RESUMO
PURPOSE: Placement of an external ventricular drainage (EVD) is one of the most frequent procedures in neurosurgery, but it has specific challenges and risks in the pediatric population. We here investigate the indications, management, and shunt conversion rates of an EVD. METHODS: We retrospectively analyzed the data of a consecutive series of pediatric patients who had an EVD placement in the Department of Neurosurgery at Hannover Medical School over a 12-year period. A bundle approach was introduced to reduce infections. Patients were categorized according to the underlying pathology in three groups: tumor, hemorrhage, and infection. RESULTS: A total of 126 patients were included in this study. Seventy-two were male, and 54 were female. The mean age at the time of EVD placement was 5.2 ± 5.0 years (range 0-17 years). The largest subgroup was the tumor group (n = 54, 42.9%), followed by the infection group (n = 47, 37.3%), including shunt infection (n = 36), infected Rickham reservoir (n = 4), and bacterial or viral cerebral infection (n = 7), and the hemorrhage group (n = 25, 19.8%). The overall complication rate was 19.8% (n = 25/126), and the total number of complications was 30. Complications during EVD placement were noted in 5/126 (4%) instances. Complications during drainage time were infection in 9.5% (12 patients), dysfunction in 7.1% (9 patients), and EVD dislocation in 3.2% (4 patients). The highest rate of complications was seen in the hemorrhage group. There were no long-term complications. Conversion rates into a permanent shunt system were 100% in previously shunt-dependent patients. Conversion rates were comparable in the tumor group (27.7%) and in the hemorrhage group (32.0%). CONCLUSION: EVD placement in children is an overall safe and effective option in children. In order to make further progress, carefully planned prospective and if possible randomized studies are needed controlling for multivariable aspects.
Assuntos
Drenagem , Humanos , Masculino , Feminino , Criança , Adolescente , Pré-Escolar , Lactente , Estudos Retrospectivos , Recém-Nascido , Drenagem/métodos , Hidrocefalia/cirurgia , Derivações do Líquido Cefalorraquidiano/métodos , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologiaRESUMO
BACKGROUND: The semi-sitting position offers advantages for surgeries in the posterior cranial fossa. However, data on its safety and effectiveness for clipping aneurysms in the posterior cerebral circulation are limited. This retrospective cohort study evaluates the safety and effectiveness of using the semi-sitting position for these surgeries. METHODS: We conducted a retrospective study of 17 patients with posterior cerebral circulation aneurysms who underwent surgical clipping in the semi-sitting position in the Department of Neurosurgery at Hannover Medical School over a 10-year period. RESULTS: The mean age at surgery was 62 years (range, 31 to 75). Fourteen patients were admitted with subarachnoid hemorrhage and 3 patients had incidental aneurysmas. Fifteen patients had PICA aneurysms, and two had aneurysms of the vertebral artery and the superior cerebellar artery, respectively. The median diameter of the aneurysms was 5 mm (range 3-17 mm). Intraoperative venous air embolism (VAE) occurred in 4 patients, without affecting the surgical or clinical course. VAE was associated with a mild decrease of EtCO2 levels in 3 patients and in 2 patients a decrease of blood pressure occurred which was managed effectively. Surgical procedures proceeded as planned in all instances. There were no complications secondary to VAE. Two patients died secondary to respiratory problems (not related to VAE), and one patient was lost to follow-up. Eleven of fourteen patients were partially or completely independent (Barthel index between 60 and 100) at a median follow-up duration of 13.5 months (range, 3-103 months). CONCLUSION: The semi-sitting position is a safe and effective technique for the surgical clipping of aneurysms in the posterior cerebral circulation. The incidence of VAE is comparable to that seen in tumor surgery. However, it is crucial for the surgical and anesthesiological team to be familiar with potential complications and to react immediately in case of an occurrence of VAE.
