Recurrent Arteriovenous Malformations Research Articles

New nidus formation adjacent to the target site of an arteriovenous malformation treated by Gamma Knife surgery

New nidi are rarely found adjacent to the resection margin following treatment for an arteriovenous malformation (AVM), especially in adults. In addition, there are no reports in adults of new nidus formation adjacent to the targeted site of an AVM that angiography has verified to be completely obliterated by radiosurgery. The authors present their experience with recurrent AVMs following AVM radiosurgery in 3 patients whose ages were 9 years, 10 years, and 33 years. None of the patients had been treated with embolization before radiosurgery. Two patients had a history of intracerebral hemorrhage before radiosurgery. New lesions developed around the obliterated nidi in all 3 cases. Angiography performed after the first radiosurgery confirmed complete removal of the nidus in all 3 patients, and new nidus formation was detected 31, 132, and 36 months after the initial GKS. The new lesions were also treated by GKS. Occasionally, in patients with recurrent AVMs, such as those described in this paper, long-term clinical and angiographic follow-up may be required, even if complete occlusion is originally shown on angiograms.

Influence of the combination of patient age and deep venous drainage on brain arteriovenous malformation recurrence after surgery

Case reports suggest that young age is a critical factor in determining recurrence of brain arteriovenous malformations (AVMs) after surgery. However, other factors that may contribute to the increased risk of recurrence have not been considered. In this study, the authors' goal was to ascertain the risk and risk factors of recurrence after resection of AVMs of the brain. A consecutive case series (prospectively collected data) of 600 cases of resection of brain AVMs was retrospectively analyzed. Radiological evidence of recurrence or nonrecurrence, as well as clinical evidence of recurrence, could be established in 427 of these cases that underwent follow-up for more than 350 days after initial surgery. These cases were analyzed using Kaplan-Meier curves and Cox regression with respect to age and the presence of deep venous drainage. Nine recurrent AVMs were found in 8 patients. By analysis of the Kaplan-Meier curves, the 10-year recurrence rate was 14% for those with deep venous drainage, compared with 4% for those without deep venous drainage. Stratifying by age, in the 0- to 20-year age group, the 10-year recurrence rates were 63% and 13% for those with and without deep venous drainage, respectively. In the 20- to 39-year age group, the rates were 5% and 0% respectively, and in the 40-year and older age group they were 0% and 3%, respectively. The hazard ratio for deep venous drainage, adjusted for age, was 5.97 (95% CI 1.20-29.69, p = 0.029). The risk of recurrence after AVM resection is significant for young patients with deep venous drainage.

Long Term Outcome of Arteriovenous Malformation Bleeding in Patients with HeartMateII Left Ventricular Assist Device

Arteriovenous malformation (AVM) has been well recognized as a cause of recurrent gastrointestinal (GI) bleeding in patients with continuous flow left ventricular assist device (LVAD). We compared the long-term outcome of patients with AVM bleeding vs. non-AVM bleeding in HeartMateII (HMII) recipients. AVM bleeding in LVAD patients may be associated with worse clinical outcome than non-AVM GI bleeding. Twenty-four consecutive patients who were admitted for GI bleeding were identified from retrospective chart review of all HMII implants. Patients with GI bleeding during the admission for LVAD implantation were excluded. Recurrence was defined as an episode of GI bleeding necessitating hospitalization. Mean age was 64±9 years (58% bridge to transplantation). Of 24 patients with GI bleeding, 16 (67%) patients had AVM and 8 patients (33%) had other diagnoses (3 gastric ulcer or gastritis, 2 hemorrhoid, 1 rectal ulcer, 1 diverticular, 1 ischemic colitis). Demographic variables such as age, previous GI bleeding history and target INR were similar (p=NS) in both groups. Time period from LVAD implantation to first bleeding was also comparable (day 351±424 vs. day 296±459, respectively; p=NS). However, patients with AVM bleeding tended to have lower mean blood pressure (73±16 vs. 88±11; p=0.04) and lower nadir hemoglobin level (6.6±1.5 vs. 8.6±2.1; p=0.01). In addition, patients with AVM bleeding required more units of red cell transfusion (5.5±4.3 vs. 1.6±2.4; p=0.03). Over 2.9±2.0 years of follow-up, 12 (75%) patients had recurrent AVM bleeding compared to 3 (50%) patients with rebleeding from other causes (p=0.01). However, 3-year survival rate was not different in both groups (log-rank test, p=0.26). HMII patients who have GI bleeding from AVM were observed to have a lower mean BP at presentation, require more red cell transfusions and have a higher rate or recurrent bleeding compared to those with non-AVM bleeding. However, mortality at 3 year follow-up is comparable in both groups.

