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1.
Childs Nerv Syst ; 2020 Feb 04.
Artigo em Inglês | MEDLINE | ID: mdl-32020269

RESUMO

INTRODUCTION: The prognosis of diffuse intrinsic pontine glioma (DIPG) is poor. The role of biopsy in DIPG remains controversial since the diagnosis may be established with imaging alone. Recent advances in understanding molecular biology and targeting of brain tumors have created a renewed interest in biopsy for DIPG. The Neurosurgery Working Group (NWG) of the SIOP-Europe Brain Tumor Group (BTG) undertook a survey among international pediatric neurosurgeons to define their current perceptions and practice regarding DIPG biopsy. METHODS: The NWG developed a 20-question survey which was emailed to neurosurgeons in the International Society for Pediatric Neurosurgery (ISPN). The questionnaire included questions on diagnosis, indications, and techniques for biopsy, clinical trials, and healthcare infrastructure. RESULTS: The survey was sent to 202 neurosurgeons and 73 (36%) responded. Consensus of > 75% agreement was reached for 12/20 questions, which included (1) radiological diagnosis of DIPG is sufficient outside a trial, (2) clinical trial-based DIPG biopsy is justified if molecular targets are investigated and may be used for treatment, and (3) morbidity/mortality data must be collected to define the risk:benefit ratio. The remaining 8/20 questions proved controversial and failed to reach consensus. CONCLUSIONS: Routine DIPG biopsy continues to be debated. Most neurosurgeons agreed that DIPG biopsy within a clinical trial should be supported, with the aims of defining the procedure risks, improving understanding of tumor biology, and evaluating new treatment targets. Careful family counseling and consent remain important.

2.
J Neurooncol ; 145(1): 177-184, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31522324

RESUMO

INTRODUCTION: Diffuse intrinsic pontine glioma (DIPG) is a rare clinically, neuro-radiologically, and molecularly defined malignancy of the brainstem with a median overall survival of approximately 11 months. Our aim is to evaluate the current tendency for its treatment in Europe in order to develop (inter)national consensus guidelines. METHODS: Healthcare professionals specialized in DIPG were asked to fill in an online survey with questions regarding usual treatment strategies at diagnosis and at disease progression in their countries and/or their centers, respectively. RESULTS: Seventy-four healthcare professionals responded to the survey, of which 87.8% were pediatric oncologists. Only 13.5% of the respondents biopsy all of their patients, 41.9% biopsy their patients infrequently. More than half of the respondents (54.1%) treated their patients with radiotherapy only at diagnosis, whereas 44.6% preferred radiotherapy combined with chemotherapy. When the disease progresses, treatment strategies became even more diverse, and the tendency for no treatment increased from 1.4% at diagnosis to 77.0% after second progression. 36.5% of the healthcare professionals treat children younger than 3 years differently than older children at diagnosis. This percentage decreased, when the disease progresses. Most of the participants (51.4%) included less than 25% of their patients in clinical trials. CONCLUSION: This survey demonstrates a large heterogeneity of treatment regimens, especially at disease progression. We emphasize the need for international consensus guidelines for the treatment of DIPG, possible by more collaborative clinical trials.

3.
Sci Rep ; 9(1): 9973, 2019 Jul 10.
Artigo em Inglês | MEDLINE | ID: mdl-31292491

RESUMO

EPNs comprise a heterogeneous group of neuroepithelial tumors, accounting for about 10% of all intracranial tumors in children and up to 30% of brain tumors in those younger than 3 years. Actually, the pattern therapy for low-grade EPNs includes complete surgical resection followed by radiation therapy. Total surgical excision is often not possible due to tumor location. The aim of this study was to evaluate, for the first time, the anti-tumor activity of Amblyomin-X in 4 primary cultures derived from pediatric anaplastic posterior fossa EPN, Group A (anaplastic, WHO grade III) and one primary culture of a high grade neuroepithelial tumor with MN1 alteration, which was initially misdiagnosed as EPN: i) by in vitro assays: comparisons of temozolomide and cisplatin; ii) by intracranial xenograft model. Amblyomin-X was able to induce cell death in EPN cells in a more significant percentage compared to cisplatin. The cytotoxic effects of Amblyomin-X were not detected on hFSCs used as control, as opposed to cisplatin-treatment, which promoted a substantial effect in the hAFSCs viability. TEM analysis showed ultrastructural alterations related to the process of cell death: mitochondrial degeneration, autophagosomes and aggregate-like structures. MRI and histopathological analyzes demonstrated significant tumor mass regression. Our results suggest that Amblyomin-X has a selective effect on tumor cells by inducing apoptotic cell death and may be a therapeutic option for Group AEPNs.

