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1.
World Neurosurg ; 2020 Jun 22.
Artigo em Inglês | MEDLINE | ID: mdl-32585377

RESUMO

OBJECTIVE: Two degrees of posterior cranial fossa (PCF) maldevelopment can be hypothesized in children with myelomeningocele (MMC). This paper investigate the PCF deformation by quantitative MRI analysis in MMC subjects with and without Chiari 2 malformation (CM2). METHODS: Posterior cranial fossa volume (PCFV), posterior cranial fossa brain volume (PCFBV), lengths of PCF, ventriculomegaly, level and extension of the dysraphism were analyzed in magnetic resonance imaging (MRI) of 51 newborns with MMC surgically repaired at birth (and 41 controls). The possible correlation among PCF hypoplasia, level/extension of the spinal dysraphism and ventriculomegaly was assessed. RESULTS: In MMC and CM2, the dysraphism level was above L4 in 30 and below L4 in 10 subjects. PCFV/PCFBV ratio, supraocciput and exocciput lengths were significantly reduced; foramen magnum diameters, mammillo-pontine distance and pons length were significantly increased (p < 0.05). In isolated MMC, the dysraphism level was below L4 in all cases. PCFV/PCFBV ratio and supraocciput length were significantly reduced; pons length was significantly increased (p < 0.05). The lower the MMC level, the lower the incidence of CM2. A positive correlation was found between PCF hypoplasia and MMC level above L4 (p < 0.001), while a negative correlation was found between PCF hypoplasia and MMC extension (p = 0.006), PCF hypoplasia and ventriculomegaly (p = 0.02). CONCLUSIONS: PCF hypoplasia has to be considered a dynamic maldevelopment process in the two cohorts rather than two separated entities. The level of MMC is the main but not the unique cause influencing the severity of PCF maldevelopment.

3.
Childs Nerv Syst ; 2020 May 09.
Artigo em Inglês | MEDLINE | ID: mdl-32388813

RESUMO

PURPOSE: For selected children with medically intractable epilepsy, hemispherectomy can be an excellent treatment option and its efficacy in achieving seizure freedom or reduction in seizure frequency has been shown in several studies, but patients' selection could not be straightforward and often it is taken on subjective basis. We described a multimodal approach to assess patient eligible for hemispherectomy and possibly predicting post-surgical outcomes. METHODS: We describe pre- and post-surgical clinical features along with neuroradiological results by magnetic resonance imaging (MRI), functional magnetic resonance imaging (fMRI), MR-tractography (MRT), and neurophysiological study by single and paired pulses transcranial magnetic stimulation (TMS) in a child with cerebral palsy with epileptic encephalopathy, eligible for epilepsy surgery. RESULTS: Presurgical TMS evaluation showed a lateralization of motor function on the left motor cortex for both arms, and results were confirmed by MRI studies. Interestingly, after surgery, both epilepsy and motor performances improved and TMS showed enhancement of intracortical inhibition and facilitation activity. CONCLUSION: Functional hemispherectomy is an effective treatment for drug-resistant epilepsy, and multimodal presurgical assessment may be a useful approach to guide surgeons in selecting patients. Moreover, pre- and post-surgical evaluation of these patients may enhance our understanding of brain plasticity phenomena.

4.
J Comput Assist Tomogr ; 44(3): 374-379, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32176157

RESUMO

The aim of this study was to identify quantitative tools to classify the severity of trigonocephaly to guide surgical management and predict outcome. METHODS: We reviewed high-resolution computed tomography images of 59 patients with metopic synostosis. We assessed the craniofacial sutural pattern as well as interfrontal and metopic angles, and we related the frontal angulation degree with the sutural pattern, the surgical management, and clinical outcome. RESULTS: We identified 3 groups according to the severity of trigonocephaly. No difference was found between the sutural pattern of nasion complex and severity, whereas the closure of zygomatic maxillary sutures increased with the severity degree (P < 0.05). The operative management was related to the severity degree (P < 0.001) and to the reduced age (P = 0.009). CONCLUSIONS: Interfrontal and metopic angles are complementary measurements to evaluate with high accuracy the degree of frontal angulation. In preoperative assessment, they may guide surgery decision in particular when the choice is not straightforward.