Follow-up imaging to detect recurrence of surgically treated pediatric arteriovenous malformations

The true postoperative incidence of arteriovenous malformation (AVM) recurrence in the pediatric population remains largely unreported. Some literature suggests that delayed imaging studies should be obtained at 6 months to 1 year after negative findings on a postoperative angiogram. The aim of this study was to describe the timing of AVM recurrences after resection and the neuroimaging modalities on which the recurrences were detected. This study was performed in a retrospective cohort of all pediatric patients treated surgically for AVM resection by a single neurosurgeon between 2005 and 2010. Patients were followed after resection with MR angiography (MRA) or conventional angiography, when possible, at various time points. A visual scale for compactness of the initial AVM nidus was used, and the score was correlated with probability of recurrence after surgery. A total of 28 patients (13 female, 15 male) underwent an AVM resection. In 18 patients (64.3%) an intraoperative angiogram was obtained. In 4 cases the intraoperative angiogram revealed residual AVM, and repeat resections were performed immediately. Recurrent AVMs were found in 4 children (14.3%) at 50, 51, 56, and 60 weeks after the initial resection. Recurrence risk was 0.08 per person-year. No patient with normal results on an angiogram obtained at 1 year developed a recurrence on either a 5-year angiogram or one obtained at 18 years of age. All patients with recurrence had a compactness score of 1 (diffuse AVM); a lower compactness score was associated with recurrence (p = 0.0003). All recurrences in this cohort occurred less than 15 months from the initial resection. The authors recommend intraoperative angiography to help ensure complete resection at the time of the surgery. Follow-up vascular imaging is crucial for detecting recurrent AVMs, and conventional angiography is preferred because MRA can miss smaller AVMs. One-year follow-up imaging detected these recurrences, and no one who had negative results on an angiogram obtained at 1 year had a late recurrence. However, not all of the patients have been followed for 5 years or until 18 years of age, so longer follow-up is required for these patients. A lower compactness score predicted recurrent AVM in this cohort.

Endovascular treatment combined with emboloscleorotherapy for pelvic arteriovenous malformations

To describe the clinical features and treatment outcomes after combined endovascular and embolosclerotherapy treatment of pelvic arteriovenous malformations (AVMs). From November 1996 to May 2011, we treated 12 patients (seven males, five females; mean age, 38.1 ±14.6 years; age range, 23-70 years) with pelvic AVMs. Our treatment strategy was coil embolization of a dilated draining vein of the AVM to reduce blood flow velocity in the AVM lesion and sclerotherapy of the residual arteriovenous fistulae with high-concentration ethanol to eradicate potential AVM recurrence. To ensure effective endovascular treatment, we used transarterial, transvenous, percutaneous, or combined access routes. Treatment outcomes were assessed with periodic computed tomography angiograms and clinical examinations. During the follow-up period (mean, 33.2 months; median, 21.3 months; range, 1-96 months) after embolosclerotherapy, we observed complete remission (no residual or recurrent AVM lesion on follow-up computed tomography and complete symptomatic relief) in 10 (83.3%) patients and partial remission in two (16.7%) patients. One major complication of focal bladder necrosis occurred (1/22 sessions, 4.5%, 1/12 patients, 8.3%), but this resolved with conservative treatment. As early procedure-related complications, transient pulmonary hypertension and/or hemoglobinuria developed in 54.5% (12/22 sessions) and 22.7% (5/22 sessions) of patients, respectively. Combined embolosclerotherapy of pelvic AVMs with coils and ethanol was efficacious at achieving complete remission.

