scholarly journals Growing Dural Sinus Malformation with Associated Developmental Venous Anomaly, Multiple Cavernomas and Facial Venous Malformation in an Infant

2002 ◽  
Vol 8 (4) ◽  
pp. 421-430 ◽  
Author(s):  
Z. Mohamed ◽  
LL Batista ◽  
M. Sachet ◽  
J. Mahadevan ◽  
H. Alvarez ◽  
...  

This is an unusual case report of an infant, who initially presented with a facial haemangioma and was later diagnosed to have a dural sinus malformation (DSM) involving the torcula. The DSM increased in size lateralising to the right transverse sinus at three months of age. Postnatal enlargement of the dural sinus has not been described before suggesting a delay in the maturation of the dural sinus which normally would occur antenatally. There was a further association with a complex developmental venous anomaly (DVA) draining the right cerebral hemisphere into the deep cerebral vein and multiple cavernous malformations. The DVA was not clearly demonstrated at age one month but was more obvious at age three months. This would be the first reported case of DSM associated with a DVA. Increasing venous hypertension probably contributed to the poor opacification of the DVA on follow-up angiography at age six months and to the haemorrhagic changes within the cavernomas on magnetic resonance imaging (MRI). The therapeutic goal was to correct venous hypertension by partially embolising the dural shunts to remodel the cerebral vasculature and preserve the patent sinus. The treatment strategy and possible link between the complex disease entities presented in this infant are discussed. Despite these attemps, the lesion continued to grow compressing the posterior fossa structures. The infant died at nine months of age.

2021 ◽  
pp. 197140092110428
Author(s):  
Nimisha Parikh ◽  
Richard Williamson ◽  
Matthew Kulzer ◽  
Albert Sohn ◽  
Warren M Chang ◽  
...  

Cavernous malformations are angiographically occult vascular malformations. They are often associated with a developmental venous anomaly through poorly understood mechanisms. We present an unusual case of a gradually enlarging cavernous malformation associated with a developmental venous anomaly with arteriovenous shunting, suggesting venous hypertension or reflux as a potential cause of progressive growth.


2017 ◽  
Vol 23 (5) ◽  
pp. 510-515 ◽  
Author(s):  
Katsuhiro Mizutani ◽  
Tomoru Miwa ◽  
Takenori Akiyama ◽  
Tokunori Kanazawa ◽  
Hideaki Nagashima ◽  
...  

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.


Neurosurgery ◽  
2001 ◽  
Vol 49 (5) ◽  
pp. 1246-1250 ◽  
Author(s):  
Richard E. Clatterbuck ◽  
İlhan Elmacı ◽  
Daniele Rigamonti

ABSTRACT OBJECTIVE AND IMPORTANCE Capillary telangiectasias, cavernous malformations, and developmental venous anomalies are all vascular malformations that occur on the capillary-venous side of the cerebral circulation. The associations of capillary telangiectasias with venous malformations, cavernous malformations with venous malformations, and capillary telangiectasias with cavernous malformations have all been described; however, the association of all three lesions in a single patient is extremely rare. CLINICAL PRESENTATION A 52 year-old Caucasian woman presented to our clinic with an extended history of confusion, distorted visual perceptions, photophobia, neck pain, swallowing problems, and poor balance. The patient's examination was remarkable for difficulty concentrating, mild rotatory nystagmus, subtle decreased sensation over the left side of the face and body, and brisk reflexes. Review of the patient's magnetic resonance imaging examination demonstrated a cavernous malformation, a capillary telangiectasia, and a developmental venous anomaly located adjacent to one another in the brainstem. INTERVENTION Given the patient's complex constellation of symptoms and relatively mild neurological findings, it was difficult to ascribe any one of them to a specific vascular malformation. Conservative management of this patient's vascular malformations was decided upon. CONCLUSION Juxtaposition of these three different vascular lesions in the brainstem of an otherwise normal individual suggests a relationship among them. Although there are several theories that link similar associations through physiological mechanisms such as venous hypertension, we propose that a developmental event disrupting local capillary-venous pattern formation is a plausible alternative.


2015 ◽  
Vol 86 (11) ◽  
pp. e4.99-e4
Author(s):  
Katherine Dodd ◽  
Emily Pegg ◽  
Sachin Mathur ◽  
Chhetri Suresh

Developmental venous anomaly (DVA) is a commonly encountered congenital abnormality of the venous drainage system. Spontaneous thrombosis of DVA is rare. We present a case of thrombosed brainstem DVA leading to venous infarction and oedema within the posterior fossa.A 49 year old, previously fit gentleman presented to the local hospital with a one day history of headache, slurred speech and incoordination. Examination demonstrated GCS of 13/15, cerebellar dysarthria, horizontal nystagmus to the left, mild right sided pyramidal weakness, right sided cerebellar ataxia and bilateral extensor plantars.CT venogram revealed a cerebellar DVA with thrombosis of one of the veins. There was surrounding venous infarction and oedema within the posterior fossa, causing compression of the fourth ventricle and dilatation of the lateral ventricles. MRI demonstrated extensive T2 change in the right cerebellar hemisphere, dorsal pons and right cerebral peduncle. No underlying thrombotic tendency was identified. He was treated successfully with intravenous heparin infusion. He improved over the next 3 weeks, and was discharged on warfarin with mild right sided ataxia and cerebellar dysarthria.Our case demonstrates that DVAs, generally considered as common insignificant anatomical variants, can uncommonly lead to significant complications.


2016 ◽  
Vol 29 (6) ◽  
pp. 458-464 ◽  
Author(s):  
Hariprakash Chakravarthy ◽  
Tzu-Kang Lin ◽  
Yao-Liang Chen ◽  
Yi-Ming Wu ◽  
Chin-Hua Yeh ◽  
...  

This is a case report of de novo development of two cerebral cavernous malformations adjacent to existing developmental venous anomalies. The development of cavernomas was noted over a follow-up period of 10 years. These developments happened during the course of staged endovascular management of a complex dural arterio-venous fistula along the right sphenoid wing. The patient presented with a proptosis secondary to lympho-haemangiomatous lesion of the fronto-orbital region and a high-flow right sphenoid wing dural arterio-venous fistula. During the initial period of conservative management of the dural arterio-venous fistula, he developed de novo cavernous malformations in the left mesial temporal lobe adjacent to a developmental venous anomaly in the temporal lobe, and along with this there was engorgement of deep veins related to another existing developmental venous anomaly in the brainstem. Later during the course of endovascular treatment of the dural arterio-venous fistula, a large brainstem cavernoma developed adjacent to the brainstem developmental venous anomaly. This case report discusses the cause-effect relationship of venous pressure changes related to management of dural arterio-venous fistula and de novo formation of cerebral cavernous malformations adjacent to existing developmental venous anomalies.


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.


2013 ◽  
Vol 19 (1) ◽  
pp. 67-72 ◽  
Author(s):  
Jmn Enslin ◽  
D. Lefeuvre ◽  
A. Taylor

Developmental venous anomalies (DVA) drain normal neural tissue and are mostly discovered incidentally. We describe a young patient with a left hemisphere superficial to deep DVA and right hemisphere venous outflow restriction presenting with a seizure. The right hemisphere drainage variation is not typical of a DVA but represents another drainage pattern on the border of normality.


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