Arteries and Veins of the Sylvian Fissure and Insula: Microsurgical Anatomy.

We present a vascular anatomical study of the arteries and veins of the sylvian fissure and insula.A good knowledge of the sylvian fissure, the insula, and their vascular relationship would seem mandatory before performing surgery in this area, whatever the type of surgery (aneurysms, arteriovenous malformations, insular tumors).We start with the sylvian fissure and insula morphology, followed by the MCA description and its perforators, with special attention paid to the insular perforators. We demonstrate that the long insular perforators penetrating in the superior part of the posterior short gyrus and long gyri vascularize, respectively, the corticonuclear and corticospinal fasciculi. We particularly insist too on three anatomical constants regarding the vascularization of the insula, already described in the literature: The superior periinsular sulcus is the only sulcus on the lateral surface of the brain without an artery along its axis; the superior branch of the MCA supplies the anterior insular pole and both the anterior and middle short gyri in 100 % of cases; in at least 90 % of cases, the artery that supplied the central insular sulcus continued on to become the central artery.We end with the anatomical study of the veins and cisterns.

Sporadic intracranial haemangioblastomas: surgical outcome in a single institution series.

BACKGROUND: Haemangioblastomas are benign vascular tumours that may appear sporadically or in von Hippel-Lindau disease. Despite their higher incidence, sporadic haemangioblastomas have been less studied than syndromic ones. In this article, we evaluate the specific features, outcome and quality of life of patients with intracranial sporadic haemangioblastomas (ISHs) operated on in our institution. METHODS: Between 1998 and 2010, 38 patients harbouring 38 ISHs were operated on in our department. Their clinical, biological, radiological and surgical features were retrospectively reviewed. All patients were contacted for a quality-of-life (QOL) survey assessed by the Short Form 36 questionnaire (SF36). The mean duration of follow-up was 40 months (13-108 months). RESULTS: ISH represented 0.9 % of primary intracranial neoplasms treated in our centre during this period. Patients comprised 23 men and 15 women with a mean age of 47 years. None had polycythaemia. Cerebellar locations accounted for 79 % of ISHs, and brainstem ISH with involvement of the floor of the fourth ventricle represented 11 % of ISHs. At last follow-up, two patients harbouring solid medulla oblongata haemangioblastoma had died following severe bulbar syndrome and five patients had died of unrelated causes. One patient had multiple surgeries for three recurrences. Tumoral control was achieved in all cases at last follow-up. Results of the SF-36 questionnaire were as follows: median physical functioning score 100 (range 0-100), median physical problems score 100 (range 0-100), median bodily pain score 100 (range 45-100), median social functioning score 100 (range 25-100), median general mental health score 84 (range 40-92), median emotional problems score 100 (range 0-100), median vitality score 70 (range 35-80) and median general health perceptions score 70 (range 35-100). Mean QOL scores were similar to the general healthy population. CONCLUSION: Surgery of ISH provides good QOL and tumoral control except for those located in the medulla oblongata. We recommend considering a careful multimodal therapeutic approach, including radiosurgery for these specific locations.

Markers of recurrence and long-term morbidity in craniopharyngioma: a systematic analysis of 171 patients.

CONTEXT: Craniopharyngiomas are often associated with an unfavorable prognosis, but data on their long-term consequences are sparse. OBJECTIVE: The aim of the study was to identify markers of recurrence and factors associated with compromised social rehabilitation and altered quality of life in a large cohort of patients with either childhood-onset (CO) or adult-onset craniopharyngioma. METHODS: Retrospective analysis was performed for 171 patients treated for craniopharyngioma in two academic centers in France between 1972 and 2009. For each subject, data were collected concerning clinical presentation, imaging features, visual sequelae, endocrine and metabolic impact, treatment modalities (surgery, radiotherapy), recurrence-free survival rate, and social insertion, as well as answers to the WHO-QOL BREF questionnaire. RESULTS: A total of 65 CO and 106 adult-onset patients were reviewed. If CO was diagnosed before the age of 10 yr, this was associated with a higher incidence of obesity, blindness, and panhypopituitarism, and only 40.7% of subjects had adequate work or school attendance compared to 72.4% of patients with later disease onset. Initial symptoms of intracranial hypertension (SIHT), pterional surgery, and multiple surgery were associated with obesity and poorer social insertion. No determinant of quality of life was identified. In the subgroup of patients treated in the 1990s and later, the progression rate was 59.4% in patients with residual tumor on magnetic resonance imaging compared with a 19.8% recurrence rate in the group with apparently complete resection. Recurrence/progression correlates significantly with male gender, early onset (before 10 yr), and SIHT, but only SIHT at presentation remained a significant predictor with multivariate analysis. CONCLUSIONS: Craniopharyngioma continues to be associated with severe outcomes. Higher morbidity rates are found in patients with early-onset disease (before 10 yr), initial SIHT, or in whom pterional surgery was required. Markers of recurrence are difficult to identify, with SIHT being the most powerful predictor.