Assuntos
Aneurisma Intracraniano , Procedimentos Neurocirúrgicos , Humanos , Pessoa de Meia-Idade , Feminino , Aneurisma Intracraniano/cirurgia , Masculino , Idoso , Adulto , Estudos Retrospectivos , Procedimentos Neurocirúrgicos/métodos , Postura Sentada , Instrumentos Cirúrgicos , Resultado do Tratamento , Hemorragia Subaracnóidea/cirurgiaRESUMO
PURPOSE: The semi-sitting position for resection of posterior fossa tumors is a matter of ongoing debate. Here we report about our experience with this approach in children younger than 4 years of age. METHODS: We retrospectively analyzed data of children younger than 4 years of age operated on in our institution in the semi-sitting position over a 15-year period. Patients were intraoperatively monitored for venous air embolism (VAE) by transthoracic Doppler (TTD) or transesophageal echocardiography (TEE). The severity of VAE was classified according to the Tübingen grading scale. Intraoperative incidents of VAE were recorded and the patients' course was followed postoperatively with a special focus on possible complications. RESULTS: Twenty-four children (18 boys, 6 girls) were operated on in the semi-sitting position (26 operations). Mean age was 2.2 years (± 1.0), range between 0.4 and 3.9 years. External ventricular drains were inserted in 18 children with hydrocephalus preoperatively. VAE was detected in 6 instances during surgery (6/26 (23.1%)). In 3 patients with grade 1 VAE, no additional treatment was necessary. In one patient with grade 2 VAE, intracardiac air suction via the central venous catheter was performed, and in two patients with grade 4 VAE, additional cathecholamine-infusion was administered. No major intraoperative complications occurred. Postoperative CT images showed pneumocephalus in all children. In two children, small asymptomatic impression skull fractures at the site of the Mayfield pin occurred. Revision surgery was necessary in one child with a suboccipital CSF fistula. CONCLUSION: The semi-sitting position for resection of tumors in the posterior fossa in children younger than 4 years of age can be safely performed in experienced centers taking special caution to detect and treat potential complications in an interdisciplinary setting.
Assuntos
Neoplasias Encefálicas , Embolia Aérea , Neoplasias Infratentoriais , Masculino , Feminino , Humanos , Criança , Pré-Escolar , Postura Sentada , Procedimentos Neurocirúrgicos/métodos , Estudos Retrospectivos , Posicionamento do Paciente/efeitos adversos , Neoplasias Encefálicas/complicações , Neoplasias Infratentoriais/complicações , Embolia Aérea/etiologiaRESUMO
OBJECTIVE: Posterior fossa or midline tumors are often associated with hydrocephalus and primary tumor removal with or without perioperative placement of an external ventricular drain (EVD) is commonly accepted as first-line treatment. Shunting prior to posterior fossa surgery (PFS) is mostly reserved for symptomatic cases or special circumstances. There are limited data regarding the anticipated risk for symptomatic pneumocephalus and the perioperative management using the semi-sitting position (SSP) in such a scenario. Here, we therefore assessed the safety of performing PFS in a consecutive series of patients over a period of 15 years to allow the elaboration of recommendations for perioperative management. METHODS: According to specific inclusion and exclusion criteria a total of 13 patients who underwent 17 operations was identified. Supratentorial pneumocephalus was evaluated with semiautomatic-volumetric segmentation. The volume of pneumocephalus was evaluated according to age and ventricular size. RESULTS: Ten of the 13 patients had a programmable valve (preoperative valve setting range 6-14 cmH20; mean 7.5 cmH20) while 3 patients had non programmable valves. A variable amount of supratentorial air collection was evident in all patients postoperatively (range 3.2-331 ml; mean 122.32 ml). Positive predictors for the volume of postoperative pneumocephalus were higher age and a preoperative Evans ratio > 0.3. In our series, we encountered no cases of tension pneumocephalus necessitating an air replacement procedure as well as no obstruction, disconnection, infection or hardware malfunction of the shunt system. CONCLUSIONS: Our findings indicate that a CSF shunt in situ is not a contraindication for performing PFS in the semi-sitting position and it does not increase the pre-existing risk for postoperative tension pneumocephalus. In cases of primary shunting for hydrocephalus associated with posterior fossa tumors a programmable valve set at a medium opening pressure with a gravitational device is a valid option when PFS in the semi-sitting position is opted. In patients with an indwelling shunt diversion system special caution is indicated in order to prevent and detect overdrainage especially in not adjustable valves or shunts without antisiphon devices.
