Intracranial lipomas: clinical appearances on neuroimaging and clinical significance

Intracranial lipomas are rare congenital malformations which are neither hematomas nor true neoplasms. They result from the abnormal persistence and maldifferentiation of the meninx primitiva. The majority of such lesions occur near the midline. Around 55% of intracranial lipomas are associated with brain malformations of varying severity. Although they are usually an incidental finding, symptomatic intracranial lipomas are sometimes observed. Surgical excision may result in high morbidity and mortality due to the highly vascular nature of intracranial lipomas and the latter’s strong adhesion to the surrounding tissue and is very rarely indicated.     

Vertex cephaloceles: a review

Introduction

Vertex cephaloceles (VCs), also known as midline parietal cephaloceles, are among the most common midline scalp masses. Usually composed by a meningeal wall herniating from a vertex skull defect and covered by skin, VCs may also contain both anomalous vessels and neural elements. In spite of their harmless appearance, VCs often hide complex intracranial venous and/or brain malformations so that they represent a “tip of the iceberg”. Vertical embryonic positioned straight sinus, elongation of the vein of Galen, persistence of the falcine sinus, fenestration of the superior sagittal sinus, corpus callosum agenesis, intracranial cysts, tentorial malformations, cerebellar vermis agenesis, hydrocephalus, and gray matter heterotopia are some of such associated anomalies.

Methods

The treatment of VCs is surgical. It is indicated to prevent the rupture of the malformation or in case of pain or cosmetic impact. A careful preoperative radiological work up is mandatory to investigate the relationship between the VC and the sagittal sinus and/or the possible communication with the brain. The surgical procedure is usually carried out without significant complications.

Conclusion

The prognosis of VCs is good even though the overall outcome is affected by the associated brain malformations.     

Facial hemangioma, and associated malformations: a case report

Facial hemangioma can be isolated lesions or associated with a wide variety of systemic findings. We report a 9-month-old girl who shows an extensive facial hemangioma, intracranial and extracranial vascular malformations, a Dandy-Walker malformation and congenital cardiac malformations. This patient serves as an ample reason why children with similar cutaneous lesions should be carefully evaluated for other associated defects.     

Spontaneous regression of cerebral arteriovenous malformations: case report and review of the literature

Spontaneous regression of intracranial arteriovenous malformations (AVMs) is a rare phenomenon. Such an event is more likely to occur with small AVMs that present with intracranial hemorrhage, which are fed by a unique artery and drained through a single vein. The factors responsible for AVMs regression remain unclear. Thrombosis of the AVM secondary to intracranial hemorrhage ha been the most commonly associated factor. Other possible causes are the gliosis around the clot secondary to repeated frequent microbleedings or occlusion of the feeding arteries by small emboli. We report a new case of spontaneous regression of a AVM and review the literature related to this entity.     

Neuroimaging of hemorrhage and vascular malformations

Nontraumatic spontaneous intracranial hemorrhage occurs most commonly into the subarachnoid space and brain parenchyma, in contrast to subdural and epidural hematomas that are usually traumatic. The differential diagnosis of nontraumatic subarachnoid hemorrhage includes intracranial aneurysm rupture and vascular malformations, both of which may be investigated noninvasively with computed tomography and magnetic resonance imaging. An isolated intraparenchymal hematoma may be caused by hypertensive vasculopathy, amyloid angiopathy, vascular malformations, or by primary or secondary neoplasms. Knowledge of the appearance of intracerebral vascular malformations will help clinicians request appropriate further imaging and direct treatment.     

Primary brain neoplasms associated with vascular malformations: anatomo-pathologic study of 2 cases

The association of intracerebral vascular malformations and primary cerebral neoplasm is rare. The most commonly found vascular malformation with neoplasm is intracranial arterial aneurysm. We describe two cases of vascular malformations associated with primary cerebral neoplasms, with histologic and immunohistochemical studies.     

