✓ The authors report the case of a 21-year-old woman who presented with headaches, frequent sensations of loss of equilibrium, and intermittent strabismus. A tectal arteriovenous malformation (AVM) was diagnosed based on magnetic resonance (MR) imaging findings. The AVM drained toward the straight sinus and was associated with a tonsillar prolapse (Chiari malformation Type I [CM-I]) and cervical syringomyelia. The tectal AVM was embolized with N-butyl cyanoacrylate, and disconnection of about 80% of the lesion was obtained. All clinical symptoms resolved after embolization, and radiosurgery was proposed to treat the malformation remnant. A control MR image confirmed the regression of the tonsillar prolapse and the disappearance of the syrinx. This report emphasizes that CM-I and syringomyelia may be acquired and related to hydrovenous disorders.
Reversible tonsillar prolapse and syringomyelia after embolization of a tectal arteriovenous malformation
Case report and review of the literature
Georges Rodesch, Bernard Otto, Micheline Mouchamps, and Jacques Born
Raphaël Vialle, Fabrice Parker, Jean-François Lepeintre, Georges Rodesch, Jean-Louis Tassin, and Marc Tadié
Pierre Lasjaunias, Georges Rodesch, Phillippe Pruvost, Françoise Grillot Laroche, and Pierre Landrieu
✓ The authors report the case of a vein of Galen aneurysmal malformation in a 1-year-old baby presenting with an enlarging head. The lesion was a direct arteriovenous fistula in a dilated vein of Galen and was treated by complete embolization in one session. Four months after occlusion of the shunt, the ectatic vein of Galen and torcular were normal, and the head circumference had stabilized. This case serves as an opportunity to emphasize the quality of results that can be obtained with endovascular techniques. Proper analysis of the vein of Galen angioarchitecture allows planning for appropriate treatment with the lowest possible risk of morbidity and mortality.
Ronit Agid, Karel TerBrugge, Georges Rodesch, Tommy Andersson, and Michael Söderman
Dural arteriovenous fistulas (DAVFs) of the anterior cranial fossa are rare lesions that can cause intracranial hemorrhage. Authors of previous reports mostly have described open surgical treatment for this fistula type. The authors' purpose in the present study was to describe their experience with anterior cranial fossa DAVFs, including their endovascular treatment.
All patients with anterior cranial fossa DAVFs diagnosed and treated in 3 separate institutions during the last 23 years were retrospectively identified. Clinical charts, imaging studies, and procedural notes were evaluated.
Twenty-four patients (22 males and 2 females), ranging in age from 3 to 77 years, harbored 24 DAVFs in the anterior cranial fossa. Eleven patients were primarily treated with surgical disconnection and 2 with radiosurgery. Eleven patients were treated endovascularly; 7 of these patients (63.6%) were cured. In 4 cases of failed embolization, final disconnection was achieved through surgery. In fact, surgery was effective in disconnecting the fistula in 100% of cases. All endovascular procedures consisted of transarterial injections of diluted glue (N-butyl cyanoacrylate [NBCA]), and there were no complications. Brain edema developed around the venous pouch and confusion was apparent after venous disconnection in 1 surgically treated patient. No patient suffered a hemorrhage during the follow-up period.
Disconnection of an anterior cranial fossa DAVF by using transarterial catheterization through the ophthalmic artery and subsequent injection of NBCA is possible with a reasonable success rate and low risk for complications. In patients with good vascular access this procedure could be the treatment of choice, to be followed by open surgery in cases of embolization failure.
João Ferreira de Melo Neto, Eduardo E. Pelinca da Costa, Nilson Pinheiro Junior, André L. Batista, Georges Rodesch, Serge Bracard, and Antônio G. Oliveira
Dural arteriovenous fistulas (DAVFs) are abnormal, acquired arteriovenous connections within the dural leaflets. Their associated symptoms may be mild or severe and are related to the patient’s venous anatomy. With the hypothesis that the patient’s venous anatomy determines the development of symptoms, the authors aimed to identify which venous anatomy elements are important in the development of major symptoms in patients with a DAVF.
A multicenter study was performed based on the retrospective analysis of cerebral angiographies with systematic assessment of brain drainage pathways (including fistula drainage) in patients over 18 years of age with a single DAVF. The patients were divided into two groups: those with minor (group 1, n = 112) and those with major (group 2, n = 89) symptoms. Group 2 was subdivided into two groups: patients with hemorrhage (group 2a, n = 47) and patients with severe nonhemorrhagic symptoms (group 2b, n = 42).