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Hidrocefalia , Neoplasias Infratentoriais , Pneumocefalia , Humanos , Postura Sentada , Pneumocefalia/diagnóstico por imagem , Pneumocefalia/etiologia , Pneumocefalia/prevenção & controle , Procedimentos Neurocirúrgicos/métodos , Neoplasias Infratentoriais/cirurgia , Hidrocefalia/cirurgia , Hidrocefalia/complicações , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/prevenção & controle , Complicações Pós-Operatórias/diagnóstico , Derivação Ventriculoperitoneal/efeitos adversosRESUMO
Structural and functional abnormalities in the cerebellar midline region, including the fastigial nucleus, have been reported in neuropsychiatric disorders, also comprising the cerebellar cognitive affecting syndrome. In rats, early fastigial lesions reduce social interaction during development and lead to cognitive and emotional deficits in adults, accompanied by compromised neuronal network activity. Since epigenetic mechanisms are implicated in the etiology of neuropsychiatric disorders, we investigated whether fastigial nucleus lesions in juvenile rats would impact epigenetic regulation of neural transmission. The fastigial nucleus was lesioned bilaterally in 23-day-old male rats. Sham-lesion and naïve rats served as controls. DNA methylation was investigated for target genes of the GABAergic, dopaminergic, glutamatergic and oxytocinergic systems in brain regions with anatomic connections to the fastigial nucleus, i.e., medial prefrontal cortex, nucleus accumbens, striatum, thalamus, and sensorimotor cortex. Protein expression was examined for the respective target genes in case of altered DNA methylation between lesion and control groups. Lesioning of the fastigial nucleus led to significant differences in the epigenetic regulation of glutamate decarboxylase 1 and the oxytocin receptor in the nucleus accumbens and the prefrontal cortex. No differences were found for the other target genes and brain regions. Our findings indicate that epigenetic dysregulation after lesioning of the fastigial nucleus may influence long-term recovery and the emergence of behavioral changes. Together with previous behavioral and electrophysiological investigations of this rat model, these observations can play a role in the cerebellar cognitive affective syndrome and other neuropsychiatric disorders.
Assuntos
Núcleos Cerebelares , Epigênese Genética , Animais , Núcleos Cerebelares/metabolismo , Cerebelo/fisiologia , Masculino , Córtex Pré-Frontal , Ratos , Transmissão SinápticaRESUMO
Alagille syndrome (AS) is a rare multisystem disease of the liver, heart, eyes, face, skeleton, kidneys, and vascular system. The occurrence of pseudotumor cerebri syndrome (PTCS) in patients with AS has been reported only exceptionally. Owning to its rarity and a mostly atypical presentation, the diagnosis and natural history of affected patients remain uncertain. We report an atypical case of PTCS in a 4-year-old boy with a known history of AS who presented with bilateral papilledema (PE) on a routine ophthalmological examination. Visual findings deteriorated after treatment with acetazolamide. Continuous intracranial pressure (ICP) monitoring was then utilized to investigate ICP dynamics. Successful treatment with resolution of PE was achieved after ventriculoperitoneal shunting but relapsed due to growth-related dislocation of the ventricular catheter. This report brings new insights into the ICP dynamics and the resulting treatment in this possibly underdiagnosed subgroup of PTCS patients. It also demonstrates that ventriculoperitoneal shunting can provide long-term improvement of symptoms for more than 10 years.
Assuntos
Síndrome de Alagille , Papiledema , Pseudotumor Cerebral , Síndrome de Alagille/complicações , Síndrome de Alagille/cirurgia , Pré-Escolar , Humanos , Pressão Intracraniana , Masculino , Pseudotumor Cerebral/complicações , Pseudotumor Cerebral/cirurgia , Resultado do TratamentoRESUMO
Rosette-forming glioneuronal tumor (RGNT) is a rare tumor entity which has been reported mainly occurring in the fourth ventricle. It has been described as a benign lesion with limited extension into surrounding structures, including the cerebellar vermis, midbrain, and cerebral aqueduct. More recently, few cases involving also other midline structures have been documented as well. Here, we report about diagnosis and treatment of RGNT in the septum pellucidum in a pediatric patient which has not been described previously. A 7-year-old boy had a 3-week history of headache. Magnetic resonance imaging showed a solid mass in the septum pellucidum accompanied by hydrocephalus. The tumor was resected via a transcortical approach. Histological examination revealed the typical findings of a RGNT. At 2-year follow-up, there was no tumor recurrence, and clinical outcome was unremarkable. RGNT has to be considered in the differential diagnosis of pediatric midline tumors also outside of the fourth ventricle. Surgical resection is the first-line therapy which may result in beneficial outcome in the long term. The role of adjuvant therapy needs further definition since due to the rarity of this tumor entity, available data is very limited.
