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The transorbital approach (TOA) can provide immediate access to the lateral ventricles by piercing the roof of the orbit (ROO) with a spinal needle and without the need of a drill. Reliable external landmarks for the TOA ventriculostomy have been described, however, the necessary spinal needle gauge and other relevant parameters such as the thickness of the ROO have not been evaluated. Nineteen formalin-fixed adult cadaveric heads underwent the TOA. Spinal needles of different gauges were consecutively used in each specimen beginning with the smallest gauge until the ROO was successfully pierced. The thickness of the ROO at the puncture site and around its margins was measured. Other parameters were also measured. The TOA was successfully performed in 14 cases (73.68%), where the most suitable needle gauge was 13 (47.37%), followed by a 10-gauge needle (36.84%). The mean thickness of the ROO at the puncture site, and the mean length of the needle to the puncture site were 1.7 mm (range 0.2-3.4 mm) and 15.5 mm (range 9.2-23.4 mm), respectively. A ROO thickness of greater than 2.0 mm required a 10-gauge needle in seven cases, and in five cases, a 10-gauge needle was not sufficient for piercing the ROO. The presence of hyperostosis frontalis interna (HFI) (21.05%) was related to the failure of this procedure (80%; p < 0.00). Using a 13/10-gauge spinal needle at Tubbs' point for TOA ventriculostomy allowed for external ventricular access in most adult specimens. The presence of HFI can hinder this procedure. These findings are important when TOA ventriculostomy is considered.
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Drenagem , Formaldeído , Adulto , Humanos , Ventrículos Laterais , Agulhas , VentriculostomiaRESUMO
Patients are at risk of increased intracranial pressure (ICP) during manipulation for endotracheal intubation. The aim of this cadaveric study was to quantify this relationship. Five fresh frozen adult cadaveric heads were used. The internal carotid artery (ICA) and internal jugular vein (IJV) were dissected bilaterally. All vessels were occluded on the right side. To mimic central venous pressures, the left IJV was cannulated and filled with water to maintain a pressure of approximately 7 cm H2O. The ICA cannula was attached to an oscillating water pump. Next, an ICP monitor was placed. Normophysiological volumes of water were infused to maintain normal ICP. Baseline ICP and IJV pressures were recorded. The heads were then placed in the sniffing position and in neutral position for nasal intubation, and finally an oral endotracheal tube was placed using standard head/neck maneuvers. The ICP was recorded. Mean baseline ICP was 13 mmHg (range 4-18 mmHg) and mean IJV pressure was 6 cm H2O. In the sniffing position, ICP increased in all specimens by a mean of 5 mmHg. In the neutral position, after intubation, the ICP increased in all specimens by a mean of 3 mmHg. Nasal intubation resulted in an increase in four of the five specimens. The mean increase in ICP with this maneuver was 2 mmHg. We present a cadaveric model of ICP measurement during neck manipulation using intracranial fluid dynamics. The ICP increased consistently. Following clinical studies, physicians involved in intubating patients with concern for raised ICP should change the technique to avoid complications.
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Pressão Intracraniana , Intubação Intratraqueal , Adulto , Humanos , Cadáver , Hipertensão Intracraniana , Intubação Intratraqueal/métodos , Veias Jugulares/fisiologiaRESUMO
PURPOSE: Inflammatory myofibroblastic tumor (IMT) is a rare neoplastic tumor type of intermediate biological potential, only recently distinguished from the non-neoplastic category of inflammatory pseudotumor (IP). The literature describes very few cases of IMTs arising in the central nervous system (CNS), and the distinguishing clinical, pathological, and molecular features of IMT-CNS are not well understood. Our purpose is to publish a case of an IMT-CNS with a novel DCTN1-ALK gene fusion, furthering in the literature's characterization of a rare tumor type. METHODS: Review of the literature included a PubMed Database search of articles found by the following searches: "Inflammatory myofibroblastic tumor;" "Inflammatory myofibroblastic tumor central nervous system;" "ALK gene fusion;" and "DCTN1-ALK gene fusion." Inclusion of articles discovered by these search terms was determined through critical appraisal of article relevance, number of citations, cross-citation within articles of interest, and rare findings with conflicting conclusions in an effort to reduce publication bias. RESULTS: We present a case of IMT-CNS with several distinctive molecular features including a DCTN1-ALK gene fusion, the first of its kind described in an intracranial IMT. CONCLUSION: IMT is an infrequent tumor type and its presentation within the CNS is exceedingly rare. The paucity of cases, along with the ambiguity of terminology in the literature, has stunted accurate clinical, pathological, and molecular characterization of IMT-CNS. Our case report improves the characterization of the recently appreciated category of IMT-CNS so that connections between phenotype and prognosis, and between genotype and treatment, can eventually be made.
