Dural sinus malformation in the early childhood: case report

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.

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Focal stenosis of the sigmoid sinus causing intracranial venous hypertension: Case report, endovascular management, and review of the literature

Interventional Neuroradiology ◽

10.1177/1591019915622160 ◽

2016 ◽

Vol 22 (2) ◽

pp. 240-245 ◽

Cited By ~ 2

Author(s):

Amir R Honarmand ◽

Michael C Hurley ◽

Sameer A Ansari ◽

Tord D Alden ◽

Ryan Kuhn ◽

...

Keyword(s):

Intracranial Hypertension ◽

Age Groups ◽

Venous Drainage ◽

Transverse Sinus ◽

Venous Hypertension ◽

Sigmoid Sinus ◽

Arterial System ◽

Arteriovenous Shunts ◽

Endovascular Stent Placement ◽

Focal Stenosis

Regardless of the underlying pathology, elevated intracranial pressure is the endpoint of any impairment in either cerebrospinal fluid (CSF) absorption (including arachnoid villi) or intracranial venous drainage. In all age groups, the predominant final common pathway for CSF drainage is the dural venous sinus system. Intracranial venous hypertension (ICVH) is an important vascular cause of intracranial hypertension (and its subsequent sequelae), which has often been ignored due to excessive attention to the arterial system and, specifically, arteriovenous shunts. Various anatomical and pathological entities have been described to cause ICVH. For the second time, we present a unique case of severe focal stenosis in the distal sigmoid sinus associated with concurrent hypoplasia of the contralateral transverse sinus causing a significant pressure gradient and intracranial hypertension, which was treated with endovascular stent placement and angioplasty.

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Advances in Surgical Approaches to Dural Fistulas

Neurosurgery ◽

10.1227/neu.0000000000000228 ◽

2014 ◽

Vol 74 (suppl_1) ◽

pp. S32-S41 ◽

Cited By ~ 19

Author(s):

Patrick P. Youssef ◽

Albert Jess Schuette ◽

C. Michael Cawley ◽

Daniel L. Barrow

Keyword(s):

Treatment Options ◽

Venous Drainage ◽

Venous Hypertension ◽

Surgical Approaches ◽

Dural Sinus ◽

Intraoperative Angiography ◽

Indocyanine Green Videoangiography ◽

Arteriovenous Fistulas ◽

The Past ◽

Dural Arteriovenous Fistulas

Abstract Dural arteriovenous fistulas are abnormal connections of dural arteries to dural veins or venous sinuses originating from within the dural leaflets. They are usually located near or within the wall of a dural venous sinus that is frequently obstructed or stenosed. The dural fistula sac is contained within the dural leaflets, and drainage can be via a dural sinus or retrograde through cortical veins (leptomeningeal drainage). Dural arteriovenous fistulas can occur at any dural sinus but are found most frequently at the cavernous or transverse sinus. Leptomeningeal venous drainage can lead to venous hypertension and intracranial hemorrhage. The various treatment options include transarterial and transvenous embolization, stereotactic radiosurgery, and open surgery. Although many of the advances in dural arteriovenous fistula treatment have occurred in the endovascular arena, open microsurgical advances in the past decade have primarily been in the tools available to the surgeon. Improvements in microsurgical and skull base approaches have allowed surgeons to approach and obliterate fistulas with little or no retraction of the brain. Image-guided systems have also allowed better localization and more efficient approaches. A better understanding of the need to simply obliterate the venous drainage at the site of the fistula has eliminated the riskier resections of the past. Finally, the use of intraoperative angiography or indocyanine green videoangiography confirms the complete disconnection of fistula while the patient is still on the operating room table, preventing reoperation for residual fistulas.

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Multiple Dural Arteriovenous Fistulas

Interventional Neuroradiology ◽

10.1177/159101990200800210 ◽

2002 ◽

Vol 8 (2) ◽

pp. 183-191 ◽

Cited By ~ 4

Author(s):

A.J.P. Goddard ◽

M.S. Khangure

Keyword(s):

