scholarly journals Dural Sinus Malformations (DSM) with Giant Lakes, in Neonates and Infants

2003 ◽  
Vol 9 (4) ◽  
pp. 407-424 ◽  
Author(s):  
M. Barbosa ◽  
J. Mahadevan ◽  
Y.C. Weon ◽  
Y. Yoshida ◽  
A. Ozanne ◽  
...  

Background and Purpose Dural Arteriovenous Shunt (DAVS) in children include Dural sinus malformation (DSM), infantile and adult types. They are rare and seldom reported. Our purpose was to highlight the angiographic features of the DSM sub group for prognosis of clinical evolution and outcome and to lay guidelines for management. Methods: From a dedicated neurovascular data bank, there were 52 cases of arteriovenous dural shunts in children from 1985 to 2003. Of these, there were 30 patients with DSM, which we analysed the various angioarchitecture, presentation and neurological outcome. Children clinical status was evaluated and scored at admission and follow up. Results There was an overall male dominance of 2:1. Antenatal diagnosis was obtained in 8/30 (26.7%) cases. Mean age of diagnosis was 5 months. Mean age at first consultation was 8.7 months. No patient was diagnosed during childhood. The most common clinical presentations were macrocrania 76.7%, seizures 23.3% and mental retardation 23.3%. In 14/30 (35.7%) of the patients, the therapeutic decision was to manage conservatively; in 5/14 (30.7%) with predictable favourable evolution and in 9/14 (64.3%) with irreversible poor neurological outcome. In the remaining 16/30 (53.3%) patients, endovascular treatment was performed. In 12/16 (75.0%) patients the neurological outcome was good, 3/16 (18.8%) patients had unfavourable evolution despite embolization. There was no morbidity mortality related to the procedures themselves. 1/16 (6.3%) patient was lost to follow-up. Overall 12/29 (45.8%) patients had an unfavourable neurological outcome with 11 patients dead and 1 with severe neurological deficit. In the surviving group of children, 17/18 (94.4%) have a good neurological outcome; in 10/18 (55.5%) the lesion is morphologically excluded. Conclusion DSM is rare disease with high mortality. They usually proceed to either total or partial spontaneous thrombosis before the age of 2 thus compromising normal cerebral venous drainage. DSM away from the torcular, good cavernous sinus, cavernous capture of sylvian veins, absence of pial veins, straight sinus or superior sagital sinus (SSS) reflux and absence of jugular bulb dys-maturation represent factors of good prognosis. Such patients will highly benefit for endovascular treatment. In partial endovascular approach the aim being is to separate the brain drainage from DSM drainage. This will be achieved by the transarterial approach to the associated mural arteriovenous shunts (AVS) and by disconnecting the pial reflux by transvenous route.

2022 ◽  
Author(s):  
Zeferino Demartini Jr. ◽  
Adriano Keijiro Maeda ◽  
Gelson Luis Koppe ◽  
Ricardo Munhoz da Rocha Guimarães ◽  
Luana A.Maranha Gatto ◽  
...  

The dural sinus malformation (DSM) are rare congenital vascular anomalies with variable anatomic features, clinical conditions, and outcomes. There are two forms of disease: a lateral subtype, affecting the jugular bulb with associated high flow arteriovenous fistula; and a midline subtype, called torcular DSM. The torcular subtype is more common and characterized by a giant dural sinus lake involving the confluens sinuum (torcular Herophili). We present a case of a 28-year-old woman with an ultrasound at 32 weeks’ gestation showing a fetal intracranial thrombosed lesion, measuring 6x4 cm. An elective c-section was performed at 38 weeks’ gestation, and a male neonate was born. He remained asymptomatic, and a brain MRI performed 9 days later showed a thrombosed midline mass due to a torcular DSM. The outcome with conservative treatment was favorable, and further development was unremarkable. The 2-year follow-up imaging demonstrated spontaneous involution of the clot. The torcular DSM is frequently diagnosed prenatally as an intraluminar lake thrombosis, and differential diagnosis include tumors, subdural collections, vein of Galen aneurysmal malformations, pial malformations, arachnoid and dermoid cysts. A literature review involving 126 patients with torcular DSM found an overall mortality of 22.1%. Torcular DSM seems to have better prognosis than those having dural arteriovenous shunts, and the worst outcomes were associated to brain damage and patent feeders. In addition, antenatal diagnosis and thrombosis of pouch have good prognosis, possibly because the clot may spontaneously obliterate the fistulas. Treatment options must be individualized, and include conservative, embolization, ventriculoperitoneal shunt and endoscopic third ventriculostomy. Although systemic anticoagulation is controverse in infants, it should be considered for patients with sinovenous thrombosis affecting outflow pathways, especially those aggravated by venous congestion. Intervention is recommended for grade III patients because they progress to grade IV, and endovascular treatment is the gold standard therapy due to the risk of fatal intraoperative exsanguination. Whenever possible, embolization should be prioritized over hydrocephalus treatment because it may avoid ventriculoperitoneal shunt. Close follow-up imaging is recommended to detect any changes, with lesion reduction indicating favorable outcome.