Supernova hemorrhage: obliterative hemorrhage of brain arteriovenous malformations following gamma knife radiosurgery

Hemorrhage represents the most feared complication of cerebral arteriovenous malformations (AVMs) in both untreated patients and those treated with gamma knife radiosurgery. Radiosurgery does not immediately lead to obliteration of...

Asymptomatic arteriovenous malformation recurrence in an adult

Dear Editor,Consensus exists that angiographically confirmed completeresection of cerebral arteriovenous malformations (AVM)can be regarded as the definite treatment. Only a fewreports of AVM recurrences—mostly in children—areavailable so far [8, 9, 13, 14]. AVM recurrence in adultsseems to be extremely rare, with only five case reportsexisting to date [1, 3, 4, 6, 11]. We present the first reportedcase of asymptomatic AVM recurrence in an adult.In June 2008, a 30-year-old female suffered a spontane-ous intracerebral hematoma in the left parietal lobeinvolving the corpus callosum and the cingulate gyrus,leading to acute headaches, vomiting, coordination disorder,and right-sided hemihypaesthesia. The haemorrhage wasinduced by a small AVM in the left parietal lobe (Fig. 1a).Surgery was performed 1 month after the bleeding withoutprevious embolization. Angiography (DSA) on postopera-tive day 6 confirmed the complete removal of the AVM(Fig. 1b). Postoperatively, the patient was able to return toall activities of daily life, including work, without perma-nent impairment. In November 2010, MRI performedbecause of chronically persistent headaches revealed anew vascular convolute at the dorsolateral resection margin(Fig. 1c, d) confirmed by a subsequent DSA as an AVMrecurrence (Fig. 1e, f). The patient underwent a secondsurgery, again with angiographically verified completeAVM resection (Fig. 1g).Our case confirms that even adults are at risk for AVMrecurrence despite initial inconspicuous postoperative angio-graphic control. In the previously reported cases, AVMrecurrence was accompanied by haemorrhagic events orseizures [1, 3, 4, 6, 11]. Codd et al. even described a doublerecurrence. In this case the second recurrence was detectedon a routine DSA indicated because of the first one [1]. Inour patient the recurrent AVM was detected by MRI carriedout because of chronically persistent headaches with no otherspecific symptoms.Among the described recurrent cases in adults [1, 3, 4, 6,11], includingourcase,AVMswerelocalizedbothineloquentand non-eloquent regions, with both deep and superficialvenous drainage, respectively, rendering these parametersobviously not relevant for recurrence. Time to recurrenceranged between 10 months and 9 years. Patient age rangedbetween 17 and 33 years at the time of the initial surgery, andbetween 21 and 40 years at the detection of the recurrence.Remarkably, all of them were small in size (<3 cm).Various possible mechanisms of AVM recurrence arediscussed in the literature. One explanation is an incompleteresection disguised by vasospasm or thrombosis withinresidual AVM compartments [6]. The second theoryassumes genetically predisposed abnormal angiogenesismediated by increased vascular endothelial growth factor(VEGF) [12] or cellular signalling proteins pERK (phos-phorylated extracellular signal–regulated kinase) andCD105 (endoglin), respectively [13]. The third possibleexplanation is a “hidden compartment” [10]: AVMs, beingmulticompartmental, may comprise a “reserve nidus”,which remains angiographically hidden because of verylow or absent blood flow in its vessels as a result of internalsteel as long as “active compartments” seen on angiogramare not resected.Regardless of the pathological mechanism, AVM recur-rences remain a rare but potentially serious sequel aftercomplete surgical obliteration. The actual rate of recurrence

Arteriovenous malformation of the conus supplied by the artery of Desproges-Gotteron

The artery of Desproges-Gotteron is rarely mentioned in the literature and is unfamiliar to most neurosurgeons. The authors report a unique case of an arteriovenous malformation (AVM) of the conus in an adult woman, which received blood supply from an artery of Desproges-Gotteron. The patient presented with intermittent pain radiating down the right posterior thigh and foot and transient bladder incontinence. On examination, there was weakness of the right lower limb with hypalgesia of the plantar aspect of the right foot. Magnetic resonance imaging revealed a mass near the anterior aspect of the conus medullaris and angiography confirmed a spinal AVM at the L-1 level and a shunt located at the inferior L-3 level. The patient underwent transarterial embolization, and at 2-year follow-up, repeat angiography demonstrated no evidence of residual or recurrent spinal AVM, intermittent and tolerable pain without treatment interventions, and a normal neurological examination. The artery of Desproges-Gotteron appears to be a rare arterial variation. Moreover, the authors believe this to be the first case of a conal AVM supplied by such an artery. The anatomy and implications of such an arterial variant are discussed.

