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1.
Pan Afr Med J ; 37: 59, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33209186

RESUMO

Chordoma is a rare malignant tumor of the spine. We report the case of a 26-year-old man who presented with facial paralysis and upper limbs paresthesia. Cerebral CT-scan and cerebro-spinal MRI revealed a 58mm locally advanced middle clival mass with deviation of median cerebral structures. Endoscopic biopsy concluded to a chondroid chordoma. Skeletal survey and thoraco-abdomino-pelvic CT-scan were normal. Treatment consisted in complete surgical removal of the tumor followed by adjuvant radiotherapy. The patient is alive free of disease with a follow up of 12 months.


Assuntos
Cordoma/diagnóstico por imagem , Fossa Craniana Posterior/diagnóstico por imagem , Neoplasias da Base do Crânio/diagnóstico por imagem , Adulto , Cordoma/patologia , Cordoma/terapia , Fossa Craniana Posterior/patologia , Seguimentos , Humanos , Imagem por Ressonância Magnética , Masculino , Radioterapia Adjuvante , Neoplasias da Base do Crânio/patologia , Neoplasias da Base do Crânio/terapia , Tomografia Computadorizada por Raios X
2.
World Neurosurg ; 141: 188-191, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32569764

RESUMO

BACKGROUND: An intracranial schwannoma originating in the dura mater is extremely rare. Herein, we report a case of schwannoma arising from the dura mater of the petrosal surface in the posterior cranial fossa. CASE DESCRIPTION: A 48-year-old man presented with slight gait disturbance and papilledema. Magnetic resonance imaging showed a T1- and T2-weighted mixed-intensity extra-axial tumor, approximately 4 cm in the maximum diameter with multiple small cysts, in the left posterior cranial fossa. The tumor was heterogeneously enhanced with gadolinium. Operative findings via a lateral suboccipital approach revealed that the tumor did not adhere to any cranial nerves but to the dura mater of the petrosal surface. The tumor was completely excised without any neurologic deficits, and the histologic diagnosis was schwannoma. CONCLUSIONS: The operative and postoperative findings suggested that the schwannoma originated in the meningeal branch of the lower cranial nerves or upper cervical nerves in the dura mater of the petrosal surface in the posterior cranial fossa.


Assuntos
Fossa Craniana Posterior/patologia , Dura-Máter/patologia , Neoplasias Meníngeas/patologia , Neurilemoma/patologia , Humanos , Masculino , Pessoa de Meia-Idade
3.
World Neurosurg ; 141: e989-e997, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32585377

RESUMO

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


Assuntos
Malformação de Arnold-Chiari/complicações , Fossa Craniana Posterior/crescimento & desenvolvimento , Fossa Craniana Posterior/patologia , Meningomielocele/cirurgia , Fossa Craniana Posterior/diagnóstico por imagem , Feminino , Humanos , Recém-Nascido , Imagem por Ressonância Magnética , Masculino
4.
World Neurosurg ; 138: 521-529.e2, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32156591

RESUMO

BACKGROUND: Chiari malformation (CM) is defined as a herniation of cerebellar tonsils below the foramen magnum and is considered to be due to underdevelopment of posterior fossa (PF) bone components. There is overcrowding of neural structures, and normal cerebrospinal fluid circulation is frequently blocked. Although several publications exist of measurements of dimensions and volumes from bone components of PF in CM, there is no systematic review evaluating quantitatively these dimensions. The aim of this study was to evaluate PF dimensions and volumes in a meta-analysis. METHODS: An electronic search using PubMed and Google Scholar was done. Study eligibility criteria were defined by the PICO strategy, which selected patients and interventions. Studies comparing PF dimensions between patients with CM and normal subjects were selected. A meta-analysis of pooled data was done using statistical software. RESULTS: Data for pooled analysis were obtained from 12 studies. Data synthesis was based on small published sample sizes in the majority of studies. Key findings included smaller measurements of clivus, supraoccipital bone, and PF area dimensions in patients with CM compared with normal subjects. Brainstem and cerebellar length was not different between groups. CONCLUSIONS: This review provides evidence of smaller measurements of clivus, supraoccipital bone, and PF area dimensions in patients with CM compared with normal subjects. An implication of the key findings is that surgical treatment of CMs should consider the smaller dimensions of PF in planning.


