Anomalía venosa del desarrollo cerebral trombosada: hallazgos en la resonancia magnética / Developmental cerebral thrombosed venous anomaly: findings in magnetic resonance imaging

Introducción: Las anomalías venosas del desarrollo son las malformaciones vasculares cerebrales más comunes, suelen ser incidentales y benignas, aunque alrededor del 40% se asocian a malformaciones cavernosas, por lo que es esencial buscar otras anomalías vasculares o neurocutáneas asociadas. Caso clínico: Se presenta una mujer de 34 años embarazada que acude por convulsiones y disartria, a la cual se le realiza una resonancia magnética de cráneo urgente. En ella se aprecia el clásico signo de la ‘cabeza de Medusa’, y se concluye como diagnóstico anomalía venosa del desarrollo con trombosis parcial periférica y flujo lento proximal. Conclusiones: La complicación con trombosis de las anomalías venosas del desarrollo es rara y los informes de casos de la bibliografía sugieren que deben manejarse de forma conservadora, como una trombosis del seno venoso, dejando la cirugía para otras complicaciones asociadas. Como radiólogos, debemos conocer las principales características por imagen para elaborar un diagnóstico certero.(AU)

Introduction: Developmental venous anomalies are the most common cerebral vascular malformations. They are usually incidental and benign, although about 40% are associated with cavernous malformations, and so it is essential to look for other associated vascular or neurocutaneous anomalies. Case report: We report the case of a 34-year-old pregnant woman who presented with seizures and dysarthria, and was submitted to an urgent cranial MRI scan. She showed the classic ‘Medusa head’ sign and was diagnosed with developmental venous anomaly with partial peripheral thrombosis and slow proximal flow. Conclusions: Thrombosis rarely occurs as a complication of developmental venous anomalies and case reports in the literature suggest that they should be managed conservatively, leaving surgery for other associated complications. As radiologists, we must be aware of the main imaging features so as to be able to make an accurate.(AU)

Biventricular hydrocephalus secondary to aqueductal developmental venous anomaly.

Developmental venous anomaly (DVA) is the most common type of intracranial vascular malformation. These lesions are benign and are considered to be non-pathological variants of normal deep parenchymal veins. Although most of them are asymptomatic, a small subset of them located in aqueductal region have been reported to cause obstructive hydrocephalus. The authors present an interesting case of biventricular hydrocephalus secondary to a DVA located on the proximal aqueduct in an adolescent patient. This case is discussed with in corroboration with current literature and management recommendations.

Klippel-Trenaunay-Weber Syndrome with Atypical Presentation of Cerebral Cavernous Angioma: A Case Report and Literature Review.

BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare syndrome characterized by the triad of cutaneous hemangiomas, venous varicosities, and osseous-soft tissue hypertrophy of the affected limb. Clinical manifestations, genetic testing, and radiologic imaging are the key steps in diagnosing this syndrome. CASE DESCRIPTION: An 18-month-old boy was brought for follow-up brain magnetic resonance imaging (MRI) with a history of right lower limb hypertrophy, cutaneous varicosities, and hemangiomas diagnosed at birth. A baseline MRI at 12 months revealed multiple hemorrhagic lesions within the cerebrum, the largest in the right temporal lobe, which was treated surgically at the age of 18 months because of its rapid growth. This is the youngest patient with KTWS treated surgically for intracranial hemangiomas. CONCLUSION: KTWS is a rare disease with a wide range of manifestations. Multisystemic evaluation of this group of patients should be performed to identify cavernous hemangiomas at the early stage of life and adequately treat them in the future. Treatment of KTWS patients with cavernous hemangiomas should not be different from the treatment of patients with any other hemangiomas, and surgical intervention should be considered on a case-to-case bases.

Evaluation of sporadic intracranial cavernous malformations for detecting associated developmental venous anomalies: added diagnostic value of C-arm contrast-enhanced cone-beam CT to routine contrast-enhanced MRI.

