Intracranial Arteriovenous Malformation

Author: David Altschul, MD, Neurological Surgery Resident, Montefiore Medical Center Coauthor(s): Michael Smith, MD, Staff Physician, Department of Neurosurgery, Hospital of the University of Pennsylvania; Grant P Sinson, MD, ssociate Professor, Department of Neurosurgery, Medical College of !isconsin Contri"utor #nformation and Disclosures Updated$ Mar %&, %''(

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Introduction
"normalities of the vascular structures of the head and "rain have long "een recogni+ed, McCormicpu"lished an influential classification system in his .(&& paper, /)he Pathology of 0ascular 12 rteriovenous23 Malformations,/. He descri"ed the arteriovenous malformation 1 0M3, cavernous malformation, venous malformation, telangiectasia, and vari4, #ntracranial varices are not of significant current clinical concern, )his overvie5 focuses on the previous 6 classes, 667year7old 5oman presented 5ith left7sided hemiparesis and intraventricular hemorrhage on her head C), )his a4ial )%7 5eighted MR# sho5s arteriovenous malformation nidus on the right side, )he arro5s demonstrate large draining veins 5ithin the right lateral ventricle,

A 44- ear-old !oman "resented !ith left-sided hemi"aresis and intraventricular hemorrha#e on her head C$% $his a&ial $'-!ei#hted M(I sho!s arteriovenous malformation nidus on the ri#ht side% $he arro!s demonstrate lar#e drainin# veins !ithin the ri#ht lateral ventricle% 8 C9:S* !#ND:! ;

A 44- ear-old !oman "resented !ith left-sided hemi"aresis and intraventricular hemorrha#e on her head C$% $his a&ial $'-!ei#hted M(I sho!s arteriovenous malformation nidus on the ri#ht side% $he arro!s demonstrate lar#e drainin# veins !ithin the ri#ht lateral ventricle%

Arteriovenous Malformation
)istor 9usch-a and 0ircho5 originally descri"ed arteriovenous malformations 1 0Ms3 in the mid .<''s, :livecrona performed the first surgical e4cision in .(=%, Definition 9esions of the cere"ral vasculature develop such that "lood flo5s directly from the arterial system to the venous system 5ithout passing through a capillary system, )he arteriovenous 1 03 shunt is the definitive characteristic of these lesions, *re+uenc )he estimated incidence of 0M in the US general population is ',.6> 1.6' cases per .'',''' persons or . case per ?'' persons3, )his is appro4imately one fifth to one seventh the incidence of intracranial aneurysms, ,tiolo# :ne or more persisting direct connections from the arterial to the venous systems are present, 0Ms are considered congenital lesions and are characteri+ed "y a failure of the em"ryonic vascular ple4us to fully differentiate and develop a mature capillary "ed in the affected area, )he formation of 0Ms pro"a"ly relates to se@uential formation and resorption of cere"ral surface veins, )heir structures may change and gro5 postnatally "ut only in relation to a prenatally e4tant lesion, Molecular "iologic factors are thought to "e important to 0M development, )hese may include vascular endothelial gro5th factor 10*AB3 and "asic fi"ro"last gro5th factor 1"BAB3, )issues adCacent to the 0M may "e persistently mildly hypo4ic "ecause the malformation may steal "lood from adCacent healthy tissue, further promoting angiogenesis, Patho"h siolo# )he direct connection "et5een the arterial and venous systems supplies a lo57resistance shunt for arterial "lood and e4poses the venous system to a"normally high pressures, )his results in a system of enlarged feeding vessels, the tangled nidus of the 0M itself, and enlarged draining venous structures, Clinical "resentation

Hemorrhage 1D=> of cases3 cute onset of severe headache May "e descri"ed as the 5orst headache of the patient2s life Depending on the location of hemorrhage, may "e associated 5ith ne5 fi4ed neurologic deficit Sei+ure 16&>3 Headache 1=6>3 Progressive neurologic deficit 1%.>3 May "e caused "y mass effect or ischemia resulting from local vascular steal phenomenon

Presence and nature of deficit depend on location of lesion Pediatric patients Heart failure Macrocephaly Prominent scalp veins

