Category Archives: Vascular

Book: Moyamoya Disease Explored Through RNF213: Genetics, Molecular Pathology, and Clinical Sciences

Moyamoya Disease Explored Through RNF213: Genetics, Molecular Pathology, and Clinical Sciences (Current Topics in Environmental Health and Preventive Medicine)

Moyamoya Disease Explored Through RNF213: Genetics, Molecular Pathology, and Clinical Sciences (Current Topics in Environmental Health and Preventive Medicine)

List Price: $159.00
This book presents the latest findings on biological, epidemiological, and clinical investigations of RNF213, which is thought to be involved in many biological processes and plays a key role in cerebro- and cardiovascular disease . By discussing the epidemiology and genetic epidemiology of the disease with a particular focus on the molecular function of RNF213, research using animal models, diagnosis, therapy and clinical management around the world, this work makes a valuable contribution to the study of the disease.
Moyamoya Disease Explored Through RNF213 is an indispensable resource for both beginning and experienced researchers, pediatricians, neurologists, and neurosurgeons who are seeking comprehensive information on adult and childhood stroke.

Product Details

  • Published on: 2017-03-22
  • Original language: English
  • Number of items: 1
  • Dimensions: 9.30″ h x .0″ w x 6.10″ l,
  • Binding: Hardcover
  • 185 pages

Akio Koizumi, Department of Health and Environmental Sciences, Graduate School of Medicine, Kyoto University

Kazuhiro Nagata, Laboratory of Molecular and Cellular biology, Faculty of Life Sciences, Kyoto Sangyo University

Kiyohiro Houkin, Department of Neurosurgery, Graduate School of Medicine, Hokkaido University

Teiji Tominaga, Department of Neurosurgery, Tohoku University School of Medicine
Susumu Miyamoto, Department of Neurosurgery, Graduate School of Medicine, Kyoto University
Shigeo Kure, Department of Pediatrics, Tohoku University School of Medicine
Elizabeth Tournier‐Lasserve, CHU Paris‐GH St‐Louis Lariboisière F‐Widal ‐ Hôpital Lariboisière

Update: Molsidomine

Molsidomine is an orally active, long acting vasodilator. Molsidomine is metabolized in the liver to the active metabolite linsidomine. Linsidomine is an unstable compound that releases nitric oxide (NO) upon decay as the actual vasodilating compound.

Belongs to the drug class of sydnones . SIN-1A metabolite of Molsidomine has pharmacologically active group of NO, which by increasing levels of cGMP, decreases levels of intracellular calcium ions in smooth muscle cells. This effect leads to relaxation of smooth muscle vasculature, inhibits platelets aggregation and has indirect antiproliferative effect. In clinical observations no effect of tolerance to the drug was observed. Experimental data show additional mechanism of action of the drug: SIN-1C metabolites protects the reoxygenated cardiomyocyte from post-reperfusion damage. Indications for use of Molsidomine are: ischaemic heart disease, chronic heart failure and pulmonary hypertension. Effects of Molsidomine use in acute myocardial infarction and unstable angina were compared in clinical trials to effects of nitroglycerin use. Both drugs were found equally potent, but authors underline the fact of better Molsidomine tolerability comparing NTG, but longer serum half-time of Molsidomin effects that control of the treatment is worse. In clinical trials it was suggested that intravenous use of Molsidomine metabolite SIN-1 during PTCA procedures is more effective than use of isosorbide dinitrate in the same procedures. In other clinical trials molsidomin was found to produce beneficial effects in patients with heart failure due to ischaemic cardiomyopathy, dilatative cardiomyopathy, in essential hypertension, pulmonary artery hypertension in COPD patients and in congestive heart failure 1).

Ehlers et al. examined the effects of treatment with molsidomine with regard to decreasing the incidence of spasm-related delayed cerebral infarctions and improving clinical outcome in patients with SAH.

Seventy-four patients with spontaneous aneurysmal subarachnoid hemorrhage (SAH) were included in this post hoc analysis. Twenty-nine patients with SAH and proven cerebral vasospasm (CVS) received molsidomine in addition to oral or intravenous nimodipine. Control groups consisted of 25 SAH patients with proven vasospasm and 20 SAH patients without. These patients received nimodipine therapy alone. Cranial computed tomography (CCT) before and after treatment was analyzed for CVS-related infarcts. A Modified National Institutes of Health Stroke Scale (mNIHSS) and the modified Rankin Scale (mRS) were used to assess outcomes at a 3-month clinical follow-up.

Four of the 29 (13.8%) patients receiving molsidomine plus nimodipine and 22 of the 45 (48%) patients receiving nimodipine therapy alone developed vasospasm-associated brain infarcts (p < 0.01). Follow-up revealed a median mNIHSS score of 3.0 and a median mRS score of 2.5 in the molsidomine group compared with scores of 11.5 and 5.0, respectively, in the nimodipine group with CVS (p < 0.001). One patient in the molsidomine treatment group died, and 12 patients in the standard care group died (p < 0.01).

In this post hoc analysis, patients with CVS who were treated with intravenous molsidomine had a significant improvement in clinical outcome and less cerebral infarction. Molsidomine offers a promising therapeutic option in patients with severe SAH and CVS and should be assessed in a prospective study 2).

Durak et al., investigated the protective and therapeutic effects of molsidomine (MOL) in a rat model of whole brain radiotherapy (RT). Forty female rats were divided into five groups of eight: group 1, control; group 2, 15 Gy single dose RT (RT); group 3, 4 mg/kg MOL treated for 5 days (MOL); group 4, 4 mg/kg MOL for 5 days, 10 days after RT treatment (RT + MOL); group 5, 4 mg/kg MOL treatment for 5 days before RT treatment and for 5 days after RT treatment (MOL + RT). All rats were sacrificed on day 16. Neurodegenerative changes in the brain and tissue levels of oxidants and antioxidants were evaluated. The oxidative parameters were increased and antioxidant status was decreased in group RT compared to groups MOL + RT and RT + MOL. Histopathological examination showed that treatment with MOL after RT application and treatment with MOL before RT treatment decreased neuronal degeneration. No difference in neuronal appearance was found between groups RT + MOL and MOL + RT. MOL treatment protected the nervous system of rats and may be a treatment option for preventing RT induced neural injury 3)

1) Kmieć M, Ochmański W. [Molsidomine: importance in treatment of circulation disorders]. Przegl Lek. 1998;55(10):532-6. Review. Polish. PubMed PMID: 10224868.
2) Ehlert A, Schmidt C, Wölfer J, Manthei G, Jacobs AH, Brüning R, Heindel W, Ringelstein EB, Stummer W, Pluta RM, Hesselmann V. Molsidomine for the prevention of vasospasm-related delayed ischemic neurological deficits and delayed brain infarction and the improvement of clinical outcome after subarachnoid hemorrhage: a single-center clinical observational study. J Neurosurg. 2016 Jan;124(1):51-8. doi: 10.3171/2014.12.JNS13846. Epub 2015 Jul 10. PubMed PMID: 26162034.
3) Durak MA, Parlakpinar H, Polat A, Vardi N, Ekici K, Ucar M, Ozhan O, Yildiz A, Pasahan R. Protective and therapeutic effects of molsidomine on radiation induced neural injury in rats. Biotech Histochem. 2017 Feb 6:1-10. doi: 10.1080/10520295.2016.1271454. [Epub ahead of print] PubMed PMID: 28166419.


The British Neurovascular Group evolved from informal groupings
of interested clinicians and researchers within the Society of
British Neurological Surgeons.
They initially assembled under the banner of the Spontaneous
Intracerebral Haemorrhage Group and members conceived or
facilitated important British contributions to the care of patients
with neurovascular disease including the STICH trials.

