Torcular dural sinus malformation

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.

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.


2021 ◽  
Vol 10 (4) ◽  
pp. 205846012110063
Author(s):  
Hana Shabana ◽  
Johannes Leidinger ◽  
Johan Wikström ◽  
Ove Axelsson

Dural sinus malformation is a rare condition. We describe a prenatally detected case followed by repeated ultrasound scans and a prenatal magnetic resonance imaging examination. A substantial spontaneous regression was observed, which is associated with a favorable outcome. We believe that our observations, including a long postnatal follow-up, will add to the present knowledge of prenatally detected cases, and thus improve management of the pregnancies as well as our possibilities to counsel the parents-to-be.


Author(s):  
Matthew McInnes ◽  
Katherine Fong ◽  
Andrea Grin ◽  
Karel ter Brugge ◽  
Susan Blaser ◽  
...  

Background:Dural sinus malformation (DSM) is a term used to describe congenital vascular malformations characterized by massive dilation of one or more dural sinuses: these dilatations are typically associated with arteriovenous shunts. Such malformations can present antenatally but their early natural history and anatomy is poorly defined.Methods:We reviewed five years of autopsy experience and retrieved three primary vascular malformations of the fetal dural sinuses with ultrasound, magnetic resonance imaging (MRI) and post-mortem correlation.Results:Fetal ultrasound and MRI obtained between 19 and 23 weeks gestational age demonstrated in all cases dilation of the dural sinuses. In two cases vascular thromboses were present in either the dilated dural sinus (one of three) or the associated arteriovenous fistula (one of three). All cases were autopsied at 22-23 weeks gestational age. In one there was imaging and autopsy evidence of remodeling of the dural sinuses associated with a pial arteriovenous fistula. In two cases, no arteriovenous malformation was identified on initial imaging, but only became evident at autopsy. One case showed morphological overlap with vein of Galen aneurysmal malformation, with a midline arteriovenous shunt and vein of Galen ectasia. The other demonstrated a perisylvian dural arteriovenous fistula.Conclusion:In utero thrombosis of feeding vascular malformations or of the dural sinus malformation may be prominent. The early in utero developmental trajectory of dural sinus malformation (DSM) is poorly defined and deserves further study.


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.


Neurosurgery ◽  
2019 ◽  
Vol 66 (Supplement_1) ◽  
Author(s):  
Bamidele O Adebayo ◽  
Okezie Kanu ◽  
Olufemi B Bankole ◽  
Omotayo Ojo

Abstract INTRODUCTION Myelomeningocele is associated with hydrocephalus in 35% to 90% of cases. Hydrocephalus is usually treated with insertion of ventriculoperitoneal shunt. However, endoscopic third ventriculostomy with or without choroid plexus cauterization is a viable alternative. METHODS A prospective longitudinal study from January 2016 to December 2018 on patients with myelomeningocele who presented to Lagos University Teaching Hospital, Nigeria and who had hydrocephalus.Informed consent for research and surgery were obtained. ETV with or without CPC or VPS was done with general anesthesia using standard operative techniques. Patients were followed up monthly for at least 6 mo. The surgery was adjudged failed if there was progressive head enlargement as well as other signs of hydrocephalus necessitating another surgery for hydrocephalus. RESULTS A total of 48 patients completed the study. A total of Six patients were lost to follow-up. Age range of the patients was 1 to 44 wk. A total of Five patients had ETV, 21 had ETV/CPC, and 22 patients had VPS. The mean OFC at the time of surgery were 45.3 cm and 44.9 cm for the endoscopic and VPS arms respectively. There were 2 (7.6%) cases of CSF leak in the endoscopic arm and 3 (13.6%) cases of wound dehiscence, with 1 (4.5%) case of CSF leak in the VPS arm. Mean age at surgery for ETV/CPC arm was 12.3 wand 11.5 wk for the VPS arm. Mean time to failure was 9.9 and 6.3 wk for endoscopic and VPS arms respectively. Four (80%) of the patients who had ETV had a successful outcome, 11 (52.3%) of those who had ETV/CPC had a successful outcome and 13 (59%) of those who had VPS had a successful outcome. At 6 mo follow up, overall success rate for the endoscopic arm was 57.6% and 59% for the VPS. CONCLUSION Endoscopic third ventriculostomy with or without choroid plexus cauterization had similar success rate with VPS at 6 mos.


2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi59-vi60
Author(s):  
Timothy Crook ◽  
Darshana Patil ◽  
Dadasaheb Akolkar ◽  
Anantbhushan Ranade ◽  
Amit Bhatt ◽  
...  