4.
Neurosurgery ; 2019 Jun 24.
Artigo em Inglês | MEDLINE | ID: mdl-31232427

RESUMO

BACKGROUND: New-onset hydrocephalus following foramen magnum decompression (FMD) for Chiari I malformation (CM-I) is rare; its natural history and pathophysiology are poorly understood. OBJECTIVE: To describe a series of patients who developed hydrocephalus following FMD for CM-I, provide possible explanations of this phenomenon, and outline treatment options. METHODS: Out of patients undergoing FMD for CM-I from 6 different tertiary centers, we evaluated patients presenting with new-onset hydrocephalus following FMD. The retrospectively collected data included demographics, clinical, and radiological findings of the CM-I and hydrocephalus patients. Time from FMD and hydrocephalus onset, treatment, and surgical techniques were assessed. RESULTS: Of 549 patients who underwent FMD for CM-I, 28 (5.1%) subsequently developed hydrocephalus (18 females, 10 males), with a mean age of 11.7 ± 11.9 yr (range 6 mo to 52 yr). Hydrocephalus occurred on average 2.2 ± 2.6 mo after FMD (range 1 wk to 8 mo). Four patients did not have a violation of the arachnoid during the FMD surgery. Main presenting symptoms of hydrocephalus were headaches (17, 41%), vomiting (10, 24.4%), and cerebrospinal fluid (CSF) leak or pseudomeningocele (7, 17%).Overall, 23 patients (82.1%) underwent CSF shunting, 1 patient (3.5%) had an endoscopic third ventriculostomy, 3 patients (10.7%) temporary CSF diversion only, and 1 patient (3.5%) was treated with acetazolamide. CONCLUSION: Hydrocephalus following FMD for CM-I is uncommon, but important. Based on our series and literature review, its incidence is about 5% to 7% and most likely will require further surgery. Shunting appears to be the favored treatment option.

5.
Klin Padiatr ; 231(3): 107-135, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-31108561

RESUMO

Low grade gliomas (LGGs) constitute the largest, yet clinically and (molecular-) histologically heterogeneous group of pediatric brain tumors of WHO grades I and II occurring throughout all pediatric age groups and at all central nervous system (CNS) sites. The tumors are characterized by a slow growth rate and may show periods of growth arrest. Around 40% of all LGG patients can be cured by complete neurosurgical resection and are followed by close observation. In case of relapse, second resection often is possible. Following incomplete resection observation is recommended, as long as there is no radiologic tumor growth and the patient does not suffer from significant, tumor-related symptoms. This also applies to patients with a diagnosis of LGG on the basis of radiological criteria. By contrast, clinical worsening and / or radiologic progression are an indication to treatment with either chemo- or radiotherapy. Overall survival is around 90%, and many patients survive with residual tumor, i. e. they suffer from chronic disease. All patients need comprehensive neuro-oncological care, the principles and details of which are summarized in the current guidelines. These represent standard of care for diagnostic work-up (including neuroimaging and neuropathology), and for therapeutic decisions (including the indications to non-surgical treatment) as well as concepts for neurosurgical intervention, chemotherapy and radiotherapy as well as surveillance and rehabilitation. The current treatment algorithm was compiled by members of the LGG working group of the SIOP-E brain tumor group (SIOP-E-BTG) and is based upon the results of previous European LGG studies and international reports.


Assuntos
Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/terapia , Glioma/diagnóstico , Glioma/terapia , Guias de Prática Clínica como Assunto , Adolescente , Criança , Progressão da Doença , Humanos , Recidiva Local de Neoplasia , Sociedades Médicas
6.
Eur J Cancer ; 114: 27-35, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31022591

RESUMO

PURPOSE: Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive paediatric brain tumour with fatal outcome. The Individualised Therapy For Relapsed Malignancies In Childhood (INFORM) registry study offers comprehensive molecular profiling of high-risk tumours to identify target alterations for potential precision therapy. We analysed molecular characteristics and clinical data after brainstem biopsy of all enrolled newly diagnosed DIPGs. PATIENTS AND METHODS: From -February 2015 to February 2018, 21 subsequent primary DIPG cases were enrolled in the nation-wide multicentre INFORM registry study after brainstem biopsy. Whole-genome, whole-exome sequencing and DNA methylation analysis were performed, and RNA-sequencing was added in case of sufficient material. Clinical data were obtained from standardised questionnaires and the INFORM clinical data bank. RESULTS: Tumour material obtained from brainstem biopsy was sufficient for DNA analysis in all cases and RNA analysis in 16 of 21 cases. In 16 of 21 cases (76%), potential targetable alterations were identified including highly relevant MET and NTRK1 fusions as well as an EZH2 alteration not previously described in DIPG. In 5 of 21 cases, molecular information was used for initiation of targeted treatment. The majority of patients (19/21) presented with neurological deficits at diagnosis. Newly arising or worsening of neurological deficits post-biopsy occurred in nine patients. Symptoms were reversible or improved notably in eight cases. CONCLUSION: In this multicentre study setting, brainstem biopsy of DIPG was feasible and yielded sufficient material for comprehensive molecular profiling. Relevant molecular targets were identified impacting clinical management in a substantial subset. Death or severe bleeding occurred in none of the cases. One of 20 patients experienced unilateral paraesthesia possibly related to biopsy.