OBJECTIVE: Our purpose was to investigate the added diagnostic value of C-arm contrast-enhanced cone-beam CT (CE-CBCT) to routine contrast-enhanced MRI (CE-MRI) in detecting associated developmental venous anomalies (DVAs) in patients with sporadic intracranial cavernous malformations (ICMs). METHODS: Fifty-six patients (53 with single and three with double ICMs) met the inclusion criteria. All patients had routine CE-MRI scans performed at 1.5 Tesla. The imaging studies (CE-MRI and CE-CBCT) were retrospectively and independently reviewed by two observers, with consensus by a third. Group difference, intra- and interobserver agreement, and diagnostic performance of the modalities in detecting associated DVAs were calculated. Reference standard was CE-MRI. RESULTS: On CE-MRI and CE-CBCT, 37 (66%; of 56) and 47 patients (84%; of 56) had associated DVAs, respectively. In 10 patients (52.6%; of CE-MRI negatives [n=19]), CE-CBCT improved the diagnosis. Nine patients (16%; of 56) had no DVA on both imaging techniques. Difference in proportions of associated DVAs on CE-MRI and CE-CBCT was statistically significant, p < 0.05. Sensitivity, specificity, positive likelihood ratio, and area under the curve of CE-CBCT were 100% (95% confidence interval [CI]: 90.5-100%), 47.3% (95% CI: 24.4-71.1%), 1.9 (95%CI: 1.240-2.911), 0.737 (95%CI: 0.602-0.845), respectively. Intraobserver agreement was excellent for CE-MRI, kappa (κ) coefficient = 0.960, and CE-CBCT, κ=0.931. Interobserver agreement was substantial for CE-MRI, κ=0.803, and excellent for CE-CBCT, κ=0.810. CONCLUSIONS: CE-CBCT is a useful imaging technique especially in patients with negative routine CE-MRI in terms of detecting associated DVAs. In nearly half of these particular patients, it reveals an associated DVA as a new diagnosis. KEY POINTS: • Although it is known to be the gold standard, some of the DVAs associated with ICMs are underdiagnosed with CE-MRI. • In nearly half of the patients with negative routine CE-MRI, CE-CBCT reveals an associated DVA as a new diagnosis. • Intra- and interobserver agreement on CE-CBCT is excellent in terms of detecting associated DVAs.

Developmental venous anomalies and brainstem cavernous malformations: a proposed physiological mechanism for haemorrhage.

The incidental diagnosis of both developmental venous anomalies (DVAs) and cavernous malformations (CMs) in the central nervous system is increasing with improved imaging techniques. While classically silent diseases, these cerebrovascular pathologies can follow an aggressive course, particularly when present in the brainstem. In the last decade, substantial research has focussed on KRIT1-mediated tight junction gene expression and their role in CM development. However, our understanding of the physiologic conditions precipitating symptomatic CM development or CM haemorrhage with and without concomitant DVAs, remains lacking. The only established risk factor for CM haemorrhage is a previous history of haemorrhage, and literature currently reports trauma as the only precipitant for symptomatic events. While plausible, this occurs in a minority, with many patients experiencing occult events. This manuscript presents a hypothesis for symptomatic CM events by first discussing the anatomical pathways for intracranial venous outflow via the internal jugular veins (IJV) and vertebral venous plexus (VVP), then exploring the role of venous flow diversion away from the IJVs under physiologic stress during dynamic postural shift. The resultant increase in intracranial venous pressure can exacerbate normal and pre-existing structural DVA pathologies, with repeated exposure causing symptomatic or CM-inducing events. This pathophysiological model is considered in the context of the role of the autonomic nervous system (ANS) in postural intracranial venous outflow diversion, and how this may increase the risk of DVA or CM events. It is hoped that this hypothesis invokes further investigation into precipitants for DVA or CM events and their sequela and, also, furthers the current knowledge on pathophysiological development of DVAs and CMs.

Developmental venous anomaly as a rare cause of obstructive hydrocephalus.