Surgical treatment of rare cauda equina tumours.

BACKGROUND: Cauda equina tumours (CET) are rare and usually benign. Treatment of schwannomas and benign ependymomas, which are the most frequent histopathological types of CET, is now well established. However, management of other presumed histopathological types of CET is still a matter of debate. The aim of this study was to assess the incidence and the surgical treatment of rare CET. METHOD: A retrospective study was carried out on 176 adult patients surgically treated for CET in our two departments from 1994 to 2010. We reviewed pre- and postoperative symptoms, magnetic resonance imaging aspects, surgical findings, outcome including operative neurological morbidity, local recurrence rate and operative mortality, and incidence of rare CET. FINDINGS: Seventeen percent (30 patients) of CETs operated on were neither schwannomas nor benign ependymomas. Half of these cases were benign tumours, with paragangliomas being the most common. Two patients were in poorer clinical condition after surgery, one patient experienced a local recurrence, and one died following surgery, from the progress of his disease (Von Hippel-Lindau disease). The other half were malignant tumours, with metastases being the most common. One third of the patients were worsened by surgery, and the mortality rate was 1/3 at 8 months (1-27 months). CONCLUSIONS: Roughly one in six CET were neither schwannomas nor benign ependymomas. This study demonstrated the efficiency of surgery for rare benign CET with a low local recurrence rate. Surgical treatment of rare malignant CET led to a high rate of increased postoperative neurological deficit in patients with a reduced life expectancy.

Olfactory ensheathing cell tumour: case report and literature review.

Olfactory ensheathing cell tumour (OECT) and olfactory groove schwannoma (OGS) are among the rarest intracranial tumour types that develop within anterior cranial fossa. These tumours share several similarities, including radiological and histological aspects, and only immunohistochemical staining can differentiate between them. We report a case of OECT occurring in a 28-year-old woman with a history of complex partial seizures, emotional lability and anosmia. Radiological features showed a predominantly left subfrontal extra-axial mass. Total excision of the tumour, connected to the cribriform plate and contiguous to the left olfactory bulb, was performed. Histological examination suggested an atypical schwannoma; however, immunohistochemical staining was strongly positive for S-100 protein but negative for both epithelial membrane antigen (EMA) and CD 57 (Leu-7). The final diagnosis was olfactory ensheathing cell tumour. We describe the third case of OECT and emphasize the important role of immunohistochemical staining in diagnosis: awareness of this entity, and use of immunohistochemistry help to distinguish it from OGS.

SPECT and PET analysis of subthalamic stimulation in Parkinson's disease: analysis using a manual segmentation.

The subthalamic nucleus (STN) has become an effective target of deep-brain stimulation (DBS) in severely disabled patients with advanced Parkinson's disease (PD). Clinical studies have reported DBS-induced adverse effects on cognitive functions, mood, emotion and behavior. STN DBS seems to interfere with the limbic functions of the basal ganglia, but the limbic effects of STN DBS are controversial. We measured prospectively resting regional cerebral metabolism (rCMb) with 18-fluorodeoxyglucose and PET, and resting regional cerebral blood flow (rCBF) with HMPAO and SPECT in six patients with Parkinson's disease. We compared PET and SPECT 1 month before and 3 months after STN DBS. On cerebral MRI, 13 regions of interest (ROI) were manually delineated slice by slice in frontal and limbic lobes. We obtained mean rCBF and rCMb values for each ROI and the whole brain. We normalized rCBF and rCMB values to ones for the whole brain volume, which we compared before and following STN DBS. No significant difference emerged in the SPECT analysis. PET analysis revealed a significant decrease in rCMb following STN DBS in the superior frontal gyri and left and right dorsolateral prefrontal cortex (p < 0.05). A non-significant decrease in rCMb in the left anterior cingulate gyrus appeared following STN DBS (p = 0.075). Our prospective SPECT and PET study revealed significantly decreased glucose metabolism of the two superior frontal gyri without any attendant perfusion changes following STN DBS. These results suggest that STN DBS may change medial prefrontal function and therefore the integration of limbic information, either by disrupting emotional processes within the STN, or by hampering the normal function of a limbic circuit.