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Granuloma de Células Plasmáticas , Quinase do Linfoma Anaplásico , Sistema Nervoso Central , Complexo Dinactina , Fusão Gênica , Granuloma de Células Plasmáticas/diagnóstico por imagem , Granuloma de Células Plasmáticas/genética , Granuloma de Células Plasmáticas/cirurgia , Humanos , Receptores Proteína Tirosina Quinases/genéticaRESUMO
OBJECTIVE: The Global Burden of Disease (GBD) is an international collaboration and the largest comprehensive investigation of global health disease burden ever conducted. It has been particularly insightful for understanding disease demographics in middle-income nations undergoing rapid development, such as Vietnam, where 6 of the top 10 causes of death are relevant to the neurosurgeon. The burden of stroke-the number one cause of death in Vietnam-is particularly impressive. Likewise, road injuries, with a disproportionate rate of traumatic brain injury, continue to increase in Vietnam following economic development. Low-back and neck pain is the number one cause of disability. Simultaneously, more patients have access to care, and healthcare spending is increased. METHODS: It is imperative that neurosurgical capital and infrastructure keep pace with Vietnam's growth. The authors searched the existing literature for assessments of neurosurgical infrastructure or initiatives to address neurosurgical disease burden. Using GBD data, the authors also abstracted data for death by cause and prevalence of years of life lost due to disability (YLD) for common neurosurgical pathologies for Vietnam and comparison nations. RESULTS: Interventions aimed at primary prevention of risk factors for neurosurgical disease and focused on the transference of self-sustainable technical skills were found to be analogous to those that have been successful in other regions. Efforts toward stroke prevention have been focused on causal risk factors. Multiple investigators have found that interventions aimed at increasing helmet use were successful in preventing traumatic brain injury. Government-led reforms and equipment donation programs have improved technical capacity. Nevertheless, Vietnam lags behind other nations in neurosurgeons per capita; cause-attributable death and YLD attributable to neurosurgical disease are considerably higher in Vietnam and middle-income nations compared to both lower-income nations and upper-income nations. CONCLUSIONS: More than two-thirds of deaths attributable to neurosurgical pathologies in Vietnam and other middle-income nations were due to stroke, and one-fifth of both cause-attributable death and YLD was associated with neurosurgical pathologies. Vietnam and other middle-income nations continue to assume a global burden of disease profile that ever more closely resembles that of developed nations, with particular cerebrovascular, neurotrauma, and spinal disease burdens, leading to exponentially increased demand for neurosurgeons that threatens to outpace the training of neurosurgeons.
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Carga Global da Doença , Doenças do Sistema Nervoso/epidemiologia , Acidentes de Trânsito/estatística & dados numéricos , Lesões Encefálicas Traumáticas/epidemiologia , Humanos , Doenças do Sistema Nervoso/mortalidade , Acidente Vascular Cerebral/epidemiologia , Acidente Vascular Cerebral/mortalidade , Vietnã/epidemiologiaRESUMO
Aplasia cutis congenita is a rare scalp defect with occasional absence of underlying bone and dura. Patients with small defects respond well to conservative management. However, larger defects often can pose management and technical challenge. Successful management of a large, composite aplasia cutis congenita defect using Integra dermal regeneration matrix is presented. At 16 months, the patient showed a stable and supple scalp with evidence of calvarial regeneration.