Assuntos
Neoplasias do Ventrículo Cerebral , Glioma , Neoplasias do Ventrículo Cerebral/diagnóstico por imagem , Neoplasias do Ventrículo Cerebral/cirurgia , Criança , Quarto Ventrículo , Glioma/diagnóstico por imagem , Glioma/cirurgia , Humanos , Imageamento por Ressonância Magnética , Masculino , Recidiva Local de Neoplasia , Septo Pelúcido/diagnóstico por imagem , Septo Pelúcido/cirurgiaRESUMO
Secondary spinal manifestations of esthesioneuroblastoma are rare. A 67-year-old woman was presented with an extradural spinal manifestation at the vertebra Th7 within 8 weeks after resection of an esthesioneuroblastoma. Subtotal removal of the epidural tumour was achieved combined with dorsal transpedicular stabilization. Early screening for distant metastases may be considered in patients with esthesioneuroblastoma.
Assuntos
Estesioneuroblastoma Olfatório/secundário , Cavidade Nasal , Neoplasias Nasais , Neoplasias da Coluna Vertebral/secundário , Idoso , Estesioneuroblastoma Olfatório/cirurgia , Evolução Fatal , Feminino , Humanos , Imageamento por Ressonância Magnética , Fraturas da Coluna Vertebral/etiologia , Neoplasias da Coluna Vertebral/cirurgia , Vértebras TorácicasRESUMO
BACKGROUND: Idiopathic intracranial hypertension (IIH) is characterized by increased cerebrospinal fluid (CSF) pressure and normal or slit ventricles. Lumboperitoneal shunting had been favored by many investigators for CSF diversion in IIH for decades; however, it has been associated with various side effects. Because of the small ventricular size adequate positioning of a ventricular catheter is challenging. OBJECTIVES: Here, we investigated the usefulness of electromagnetic (EM)-guided ventricular catheter placement for ventriculoperitoneal shunting in IIH. METHODS: Eighteen patients with IIH were included in this study. The age of patients ranged from 5 to 58 years at the time of surgery (mean age: 31.8 years; median: 29 years). There were 2 children (5 and 11 years old) and 16 adults. Inclusion criteria for the study were an established clinical diagnosis of IIH, lack of improvement with medication, and the presence of small ventricles. In all patients EM-navigated placement of the ventricular catheter was performed using real-time tracking of the catheter tip for exact positioning close to the foramen of Monro. Postoperative CT scans were correlated with intraoperative screen shots to validate the position of the catheter. RESULTS: In all patients EM-navigated ventricular catheter placement was achieved with a single pass. There were no intraoperative or postoperative complications. Postoperative imaging confirmed satisfactory positioning of the ventricular catheter. No proximal shunt failure was observed during the follow-up at a mean of 41.5 months (range: 7-90 months, median: 40.5 months). CONCLUSIONS: EM-navigated ventricular catheter placement in shunting for IIH is a safe and straightforward technique. It obviates the need for sharp head fixation, the head of the patient can be moved during surgery, and it may reduce the revision rate during follow-up.
Assuntos
Derivações do Líquido Cefalorraquidiano , Procedimentos Neurocirúrgicos/métodos , Pseudotumor Cerebral/cirurgia , Adulto , Criança , Pré-Escolar , Fenômenos Eletromagnéticos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neuronavegação , Pseudotumor Cerebral/diagnóstico por imagem , Resultado do Tratamento , Adulto JovemRESUMO
PURPOSE: Desmoplastic infantile gliomas (DIG) are rare tumors that occur in infants aged between 1 and 24 months. The tumor in general has a favorable prognosis after surgical resection. There are no treatment algorithms, however, for patients with multiple intracranial and intraspinal presentations. CASE REPORT: In an 11-month-old girl with a history of nystagmus, magnetic resonance imaging (MRI) demonstrated contrast-enhancing lesions in the suprasellar region, the cerebellar vermis, and the spinal axis. The tumor in the cerebellar vermis was removed via a suboccipital midline approach. The histological examination revealed a desmoplastic infantile astrocytoma (DIA) WHO grade I. Postoperatively, it was decided to adopt a wait-and-see strategy. Further development, up to 16 months after surgery, was unremarkable. Follow-up MRI showed no recurrence of the posterior fossa tumor, mild progress of the suprasellar tumor, and significant regression of the spinal tumors. CONCLUSION: DIA is a rare mostly benign brain tumor found in infants. The final diagnosis always relies on histology. Surgical resection is the recommended therapy for symptomatic tumors; however, more experience is needed to develop treatment recommendations for multiple-site tumors.