Gowers intrasyringal hemorrhage. Case report and review of the literature.

The concept of hemorrhage in a preexisting syringomyelic cavity was first described by Gowers in 1904. Since its first description only 13 cases have been reported. The aims of this report are to describe a new case, bring this entity to wider attention, and summarize the existing literature on the subject. This 36-year-old woman presented with progressive gait disturbance and unsteadiness. Physical examination revealed incomplete quadriparesis, predominantly on the left side, and hypesthesia below C-7. Magnetic resonance imaging revealed hematomyelia characterized by a heterogeneous hyperintense signal within the central cervical cord. A liquefied well-limited hematoma was evacuated. The postoperative course was uneventful; a near-complete recovery was observed at the 7-year follow-up examination. Most cases of intrasyringal hemorrhage (ISH) have occurred in syringomyelic cavities associated with scoliosis or Chiari malformation Type I. Although there is no specific clinical picture associated with this entity, it can be characterized by three neurological forms: 1) sudden onset or rapid development of signs and symptoms, 2) acute worsening of symptoms that may improve but leaving greater neurological dysfunction than before the previous episode, and 3) ISH may initiate progressive deterioration in a patient with known syringomyelia. Intrasyringal bleeding is most probably caused by a sudden dilation of the syringomyelic cavity, which may provoke rupture of the intrasyringal vessels by an acute distension of the accompanying strands. Magnetic resonance imaging is the most accurate diagnostic modality, and recognition of ISH can lead to early, safe, and efficient surgical treatment.

Asymptomatic syringomyelia in the course of medulloblastoma.

Syringomyelia is frequently associated with Chiari malformation or one of many other pathological conditions. Its co-occurrence with medulloblastoma is rare, and to our knowledge, only 4 patients have been reported, although some reports have documented on syringomyelia associated with intracranial processes or intramedullary tumor. The authors describe an unusual case of asymptomatic thoracic syringomyelia complicated by an intrasyringal hemorrhage in a child with medulloblastoma. This report illustrates that, although unusual, syringomyelia is a potential complication in the natural history of medulloblastoma, and the authors consider the possible pathogenesis of syrinx enlargement.

Primary liposarcoma of the thoracic spine: case report.

Liposarcoma is a malignant tumor of soft tissue. The thoracic spine is an unusual location, even for metastasis, and to our knowledge, no case of primary pleomorphic liposarcoma of the vertebral body has been reported until now. A female patient presented with paraplegia. She had a previous medical history of mental depression, and complained of dorsal pain for three months following a road accident. Magnetic Resonance Imaging (MRI) revealed a collapse of T7-T8, and the diagnosis of plasmocytoma was made. She was treated with decompressive laminectomy and posterior instrumentation. Histological examination revealed a pleomorphic liposarcoma. She received a course of radiotherapy. At 13 months follow-up she developed pulmonary metastases and rib involvement. The spine is an unusual location for pleomorphic liposarcoma, even as metastasis. The differential diagnoses of this rare entity are discussed, as well as the criteria for diagnosing primary spinal liposarcoma. Although rare, our case demonstrates that liposarcoma should be considered in the differential diagnosis of spinal tumors.

Pathophysiology of capillary haemangioma growth after birth.