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Displasia Ectodérmica/cirurgia , Procedimentos de Cirurgia Plástica/métodos , Couro Cabeludo/cirurgia , Crânio/cirurgia , Humanos , Recém-Nascido , Couro Cabeludo/anormalidades , Crânio/anormalidadesRESUMO
BACKGROUND: Spontaneous resolution of syringomyelia has rarely been reported in the literature. Rarer still are cases wherein this process is associated with pregnancy and parturition. We review theories on syringomyelia development and spontaneous resolution to better understand the role pregnancy and parturition may play in both processes. METHODS: We present a 30-year-old female with MRI-confirmed spontaneous syrinx regression following caesarean delivery of a full-term pregnancy. We additionally review the literature to identify previously reported cases of spontaneous syrinx regression both independent of and associated with pregnancy. RESULTS: Including the present case, 39 cases describing spontaneous regression of syringomyelia have been reported in the literature, of which only four are associated with pregnancy and parturition. 75% of all reported cases were associated with type I Chiari malformation, though several disorders of the craniocervical junction and spinal canal were implicated. Complete syrinx regression was achieved in 33.3% of cases and 5% of cases described recurrence of syringomyelia following the spontaneous resolution. CONCLUSION: Syringomyelia likely develops due to disturbance of the physiologic flow of cerebrospinal fluid around the craniocervical junction and the obex. Several mechanisms including fissuring of the spinal cord parenchyma and reduction of subarachnoid scarring are likely involved in this process. In the setting of pregnancy, additional mechanisms surrounding the increased intraabdominal forces imparted by a growing fetus, Valsalva-like strain experienced during labor, and hemodynamic changes that occur to accommodate gestation are likely implicated. Nevertheless, patients should continue to be monitored periodically for syrinx recurrence.
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Malformação de Arnold-Chiari , Siringomielia , Gravidez , Feminino , Humanos , Adulto , Siringomielia/complicações , Siringomielia/diagnóstico por imagem , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico por imagem , Espaço Subaracnóideo , Imageamento por Ressonância Magnética , PartoRESUMO
BACKGROUND: Despite the success of folic acid fortification programs, neural tube defects (NTDs) such as spina bifida, encephalocele, and anencephaly remain among the most substantial causes of childhood morbidity and mortality worldwide. Although these are complicated conditions that require an interdisciplinary approach to care, definitive treatment of survivable NTDs is often neurosurgical. METHODS: Using Global Burden of Disease data, we examined the global burden of NTDs as related to a nation's wealth, health care quality, and access to neurosurgical care. We abstracted data for death by cause, years lived with disability (YLD), gross domestic product (GDP), United Nations geoscheme, Food Fortification Initiative participation, and Healthcare Access and Quality Index. We compared means using 1-way analysis of variance and proportions using Fisher exact tests, with statistical significance as α = 0.05. RESULTS: Seventeen of 20 (85%) nations with the most deaths caused by NTDs (P < 0.0001) and 15/20 (75%) nations with the highest YLD (P < 0.0001) were in the lowest GDP quartile. Deaths and YLD were negatively correlated with increasing GDP and Healthcare Access and Quality Index (P < 0.0001). The nations with the highest disease burdens also had the fewest neurosurgeons per capita. CONCLUSIONS: Despite the success of folic acid fortification programs, greater global public health efforts should be placed on improving access to neurosurgical care in low and middle-income nations through sustainable initiatives such as surgeon exchange programs and the establishment of neurosurgery residency training programs.
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Anencefalia/cirurgia , Ácido Fólico/metabolismo , Defeitos do Tubo Neural/cirurgia , Procedimentos Neurocirúrgicos , Efeitos Psicossociais da Doença , Humanos , Neurocirurgiões , Neurocirurgia/métodosRESUMO
Idiopathic Intracranial Hypertension (IIH) typically occurs in obese (BMI >30 kg/m2) females of childbearing age in the absence of any apparent intracranial space-occupying lesion. Patients typically present with headache, nausea, vomiting, tinnitus, and blurry vision secondary to increased intracranial pressure, with more severe cases involving cranial neuropathies and ophthalmological manifestations. Complete ophthalmoplegia is a rare event in IIH. In such cases, aggressive management with pharmacological, endovascular, and surgical intervention is essential to hasten recovery and limit long-term neurological and visual deficits. Herein, we present a rare case of a patient with IIH associated with third, fourth, and sixth cranial nerve palsies, resulting in complete unilateral ophthalmoplegia, who underwent dural sinus stenting and 2.5-year follow-up revealed complete resolution with full extraocular movements. We also perform a systematic literature review of complete and partial ophthalmoplegia secondary to IIH, highlighting the associated presentations, pathophysiology, management, and outcomes.