Neurologic findings before and after removal of cerebrovascular abnormalities

In a retrospective serie the results of 72 cerebral vascular malformations, operated on consecutively were reviewed: 57 AV-malformations (in 55 patients), 1 dura AV-malformation, 9 cavernomas, 1 telangiectasia, 2 venous angiomas and 2 mixed malformations. The most frequent initial symptoms were headache, epileptic seizures and intracranial bleeding. In 5 cases the initial symptom was loss of consciousness with coma. A total resection could be proved angiographically in all cases. Mortality was 0%, neurological morbidity, i.e., new deficits or increase of existing deficits, was 8.6%. There were no recurrences of bleeding in the postoperative period. Quality of life, as assessed by the Karnofsky Index 6 months postoperatively, was satisfactory, approximately 80% of all patients being back at work. In this group of selectively operated patients it could be shown that localization of the malformation in or close to an eloquent brain area (n = 32) is rarely associated with significant persisting new deficits. Taking into consideration the natural history of AV malformations, with an 8% morbidity and 2% mortality each year, as compared to the surgical results, it is concluded that surgery may be more often indicated for AV malformations than was previously thought, even when they are near eloquent brain areas and relatively large in diameter.     

Literature Review: Sinus pericranii

Abstract

Sinus pericranii is a rare circumscribed fluctuating vascular swelling of the scalp that communicates with the intracranial venous system. It has been associated with other intracranial vascular malformations. The tumour is usually round, fluctuant, nonpulsatile, and disappears with compression. Its size increases during manoeuvers that increase the intracranial pressure. Approximately 100 cases were reported in the literature. The antecedents, different surgical procedures, differential diagnoses, and associated malformations were reviewed. [Neurol Res 1994; 16: 471–474]     

Hypersensitivity to glucocorticoids in patients with raised ICP : report of two cases.

Corticosteroids are widely used in patients with raised intracranial pressure associated with cerebral neoplasms, cerebral vascular malformations, cerebral ischaemia and benign intracranial hypertension. In general clinical practice, anti-allergic, anti-inflammatory and immuno-suppressive properties of corticosteroids are commonly utilised in the management of allergic and immunological diseases. However in exceptionally rare circumstances, steroids may be the cause of hypersensitive reactions. Authors report two patients with raised intracranial pressure who developed steroid hypersensitivity. A review of the relevant literature is discussed.     

Haemangioma calcificans presenting as intracranial haemorrhages during pregnancy

A 33-year-old female with intracerebral haemorrhage during pregnancy, associated with subarachnoid and intraventricular haemorrhages, is presented. Intracranial haemorrhages during pregnancy are known to frequently result from cerebral aneurysms or arteriovenous malformations. We believe that this is caused by haemangioma calcificans and is noteworthy in considering the indications for surgical treatment for intracranial calcified lesions suggestive of cavernous haemangioma.     

A sixty-eight-year-old man with giant intracranial arteriovenous malformation and right-sided hemicrania -- a case report

Arteriovenous malformations (AVMs) are the most dangerous congenital vascular malformations. Intracranial AVMs occur in about 0.1 percent of the population and account for 1 to 2 percent of all strokes. The angioarchitecture of AVMs consists of direct arterial to venous connections without an intervening capillary network. The main symptom reported by half of all patients with arteriovenous malformations is an episodic headache. The headache is associated with nausea and vomiting and due to this resembles the hemicrania or cluster headache attacks. Although cerebral angiography is considered as the gold standard for the diagnosis, planned treatment and after treatment observation, AVMs are usually identified with advanced visual imaging techniques such as magnetic resonance imaging, computed tomography, and computed angiotomography. The treatment options consist of surgery, radiosurgery and endovascular embolization. We report a case of a 68-year-old man with giant intracranial arteriovenous malformation and right-sided hemicrania.     

Intracranial arterial and arteriovenous malformations presenting with infarction. Lausanne Stroke Registry study.

Cerebral aneurysms and arteriovenous malformations (AVMs) are well-known sources of intracranial hemorrhage, but can also manifest as other clinical symptoms or remain clinically asymptomatic. The aim was to document and analyze cases of aneurysm or AVM with brain infarction. Survey on 4804 stroke patients treated at the Department of Neurology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland between 1978 and 2000 using the Lausanne Stroke Registry. Twenty patients presented with cerebral aneurysm and 21 with cerebral AVM. Hemorrhage was present in 100% of the AVM and in 75% of the aneurysm patients; in one (5%) of the remaining aneurysm patients, aneurysm and infarction were located in different territories. Infarction associated with Sylvian artery aneurysm was found in three (15%), vertebrobasilar ischemia because of fusiform left vertebral artery aneurysm in one (5%), and dural fistula draining to the distal transversal and left sigmoid sinus associated with a stroke in the territory of the left anterior inferior cerebellar artery in one patient. Ischemic stroke is infrequent, but important, complication in unruptured intracranial aneurysms and AVMs. The early recognition and therapy of these vascular malformations in selected patients can avoid a major neurological deficit or death caused by their rupture.     