Assuntos
Malformação de Arnold-Chiari/patologia , Cefalometria , Fossa Craniana Posterior/patologia , Osso Occipital/patologia , Malformação de Arnold-Chiari/diagnóstico por imagem , Estudos de Casos e Controles , Fossa Craniana Posterior/anatomia & histologia , Fossa Craniana Posterior/diagnóstico por imagem , Humanos , Imagem por Ressonância Magnética , Osso Occipital/anatomia & histologia , Osso Occipital/diagnóstico por imagem , Tamanho do Órgão , Platibasia/diagnóstico por imagem , Platibasia/patologia , Valores de Referência , Tomografia Computadorizada por Raios X
6.
World Neurosurg ; 133: e259-e266, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31513955

RESUMO

OBJECTIVE: How Chiari malformation type I (CM-I) affects posterior fossa brain structures and produces various symptoms remains unclear. The fourth ventricle is surrounded by critical structures required for normal function. The foramen of Magendie can be obstructed in CM-I; therefore, fourth ventricle changes may occur. To test this hypothesis, we assessed fourth ventricle volume in CM-I compared with healthy controls. METHODS: Using our database from 2007-2016, we studied 72 patients with CM-I and 30 age-matched healthy control subjects. Fourth and lateral ventricle volumes and posterior fossa volumes (PFV) were assessed and correlated with clinical signs and symptoms. Statistical analysis was performed. RESULTS: Patients with CM-I had larger fourth ventricle volumes compared with control subjects (1.31 vs. 0.95 mL; P = 0.012). There were no differences in lateral ventricle volume or PFV. CM-I fourth ventricle volume was associated with tonsillar descent (P = 0.030). CM-I fourth ventricle volume variance was larger than healthy controls (F71,29 = 8.33; P < 0.0001). Patients with CM-I with severe signs and symptoms had a significantly larger fourth ventricle than patients with CM-I with mild signs and symptoms (1.565 vs. 1.015 mL; P = 0.0002). CONCLUSIONS: The fourth ventricle can be enlarged in CM-I independent of lateral ventricle size and is associated with greater tonsillar descent. Most importantly, fourth ventricle enlargement was associated with a worse clinical and radiographic presentation independent of PFV. Fourth ventricle enlargement can affect critical structures and may be a mechanism contributing to symptoms unexplained by tonsil descent. Fourth ventricle enlargement is a useful adjunct in assessing CM-I.


Assuntos
Malformação de Arnold-Chiari/patologia , Fossa Craniana Posterior/patologia , Quarto Ventrículo/patologia , Adolescente , Adulto , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico por imagem , Criança , Pré-Escolar , Fossa Craniana Posterior/diagnóstico por imagem , Dilatação Patológica/diagnóstico por imagem , Dilatação Patológica/patologia , Encefalocele/diagnóstico por imagem , Encefalocele/etiologia , Feminino , Quarto Ventrículo/diagnóstico por imagem , Humanos , Imageamento Tridimensional , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neuroimagem , Tamanho do Órgão , Estudos Prospectivos , Adulto Jovem
7.
Cancer Genet ; 240: 66-72, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31794935