Assuntos
Suturas Cranianas/diagnóstico por imagem , Craniossinostoses/diagnóstico por imagem , Craniossinostoses/cirurgia , Tomografia Computadorizada por Raios X , Estudos de Coortes , Suturas Cranianas/patologia , Craniossinostoses/classificação , Craniossinostoses/patologia , Feminino , Humanos , Lactente , Masculino , Índice de Gravidade de Doença
5.
Childs Nerv Syst ; 36(6): 1263-1273, 2020 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31845029

RESUMO

PURPOSE: Craniofacial dysmorphology varies significantly along a wide spectrum of severity in metopic cranial synostosis (MCS). This study aimed to quantify craniofacial changes, in MCS, to investigate their relationships with the severity of trigonocephaly. METHODS: By combining the metopic ridge and interfrontal angles, we identified three groups of trigonocephaly severity (mild group n.14, moderate group n.19, severe group n.18). We perform a quantitative analysis using high-resolution CT images evaluating (1) cranial fossae dimensions; (2) vault indices and ratios: interparietal/ intercoronal (IPD/ICD), interparietal/intertemporal (IPD/ITD), cephalic index, vertico-longitudinal index; (3) orbito-facial distances (midfacial depth, maxillary height, upper facial index, orbital distances, globe protrusions), maxilla and orbital volumes; (4) supratentorial (ICV) and infratentorial (PCFV) cranial volumes and supratentorial (WBV) and infratentorial (PCFBV) brain volumes. RESULTS: In all groups, middle skull base lengths and upper midface index were increased. In moderate and severe groups: anterior hemifossa lengths were reduced, IPD/ICD and vertico-longitudinal index were changed; midfacial depth, anterior, mild, and lateral interorbital distances were reduced; globe protrusions were increased. The comparison between moderate and severe groups showed an increase of both globe protrusions and IPD/ICD. Among all groups, ICV and WBV were reduced in the severe group. CONCLUSION: This morpho-volumetric study provides new insights in understanding the craniofacial changes occurring in infants at different severity of trigonocephaly. The increase of globe protrusions and the reduction of supratentorial volumes found in the severe group reflect the severity of trigonocephaly; these findings might have a clinical and surgical relevance.

6.
Childs Nerv Syst ; 35(10): 1649-1650, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31392454
7.
Childs Nerv Syst ; 35(8): 1351-1356, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-31190181

RESUMO

PURPOSE: This study aimed to develop a novel approach to assess the severity of skull dysmorphology in infants with isolated sagittal synostosis (ISS) and its relationship with the surgical results. METHODS: We divided 66 infants with ISS into three groups by combining the scaphocephalic (SSI-A) and platycephalic (VLI) indices as descriptors of the relation between length, width, and height. We evaluated each skull for morphology as hyperdolichocephalic (< 66%) versus dolichocephalic (66-77%) and as hyperplatycephalic (< 78%) versus platycephalic skull (78-85%). A score system was developed as follows: 2 points for values < 66% and < 78% and 1 point for values between 66 and 77% and 78 and 85% in SSI-A and VLI, respectively. The overall score was calculated and it was used to classify our patients on a 4-point ordinal scale, according to the severity of head shape (2 = mild, 3 = moderate, 4 = severe). RESULTS: Thirty-two infants resulted in mild group, 17 in moderate group, and 17 in severe group. SSI-A and VLI were reduced according to the severity of ISS. We demonstrated a positive correlation between SSA-A and VLI in mild subgroup of patients while we found a negative correlation between SSA-A and VLI in moderate and in severe subgroups. Moreover, a positive correlation was found between severe subgroup and Sloan III class of surgical results. CONCLUSION: This study describes a simple tool to better classify infants with ISS, considering the three-dimensional morphology of the skull, because it evaluates both the dolichocephalic and platycephalic component.