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Cavidades Cranianas/cirurgia , Oftalmoplegia/cirurgia , Pseudotumor Cerebral/cirurgia , Stents , Adulto , Feminino , Humanos , Oftalmoplegia/etiologia , Pseudotumor Cerebral/complicações , Resultado do TratamentoRESUMO
BACKGROUND AND OBJECTIVE: Pigmented villonodular synovitis (PVNS) is a lesion of uncertain etiology that involves the synovial membranes of joints or tendon sheaths, representing a diffuse and non-encapsulated form of the more common giant cell tumors of the synovium (GCTTS). PVNS was reclassified to denote a diffuse form of synovial giant cell tumor (TSGCT), while 'giant cell tumor of the tendon sheath (GCTTS)' was used for localized lesions. These pathologies rarely affect the axial skeleton. We provide an unprecedented and extensive systematic review of both lesions highlighting presentation, diagnostic considerations, treatment, prognosis, and outcomes, and we report a short case-series. METHOD: We describe two-cases and conduct a systematic review in accordance with PRISMA guidelines. RESULT: PVNS was identified in most of the cases reviewed (91.6 %), manifesting predominantly in the cervical spine (40 %). Patients commonly presented with neck pain (59 %), back pain (53 %), and lower back pain (81.2 %) for cervical, thoracic, and lumbar lesions, respectively. GTR occurred at rates of 94 %, 80 %, and 87.5 %. Recurrence was most common in the lumbar region (30.7 %). GCTTS cases (8%) manifested in the cervical and thoracic spine at the same frequency. We reported first case of GCTTS in the lumbosacral region. Both poses high rate of facet and epidural involvements. CONCLUSION: Spinal PVNS and GCTTS are rare. These lesions manifest most commonly as PVNS within the cervical spine. Both types have a high rate of facet and epidural involvement, while PVNS has the highest rate of recurrence within the lumbar spine. The clinical and radiological features of these lesions make them difficult to differentiate from others with similar histogenesis, necessitating tissue diagnosis. Proper management via GTR resolves the lesion, with low rates of recurrence.
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Tumor de Células Gigantes de Bainha Tendinosa/diagnóstico por imagem , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Sinovite Pigmentada Vilonodular/diagnóstico por imagem , Adulto , Dor nas Costas/fisiopatologia , Feminino , Transtornos Neurológicos da Marcha/fisiopatologia , Tumor de Células Gigantes de Bainha Tendinosa/patologia , Tumor de Células Gigantes de Bainha Tendinosa/fisiopatologia , Tumor de Células Gigantes de Bainha Tendinosa/cirurgia , Humanos , Hipestesia/fisiopatologia , Dor Lombar/fisiopatologia , Vértebras Lombares/diagnóstico por imagem , Vértebras Lombares/cirurgia , Imageamento por Ressonância Magnética , Debilidade Muscular/fisiopatologia , Cervicalgia/fisiopatologia , Procedimentos Neurocirúrgicos , Sacro/diagnóstico por imagem , Sacro/cirurgia , Fusão Vertebral , Neoplasias da Coluna Vertebral/patologia , Neoplasias da Coluna Vertebral/fisiopatologia , Neoplasias da Coluna Vertebral/cirurgia , Sinovite Pigmentada Vilonodular/patologia , Sinovite Pigmentada Vilonodular/fisiopatologia , Sinovite Pigmentada Vilonodular/cirurgia , Vértebras Torácicas/diagnóstico por imagem , Vértebras Torácicas/cirurgia , Adulto JovemRESUMO
BACKGROUND: Medical subspecialties including neurosurgery have seen a dramatic shift in operative volume in the wake of the coronavirus disease 2019 (COVID-19) pandemic. The goal of this study was to quantify the effects of the COVID-19 pandemic on operative volume at 2 academic neurosurgery centers in New Orleans, Louisiana, USA from equivalent periods before and during the COVID-19 pandemic. METHODS: A retrospective review was conducted analyzing neurosurgical case records for 2 tertiary academic centers from March to June 2020 and March to June 2019. The records were reviewed for variables including institution and physician coverage, operative volume by month and year, cases per subspecialty, patient demographics, mortality, and morbidity. RESULTS: Comparison of groups showed a 34% reduction in monthly neurosurgical volume per institution during the pandemic compared with earlier time points, including a 77% decrease during April 2020. There was no change in mortality and morbidity across institutions during the pandemic. CONCLUSIONS: The COVID-19 pandemic has had a significant impact on neurosurgical practice and will likely continue to have long-term effects on patients at a time when global gross domestic products decrease and relative health expenditures increase. Clinicians must anticipate and actively prepare for these impacts in the future.