Hemorrhage is more li-ely to "e caused "y small lesions, 5hile sei+ures are more li-ely to "e caused "y large lesions, n increasing num"er of lesions no5 are found incidentally upon "rain imaging, )he annual ris- of intracranial hemorrhages associated 5ith 0Ms is %7=>, )he mortality rate associated 5ith the initial "leed is .'>, )he mortality rate associated 5ith the second "leed is .=>, and the rate increases to %'> for each su"se@uent "leed, )he incidence of ne5 neurologic deficit occurring 5ith each "leed is D'>, )hese num"ers are generali+ed for all 0Ms, )he location and si+e of each patient2s lesion greatly affects their ris- of mor"idity and mortality, Patients 5ith an 0M have an increased ris- of developing a cere"ral aneurysm, ppro4imately ?,&> of patients 5ith an 0M develop an aneurysm, lternatively, .,.> of patients 5ith an aneurysm are found to have an 0M, Most commonly, aneurysms are found on arteries feeding the 0M, Rare case reports descri"e multiple intracranial 0Ms andEor concomitant intracranial and intraspinal 0Ms, "ut these are too rare to "e 5ell characteri+ed, 0Ms may present as part of a neurocutaneous syndrome, including Sturge7!e"er syndrome or Rendu7:sler7!e"er syndrome, -or.u"

9a" studies 1these are for routine preoperative evaluation and not for diagnosis, per se3 CFC count Prothrom"in time 1P)3Eactivated partial throm"oplastin time 1aP))3 )yping Screening #maging studies C) scan is the first imaging choice in an emergent setting in 5hich a patient is thought to have an intracranial "leed, #t is fast and 5idely availa"le, C) angiography provides "etter vascular detail than MR# or magnetic resonance angiography 1MR 3, and can "e performed in most emergency rooms, #t can "e a useful non7invasive alternative to cere"ral angiography, MR# is the first imaging choice in a nonemergency setting 1eg, 5or-up of sei+ure, headache, or neurologic deficit3, #t provides significantly greater resolution and fle4i"ility in diagnosis, MR s can reveal the anatomy of the 0M noninvasively "ut have lo5er resolution than conventional angiography and do not provide functional information, MR# is useful compared to computed tomoangiography 1C) 3 or cere"ral angiography in that it provides "etter visuali+ation of the surrounding cere"ral structures, Bunctional MR# can assist in treatment planning "y defining the functionality of adCacent "rain, Cere"ral angiography provides definitive diagnosis, #t documents a functional 0 shunt; ho5ever, "ecause it is an invasive test, it is not performed as the first imaging study, Cere"ral angiography also allo5s grading of the 0M via the follo5ing Spet+ler and Martin criteria, )he Spet+ler7Martin grading system descri"ed in )a"le . helps predict the li-elihood of satisfactory outcome if an attempt at surgical resection is made, )he Spet+ler7Martin grade is determined "y adding the = individual scores from the ta"le, High7grade 0Ms are more difficult to resect, and, therefore, neurologic deficits from the surgery itself are more li-ely, :ther grading systems have "een proposed for radiosurgery planning,

Spet+ler7Martin Arading System for 0Ms :pen ta"le in ne5 5indo5 8 C9:S* !#ND:! ;
$a/le

Si0e of A1M2 ,lo+uence of ad3acent /rain4 Pattern of venous draina#e5 Small 1G= cm3 . Nonelo@uent ' Superficial only ' Medium 1=7& cm3 % *lo@uent . Deep component . 9arge 1H& cm3 = I I 4 Si0e of A1M2 ,lo+uence of ad3acent /rain Pattern of venous draina#e5 Small 1G= cm3 . Nonelo@uent ' Superficial only ' Medium 1=7& cm3 % *lo@uent . Deep component . 9arge 1H& cm3 = I I J Measure the largest diameter of the nidus of the lesion on angiography, K *lo@uent areas include sensorimotor, language, visual, thalamus, hypothalamus, internal capsule, "rain stem, cere"ellar peduncles, and deep cere"ellar nuclei, L )he lesion is considered superficial only if all drainage is via the cortical drainage system, $reatment )rials directly comparing treatment approaches are lac-ing, and information on outcomes derives largely from case series, Complete o"literation is the goal of treatment as partial o"literation does not affect the rate of hemorrhage,

Surgery 1craniotomy3 dvantages$ Cure is immediate and permanent after complete resection "y craniotomy, Surgery is generally recommended for grade ., %, and = lesions, sometimes for grade 6 lesions, and not for grade D lesions, Disadvantages$ )he potential for significant intraoperative "leeding, damage to adCacent neural tissue, and ischemic stro-e are disadvantages, )he /arteries of passage/ supply intact neural tissue and must not "e destroyed 5hile attempting to interrupt the arterial supply to the 0M, ris- also e4ists for perfusion7"rea-through "leeding, ie, hemorrhage into the healthy part of the "rain caused "y sudden hemodynamic shifts, 5hich results from the removal of a large 0 shunt and the su"se@uent increased flo5 to previously underperfused vessels, Reports of rates of permanent neurologic deficit range from '7.D>, 5hile mortality rates are close to +ero in Spet+ler7Martin grades .7= 0Ms, Much higher mor"idity and mortality rates are reported 5ith grades 67D 0Ms, *ndovascular neurosurgery 1o"literating vessels 5ith glues or particles delivered via arterial catheter in the angiography suite3 dvantages$ Significant reduction of pathologic "lood flo5 through the lesion can "e achieved, #ts main use is as adCuvant therapy prior to craniotomy to decrease intraoperative "leeding and technical difficulty, #t has also "een used to decrease the si+e of an 0M to ma-e it sufficiently compact for effective targeting "y stereotactic radiosurgery, *m"oli+ation may "e curative in lesions less than . cm in diameter that are fed "y a single artery, #mproved o"literation rates of appro4imately %'> have "een reported 5hen using the em"olic agent :ny4,

