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.

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.

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.

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.

Serial imaging and SWAN sequence of developmental venous anomaly thrombosis with hematoma: Diagnosis and follow-up.

Developmental venous anomalies (DVAs) are usually asymptomatic. We report a case of DVA thrombosis with recurrent tiny frontal hematoma in a 24-year-old man. The contribution of T2-GRE and SWAN sequences are discussed. Follow-up attested complete recanalization after anticoagulation.

Enfermedad quística subadventicial en vena femoral común / Subadventitial cystic disease of common femoral vein

No disponible

Epidural Venous Angioma Presenting with Spinal Cord Compression in a 42-Year-Old Woman with Previous History of Ovarian Malignancy.

BACKGROUND: Venous angioma is an extremely rare vascular malformation of the epidural space. To the best of our knowledge, only 5 cases have been documented to date and none has been reported in the setting of a previous malignancy. CASE DESCRIPTION: We report the case of a 42-year-old woman with a previous history of ovarian cancer, treated by surgery plus chemotherapy; who presented with signs of spinal cord compression for 3 weeks. Magnetic resonance imaging showed an intensely enhancing epidural mass at the T2-T6 level causing major spinal cord compression, for which urgent surgery was indicated. During surgery, the tumor was extremely hemorrhagic and the hemostasis was hazardous. Blood loss was estimated at 1.5 L, causing hemodynamic instability and requiring intensive resuscitation with fluids and blood transfusions. Gross total resection was achieved and the pathologic examination confirmed the diagnosis of venous angioma. The patient recovered quickly postoperatively and was able to walk independently within 2 weeks of starting intensive rehabilitation. She was symptom free with no clinical or radiologic evidence of recurrence at 1 year follow-up. CONCLUSIONS: Venous angioma should be included in the differential diagnosis of spinal epidural masses even in case of previous malignancy. Subtle imaging features should alert clinicians to this rare yet potentially life-threatening condition. Surgery remains the cornerstone of the treatment and can result in remarkable recovery.

Intracerebral hemorrhage due to developmental venous anomalies.

Developmental venous anomalies (DVA) and cavernous malformations (CM) are a common form of mixed vascular malformation. The relationship between DVA, CM and hemorrhage is complicated. It is important to differentiate hemorrhagic CM and hemorrhagic DVA. A retrospective review of all patients with acute spontaneous intracerebral hemorrhages (ICH) between 1 May 2008 and 1 May 2013 was performed. ICH due to DVA or CM were identified and compared for demographic features, clinical symptoms, neurological deficits, and radiological findings. A total of 1706 patients with acute spontaneous ICH were admitted to our hospital during the study period. Among these, 10 (0.59%) were caused by DVA and 42 (2.47%) were caused by CM. No significant differences were found in age (p=0.252) or sex ratio (p=1.000) between the two groups. Compared with CM-induced ICH, DVA-induced ICH were characterized by cerebellar predominance (p=0.000) and less severe neurological deficits (p=0.008). Infratentorial hemorrhagic DVA are characterized by cerebellar predominance and benign clinical course. Infratentorial hemorrhagic CM are mainly located in the brainstem. DVA should be given suspected rather than CM when considering the etiology of a cerebellar hemorrhage, especially in young adults.

Trigeminal Neuralgia Caused by Venous Angioma: A Case Report and Review of the Literature.

BACKGROUND: Trigeminal neuralgia typically occurs in the middle-aged to elderly population, is believed to be related to abnormal conduction within the trigeminal nerve, and is possibly is attributable to changes in myelin induced by pulsatile mechanical trauma from an adjacent vessel. At the point just before it enters the brainstem, there is a short segment at which nerve axons are still ensheathed in central myelin (produced by oligodendrocytes), but after a few millimeters, there is a transition to peripheral myelin (produced by Schwann cells). The region of this transition is called the Obersteiner-Redlich zone. It is thought that the area of the nerve containing the central form of myelin is especially susceptible to pathologic changes from vascular contact that result in demyelination and altered conduction. When associated with a venous angioma at the root entry zone, trigeminal neuralgia usually presents at a younger age. METHODS: We report a 34-year-old man with a complaint of left hemifacial stabbing pain in maxillomandibular area that was triggered by chewing and speaking. On examination, no neurologic deficit was detected. The pain was not relieved significantly, even with the administration of carbamazepine. Magnetic resonance imaging demonstrated venous angioma in the left cerebello-pontine region. RESULTS: Microvascular decompression was performed uneventfully. The patient's pain was completely relieved without neurologic deficits. CONCLUSION: It seems that the trigeminal neuralgia caused by venous angioma may occur in the younger population. In most cases, the vessel that caused compression can be identified with magnetic resonance imaging without the need for intensive conventional angiography.