2014 ◽  
Vol 37 (v1supplement) ◽  
pp. 1 ◽  
Author(s):  
Michael R. Levitt ◽  
Joshua W. Osbun ◽  
John D. Nerva ◽  
Louis J. Kim

A 71-year-old woman presented with headache and dilated vessels on CTA. Angiography demonstrated a complex dural arteriovenous fistula with retrograde cortical venous hypertension, supplied by branches of internal and external carotids bilaterally into a fistulous pouch paralleling the left transverse and sigmoid sinuses, which was occluded at the jugular bulb. The patient refused treatment and was lost to follow-up, returning with sudden confusion and hemianopsia from left temporo-occipital hemorrhage. Transvenous endovascular embolization was performed using the dual-microcatheter technique with a combination of coiling and Onyx copolymer, completely occluding the sinus and fistula while preserving normal venous drainage.The video can be found here: http://youtu.be/u_4Oc7tSmDM.


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.


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.


Neurosurgery ◽  
2005 ◽  
Vol 57 (5) ◽  
pp. 973-983 ◽  
Author(s):  
Georges Rodesch ◽  
Michel Hurth ◽  
Hortensia Alvarez ◽  
Marc Tadie ◽  
Pierre Lasjaunias

Abstract OBJECTIVE: To review our series of intradural spinal cord arteriovenous fistulas (SCAVFs), analyzing symptoms and related angioarchitecture, and to study the morphological and clinical results of embolization. METHODS: Thirty-two SCAVFs (in 22 adults and 10 children) were treated between 1981 and 2000. These lesions were classified as microarteriovenous fistulas (mAVFs) or macroarteriovenous fistulas (MAVFs) according to shunt morphology. Location, architecture, presenting symptoms, and age group were detailed. The selection of patients for endovascular versus surgical treatments was analyzed, as were the anatomic and clinical results obtained by embolization with n-butylcyanoacrylate. Clinical status was evaluated according to the Karnofsky Performance Scale score. RESULTS: Ten SCAVFs were found in the pediatric population (four mAVFs and six MAVFs). All four mAVFs presented with acute symptoms. Three mAVFs (two cervical and one thoracic) presented hematomyelia; in one patient with a thoracic AVF, subarachnoid hemorrhage was suspected. All six MAVFs were located in the thoracolumbar cord (five associated with hereditary hemorrhagic telangiectasias). Four of the six MAVFs presented with hemorrhage. In the adult population, there were 21 mAVFS (95%) and one MAVF (5%). Only two mAVFs were found in the cervical cord, all other shunts affecting the thoracolumbar region. Hemorrhage was present in 6 of the 22 cases seen in adults (27%). The symptoms of SCAVFs did not differ from those found in spinal cord arteriovenous shunts of nidus type. Pial venous reflux and congestion were the most frequently encountered features in both the adult and pediatric groups. Arterial aneurysms (different from false aneurysms) were not found in association with hemorrhagic presentation of SCAVFs. Mean follow-up in our series was 3.3 years. Of the MAVFs, 86% were embolized, with 67% cured. The others had more than 75% occlusion. All patients followed up improved significantly. Of the mAVFs, 48% were treated endovascularly. Successful embolization was performed in 75% of patients. One patient was not embolized because of vasospasm, whereas 67% percent of mAVFs were completely occluded, 22% were more than 90% occluded, and 11% were 75% occluded. Complementary surgery was deemed unnecessary. All patients with mAVFs improved significantly at follow-up. Transient complications occurred in 22% of all patients, with no permanent morbidity or mortality. No patient bled or rebled after embolization. Thirty-six percent of mAVFs were operated on because of anticipated technical difficulties for endovascular approach or distal localization of the shunt. CONCLUSION: Endovascular treatment of SCAVFs stabilizes, normalizes, or improves neurological symptoms in all patients at long-term follow-up, with no bleeds or rebleeds. Embolization of SCAVFs with glue is a safe treatment that compares favorably with other approaches and significantly improves the poor natural history of the disease.