7.
Acta Neuropathol ; 136(2): 273-291, 2018 08.
Artigo em Inglês | MEDLINE | ID: mdl-29564591

RESUMO

Tumors with histological features of pilocytic astrocytoma (PA), but with increased mitotic activity and additional high-grade features (particularly microvascular proliferation and palisading necrosis) have often been designated anaplastic pilocytic astrocytomas. The status of these tumors as a separate entity has not yet been conclusively demonstrated and molecular features have only been partially characterized. We performed DNA methylation profiling of 102 histologically defined anaplastic pilocytic astrocytomas. T-distributed stochastic neighbor-embedding (t-SNE) and hierarchical clustering analysis of these 102 cases against 158 reference cases from 12 glioma reference classes revealed that a subset of 83 of these tumors share a common DNA methylation profile that is distinct from the reference classes. These 83 tumors were thus denominated DNA methylation class anaplastic astrocytoma with piloid features (MC AAP). The 19 remaining tumors were distributed amongst the reference classes, with additional testing confirming the molecular diagnosis in most cases. Median age of patients with MC AAP was 41.5 years. The most frequent localization was the posterior fossa (74%). Deletions of CDKN2A/B (66/83, 80%), MAPK pathway gene alterations (49/65, 75%, most frequently affecting NF1, followed by BRAF and FGFR1) and mutations of ATRX or loss of ATRX expression (33/74, 45%) were the most common molecular alterations. All tumors were IDH1/2 wildtype. The MGMT promoter was methylated in 38/83 tumors (45%). Outcome analysis confirmed an unfavorable clinical course in comparison to PA, but better than IDH wildtype glioblastoma. In conclusion, we show that a subset of histologically defined anaplastic pilocytic astrocytomas forms a separate DNA methylation cluster, harbors recurrent alterations in MAPK pathway genes in combination with alterations of CDKN2A/B and ATRX, affects patients who are on average older than those diagnosed with PA and has an intermediate clinical outcome.


Assuntos
Astrocitoma/genética , Neoplasias Encefálicas/genética , Isocitrato Desidrogenase/genética , Transdução de Sinais/genética , Adolescente , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Criança , Pré-Escolar , Inibidor p16 de Quinase Dependente de Ciclina/genética , Metilação de DNA/genética , Metilases de Modificação do DNA/metabolismo , Enzimas Reparadoras do DNA/metabolismo , Feminino , Histonas/genética , Histonas/metabolismo , Humanos , Lactente , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Quinases de Proteína Quinase Ativadas por Mitógeno/genética , Mutação/genética , Estudos Retrospectivos , Proteínas Supressoras de Tumor/metabolismo , Proteína Nuclear Ligada ao X/genética , Adulto Jovem
8.
J Neurosurg ; 129(6): 1407-1415, 2018 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-29424646

RESUMO

OBJECTIVEHemorrhage (also known as apoplexy) in optic pathway gliomas (OPGs) is rare. Because of the variable presentations and low incidence of OPG hemorrhages, little is known about their clinical course and the best treatment options. The aim of this work was to review risk factors, clinical course, and treatment strategies of optic glioma hemorrhages in the largest possible number of cases.METHODSA total of 34 patients were analyzed. Nine new cases were collected, and 25 were identified in the literature. Data regarding demographics, radiological and histological features, treatment, and outcome were retrospectively reviewed.RESULTSThe majority of patients were younger than 20 years. Only 3 patients were known to have neurofibromatosis. The histopathological diagnosis was pilocytic astrocytoma in the majority of cases. Five patients had intraorbital hemorrhages, whereas 29 patients had intracranial hemorrhage; the majority of intracranial bleeds were treated surgically. Six patients, all with intracranial hemorrhage, died due to recurrent bleeding, hydrocephalus, or surgical complications. No clear risk factors could be identified.CONCLUSIONSIntracerebral OPG hemorrhages have a fatal outcome in 20% of cases. Age, hormonal status, neurofibromatosis involvement, and histopathological diagnosis have been suggested as risk factors for hemorrhage, but this cannot be reliably established from the present series. The goals of surgery should be patient survival and prevention of further neurological and ophthalmological deterioration.


Assuntos
Astrocitoma/patologia , Neoplasias Encefálicas/patologia , Ganglioglioma/patologia , Hemorragias Intracranianas/patologia , Glioma do Nervo Óptico/patologia , Astrocitoma/diagnóstico por imagem , Neoplasias Encefálicas/diagnóstico por imagem , Criança , Pré-Escolar , Ganglioglioma/diagnóstico por imagem , Humanos , Hemorragias Intracranianas/diagnóstico por imagem , Imagem por Ressonância Magnética , Masculino , Glioma do Nervo Óptico/diagnóstico por imagem
9.
World Neurosurg ; 107: 623-629, 2017 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-28842232