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Centros Médicos Acadêmicos/tendências , COVID-19/epidemiologia , Internato e Residência/tendências , Procedimentos Neurocirúrgicos/educação , Procedimentos Neurocirúrgicos/tendências , Tempo para o Tratamento/tendências , Centros Médicos Acadêmicos/métodos , Adulto , Idoso , COVID-19/prevenção & controle , Feminino , Humanos , Internato e Residência/métodos , Tempo de Internação/tendências , Masculino , Pessoa de Meia-Idade , Neurocirurgia/educação , Neurocirurgia/métodos , Neurocirurgia/tendências , Procedimentos Neurocirúrgicos/métodos , Nova Orleans/epidemiologia , Pandemias/prevenção & controle , Estudos RetrospectivosRESUMO
Papillary tumors of the pineal region (PTPR) are a rare tumor entity first described in 2003, later codified in the 2007 WHO tumor classification system. PTPRs most commonly occur in the third and fourth decades, with exceedingly rare presentations in pediatric populations. Herein, we present an additional case of a 10-year-old female found to have PTPR in conjunction with Trisomy 21 managed successfully with cerebrospinal fluid diversion and gross total resection (GTR). Three years after resection she has returned to baseline without recurrence. We also performed a comprehensive review of the current literature discussing the diagnosis, treatment, and pathophysiologic correlations in children. Diagnosis and management of PTPRs is a topic that is increasingly garnering attention in the literature given the recent characterization of this tumor entity. However, relatively little is known about the presentation of PTPRs in pediatric populations. In adults, PTPRs have been linked with several chromosomal and genetic abnormalities; however this correlation is limited in pediatric literature. Although GTR is the mainstay for treatment, the application of adult treatment protocols may not be advisable due to age and the developmental changes of the CNS in children.
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Neoplasias Encefálicas/cirurgia , Recidiva Local de Neoplasia/cirurgia , Glândula Pineal/cirurgia , Pinealoma/cirurgia , Adolescente , Neoplasias Encefálicas/patologia , Criança , Feminino , Humanos , Masculino , Recidiva Local de Neoplasia/patologia , Pediatria , Glândula Pineal/patologia , Pinealoma/diagnósticoRESUMO
Split notochord syndrome (SNS) is a rare congenital defect of the central nervous system and has been associated with several anomalies affecting multiple organ systems. One association has been communication with the gastrointestinal tract and the spine, previously identified as a neuroenteric fistula (NEF). Here, the authors describe the unique case of a female infant with SNS and NEF treated with a multistage surgical repair. The three-stage operative plan included a two-stage repair of the defect and temporary subgaleal shunting followed by delayed ventriculoperitoneal shunt placement. The infant recovered well postsurgery and over a 5-year follow-up. A case description, surgical techniques, and rationale are reported. Additionally, a systematic review of the literature utilizing the MEDLINE database was performed.Treatment of SNS with NEF using a multidisciplinary multistaged approach to repair the intestinal defect, close the neural elements, and divert cerebrospinal fluid to the peritoneum is shown to be a safe and viable option for future cases.
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Fístula/cirurgia , Trato Gastrointestinal/cirurgia , Procedimentos Neurocirúrgicos/métodos , Notocorda/anormalidades , Notocorda/cirurgia , Doenças da Medula Espinal/cirurgia , Feminino , Humanos , Recém-Nascido , Espinha Bífida Cística/complicações , Espinha Bífida Cística/cirurgia , Disrafismo Espinal/complicações , Disrafismo Espinal/cirurgia , Síndrome , Resultado do Tratamento , Derivação VentriculoperitonealRESUMO
BACKGROUND: The coronavirus disease of 2019 (COVID-19), which is caused by infection with the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has recently been designated a pandemic by the World Health Organization, affecting 2.7 million individuals globally as of April 25, 2020, with more than 187,000 deaths. An increasing body of evidence has supported central nervous system involvement. METHODS: We conducted a review of the reported data for studies concerning COVID-19 pathophysiology, neurological manifestations, and neuroscience provider recommendations and guidelines. RESULTS: Central nervous system manifestations range from vague nonfocal complaints to severe neurological impairment associated with encephalitis. It is unclear whether the neurological dysfunction results from direct viral injury or systemic disease. The virus could affect brainstem pathways that lead to indirect respiratory dysfunction, in addition to direct pulmonary injury. Necessary adaptations in patient management, triage, and diagnosis are evolving in light of the ongoing scientific and clinical findings. CONCLUSIONS: The present review has consolidated the current body of data regarding the neurological impact of coronaviruses, discussed the reported neurological manifestations of COVID-19, and highlighted the recommendations for patient management. Specific recommendations pertaining to clinical practice for neurologists and neurosurgeons have also been provided.