Capillary haemangiomas (CHs) are the most common soft tissue tumours of infancy. It is generally believed that the primary defect in CHs is intrinsic to endothelial cells, but their pathogenesis is yet poorly understood. The relatively low oxygen environment, in which the human foeto-placental unit develops, during the first trimester, is necessary to induce vasculo-angiogenesis via embryonic endothelial cells proliferation, since these cells are sensitive to hypoxia and acidosis. In newborn infants with haemangioma, persistent embryonic primitive endothelial cells trapped in the intimae underneath the developing vessels, and representing "leader" endothelial cells, can stabilise the labile vascular endothelial growth factor mRNA (VEGF mRNA), produce other angiogenic factors, degrade the underlying basement membrane and invade into the stroma of the neighbouring tissue. With bearing down, the transition from intra- to extra-uterine life is accompanied by more or less pronounced hypoxia. Consequently, in babies with haemangioma, hypoxia can act as a switch to activate these "leader" endothelial cells and thereby initiate a cascade of reactions leading to CH proliferation. As they are regulated by embryonic cells, the haemangioma growth mechanisms pursue the pathway of embryonic angiogenesis and it will stop at the end of the embryonic endothelial cell cycle. Addressing this mechanism in vivo has partly been done (the angiogenic peptide bFGF varies with haemangioma growth). Thus, early treatment seems necessary in infants with haemangioma, before the endothelial cells achieve their proliferative stage. The use of an antibody to interfere with VEGF receptors provides a particular attractive strategy.

Cervical spondylotic myelopathy in athetoid cerebral palsy patients: about five cases.

UNLABELLED: We herein report five cases of cerebral palsy athetosic patients with spondyloid cervical myelopathy. Four of them underwent decompressive surgery. The level of cervicarthrosis differs from a control population with a more frequent osteoarthritis on the lower cervical spine. The diagnosis of spondylotic cervical myelopathy is frequently overlooked because of the insidious progression of neurologic disorders and of the pre-existent neurological handicap. Depressive syndrome is often evoked in such a situation, and thus responsible for a delay of diagnosis. The presence of an hypersignal in T2 MRI sequences is still controversial. For some authors it is an indication for surgery, which is the treatment with the best functional results. CONCLUSION: Cervical spondylotic myelopathy must be evoked in patients with athetoid cerebral palsy who complain about a decrease of their functional ability.

Capillary haemangioma arising from the anterior choroidal artery.

BACKGROUND: Capillary haemangioma of the central nervous system is extremely rare. Histologically proven cases developed in the dura mater and choroid plexus, or were typically intracranial extensions of an extra-cranial lesion. FEATURES: This report details a case that developed in the anterior choroidal artery of a newborn infant and manifested as a lethal intra-cerebral haemorrhage. Pathological criteria for the diagnosis of vascular malformations should be carefully investigated and the differential diagnoses of the present case are discussed. PROGNOSIS: Intracranial haemangioma presents a diagnostic challenge and the treatment of deep lesions remains problematic.

Results in the operative treatment of elderly patients with spinal meningiomas.

STUDY DESIGN: Thirty consecutive patients over 70 years of age who were operated on for spinal meningiomas are presented to assess surgical criteria and postoperative outcome. OBJECTIVES: To assess the outcome of surgically treated spinal meningiomas in patients in their eighth or ninth decade of life and to analyze the possible role of some prognostic factors. SUMMARY OF BACKGROUND DATA: The ageing of the population in the industrial nations has led to increased findings of benign spinal tumors, namely meningiomas, in the elderly. These slow-growing tumors may prevent these patients from leading an autonomous life. Little is known about the outcome of spinal meningioma surgery in patients of 70 years or older because the number of these patients, although increasing, is still small. METHODS: Thirty consecutive patients over 70 years of age (mean age, 77.1 years) were operated on for spinal meningiomas between 1986 and 2001. The preoperative neurologic status of the patients was graded with the Solero score, and the general health conditions were staged according to the American Society of Anesthesiology classification. Total tumor removal was achieved in 27 patients. The follow-up period ranged from 17 to 173 months (mean, 62.7 months). RESULTS: There was no operative mortality, and morbidity was not significant. Although not significant, neurologic outcome was better in patients with a low preoperative Solero score than those with a high one (P = 0.06). Clinical outcome was not influenced by the duration of symptoms before surgery or preoperative American Society of Anesthesiology classification. No recurrence was observed during the follow-up period. CONCLUSIONS: Neurologic outcome following surgery was favorable in the vast majority of patients, with no mortality or significant morbidity. Surgery is the only treatment in elderly patients with symptomatic spinal meningiomas, even those with a poor preoperative neurologic condition, whenever there is an acceptable risk from an anesthesiological point of view.

Caudal spinal cord ischemia after lumbar vertebral manipulation.

Neurological complications after lumbar spine manipulation are uncommon. The cause is usually a herniated disk or displaced bony structure. We report a case of paraplegia that developed a few hours after manipulation of the lumbar spine. Magnetic resonance imaging was consistent with ischemia of the caudal spinal cord. No disk fragment or bony structure impinging on the spinal cord was seen. Spinal cord ischemia may deserve to be added to the list of possible adverse events after lumbar spine manipulation.