De Novo ◽

Disease Process ◽

Venous Drainage ◽

Transverse Sinus ◽

Dural Sinus ◽

External Carotid ◽

Unique Case ◽

Arteriovenous Fistulas ◽

Type Iv ◽

Dural Arteriovenous Fistulas

Dural arteriovenous fistulas are most probably acquired lesions. However, they have been rarely encountered de novo. We present a unique case of a 71-year-old woman who initially presented with right-sided dural arteriovenous fistula (DAVF), which spontaneously resolved after diagnostic arteriography. She later developed asymptomatic occlusion of the left transverse sinus. Five years after her initial presentation she developed left-sided pulse-synchronous tinnitus. MRA and catheter angiography showed a complex type IV DAVF between the left transverse sinus and multiple dural branches arising from both left and right external carotid arteries. The left transverse sinus was isolated from the torcula herophili, with stenosis of the sigmoid sinus. Extensive cortical venous drainage was demonstrated. Endovascular cure was effected by polyvinyl alcohol particle and absolute alcohol occlusion of the dominant dural supply, and transvenous coil occlusion of the left transverse sinus. The patient's symptoms resolved almost immediately. This unique case demonstrates that dural sinus occlusion and DAVFs may co-exist, but there may not be a causal relationship. It is likely that both DAVFs and sinus occlusion are manifestations of the same disease process characterised by a pro-thrombotic state and secondary angiogenesis. It is important to recognise that changes in symptomatology, even long after apparent disappearance of a lesion may indicate recurrence, and careful follow up is advocated.

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A variant of arteriovenous fistulas within the wall of dural sinuses. Results of combined surgical and endovascular therapy

Journal of Neurosurgery ◽

10.3171/jns.1991.74.2.0199 ◽

1991 ◽

Vol 74 (2) ◽

pp. 199-204 ◽

Cited By ~ 92

Author(s):

Stanley L. Barnwell ◽

Van V. Halbach ◽

Christopher F. Dowd ◽

Randall T. Higashida ◽

Grant B. Hieshima ◽

...

Keyword(s):

Venous Drainage ◽

Venous Hypertension ◽

Neurological Dysfunction ◽

Dural Sinus ◽

Unique Type ◽

Content Type ◽

Av Fistula ◽

Arteriovenous Fistulas ◽

Av Fistulas ◽

Cortical Venous Drainage

✓ Dural arteriovenous (AV) fistulas are thought to be acquired lesions that form in an area of thrombosis within a sinus. If the sinus remains completely thrombosed, venous drainage from these lesions occurs through cortical veins, or, if the sinus is open, venous drainage is usually into the involved sinus. Among 105 patients with dural A V fistulas evaluated over the the past 5 years, seven had a unique type of dural AV fistula in the superior sagittal, transverse, or straight sinus in which only cortical venous drainage occurred despite a patent involved sinus; the fistula was located within the wall of a patent dural sinus, but outflow was not into the involved sinus. This variant of dural AV fistulas puts the patient at serious risk for hemorrhage or neurological dysfunction caused by venous hypertension. Three patients presented with hemorrhage, one with progressive neurological dysfunction, one with seizures, and two with bruit and headaches. A combination of surgical and endovascular techniques was used to close the fistula while preserving flow through the sinus.

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Postnatal delayed exacerbation of dural sinus malformation associated with brainstem cavernous malformations: A case report

Interventional Neuroradiology ◽

10.1177/1591019917720806 ◽

2017 ◽

Vol 23 (5) ◽

pp. 510-515 ◽

Cited By ~ 2

Author(s):

Katsuhiro Mizutani ◽

Tomoru Miwa ◽

Takenori Akiyama ◽

Tokunori Kanazawa ◽

Hideaki Nagashima ◽

...

Keyword(s):

Case Report ◽

Vascular Malformation ◽

De Novo ◽

Clinical Course ◽

Cavernous Malformation ◽

Venous Hypertension ◽

Dural Sinus ◽

Cavernous Malformations ◽

Dural Sinus Malformation ◽

Brainstem Cavernous Malformation

Dural sinus malformation (DSM) is a rare paediatric vascular malformation characterised by abnormal dilation of the posterior dural sinus. Owing to its rarity, the pathophysiology of DSM has not been fully elucidated. We report a case of prenatally diagnosed DSM with an unusual clinical course. We detected DSM in a male foetus in the 26th week of gestation by using foetal ultrasonography. Although the DSM regressed during the foetal stage and the arteriovenous shunt was insignificant in the neonate, the shunt rapidly developed four months after birth. The neonate also had postnatal de novo brainstem cavernous malformation (CM), which also developed rapidly, supposedly due to the aggravated venous hypertension resulting from the DSM. We successfully treated the aggravated shunts by endovascular transarterial and transvenous embolisation six times over two years and, subsequently, the clinical condition and the size of the brainstem CM became stable. The DSM and CM seemed to have a metameric origin. Such aberrant cases could help to further the understanding of DSM.