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Betacoronavirus , Infecções por Coronavirus/complicações , Infecções por Coronavirus/diagnóstico , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/etiologia , Neurociências/tendências , Pneumonia Viral/complicações , Pneumonia Viral/diagnóstico , Adulto , Idoso , COVID-19 , Infecções por Coronavirus/terapia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/terapia , Pandemias , Pneumonia Viral/terapia , SARS-CoV-2RESUMO
BACKGROUND: Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant central nervous system neoplasm classified as an embryonal grade IV neoplasm by the World Health Organization. ATRT generally occurs in children younger than 3 years, with 85 pathologically confirmed cases reported in adults. It is most commonly supratentorial, with only 9 confirmed adult cases localized to the pineal region. CASE DESCRIPTION: The case is described of a 29-year-old man with a history of chronic migraines and a previously negative computed tomography scan of the head presenting with worsening headaches and new-onset diplopia with upward gaze palsy. Computed tomography and magnetic resonance imaging showed a hemorrhagic pineal mass with extension into the right thalamus. After resection, the immunohistochemical staining and cytogenetic profile proved consistent with ATRT, making it the ninth reported case of pineal ATRT in an adult, which was treated aggressively with good outcome. CONCLUSIONS: Adult ATRT is rare, especially in the pineal region, with only 9 cases reported. Because of the aggressiveness, ATRT must be considered in the differential diagnosis of pineal region lesions because early diagnosis and aggressive treatment are key to prolonged survival.
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Quimiorradioterapia/métodos , Procedimentos Neurocirúrgicos/métodos , Tumor Rabdoide/diagnóstico por imagem , Tumor Rabdoide/cirurgia , Teratoma/diagnóstico por imagem , Teratoma/cirurgia , Adulto , Humanos , Masculino , Tumor Rabdoide/tratamento farmacológico , Tumor Rabdoide/radioterapia , Teratoma/tratamento farmacológico , Teratoma/radioterapiaRESUMO
Background: Cholangiocarcinoma (CCC), a rare tumor arising from the viscera, has a poor prognosis. Although CCC is prone to metastasis, spread to the cranium and spine is exceedingly rare. Treatment for metastatic disease is palliative, with total resection of the primary lesion the only cure. We describe a case of metastatic CCC to the spine and cranium treated with surgical resection. Case Report: A 61-year-old male with a history of hepatitis C with liver transplant and incidental discovery of CCC presented with gradually increasing back pain. Physical examination revealed a palpable nontender mass in the parieto-occipital area. Computed tomography survey of the spine and head revealed mixed sclerotic and lytic lesions of the T9, T11, L2, and L5 vertebral bodies, a lytic lesion on the T6 vertebral body, and a 1.4-cm lesion in the right occipital calvarium. The patient underwent right occipital craniotomy for excisional biopsy of the calvarial mass with gross total resection and immunohistochemical confirmation of CCC. The patient was started on gemcitabine chemotherapy and radiation therapy for spinal metastases. Three months later, the patient died from metastatic disease complications. Conclusion: To our knowledge, only 6 cases of cranial CCC have been reported, and only 2 reported mixed cranial/spinal involvement. We report a rare case of CCC metastasis to the spine and cranium that was treated with surgery, chemotherapy, and radiotherapy. CCC should be considered an exceedingly rare etiology with treatment options aimed solely at palliation. This case supplements the existing literature to inform medical and surgical decision-making.