2010 ◽  
Vol 29 (04) ◽  
pp. 143-146
Author(s):  
João Paulo Mattos ◽  
João Paulo C. de Almeida ◽  
Lucas Alverne F. Albuquerque ◽  
Marcelo Otoch ◽  
Ricardo Leite de Aquino ◽  
...  

Abstract Background: Arteriovenous malformations (AVM) are rare disease in pediatric age group and dural sinus malformation (DSM) has even a lower incidence rate. DSMs are associated with a mild male dominance and onset symptoms appear around 5 months of age. The most common clinical presentation is macrocrania, seizures, psychomotor delay, intracranial hemorrhage, congestive heart failure and brain ischemia. Early recognition of these lesions is essential to prevent brain injury for ischemia and intracranial hypertension. Case description: We discuss the case of a 4 month-old boy presenting with macrocrania and signs of intracranial hypertension secondary to a transverse sinus dural arteriovenous malformation. This case was successfully treated by endovascular procedure reaching the goal of the treatment that is to obliterate the arterial portion of the fistula while preserving cerebral venous drainage to reduce the pial reflux in order to prevent venous hypertension and ischemic complications.


2019 ◽  
Vol 11 (10) ◽  
pp. 1040-1044 ◽  
Author(s):  
Stanimir Sirakov ◽  
Alexander Sirakov ◽  
Krasimir Minkin ◽  
Marin Penkov ◽  
Kristian Ninov ◽  
...  

Background and PurposeLiquid embolic agents (LEAs) are the determinant tool for successful embolization of cranial arteriovenous shunts. There are few currently available LEAs. The aim of the study was to summarize our initial experience with a recently introduced non-adhesive ethylene vinyl alcohol (EVOH) copolymer based LEA (Menox 18) in the endovascular treatment of cerebral arteriovenous malformations.MethodsFrom April 2018 to November 2018, 24 patients harboring cerebral arteriovenous malformations underwent endovascular embolization with Menox 18. Clinical features, angiographic results, procedural details, complications, and follow-up details were prospectively collected and retrospectively analyzed.ResultsCurative embolization in one endovascular session was achieved in 14/24 (58.3%) of the treated patients. Partial embolization was achieved in 10 patients (42.6%) in whom staged treatment with radiosurgery or microsurgical resection was planned. No mortality was recorded in our series. Clinical complications after embolization occurred in 1/24 (4.66%) patients. No technical complications were notedConclusionsOur pilot study suggests that the Menox embolization system offers similar technical and clinical results in comparison with the other currently available LEAs. Further studies with larger cohorts and long term follow-up data are needed to fully evaluate its efficacy


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.


2019 ◽  
Vol 26 (3) ◽  
pp. 260-267 ◽  
Author(s):  
Benjamin Mine ◽  
Thomas Bonnet ◽  
Juan Carlos Vazquez-Suarez ◽  
Noémie Ligot ◽  
Boris Lubicz

Introduction Stent-assisted coiling has widened indications and improved stability of endovascular treatment of intracranial aneurysms. However, stent-assisted coiling is usually not used to treat acutely ruptured intracranial aneurysms to avoid antiplatelet therapy. The objective of this study is to evaluate a strategy of staged endovascular treatment of ruptured intracranial aneurysms including coiling at the acute phase with complementary stenting with or without coiling at the subacute phase. Material and methods Between 2012 and 2017, we retrospectively identified, in our prospectively maintained database, all patients treated for a ruptured intracranial aneurysm based on this staged stenting strategy. Clinical charts and imaging follow-up were analyzed to assess the procedural safety and feasibility as well as clinical and anatomical outcome. Results We identified 23 patients with 23 intracranial aneurysms including 15 (65.2%) women with a mean age of 50 years (range 24–69 years). No rebleeding occurred during the mean delay of 24.3 days between initial coiling and stenting. All procedures were successful and additional coiling was performed in 5/23 procedures (21.7%). Clinical status was unchanged in all patients. At follow-up, the modified Rankin scale was graded 0 in 19/23 (82.6%), 1 in 2/23 (8.7%), and 2 in 2/23 (8.7%) patients, respectively. The rate of complete occlusion rose from 30.4% before the stenting procedure to 52.2% immediately after and 72.7% at follow-up. Conclusion This strategy of early staged stenting in selected patients is safe and improves immediate intracranial aneurysm occlusion and long-term stability in this population at high risk of intracranial aneurysm recurrence with coiling alone.


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