Assuntos
Astrocitoma/terapia , Neoplasias Encefálicas/terapia , Neoplasias da Coluna Vertebral/terapia , Astrocitoma/diagnóstico , Neoplasias Encefálicas/diagnóstico , Feminino , Seguimentos , Humanos , Lactente , Imageamento por Ressonância Magnética , Neoplasias da Coluna Vertebral/diagnósticoRESUMO
PURPOSE: Navigated intracranial endoscopy with conventional technique usually requires sharp head fixation. In children, especially in those younger than 1 year of age and in older children with thin skulls due to chronic hydrocephalus, sharp head fixation is not possible. Here, we studied the feasibility, safety, and accuracy of electromagnetic (EM)-navigated endoscopy in a series of children, obviating the need of sharp head fixation. METHODS: Seventeen children (ten boys, seven girls) between 12 days and 16.8 years (mean age 4.3 years; median 14 months) underwent EM-navigated intracranial endoscopic surgery based on 3D MR imaging of the head. Inclusion criteria for the study were intraventricular cysts, arachnoid cysts, aqueduct stenosis for endoscopic third ventriculostomy (ETV) with distorted ventricular anatomy, the need of biopsy in intraventricular tumors, and multiloculated hydrocephalus. A total of 22 endoscopic procedures were performed. Patients were registered for navigation by surface rendering in the supine position. After confirming accuracy, they were repositioned for endoscopic surgery with the head fixed slightly on a horseshoe headholder. EM navigation was performed using a flexible stylet introduced into the working channel of a rigid endoscope. Neuronavigation accuracy was checked for deviations measured in millimeters on screenshots after the referencing procedure and during surgery in the coronal (z = vertical), axial (x = mediolateral), and sagittal (y = anteroposterior) planes. RESULTS: EM-navigated endoscopy was feasible and safe. In all 17 patients, the aim of endoscopic surgery was achieved, except in one case in which a hemorrhage occurred, blurring visibility, and we proceeded with open surgery without complications for the patient. Navigation accuracy for extracranial markers such as the tragus, bregma, and nasion ranged between 1 and 2.5 mm. Accuracy for fixed anatomical structures like the optic nerve or the carotid artery varied between 2 and 4 mm, while there was a broader variance of accuracy at the target point of the cyst itself ranging between 2 and 9 mm. CONCLUSIONS: EM-navigated endoscopy in children is a safe and useful technique enhancing endoscopic intracranial surgery and obviating the need of sharp head fixation. It is a good alternative to the common opto-electric navigation system in this age group.
Assuntos
Cistos Aracnóideos/cirurgia , Fenômenos Eletromagnéticos , Endoscopia/métodos , Hidrocefalia/cirurgia , Neuronavegação/métodos , Ventriculostomia/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Posicionamento do Paciente , Estudos Retrospectivos , Resultado do TratamentoRESUMO
The surgical treatment of distal anterior cerebral artery (DACA) aneurysms still presents a challenge for neurosurgeons because of their small size and their location in the depth of the narrow frontal interhemispheric fissure. This study aimed to investigate feasibility, safety, accuracy, and usefulness of electromagnetic (EM) navigation to aid clipping of DACA aneurysms. Eight patients (age between 2 and 68 years, mean age 49.8 years) with a DACA aneurysm underwent EM-guided neuronavigated microsurgery for clipping of the aneurysm. All patients underwent craniocervical 3D-CT angiography preoperatively. After planning the optimal approach and surgical trajectory avoiding opening of the frontal sinus, the head was fixed. Intraoperative screenshots were correlated with the microscopical view of the DACA aneurysms before clipping. EM-guided neuronavigation using CT angiography for DACA aneurysms enabled fast and accurate referencing of the patient and planning of a tailored craniotomy without opening of the frontal sinus. Intraoperative accuracy was highly reliable except in one instance due to dislocation of the dynamic reference frame (DRF). There was a good correlation between the 3D-CT angiography-based navigation data sets and the intraoperative vascular anatomy. In all patients, bridging veins were spared. The aid of EM neuronavigation was considered useful in all instances. EM-guided neuronavigation using CT angiography for surgery of DACA aneurysms is a useful tool optimizing the surgical approach directly to the aneurysm minimizing additional damage to the surrounding tissue during preparation of the aneurysm and the parent vessel.