The prevalence of stenosis in DAVF venous drainage and the identification of tiny anastomoses between venous territories were significantly higher in group 2 (32.6% and 19.1%, respectively) compared with group 1 (2.68% and 5.36%, respectively). Stenosis of DAVF venous drainage was significantly more frequent in group 2a than in group 2b (51.1% vs 11.9%, p < 0.001). Group 2b patients had increased prevalence of shared use of the cerebral main drainage pathway (85.0% vs 53.2%, p = 0.002), the absence of an alternative route (45.0% vs 17.0%, p = 0.004), and the presence of contrast stagnation (62.5% vs 29.8%, p = 0.002) compared with group 2a patients. In patients with high-grade fistulas, the group with major symptoms had increased prevalence of a single draining direction (31.3% vs 8.33%, p = 0.003), stenosis in the draining vein (35.0% vs 6.25%, p = 0.000), the absence of an alternative pathway for brain drainage (31.3% vs 12.5%, p = 0.017), and the presence of contrast stagnation (48.8% vs 22.9%, p = 0.004).
Major symptoms were observed when normal brain tissue venous drainage was impaired by competition with DAVF (predominance in group 2b) or when DAVF venous drainage had anatomical characteristics that hindered drainage, with consequent venous hypertension on the venous side of the DAVF (predominance in group 2a). The same findings were observed when comparing two groups of patients with high-grade lesions: those with major versus those with minor symptoms.
Katsuhiro Mizutani, Arturo Consoli, Federico Di Maria, Stéphanie Condette Auliac, Anne Boulin, Oguzhan Coskun, Julie Gratieux, and Georges Rodesch
Few classifications of intradural spinal arteriovenous shunts (ID-SAVSs) have considered their anatomical localization in relation to their phenotype and angioarchitectonics. The authors propose another vision of ID-SAVSs allowing a reappraised classification based on analysis of the anatomical disposition, angioarchitecture, and histogenetic location of these vascular malformations.
The radiological and clinical records of 210 patients with ID-SAVSs were retrospectively reviewed, considering their localization, vascular architectonics, and correlation with the 5 histogenetic units of the spinal cord. Among these, 183 files with complete data allowed precise analysis of the ID-SAVSs.
Among these 183 files (162 and 21 cases with single and multiple lesions, respectively), different entities were identified: 13 pial macro arteriovenous fistulas (MAVFs), 92 pial micro arteriovenous fistulas (mAVFs), 33 superficial pial niduses, and 69 intramedullary niduses. Thirteen sulcal shunts (either fistulas or niduses) were considered subtypes of pial lesions. Among the 21 multiple cases, 11 were monomyelomeric while 10 were multimyelomeric. Pial lesions, either fistulas or niduses, were dominantly vascularized by pial arteries (anterior or posterior depending on the localization of the shunt) and occasionally (except for MAVFs) by transmedullary arteries. Pial niduses occasionally extended into the funiculus by recruiting intrinsic veins or by extension of the nidus itself inside the white matter. Intramedullary niduses were always vascularized by both centrifugal and centripetal feeders, respectively, from sulcal arteries (SAs) and pial arteries. Sulcal lesions are pial lesions located within the ventral median sulcus and vascularized by SAs and veins. Single or multiple ID-SAVSs can be part of various syndromes such as hereditary hemorrhagic telangiectasia, Parkes-Weber, RASA1, CLOVES, and spinal arteriovenous metameric syndromes. Histogenetic analyses revealed a specific distribution of each ID-SAVS in the 5 histogenetic units of the spinal cord: intramedullary niduses were found almost equally from cervical to thoracic units, while MAVFs and mAVFs were mostly found from thoracic to postcrural ones. Pial niduses showed intermediate features between intramedullary and fistulous lesions and were mostly distributed from brachial to crural segments.
Precise analysis of the anatomical disposition of ID-SAVSs in relation to functional histogenetic units allows a better understanding of these lesions and improved therapeutic management.
Marlise P. dos Santos, Jingwen Zhang, Diana Ghinda, Rafael Glikstein, Ronit Agid, Georges Rodesch, Donatella Tampieri, and Karel G. terBrugge
Intraspinal tumors comprise a large spectrum of neoplasms, including hemangioblastomas, paragangliomas, and meningiomas. These tumors have several common characteristic imaging features, such as highly vascular mass appearance in angiography, hypointense rim and serpentine flow voids in MRI, and intense enhancement after intravenous contrast administration. Due to their rich vascularity, these tumors represent a special challenge for surgical treatment. More recently, the surgical treatment of intraspinal vascular tumors has benefited from the combination of endovascular techniques used to better delineate these lesions and to promote preoperative reduction of volume and tissue blood flow. Endovascular embolization has been proven to be a safe procedure that facilitates the resection of these tumors; hence, it has been proposed as part of the standard of care in their management.