Recurrence of a cerebral arteriovenous malformation following complete surgical resection: A case report and review of the literature

Background:Angiography-confirmed complete resection of an arteriovenous malformation (AVM) has traditionally been considered curative. However, recurrence of AVM following angiographically proven complete resection does exist, especially in children. This rare occurrence has been reported 29 times in the English language literature. Although recurrence may be asymptomatic, many reported cases result in epilepsy or intracranial hemorrhage anywhere from 0.5 to 9 years following complete resection. We report a rare case of AVM recurrence that became symptomatic 16 years after complete resection. We review the literature and discuss the relevance of performing follow-up imaging to detect AVM recurrence.Case Description:An 8-year-old girl presented with a right occipital hemorrhage with intraventricular extension from a ruptured AVM of the right occipital lobe. She underwent AVM resection through a right occipital craniotomy. Postoperative angiography confirmed complete resection and she made an uneventful recovery. Sixteen years later, she presented with a 2-month history of headaches, nausea and dizziness. Angiography revealed recurrence of the AVM which was completely resected, as documented on postoperative angiography.Conclusion:In children, an AVM may recur after angiography-proven complete resection. Recurrence may be due to persistence and growth of an initially angiographically occult arteriovenous shunt left in place during surgery or the development of a new AVM. In addition to obtaining follow-up angiography 6-12 months after surgery, a late angiography 5 years after resection may be warranted in patients at risk for recurrence. Asymptomatic recurrence detection allows treatment and may prevent the morbidity associated with intracranial hemorrhage.

Multiple, Recurrent, Refractory Vascular Malformations as the Primary Presenting Feature of a PTEN Mutation

Complete history and physical examinations are very important in patients with a vascular anomaly. This brief report highlights the case of a five-year-old male who presented with recurrent arteriovenous malformations of the hand and forearm. The patient also demonstrated additional clinical and diagnostic imaging features consistent with a PTEN Hamartoma-Tumor syndrome. The prompt identification of individuals with this condition is important, given the more locally aggressive nature to the malformations and the increased potential for future malignant disease. The etiology and clinical features associated with PTEN Hamartoma-Tumor syndromes are reviewed in this case.

Intramuscular Arteriovenous Malformation of the Gluteus Maximus Muscle

Extracranial arteriovenous malformations (AVMs) are far less common than intracranial AVMs. AVMs mostly involve the skin, subcutaneous tissue and even the musculoskeletal system extensively. The goal of treatment is to control the AVM, using tumor debulking or arterial embolization; complete tumor excision is usually not possible and the local recurrence rate is high. Intramuscular AVMs are rare and these lesions can be curable with complete surgical resection. We present a case of a rare intramuscular AVM of the right gluteus maximus muscle in a 33-year-old man. The AVM had undergone progressive enlargement and produced pulsation and pain. We performed selective embolization and radical excision of the AVM with removal of the entire muscle. There was no local recurrence of the AVM and the patient’s right hip extension and external rotation had recovered well at a 12-month follow-up. AVMs are extremely rare in skeletal muscle and less than 1% of vasoformative tumors throughout the body occur in such locations. The treatment of choice is a combination of selective embolization and radical excision to avoid recurrence. In fact, intramuscular AVMs are curable compared with those in other sites.

O-007 Treatment of pial arteriovenous malformations: single institution experience at Children's hospital boston

IntroductionPediatric cerebral arteriovenous malformations (AVMs) present an ongoing risk of hemorrhage compounded by long life expectancy. AVMs can be treated with microsurgery, embolization or radiosurgery, alone or in combination. We...