8.
Childs Nerv Syst ; 2019 Jun 14.
Artigo em Inglês | MEDLINE | ID: mdl-31201497

RESUMO

BACKGROUND: The association between preoperative language impairment and development of cerebellar mutism after surgical treatment of posterior fossa tumors has gained increasing interest in recent years based on the concept that both local compression/infiltration of the infratentorial periventricular anatomical structures involved in speech and language, as well as an increased distraction of supratentorial periventricular anatomical structures, due to an associated hydrocephalus, involved in the coordination of speech circuits, might lead to the subclinical presence of language disturbances already at diagnosis, predicting the development of a cerebellar mutism syndrome after tumor removal. METHODS: A thorough review of the literature on the subject has been performed, together with a review of our institutional experience reporting the related long-term (10 years) results. RESULTS AND CONCLUSIONS: According to our institutional experience, 20/70 (28.5%) children presented preoperative language impairment and developed cerebellar mutism after surgery. A residual impairment persists in 75% of the cases at a follow-up of 2 years, and in 50% of the cases at 10 years, a finding which actually depicts cerebellar mutism no more as a transient deficit but among long-term sequelae. The appearance of complex dysarthria in the postoperative period is a negative prognostic factor for the long-term persistence of speech disturbances.

9.
Childs Nerv Syst ; 35(10): 1701-1709, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31227858

RESUMO

PURPOSE: The association between Chiari type I malformation (CIM) and hydrocephalus raises a great interest because of the still unclear pathogenesis and the management implications. The goal of this paper is to review the theories on the cause-effect mechanisms of such a relationship and to analyze the results of the management of this condition. METHODS: A review of the literature has been performed, focusing on the articles specifically addressing the problem of CIM and hydrocephalus and on the series reporting about its treatment. Also, the personal authors' experience is briefly discussed. RESULTS: As far as the pathogenesis is concerned, it seems clear that raised intracranial pressure due to hydrocephalus can cause a transient and reversible tonsillar caudal ectopia ("pressure from above" hypothesis), which is something different from CIM. A "complex" hypothesis, on the other hand, can explain the occurrence of hydrocephalus and CIM because of the venous engorgement resulting from the hypoplasia of the posterior cranial fossa (PCF) and the occlusion of the jugular foramina, leading to cerebellar edema (CIM) and CSF hypo-resorption (hydrocephalus). Nevertheless, such a mechanism can be advocated only in a minority of cases (syndromic craniosynostosis). In non-syndromic CIM subjects, the presence of hydrocephalus could be explained by an occlusion of the basal CSF pathways, which would occur completely in a minority of cases (only 7-10% of CIM patients show hydrocephalus) while it would be partial in the remaining cases (no hydrocephalus). This hypothesis still needs to be demonstrated. As far as the management is concerned, the strategy to treat the hydrocephalus first is commonly accepted. Because of the "obstructive" origin of CIM-related hydrocephalus, the use of endoscopic third ventriculostomy (ETV) is straightforward. Actually, the analysis of the literature, concerning 63 cases reported so far, reveals very high success rates of ETV in treating hydrocephalus (90.5%), CIM (78.5%), and syringomyelia symptoms (76%) as well as in giving a radiological improvement of both CIM (74%) and syringomyelia (89%). The failures of ETV were not attributable to CIM or syringomyelia. Only 11% of cases required PCF decompression after ETV. CONCLUSIONS: The association between CIM and hydrocephalus probably results from different, multifactorial, and not yet completely understood mechanisms, which place the affected patients in a peculiar subgroup among those constituting the heterogeneous CIM population. ETV is confirmed as the best first approach for this subset of patients.