Mr Stuart Ross
Consultant Neurosurgeon, Leeds General Infirmary, Leeds
Organising Committee

Mr Edward Jerome St George
Consultant Neurosurgeon, Southern General Hospital, Glasgow

Mr Nigel Suttner
Consultant Neurosurgeon, Queen Elizabeth University Hospital,

Invited Speakers
Jeremiah N. Johnson, MD, Dept. of Neurosurgery, UT Health
Science Centre San Antonio, San Antonio, Texas, USA

Mr. Mario Teo, Consultant Neurosurgeon, Bristol, UK

The hybrid endovascular neurosurgeon in practice
• Legislative and bureaucratic hurdles to combined endovascular/
neurosurgical training in the UK
• Moyamoya disease



13:10 Prevalence of cerebral vascular abnormalities in spontaneous subarachnoid haemorrhage I Phang

13:20 Is digital subtraction angiography (DSA) necessary in cases of perimesencephalic J Walker
subarachnoid haemorrhage (PMSAH) if CT angiogram (CTA) is negative – A systematic review Page 5
13:30 Associations between outcome and radiological screening practices for delayed cerebral ischaemia M Hollingworth

13:40 The role of semi-quantitative flow analysis with indocyanine green videoangiography in A Ghosh
cerebrovascular surgery

13:50 Risk factors of shunt dependent hydrocephalus in aneurysmal subarachnoid hemorrhage – M Blagia
Our experience and literature review

14:00 Guest Lecture J Johnson
The comprehensive (dual-trained) cerebrovascular neurosurgeon. Is one better than two?

15:10 Seminar – Dual training in neurovascular surgery H Patel, J Johnson

15:40 A single centre experience of intra operative angiogram use in arteriovenous malformation excision G Dobson

15:50 Does the availability of a microvascular neurosurgeon impact on the management and outcomes in K Ageymang
patients with an aneurysmal clot?

16:00 Surgical outcomes of very poor grade subarachnoid haemorrhage M Kommer

16:10 Sylvian fissure haematomas – A single unit experience R Chave-Cox

16:20 Cryptogenic subarachnoid haemorrhage – What’s new, MR? C Li


09:00 Guest lecture M Teo
Moyamoya disease – The last frontier for brain bypass surgery

09:30 Moyamoya disease in Denmark – A population based register study P Birkeland

09:40 EC-IC bypass – A single centre experience C Robson

09:50 Neurovascular simulator – A novel training aid A Sheikh

10:00 MSN – 24/7 Scottish coiling service D Seok-Lee

10:30 A neuroradiologist’s perspective on INR training M Puthuran

11:00 Guest lecture J Johnson
Neurovascular training – A United States perspective

11:15 Guest Lecture M Teo
USA neurovascular fellowship – A United Kingdom perspective

11:30 MISTIE III – Update on progress B Gregson

11:40 The SCIL-SAH Phase II study – Results and implications for future trials J Galea

11:50 In-hospital outcomes of aneurysmal SAH in the UK and Ireland H Patel

12:00 Treatment of poor grade subarachnoid haemorrhage trial – TOPSAT 2 – Update on progress B Gregson

Venous sinus stenting for idiopathic intracranial hypertension

Trials suggest that venous sinus stenting offers both comparable rates of efficacy – with improved papilledema in 97% of patients, resolved headache in 83%, and improved visual acuity in 78% .

Patients whose sight is threatened by medically refractory IIH must often consider invasive procedures to control their disease. Venous sinus stenting may offer equal efficacy and lower failure and complication rates than traditional surgical approaches such as optic nerve sheath fenestration and cerebrospinal fluid diversion 3).




A systematic review of the surgical treatment of IIH was carried out. Cochrane Library, MEDLINE and EMBASE databases were systematically searched from 1985 to 2014 to identify all relevant manuscripts written in English. Additional studies were identified by searching the references of retrieved papers and relative narrative reviews.

Forty-one (41) studies were included (36 case series and 5 case reports), totalling 728 patients. Three hundred forty-one patients were treated with optic nerve sheath fenestration (ONSF), 128 patients with lumboperitoneal shunting (LPS), 72 patients with ventriculoperitoneal shunting (VPS), 155 patients with venous sinus stenting and 32 patients with bariatric surgery. ONSF showed considerable efficacy in vision improvement, while CSF shunting had a superior headache response. Venous sinus stenting demonstrated satisfactory results in both vision and headache improvement along with the best complication profile and low relapse rate, but longer follow-up periods are needed. The complication rate of bariatric surgery was high when compared to other interventions and visual outcomes have not been reported adequately. ONSF had the lowest cost.

No surgical modality proved to be clearly superior to any other in IIH management. However, in certain contexts, a given approach appears more justified. Therefore, a treatment algorithm has been formulated, based on the extracted evidence of this review. The traditional treatment paradigm may need to be re-examined with sinus stenting as a first-line treatment modality 4).


Kanagalingam et al., review the role of cerebral venous sinus stenting in the management of patients with medically refractory pseudotumor cerebri. Although long- term studies are needed in this field, the current reports indicate a favorable outcome for preventing vision loss and symptom control 5).


In 2013, a review of the literature was performed which identified patients with IIH treated with venous sinus stenting. The procedural data and outcomes are presented. A total of 143 patients with IIH (87% women, mean age 41.4 years, mean body mass index 31.6 kg/m(2)) treated with venous sinus stenting were included in the analysis. Symptoms at initial presentation included headache (90%), papilledema (89%), visual changes (62%) and pulsatile tinnitus (48%). There was a technical success rate of 99% for the stent placement procedure with a total of nine complications (6%). At follow-up (mean 22.3 months), 88% of patients experienced improvement in headache, 97% demonstrated improvement or resolution of papilledema, 87% experienced improvement or resolution of visual symptoms and 93% had resolution of pulsatile tinnitus. In patients with IIH with focal venous sinus stenosis, endovascular stent placement across the stenotic sinus region represents an effective treatment strategy with a high technical success rate and decreased rate of complications compared with treatment modalities currently used 6).

Teleb et al., aimed to review all published cases and case series of dural sinus stenting for IIH, with analysis of patient presenting symptoms, objective findings (CSF pressures, papilledema, pressure gradients across dural sinuses), follow-up of objective findings, and complications.

A Medline search was performed to identify studies meeting pre-specified criteria of a case report or case series of patients treated with dural sinus stent placement for IIH. The manuscripts were reviewed and data was extracted.

A total of 22 studies were identified, of which 19 studies representing 207 patients met criteria and were included in the analysis. Only 3 major complications related to procedure were identified. Headaches resolved or improved in 81% of patients. Papilledema improved the (172/189) 90%. Sinus pressure decreased from an average of 30.3 to 15 mm Hg. Sinus pressure gradient decreased from 18.5 (n=185) to 3.2 mm Hg (n=172). Stenting had an overall symptom improvement rate of 87%.

Although all published case reports and case series are nonrandomized, the low complication and high symptom improvement rate make dural sinus stenting for IIH a potential alternative surgical treatment. Standardized patient selection and randomization trials or registry are warranted 7).

Case series


Seventeen patients underwent dural venous sinus stenting (DVSS). Average pre- and post-intervention pressure gradients were 23.06 and 1.18 mmHg, respectively (p < 0.0001). Sixteen (94%) noted improvement in headache, fourteen (82%) had visual improvement and all (100%) patients had improved main symptom. Of 11 patients with optical coherence tomography, 8 showed decreased RNFL thickness and 3 remained stable; furthermore, these 11 patients had improved vision with improved papilledema in 8, lack of pre-existing papilledema in 2 and stable, mild edema in 1 patient.

This series of patients with dural sinus stenosis demonstrated improvement in vision and reduction in RNFL thickness. DVSS appears to be a useful treatment for IIH patients with dural sinus stenosis 8).