Assuntos
Artéria Cerebral Anterior/cirurgia , Aneurisma Intracraniano/cirurgia , Neuronavegação/métodos , Procedimentos Neurocirúrgicos/métodos , Adolescente , Adulto , Idoso , Aneurisma Roto/cirurgia , Angiografia Cerebral , Criança , Pré-Escolar , Campos Eletromagnéticos , Feminino , Humanos , Processamento de Imagem Assistida por Computador , Masculino , Microcirurgia/métodos , Pessoa de Meia-Idade , Neuronavegação/efeitos adversos , Procedimentos Neurocirúrgicos/efeitos adversos , Estudos Prospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Electromagnetic (EM)-guided neuronavigation is an innovative technique and a viable alternative to opto-electric navigation. We have performed a safety and feasibility study using EM-guided neuronavigation for posterior fossa surgery in the semi-sitting position in a selected subset of patients. METHODS: Out of 284 patients with posterior fossa tumours operated upon over a period of 40 months, a subset of 15 patients was thought to possibly benefit from EM navigational guidance and was included in this study. There were six children and nine adults (aged between 8 and 84 years; mean age, 34.6 years) with different neoplasms in the brainstem or close to the midline. All patients had contrast-enhanced three-dimensional (3D) magnetic resonance imaging (MRI) of the head preoperatively. EM-guided navigation was used to identify and preserve the venous sinuses during craniotomy and to determine the trajectory to the lesion using various approaches. Neuronavigation accuracy was repeatedly checked for deviations measured in millimetres on screen shots during surgery before and after dural opening in the coronal (z = vertical), axial (x = mediolateral) and sagittal (y = anteroposterior) plane. RESULTS: Referencing of the patient in the supine position was fast and easy. There was no loss of navigation accuracy after repositioning of the patient in the semi-sitting position (mean, 2.5 mm ± 0.92 mm). Identification of the pathological structure using EM navigation was achieved in all instances. Optimal angulation of the neck was selected individually to permit a comfortable position for the surgeon with full access to the lesion avoiding over-flexion. Deviation of accuracy at the surface of the target lesion ranged between 2.5 and 5.8 mm (mean, 3.9 mm ± 1.1 mm). CONCLUSIONS: EM-guided neuronavigation in the semi-sitting position was safe and technically feasible. It enabled fast and accurate referencing without loss of navigation accuracy despite repositioning of the patient. In contrast to conventional opto-electric neuronavigation there were no line of sight problems.
Assuntos
Fossa Craniana Posterior/cirurgia , Campos Eletromagnéticos , Neuronavegação/métodos , Procedimentos Neurocirúrgicos/métodos , Posicionamento do Paciente/métodos , Neoplasias da Base do Crânio/cirurgia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Fossa Craniana Posterior/patologia , Campos Eletromagnéticos/efeitos adversos , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neuronavegação/efeitos adversos , Procedimentos Neurocirúrgicos/efeitos adversos , Adulto JovemRESUMO
PURPOSE: Fanconi anemia is associated with a high risk for developing malignant tumors. The occurrence of primary intracranial leiomyosarcoma, however, which in general has a poor prognosis, has not been described thus far. The purpose of this study is to report on management and outcome of leiomyosarcoma of the torcular Herophili associated with Fanconi anemia in a pediatric patient. CASE REPORT: A 12-year-old girl with Fanconi anemia presented with a primary intracranial leiomyosarcoma arising from the torcular Herophili and infiltrating the adjacent venous sinuses after previous allogenic hematopoietic stem cell transplantation. Radical tumor resection followed by radiotherapy resulted in tumor-free survival and good outcome at a 2-year follow-up. CONCLUSION: Despite occurrence of leiomyosarcoma in a site thought unfavorable for surgery, combined tumor resection and radiosurgery may yield excellent outcome.
Assuntos
Cavidades Cranianas/patologia , Anemia de Fanconi/complicações , Anemia de Fanconi/cirurgia , Leiomiossarcoma/complicações , Leiomiossarcoma/cirurgia , Transplante de Células-Tronco/métodos , Proteínas de Ligação a Calmodulina/metabolismo , Criança , Desmina/metabolismo , Feminino , Gadolínio , Humanos , Imageamento por Ressonância MagnéticaRESUMO
BACKGROUND AND OBJECTIVES: Cavernous sinus meningiomas (CSM) pose one of the most difficult to treat subgroup of skull base meningiomas. The purpose of this study was to evaluate the efficacy of an interdisciplinary treatment approach for symptomatic CSM which incorporated conservative function preserving microsurgery and routine adjuvant fractionated stereotactic radiotherapy (FSRT). METHODS: A homogenous group of patients with symptomatic primary CSM with extracavernous extension was treated between 2005 and 2012. All patients were available for a minimum follow-up of 5 years. Clinical follow-up included detailed examination of oculomotor deficits, visual status, and endocrinologic function. Radiologic follow-up was conducted by tumor volumetry. RESULTS: Overall, 23 patients were included in this study (78.3% women; median age 58 years). Diplopia was the most common presenting symptom, followed by headache and visual disturbances. Surgical morbidity was low (3/23; 13%). FSRT was applied after a median of 2 months after surgery. At a median clinical follow-up of 113 months, 70.45% of the presenting symptoms had improved, 25% remained unchanged, and in 2 cases (4.54%), worsening occurred. Overall tumor regression was evident in 19/21 World Health Organization 1 and in 1/2 of World Health Organization 2 CSM, respectively, at a median radiological follow-up of 103 months. CONCLUSION: Our findings demonstrate the efficacy of an interdisciplinary treatment approach for symptomatic primary CSM with extracavernous extension with decompression of neurovascular elements followed by FSRT. Precise preoperative planning and intraoperative decision making in combination with routine postoperative radiotherapy can achieve excellent tumor control, improve neurologic function, and minimize long-term morbidity.
