Gustavo Soto-Ares

Cavernous malformations after cerebral irradiation during childhood: report of nine cases

IntroductionCavernous hemangioma is increasingly recognized as a late complication of cerebral irradiation (CI); however, the significance of the problem, especially the risk of hemorrhage, is not documented in the literature. In order to discover this, we reviewed our experience of radiation-induced cavernous hemangiomas (RICH) in patients who had received CI during childhood.MethodsWe reviewed retrospectively our pediatric database of 965 brain tumors, 419 of which were irradiated between 1964 and 2003. We collected nine cases of RICH diagnosed in patients who had received CI during childhood.ResultsThe CI dosage ranged between 25 and 55xa0Gy. The interval between CI and the diagnosis of RICH ranged from 4 to 22 years. The RICH was the cause of brain hemorrhage in five cases, three of which required surgical evacuation.DiscussionRadiation-induced cavernous hemangiomas are an underestimated problem, and systematic screening of irradiated patients with gradient-echo MRI will probably yield more asymptomatic cases. We think that not all RICH require surgery, but only those responsible for intracerebral hemorrhage or that show radiological progression. ConclusionWith respect for the long interval between CI and diagnosis of RICH, we advise control with MRI, including gradient-echo sequence, as late as 15xa0years after CI, and closer monitoring of asymptomatic RICH.

Traumatic intracranial hemorrhage in newborns

IntroductionNeonatal traumatic head injuries (NTHI) can be life-threatening and require aggressive treatment. The indications, techniques, and results of brain decompression are not well defined in the literature.MethodsWe studied prospectively cases of NTHI with intracranial traumatic lesions; skull fractures without underlying lesions were not included. We treated 17 cases of NTHI: 7 patients had a subdural hematoma, 3 had an extradural hematoma, and the others had subarachnoid hemorrhage. Surgical evacuation of intracranial clots was performed in 7 cases, by needle aspiration in 5 and by craniotomy in 2 patients with extradural hematomas.ResultsThe outcome was favorable in all but one patient, who had hemophilia A, and died of rebleeding at the age of 2xa0months.Conclusion Surgical decompression of intracranial hematomas due to NTHI is often unnecessary; however, it may be required in emergency because of poor clinical tolerance. Whenever possible, percutaneous needle aspiration is the treatment of choice.

Posterior fossa volume increase after surgery for Chiari malformation Type I: a quantitative assessment using magnetic resonance imaging and correlations with the treatment response.

OBJECTnThe aim of this paper was to measure the posterior fossa (PF) volume increase resulting from a given-sized occipital craniectomy in Chiari malformation Type I surgery and to analyze its correlations with the PF size and the treatment response, with the perspective of tailoring the amount of bone removal to the patient-specific PF dimensions.nnnMETHODSnBetween January 2005 and June 2006, 11 adult patients with symptomatic Chiari malformation Type I underwent a standardized PF decompression. A prospective evaluation with clinical examination, functional grading, and MR imaging measurement protocols was performed pre- and postoperatively. A method is reported for the measurement of PF volume (PFV) after surgery. The degree of PFV increase was compared with the preoperative size of the PF and with the clinical outcome.nnnRESULTSnAll 11 patients improved postoperatively, with complete and partial recovery in 4 and 7 patients, respectively. No postoperative complication occurred after a mean follow-up period of 45 months. The mean relative increase in PFV accounted for 10% (range 1.5%-19.7%) of the initial PFV; the increase was greater in cases in which the PF was small (r = -0.52, p = 0.09) and the basiocciput was short (r = -0.37, p = 0.2). A statistically significant positive correlation was found between the degree of PFV increase and the treatment response (p = 0.014); complete recovery was observed with a PFV increase of 15% and partial recovery with an increase of 7%.nnnCONCLUSIONSnThe treatment response is significantly influenced by the degree of PFV increase, which is dependent on the size of the PF and the extent of the craniectomy, suggesting that the optimal patient-specific PFV increase could be predicted on the basis of preoperative MR imaging and enhancing the perspective that the craniectomy size could be tailored to the individual PFV.

Fluid-attenuated inversion recovery (FLAIR) sequences for the assessment of acute stroke: inter observer and inter technique reproducibility.