Ten patients for whom medical therapy had failed were prospectively followed. Ophthalmological examinations were assessed, and patients with venous sinus stenosis on MR angiography proceeded to catheter angiography, venography with assessment of pressure gradient, and ICP monitoring. Patients with elevated ICP measurements and an elevated pressure gradient across the stenosis were treated with stent placement. RESULTS All patients had elevated venous pressure (mean 39.5 ± 14.9 mm Hg), an elevated gradient across the venous sinus stenosis (30.0 ± 13.2 mm Hg), and elevated ICP (42.2 ± 15.9 mm Hg). Following stent placement, all patients had resolution of the stenosis and gradient (1 ± 1 mm Hg). The ICP values showed an immediate decrease (to a mean of 17.0 ± 8.3 mm Hg), and further decreased overnight (to a mean of 8 ± 4.2 mm Hg). All patients had subjective and objective improvement, and all but one improved during follow-up (median 23.4 months; range 15.7-31.6 months). Two patients developed stent-adjacent stenosis; retreatment abolished the stenosis and gradient in both cases. Patients presenting with papilledema had resolution on follow-up funduscopic imaging and optical coherence tomography (OCT) and improvement on visual field testing. Patients presenting with optic atrophy had optic nerve thinning on follow-up OCT, but improved visual fields. CONCLUSIONS For selected patients with IIH and venous sinus stenosis with an elevated pressure gradient and elevated ICP, venous sinus stenting results in resolution of the venous pressure gradient, reduction in ICP, and functional, neurological, and ophthalmological improvement. As patients are at risk for stent-adjacent stenosis, further follow-up is necessary to determine long-term outcomes and gain an understanding of venous sinus stenosis as a primary or secondary pathological process behind elevated ICP 9).

El Mekabaty et al., retrospectively reviewed a prospectively maintained database spanning December 2011 to May 2015 of all patients with idiopathic intracranial hypertension who were screened for possible venous sinus stenting, including only patients who received a stent, noting symptomatic improvements, changes in opening lumbar puncture pressure, demographic characteristics, and any subsequent intervention after stent placement. Fisher’s exact test and logistic regression were used to test each of seven potential predictors for retreatment. RESULTS: There were eight revisions in 31 patients (25.8%). Among Caucasians, 8.0% required a revision compared with 100% of African-Americans (p<0.001). The c-index for race was 0.857. Body mass index (BMI) was also a significant predictor of revision (p=0.031): among class III obese patients the revision rate was 46.2% compared with 16.7% among class I and II obese patients and 0% among overweight to normal weight patients.

BMI was a significant predictor of revision, suggesting that higher BMI may have a higher risk of revision. The small number of African-Americans in the study makes interpretation of the practical significance of the revision rate in these patients uncertain. None of the other studied factors was statistically significant. 10).

A written informed consent approved by the Weill Cornell institutional review board was signed and obtained from the study participants. Thirty-seven consecutive patients with IIH and venous sinus stenosis who were treated with venous sinus stenting between Jan.2012-Jan.2016 were prospectively evaluated. Patients without pulsatile tinnitus were excluded. Tinnitus severity was categorized based on “Tinnitus Handicap Inventory” (THI) at pre-stent, day-0, 1-month, 3-month, 6-month, 12-month, 18-month and 2-year follow-up. Demographics, body-mass index (BMI), pre and post VSS trans-stenotic pressure gradient were documented. Statistical analysis performed using Pearson’s correlation, Chi-square analysis and Fischer’s exact test.

29 patients with a mean age of 29.5±8.5 years M:F = 1:28. Median (mean) THI pre and post stenting were: 4 (3.7) and 1 (1) respectively. Median time of tinnitus resolution post VSS was 0-days. There was significant improvement of THI (Δ Mean: 2.7 THI [95% CI: 2.3-3.1 THI], p<0.001) and transverse-distal sigmoid sinus gradient (Δ Mean: -15.3 mm Hg [95% CI: 12.7-18 mm Hg], p<0.001) post-stenting. Mean follow-up duration of 26.4±9.8 months (3-44 months). VSS was feasible in 100% patients with no procedural complications. Three-patients (10%) had recurrent sinus stenosis and tinnitus at mean follow-up of 12 months (6-30 months).

Venous sinus stenting is an effective treatment for pulsatile tinnitus in patients with IIH and venous sinus stenosis 11).


Fields et al reviewed all cases of dural stents for IIH. Eligibility criteria included medically refractory IIH with documented papilledema and dural venous sinus stenosis of the dominant venous outflow system (gradient ≥10 mm Hg).

Fifteen cases (all women) of mean age 34 years were identified. All had failed medical therapy and six had failed surgical intervention. Technical success was achieved in all patients without major periprocedural complications. The mean preprocedural gradient across the venous stenosis was reduced from 24 mm Hg before the procedure to 4 mm Hg after the procedure. Headache resolved or improved in 10 patients. Papilledema resolved in all patients and visual acuity stabilized or improved in 14 patients. There were no instances of restenosis among the 14 patients with follow-up imaging.

In this small case series, dural sinus stenting for IIH was performed safely with a high degree of technical success and with excellent clinical outcomes. These results suggest that angioplasty and stenting for the treatment of medically refractory IIH in patients with dural sinus stenosis warrants further investigation as an alternative to LPS, VPS and ONSF 12).

1) , 4) Kalyvas AV, Hughes M, Koutsarnakis C, Moris D, Liakos F, Sakas DE, Stranjalis G, Fouyas I. Efficacy, complications and cost of surgical interventions for idiopathic intracranial hypertension: a systematic review of the literature. Acta Neurochir (Wien). 2017 Jan;159(1):33-49. doi: 10.1007/s00701-016-3010-2. Review. PubMed PMID: 27830325.
2) Dinkin MJ, Patsalides A. Venous Sinus Stenting for Idiopathic Intracranial Hypertension: Where Are We Now? Neurol Clin. 2017 Feb;35(1):59-81. doi: 10.1016/j.ncl.2016.08.006. Review. PubMed PMID: 27886896.
3) Chaudhry S, Bryant TK, Peeler CE. Venous sinus stenting in idiopathic intracranial hypertension: a safer surgical approach? Curr Opin Ophthalmol. 2016 Nov;27(6):481-485. Review. PubMed PMID: 27585210.
5) Kanagalingam S, Subramanian PS. Cerebral venous sinus stenting for pseudotumor cerebri: A review. Saudi J Ophthalmol. 2015 Jan-Mar;29(1):3-8. doi: 10.1016/j.sjopt.2014.09.007. Review. PubMed PMID: 25859134; PubMed Central PMCID: PMC4314570.
6) Puffer RC, Mustafa W, Lanzino G. Venous sinus stenting for idiopathic intracranial hypertension: a review of the literature. J Neurointerv Surg. 2013 Sep 1;5(5):483-6. doi: 10.1136/neurintsurg-2012-010468. Review. PubMed PMID: 22863980.
7) Teleb MS, Cziep ME, Lazzaro MA, Gheith A, Asif K, Remler B, Zaidat OO. Idiopathic Intracranial Hypertension. A Systematic Analysis of Transverse Sinus Stenting. Interv Neurol. 2013;2(3):132-143. PubMed PMID: 24999351; PubMed Central PMCID: PMC4080637.
8) Smith KA, Peterson JC, Arnold PM, Camarata PJ, Whittaker TJ, Abraham MG. A case series of dural venous sinus stenting in idiopathic intracranial hypertension: association of outcomes with optical coherence tomography. Int J Neurosci. 2017 Feb;127(2):145-153. PubMed PMID: 26863329.
9) Liu KC, Starke RM, Durst CR, Wang TR, Ding D, Crowley RW, Newman SA. Venous sinus stenting for reduction of intracranial pressure in IIH: a prospective pilot study. J Neurosurg. 2016 Dec 23:1-8. doi: 10.3171/2016.8.JNS16879. [Epub ahead of print] PubMed PMID: 28009240.
10) El Mekabaty A, Obuchowski NA, Luciano MG, John S, Chung CY, Moghekar A, Jones S, Hui FK. Predictors for venous sinus stent retreatment in patients with idiopathic intracranial hypertension. J Neurointerv Surg. 2016 Dec 13. pii: neurintsurg-2016-012803. doi: 10.1136/neurintsurg-2016-012803. [Epub ahead of print] PubMed PMID: 27965382.
11) Boddu S, Dinkin M, Suurna M, Hannsgen K, Bui X, Patsalides A. Resolution of Pulsatile Tinnitus after Venous Sinus Stenting in Patients with Idiopathic Intracranial Hypertension. PLoS One. 2016 Oct 21;11(10):e0164466. doi: 10.1371/journal.pone.0164466. PubMed PMID: 27768690; PubMed Central PMCID: PMC5074492.
12) Fields JD, Javedani PP, Falardeau J, Nesbit GM, Dogan A, Helseth EK, Liu KC, Barnwell SL, Petersen BD. Dural venous sinus angioplasty and stenting for the treatment of idiopathic intracranial hypertension. J Neurointerv Surg. 2013 Jan 1;5(1):62-8. doi: 10.1136/neurintsurg-2011-010156. PubMed PMID: 22146571.