RESUMO

BACKGROUND: Hydrocephalus in patients with neurofibromatosis (NF) type 1 is usually obstructive and may arise secondary to tumoral or nontumoral causes. Treatment of hydrocephalus in these patients is often challenging owing to combined pathologies and unique anatomic changes. The use of endoscopic third ventriculostomy (ETV) as treatment has rarely been described in this group. We aimed to characterize indications, considerations, and outcome of ETV in patients with NF 1 gathered in a multicenter international cohort. METHODS: Five centers participated in this retrospective study. Following institutional review board approval, data and images were collected. Patients of all ages with NF 1 who underwent ETV for treatment of obstructive hydrocephalus were included. Patients who had no postoperative radiologic or clinical follow-up were excluded. ETV failure was defined as recurrent clinical or radiologic signs of hydrocephalus. RESULTS: The study included 42 patients. Common etiologies for hydrocephalus were aqueductal/tectal tumor (31%), aqueductal web (26%), and aqueductal stenosis owing to NF-related changes (14%). Ten patients had a preoperative diagnosis of optic pathway glioma. ETV failures were identified in 6 patients within 1 month, in 3 patients within 9 months, and in 1 patient within 4 years. ETV was successful in 32 patients (76%) with a mean follow up of 59.4 months ± 50.9 (range, 4 months to 15 years). CONCLUSIONS: ETV is a safe treatment for selected patients with NF 1 and obstructive hydrocephalus. Individual anatomic and pathologic aspects should be taken into consideration.


Assuntos
Neoplasias Encefálicas/cirurgia , Neuroendoscopia/métodos , Neurofibromatose 1/cirurgia , Ventriculostomia/métodos , Adolescente , Adulto , Assistência ao Convalescente , Neoplasias Encefálicas/patologia , Criança , Pré-Escolar , Feminino , Humanos , Hidrocefalia/etiologia , Hidrocefalia/patologia , Hidrocefalia/cirurgia , Lactente , Complicações Intraoperatórias/etiologia , Estimativa de Kaplan-Meier , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neurofibromatose 1/patologia , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Falha de Tratamento , Derivação Ventriculoperitoneal/métodos , Adulto Jovem
10.
Acta Neurochir (Wien) ; 159(6): 1033-1041, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28389876

RESUMO

BACKGROUND: Individual planning of the entry point and the use of navigation has become more relevant in intraventricular neuroendoscopy. Navigated neuroendoscopic solutions are continuously improving. OBJECTIVE: We describe experimentally measured accuracy and our first experience with augmented reality-enhanced navigated neuroendoscopy for intraventricular pathologies. PATIENTS AND METHODS: Augmented reality-enhanced navigated endoscopy was tested for accuracy in an experimental setting. Therefore, a 3D-printed head model with a right parietal lesion was scanned with a thin-sliced computer tomography. Segmentation of the tumor lesion was performed using Scopis NovaPlan navigation software. An optical reference matrix is used to register the neuroendoscope's geometry and its field of view. The pre-planned ROI and trajectory are superimposed in the endoscopic image. The accuracy of the superimposed contour fitting on endoscopically visualized lesion was acquired by measuring the deviation of both midpoints to one another. The technique was subsequently used in 29 cases with CSF circulation pathologies. Navigation planning included defining the entry points, regions of interests and trajectories, superimposed as augmented reality on the endoscopic video screen during intervention. Patients were evaluated for postoperative imaging, reoperations, and possible complications. RESULTS: The experimental setup revealed a deviation of the ROI's midpoint from the real target by 1.2 ± 0.4 mm. The clinical study included 18 cyst fenestrations, ten biopsies, seven endoscopic third ventriculostomies, six stent placements, and two shunt implantations, being eventually combined in some patients. In cases of cyst fenestrations postoperatively, the cyst volume was significantly reduced in all patients by mean of 47%. In biopsies, the diagnostic yield was 100%. Reoperations during a follow-up period of 11.4 ± 10.2 months were necessary in two cases. Complications included one postoperative hygroma and one insufficient fenestration. CONCLUSIONS: Augmented reality-navigated neuroendoscopy is accurate and feasible to use in clinical application. By integrating relevant planning information directly into the endoscope's field of view, safety and efficacy for intraventricular neuroendoscopic surgery may be improved.


Assuntos
Neuroendoscopia/efeitos adversos , Ventriculostomia/efeitos adversos , Adolescente , Adulto , Idoso , Neoplasias Encefálicas/cirurgia , Criança , Pré-Escolar , Feminino , Humanos , Hidrocefalia/cirurgia , Lactente , Masculino , Pessoa de Meia-Idade , Neuroendoscopia/instrumentação , Neuroendoscopia/métodos , Complicações Pós-Operatórias/epidemiologia , Reoperação/estatística & dados numéricos , Ventriculostomia/instrumentação , Ventriculostomia/métodos
11.
Childs Nerv Syst ; 33(5): 759-765, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28332153

RESUMO

OBJECTIVE: Overdrainage in children is a long-term problem for shunted patients which might lead to chronic anatomical changes. In order to prevent these problems, valve exchange is performed on a regular basis in patients without hydrostatic units towards a valve with both an adjustable and a gravitational unit. The clinical outcome of these patients is reported in a retrospective study. METHODS: Between 2009 and 2014, the in-house database was analyzed for patients who received a valve exchange towards an adjustable differential pressure valve with gravitational unit. The study protocol included the patients shunt history, image analysis for ventricular width, and necessity of revision surgery after valve exchange. A questionnaire was sent to the patients in order to ask for their subjective experience for symptom changes and treatment experience. RESULTS: Forty-six patients were identified (26 girls, mean age 11.8 ± 6.1 years) with a mean follow-up of 36.3 ± 15 months. The ventricular width did increase after valve exchange as measured in frontal and occipital horn ratio (0.364 ± 0.032 vs. 0.402 ± 0.09, p = 0.0017). Of the patients suffering from acute symptoms, 89% improved after treatment. The shunt and valve survival rates were 88 and 95%, respectively, after 12 months. Comparing the total amount of revisions before and after valve exchange, a significant reduction was seen in total but a no significant difference was analyzed in amount of revisions to time ratio. CONCLUSION: Valve exchange might be cautiously decided if patients seem to perform clinically well. In our study, we were able to show that the strategy of valve exchange to prevent chronic overdrainage is well tolerated and seem to improve patient's clinical outcome in terms of ventricular width, symptom relieve, and revision rate.