Complete angiographic obliteration of intracranial AVMs with endovascular embolization: incomplete embolic nidal opacification is associated with AVM recurrence

BackgroundEmbolization of intracranial arteriovenous malformations (AVMs) is generally a preoperative adjunctive procedure in the USA. However, sometimes embolization can result in complete angiographic obliteration of the AVM. There is significant...

Endovascular Treatment Increases but Gamma Knife Radiosurgery Decreases Angiogenic Activity of Arteriovenous Malformations

To compare the angiogenic potentials of embolized, gamma knife-treated or untreated cerebral arteriovenous malformations (AVMs), using a rat cornea angiogenesis model. Tissue samples from cerebral AVM patients who were either untreated or had previously been treated with embolization or gamma knife radiosurgery and who had undergone operations for hemorrhage at the Neurosurgery Department or the Neurological Sciences Institute of Marmara University were used. For the macroscopic evaluation of angiogenesis, tissue samples were inoculated in a micropocket created on the rat eye, and the level of angiogenic activity was graded macroscopically for 15 days, with glioblastoma multiforme and normal brain artery tissues serving as positive and negative controls, respectively. For the other part of the experiment, eyes of another set of rats were inoculated with the study samples only using the same cornea angiogenesis model, in which microvessel count and vascular endothelial growth factor assessment was done at days 3, 7, 11, and 15. Based on our macroscopic findings in the cornea angiogenesis model, embolized AVMs exhibited the highest angiogenic activity, followed by untreated AVMs and gamma knife-treated AVMs. Evaluations of vascular endothelial growth factor expression and microvessel counts showed a similar relation among the 3 tissue groups with regard to the level of angiogenic activity, supporting the results of macroscopic examinations. This study, for the first time, provides experimental semiquantitative data to compare the angiogenic potentials of embolized and gamma knife-treated AVM tissues. Embolization may increase angiogenic activity, and gamma knife radiosurgery may decrease it when compared with activity in previously untreated AVMs. These data can be useful to understand why recurrence of AVMs after angiographically demonstrated endovascular occlusion is common but after gamma knife occlusion is rare.

Postnatal Development of Arteriovenous Malformations

Background: Arteriovenous malformations (AVMs) are commonly thought to be congenital malformations; however, patients usually present in adolescence or adulthood. This study was carried out to better understand the development of AVMs between birth and presentation. Methods: A retrospective review was performed of the medical records of all patients under 25 presenting to a single institution with an AVM or spontaneous intracerebral hemorrhage from 2000 to 2007. Results: Out of a total of 34 cases, 3 children were identified with delayed de novo appearance of an AVM after an intracerebral hemorrhage and normal initial angiography. The clinical and angiographic features are presented for these 3 patients and for an additional patient with AVM recurrence after complete surgical excision. Conclusions: In the light of these cases and a review of the literature, we suggest a hypothesis that AVMs develop postnatally, undergoing a period of growth in childhood or early adulthood, and that they may become symptomatic from the time of their earliest development. The trigger to growth may be shear stress stimulating growth factor expression by endothelial cells lining an arteriovenous fistula. Alternative stimuli, such as venous hypertension, cannot be ruled out.

Linear vein–based arteriovenous malformations in children

The authors reviewed the experience of a single surgeon in the surgical treatment of arteriovenous malformations (AVMs) in children, with specific attention to the angioarchitectural appearance of these lesions. Methods The authors performed a retrospective review of pediatric cases of AVM treated at a single institution over a 15-year period. Inclusion criteria consisted of pediatric age at time of treatment and resection of a pial AVM. The AVMs were considered linear-based if they had a single, centrally located, dominant vein with a linear configuration rather than the typical central nidus. Sixty-seven patients, ranging in age from 6 months to 17 years, underwent surgical treatment of an AVM between January 1, 1990, and December 30, 2005. In these patients, 31 AVMs (46%) had a distinct linear configuration that was centered on a dominant draining vein ("vein-based"). These AVMs presented with hemorrhage (61%), incidental findings on imaging (16%), or seizures (13%). In 4 cases, AVM recurrence was noted on angiographic follow-up after postoperative angiography showed no residual lesion. Recurrence was seen at 1 year in 2 cases, and at 3 and 11 years in 1 case each. A linear vein-based morphological pattern is common in pediatric AVMs and may recapitulate the embryological origins of the cerebral circulation. Surgical treatment in these cases can also be vein-based, through a narrow surgical corridor, which leads to minimal cortical disruption.