Abstract Brain metastasis in solid organ cancers is associated with adverse prognosis, which is further aggravated by limited systemic treatment options. Such patients are also often excluded from clinical trials since their poor prognosis is perceived to unfavorably impact trial outcomes and misrepresent efficacy data. We retrospectively evaluated the efficacy of treatment guided by Encyclopedic Tumor Analysis (ETA) in patients with advanced refractory malignancies and brain metastases to determine the impact on outcomes. Freshly biopsied tumor tissue (primary / lymph node / liver) and peripheral blood of patients were used for integrational multi-analyte investigations as part of ETA, which included gene mutations, gene expression, and in vitro chemosensitivity profiling of viable tumor cells. Based on ETA, patients received individualized therapy recommendations. All patients underwent a PET-CT scan as well as MRI scan prior to treatment start to determine extent of disease. All patients underwent follow-up PET-CT scans and brain MRI scans every 6–8 weeks. Of the ten patients with brain metastases, which were evaluated after receiving ETA-guided treatment, the median follow-up duration was 97 days (range 79 – 180 days) during which all ten patients remained progression-free. Median time to progression for these patients on the last (failed) line of treatment was 91 days (range 30 - 176 days). Five patients showed partial response and five patients showed stable disease while on ETA-guided treatment. During the follow-up period, all brain metastases were either stable (n=7) or had regressed (n=3), and none of the patients reported new brain lesions. Personalized ETA guided treatments imparted clinical benefit by halting disease progression in this cohort of high-risk patients who would have otherwise been considered for palliative regimens due to perceived unfavorable prognosis.


2007 ◽  
Vol 18 (4) ◽  
pp. 692-699 ◽  
Author(s):  
Valérie Merzoug ◽  
Sabrina Flunker ◽  
Cyrine Drissi ◽  
Danielle Eurin ◽  
Gilles Grangé ◽  
...  

2001 ◽  
Vol 7 (4) ◽  
pp. 319-323 ◽  
Author(s):  
C. Kwong Yau ◽  
H. Alvarez ◽  
P. Lasjaunias

A rare case of dural sinus malformation with dural arteriovenous fistula in an infant is presented. Presenting symptom was progressive macrocrania without hydrocephalus. A high flow arteriovenous of the sigmoid sinus associated with jugular bulb diaphragm was demonstrated. Reflux in the intracranial sinus was present. The shunt was successfully occluded by transarterial embolization. The natural history and treatment strategy are discussed.


Cephalalgia ◽  
2013 ◽  
Vol 33 (11) ◽  
pp. 948-950 ◽  
Author(s):  
Raquel Gil-Gouveia

Introduction: Orthostatic headache is very suggestive of intracranial hypotension. It has a good prognosis as it usually responds to conservative treatment or epidural blood patches. Case report: A 36-year-old female presented with severe and prolonged orthostatic headache starting after a seizure. No stigma of intracranial hypotension was detected on brain MRI, and intracranial pressure was within normal range. No imaging evidence of a fistula was found. She was refractory to symptomatic treatment including five epidural blood patches. Progressive improvement occurred simultaneously to the introduction of vitamin A supplementation. Discussion: A series of six similar patients is discussed, in which five patients remained severely symptomatic and workdisabled at an average follow-up of four years. It is proposed that the pathophysiological mechanism producing orthostatic headache might not be dependent on intracranial hypotension and could respond to vitamin A.


2021 ◽  
Vol 26 (1) ◽  
Author(s):  
Matheus Melo PITHON

ABSTRACT Introduction: Orthodontic treatment in patients with traumatized teeth is a condition that needs good planning in order to achieve satisfactory results. Objective: To discuss approaches to orthodontic treatment of malocclusions associated with trauma followed by avulsion of anterior teeth, reimplanted after a short period of time. Case report: The treatment started with the distalization of upper posterior teeth, with the aid of mini-implants and sliding jigs, followed by the inclusion of anterior teeth in the arch, followed by intrusion of these teeth. Results: With the treatment, improved mobility of the anterior teeth was achieved, with better insertion into bone tissue. The most important factor for satisfactory treatment and a good prognosis for avulsion is the time the tooth remains outside the socket. Orthodontic treatment in patients with traumatized teeth is not contraindicated; however, clinical and radiographic aspects must be considered. Conclusion: Among the feasible orthodontic treatment options, the conservative approach can be a very favorable treatment alternative.


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