Assuntos
Desenho de Equipamento/instrumentação , Gravitação , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/cirurgia , Pressão , Derivação Ventriculoperitoneal/instrumentação , Adolescente , Pressão do Líquido Cefalorraquidiano/fisiologia , Criança , Pré-Escolar , Desenho de Equipamento/métodos , Feminino , Seguimentos , Humanos , Masculino , Estudos Retrospectivos , Inquéritos e Questionários , Resultado do Tratamento , Derivação Ventriculoperitoneal/métodos , Adulto Jovem
12.
Clin Neurol Neurosurg ; 137: 132-6, 2015 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-26196478

RESUMO

OBJECTIVE: Cerebrospinal fluid (CSF) overdrainage is a major problem in shunt therapy for hydrocephalus. The adjustable gravitational valve proSA allows for the first time a targeted compensation for overdrainage in the upright position without interfering with the differential pressure valve. To evaluate benefit, safety and reliability, the multicenter prospective registry PROSAIKA was conducted in 10 German neurosurgical centers. METHODS: Between March 2009 and July 2010, 120 hydrocephalic patients undergoing first time shunt implantation or shunt revision using proSA entered the study. 93 patients completed the 12 months follow-up. RESULTS: Hydrocephalus symptoms were improved in 86%, unchanged in 9% and deteriorated in 3%. In 51%, the proSA opening pressure was readjusted one or several times to treat suspected suboptimal shunt function, this resulted in clinical improvement in 55%, no change in 25%, and deterioration in 20% of these patients. The 1 year censored proSA shunt survival rate was 89%. Device related shunt failure was seen in two cases. CONCLUSIONS: This is the first clinical report on the implantation of the adjustable gravitational valve proSA with a follow-up of 12 months in a substantial number of patients. Irrespective of different hydrocephalus etiologies and indications for shunt surgery, the overall results after 12 months were very satisfying. The high frequency of valve readjustments underlines the fact that preoperative selection of the appropriate valve opening pressure is difficult. The low number of revisions and complications compared to other valves proves that proSA implantation adds no further risk; this valve is reliable, helpful and safe.


Assuntos
Derivações do Líquido Cefalorraquidiano , Desenho de Equipamento , Hidrocefalia/cirurgia , Derivação Ventriculoperitoneal , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Derivações do Líquido Cefalorraquidiano/instrumentação , Criança , Pré-Escolar , Falha de Equipamento , Feminino , Seguimentos , Gravitação , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Sistema de Registros , Derivação Ventriculoperitoneal/instrumentação , Adulto Jovem
13.
Childs Nerv Syst ; 31(8): 1401-6, 2015 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25925579

RESUMO

PURPOSE: Hamartomas are well described but yet incompletely understood sporadic benign lesions that can arise in various locations. Hypothalamic hamartomas of infancy are often associated with severe developmental disturbances. We present a case of an infant boy with a hamartoma that arises from the optic nerve and lead to progressive unspecific seizure activity, behavioral problems and precautious puberty. METHODS: A 1-year-old male patient was presented with horizontal nystagmus and developmental retardation. Magnetic resonance imaging (MRI) with contrast revealed an isointense mass ventral of the chiasm consistent with a hamartoma. Soon after the MRI, the mother of the patient reported gelastic-like seizures. The patient was evaluated by an interdisciplinary team, and surgery was recommended. Intraoperatively, a firm attachment to the optic nerve was recognized and a thin remnant layer of tissue was left behind. RESULTS: After an uncomplicated near total resection, the patient improved significantly. After 6 months, the frequency of seizures reoccurred, which were again unresponsive to antiepileptic medication. In a second operation, a complete resection of the remnants was performed, and the patient showed lasting clinical improvement. CONCLUSION: We conclude that hamartomas mimicking hypothalamic symptoms can also arise from the optic pathway and that a reoperation, if feasible, of even small remnants is essential in order to achieve lasting symptom relieve.