Update: Intraventricular cavernous malformation


Intraventricular cavernomas (IVCs) are rare, and only occur in 2.8 to 10% of patients with cerebral cavernous malformations.

Reports concerning IVC are scarce and are limited mostly to sporadic case reports.

Till 2017, among 136 IVC cases, the mean age of the patients was 36.5 years; the male-to-female ratio was 0.8. The most frequent location was the lateral ventricle (52.6%), and most of the clinical symptoms (74%) were related to mass effects on adjacent brain tissues. Intraventricular hemorrhage occurred in 22.9% of cases 1).

Till 2003, only 10 pediatric cases out of 46 well-documented cases have been published 2).


In a review of literature by Reyns et al., of the 45 cases reported in literature, 44% of intraventricular cavernomas were found to be located in the third ventricle, 27% in the lateral ventricle, 20% in the trigone, and 9% in the fourth ventricle 3).

Third ventricle cavernous malformation

Foramen of Monro

Trigone cavernous malformation

Of intraventricular CM, only about 20% are located in the trigone of the lateral ventricle 4).

The first report of trigonal CM was published in 1977 by Coin 5).

Ohbuchi et al., conducted a PubMed search for trigonal CM and found 17 cases 6).

Clinical features

Although IVCs share some common characteristics with intraparenchymal cavernomas, they also have some distinct features involving structure, clinical symptoms, radiologic appearance, and onset of symptoms 7).

Differential diagnosis

The important differentials of the lesions occurring at the foramen of Monro include colloid cyst, central neurocytoma, giant cell astrocytoma, and oligodendroglioma. Cystic and hemorrhagic metastases, however uncommon, may also occur at the foramen of Monro 8).

Colloid cysts are oval-to-round lesions. Approximately two thirds are homogeneously hyperdense compared to brain on plain scans and one-third of them are isodense. On MRI, the signal characteristics of colloid cysts vary. Most commonly, these appear hyperintense on T1WI and hypointense on T2WI. Central neurocytomas are inhomogeneous, partially calcified, and mildly enhancing lateral ventricular masses. On MRI, these are seen to be inhomogeneously isointense on T1WI with variable appearance on T2WI. Giant cell astrocytomas are seen in association with tuberous sclerosis and appear iso-to- hypodense on CT scans with focal areas of calcification and cyst formation. These demonstrate hypo- to isointense signal on T1WI and are iso- to hyperintense on T2WI. Oligodendrogliomas most commonly show dense areas of calcification. These appear hypo- to isointense on T1WI and hyperintense on T2WI, showing moderate patchy enhancement 9).

Jin et al., report a case of trigonal cavernous malformation (CM) radiologically mimicking meningioma. The computed tomographic (CT) head angiography and magnetic resonance imaging (MRI) showed a partially calcified lesion with slight contrast enhancement located in the area of the left atrium of lateral ventricle. The lesion was completely removed using microsurgery with a parieto-occipital transcortical approach. The resected mass was histologically confirmed as CM. CM should be considered as differential diagnosis in case of the atrial mass lesion due to lack of hemosiderin ring characteristically seen other seated CM 10).

Dey et al., present the first report of an intraventricular encapsulated hematoma, mimicking cavernous malformation on imaging and gross lesion appearance. A 47-year-old female on anticoagulation therapy for atrial fibrillation presented with left upper extremity apraxia, neglect, and mild gait imbalance. Her brain CT scan and MRI revealed multilobulated lesions involving the choroid plexus in the atria of both lateral ventricles. The intraoperative appearance was that of encapsulated mass, with blood clots at different stages of liquefaction and organization, all consistent with the gross appearance of a cavernous malformation. However, histopathologic examination demonstrated hematoma with components at different ages, and normal vessel infiltration without any hint of cavernous malformation histology, or underlying neoplasia. Encapsulated hematoma should be considered in the differential diagnosis of hemorrhagic intraventricular masses 11).


Most of the articles concluded that complete surgical resection is the treatment of choice.The microsurgical approach is currently considered the gold standard for IVC resection 12).


As neuroendoscopy evolves, we see that lesions considered impossible to be managed by neuroendoscopy before today are being treated with the advantages that the minimally invasive surgery offers 13).

However, tumor size and vascular nature are considered restrictive factors.

Till 2013, there are two cases in the literature of intraventricular cavernoma resection done by neuroendoscopy.

Giannetti describe a case of a man who presented with hydrocephalus secondary to a mesencephalic cavernoma and a second cavernous angioma located at the dorsum thalamus. This second lesion was successfully removed using a pure neuroendoscopic technique at the same time as a third ventriculostomy was performed 14).

Using the neuroendoscope and neuronavigational guidance and based on the biological characteristics of the IVC, proper lesion size, and dilated ventricles, Shirvani et al., totally resected the lesion in all his three cases. Neuroendoscopy can be considered as an alternative to microsurgery of IVCs. However, Shirvani et al., believe a larger series of cases is necessary to demonstrate when microsurgery and when neuroendoscopy should be performed for IVC resection 15).



Stavrinou et al., performed a search of the literature of the last 30 years and identified all cases of intraventricular cavernous angiomas. Trigonal cavernomas were separately identified and analysed. Our search yielded a total of 13 trigonal cavernomas.

A total of 61 intraventricular cases, 13 were located in the trigone of the lateral ventricles. The most prominent presenting symptom was intracranial hypertension (68.9%), followed by seizures (18.2%) and hemorrhage (13.1%).The literature review revealed a trend of intraventricular cavernomas to present with intracranial hypertension rather than seizures or focal neurologic deficit, unlike their intraparenchymal counterparts. We feel that this difference has received little attention in the international literature. We discuss a possible pathogenetic mechanism for the presence of intracranial hypertension and address different aspects of diagnosis and treatment of this benign lesion.

Trigonal cavernomas are benign lesions that have an excellent outcome after radical excision. Symptoms and signs of intracranial hypertension and hydrocephalus may be the prominent initial presentation of this rare ailment. 16).

Case series


Faropoulos et al., present there experience, consisting of five IVC cases over a period of 11 years. They describe the symptoms leading to hospital admission, the main radiologic findings, the management of each ICV case, and the patients’ clinical status after surgery. They also reviewed the international literature on IVC, presenting the main demographic characteristics, their most common location in the ventricular system, and the main signs and symptoms. Finally, They present the management options according to the current literature, the advantages and disadvantages of every management option, accompanied by a brief follow-up of most IVC cases, whether the cavernoma was treated surgically or conservatively 17).


Kivelev et al., present a series of 12 patients with IVCs that were treated at a single neurosurgical department. In addition, the authors reviewed the literature.

All clinical data were analyzed retrospectively. Follow-up questionnaires were sent to all patients. Outcome was assessed using the Glasgow Outcome Scale. The authors also conducted a PubMed search and found 77 cases of IVC.

The patients’ median age was 47 years, and the male/female ratio was 2:1. A cavernoma occurred in the lateral ventricle in 6 patients, in another 5 it was in the fourth ventricle, and 1 had a lesion in the third ventricle. Almost all patients presented with acute headache on admission and in more than half, the symptoms were related to cavernoma bleeding. In total, 8 rebleedings occurred in 5 patients during a median of 0.4 years. Three patients with a cavernoma of the fourth ventricle presented with a cranial nerve deficit. In 8 cases, a cavernoma was surgically treated an average of 1.3 years after the diagnosis. Only 1 patient underwent surgery in the acute phase after a major intraventricular/intracerebral hemorrhage. The median follow-up time was 2 years. No patient was lost to follow-up, and no patient died. In total, on follow-up 9 patients improved and 3 had a persistent neurological deficit, of which 2 existed before surgery.