RESUMO

Ependymomas are neuroepithelial tumors that differentiate along the ependymal cell lineage, a lining of the ventricles of the brain and the central canal of the spinal cord. They are rare in adults, but account for around 9% of brain tumors in children, where they usually have an aggressive course. Efficient stratification could lead to improved care but remains a challenge even in the genomic era. Recent studies proposed a multivariate classification system based on tumor location, age, and broad genomic findings like global patterns of methylation and copy number variants (CNVs). This system shows improved prognostic utility, but is relatively impractical in the routine clinical setting because it necessitates multiple diagnostic tests. We analyzed 13 intracranial grade II and III ependymoma specimens on a DNA microarray to identify discrete CNVs that could support the existing classification. The loss of chr22 and the gain of 5p15.31 were common throughout our cohort (6 and 11 cases, respectively). Other CNVs correlated well with the previously proposed classification system. For example, gains of chr20 were unique to PF-EPN-B tumors of the posterior fossa and may differentiate them from PF-EPN-A. Given the ease of detecting CNVs using multiple, clinically validated methods, these CNVs should be further studied to confirm their diagnostic and prognostic utility, for incorporation into clinical testing algorithms.


Assuntos
Biomarcadores Tumorais/genética , Variações do Número de Cópias de DNA , Ependimoma/diagnóstico , Neoplasias Infratentoriais/diagnóstico , Neoplasias Supratentoriais/diagnóstico , Adulto , Criança , Pré-Escolar , Cromossomos Humanos Par 20/genética , Cromossomos Humanos Par 22/genética , Cromossomos Humanos Par 5/genética , Fossa Craniana Posterior/patologia , Diagnóstico Diferencial , Ependimoma/genética , Ependimoma/mortalidade , Ependimoma/patologia , Estudos de Viabilidade , Feminino , Humanos , Lactente , Neoplasias Infratentoriais/genética , Neoplasias Infratentoriais/mortalidade , Neoplasias Infratentoriais/patologia , Masculino , Gradação de Tumores , Análise de Sequência com Séries de Oligonucleotídeos , Prognóstico , Intervalo Livre de Progressão , Estudos Retrospectivos , Neoplasias Supratentoriais/genética , Neoplasias Supratentoriais/mortalidade , Neoplasias Supratentoriais/patologia
8.
Neurocirugía (Soc. Luso-Esp. Neurocir.) ; 30(6): 305-308, nov.-dic. 2019. ilus
Artigo em Espanhol | IBECS | ID: ibc-186961

RESUMO

Presentamos el caso de una mujer de 52 años, sin antecedentes de interés, que fue derivada a nuestro centro tras ser diagnosticada de una lesión ocupante de espacio en el hemisferio cerebeloso derecho. La sospecha inicial era de metástasis. En la RMN cerebral, sin embargo, se apreciaba una marcada restricción en la secuencia de difusión concordante con un absceso cerebeloso. La paciente se había sometido a una limpieza dental tres semanas antes. El análisis microbiológico tras la evacuación quirúrgica de la lesión mostró la presencia de Streptococcus intermedius


A 52-year-old woman with no relevant previous medical history was diagnosticated of an infratentorial bulky cerebellar mass. The mass showed restricted diffusion on MR images, which was consistent with cerebellar abscess. The patient had undergone a minor dental procedure three weeks before. Microbiological analysis after surgical evacuation of the mass confirmed the presence of Streptococcus intermedius


Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Profilaxia Dentária/efeitos adversos , Streptococcus intermedius/patogenicidade , Fossa Craniana Posterior/diagnóstico por imagem , Abscesso/microbiologia , Fossa Craniana Posterior/patologia , Cérebro/diagnóstico por imagem , Cérebro/patologia , Craniotomia/métodos , Neuroimagem/métodos , Abscesso/tratamento farmacológico
9.
World Neurosurg ; 132: e124-e132, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31518744