11.
Childs Nerv Syst ; 34(9): 1725-1733, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29761256

RESUMO

PURPOSE: The effects of premature fusion of one coronal suture cause skull and orbital alterations in term of side-to-side asymmetry. This study aimed to quantify the cranio-orbital complex changes related to the severity of skull base dysmorphology in patients with unicoronal synostosis. METHODS: Twenty-four infants affected by unicoronal synostosis were subdivided in three subgroups according to the severity of skull base deformity and their high-resolution CT images were quantitatively analyzed (groups IIa, IIb, III). Dimensions of cranial fossae, intracranial volume (ICV), ICV synostotic and ICV non synostotic side, whole brain volume (WBV), orbital volumes (OV), ICV/WBV, ICVsynostotic/ICVnon-synostotic, and OVsynostotic/OVnon-synostotic were evaluated. RESULTS: Asymmetry and reduction in the growth of the anterior and middle fossae were found in all groups while asymmetry of the posterior cranial fossa was found only in IIb and III groups. In all groups, ICV, WBV, and ICV/WBV were not significantly different while ICVsynostotic/ICVnon-synostotic and OVsynostotic/OVnon-synostotic resulted significant difference (p < 0.05). ICVsynostotic side resulted reduction only in group III. OV on the synostotic side was not significantly reduced although a trend in progressively reducing volumes was noted according to the severity of the group. CONCLUSION: Skull and orbital changes revealed a side-to-side asymmetry but the effects of the premature synostosis were more severe in group III suggesting an earlier timing of premature unicoronal synostosis in group III with respect to the other groups. The assessment of the skull base deformity might be an indirect parameter of severity of skull orbital changes and it might be useful for surgical planning.


Assuntos
Suturas Cranianas/diagnóstico por imagem , Craniossinostoses/diagnóstico por imagem , Órbita/diagnóstico por imagem , Base do Crânio/diagnóstico por imagem , Tomografia Computadorizada por Raios X/normas , Suturas Cranianas/cirurgia , Craniossinostoses/cirurgia , Feminino , Humanos , Lactente , Masculino , Órbita/cirurgia , Estudos Retrospectivos , Base do Crânio/cirurgia , Tomografia Computadorizada por Raios X/métodos
12.
Bone ; 112: 58-70, 2018 07.
Artigo em Inglês | MEDLINE | ID: mdl-29674126

RESUMO

Nonsyndromic craniosynostosis (NCS) is the premature ossification of skull sutures, without associated clinical features. Mutations in several genes account for a small number of NCS patients; thus, the molecular etiopathogenesis of NCS remains largely unclear. Our study aimed at characterizing the molecular signaling implicated in the aberrant ossification of sutures in NCS patients. Comparative gene expression profiling of NCS patient sutures identified a fused suture-specific signature, including 17 genes involved in primary cilium signaling and assembly. Cells from fused sutures displayed a reduced potential to form primary cilia compared to cells from control patent sutures of the same patient. We identified specific upregulated splice variants of the Bardet Biedl syndrome-associated gene 9 (BBS9), which encodes a structural component of the ciliary BBSome complex. BBS9 expression increased during in vitro osteogenic differentiation of suture-derived mesenchymal cells of NCS patients. Also, Bbs9 expression increased during in vivo ossification of rat sutures. BBS9 functional knockdown affected the expression of primary cilia on patient suture cells and their osteogenic potential. Computational modeling of the upregulated protein isoforms (observed in patients) predicted that their binding affinity within the BBSome may be affected, providing a possible explanation for the aberrant suture ossification in NCS.


Assuntos
Cílios/metabolismo , Suturas Cranianas/patologia , Craniossinostoses/genética , Proteínas de Neoplasias/genética , Osteogênese/genética , Processamento Alternativo/genética , Animais , Diferenciação Celular/genética , Proteínas do Citoesqueleto , Éxons/genética , Feminino , Perfilação da Expressão Gênica , Regulação da Expressão Gênica , Técnicas de Silenciamento de Genes , Proteínas Hedgehog/metabolismo , Humanos , Lactente , Masculino , Proteínas de Neoplasias/química , Isoformas de Proteínas/química , Isoformas de Proteínas/genética , Isoformas de Proteínas/metabolismo , Ratos Wistar , Transdução de Sinais
13.
J Neurosurg Pediatr ; 21(2): 119-123, 2018 02.
Artigo em Inglês | MEDLINE | ID: mdl-29171799