Assuntos
Seio Cavernoso , Neoplasias Meníngeas , Meningioma , Microcirurgia , Radiocirurgia , Humanos , Feminino , Pessoa de Meia-Idade , Masculino , Meningioma/cirurgia , Meningioma/radioterapia , Meningioma/diagnóstico por imagem , Radiocirurgia/métodos , Idoso , Seio Cavernoso/cirurgia , Seio Cavernoso/diagnóstico por imagem , Resultado do Tratamento , Neoplasias Meníngeas/radioterapia , Neoplasias Meníngeas/cirurgia , Neoplasias Meníngeas/diagnóstico por imagem , Neoplasias Meníngeas/patologia , Microcirurgia/métodos , Adulto , Radioterapia Adjuvante/métodos , Seguimentos , Estudos Retrospectivos , Descompressão Cirúrgica/métodos , Fracionamento da Dose de RadiaçãoRESUMO
OBJECTIVE AND IMPORTANCE: Trochlear nerve schwannomas without neurofibromatosis are very rare. No more than 33 cases have been pathologically verified. None of the reported cases showed postoperative improvement of trochlear palsy. The authors present a case of trochlear palsy due to a trochlear nerve schwannoma with subsequent improvement of trochlear palsy after subtotal excision of the tumour. CLINICAL PRESENTATION: A 65-year-old woman presented with diplopia, vertigo and left arm weakness. There was no history of neurofibromatosis. MR imaging demonstrated a tumour in the right perimesencephalic/ambient cistern. INTERVENTION: The tumour was operated by a pterional approach, and subtotal excision was achieved preserving the tumour capsule with the trochlear nerve. The tumour was pathologically diagnosed as a schwannoma. CONCLUSION: At 2-year follow-up, there was no double vision and MR imaging showed a small, stable tumour remnant. To our knowledge, this is the first reported case of a trochlear schwannoma in whom postoperative improvement of trochlear function was achieved.
Assuntos
Neoplasias dos Nervos Cranianos/cirurgia , Diplopia/cirurgia , Neurilemoma/cirurgia , Doenças do Nervo Troclear/cirurgia , Nervo Troclear , Idoso , Neoplasias dos Nervos Cranianos/complicações , Diplopia/etiologia , Tontura/etiologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Neurilemoma/complicações , Resultado do Tratamento , Doenças do Nervo Troclear/complicações , Vertigem/etiologiaRESUMO
BACKGROUND: Mannitol is used in the treatment of raised intracranial pressure (ICP). The aim of this study was to investigate whether mannitol (MAN) leads to a relevant deterioration in platelet function in routine neurosurgical procedures. METHODS: Thirty-eight patients undergoing elective craniotomy due to a brain tumor with elevated ICP were included. After induction of anaesthesia a blood sample was taken (T1). The patients then received 1 g-kg-1 MAN within 30 minutes. The second blood sample (T2) was obtained 60 minutes after T1. Blood samples were examined by means of aggregometry (Multiplate®) and PFA-100® tests. RESULTS: No patient had clinical signs of increased bleeding. We could not find any deterioration in the aggregometry using Multiplate®, neither in the adenosinediphosphate (ADP), the arachidonic acid (ASPI), or the thrombin receptor activating protein (TRAP) test. PFA-100® closing times (cT) showed a significant prolongation between T1 and T2: collagen/adenosindiphosphate (COL/ADP) test 79s [70/99] and 91s [81/109]; p=0.002); collagen/epinephrine (COL/EPI) test 109s [92/129] and 122s [94/159]; p=0.0004). A subgroup analysis showed that the patients who received isotonic balanced infusions only, had no prolongation of cT, whereas the patients who received additionally gelatine solution had a significant prolongation. COL/ADP: 78s [70/98] and 91s [82/133]; p=0.0004). COL/EPI: test 111s [92/128] and 127s [103/146]; p=0.0026). Except for individual outliers, the measured values were in the normal range. CONCLUSIONS: In this study, we found no clinically relevant deterioration of platelet function in neurosurgical patients with increased ICP after administration of MAN. Changes that occurred were all within normal ranges.