Assuntos
Hamartoma/fisiopatologia , Hamartoma/terapia , Doenças do Nervo Óptico/fisiopatologia , Doenças do Nervo Óptico/terapia , Proteína Glial Fibrilar Ácida/metabolismo , Hamartoma/diagnóstico , Humanos , Lactente , Filamentos Intermediários/metabolismo , Antígeno Ki-67/metabolismo , Imagem por Ressonância Magnética , Masculino , Quiasma Óptico/patologia , Doenças do Nervo Óptico/diagnóstico
14.
Pediatr Blood Cancer ; 62(7): 1252-8, 2015 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-25850573

RESUMO

BACKGROUND: Disease and therapy cause brain damage and subsequent functional loss in pediatric patients with posterior fossa tumors. Treatment-related toxicity factors are resection in patients with pilocytic astrocytoma (PA) and, additionally, cranio-spinal irradiation together with chemotherapy in patients with medulloblastoma (MB). We tested whether damage to white matter (WM) as revealed by diffusion tensor MR imaging (DTI) correlated with specific cognitive and motor impairments in survivors of pediatric posterior fossa tumors. PROCEDURES: Eighteen MB (mean age ± SD, 15.2 ± 4.9 y) and 14 PA (12.6 ± 5.0 y) survivors were investigated with DTI on a 3-Tesla-MR system. We identified fractional anisotropy (FA) of WM, the volume ratio of WM to gray matter and cerebrospinal fluid (WM/GM + CSF), and volume of specific frontocerebellar tracts. Ataxia was assessed using the International Cooperative Ataxia Rating Scale (ICARS), while the Wechsler Intelligence Scale for Children determined full-scale intelligence quotients (FSIQ). Amsterdam Neuropsychological Tasks (ANT) was used to assess processing speed. Handwriting automation was analyzed using a digitizing graphic tablet. RESULTS: The WM/GM + CSF ratio correlated significantly with cognitive measures (IQ, P = 0.002; ANT baseline speed, P = 0.04; ANT shifting attention, P = 0.004). FA of skeletonized tracts correlated significantly with FSIQ (P = 0.008), ANT baseline speed (P = 0.028) and ANT shifting attention (P = 0.045). Moreover, frontocerebellar tract volumes correlated with both the FSIQ (P = 0.011) and ICARS (P = 0.007). CONCLUSION: DTI provides a method for quantification of WM damage by tumor and by therapy-associated effects in survivors of pediatric posterior fossa tumors. DTI-derived WM integrity may be a representative marker for cognitive and motor deterioration.


Assuntos
Astrocitoma/complicações , Ataxia/diagnóstico , Transtornos Cognitivos/diagnóstico , Imagem de Tensor de Difusão/métodos , Neoplasias Infratentoriais/complicações , Meduloblastoma/complicações , Substância Branca/patologia , Adolescente , Anisotropia , Astrocitoma/patologia , Ataxia/etiologia , Neoplasias Cerebelares/complicações , Neoplasias Cerebelares/patologia , Criança , Transtornos Cognitivos/etiologia , Feminino , Seguimentos , Humanos , Neoplasias Infratentoriais/patologia , Testes de Inteligência , Masculino , Meduloblastoma/patologia , Testes Neuropsicológicos , Prognóstico , Taxa de Sobrevida , Sobreviventes
15.
Methods Mol Biol ; 1256: 405-17, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-25626554

RESUMO

Mobile technology has recently been introduced for blood pressure measurements or glucose level controls. In surgical disciplines the use of smartphone applications is mostly restricted as training tools or knowledge resources. Simple surgical procedures which are performed often in certain disciplines may be performed with limited accuracy since routine and overwork of medical staff lead to less awareness to possible mistakes. In these cases simple and effective means are necessary to achieve better patient safety.In this context, a surgical instrument for ventricular catheter placement in neurosurgical patients was designed which is assisted by measurements undertaken in a smartphone software application specifically visualizing the use of this instrument and achieving better accuracy for catheter positioning. On theoretical ground, the angulation of the catheter trajectory towards the surface of the skull in a coronal reconstructed CT or MR image is determined as the simplified but the most relevant individual parameter for correct ventricular catheter placement. Transfer of a CT/MRI image onto the smartphone can be performed via mail as anonymous file. Using this image, the trajectory measurement can be performed individually in a few steps by calibration of the image size, definition of the frontal entry point, and virtual placement of the instrument on the surface of the skull. Then the angulation can be adjusted and measured to place the catheter's trajectory towards the ipsilateral ventricle and the catheter length is determined. The parameters are now given by the app and visualized on the image in order to be applied to the surgical site of the patient.The tool represents a widely available and cost-effective solution as navigation technique which is simple to apply in order to achieve better accuracy in ventricular catheter placement for higher safety in a large cohort of neurosurgical patients.


Assuntos
Telefone Celular/instrumentação , Neuronavegação/instrumentação , Neurocirurgia/instrumentação , Software , Cateteres de Demora , Ventrículos Cerebrais/anatomia & histologia , Ventrículos Cerebrais/cirurgia , Processamento Eletrônico de Dados , Humanos , Internet , Neuronavegação/métodos , Técnicas Estereotáxicas/instrumentação , Telemedicina
16.
J Neurosurg Pediatr ; 13(3): 319-23, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24437987

RESUMO

The authors report on the first experiences with the prototype of a surgical tool for cranial remodeling. The device enables the surgeon to transfer statistical information, represented in a model, into the disfigured bone. The model is derived from a currently evolving databank of normal head shapes. Ultimately, the databank will provide a set of standard models covering the statistical range of normal head shapes, thus providing the required template for any standard remodeling procedure as well as customized models for intended overcorrection. To date, this technique has been used in the surgical treatment of 14 infants (age range 6-12 months) with craniosynostosis. In all 14 cases, the designated esthetic result, embodied by the selected model, has been achieved, without morbidity or mortality. Frame-based reconstruction provides the required tools to precisely realize the surgical reproduction of the model shape. It enables the establishment of a self-referring system, feeding back postoperative growth patterns, recorded by 3D follow-up, into the model design.


