal brain tissue in the targeted le-sions could also be included to a greater extent and af-fected by the radiation more than that of the AVM, which has a tightly packed nidus. Inoue9 reported that low-dose GKS (≤ 20-Gy margin dose) was feasible for the treatment of AVMs and could decrease the long-term adverse effects to surrounding tissue. Accordingly, we chose a low radia-tion dose targeted to the vp-AVM.

    Our study has several distinctive features. First, our study included the largest sample size of patients with vp-AVMs to date (n = 16). Second, these patients with

    vp-AVMs have been followed for a mean of 79.9 months. Although no direct comparative test among the treatment modalities was conducted due to the small number of cas-es, information on long-term treatment outcomes would be beneficial when selecting the treatment modality.

    conclusionsPoor lesion localization of vp-AVMs makes them chal-

    lenging to treat. Symptomatic patients with a high-flow shunt are supposedly best treated with GKS, despite the fact that only 87.5% of the vp-AVMs treated this way showed a reduction in the malformation volume, and none were cured.

    acknowledgmentWe would like to thank Sung-Eun Kim for help with the data

    collection.

    references 1. Aksoy FG, Gomori JM, Tuchner Z: Association of intracere-

    bral venous angioma and true arteriovenous malformation: a rare, distinct entity. Neuroradiology 42:455–457, 2000

    2. Awad IA, Robinson JR Jr, Mohanty S, Estes ML: Mixed vas-cular malformations of the brain: clinical and pathogenetic considerations. Neurosurgery 33:179–188, 1993

    3. Goulao A, Alvarez H, Garcia Monaco R, Pruvost P, Lasjau-nias P: Venous anomalies and abnormalities of the posterior fossa. Neuroradiology 31:476–482, 1990

    4. Han JH, Kim DG, Chung HT, Paek SH, Park CK, Jung HW: Radiosurgery for large brain metastases. Int J Radiat Oncol Biol Phys 83:113–120, 2012

    5. Headache Classification Committee of the International Headache Society: The International Classification of Head-ache Disorders, 3rd edition (beta version). Cephalalgia 33:629–808, 2013

    6. Hirata Y, Matsukado Y, Nagahiro S, Kuratsu J: Intracerebral venous angioma with arterial blood supply: a mixed angioma. Surg Neurol 25:227–232, 1986

    7. Huang YP, Robbins A, Patel SC, Chaudhary M: Cerebral venous malformations and a new classification of cerebral vascular malformations, in Kapp JP, Schmidek HH (eds): The Cerebral Venous System and Its Disorders. Orlando: Grune & Stratton, pp 373–474, 1984

    8. Im SH, Han MH, Kwon BJ, Ahn JY, Jung C, Park SH, et al: Venous-predominant parenchymal arteriovenous malforma-tion: a rare subtype with a venous drainage pattern mimick-ing developmental venous anomaly. J Neurosurg 108:1142–1147, 2008

    9. Inoue HK: Long-term results of Gamma Knife surgery for arteriovenous malformations: 10- to 15-year follow up in patients treated with lower doses. J Neurosurg 105 Sup-pl:64–68, 2006

    10. Komiyama M, Yamanaka K, Iwai Y, Yasui T: Venous an-giomas with arteriovenous shunts: report of three cases and review of the literature. Neurosurgery 44:1328–1335, 1999

    11. Kurita H, Sasaki T, Tago M, Kaneko Y, Kirino T: Successful radiosurgical treatment of arteriovenous malformation ac-companied by venous malformation. AJNR Am J Neurora-diol 20:482–485, 1999

    12. Lasjaunias P, Burrows P, Planet C: Developmental venous anomalies (DVA): the so-called venous angioma. Neurosurg Rev 9:233–242, 1986

    13. Lindquist C, Guo WY, Karlsson B, Steiner L: Radiosurgery for venous angiomas. J Neurosurg 78:531–536, 1993

    14. Mullan S, Mojtahedi S, Johnson DL, Macdonald RL: Cere-

    J Neurosurg  Volume 124 • April 2016 1105

    Unauthenticated | Downloaded 07/01/21 05:10 PM UTC

  • J. p. Jeon et al.

    bral venous malformation-arteriovenous malformation transi-tion forms. J Neurosurg 85:9–13, 1996

    15. Oran I, Kiroglu Y, Yurt A, Ozer FD, Acar F, Dalbasti T, et al: Developmental venous anomaly (DVA) with arterial compo-nent: a rare cause of intracranial haemorrhage. Neuroradiol-ogy 51:25–32, 2009

    16. Pereira VM, Geibprasert S, Krings T, Aurboonyawat T, Ozanne A, Toulgoat F, et al: Pathomechanisms of symptom-atic developmental venous anomalies. Stroke 39:3201–3215, 2008

    17. Roh JE, Cha SH, Lee SY, Jeon MH, Cho BS, Kang MH, et al: Atypical developmental venous anomaly associated with single arteriovenous fistula and intracerebral hemorrhage: a case demonstrated by superselective angiography. Korean J Radiol 13:107–110, 2012

    18. Ruíz DS, Yilmaz H, Gailloud P: Cerebral developmental ve-nous anomalies: current concepts. Ann Neurol 66:271–283, 2009

    disclosureThe authors report no conflict of interest concerning the materi-als or methods used in this study or the findings specified in this paper.

    author contributionsConception and design: JE Kim. Acquisition of data: Ahn, WS Cho, Kang, Chung. Analysis and interpretation of data: JE Kim, Jeon, Sohn, YD Cho, Chung, DG Kim. Drafting the article: Jeon. Critically revising the article: Son, Bang, Oh.

    correspondenceJeong Eun Kim, Department of Neurosurgery, Seoul National University College of Medicine, Seoul National University Hos-pital, 101 Daehak-ro, Jongno-gu, Seoul 110-744, Korea. email: [email protected].

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