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Dural Arteriovenous Malformations and Papilledema

Neurosurgery ◽

10.1227/00006123-198806010-00020 ◽

1988 ◽

Vol 22 (6P1-P2) ◽

pp. 1079-1084 ◽

Cited By ~ 24

Author(s):

Mark J. Gelwan ◽

In Sup Choi ◽

Alejandro Berenstein ◽

John M. D. Pile-Spellman ◽

Mark J. Kupersmith

Keyword(s):

Arteriovenous Malformations ◽

Sinus Thrombosis ◽

Arterial Embolization ◽

Venous Drainage ◽

Transverse Sinus ◽

Venous Hypertension ◽

Venous Sinus Thrombosis ◽

Selective Arterial Embolization ◽

First Case ◽

Cerebrospinal Fluid Absorption

Abstract We examined and managed two patients with posterior fossa dural arteriovenous malformations (DAVMs) and papilledema. Both DAVMs had venous drainage into the transverse, straight, and sigmoid dural venous sinuses. The mechanism of papilledema in the first case was presumed venous hypertension resulting in impaired cerebrospinal fluid absorption, as the malformation drained into the single transverse sinus. This was cured by selective arterial embolization of the causative DAVM. The second patient had venous sinus thrombosis that impaired venous drainage despite embolization. A lumboperitoneal shunt was necessary to treat the elevated intracranial pressure.

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Endovascular treatment of intracranial dural arteriovenous fistulas with spinal perimedullary venous drainage

Journal of Neurosurgery ◽

10.3171/jns.1992.77.5.0718 ◽

1992 ◽

Vol 77 (5) ◽

pp. 718-723 ◽

Cited By ~ 64

Author(s):

Y. Pierre Gobin ◽

Andre Rogopoulos ◽

Armand Aymard ◽

Mazen Khayata ◽

Daniel Reizine ◽

...

Keyword(s):

Endovascular Treatment ◽

Endovascular Therapy ◽

Sinus Thrombosis ◽

Venous Drainage ◽

Transverse Sinus ◽

Venous Hypertension ◽

Arterial Phase ◽

Content Type ◽

Arteriovenous Fistulas ◽

Av Fistulas

✓ Intracranial dural arteriovenous (AV) fistulas with spinal perimedullary venous drainage are rare lesions that have distinctive clinical, radiological, and therapeutic aspects. Five patients presented with an ascending myelopathy, which extended to involve the brain stem in three cases. Myelography and magnetic resonance imaging showed slightly dilated spinal perimedullary vessels. Spinal angiograms were normal in the arterial phase. Diagnosis was only possible after cerebral angiography, which demonstrated posterior fossa AV fistulas fed by meningeal arteries and draining into spinal perimedullary veins. Endovascular treatment alone resulted in angiographic obliteration of the lesion in three patients. Two patients required surgery in addition to endovascular therapy. One patient died postoperatively, and in one a transient complication of embolization was observed. Improvement after treatment was good in two cases and fair in two. Transverse sinus thrombosis was observed in three cases and was probably the cause of the aberrant venous drainage of the fistula into the spinal perimedullary veins. The pathophysiology is related to spinal cord venous hypertension. These lesions were classified as Type 5 in the Djindjian and Merland classification of dural intracranial AV fistulas. Endovascular therapy is a safe effective method in the treatment of these fistulas and should be tried first.

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Headache and Sleep-Related Breathing Disorders Among Patients With Sclerosteosis and Disease Carriers: The First Family in Italy

10.21203/rs.3.rs-80061/v1 ◽

2020 ◽

Author(s):

Laura Rapisarda ◽

Monica Gagliardi ◽

Caterina Bombardieri ◽

Radha Procopio ◽

Giulio Demonte ◽

...

Keyword(s):