In the present series, the IVCs had a high tendency for rehemorrhage. Surgery is advocated when hemorrhages are frequent, and the mass effect causes progressive neurological deficits. Microsurgical removal of the IVC is safe, but in the fourth ventricle it can carry increased risk for cranial nerve deficits 18).


The clinical and radiographic presentations of 3 patients with intraventricular cavernous hemangioma are described. The accumulated total of 19 cases from the literature are compared to determine whether there is a common clinical and radiographic presentation for this benign intraventricular lesion. The differential diagnosis of intraventricular cavernous hemangioma includes intraventricular meningioma, choroid plexus papilloma, arteriovenous malformation, low grade astrocytoma, and ependymoma 19).

Case reports


Shirvani et al., describe three IVC cases and briefly review previously documented IVC cases in PubMed. Among 136 IVC cases, the mean age of the patients was 36.5 years; the male-to-female ratio was 0.8. The most frequent location was the lateral ventricle (52.6%), and most of the clinical symptoms (74%) were related to mass effects on adjacent brain tissues. Intraventricular hemorrhage occurred in 22.9% of cases. Most of the articles concluded that complete surgical resection is the treatment of choice.The microsurgical approach is currently considered the gold standard for IVC resection. Using the neuroendoscope and neuronavigational guidance and based on the biological characteristics of the IVC, proper lesion size, and dilated ventricles, we totally resected the lesion in all three cases. Neuroendoscopy can be considered as an alternative to microsurgery of IVCs. However, we believe a larger series of cases is necessary to demonstrate when microsurgery and when neuroendoscopy should be performed for IVC resection 20).


A 64-year-old woman who was evaluated after being found unresponsive. Imaging revealed a foramen of Monro cavernoma resulting in hydrocephalus. Supratentorial cavernomas are most frequently found in the cerebral cortex, and although ventricular cavernomas do occur, they are rarely located in the foramen of Monro. Foramen of Monro cavernomas are extremely dangerous, requiring aggressive management when identified 21).


Bhatia et al., present a case of cavernous hemangioma located at foramen of Monro, with its radiopathological confirmation 22).


A case of trigonal cavernous malformation (CM) with intraventricular hemorrhage. This 67-year-old woman experienced sudden onset of loss of consciousness and her Glasgow Coma Scale (GCS) was 5 points (E1V1M3) on admission. CT scan demonstrated intraventricular hemorrhage and acute hydrocephalus. Angiography did not demonstrate any vascular abnormality. Ventricular drainage was performed for acute hydrocephalus and the postoperative course was good. CT showed a hyperdense lesion in the left trigone, which was contrast-enhanced on T1-weighted MR. Removal of CM was performed via the left middle temporal sulcus 23).


Intraventricular cavernous malformation with superficial siderosis 24).


A case of intraventricular cavernoma in the region of the foramen of Monro with the aim of illustrating the difficulties involved in the diagnosis of this rare lesion 25).


Jin et al., report a case of trigonal cavernous malformation (CM) radiologically mimicking meningioma. The computed tomographic (CT) head angiography and magnetic resonance imaging (MRI) showed a partially calcified lesion with slight contrast enhancement located in the area of the left atrium of lateral ventricle. The lesion was completely removed using microsurgery with a parieto-occipital transcortical approach. The resected mass was histologically confirmed as CM. CM should be considered as differential diagnosis in case of the atrial mass lesion due to lack of hemosiderin ring characteristically seen other seated CM 26).

Muccio et al., report the MRI findings and histological features of an uncommon case of a single giant (maximum diameter: >6 cm) cystic CCM of the left lateral ventricle occurring in a 26-year-old man who had undergone 30 Gy cranial irradiation for acute leukemia at the age of six years. Large cystic CCMs must be included in the neuroradiological differential diagnosis of intraventricular hemorrhagic cystic lesions 27).

A 56 years old patient was admitted with progressive and intractable headache of 10 days of evolution. He was known to suffer familial multiple cavernomatosis. Magnetic resonance imaging (MRI), revealed obstructive hydrocephalus due to a cavernoma located in the area of the left foramen of Monro. Under neuronavigation guidance, complete endoscopic resection of the cavernoma was performed and normal ventricular size achieved. The patient experienced transient recent memory loss that resolved within a month after surgery. In the literature attempted endoscopic resection is reported to be abandoned due to bleeding and ineffectiveness of piecemeal endoscopic resection. In this case, the multiplicity of the lesions made it advisable to resect the lesion endoscopically, to avoid an open procedure in a patient with multiple potentially surgical lesions. Endoscopic resection was uneventful with easy control of bleeding with irrigation, suction, and bipolar coagulation despite dense vascular appearance of the lesion. During the procedure, precise visualization of the vascular structures around the foramen of Monro allowed complete resection with satisfactory control of the instruments. To the best of the authors’ knowledge, this is the first published cavernoma of foramen of Monro successfully resected using an endoscopic approach 28).


A 25-year-old male patient presented with a predominantly intralesional haemorrhage. Neuroimaging led to an accurate preoperative diagnosis although the typical low intensity perilesional ring of gliosis and hemosiderin was not present. The lesion was microsurgically removed using an stereotactically guided posterior temporal transsulcal approach 29).


A eight-year old boy with a rare third ventricular cavernous angioma that hemorrhaged presenting with symptoms of acute hydrocephalus. Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) showed a heterogenous ill-defined, solid and cystic intraventricular mass in the third ventricle which was mildly enhanced with contrast and there was associated hydrocephalus. The mass was removed with success and follow up after two years revealed no neurological abnormalities 30).

Longatti et al., report on a patient who presented with an intraventricular mass located at the level of the foramen of Monro. The clinical presentation and neuroimaging appearance of the mass led to an initial diagnosis of colloid cyst. A neuroendoscopic approach offered a direct view of the ventricular lesion, which was found to be a cavernous angioma partially occluding the foramen of Monro. The lesion was then removed using microsurgery. In this report the authors highlight possible pitfalls in the diagnosis of some lesions of the third ventricle, and the possible advantages of using a combined endoscopic and microsurgical technique when approaching such lesions 31).

Three cases of trigonal cavernous angiomas who presented with raised intracranial pressure or seizures and who underwent total excision with a good recovery. We also review the literature and discuss surgical approaches.

On magnetic resonance imaging, intraventricular cavernous angiomas lack the hemosiderin ring characteristically seen around parenchymal cavernous angiomas. This explains why trigonal cavernous angiomas can mimic malignant neoplasm on imaging, and they should be considered in the differential diagnosis of intraventricular masses. Total excision should be the goal of surgery 32).

A 47-year-old woman presented with unilateral ventricular enlargement detected by magnetic resonance imaging during a medical checkup. Neuroendoscopic exploration identified a multilocular lesion in which dark red fluid formed a niveau near the right side of the foramen of Monro. The diagnosis was intraventricular cavernous angioma. Restricted flow of cerebrospinal fluid at the foramen of Monro was observed. Xanthochromia, which seemed to be due to previous bleeding, was observed at the fornix. When the neuroendoscope touched the angioma, the wall collapsed and bled. Endoscopic removal of the angioma was abandoned, and craniotomy and resection of the angioma were performed. No new neurological anomalies were observed after surgery. Preoperative diagnosis of intraventricular cavernous angioma is difficult based on neuroimaging. Neuroendoscopy is effective for diagnosis and the decision-making process regarding treatment 33).

A 51-year-old woman was admitted with obstructive hydrocephalus-related symptoms. The computed tomography (CT) and magnetic resonance imaging (MRI) revealed a partly calcified lesion with slight contrast enhancement located in the area of the right foramen of Monro. The lesion was completely removed by surgical resection with a transfrontal transventricular approach. The resected mass was histologically diagnosed as cavernous hemangioma. The patient’s symptoms resolved immediately after operation. Cavernous hemangioma at the foramen of Monro in the present case had common MRI features as previously reported. Although MRI can provide initial diagnosis for such unusually localized tumor, it should be confirmed histopathologically 34).


Tatsui et al., present two cases with diagnosis made only by histopathologic examination, due to a lack of classic image findings. Cavernous hemangiomas must be included in the differential diagnosis of intraventricular tumors, and total surgical resection is the treatment of choice. Perilesional ring as demonstrated by MRI, must not be expected when dealing with such lesions 35).