Background and purposeDiffusion–weighted magnetic resonance (MR) imaging (DWI), and three–dimensional (3D) time–of–flight (TOF) MR angiography (MRA), are highly sensitive for the early detection of stroke and arterial occlusion. However, only a few studies have evaluated the sensitivity of conventional MR sequences that are usually included in the imaging protocol. The aim of this study was to evaluate interobserver and intertechnique reproducibility of Fluid–Attenuated Inversion Recovery (FLAIR) sequences for the diagnosis of early brain ischemia and arterial occlusion.MethodsOver a 30–month period, brain MR examinations were performed in 34 patients within 12 hours after stroke onset. Imaging protocol included FLAIR sequences, DWI and 3D TOF MRA. Ten observers including radiologists and neurologists, performed separately a visual interpretation of FLAIR images for the detection of brain ischemia and arterial occlusion seen as an arterial high signal. DWI and 3D TOF MRA were used as reference and interpreted independently by two senior radiologists. Interobserver agreement was assessed for image quality, detectability and conspicuity of lesions whereas intertechnique agreement was only judged for lesion detectability.ResultsOn FLAIR sequences, interobserver agreement for the detection of brain ischemia and arterial occlusion was excellent (κ = 0.81 and 0.87 respectively). The concordance between FLAIR and DWI sequences for the detection of brain ischemia and between FLAIR and 3D TOF MRA for the detection of arterial occlusion were judged as excellent for all observers (κ = 0.91 and 0.89 respectively).ConclusionAlthough DWI is the most sensitive technique with which to detect acute stroke, FLAIR imaging may also be useful to demonstrate both acute ischemia and arterial occlusion with an excellent interobserver reproducibility.

Disappearance of a middle cerebral artery aneurysm associated with Moyamoya syndrome after revascularization in a child: case report.

IntroductionPediatric Moyamoya disease is rarely associated with intracranial aneurysms. We report a case of a 7-year-old girl with an antecedent of persistent craniopharyngeal canal, who presented with a history of choreiform movements.Materials and methodsA Moyamoya disease was found with an unruptured left middle cerebral artery aneurysm on her first angiography. Conservative treatment was chosen for the aneurysm and she underwent indirect revascularization by encephalosynangiosis using the multiple bur-hole technique for her Moyamoya disease. Abnormal movements were improved. Control angiogram at 6xa0months showed development of intracranial–extracranial anastomoses with complete resolution of the aneurysm. Aneuryms including the major arteries of the basal arterial circle occur as a by-product of the high velocity and blood flow secondary to the arterial stenosis. Blood flow modification after revascularization often lead to spontaneous regression and disappearance of these aneurysms.ConclusionTherefore, a conservative treatment of these proximal aneurysms must be chosen after encephalosynangiosis.

Progressive myelopathy due to meningeal thickening in shunted patients: description of a novel entity and the role of surgery

IntroductionSpinal cord compression due to meningeal thickening is a rare occurrence in shunted patients. Because of the long delay to clinical onset, this complication has not been identified as yet.AimsWe report on nine cases of shunt-related progressive myelopathy due to meningeal thickening (SPMMT).Materials and methodsWe reviewed our database of shunted children, for cases having developed progressive tetraparesis due to cervical meningeal thickening.ResultsWe identified nine observations of SPMMT, eight of these with hydrocephalus due to neonatal meningitis; the last case had Dandy–Walker malformation shunted at birth and suffered postoperative meningitis. The age of clinical onset of myelopathy was between 6 and 20xa0years (median 12.8). All patients presented with slowly progressive walking difficulties with falls and no spinal pain. Magnetic resonance imaging (MRI) showed typically a thickened dura mater with collapse of the arachnoid space, compensatory expansion of the epidural fat, and T2 hyperintensity in the spinal cord. We operated on seven patients for surgical decompression and arachnoidolysis: One died postoperatively because of shunt malfunction, and two others died later of complications of tetraplegia. Three patients were aggravated after surgery, three experienced partial improvement, but one of these subsequently deteriorated again.ConclusionSPMMT appears to be a novel and well-defined clinical and pathological entity; its pathological and radiological features are stereotyped; however, the diagnosis is delayed because of the slow pace of the disease. Although surgical decompression may be the only option, its results were poor in our experience; earlier surgery might improve this grim prognosis.

