scholarly journals Therapeutic progress in pediatric intracranial dural arteriovenous shunts: A review

2016 ◽  
Vol 22 (5) ◽  
pp. 548-556 ◽  
Author(s):  
Jinlu Yu ◽  
Xianli Lv ◽  
Youxiang Li ◽  
Zhongxue Wu

Pediatric dural arteriovenous shunts (dAVSs) are a rare form of vascular disease: Fewer than 100 cases are reported in PubMed and the understanding of pediatric dAVS is limited. For this study, we searched in PubMed, reviewed and summarized the literature related to pediatric dAVSs. Our review revealed that pediatric dAVSs have an unfavorable natural history: If left untreated, the majority of pediatric dAVSs deteriorate. In a widely accepted classification scheme developed by Lasjaunias et al., pediatric dAVSs are divided into three types: Dural sinus malformation (DMS) with dAVS, infantile dAVS (IDAVS) and adult-type dAVS (ADAVS). In general, the clinical manifestations of dAVS can be summarized as having symptoms due to high-flow arteriovenous shunts, symptoms from retrograde venous drainage, symptoms from cavernous sinus involvement and hydrocephalus, among other signs and symptoms. The pediatric dAVSs may be identified with several imaging techniques; however, the gold standard is digital subtraction angiography (DSA), which indicates unique anatomical details and hemodynamic features. Effectively treating pediatric dAVS is difficult and the prognosis is often unsatisfactory. Transarterial embolization with liquid embolic agents and coils is the treatment of choice for the safe stabilization and/or improvement of the symptoms of pediatric dAVS. In some cases, transumbilical arterial and transvenous approaches have been effective, and surgical resection is also an effective alternative in some cases. Nevertheless, pediatric dAVS can have an unsatisfactory prognosis, even when timely and appropriate treatment is administered; however, with the development of embolization materials and techniques, the potential for improved treatments and prognoses is increasing.

2010 ◽  
Vol 112 (3) ◽  
pp. 603-610 ◽  
Author(s):  
Pierre Guedin ◽  
Stephan Gaillard ◽  
Anne Boulin ◽  
Stephanie Condette–Auliac ◽  
Frederic Bourdain ◽  
...  

Object 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. Methods 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. Results 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. Conclusions 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.


2007 ◽  
Vol 13 (1) ◽  
pp. 67-74 ◽  
Author(s):  
S. Geibprasert ◽  
T. Krings ◽  
V. Pereira ◽  
P. Lasjaunias

A three-year-old girl with an incidentally discovered infantile type of dural arteriovenous shunts (DAVs) along the superior sagittal sinus during investigation of a minor head trauma is presented. The DAVs drained into a developmental venous anomaly of the right cerebral hemisphere. In addition, there was a small cavernoma within the territory drained by the DVA. The patient underwent multiple transarterial embolizations to decrease the shunt flow and thus the constrained venous drainage of the DVA and right cerebral hemisphere. Pediatric dural arteriovenous shunts are a different entity from the adult type DAVs and should be managed according to the growth and development of the child. DVAs are extreme variations of the venous system with reduced flexibility to increased venous drainage. Regardless of the type of treatment employed, the DVA and its drainage pathway must be preserved.


1996 ◽  
Vol 2 (3) ◽  
pp. 179-191 ◽  
Author(s):  
P. Lasjaunias ◽  
G. Magufis ◽  
A. Goulao ◽  
R. Piske ◽  
S. Suthipongchai ◽  
...  

We review 29 children (presenting between 1985–1996) with dural arteriovenous shunts. By analysing the anatomical features from axial and angiographic imaging and examining the clinical history and pathophysiological characteristics, we hypothesize that different diseases can be distinguished and divided into three groups: dural sinus malformation (DSM), infantile type of dural arteriovenous shunts (IDAVS) and adult type of dural arteriovenous shunts (ADAVS). It was helpful to classify these diseases when assessing the treatment options and long-term prognosis. Our group of 29 children comprised 19 DSM, 7 IDAVS, 3 ADAVS. A slight male preponderance was noted in the DSM group. The range of symptoms encountered included mild cardiac failure and coagulopathies, macro-crania, developmental delay, mental retardation, seizures and focal neurological deficits (in the neonates and early infancy age group) with or without haemorrhagic venous infarctions secondary to venous outlet restriction. We found all types of lesion in the neonatal age group, but in general the different types of lesion correspond to the paediatric subgroups with DSM occurring in the neonatal age group, IDAVS in infancy and ADAVS in children. DSMs are revealed in the first few months of live and the prognosis is good if the torcular is not involved. Two types can be seen: 1) DSM involving the posterior sinus with or without the confluens sinusum, with giant dural lakes and slow flow mural AV shunting. Spontaneous thrombosis may further restrict cerebral venous drainage and subsequently lead to intraparenchymatous haemorrhagic infarction. 2) DSM involving the jugular bulb with otherwise normal sinuses but associated with a high flow sigmoïd sinus AVF. The prognosis is excellent with embolisation treament. IDAVS are high flow and low pressure. The sinuses are large and patent with no lakes. Clinical onset is seen in the first few years of life and the shunts are initially well tolerated. Progressive symptoms (symptoms of raised ICP and venous ischaemia) develop at a later age and initially respond to partial embolisation. The long term prognosis is poor with neurological deterioration in early adulthood. ADAVS present in all age groups and almost all of them are located in the cavernous venous plexus. Post embolisation outcome is excellent.