Assuntos
Craniossinostoses/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Crânio/anormalidades , Crânio/cirurgia , Beleza , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Lactente , Masculino , Base do Crânio/cirurgia , Resultado do Tratamento
17.
Childs Nerv Syst ; 29(3): 425-31, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23135777

RESUMO

OBJECT: Overdrainage is a chronic complication in shunted pediatric patients with hydrocephalus. The use of adjustability of differential pressure (DP) valves in combination with antisiphoning devices may help to overcome this sequela and may diminish the rate of possible shunt failures. The purpose of this retrospective study is to report our experience on shunt survival and infection rate with an adjustable DP valve with integrated gravitational unit in pediatric hydrocephalus. METHODS: The proGAV consists of an adjustable differential pressure (DP) valve and a gravitational unit. During the time period of July 2004 and December 2009, a total of 237 adjustable gravitational valves were used in 203 children (age, 6.5 ± 6.54; 0-27 years). In the follow-up period, valve and shunt failures as well as rate of infection were recorded. RESULTS: Within the average follow-up time of 21.9 ± 10.3 months (range, 6-72 months), the valve survival rate was 83.8 %. The overall shunt survival rate including all necessary revisions was 64.3 %. Looking at the group of infants (<1 year of age) within the cohort, the valve survival rate was 77.3 % and the shunt survival rate was 60.9 %. The overall infection rate was 4.6 %. CONCLUSION: In a concept of avoiding chronic overdrainage by using the proGAV in hydrocephalic children, we observed a good rate of valve and shunt survival. Compared to previous reported series, we experienced the proGAV as a reliable tool for the treatment of pediatric hydrocephalus.


Assuntos
Derivações do Líquido Cefalorraquidiano/efeitos adversos , Drenagem/efeitos adversos , Hidrocefalia/cirurgia , Hipotensão Intracraniana/prevenção & controle , Adolescente , Adulto , Fatores Etários , Derivações do Líquido Cefalorraquidiano/instrumentação , Derivações do Líquido Cefalorraquidiano/métodos , Criança , Pré-Escolar , Estudos de Coortes , Drenagem/instrumentação , Desenho de Equipamento , Análise de Falha de Equipamento , Feminino , Seguimentos , Gravitação , Humanos , Hidrodinâmica , Lactente , Recém-Nascido , Hipotensão Intracraniana/etiologia , Estimativa de Kaplan-Meier , Masculino , Pressão , Resultado do Tratamento , Adulto Jovem
18.
Childs Nerv Syst ; 28(5): 707-13, 2012 May.
Artigo em Inglês | MEDLINE | ID: mdl-22391876

RESUMO

OBJECTIVE: Diffuse intrinsic brainstem gliomas are considered to be inoperable. We report our initial experience of temozolomide (TMZ) administration into brainstem by intracerebral (i.c.) microinfusion using a rat brainstem glioblastoma allograft model. METHODS: Forty-eight Fischer 344 female rats were used. In a feasibility study, various doses of i.c.-TMZ (1-10 mg) were administered into the brainstem using AlzetTM pumps in order to evaluate survival rates and neurotoxicity. For tumor implantation, rats received an injection of 10(5) 9 L gliosarcoma cells. For local therapy, 5 days after inoculation, a total amount of 1 mg of TMZ or saline was administered into the brainstem at 1 µl/h over 7 days (n = 8/group). For systemic therapy, rats were treated with an orally administered maximum daily dose of 50 mg/kg TMZ for 5 consecutive days. Survival time and neurological deficit were recorded as outcome parameters. RESULTS: In the neurotoxicity study, low dose TMZ (1 mg) was feasible to be administered into brainstem over 7 days without neurological deficit. Using high dose TMZ (5-10 mg), marked neurotoxic effect was observed. In the brainstem tumor study, survival was significantly prolonged in low dose i.c.-TMZ group compared to control rats (median survival 23.5 versus 29.5 days; p < 0.01). Systemic therapy with maximal oral-TMZ dose resulted in longer survival time compared to low dose i.c.-TMZ group (median survival 33.5 versus 29.5 days; p < 0.01). CONCLUSIONS: i.c.-TMZ is feasible and effective against rat brainstem glioblastoma allograft. However, we could not show superior potential of i.c.-TMZ compared to oral-TMZ administration. Modification of TMZ infusion with systemic therapy warrants future investigations.