A 11-year-old girl operated on for a voluminous cavernoma at the ventricular trigone which was diagnosed after absence seizures.

Interestingly, the case we report showed a hypointense rim on T2-weighted magnetic resonance images which has not been a common finding in the cases previously reported 36).

A clinicopathological review of a giant intraventricular cavernous malformation 37).


Intraventricular cavernoma in the region of the foramen of monro 38).


A 15-years-old female patient, who suffered of mild chronic headache for 8 months, followed by two episodes of sudden intensive headache and stupor with complete recovery after 48 hours. CT scan was performed and revealed a voluminous size, hiperdense mass in the frontal horn of the lateral ventricle. The surgical access to site was through transcallosal interhemisphere approach. The patient had a good recovery without complications.

Although lateral ventricle cavernomas are rare they should be considered in the differential diagnosis of intraventricular tumors. A wrong preoperative diagnosis has sometimes induced a wrong therapy, such as radiotherapy, for these surgically curable benign 39).


A 16-year-old female patient presented with a sudden distal deficit of the left superior limb. She had a voluminous tumor involving the two lateral ventricles, with radiological evidence of recent hemorrhage. A 30-year-old man presented with generalized seizures and a right hemiplegia related to a 4-cm-diameter cavernoma in the two lateral ventricles involving the interhemispheric scissure through the corpus callosum and left centrum ovale. The radiological appearance was not typical and did not allow the diagnosis. A 42-year-old man had a cavernoma in the third ventricle, which was responsible for his short-term memory loss. This cavernoma had been revealed by computed tomography that was performed after intracerebral hemorrhage related to another cavernoma in the right parietal lobe occurred.

Stereotactic biopsies allowed the diagnosis of intraventricular cavernoma in the first case. Surgical removal via a right transcortical transventricular approach and a transcallosal approach in the first and second cases, respectively, was complete, resulting in good outcomes. Surgical removal via a right transcortical transventricular approach in the third case was partial.

Intraventricular cavernomas are so uncommon that only 42 well-documented cases have been previously reported in the literature. It seems that their radiological diagnosis may be difficult because of their uncommon location in the ventricular system and their voluminous size. A wrong preoperative diagnosis has sometimes been the cause of inefficient therapy, such as radiotherapy, for these surgically curable benign lesions 40).


A case of a cavernous haemangioma that appeared as an intraventricular mass at the foramen of Monro. Despite the unusual location the diagnosis was established by MRI because of the typical appearance. The differential diagnosis included primary and secondary neoplasms at the foramen of Monro 41).


Four cases of cavernous malformations of the third ventricle. Patients presented with symptoms of hydrocephalus, memory loss, and signs of hypothalamic dysfunction. Magnetic resonance imaging and computed tomography provided characteristic images of the three lesions preoperatively. All patients underwent direct surgical excision of the malformations. Two patients had a transcallosal, transventricular approach, the third underwent a transcortical, transventricular approach, and the fourth had an infratentorial supracerebellar approach. Postoperatively, the patient with hypothalamic dysfunction has not improved and underwent ventriculoperitoneal shunting. The second patient did well initially; however, 8 days postoperatively, she became comatose and later died. The presumed cause of her deterioration was a hypothalamic venous infarction. The third and fourth patients have returned to their normal neurological baseline. The presenting signs and symptoms, magnetic resonance imaging and computed tomography findings, and treatment options for this rare lesion are discussed and illustrated 42).


A 54-year-old female presented with a cryptic arteriovenous malformation (AVM) of the choroid plexus of the fourth ventricle causing intraventricular hemorrhage. Computed tomography and magnetic resonance imaging disclosed the lesion near the fourth ventricle, but bilateral vertebral angiograms showed no abnormalities. The preoperative diagnosis was cavernous angioma. The mass was removed completely, and histological examination demonstrated an AVM of the choroid plexus. Vascular malformations of the choroid plexus of the fourth ventricle are extremely rare. The possibility of this lesion being the cause of primary intraventricular hemorrhage of unknown origin should always be considered 43).


A case located in the trigone of the lateral ventricle in the dominant hemisphere and drained into the longitudinal caudate vein of Schlesinger via deep medullary veins. By a transsylvian transventricular approach, the CM was totally removed with successful preservation of the medullary venous malformation. This approach is available for trigonal lesions, especially in cases with enlarged inferior horn. They stress that CM removal can be conducted with preservation of the adjacent medullary venous malformation 44).


Since the availability of CT diagnosis 23 cases of intraventricular cavernous angioma (IVCA) have been published in the literature till 1991. Three additional cases have been operated upon in the Neurochirurgische Klinik, Krankenhaus Nordstadt, Hannover, Federal Republic of Germany. Based on these 26 cases the clinical data, radiological findings, treatment and outcome of IVCAs are reviewed 45).

A rare case of cavernous angioma located in the fourth ventricular floor occurred in a 44-year-old female complaining of occipital headache, vomiting, diplopia, and dysarthria. Computed tomographic scans demonstrated a high-density area in the fourth ventricle and slight hydrocephalus. Magnetic resonance (MR) imaging showed a mixed intensity mass on T2-weighted images and high- or isointensity regions on T1-weighted images. The tumor was totally removed and histologically diagnosed as cavernous angioma. Postoperatively, ataxic gait, nausea, and vomiting disappeared gradually. MR imaging was useful to accurately evaluate the anatomic relationship between the lesion and the brainstem 46).


Cavernous angioma arising in the third ventricle is an extremely rare disease. Ogawa et al., reviewed five cases previously reported, as well as they own two cases, and discuss the clinical characteristics of and surgical approach to cavernous angioma at this site. The bifrontal craniotomy and interhemispheric translamina terminalis approach minimize the damage to the brain and allow for an approach to the third ventricle in a wide operative field with minimal compression of the brain itself. They have found this approach to be suitable for surgery on angiomas of the anterior half of the third ventricle 47).