2006 ◽  
Vol 104 (6) ◽  
pp. 867-875 ◽  
Author(s):  
Michael Söderman ◽  
Göran Edner ◽  
Kaj Ericson ◽  
Bengt Karlsson ◽  
Tiit Rähn ◽  
...  

Object The aim of this study was to assess the clinical efficacy of gamma knife surgery (GKS) in the treatment of dural arteriovenous shunts (DAVSs). Methods From a database of more than 1600 patients with intracranial arteriovenous shunts that had been treated with GKS, the authors retrospectively and prospectively identified 53 patients with 58 DAVSs from the period between 1978 and 2003. Four patients were lost to follow-up evaluation and were excluded from the series. Thus, this study is based on the remaining 49 patients with 52 DAVSs. Thirty-six of the shunts drained into the cortical venous system, either directly or indirectly, and 22 of these were associated with intracranial hemorrhage on patient presentation. The mean prescription radiation dose was 22 Gy (range 10–28 Gy). All patients underwent a clinical follow-up examination. In 41 cases of DAVS a follow-up angiography study was performed. At the 2-year follow-up visit, 28 cases (68%) had angiographically proven obliteration of the shunt and in another 10 cases (24%) there was significant flow regression. Three shunts remained unchanged. There was one immediate minor complication related to the administration of radiation. Furthermore, one patient had a radiation-induced complication 10 years after treatment, although she recovered completely. There was one posterior fossa bleed 2 months after radiosurgery; a hematoma, as well as a lesion, was evacuated, and the patient recovered uneventfully. A second patient had an asymptomatic occipital hemorrhage approximately 6 months postradiosurgery. The clinical outcome after GKS was significantly better than that in patients with naturally progressing shunts (p < 0.01, chi-square test); figures on the latter have been reported previously. Conclusions Gamma knife surgery is an effective treatment for DAVSs, with a low risk of complications. Major disadvantages of this therapy include the time elapsed before obliteration and the possibility that not all shunts will be obliterated. Cortical venous drainage from a DAVS, a risk factor for intracranial hemorrhage, is therefore a relative contraindication. Consequently, GKS can be used in the treatment of both benign DAVSs with subjectively intolerable bruit and aggressive DAVSs not responsive to endovascular treatment or surgery.


2001 ◽  
Vol 7 (1) ◽  
pp. 51-60 ◽  
Author(s):  
P. Vilela ◽  
R. Willinsky ◽  
K. terBrugge

The infantile dural arteriovenous shunts are multifocal involving different dural sinuses and progress to an occlusive venopathy with sigmoid sinus and/or jugular bulb stenosis and subsequent occlusion. We report a successful angioplasty and stent placement of a sigmoid sinus — jugular bulb stenosis due to venous occlusive disease in a patient with infantile dural arteriovenous shunts. A five-year-old patient presented with status epilepticus due to severe venous congestive encephalopathy. The angiogram revealed multifocal dural arteriovenous shunts, occlusion of the right sigmoid sinus, absence of cavernous sinuses and significant stenosis of the left sigmoid sinus — jugular bulb. By transvenous approach, percutaneous transluminal balloon angioplasty and stent placement of the stenosed left sigmoid sinus — jugular bulb segment was performed. This resulted in a significant decrease of the venous pressure gradient across the stenosis and allowed a dramatic clinical recovery. Dural sinus angioplasty and stent placement appears to be a safe and effective procedure and should be considered in the treatment of the venous occlusive disease associated with infantile dural arteriovenous shunts.


2014 ◽  
Vol 38 (2) ◽  
pp. 283-291 ◽  
Author(s):  
Gerasimos Baltsavias ◽  
Alex Spiessberger ◽  
Torsten Hothorn ◽  
Anton Valavanis

1998 ◽  
Vol 89 (5) ◽  
pp. 755-761 ◽  
Author(s):  
Hiroyuki Kinouchi ◽  
Kazuo Mizoi ◽  
Akira Takahashi ◽  
Yoshihide Nagamine ◽  
Keiji Koshu ◽  
...  