Conduite à tenir devant une première crise convulsive

Resume La conduite a tenir neuroradiologique devant une premiere crise convulsive est determinee par les donnees obtenues par l’anamnese, l’examen neurologique, la biologie sanguine et la realisation d’un electro-encephalogramme (EEG). Il s’agit dans un premier temps de dissocier la crise convulsive vraie d’une manifestation clinique d’apparence semblable, d’exclure une crise convulsive occasionnelle declenchee par un facteur exogene puis, de s’orienter vers une etiologie organique responsable de la crise vraie. Cette derniere peut etre lesionnelle ou inaugurale de l’epilepsie. La realisation d’une imagerie cerebrale en urgence n’est indiquee que chez les patients presentant un deficit neurologique focal, une alteration de l’etat de conscience persistante ou devant des elements cliniques et biologiques faisant suspecter une pathologie cerebrale vasculaire ou infectieuse. Le scanner est l’imagerie la plus facilement accessible en urgence pour identifier la lesion causale. Son interet est d’orienter la prise en charge therapeutique immediate et de differer la realisation de l’IRM. Toutefois, une IRM cerebrale doit etre envisagee en urgence lorsque le scanner est peu contributif malgre un tableau clinique grave ou en cas de suspicion de thrombose veineuse cerebrale. Les modalites d’examen sont alors fonction des donnees cliniques, du type d’epilepsie suspecte et de l’âge du patient.

Natural history of traumatic meningeal bleeding in infants: semiquantitative analysis of serial CT scans in corroborated cases

BackgroundThe natural history of posttraumatic meningeal bleeding in infants is poorly documented, and the differences between inflicted head injury (IHI) and accidental trauma (AT) are debated. Autopsy findings have suggested that anoxia also plays a role in bleeding; however, these findings may not reflect what occurs in live trauma patients.PurposeWe studied the natural history of traumatic meningeal bleeding in infants using serial computed tomography (CT) scans in corroborated IHI and AT.Materials and methodsFrom our prospective series, we selected corroborated cases (confessed IHI or AT having occurred in public), who underwent at least three CT scans in the acute phase. We performed a semiquantitative analysis of meningeal bleeding using a four-tier scale (absent, faint, frank, and thick) derived from the Fisher grading for aneurysmal bleeding in four regions of interest (convexity, falx cerebri, sagittal sinus, and tentorium cerebelli).ResultsWe studied 20 cases: ten IHI and ten AT. Bleeding was maximal at the convexity initially, then increased along the falx and sagittal sinus, and then along the tentorium. Decrease and disappearance of blood was variable according to the site and the initial quantity of blood. We found no difference between IHI and AT.ConclusionOur findings suggest that the primary site of meningeal bleeding in infantile head trauma is the convexity of the brain; blood cells then migrate toward the midline following the flow of cerebrospinal fluid circulation and inferiorly following gravity. The pattern of bleeding in traumatic cases appears similar in IHI and AT but different from anoxic lesions.

Imagerie par résonance magnétique du retard mental non spécifique

Resume Le retard mental (RM) est considere idiopathique ou non specifique lorsqu’aucune etiologie n’est identifiee malgre un bilan exhaustif, clinique, biologique, metabolique et genetique. Chez ces patients, l’indication d’une imagerie cerebrale par resonance magnetique (IRM) est posee devant la presence d’anomalies du perimetre crânien, de malformations crâniofaciales et somatiques, de stigmates neurocutanes, d’epilepsie, de signes neurologiques focaux et de troubles du comportement et/ou du developpement. En effet, les anomalies encephaliques font desormais partie de l’un des groupes etiologiques principaux. L’exploration par IRM doit comporter des coupes axiales sur l’ensemble de l’encephale, des coupes sagittales centrees sur la ligne mediane et des coupes coronales centrees sur la fosse posterieure et/ou l’ensemble de l’encephale. Elle permet d’identifier des malformations cerebrales majeures et/ou mineures et alors multiples. Dans la litterature, les anomalies les plus frequemment decrites interessent : 1/ le corps calleux (aspect dysplasique, court, verticalise, hypoplasique), 2/ le septum pellucidum (cavum du septum), 3/ le systeme ventriculaire (ventriculomegalie), 4/ le cortex cerebral (dysplasies corticales), 5/ le cervelet (hypoplasie) et 6/ les espaces liquidiens pericerebraux (elargissement). L’etude de la population etudiee dans notre institution a revele la presence de dysplasies corticales, interessant le cervelet et le vermis cerebelleux, anomalies jusqu’alors non decrites dans le RM. L’IRM permet d’identifier, des anomalies morphologiques mineures et multiples. La plupart de celles-ci, considerees classiquement des variantes de la normale, se sont revelees des marqueurs de dysgenesie cerebrale et, a l’heure actuelle, representent la seule anomalie observee au cours du bilan des enfants deficients. Leur role dans l’etiopathogenie du RM est en cours d’evaluation.

Neuropsychological Evaluation and Follow-Up of Children with Cerebellar Cortical Dysplasia.

Aimu2002 To describe neuropsychological disturbances and the developmental course associated with cerebellar cortical dysplasia (CCD).