1) , 15) , 20) Shirvani M, Hajimirzabeigi A. Intraventricular Cavernous Malformation: Review of the Literature and Report of Three Cases with Neuroendoscopic Resection. J Neurol Surg A Cent Eur Neurosurg. 2017 Jan 9. doi: 10.1055/s-0036-1594235. [Epub ahead of print] PubMed PMID: 28068754.
2) , 36) Nieto J, Hinojosa J, Muñoz MJ, Esparza J, Ricoy R. Intraventricular cavernoma in pediatric age. Childs Nerv Syst. 2003 Jan;19(1):60-2. PubMed PMID: 12541090.
3) , 4) , 40) Reyns N, Assaker R, Louis E, Lejeune JP. Intraventricular cavernomas: three cases and review of the literature. Neurosurgery. 1999 Mar;44(3):648-54; discussion 654-5. Review. PubMed PMID: 10069603.
5) Coin CG, Coin JW, Glover MB. Vascular tumors of the choroid plexus: diagnosis by computed tomography. J Comput Assist Tomogr. 1977 Jan;1(1):146-8. PubMed PMID: 615888.
6) , 23) Ohbuchi H, Osaka Y, Ogawa T, Nanto M, Nakahara Y, Katsura K, Tenjin H, Kasuya H. Trigonal cavernous malformation with intraventricular hemorrhage: a case report and literature review. J Med Invest. 2012;59(3-4):275-9. Review. PubMed PMID: 23037200.
7) , 17) Faropoulos K, Panagiotopoulos V, Partheni M, Tzortzidis F, Konstantinou D. Therapeutic management of intraventricular cavernoma: case series and review of the literature. J Neurol Surg A Cent Eur Neurosurg. 2015 May;76(3):233-9. doi: 10.1055/s-0034-1389093. Review. PubMed PMID: 25798802.
8) Lee BJ, Choi CY, Lee CH. Intraventricular cavernous hemangiomas located at the foramen of monro. J Korean Neurosurg Soc. 2012 Aug;52(2):144-7. doi: 10.3340/jkns.2012.52.2.144. Erratum in: J Korean Neurosurg Soc. 2012 Nov;52(5):505. PubMed PMID: 23091674; PubMed Central PMCID: PMC3467373.
9) , 22) Bhatia S, Kapoor AK, Gupta R, Sahni T. Cavernous hemangioma located at the foramen of Monro: Radiopathological correlation. Indian J Radiol Imaging. 2013 Jul;23(3):202-4. doi: 10.4103/0971-3026.120259. PubMed PMID: 24347847; PubMed Central PMCID: PMC3843325.
10) , 26) Jin SC, Ahn JS, Kwun BD, Kwon DH. Intraventricular cavernous malformation radiologically mimicking meningioma. J Korean Neurosurg Soc. 2008 Nov;44(5):345-7. doi: 10.3340/jkns.2008.44.5.345. PubMed PMID: 19119474; PubMed Central PMCID: PMC2612575.
11) Dey M, Turner MS, Pytel P, Awad IA. A “pseudo-cavernoma” – an encapsulated hematoma of the choroid plexus. J Clin Neurosci. 2011 Jun;18(6):846-8. doi: 10.1016/j.jocn.2010.09.018. PubMed PMID: 21435884; PubMed Central PMCID: PMC3094469.
12) Alves de Sousa A. [Deep-seated (corpus callosum, intraventricular, basal ganglia and insula) and brain stem cavernous angiomas. Experience in Brazil]. Neurochirurgie. 2007 Jun;53(2-3 Pt 2):182-91. Review. French. PubMed PMID: 17507054.
13) , 14) Giannetti AV. Purely neuroendoscopic resection of an intraventricular cavernous angioma: case report. J Neurol Surg A Cent Eur Neurosurg. 2013 Jan;74(1):47-50. doi: 10.1055/s-0032-1325632. PubMed PMID: 23027434.
16) Stavrinou LC, Stranjalis G, Flaskas T, Sakas DE. Trigonal cavernous angioma: a short illustrated review. Acta Neurochir (Wien). 2009 Nov;151(11):1517-20. doi: 10.1007/s00701-009-0252-2. Review. PubMed PMID: 19300903.
18) Kivelev J, Niemelä M, Kivisaari R, Hernesniemi J. Intraventricular cerebral cavernomas: a series of 12 patients and review of the literature. J Neurosurg. 2010 Jan;112(1):140-9. doi: 10.3171/2009.3.JNS081693. Review. PubMed PMID: 19408982.
19) Chadduck WM, Binet EF, Farrell FW Jr, Araoz CA, Reding DL. Intraventricular cavernous hemangioma: report of three cases and review of the literature. Neurosurgery. 1985 Feb;16(2):189-97. PubMed PMID: 3974830.
21) Winslow N, Abode-Iyamah K, Flouty O, Park B, Kirby P, Howard M 3rd. Intraventricular foramen of Monro cavernous malformation. J Clin Neurosci. 2015 Oct;22(10):1690-3. doi: 10.1016/j.jocn.2015.03.043. PubMed PMID: 26113004.
24) Sabat SB. Intraventricular cavernous malformation with superficial siderosis. Arch Neurol. 2010 May;67(5):638-9. doi: 10.1001/archneurol.2010.53. PubMed PMID: 20457968.
25) Meilán Martínez A, Vega Valdés P, Santamarta Liébana E, Rial Basalo JC. [Intraventricular cavernoma in the foramen of Monro: particularities due to its atypical location]. Radiologia. 2009 Nov-Dec;51(6):605-9. doi: 10.1016/j.rx.2009.04.009. Spanish. PubMed PMID: 19646725.
27) Muccio CF, Catapano G, Di Blasi A, Esposito G, Cerase A. Giant cystic intraventricular cerebral cavernous malformation: MRI with pathologic correlation. A case report. Neuroradiol J. 2008 Oct 1;21(4):547-50. PubMed PMID: 24256962.
28) Prat R, Galeano I. Endoscopic resection of cavernoma of foramen of Monro in a patient with familial multiple cavernomatosis. Clin Neurol Neurosurg. 2008 Sep;110(8):834-7. doi: 10.1016/j.clineuro.2008.05.011. PubMed PMID: 18584950.
29) González-Darder JM, Pesudo-Martínez JV, Merino-Peña J. [Trigonal cavernous angioma: case report]. Neurocirugia (Astur). 2007 Aug;18(4):330-2. Spanish. PubMed PMID: 17882341.
30) Zakaria MA, Abdullah JM, George JP, Mutum SS, Lee NN. Third ventricular cavernous angioma. Med J Malaysia. 2006 Jun;61(2):229-32. PubMed PMID: 16898318.
31) Longatti P, Fiorindi A, Perin A, Baratto V, Martinuzzi A. Cavernoma of the foramen of Monro. Case report and review of the literature. Neurosurg Focus. 2006 Jul 15;21(1):e13. PubMed PMID: 16859251.
32) Kumar GS, Poonnoose SI, Chacko AG, Rajshekhar V. Trigonal cavernous angiomas: report of three cases and review of literature. Surg Neurol. 2006 Apr;65(4):367-71, discussion 371. PubMed PMID: 16531197.
33) Sato K, Oka H, Utsuki S, Shimizu S, Suzuki S, Fujii K. Neuroendoscopic appearance of an intraventricular cavernous angioma blocking the foramen of monro – case report. Neurol Med Chir (Tokyo). 2006 Nov;46(11):548-51. PubMed PMID: 17124371.
34) Chen CL, Leu CH, Jan YJ, Shen CC. Intraventricular cavernous hemangioma at the foramen of Monro: Case report and literature review. Clin Neurol Neurosurg. 2006 Sep;108(6):604-9. Review. PubMed PMID: 15916846.
35) Tatsui CE, Koerbel A, Prevedello DM, Hanel RA, Grande CV, Moro MS, Araújo JC. [Magnetic resonance imaging of the intraventricular cavernomas: diagnostic aspects]. Arq Neuropsiquiatr. 2003 Mar;61(1):79-82. Portuguese. PubMed PMID: 12715024.
37) Anderson RC, Connolly ES Jr, Ozduman K, Laurans MS, Gunel M, Khandji A, Faust PL, Sisti MB. Clinicopathological review: giant intraventricular cavernous malformation. Neurosurgery. 2003 Aug;53(2):374-8; discussion 378-9. PubMed PMID: 12925254.
38) Suess O, Hammersen S, Brock M. Intraventricular cavernoma: unusual occurrence in the region of the foramen of monro. Br J Neurosurg. 2002 Feb;16(1):78-9. PubMed PMID: 11926476.
39) Fagundes-Pereyra WJ, Marques JA, Sousa LD, Carvalho GT, Sousa AA. [Cavernoma of the lateral ventricle: case report]. Arq Neuropsiquiatr. 2000 Sep;58(3B):958-64. Review. Portuguese. PubMed PMID: 11018841.
41) Kaim A, Kirsch E, Tolnay M, Steinbrich W, Radü EW. Foramen of Monro mass: MRI appearances permit diagnosis of cavernous haemangioma. Neuroradiology. 1997 Apr;39(4):265-9. PubMed PMID: 9144673.
42) Sinson G, Zager EL, Grossman RI, Gennarelli TA, Flamm ES. Cavernous malformations of the third ventricle. Neurosurgery. 1995 Jul;37(1):37-42. Review. PubMed PMID: 8587688.
43) Tamaki M, Ohno K, Asano T, Niimi Y, Uchihara T, Hirakawa K. Cryptic arteriovenous malformation of the choroid plexus of the fourth ventricle–case report. Neurol Med Chir (Tokyo). 1994 Jan;34(1):38-43. Review. PubMed PMID: 7514753.
44) Miyagi Y, Mannoji H, Akaboshi K, Morioka T, Fukui M. Intraventricular cavernous malformation associated with medullary venous malformation. Neurosurgery. 1993 Mar;32(3):461-4; discussion 464. PubMed PMID: 8455774.
45) Tatagiba M, Schönmayr R, Samii M. Intraventricular cavernous angioma. A survey. Acta Neurochir (Wien). 1991;110(3-4):140-5. Review. PubMed PMID: 1927606.
46) Itoh J, Usui K. Cavernous angioma in the fourth ventricular floor–case report. Neurol Med Chir (Tokyo). 1991 Feb;31(2):100-3. PubMed PMID: 1715036.
47) Ogawa A, Katakura R, Yoshimoto T. Third ventricle cavernous angioma: report of two cases. Surg Neurol. 1990 Dec;34(6):414-20. Review. PubMed PMID: 2244306.