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Background: Pleomorphic xanthoastrocytoma (PXA) is a rare and often focal glioma that most commonly affects children and young adults. Lesions are preferentially supratentorial and superficial, although infratentorial masses have been described, along with occasional involvement of the leptomeninges. The World Health Organization (WHO) categorizes these tumors as grade II, with surgical resection carrying a favorable prognosis. However, these tumors may undergo malignant degeneration and must be identified for appropriate treatment and prognosis. PXA has been associated with neurofibromatosis type 1 (NF1), although it is not the classic phenotype of NF1. We present a novel report of PXA, atypically located in the pineal region of a patient with a history of NF1. Case Report: A 17-year-old male with a history of NF1 presented with 1 month of bifrontal headaches. Magnetic resonance imaging was significant for a heterogeneous tectal mass, suspicious for a glioma extending to the fourth ventricle and causing displacement of the cerebral aqueduct without obstructive hydrocephalus. Following an infratentorial-supracerebellar approach for tumor resection, histopathology confirmed a low-grade variable neoplasm consistent with PXA. Postoperative imaging confirmed gross total resection with no evidence of recurrence at 9 months postoperatively. Conclusion: To our knowledge, this case is the fifth report of pineal PXA and the first associated with NF1. Because PXA presents similarly to other NF1-related intracranial tumors, careful diagnosis via immunohistochemistry is imperative. Gross tumor resection is usually curative; however, PXA has the propensity to undergo malignant degeneration and may require adjuvant treatment.
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BACKGROUND: Chiari I malformation (CIM) is a disorder characterized by caudal displacement of the cerebellar tonsils below the foramen magnum. It is often associated with syringomyelia and occasionally with hydrocephalus. CIM is commonly treated by posterior fossa decompression with or without removal of the posterior arch of C1 and duraplasty, but the treatment for infants with symptomatic CIM is not well established. We present a case of symptomatic CIM in an infant that was successfully treated with a ventriculoperitoneal shunt (VPS) and discuss the importance of the pathophysiology in management decisions. CASE DESCRIPTION: A 6-month-old male with a CIM and a cervicothoracic syrinx presented with stridor, lower cranial nerve dysfunction, and increased tone that worsened with crying. Magnetic resonance imaging studies revealed cerebellar tonsillar displacement extending to the level of C3-C4 with a syrinx extending from C4 to T4. In addition, there was compression of the cervicomedullary junction, fourth ventricular outflow obstruction, and obstructive hydrocephalus. The decision was made to place a ventriculoperitoneal shunt (VPS) instead of performing decompressive surgery as the initial treatment intervention. The infant had significant symptomatic relief at 6-, 9-, and 12-month follow-ups. Postoperative magnetic resonance imaging at 6-month follow-up revealed resolution of the syrinx and ventriculomegaly and ascent of the cerebellar tonsils. CONCLUSIONS: Ventriculoperitoneal shunting alone was successfully used to treat an infant with concurrent CIM, syrinx, and hydrocephalus. This case underscores not only the importance of hydrocephalus as the pathogenesis of CIM in some cases but also the possibility of avoiding the morbidity of decompressive surgery in infants.
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Malformação de Arnold-Chiari/cirurgia , Hidrocefalia/cirurgia , Siringomielia/cirurgia , Derivação Ventriculoperitoneal/métodos , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico por imagem , Atlas Cervical/cirurgia , Vértebras Cervicais , Descompressão Cirúrgica , Humanos , Hidrocefalia/diagnóstico por imagem , Hidrocefalia/etiologia , Lactente , Laminectomia , Imageamento por Ressonância Magnética , Masculino , Procedimentos Neurocirúrgicos , Siringomielia/complicações , Siringomielia/diagnóstico por imagem , Vértebras Torácicas , Resultado do TratamentoRESUMO
Neurosurgery training in New Orleans began when Dr Dean Echols became the head of Neurosurgery for the Ochnser Clinic and Charity Hospital in 1944. The oldest in Louisiana, the Tulane-Ochsner program has trained 63 board-certified neurosurgeons since its founding, including 5 chairmen. The program has a colorful history linked to New Orleans. Its residents have enjoyed training at Charity Hospital, Tulane Hospital and Clinics, Veterans Affairs Medical Center, and the Ochsner Clinic Foundation.