Assuntos
Neoplasias Encefálicas/tratamento farmacológico , Tronco Encefálico/efeitos dos fármacos , Dacarbazina/análogos & derivados , Modelos Animais de Doenças , Sistemas de Liberação de Medicamentos/métodos , Glioblastoma/tratamento farmacológico , Microinjeções , Animais , Antineoplásicos Alquilantes/administração & dosagem , Neoplasias Encefálicas/mortalidade , Neoplasias Encefálicas/patologia , Tronco Encefálico/patologia , Linhagem Celular Tumoral , Dacarbazina/administração & dosagem , Estudos de Viabilidade , Feminino , Glioblastoma/mortalidade , Glioblastoma/patologia , Infusões Intraventriculares , Microinjeções/métodos , Ratos , Ratos Endogâmicos F344 , Taxa de Sobrevida/tendências , Temozolomida , Transplante Homólogo , Resultado do Tratamento
19.
Acta Neurochir Suppl ; 114: 261-6, 2012.
Artigo em Inglês | MEDLINE | ID: mdl-22327705

RESUMO

INTRODUCTION: For subarachnomegaly in infants with enlarged external and internal CSF spaces clear treatment decisions, such as observation or ventriculo-peritoneal (VP) shunting, are still lacking. The aim of this study is to measure intracranial pressure (ICP) in these patients to elucidate treatment necessity. MATERIALS AND METHODS: Seven children presenting with macrocephaly, moderately enlarged ventricles, and subarachnoid spaces on magnetic resonance imaging (MRI) without neurological deficits or other CSF-associated pathological conditions were enrolled. Continuous ICP recording was conducted using an external ventricular drain. Data recordings during overnight measurements were analyzed offline to calculate baseline, mean ICP values, and a histogram distribution. RESULTS: Mean age at enrollment was 9.4 months (2-22 months). ICP monitoring was conducted for 2.7 ± 1.1 nights (range 2-5 nights) and revealed baseline values above 10 mmHg in three patients, who went on to receive a VP shunt. One patient with average values over 10 mmHg also underwent VP shunting. Three patients displayed both baseline and average ICP values of less than 10 mmHg. Observational management was chosen for this subgroup. Comparing shunted versus the non-shunted group in a histogram analysis the percentages of recorded ICP values above 8, 10, and 15 mmHg were significantly different (p < 0.05). CONCLUSION: Subarachnomegaly in infants remains a dilemma to the treating neurosurgeon. Risks and benefits of observational management options need to be weighed against those of VP shunting. Continuous ICP monitoring may help to identify patients who may potentially benefit from the surgical treatment option.


Assuntos
Pressão Intracraniana/fisiologia , Megalencefalia/patologia , Megalencefalia/fisiopatologia , Espaço Subaracnóideo/fisiopatologia , Cateterismo/métodos , Progressão da Doença , Feminino , Humanos , Lactente , Imagem por Ressonância Magnética , Masculino , Estudos Retrospectivos , Derivação Ventriculoperitoneal/métodos
20.
Acta Neurochir (Wien) ; 153(8): 1561-72, 2011 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-21461877

RESUMO

BACKGROUND: Functional connectivity analysis of resting-state functional magnetic resonance imaging data (fcrs-fMRI) has been shown to be a robust non-invasive method for localization of functional networks (without using specific tasks) and to be promising for presurgical planning. However, in order to transfer the approach to everyday clinical practice, fcrs-fMRI needs to be further validated and made easily accessible to neurosurgeons. This paper addresses the latter by presenting a software tool designed for neurosurgeons for analyzing and visualizing fcrs-fMRI data. METHODS: A prototypical interactive visualization tool was developed to enable neurosurgeons to explore functional connectivity data and evaluate its usability. The implementation builds upon LIPSIA, an established software package for the assessment of functional neuroimaging data, and integrates the selection of a region-of-interest with the computation and visualization of functionally connected areas. The tool was used to explore data from a healthy participant and eight brain lesion patients. The usability of the software was evaluated with four neurosurgeons previously unacquainted with the methodology, who were asked to identify prominent, large-scale cortical networks. FINDINGS: With this novel tool, previously published findings, such as tumor displacement of the sensorimotor cortex and other disturbances of functional networks, were reproduced. The neurosurgeons were able to consistently obtain results similar to the results of an expert, with the exception of the language network. Immediate feedback helped to pinpoint functional networks quickly and intuitively, with even inexperienced users requiring less than 3 min per network. CONCLUSIONS: Although fcrs-fMRI is a nascent method still undergoing evaluation with respect to established standards, the interactive software is nonetheless a promising tool for non-invasive exploration of individual functional connectivity networks in neurosurgical practice, both for well-known networks and for those less typically addressed.


Assuntos
Mapeamento Encefálico/métodos , Imagem por Ressonância Magnética/métodos , Rede Nervosa/fisiologia , Rede Nervosa/cirurgia , Procedimentos Neurocirúrgicos/métodos , Software/normas , Adulto , Idoso , Mapeamento Encefálico/instrumentação , Mapeamento Encefálico/tendências , Feminino , Humanos , Processamento de Imagem Assistida por Computador/métodos , Imagem por Ressonância Magnética/instrumentação , Imagem por Ressonância Magnética/tendências , Masculino , Pessoa de Meia-Idade , Procedimentos Neurocirúrgicos/instrumentação , Procedimentos Neurocirúrgicos/tendências , Cuidados Pré-Operatórios/instrumentação , Cuidados Pré-Operatórios/métodos , Cuidados Pré-Operatórios/tendências , Software/tendências , Técnicas Estereotáxicas/normas , Interface Usuário-Computador
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