Developmental venous anomalies (DVA), previously known as cerebral venous malformations or venous angiomas, are common benign entities often incidentally discovered at MRI examinations. They are non­pathologic variants of normal deep parenchymal veins that are usually asymptomatic, but they can rarely cause some complications. In this paper we described a rare case of obstructive hydrocephalus caused by a DVA located within the cerebral aqueduct and we also reviewed the previous literature on this topic. A 37­year­old man was diagnosed with symptomatic tri­ventricular hydrocephalus that during the last year caused episodes of ictal headaches accompanied by vertigo, diaphoresis and lipothimic events. The MRI and the angiography showed the presence of a large collector vein coursing within the aqueduct, which was part of a complex DVA in the posterior fossa, responsible for the aqueductal stenosis and obstructive hydrocephalus. The patient was submitted to endoscopic third ventriculostomy (ETV) and then he had a complete resolution of symptoms. A literature review was performed through Medline Pubmed on papers published in English from 1937 to 2015 with the following key words: DVA, obstructive hydrocephalus, acqueductal stenosis, venous angioma, venous malformation, medullary malformation. Including our case, the literature research identified 16 reports in the Medline database for 18 patients presenting with obstructive hydrocephalus caused by a DVA including our case. In approximately 60% (10/17) of cases, the stenosis causing obstructive hydrocephalus was present in the cerebral aqueduct. The floor of the IV ventricle and the third ventricle were the second most common locations and DVAs were identified in 20% of cases (3/17) for each of these regions. In the remaining case the venous malformation was found in the Foramen of Monro. Clinical presentation before diagnosis lasted on average 23 months ranging from 1 month to 7 years. The mean age at diagnosis was 27 (range 3 days- 58 years). The most common presenting symptom was persistent headache, representing a major complaint in 80% of cases (12/15). In 78% (14/18) of cases MRI was crucial in the diagnosis. ETV was performed in 47% (8/17) of patients, whereas no treatment or clinical follow­up was required in 18% (3/17) and VP Shunt in 12% (2/17) of cases. Instead, VA shunt, transcallosal approach and acqueductal stenting were performed in one patient each, corresponding singularly to 6% (1/17) of all cases. Despite the fact that DVAs are asymptomatic, they may rarely cause obstructive hydrocephalus because of impairment in the CSF flow. They should be considered in the differential diagnosis of any patient presenting with obstructive hydrocephalus. ETV has been demonstrated as an effective treatment option in the management of obstructive hydrocephalus due to a DVA.

"It's Not a Tumor": A Rare Case of Symptomatic Cerebellar Developmental Venous Anomaly.

Cerebral developmental venous anomalies (DVAs) are benign anatomical variants of the venous system and are commonly described as an incidental finding without clinical significance. Neurologic symptoms or abnormal examination findings are rare and usually attributed to hemorrhagic complications related to coexisting cavernous malformations. There have been limited case reports of symptomatic, uncomplicated DVAs described in the literature. The following case describes a previously healthy child who presented to the emergency department with an acute onset of altered mental status, headache, and focal neurologic examination abnormalities. Magnetic resonance imaging revealed a prominent cerebellar DVA. There was no evidence of a cavernous angioma, hemorrhage, or acute parenchymal injury. This case report illustrates a clinically symptomatic, uncomplicated posterior fossa DVA. It provides additional evidence regarding the potential for a cerebral venous malformation in causing focal neurologic deficits.

Anomalía venosa del desarrollo intracraneal. ¿Asintomática? / Intracranial developmental venous anomaly: is it asymptomatic?

La anomalía venosa del desarrollo intracraneal (AVD) representa la malformación vascular intracraneal más frecuente. En la inmensa mayoría de los casos es incidental y asintomática, y se considera benigna. No obstante, muy excepcionalmente puede presentarse con clínica neurológica. En este trabajo se presentan tres casos de pacientes con AVD que iniciaron distinta sintomatología debida a complicaciones derivadas de alteraciones en el drenaje venoso. Dichas AVD se localizaron en la ínsula izquierda, el lóbulo temporal derecho y el cerebelo. La excepcionalidad de los casos presentados, así como de las imágenes asociadas que objetivan el mecanismo productor de la clínica, radica en la baja incidencia de AVD sintomáticas descritas en la literatura

Intracranial developmental venous anomalies are the most common vascular malformation. In the immense majority of cases, these anomalies are asymptomatic and discovered incidentally, and they are considered benign. Very exceptionally, however, they can cause neurological symptoms. In this article, we present three cases of patients with developmental venous anomalies that presented with different symptoms owing to complications derived from altered venous drainage. These anomalies were located in the left insula, right temporal lobe, and cerebellum. The exceptionality of the cases presented as well as of the images associated, which show the mechanism through which the symptoms developed, lies in the low incidence of symptomatic developmental venous anomalies reported in the literature

Formation During Lifetime of Arteriovenous Shunt in Developmental Venous Anomaly That Caused Intracerebral Hemorrhage.