RESUMO
OBJECTIVE: Osteolytic lesions of the skull in children have a broad differential diagnosis including congenital, inflammatory and neoplastic lesions. Progressive osteolysis of the skull secondary to head trauma is rare and has been poorly characterized. METHODS: The pediatric database at our hospital was screened for children with osteolytic lesions who had a previous mild head injury without fracture or dural tears. We identified 2 children with circumscribed progressive cranial osteolysis after minor head trauma detected by CT and MRI scans. Three additional cases were found in a review of the published literature. RESULTS: Ten children with an osteolytic skull lesion treated between January 1998 and February 2008 were identified in our pediatric database. In 2 children there was evidence of previous mild head injury without a skull fracture. Trauma had occurred 7 and 2 months prior to presentation, respectively. The inner table of the skull was intact in both cases. In 1 case, surgery was performed, and in the other case, a wait-and-see strategy was adopted. Pathological examination in case 1 revealed an organized hemorrhage with focal papillary endothelial hyperplasia. CONCLUSIONS: Progressive osteolytic calvarial lesions may occur in both infants and adolescents after mild head injury. They involve either only the diploe and outer table of the skull or both the inner and outer tables. These lesions might be due to intradiploic or subgaleal hematomas triggering an inflammatory process. While surgical resection can be considered to confirm a histopathological diagnosis and to exclude other diagnoses, spontaneous reossification is possible.
Assuntos
Traumatismos Craniocerebrais/diagnóstico , Progressão da Doença , Osteólise/diagnóstico , Crânio/patologia , Adolescente , Criança , Pré-Escolar , Traumatismos Craniocerebrais/complicações , Feminino , Humanos , Lactente , Masculino , Osteólise/etiologia , Estudos Retrospectivos , Adulto JovemRESUMO
OBJECTIVE: Routine use of the semisitting position, which offers several advantages, remains a matter of debate. Venous air embolism (VAE) is a potentially serious complication associated with the semisitting position. In this study, the authors aimed to investigate the safety of the semisitting position by analyzing data over a 20-year period. METHODS: The incidence of VAE and its perioperative management were analyzed retrospectively in a consecutive series of 740 patients who underwent surgery between 1996 and 2016. The occurrence of VAE was defined by detection of bubbles on transthoracic Doppler echocardiography (TTDE) or transesophageal echocardiography (TEE) studies, a decrease of end-tidal CO2 (ETCO2) by 4 mm Hg or more, and/or an unexplained drop in systolic arterial blood pressure (≥ 10 mm Hg). From 1996 until 2013 TTDE was used, and from 2013 on TEE was used. The possible risk factors for VAE and its impact on surgical performance were analyzed. RESULTS: There were 404 women and 336 men with a mean age at surgery of 49 years (range 1-87 years). Surgery was performed for infratentorial lesions in 709 patients (95.8%), supratentorial lesions in 17 (2.3%), and cervical lesions in 14 (1.9%). The most frequent pathology was vestibular schwannoma. TEE had a higher sensitivity than TTDE. While TEE detected VAE in 40.5% of patients, TTDE had a detection rate of 11.8%. Overall, VAE was detected in 119 patients (16.1%) intraoperatively. In all of these patients, VAE was apparent on TTDE or TEE. Of those, 23 patients also had a decrease of ETCO2, 18 had a drop in blood pressure, and 23 had combined decreases in ETCO2 and blood pressure. VAE was detected in 24% of patients during craniotomy before opening the dura mater, in 67% during tumor resection, and in 9% during wound closure. No risk factors were identified for the occurrence of VAE. Two patients had serious complications due to VAE. Surgical performance in vestibular schwannoma surgery was not affected by the presence of VAE. CONCLUSIONS: This study shows that the semisitting position is overall safe and that VAE can be managed effectively. Persistent morbidity is very rare. The authors suggest that the semisitting position should continue to have a place in the standard armamentarium of neurological surgery.