RESUMO

OBJECTIVE Few cases of cerebellopontine angle (CPA) arachnoid cysts in pediatric patients have been described in the literature, and in only 2 of these cases were the patients described as suffering from hearing deficit. In this article, the authors report on 3 pediatric patients with CPA arachnoid cysts (2 with hearing loss and 1 with recurrent headaches) who underwent neurosurgical treatment at the authors' institution. METHODS Four pediatric patients were diagnosed with CPA arachnoid cysts at the International Neuroscience Institute during the period from October 2004 through August 2012, and 3 of these patients underwent surgical treatment. The authors describe the patients' clinical symptoms, the surgical approach, and the results on long-term follow-up. RESULTS One patient (age 14 years) who presented with headache (without hearing deficit) became asymptomatic after surgical treatment. The other 2 patients who underwent surgical treatment both had hearing loss. One of these children (age 9 years) had recent-onset hypacusia and experienced complete recovery immediately after the surgery. The other (age 6 years) had a longer history (2 years) of progressive hearing loss and showed an interruption of the deficit progression and only mild improvement at the follow-up visit. CONCLUSIONS CPA arachnoid cysts are uncommon in pediatric patients. The indication and timing of the surgical treatment are fundamental, especially when a hearing deficit is present.


Assuntos
Cistos Aracnóideos/cirurgia , Ângulo Cerebelopontino , Perda Auditiva/etiologia , Procedimentos Neurocirúrgicos/métodos , Adolescente , Cistos Aracnóideos/complicações , Criança , Pré-Escolar , Feminino , Transtornos da Cefaleia/etiologia , Humanos , Imagem por Ressonância Magnética , Masculino , Cuidados Pós-Operatórios , Resultado do Tratamento , Vômito/etiologia
14.
Neurosurgery ; 82(4): 465-472, 2018 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-28973391

RESUMO

BACKGROUND: Postoperative seizures (PSs) after neurosurgical operations are common but little is known about the role of surgical brain incision on their genesis. This topic has not been addressed so far. OBJECTIVE: To verify if the corticotomy affects the risk of PSs and postoperative epilepsy (PE) in children. METHODS: One hundred forty-three consecutive pediatric cases operated on for supratentorial lesions at the same institution in the last 15 yr have been retrospectively reviewed by dividing them into group A, 68 children who required brain corticotomy mainly for hemispheric tumors, and group B, 75 children treated through extracortical approaches mainly for suprasellar and optic tumors. Patients with possible "epileptic" biases, like preoperative seizures, were excluded. RESULTS: No significant differences have been found between group A and B as far as incidence of PSs (11.7% vs 14.5%) and PE (4.5% vs 6.5%), timing, and type of seizures are concerned after a mean 6.8 yr follow-up. The size of corticotomy in group A (<3 cm2 vs >3 cm2) had no impact on epileptogenesis as well as the other variables considered in both groups (age, sex, extent of lesion resection). CONCLUSION: This study shows that the surgical cortical "trauma" would not represent a risk factor for PSs and PE. According to the present analysis and the literature, other causes seem to be involved (namely, electrolytic imbalance and brain gliosis). This information is important for preoperative surgical planning and postoperative management. A validation by both adult series and prospective studies is needed.