Obstructive Sleep Apnea ◽

Intracranial Hypertension ◽

Venous Drainage ◽

Venous Hypertension ◽

Sleep Related Breathing Disorders ◽

Italian Family ◽

Gene Carriers ◽

Breathing Disorders ◽

Obstructive Sleep ◽

Causative Factors

Abstract BackgroundHeadache and sleep-related breathing disorders among patients with sclerosteosis and disease carriers has been little studied. We investigated the neurological and neuroimaging features of patients and gene carriers of a large Italian family with sclerosteosis.Main bodyIn this Italian family with sclerosteosis, genetic tests detected the homozygous mutation p.Gln24X (c.70C>T) of the SOST gene in the proband, and a heterozygous mutation between 9 siblings. Severe hyperostosis of the skull caused chronic headache secondary to intracranial hypertension due to venous hypertension and obstructive sleep apnea syndrome in adult homozygotes. There was transosseous intracranial-extracranial occipital venous drainage which indicated a compensatory mechanism for intracranial venous hypertension, one of the causative factors of increased CSF pressure in sclerosteosis. While mild hyperostosis of the skull was associated with headache and snoring in heterozygotes. ConclusionsHeadache and sleep-related breathing disorders are the most common clinical manifestations among patients with sclerosteosis and disease carriers. Cerebral venous hypertension leading to intracranial hypertension and facial deformities are the causative factors of headache and sleep-related breathing disorders. These data suggest that venous stenting to accommodate cerebral venous drainage could be useful in the treatment of intracranial hypertension, and correction of facial deformities to relieve obstructive sleep apnea may play a role in the treatment of patients with sclerosteosis. The data highlight that it is reasonable to extend neurological evaluation and radiological study to gene carriers.

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Endovascular Treatment for Idiopathic Intracranial Hypertension Improves Clinical Symptoms and Signs

Arquivos Brasileiros de Neurocirurgia Brazilian Neurosurgery ◽

10.1055/s-0036-1597820 ◽

2017 ◽

Vol 36 (01) ◽

pp. 01-06

Author(s):

João Silva ◽

Mario Conti ◽

Guilherme Aguiar ◽

Mauricio Jory ◽

Paulo Monzillo ◽

...

Keyword(s):

Endovascular Treatment ◽

Intracranial Hypertension ◽

Idiopathic Intracranial Hypertension ◽

Clinical Symptoms ◽

Venous Drainage ◽

Transverse Sinus ◽

Pressure Gradients ◽

Sinus Stenosis ◽

Symptoms And Signs

Introduction New physiopathological concepts regarding idiopathic intracranial hypertension (IIH) recommend the endovascular treatment in refractory patients with transverse sinus stenosis. Objectives To assess the role of the transverse sinus stenting treatment in the symptomatology of patients with IIH. Method Clinically refractory patients with impaired venous drainage of the transverse sinus were submitted to cerebral angiographies. Patients with pre and post-stenotic pressure gradients > 8 mmHg were submitted to endovascular treatment. Results Seven patients underwent cerebral angiography with manometry. Stenting was performed in six cases after pressure gradient assessment. All cases showed improvements in headache and resolution of papilledema. Discussion and Conclusion Although the role of endovascular therapy should be further studied, our data suggest it may improve the clinical symptoms and signs of IIH in selected patients.

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De Novo Pial Arteriovenous Fistula Associated With Cerebral Infarction and Venous Hypertension: Report of 2 Cases Suggesting a “Second Hit Theory”

Neurosurgery Open ◽

10.1093/neuopn/okab015 ◽

2021 ◽

Vol 2 (2) ◽

Author(s):

Tomoaki Terada ◽

Sadayoshi Nakayama ◽

Akira Wada ◽

Yuko Tanaka ◽

Hajime Yabuzaki ◽

...

Keyword(s):

Cerebral Infarction ◽

Arteriovenous Fistula ◽

De Novo ◽

Sinus Thrombosis ◽

Venous Drainage ◽

Transverse Sinus ◽

Venous Hypertension ◽

Venous Infarction ◽

Second Hit ◽

Pial Arteriovenous Fistula

ABSTRACT BACKGROUND AND IMPORTANCE The etiology of de novo pial arteriovenous fistula (AVF) is unknown. We found 2 cases of de novo pial AVF, which appeared after cerebral infarction and which was associated with venous hypertension secondary to venous sinus thrombosis with a dural AVF (dAVF). Additional angiogenic stimuli (second hit) were considered as one of the mechanisms of de novo pial AVF. CLINICAL PRESENTATION A 63-yr-old male was admitted to our hospital due to an intraventricular hemorrhage. He had a history of cerebral infarction 2 yr before. Angiography demonstrated multiple dAVFs with bilateral occlusion of the distal transverse sinus associated with prominent retrograde cortical venous drainage. A pial AVF was found at the border of his previous cerebral infarction. Both lesions were successfully treated using endovascular technique. A second case involved a 47-yr-old female who was admitted to our hospital due to venous infarction also associated with sinus thrombosis. De novo pial AVF at the border of the venous infarction and dAVF at the transverse sigmoid junction were demonstrated on angiography 6 mo later. CONCLUSION We speculate that venous hypertension associated with additional angiogenic stimuli (second hit) due to brain ischemia and/or brain injury related to infarction caused de novo pial AVF in these 2 cases.

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