Disadvantages$ )his is an invasive procedure, and its maCor ris-s are similar to those for open surgery, ie, ischemia and hemorrhage, )he main ris- is causing ischemic stro-e "y occluding a feeding vessel that also supplies normal "rain, Postem"oli+ation hemodynamic alterations can cause rupture of the 0M, resulting in ne5 neurologic deficit from su"arachnoid andEor intraparenchymal hemorrhage, analogous to the perfusion7"rea-through "leeding descri"ed a"ove, )his techni@ue is not normally used "y itself, as it rarely achieves complete eradication of the lesion and the pathologic vessels usually recanali+e over time, Stereotactic radiosurgery dvantages$ Stereotactic radiosurgery is noninvasive and can access all anatomic locations of the "rain, Ne5 techni@ues availa"le include staged radiosurgery for larger lesions 1Arade #0 and 03, 5hich has promising results, Disadvantages$ #t can only treat smaller lesions 1G= cm in diameter3 and re@uires % or more years for a full destructive effect, )he ris- for hemorrhage is not reduced during this lag time, )he ris-s of radiation necrosis of adCacent healthy tissue or cyst formation also e4ist, )he cure rates for lesions smaller than = cm range from <.7('>, )herefore, a small su"set of these lesions still hemorrhage after treatment, Com"ination therapy )otal eradication of the lesion may re@uire more than one modality, Partial treatment may increase the ris- of hemorrhage, *ndovascular neurosurgery can "e performed "efore surgical e4cision to reduce the difficulty of surgery or "efore radiosurgery to "ring the si+e of the lesion to 5ithin the limits of the machine, Radiosurgery may "e used to eradicate small residual disease left after craniotomy 1due to technical difficulty or involvement of elo@uent structures3, neurysms associated 5ith 0M neurysms on an artery that does not feed the 0M can "e managed as any unruptured intracranial aneurysm, neurysms less than D mm in si+e have "een reported to regress after treatment of 0M; in other cases, they have ruptured after treatment, Aiven concern a"out aneurysms greater than D mm, treatment via microsurgical clipping or endovascular coiling is generally done prior to the treatment of the 0M,

6utcome and "ro#nosis Prognosis for untreated 0Ms has "een reported to include either a %7=> or a 6> 1depending on study cited3 ris- of "leeding per year, 5ith an appro4imate .'> mortality rate associated 5ith the "leed, Some studies suggest a .> yearly mortality ris-, )he yearly com"ined maCor mor"idity and mortality ris- has "een reported to "e %,?>, )his figure includes only ne5 clearly fi4ed neurologic deficits, #t does not include other pro"lems, such as sei+ures, personality changes, or memory distur"ances, 5hich also may have a significant disa"ling impact on the patient2s life and functioning, Ris-s of conservative management and surgical intervention vary dramatically depending on the location and specific characteristics of individual lesions, 5hich must "e considered 5hen applying this data to each patient, )ypically, attempts should "e made to completely eradicate the lesion, Regardless of 5hich method or com"ination of methods is used for treatment, eradication is considered definitive and recurrence is not a concern 1e4cept in occasional reports in the pediatric population3, :utcomes for patients 5ith a su"arachnoid hemorrhage caused "y an 0M are "etter than outcomes for

patients 5ith a su"arachnoid hemorrhage caused "y an aneurysm, :utcome studies suggest that most patients return to full preoperative psychosocial function, *uture and controversies Multidisciplinary teams li-ely 5ill "ecome increasingly important for optimal management of each individual patient, Such teams may include neurosurgeons, interventional neuroradiologists, and stereotactic radiation specialists, Continuing advances in microsurgical, neurointerventional, and radiosurgical techni@ues 5ill affect approaches to treatment, lgorithms guiding 5hether to treat or follo5 particular lesions 5ill evolve, dvances in the efficacy and safety of the availa"le interventions may emerge, Patients2 individual psychological factors 5ill remain critical, Bor every patient, the immediate ris-s of intervention versus the li-elihood of cure must "e "alanced, Randomi+ed )rial of Unruptured Frain rteriovenous Malformations 8 RUF ;, an ongoing multicenter randomi+ed trial, is comparing treatment of unruptured lesions 5ith conservative management, )his study 5ill "e of great assistance in management decisions 5hen the results are pu"lished,%