Assuntos
Procedimentos Neurocirúrgicos/efeitos adversos , Complicações Pós-Operatórias/epidemiologia , Convulsões/etiologia , Neoplasias Supratentoriais/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Incidência , Lactente , Masculino , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Risco , Convulsões/epidemiologia
15.
Childs Nerv Syst ; 33(10): 1759-1768, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29149388

RESUMO

Cranial repair after traumatic brain injury in children is still burdened by unsolved problems and controversial issues, mainly due to the high rate of resorption of autologous bone as well as the absence of valid alternative material to replace the autologous bone. Indeed, inert biomaterials are associated to satisfactory results in the short period but bear the continuous risk of complications related to the lack of osteointegration capacity. Biomimetic materials claiming osteoconductive properties that could balance their mechanical limits seem to allow good cranial bone reconstruction. However, these results should be confirmed in the long term and in larger series. Further complicating factors that may affect cranial reconstruction after head injury should be identified in the possible associated alterations of CSF dynamics and in difficulties to manage the traumatic skin lesion and the surgical wound, which also might impact on the cranioplasty outcome. All the abovementioned considerations should be taken into account when dealing with the cranial reconstruction after decompressive craniectomy in children.


Assuntos
Lesões Encefálicas Traumáticas/cirurgia , Craniectomia Descompressiva/métodos , Procedimentos Cirúrgicos Reconstrutivos/métodos , Transplante Ósseo , Lesões Encefálicas Traumáticas/diagnóstico por imagem , Criança , Pré-Escolar , Humanos , Imagem por Ressonância Magnética , Próteses e Implantes , Fatores de Tempo
16.
Childs Nerv Syst ; 33(12): 2211, 2017 12.
Artigo em Inglês | MEDLINE | ID: mdl-28988361

RESUMO

The published version of this article unfortunately contained an error. All names of the authors have been published incorrectly. Given in this article are the corrected author names.

17.
World Neurosurg ; 104: 1050.e13-1050.e18, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-28559076

RESUMO

BACKGROUND: Intracranial pial arteriovenous fistulas (PAVFs) are direct communications between the arterial and venous system of the brain, with the characteristic absence of a plexiform nidus, as seen in the classic cerebral arteriovenous malformations. These vascular malformations, usually occurring in the pediatric population, very rarely are diagnosed in utero, because of a lack of understanding of the condition and because they may be hard to visualize. CASE DESCRIPTION: We report a rare case of a mass-effect PAVF diagnosed with fetal magnetic resonance imaging, involving the right cerebral hemisphere, fed by a pericallosal artery and associated with a giant venous dilatation. The PAVF initially was managed by the endovascular embolization. The recruitment of a middle cerebral artery feeder and the rapidly enlarging size of the venous pouch with mass effect required subsequent surgery. CONCLUSIONS: The 2-stage multimodal treatment resulted in complete disappearance of the PAVF without complications.


Assuntos
Fístula Arteriovenosa/diagnóstico por imagem , Hidrocefalia/diagnóstico por imagem , Malformações Arteriovenosas Intracranianas/diagnóstico por imagem , Pia-Máter/irrigação sanguínea , Adulto , Angiografia Digital , Fístula Arteriovenosa/complicações , Fístula Arteriovenosa/cirurgia , Angiografia Cerebral , Terapia Combinada , Procedimentos Endovasculares , Feminino , Humanos , Hidrocefalia/etiologia , Recém-Nascido , Malformações Arteriovenosas Intracranianas/complicações , Malformações Arteriovenosas Intracranianas/cirurgia , Angiografia por Ressonância Magnética , Imagem por Ressonância Magnética , Masculino , Procedimentos Neurocirúrgicos , Gravidez , Segundo Trimestre da Gravidez , Diagnóstico Pré-Natal , Falha de Tratamento , Ultrassonografia Doppler em Cores , Ultrassonografia Pré-Natal
18.
Acta Neurochir Suppl ; 124: 143-148, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28120066

RESUMO

BACKGROUND: Myelomeningocele (MMC) is a rare but severe spinal defect resulting from a failed neurulation process. Surgical repair is a delicate procedure that needs accurate neuroanatomical knowledge and adequate surgical planning. MATERIALS AND METHODS: The authors report on the main problems of the surgical management of this type of dysraphism. The technique used for reconstruction, and the management strategies, are analyzed according to the authors' experience with more than 600 cases, in comparison with the relevant literature. RESULTS: Tip and tricks of more than 30 years of MMC surgical management are reported, with special mention of the timing of surgery, the management of early hydrocephalus, and the principles of the repair, from the dissection to the closure of five separate layers (arachnoid, dura, muscular fascia, subcutaneous layer, skin). CONCLUSIONS: Accuracy and attention to the reconstruction are crucial for achieving good results and avoiding perioperative complications.