Object. A retrospective analysis was conducted of 10 patients (three women and seven men) who were treated for spinal dural arteriovenous shunts (AVSs) located at the craniocervical junction. This analysis was performed to evaluate the characteristics of this unusual location in contrast with those of the more common thoracic and lumbar AVSs. Methods. Seven patients presented with subarachnoid hemorrhage (SAH) and one with slowly progressive quadriparesis and dyspnea due to myelopathy. The other two cases were detected incidentally and included a transverse—sigmoid dural AVS and a cerebellar arteriovenous malformation. Angiographic studies revealed that the spinal dural AVSs at the C-1 and/or C-2 levels were fed by the dural branches of the radicular arteries that coursed from the vertebral artery and drained into the medullary veins. Venous drainage was caudally directed in the patient with myelopathy. In contrast, the shunt flow drained mainly into the intracranial venous system in patients with SAH. Furthermore, in four of these patients a varix was found on the draining vein. In all patients, the draining vein was interrupted surgically at the point at which this vessel entered the intradural space, using intraoperative digital subtraction angiography to monitor flow. The postoperative course was uneventful in all patients and no recurrence was confirmed on follow-up angiographic studies obtained in seven patients at 6 months after discharge. Conclusions. If computerized tomography scanning shows SAH predominantly in the posterior fossa and no abnormalities are found on intracranial four-vessel angiographic study, proximal vertebral angiography should be performed to detect dural AVS at the craniocervical junction. The results of surgical intervention for this disease are quite satisfactory.


2020 ◽  
pp. neurintsurg-2020-016888
Author(s):  
Stanislas Smajda ◽  
Michael Söderman ◽  
Georg Dorfmüller ◽  
Nathalie Dorison ◽  
Marie-Claire Nghe ◽  
...  

BackgroundTorcular dural sinus malformations (tDSMs) with arteriovenous shunts are rare congenital intracranial vascular malformations that carry a high rate of neurologic impairment and death in the neonatal, infant and young pediatric population. Their impact on brain venous drainage, especially the deep venous system, is one of the key factors in the clinical prognosis and natural history of the disease. We describe our therapeutic strategy for tDSMs, disconnecting the reflux into the deep venous system by performing an endovascular straight sinus occlusion.MethodsAmong all children with dural sinus malformations seen between 2002 and 2020, we retrospectively reviewed those with tDSM in whom straight sinus occlusion had been performed.ResultsOur databank included nine patients with tDSM that were embolized. Mean age at the clinical onset was 8.9±9.6 months (min–max=0–31). Five patients presented a significant reflux in the straight sinus on digital subtraction angiography. Those patients were initially clinically worse (mean modified Rankin Scale (mRS) 3.8) than those who did not present with reflux (mean mRS 2.25), this reflux being responsible for intraventricular hemorrhage in three patients. The reflux was suppressed by transarterial embolization in one patient and by transvenous straight sinus occlusion in four patients. Staged endovascular treatment resulted in a complete cure in six patients without complications, and clinical improvement in all patients.ConclusionStraight sinus occlusion is a feasible technique that needs to be considered in the treatment strategy for tDSM with deep venous reflux in order to avoid or minimize brain damage.


2017 ◽  
Vol 74 (2) ◽  
pp. 184-188 ◽  
Author(s):  
Dejan Marinkovic ◽  
Tamara Dragovic ◽  
Sasa Kikovic ◽  
Snezana Kuzmic-Jankovic ◽  
Zorana Djuran ◽  
...  

Introduction. Fahr?s syndrome is a rare, slowly progressive, neurodegenerative disorder, characterised by extensive, bilateral, and symmetrical basal ganglia calcification. It is associated with neuropsychiatric manifestations and gradually progressive cognitive impairment. Fahr's syndrome is the secondary form of brain calcification that is caused by various metabolic, infectious, or degenerative diseases. Case report. We presented a middle-aged male with Fahr's syndrome due to primary idiopathic hypoparathyroidism. Clinical diagnosis was based on signs and symptoms of hypocalcemia, progressive neuropsychiatric illnesses, laboratory evidence of hypoparathyroidism, and radiological signs of calcifications in the basal ganglia. The patient improved after only a few days of intravenous rehydration and calcium substitution, followed by oral supplemental calcitriol. Conclusion. Timely recognition of idiopathic and iatrogenic hypoparathyroidism allows appropriate treatment that can prevent the development and clinical manifestations of Fahr?s syndrome and potentially slow its progression.


1996 ◽  
Vol 2 (3) ◽  
pp. 223-228 ◽  
Author(s):  
M. Ezura ◽  
A. Takahashi ◽  
K. Mizoi

A 40-year-old male suffered transient global amnesia and was diagnosed as having a very rare location of dural arteriovenous shunt (dAVS) involving the right sphenoparietal sinus. The feeding pedicles of dAVS were not only arising from the right external carotid system, including the middle meningeal artery, but also the right internal carotid system including the ophthalmic artery. The dAVS drained into the right sphenoparietal sinus, right vein of Labbe, and right basal vein of Rosenthal through numerous dilated temporal cortical veins. The arteriovenous shunt itself was located at the dura around the right superior ophthalmic fissure and a large varix received direct shunting blood flow from the right sphenoparietal sinus. The dAVS was cured by drainer clipping following transarterial embolisations. A dAVS with cortical venous drainage is considered to have a higher risk of haemorrhage and venous infarction than the others and thus warrants treatment even if the clinical symptom is minimal. If the dAVS has a single drainer, it will be easily cured by way of drainer clipping. Preoperative embolisation makes clipping easier and safer. Intraoperative angiography is useful because preoperative angiography may not necessarily show the complete angioarchitecture around the lesion.


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