Retinal hemorrhages. Its significance in 100 patients with acute encephalopathy of unknown cause

A total of 94 patients had subarachnoid hemorrhage and it was strongly suspected in the remaining six patients. Acute encephalopathy associated with independent ocular hemorrhage due to hypoxia, multiple emboli, or bleeding tendencies was not a diagnostic problem during this study. Aneurysms occurred in 64 patients (combined with vascular malformations in four), isolated vascular malformations in four; "spontaneous" hematomas in 13, evidence of cryptic head trauma in six, hemorrhage from a glioblastoma in one, and no cause was identified in six patients. Retinal hemorrhages were more prominent ipsilateral to the site of intracranial bleeding. No single aneurysm location predominated and multiple aneurysms were common. The high mortality of 56% supports previous conclusions that retinal hemorrhages tend to accompany severe intracranial bleeding.     

Hemorrhagic complications in vein of Galen malformations

The authors report on a series of spontaneous intracranial hemorrhages associated with vein of Galen aneurysmal malformations (VGAMs). Thirty-four children with VGAMs have been treated at this institution since 1986. Eight children (24%) harbored the mural-type malformation, and 26 (76%) had the choroidal-type lesion. Two children (25%) with mural lesions and 1 (4%) with a choroidal lesion suffered hemorrhagic complications. Two presented with acute intracranial hemorrhage. A third child developed acute intracranial hemorrhage due to delayed dural sinus thrombosis after endovascular treatment of his choroidal-type VGAM. The subjects ranged in age from 13 days to 17 months at the time of presentation. Each patient underwent rapid radiological evaluation and treatment with endovascular surgery. Post-procedural arteriography demonstrated complete occlusion of the malformation in each patient. For the 3 patients with hemorrhage, follow-up has taken place over 49-, 107-, and 43-month intervals, respectively. Vein of Galen aneurysmal malformations can present with acute intracranial hemorrhage or develop delayed intracranial hemorrhage but respond to treatment using standard endovascular techniques. The presence of hemorrhage does not de facto portend a poor prognosis.     

Malformation vasculaire cérébrale découverte dans le bilan pré-thrombolyse d’un infarctus cérébral. Impact sur la décision thérapeutique

Intravenous recombinant tissue plasminogen activator for acute ischemic stroke is contraindicated in patients harboring an asymptomatic intracranial vascular malformation, whether it is incidentally discovered at the time of the initial cerebral imaging or previously known. Because thrombolysis is associated with a risk of serious intracerebral hemorrhage, it is theoretically possible that this treatment increases the risk of bleeding or rupture of these malformations. However, this risk seems very low in clinical practice. We report two cases, one with a probable brainstem cavernous malformation treated with alteplase for a supratentorial ischemic stroke who developed just after treatment a fatal brainstem hemorrhage, and another one with asymptomatic dural arteriovenous fistula, treated by endovascular thrombectomy solely. This approach was safe and effective, and the patient had an endovascular embolization of the fistula one month later as it became symptomatic. Based on the literature, we discuss the bleeding risk of asymptomatic intracranial vascular malformations in acute ischemic stroke patients treated with alteplase, depending on the type of malformation (intracranial aneurysm, arteriovenous and cavernous malformation or fistula), and the alternative therapeutic options.     

Stereotactic gamma knife radiosurgery. Initial North American experience in 207 patients

The first North American gamma knife for stereotactic radiosurgery of brain tumors and arteriovenous malformations entered the therapeutic armamentarium at the University of Pittsburgh (Pa) on August 14, 1987. In this article, we report our initial testing and subsequent experience with this technique. In the first 16 months of operation, 207 patients were treated (113 had arteriovenous malformations, 78 had extra-axial skull base neoplasms, 9 had glial neoplasms, and 7 had metastatic tumors). The patients' lesions either were considered previously as "inoperable" or were residual lesions after attempted surgical resection, or the radiosurgery was performed after the patient declined surgical excision. Gamma radiosurgery was associated with no surgical mortality and no significant early morbidity, and the results were encouraging during the minimum follow-up period of 6 months. Compared with treatment by conventional intracranial surgery (craniotomy), both the average length of stay and hospital charges for radiosurgery were significantly lower. Our initial experience further suggests that stereotactic radiosurgery using the gamma knife is a therapeutically effective and economically sound alternative to microneurosurgical removal of selected intracranial tumors and vascular malformations.     

