Carotid rete mirabile and pseudoxanthoma elasticum: an accidental association?

We report the case of a young female patient with a transient amaurosis due to a carotid rete mirabile (CRM), a rare congenital carotid malformation, and pseudoxanthoma elasticum (PXE), an inherited autosomal recessive systemic metabolic disorder characterised by fragmentation and mineralisation of elastic fibres in connective tissues (skin, eyes) and the vascular system. CRM is a rare form of intracranial carotid malformation whose association with PXE (6 cases at present) would appear not to be accidental. This observation suggests a new link between congenital arterial remodelling and the PXE

Transtemporal-transchoroidal approach and its transamygdala extension to the posterior chiasmatic cistern and diencephalo-mesencephalic lesions

The aim of this report is to illustrate the successful surgical management of five patients with suprasellar retrochiasmatic and diencephalo-mesencephalic tumours, using a trans-amygdala extension of the trans-temporal/trans-choroidal fissure approach.The procedure is described for five surgically treated patients with such lesions that includes three retrochiasmatic craniopharyngiomas, one hypothalamic hamartoma, and one pilocytic astrocytoma in the left crus cerebri. The management procedure included computed tomography scan (CT scan) and magnetic resonance imaging (MRI). The trans-temporal/trans-choroidal fissure approach enabled us to perform total tumour resection in four patients and a subtotal resection in one. Some technical aspects and pitfalls of the procedure are discussed.This method creates optimum conditions for a radical excision of various suprasellar retrochiasmatic and diencephalo-mesencephalic tumours without mortality and only minimum morbidity. However, no single surgical approach can provide access to the entire variety of tumours located in the suprasellar retrochiasmatic and diencephalo-mesencepalic region. Surgical approaches must be tailored to the site, type of lesion, and its extensions. This method is only another surgical option. Its successful use requires a familiarity with the anatomy of these regions and an understanding of its specificity for a radical excision of some selected tumours, as well as its limitations to access others types of lesions. Although, it seems effective, this approach needs to be evaluated by further experience, owing to the small number of patients reported in this series

Imagerie par résonance magnétique chez les patients porteurs de stimulateurs cardiaques et de défibrillateurs automatiques implantables : revue de la littérature. [Magnetic resonance imaging in patients with pacemakers and implantable cardioverter-defibrillators: a systematic review].

International audienceThe presence of a pacemaker or an implantable cardioverter-defibrillator was historically considered a contraindication to magnetic resonance imaging (MRI), due to the risks for both patient and device: reed-switch closure responsible for asynchronous pacing, inhibition of pacing, rapid ventricular pacing, heating on the lead tip or even device displacement... However, many recent studies demonstrate that if MRI is crucial for the management of the patient, it can be performed under specific monitoring and scanning conditions and after device reprogramming. The growing implication of device constructors in constructing a MRI safety device will perhaps extend in the future the indications of this imaging modality in implanted patients

Remote Cerebellar Hemorrhage Following Thoracic Spinal Surgery

A 51-year-old man underwent surgery for ossification of the ligamentum flavum at the T9-T10 levels. Intraoperatively, the dura was opened unintentionally and a subcutaneous suction drain was placed. The patient complained of severe headache and nausea postoperatively. Brain computed tomography obtained 3 days after the surgery demonstrated remote cerebellar hemorrhage and hydrocephalus. Suboccipital decompression, C1 laminectomy, and ventriculostomy were performed and his symptoms subsided 2 months later. Remote cerebellar hemorrhage following spinal surgery is extremely rare, but may occur after any type of spinal surgery resulting in dural tear or intradural manipulation. Early diagnosis is particularly important for the treatment of remote cerebellar hemorrhage following spinal surgery

Sporadic intracranial haemangioblastomas: surgical outcome in a single institution series

International audienceBACKGROUND: 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