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Centros Médicos Acadêmicos/história , Neurocirurgia/história , História do Século XIX , História do Século XX , Humanos , LouisianaRESUMO
OBJECTIVES: Deep brain stimulation (DBS) is the surgical treatment of choice for moderate to severe Parkinson's Disease (PD). However, few studies have assessed its efficacy in severe PD as defined by the modified Hoehn and Yahr scale (HY). This study evaluates long-term and medication outcomes of DBS in severe PD. PATIENTS AND METHODS: We retrospectively collected the data of 15 patients from 2008 to 2014 with severe PD treated with DBS. Retrospective assessment with the modified Hoehn and Yahr scale and motor subset of the Unified Parkinson's Disease Rating Scale (UPDRS III) were used to objectively track severity and motor function improvement, respectively. Levodopa equivalence daily doses (LEDD), number of anti-PD medications and number of daily medication doses were used to measure improvements in medication burden. Data was evaluated using univariate analyses, one sample paired t-test, two sample paired t-test, and Wilcoxon signed-rank test. RESULTS: The mean post-operative follow-up was 44.63 months, average age at diagnosis and the average age at time of DBS was 51.3 years and 61.5 years, respectively, and the time from diagnosis to treatment was 13.2 years. Significant decreases were seen in UPDRS III scores (pre-op = 44.533; post-op = 26.13; p = 0.0094), LEDD (pre-op = 1679.34 mg; post-op = 837.48 mg; p = 0.0049), and number of daily doses (pre-op = 21.266; post-op 12.2; p = 0.0046). No significant decrease was seen in the number of anti-PD medications (pre-op = 3.8; post-op = 3.2; p = 0.16). CONCLUSION: Following DBS, severe PD patients demonstrated significant improvements in motor function and medication burden during long-term follow-up. We believe our results prove that DBS is efficacious in the management of severe PD, and that further research should follow to expand DBS criteria to include severe disease.
Assuntos
Estimulação Encefálica Profunda , Doença de Parkinson/terapia , Adulto , Idade de Início , Idoso , Antiparkinsonianos/uso terapêutico , Feminino , Seguimentos , Humanos , Levodopa/administração & dosagem , Levodopa/uso terapêutico , Masculino , Testes de Estado Mental e Demência , Pessoa de Meia-Idade , Doença de Parkinson/tratamento farmacológico , Desempenho Psicomotor , Estudos Retrospectivos , Índice de Gravidade de Doença , Núcleo Subtalâmico , Resultado do TratamentoRESUMO
OBJECTIVE: Deep brain stimulation (DBS) is the procedure of choice for Parkinson's disease (PD). It has been used in PD patients younger than 70 years because of better perceived intra- and postoperative outcomes than in patients 70 years or older. However, previous studies with limited follow-up have demonstrated benefits associated with the treatment of elderly patients. This study aims to evaluate the long-term outcomes in elderly PD patients treated with DBS in comparison with a younger population. METHODS: PD patients treated with DBS at the authors' institution from 2008 to 2014 were divided into 2 groups: 1) elderly patients, defined as having an age at surgery ≥ 70 years, and 2) young patients, defined as those < 70 years at surgery. Functional and medical treatment outcomes were evaluated using the Unified Parkinson's Disease Rating Scale part III (UPDRS III), levodopa-equivalent daily dose (LEDD), number of daily doses, and number of anti-PD medications. Study outcomes were compared using univariate analyses, 1-sample paired t-tests, and 2-sample t-tests. RESULTS: A total of 151 patients were studied, of whom 24.5% were ≥ 70 years. The most common preoperative Hoehn and Yahr stages for both groups were 2 and 3. On average, elderly patients had more comorbidities at the time of surgery than their younger counterparts (1 vs 0, p = 0.0001) as well as a higher average LEDD (891 mg vs 665 mg, p = 0.008). Both groups experienced significant decreases in LEDD following surgery (elderly 331.38 mg, p = 0.0001; and young 108.6 mg, p = 0.0439), with a more significant decrease seen in elderly patients (young 108.6 mg vs elderly 331.38 mg, p = 0.0153). Elderly patients also experienced more significant reductions in daily doses (young 0.65 vs elderly 3.567, p = 0.0344). Both groups experienced significant improvements in motor function determined by reductions in UPDRS III scores (elderly 16.29 vs young 12.85, p < 0.0001); however, reductions in motor score between groups were not significant. Improvement in motor function was present for a mean follow-up of 3.383 years postsurgery for the young group and 3.51 years for the elderly group. The average follow-up was 40.6 months in the young group and 42.2 months in the elderly group. CONCLUSIONS: This study found long-term improvements in motor function and medication requirements in both elderly and young PD patients treated with DBS. These outcomes suggest that DBS can be successfully used in PD patients ≥ 70 years. Further studies will expand on these findings.