Endovascular management of arteriovenous malformations and other intracranial arteriovenous shunts in neonates, infants, and children

Purpose

We discuss the management of cerebral arteriovenous shunts in neonates, infants, and children, with emphasis on our experience with pediatric cerebral arteriovenous malformations (AVMs). The management of vein of Galen malformations is discussed in a separate chapter.

Methods

An all-inclusive retrospective chart review of the endovascular surgery operative record database at the Hyman Newman Institute for Neurology and Neurosurgery at Roosevelt Hospital in NYC was conducted. All consecutive pediatric patients (newborn to 18 years of age) with intracranial arteriovenous shunts who presented from January 1, 2004 to June 16, 2009 were included.

Results

A total of 151 consecutive pediatric patients with intracranial arteriovenous shunts were evaluated from the period of January 1, 2004 to June 16, 2009. This included 56 patients with vein of Galen malformations, 48 cerebral AVMs, 11 patients with pial arteriovenous fistulae, six patients with dural arteriovenous malformations, and 30 patients with mixed intracranial vascular malformations. Forty-four patients underwent a total of 163 endovascular embolizations. The complications rate for endovascular embolizations was 6.7% (11 in 163), 5.5% with temporary complications and 1.2% with permanent complications. The mortality rate for the group of patients (excluding patients with vein of Galen malformations) that underwent endovascular embolizations was 0.0%.

Conclusions

Careful clinical observation and timely intervention are important in the management of pediatric patients with intracranial arteriovenous shunts. Trans-arterial endovascular embolization with liquid embolic agents is the treatment of choice for safe stabilization and/or improvement of symptoms in the group of pediatric patients with intracranial arteriovenous malformations.     

Should electroconvulsive therapy be given to patients with intracranial vascular lesions?

The risk of rupture and hemorrhage of intracranial vascular lesions during electroconvulsive therapy (ECT) is currently unknown. We describe 2 cases in which ECT was discontinued because of perceptual disturbances, confusion, and the subsequent discovery of intracranial angiomas. ECT has been associated with nonconvulsive status epilepticus and prolonged altered mental status following treatment, but there has been scant documentation of side effects associated with intracranial vascular malformations. We review the literature on ECT in patients with such lesions and present 2 cases in which lesions were found in the context of perceptual disturbances and altered states of consciousness following ECT.     

BRAIN SCANNING IN DETECTION OF INTRACRANIAL ARTERIOVENOUS MALFORMATIONS

Routine brain scanning, using a scintillation camera and Tc99m pertechnetate, was performed on 42 patients with an intracranial arteriovenous malformation. Thirty-two (76 %) of them had a positive brain scan, and the location of increased uptake correlated well with the localisation of the malformation found in carotid angiography. Large malformations were detected significantly better than small malformations. The largest malformations with negative brain scanning were located in the posterior fossa and temporobasally. In 18 cases (43 %) one or several “tails” of increased activity on the scan were seen sprouting from the local uptake. These tails corresponds to the afferent and efferent vessels of the malformation seen in carotid angiography. Such tails of increased activity are highly suggestive of intracranial arteriovenous malformation.     

螺旋CT脑血管造影在急性脑血管疾病中的应用

目的探讨螺旋CT脑血管造影(CTA)在脑血管性疾病,尤其是自发性脑出血性疾病中的诊断价值。方法对28例自发性脑出血患者行CT脑血管造影3维重建技术。28例患者中发现脑动静脉畸形(AVM)16例,动脉瘤3例,动脉瘤伴AVM 1例,脑胶质瘤1例,无阳性发现者7例。结果CTA能清楚显示AVM和动脉瘤的部位、形态、供血动脉及其与周围结构的关系,对指导手术治疗具有重要参考价值。结论CTA是一种简便、快速、灵活、安全的检查方法,对寻找病灶、指导手术提供了一种客观、实用、可靠的影像资料。