Recurrent Cerebral Arteriovenous Malformation in a Child : Case Report and Review of the Literature

Arteriovenous malformations (AVM) are generally considered to be cured following angiographically proven complete resection. However, rare instances of AVM recurrence have been reported in both children and adults with negative findings on postoperative angiography. The authors present the case of a 12-year-old boy with recurrent AVM. The AVM was originally fed by the pericallosal arteries on both sides, and it showed changing patterns of supply at recurrence. The authors concluded that a negative postoperative angiogram is not necessarily indicative of a cure. Repeat angiography and regular follow-up examinations should be performed to exclude the possibility of recurrence, especially in children.

Novel Technique for Evaluating Complex Pelvic Arteriovenous Malformations with Catheter-directed Subtracted MR Angiography

Residual or recurrent arteriovenous malformation (AVM) nidal filling is not uncommon after embolization of complex peripheral AVMs. The major reason for this is incomplete embolization of the nidus owing to failure to detect all feeder vessels other than the dominant one. Herein, the authors describe a novel "intravenous minus intraarterial" subtraction magnetic resonance (MR) angiography technique that enabled the delineation of additional feeding branches not clearly identifiable at intravenous contrast-enhanced MR angiography or nonselective pelvic angiography. This technique may be particularly useful in the evaluation of complex AVMs and their subsequent management with embolization.

A case of hereditary hemorrhagic telangiectasia

Hereditary hemorrhagic telagiectasia (HHT), which is characterized by the classic triad of mucocutaneous telangiectases, arteriovenous malformations (AVMs) and inheritance, is an autosomal dominant disorder. The characteristic manifestations of HHT are all due to abnormalities of the vascular structure. This report deals with the case of a 14-year-old girl with typical features of HHT that include recurrent epistaxis, mucocutanous telangiectases, pulmonary and cerebral AVMs and a familial occurrence.

P.330 Total glossectomy reconstruction with perforator flaps from the lateral thigh

To evaluate the efficacy and safety of renal artery embolisation (RAE) for renal arteriovenous malformation (AVM) as well as its outcomes.The technical and clinical success rates, radiological and laboratory findings, and complications of RAE for 31 renal AVMs in 24 patients (M:F=9:15, mean age 46 years) at two separate medical institutions were retrospectively evaluated. Technical success was defined as complete occlusion of feeding arteries with no residual nidus seen on post-treatment angiography. Clinical failure was defined as recurrence of haematuria, presence of AVM on follow-up ultrasound or computed tomography, repeated RAE or surgery for the control of haematuria. Overall clinical success was defined as resolution haematuria or disappearance of AVM on follow-up imaging after single or multiple sessions of RAE.Types of renal AVM were AVM, arterio-venous fistula (AVF) with intranidal aneurysm, and acquired AVF in 19, 1, and 4 patients, respectively. 18 patients (75%) underwent a single session of RAE, while 6 patients (25%) had two or more sessions of RAE. The level of embolisation was feeder, segmental artery, and main renal artery in 28 (90%), 2 (6%), and 1 (4%) procedures, respectively. Coil, n-butyl 2-cyanoacrylate, and polyvinyl alcohol were the most frequently chosen embolic materials and were used in 19, 14, and 8 procedures, respectively. The clinical success rate after initial RAE was 67% (16/24). Overall clinical success rate, including multisession RAE, was 88% (21/24). The technical success rate of 31 procedures was 65% (20/31). Among 11 technical failures in 10 patients, 4 achieved clinical success without additional RAE, 3 underwent second session RAE to achieve clinical success, and 3 patients underwent nephrectomy due to recurrence.RAE is a safe and effective treatment for renal AVM. Technical failure of RAE does not always lead to clinical failure and multiple embolisation sessions may be effective for recurrent renal AVM.