Cluster headache - a symptom of different problems or a primary form? A case report.

Headache with severe, strictly one-sided unilateral attacks of pain in orbital, supraorbital, temporal localisation lasting 15-180 minutes occurring from once every two days to 8 times daily, typically with one or more autonomic symptoms, is recognized as cluster headache (CH). Headache with normal neurological examination and abnormal neuroimaging studies, mimicking cluster headache, is reported by several authors. We present an elderly woman with a cluster-like headache probably associated with other comorbidities. We differentiate between primary, but 'atypical' CH and symptomatic cluster headache due to frontal sinusitis, pontine venous angioma or vascular compression of the trigeminal nerve root. This headache is not so rare in the general population and its secondary causes must be ruled out before the diagnosis of a primary headache as cluster headache is made.

Angioma venoso asociado a hemorragia cerebelosa. Presentación de un caso / No disponible

Los angiomas venosos se consideran una anomalía del desarrollo caracterizada por persistencia en la edad adulta de un drenaje venoso embrionario. Suelen ser asintomáticos y de curso benigno, aunque pueden ocasionar convulsiones y menos frecuentemente hemorragias, normalmente por malformación cavernosa asociada. En la mayoría de los casos no es preciso tratamiento alguno, salvo circunstancias como sangrado, clínica intensa atribuible a la lesión y localización de buen acceso quirúrgico. Se presenta el caso de un varón de 11 años con un cuadro brusco de disminución del nivel de consciencia, objetivándose en estudios de imagen hematoma en hemisferio cerebeloso derecho, así como estructuras tubulares radiales compatibles con anomalía venosa del desarrollo. Se practicó una intervención quirúrgica con evacuación de hematoma, sin objetivarse otras causas que justifiquen el sangrado (AU)

[Venous angioma with associated cerebellar haemorrhage]. / Angioma venoso asociado a hemorragia cerebelosa. Presentación de un caso.

Venous angiomas are a developmental anomaly in which embryonic venous drainage is still present into adulthood. They are usually asymptomatic and benign course but they can cause seizures and less commonly bleeding, usually associated to cavernous malformation. Normally, treatment is not necessary although bleeding, severe clinical and lesions in which it is possible a favourable approach, we can consider treatment. We show a case of a 11 years old boy with acute decrease level of consciousness. We observed hematoma in the right cerebellar hemisphere with radial tubular structures consistent with developmental venous anomaly. The hematoma was evacuated without a demonstrable other reason justifying the bleeding.

Use of a micromanipulator system (NeuRobot) in endoscopic neurosurgery.

NeuRobot, a micromanipulator system with a rigid neuroendoscope and three micromanipulators, was developed for less invasive and telecontrolled neurosurgery. This system can be used to perform sophisticated surgical procedures through a small, 10-mm-diameter, window. The present study was performed to evaluate the feasibility of using NeuRobot in neuroendoscopy. Four different intraventricular neurosurgical procedures were simulated in three fixed cadaver heads using NeuRobot: (1) fenestration of the floor of the third ventricle; (2) fenestration of the septum pellucidum; (3) biopsy of the thalamus; and (4) biopsy of the choroid plexus of the lateral ventricle. Each procedure required less than 2 min, and all procedures were performed accurately. After these surgical simulations, a third ventriculostomy was carried out safely and adequately in a patient with obstructive hydrocephalus due to a midbrain venous angioma. Our results confirmed that NeuRobot is applicable to lesions in which conventional endoscopic neurosurgery is indicated. Furthermore, NeuRobot can perform more complex surgical procedures than a conventional neuroendoscope because of its maneuverability and stability. NeuRobot will become a useful neurosurgical tool for dealing with lesions that are difficult to treat by conventional neuroendoscopic surgery.