BACKGROUND: Developmental venous anomaly (DVA) or venous angioma is a common anomaly of cerebral veins that is found incidentally in the majority of cases. There are few cases of arteriovenous shunting in DVA associated with a more malignant course of the disease. Whether these DVAs with shunts are of congenital pathology or lifetime formations is unclear. CASE DESCRIPTION: We report a case of lifetime arteriovenous shunt formation in DVA that caused intracerebral hemorrhage in a child. The patient underwent 2 sequential direct surgeries: an emergency evacuation of the intracerebral hematoma and a scheduled excision of the DVA with arteriovenous shunting. CONCLUSIONS: Arteriovenous shunting in DVA may develop during a lifetime and cause intracerebral hemorrhages. This case showed that localization of DVA with arteriovenous shunting in a noneloquent area enables its complete microsurgical excision with favorable functional outcomes.

Clinical manifestations and imaging findings of thrombosis of developmental venous anomalies.

AIM: To determine clinical manifestations, imaging findings and outcome of patients with thrombosed developmental venous anomalies (DVAs). MATERIALS AND METHODS: The radiology database was searched retrospectively for thrombosed DVAs between 01/01/2000 to 07/01/2016. Demographic variables, associated risk factors, clinical manifestations, imaging findings, treatments, and follow-up were recorded. RESULTS: Six patients were found (four female and two male; age range 16-45 years with mean age, 21.3 years). The most common clinical presentation was headaches followed by neurological deficits and seizures. Venous infarction, parenchymal haemorrhage, venous congestive oedema were noted as the radiological findings. Clinical outcome was favourable in all patients with complete recovery or persistence of mild neurological symptoms. CONCLUSION: Thrombosed DVAs may occur under rare circumstances, which lead to variable symptoms. Familiarity with this entity and early recognition of associated findings including venous infarction, parenchymal haemorrhage, and venous congestive oedema would help early diagnosis and prompt treatment.

Long-Term Outcome of Gamma Knife Radiosurgery for Brain Cavernoma: Factors Associated with Subsequent De Novo Cavernoma Formation.

BACKGROUND: We aimed to evaluate the factors associated with de novo brain cavernoma formations after patients underwent gamma knife radiosurgery (GKRS) and confirmed whether developmental venous anomaly (DVA) presented with a cavernoma and whether the cavernoma was included in the GKRS target location. METHODS: From January 2003 to December 2008, 95 patients underwent radiosurgery for brain cavernoma at our institution. Of these, 15 with multiple cavernomas related to familial cavernoma or with a history of surgical treatment for cavernoma were excluded. A total of 80 patients (44 men and 36 women; average age, 39.4 years) with sporadic cavernoma were retrospectively analyzed by considering the patient characteristics, including sex, age, target volume, radiation dose, clinical symptoms, cavernoma location, radiosurgery complications, and morphology of DVA. RESULTS: The average target volume, mean radiation dose, and mean target percentage were 1019.2 mm3, 13.7 Gy, and 51.1%, respectively. Nineteen patients showed cavernomas associated with DVA; of these, de novo cavernoma formations were noticed in 4 patients at a median of 49.5 months after undergoing GKRS. All de novo cavernomas were related to the presence of DVA and were located near the brainstem or cerebral peduncle. De novo cavernomas occurred when DVAs were not included in the GKRS-target location. CONCLUSIONS: All de novo cavernomas were located near the brainstem or cerebral peduncle, and they occurred in the presence of DVAs. The presence of DVA in the radiosurgery target location might be potentially an important factor associated with de novo cavernoma formation.

Cerebellar Proliferative Angiopathy Associated with a Pontine Telangiectasia and a Developmental Venous Anomaly.