Pierre Guedin, Stephan Gaillard, Anne Boulin, Stephanie Condette–Auliac, Frederic Bourdain, Stephanie Guieu, Michel Dupuy, and Georges Rodesch
There is a strong correlation between the venous drainage pattern of intracranial dural arteriovenous shunts (ICDAVSs) and the affected patients' clinical presentation. The ICDAVSs that have cortical venous reflux (CVR) (retrograde leptomeningeal drainage: Borden Type 2 and 3 lesions) are very aggressive and have a poor natural history. Although the necessity of treatment remains debatable in ICDAVSs that drain exclusively into a sinus (Borden Type 1), lesions with CVR must be treated because of the negative effects of the retrograde venous drainage. Surgery, radiosurgery, and embolization have been proposed for management of these lesions, but endovascular therapy is considered the most appropriate therapeutic strategy in ICDAVSs. New embolic materials, such as Onyx, have been recently developed and are considered to represent a kind of “gold standard” for embolization of these lesions. The purpose of this study is to emphasize the importance of transarterial embolization using acrylic glue in the therapeutic management of ICDAVSs with CVR, and to compare the results the authors obtained using this treatment with those reported in the literature for Onyx treatment of the same type of dural shunts.
The clinical and radiological records of 53 consecutive patients suffering from ICDAVSs with CVR (Borden Types 2 or 3) were reviewed. All cases were managed with the same angiographic and therapeutic protocol. Localization of the lesions, their clinical symptoms, their angioarchitecture, their therapeutic management, and the results were analyzed.
Fourteen ICDAVSs were located at the superior sagittal sinus and/or convexity veins, 13 at the transverse and sigmoid sinuses, 10 at the tentorium, 5 in the anterior cranial fossa, 4 at the foramen magnum, 3 at the torcula, 2 at the straight sinus, and 1 at the vein of Galen. One patient presented with an infantile form of ICDAVS with multiple shunts. Hemorrhage had occurred in 36% of cases. Forty-three patients underwent transarterial embolization (42 with acrylic glue). Complete closure of the fistula was obtained in 34 patients. Suppression of the CVR with partial occlusion of the main shunt was achieved in all other cases. No mortality or permanent morbidity was observed in this series.
Intracranial dural arteriovenous shunts can be safely managed by transarterial embolization, which can be considered in most instances as an effective first-intention treatment. Acrylic glue still allows a cheap, fast, and effective treatment with high rates of cures that compare favorably to those obtained with new embolic materials.
Daniel Duran, Philipp Karschnia, Jonathan R. Gaillard, Jason K. Karimy, Mark W. Youngblood, Michael L. DiLuna, Charles C. Matouk, Beverly Aagaard-Kienitz, Edward R. Smith, Darren B. Orbach, Georges Rodesch, Alejandro Berenstein, Murat Gunel, and Kristopher T. Kahle
Vein of Galen malformations (VOGMs) are rare developmental cerebrovascular lesions characterized by fistulas between the choroidal circulation and the median prosencephalic vein. Although the treatment of VOGMs has greatly benefited from advances in endovascular therapy, including technical innovation in interventional neuroradiology, many patients are recalcitrant to procedural intervention or lack accessibility to specialized care centers, highlighting the need for improved screening, diagnostics, and therapeutics. A fundamental obstacle to identifying novel targets is the limited understanding of VOGM molecular pathophysiology, including its human genetics, and the lack of an adequate VOGM animal model. Herein, the known human mutations associated with VOGMs are reviewed to provide a framework for future gene discovery. Gene mutations have been identified in 2 Mendelian syndromes of which VOGM is an infrequent but associated phenotype: capillary malformation–arteriovenous malformation syndrome (RASA1) and hereditary hemorrhagic telangiectasia (ENG and ACVRL1). However, these mutations probably represent only a small fraction of all VOGM cases. Traditional genetic approaches have been limited in their ability to identify additional causative genes for VOGM because kindreds are rare, limited in patient number, and/or seem to have sporadic inheritance patterns, attributable in part to incomplete penetrance and phenotypic variability. The authors hypothesize that the apparent sporadic occurrence of VOGM may frequently be attributable to de novo mutation or incomplete penetrance of rare transmitted variants. Collaboration among treating physicians, patients’ families, and investigators using next-generation sequencing could lead to the discovery of novel genes for VOGM. This could improve the understanding of normal vascular biology, elucidate the pathogenesis of VOGM and possibly other more common arteriovenous malformation subtypes, and pave the way for advances in the diagnosis and treatment of patients with VOGM.