Assuntos
Terapias Fetais/métodos , Hidrocefalia/cirurgia , Meningomielocele/cirurgia , Feminino , Humanos , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/etiologia , Recém-Nascido , Meningomielocele/complicações , Meningomielocele/diagnóstico por imagem , Procedimentos Neurocirúrgicos , Gravidez , Procedimentos Cirúrgicos Reconstrutivos , Fatores de Tempo , Ultrassonografia Pré-Natal , Derivação Ventriculoperitoneal
19.
Childs Nerv Syst ; 33(2): 227-231, 2017 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-27921213

RESUMO

BACKGROUND: Primary intracranial anaplastic gangliogliomas are rare tumors in the pediatric patient group. Most of them present with symptoms of elevated pressure or symptomatic epilepsy. Extraaxial location is far more common than axial location. On MRI examination, they mimic pilocytic astrocytomas. The outcome after surgery depends mainly on the possible amount of surgical resection, and oncological therapy is necessary to prevent recurrence of the disease. CASE REPORT: An 11-year-old boy presented with headache and double vision due to obstructive hydrocephalus. MRI of the brain revealed an axial partially contrast enhancing lesion in the quadrigeminal plate extending from the cerebellum to the pineal gland and causing hydrocephalus. Subtotal removal of the lesion was performed, and the diagnosis of an anaplastic ganglioglioma was established and confirmed by the reference center. At the latest follow up (3 months), the boy is without any neurological symptoms and scheduled for radiation therapy as well as chemotherapy.


Assuntos
Neoplasias Encefálicas/complicações , Ganglioglioma/complicações , Antineoplásicos/uso terapêutico , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Criança , Ganglioglioma/diagnóstico por imagem , Ganglioglioma/cirurgia , Humanos , Indóis/uso terapêutico , Imagem por Ressonância Magnética , Masculino , Sulfonamidas/uso terapêutico , Vemurafenib
20.
Br J Neurosurg ; 31(4): 464-467, 2017 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-27619551

RESUMO

INTRODUCTION: Cavum septi pellucidi (CSP) cysts have a very low incidence (0.04%). Symptomatic patients usually present aspecific symptoms. For this reason, the management of these patients is still debated. MATERIALS AND METHODS: We selected the case of a ten year old patient, with a clinical history of frontal morning headaches and difficulty in concentration. Brain MRI documented a septum pellucidum cyst and a moderate biventricular dilation. We submitted the case, and a questionnaire concerning indications to surgery and management options to an international group of 54 pediatric neurosurgeons, analyzing the results and comparing them with the current literature. RESULTS: The majority of the participants (50%) indicated as appropriate at the early stage only a clinical observation. In case of persistence of clinical symptoms, 58% opted for intracranial pressure (ICP) monitoring, which, if raised, was considered by 91% as an adequate indication to proceed with surgical treatment. A total of 98% of the participants indicated endoscopic fenestration of the cyst as the preferred surgical strategy. CONCLUSIONS: The management of symptomatic patients with CSP cyst is controversial. Our results suggest that in most of the patients with aspecific symptoms, clinical observation and eventually ICP monitoring are adequate to identify patients for surgery.


Assuntos
Neoplasias Encefálicas/cirurgia , Cistos do Sistema Nervoso Central/cirurgia , Septo Pelúcido/cirurgia , Criança , Tratamento Conservador/estatística & dados numéricos , Transtornos da Cefaleia/etiologia , Humanos , Imagem por Ressonância Magnética , Neuroendoscopia/estatística & dados numéricos , Manejo da Dor , Padrões de Prática Médica/estatística & dados numéricos , Inquéritos e Questionários , Terapia Trombolítica/estatística & dados numéricos
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