Various mixed associations between arteriovenous malformations, cavernous malformations, developmental venous anomalies, and capillary telangiectasias have been described, and a common pathophysiologic event has been suggested to be present, although it is yet to be elucidated. We depict herein the imaging features of a patient who presented with a spontaneous cerebellar hemorrhage, in whom radiologic studies demonstrated a pontine telangiectasia, a brainstem/cerebellar developmental venous anomaly, and a cerebellar proliferative angiopathy. This unique, not previously reported combination of lesions shows that the spectrum of mixed vascular malformations continues to expand. A pathophysiologic mechanism related to the angiogenesis seen in these malformations is also hypothesized.

Intracranial developmental venous anomaly: is it asymptomatic? / Anomalía venosa del desarrollo intracraneal. ¿Asintomática?

Intracranial developmental venous anomalies are the most common vascular malformation. In the immense majority of cases, these anomalies are asymptomatic and discovered incidentally, and they are considered benign. Very exceptionally, however, they can cause neurological symptoms. In this article, we present three cases of patients with developmental venous anomalies that presented with different symptoms owing to complications derived from altered venous drainage. These anomalies were located in the left insula, right temporal lobe, and cerebellum. The exceptionality of the cases presented as well as of the images associated, which show the mechanism through which the symptoms developed, lies in the low incidence of symptomatic developmental venous anomalies reported in the literature.

Unusual location of developmental venous anomaly within fourth ventricle causing obstructive hydrocephalus - A case report.

Developmental venous anomaly (DVA) is now considered common and benign disease within the field of cerebral vascular malformation. Though symptomatic DVA is uncommon, further management is necessary to alleviate the symptoms and signs induced by symptomatic DVA, such as parenchymal hemorrhage, venous infarction, brain edema, obstructive hydrocephalus, and nerve root compression. From the viewpoint of obstructive hydrocephalus, mostly resulted from obstruction of aqueduct of Sylvius. Herein, we reported a case with presentation of obstructive hydrocephalus caused by DVA induced fourth ventricle outlet obstruction.

Clinical Outcome of Nonfistulous Cerebral Varices: the Analysis of 39 Lesions

OBJECTIVE: Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases.METHODS: From 2000 to 2015, 22 patients with 39 nonfistulous CVs (≥5 mm) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well.RESULTS: The mean age of the patients was 21 years (range, 0–78 years). On average, 1.8±1.2 CVs were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge- Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively.CONCLUSION: Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.

Clinical Outcome of Nonfistulous Cerebral Varices: the Analysis of 39 Lesions

OBJECTIVE: Cerebral varices (CVs) without an arteriovenous shunt, so called nonfistulous CVs, are very rare, and their etiology and natural course are not well understood. The aim of this study is to evaluate the clinical outcomes of nonfistulous CVs by the analysis of 39 cases. METHODS: From 2000 to 2015, 22 patients with 39 nonfistulous CVs (≥5 mm) were found by searching the medical and radiologic records of our institute. Clinical data and radiological data including numbers, sizes and locations of CVs and associated anomalies were retrospectively collected and analyzed. Previously reported cases in literature were reviewed as well. RESULTS: The mean age of the patients was 21 years (range, 0–78 years). On average, 1.8±1.2 CVs were found per patient. CVs were categorized as either fusiform or saccular depending on their shapes. Two patients had saccular type CVs, seventeen patients had fusiform types, and three patients had both fusiform and saccular CVs. Eight patients had associated compromise of the vein of Galen and the straight sinus. Four of those patients had sinus pericranii, as well. Five patients had CVs that were distal draining veins of large developmental venous anomalies. One patient had associated migration anomaly, and two patients had Sturge- Weber syndrome. Six patients with an isolated cerebral varix were observed. Of the 39 CVs in 22 patients, 20 lesions in 14 patients were followed up in outpatient clinics with imaging studies. The average follow-up duration was 6.6 years. During this period, no neurological events occurred, and all the lesions were managed conservatively. CONCLUSION: Nonfistulous CVs seemed to be asymptomatic in most cases and remained clinically silent. Hence, we suggest conservative management.