Extensive spinal cord infarction after posterior fossa surgery in the sitting position: case report.

OBJECTIVE AND IMPORTANCE: Spinal cord injury is a rare complication of neurosurgery performed with the patient in the sitting position. Previous reports showed that the level of injury is usually located at or near the C5 segmental spinal level, and the term midcervical quadriplegia has been proposed. Extensive spinal cord and lower brainstem infarction also can occur after posterior fossa surgery performed with the patient in the sitting position. CLINICAL PRESENTATION: We describe a 45-year-old woman who was operated on in the sitting position because of a fourth ventricular pilocytic astrocytoma. After surgery, the patient experienced quadriplegia. INTERVENTION: T2-weighted magnetic resonance imaging scans revealed a long, hyperintense area within the cervicothoracic spinal cord that was extended to the lower pons and was consistent with infarction. There was no evidence of previous spine disease. The patient died 6 weeks later of respiratory failure. CONCLUSION: We speculate that alteration of spinal cord blood flow by stretching of the cervical spinal cord and spinal epidural venous engorgement might have caused this devastating complication.

Epithelioid hemangioendothelioma of the infundibular-hypothalamic region: case report and literature review.

Epithelioid hemangioendothelioma (EHE) is an uncommon vascular neoplasm. Its intracranial occurrence is rare, and the literature review revealed only 23 cases (14 adults and 9 children). To our knowledge, this is the first case of EHE arising in the infundibular-hypothalamic region. A 53-year-old man presented with headaches and loss of libido. Magnetic resonance imaging revealed a supra-sellar mass, which homogeneously absorbed the contrast agent. A trans-sphenoidal craniotomy and a biopsy were performed. Adjuvant radiotherapy was administered due to intolerance to interferon. Epithelioid hemangioendothelioma is a hemorrhagic tumor. Total resection is mandatory where possible, otherwise radiotherapy seems necessary. The prognosis of intracranial location has not yet been well defined, despite the favorable outcome noted in the majority of cases.

Epidural hematoma as a complication of endoscopic biopsy and shunt placement in a patient harboring a third ventricle tumor.

Although some complications have been reported when using endoscopic techniques, epidural hematoma (EDH) has rarely been caused. The authors report a case of EDH complicating a concomitant endoscopic biopsy procedure with ventricular shunt placement in a young, 14-year-old boy harboring a third ventricle tumor with a hydrocephalus. Few cases have been reported; however, the authors warn that there is a risk that EDH may develop following an endoscopic procedure and shunt placement.

Nevoid basal cell carcinoma syndrome: relation with desmoplastic medulloblastoma in infancy. A population-based study and review of the literature.

BACKGROUND: Patients with nevoid basal cell carcinoma syndrome (NBCCS) are believed to be predisposed to develop early-onset neoplasms including medulloblastomas (MB). The desmoplastic subtype of MB is associated most commonly with NBCCS. The goals of this study were to demonstrate the relation between desmoplastic MB and NBCCS and to evaluate the concomitant diagnosis of NBCCS and MB. METHODS: The medical records of 76 consecutive children who received surgical treatment for MB between 1970 and 2000 were studied. A review of the literature was performed based on the National Library of Medicine database and bibliographies of selected articles were scanned. RESULTS: The authors reported three patients with NBCCS who received surgical treatment for an MB during infancy. The literature review identified 33 patients with NBCCS who were treated for MB at a mean age of 28 months. The desmoplastic subtype was the only histopathologic subtype of MB reported in the NBCCS population. Although patients with NBCCS are predisposed to develop multiple basal cell carcinomas and intracranial tumors in the field of irradiation, the prognosis for syndromic MBs was much better compared with the prognosis for sporadic MBs. CONCLUSIONS: Patients with NBCCS have an increased risk for other malignancies, especially radiation-induced neoplasms. Early diagnosis of this syndrome is important for the selection of appropriate adjuvant treatment and family genetic counseling. The authors did not advocate the use of radiotherapy as an adjuvant treatment in desmoplastic MB diagnosed in children younger than 5 years of age. They suggested that the desmoplastic subtype of MB in children younger than 2 years of age is a major diagnostic criterion for the diagnosis of NBCCS.