Update: Vernet’s syndrome

In contrast to the majority of classic brainstem syndromes, the interpretation of Schmidt’s syndrome (ipsilateral palsy of the IX, X, XI, and XII cranial nerves with contralateral hemiparesis) and Vernet’s syndrome (ipsilateral palsy of the IX, X, and XI nerves with contralateral hemiparesis) is controversial. They are sometimes addressed as crossed brainstem syndromes but also as syndromes due to multiple cranial nerve lesions without contralateral hemiparesis. In this study, the historic descriptions and recent publications about Schmidt’s and Vernet’s syndromes were reviewed and critically analysed. We conclude that historic descriptions and later publications describe exclusively patients with extracerebral lesions of multiple cranial nerves. “Central” syndromes of Schmidt and Vernet caused by brainstem lesion appear not to exist. An extremely extensive lesion explaining these hypothetical unilateral brainstem syndromes is theoretically possible but, however, was apparently never observed in any of the known unilateral brainstem diseases 1).


Symptoms of this syndrome are consequences of this paresis. As such, in an affected patient, you may find:


soft palate dropping

deviation of the uvula towards the normal side


loss of sensory function from the posterior 1/3 of the tongue

decrease in the parotid gland secretion

loss of gag reflex

sternocleidomastoid and trapezius muscles paresis.


A variety of neoplasms, vascular insults, infections, and trauma have been reported to cause JFS 2).

The causes of Vernet syndrome are primary tumors such as Glomus jugulare tumors (most frequently), meningioma, vestibular schwannoma, cerebellopontine angle metastases, inflammation such as meningitis and malignant otitis externa, and sarcoidosis, Guillain-Barre syndrome 3).

Trauma 4) 5).

Cholesteatoma (very rare) 6).

Obstruction of the jugular foramen due to bone diseases 7).

Varicella-zoster virus 8).

Giant cell arteritis 9) 10).

Internal jugular vein thrombosis 11).

After carotid endarterectomy 12).

Large mycotic aneurysm of the extracranial internal carotid artery after acute otitis media 13).

Systemic erythematous lupus 14).

1) Krasnianski M, Neudecker S, Zierz S. [The Schmidt and Vernet classical syndrome. Alternating brain stem syndromes that do not exist?]. Nervenarzt. 2003 Dec;74(12):1150-4. Review. German. PubMed PMID: 14647918.
2) Robbins KT, Fenton RS. Jugular foramen syndrome. J Otolaryngol. 1980 Dec;9(6):505-16. PubMed PMID: 7206037.
3) Ha SW, Kim JK, Kang SJ, Kim MJ, Yoo BG, Kim KS, et al. A case of Vernet’s syndrome caused by non-specific focal inflammation of the neck. J Korean Soc Clin Neurophysiol. 2007;9:81–84.
4) , 5) Kim HS, Ko K. Penetrating trauma of the posterior fossa resulting in Vernet’s syndrome and internuclear ophthalmoplegia. J Trauma. 1996 Apr;40(4):647-9. PubMed PMID: 8614050.
6) Erol FS, Kaplan M, Kavakli A, Ozveren MF. Jugular foramen syndrome caused by choleastatoma. Clin Neurol Neurosurg. 2005 Jun;107(4):342-6. PubMed PMID: 15885397.
7) Erol FS, Kaplan M, Kavakli A, Ozveren MF.Jugular foramen syndrome caused by choleastatoma. Clin Neurol Neurosurg. 2005 Jun;107(4):342-6.
8) Jo YR, Chung CW, Lee JS, Park HJ. Vernet syndrome by varicella-zoster virus. Ann Rehabil Med. 2013 Jun;37(3):449-52. doi: 10.5535/arm.2013.37.3.449. PubMed PMID: 23869347; PubMed Central PMCID: PMC3713306.
9) Jeret JS. Giant cell arteritis and Vernet’s syndrome. Neurology. 1999 Feb;52(3):677. PubMed PMID: 10025824.
10) Cherin P, De Gennes C, Bletry O, Lamas A, Launay M, Dubs A, Godeau P. Ischemic Vernet’s syndrome in giant cell arteritis: first two cases. Am J Med. 1992 Sep;93(3):349-52. PubMed PMID: 1524092.
11) Shima K, Iwasa K, Yoshita M, Yamada M. Vernet’s syndrome induced by internal jugular vein thrombosis. J Neurol Neurosurg Psychiatry. 2016 Nov;87(11):1252-1253. doi: 10.1136/jnnp-2015-311665. PubMed PMID: 26354943.
12) Tamaki T, Node Y, Saitoum N, Saigusa H, Yamazaki M, Morita A. Vernet’s syndrome after carotid endarterectomy. Perspect Vasc Surg Endovasc Ther. 2013 Dec;25(3-4):65-8. doi: 10.1177/1531003514525476. PubMed PMID: 24625858.
13) Amano M, Ishikawa E, Kujiraoka Y, Watanabe S, Ashizawa K, Oguni E, Saito A, Nakai Y, Ikeda H, Abe T, Uekusa Y, Matsumura A. Vernet’s syndrome caused by large mycotic aneurysm of the extracranial internal carotid artery after acute otitis media–case report. Neurol Med Chir (Tokyo). 2010 Jan;50(1):45-8. PubMed PMID: 20098025.
14) Leache Pueyo JJ, Campos del Alamo MA, Gil Paraíso P, Ortiz García A. [Vernet’s syndrome as an early manifestation of systemic erythematous lupus]. An Otorrinolaringol Ibero Am. 1997;24(2):135-41. Spanish. PubMed PMID: 9199109.

Book: Pediatric Vascular Neurosurgery: Disorders and their Management

Pediatric Vascular Neurosurgery: Disorders and their Management

Pediatric Vascular Neurosurgery: Disorders and their Management

List Price: $179.00


This book answers frequently asked questions about common pediatric neurosurgical conditions related to vascular malformations of the brain and spinal cord, in an attempt to fill in the gap and answer numerous questions that arises after a diagnosis is made.

Pediatric patients with neurosurgical conditions are almost always referred from either primary care physicians, neurologists internists or a specialist in family medicine. Recently, neurosurgeons treating adult population also refer a pediatric patient to their colleague specialized in this field.
There are over 1500 academic and private hospitals in the US who have dedicated tertiary Neurosurgery services and cater thousands of small children every year, in addition to numerous centers that have level 1 and 2 trauma care. However, there are few tertiary level Pediatric centers which can provide quality care for neurosurgical conditions.
This book is specially written and illustrated for residents, fellows and consultants/attendings in all pediatric related specialties, including but not limited to Neurosurgery, Neurology, Pediatrics, Radiology, Anesthesia.

Product Details

  • Published on: 2017-01-03
  • Original language: English
  • Number of items: 1
  • Dimensions: 9.30″ h x .0″ w x 6.10″ l, .0 pounds
  • Binding: Hardcover
  • 327 pages

Editorial Reviews

From the Back Cover
This book focuses on core concepts of vascular neurosurgery in pediatric population,. It is designed to fill the knowledge gaps and to answer the frequently sought questions on various management strategies for commonly encountered pediatric neurosurgical conditions. The chapters, authored by experts in their respective field, provide a standard of care based on current diagnostic and management guidelines for pediatric neurosurgical diseases.

Pediatric Vascular Neurosurgery – Disorders and their Management is specially written and illustrated for residents, fellows and consultants in all pediatric related specialties, including but not limited to Neurosurgery, Neurology, Pediatrics, Neuroradiology and Neuroanesthesia.

About the Author
Dr Abhishek Agrawal, M.D.: House Staff, Department of Neurosurgery/ Radiology, Brigham and Women’s’ Hospital, Harvard Medical School, Boston.

Dr Gavin Britz, MBCCh, MPH, MBA, FAANS: Chairman, Department of Neurosurgery, Methodist Neurological Institute, Houston, Texas