RESUMO

BACKGROUND: Posterior fossa tumors are rare in adults and pose a challenge to treat due to the bony contour of the posterior fossa, complex anatomical structures including deep venous sinuses, and the proximity of the fourth ventricle and brain stem. We describe our experience with laser interstitial thermal therapy (LITT) for the management of brain metastases and radiation necrosis of the posterior fossa. METHODS: We retrospectively analyzed 13 patients with metastases and radiation necrosis of the posterior fossa managed with LITT. RESULTS: Thirteen patients with histopathologically confirmed radiation necrosis (n = 5) and metastases (n = 8) of the posterior fossa underwent LITT. The median preoperative tumor was 4.66 cm3, and median postoperative ablation cavity volume was 6.29 cm3. The median volume of the ablation cavity was decreased to 2.90 cm3 at a 9-month follow-up. The median volume of peritumoral edema was 12.25 cm3, which fell to a median of 5.77 cm3 at 1-month follow-up. The median progression-free survival was 7 months (range, 3-14 months) and the mean overall survival was 40 months (range, 2-49 months) after LITT. There were no intraoperative complications. One patient experienced palsy of the seventh and eighth cranial nerves on follow-up, attributable to LITT. CONCLUSIONS: Lesions of the posterior fossa are challenging to treat given their proximity to the dura and venous sinuses. Our findings demonstrate that LITT ablation may be a safe and feasible option for metastases and radiation necrosis of the posterior fossa. Larger studies are needed to confirm the efficacy of this approach.


Assuntos
Fossa Craniana Posterior/cirurgia , Hipertermia Induzida/métodos , Neoplasias Infratentoriais/terapia , Terapia a Laser/métodos , Adulto , Idoso , Edema Encefálico/diagnóstico por imagem , Neoplasias Encefálicas/patologia , Fossa Craniana Posterior/patologia , Feminino , Seguimentos , Humanos , Neoplasias Infratentoriais/secundário , Estimativa de Kaplan-Meier , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Necrose/diagnóstico por imagem , Necrose/etiologia , Complicações Pós-Operatórias/epidemiologia , Intervalo Livre de Progressão , Radiocirurgia , Estudos Retrospectivos , Análise de Sobrevida
10.
Neurochirurgie ; 65(5): 221-227, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31557489

RESUMO

BACKGROUND: The growth of the posterior fossa in syndromic craniostenosis was studied in many papers. However, few studies described the pathophysiological growth mechanisms in non-operated infants with fibroblast growth factor receptor (FGFR) type 2 mutation (Crouzon, Apert or Pfeiffer syndrome), although these are essential to understanding cranial vault expansion and hydrocephalus treatment in these syndromes. OBJECTIVE: A review of the medical literature was performed, to understand the physiological and pathological growth mechanisms of the posterior fossa in normal infants and infants with craniostenosis related to FGFR2 mutation. DISCUSSION: Of the various techniques for measuring posterior fossa volume, direct slice-by-slice contouring is the most precise and sensitive. Posterior fossa growth follows a bi-phasic pattern due to opening of the petro-occipital, occipitomastoidal and spheno-occipital sutures. Some studies reported smaller posterior fossae in syndromic craniostenosis, whereas direct contouring studies reported no difference between normal and craniostenotic patients. In Crouzon syndrome, synchondrosis fusion occurs earlier than in normal subjects, and follows a precise pattern. This premature fusion in Crouzon syndrome leads to a stenotic foramen magnum and facial retrusion.


Assuntos
Fossa Craniana Posterior/crescimento & desenvolvimento , Fossa Craniana Posterior/patologia , Craniossinostoses/genética , Craniossinostoses/patologia , Receptor Tipo 2 de Fator de Crescimento de Fibroblastos/genética , Criança , Pré-Escolar , Humanos , Lactente , Recém-Nascido , Mutação , Crânio/anormalidades , Síndrome
11.
Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi ; 33(9): 840-844;847, 2019 Sep.
Artigo em Chinês | MEDLINE | ID: mdl-31446700

RESUMO

Summary Chiari malformation type Ⅰ(CMI) is a disorder characterized by tonsilla cerebelli herniating into an underdeveloped posterior cranial fossa, hearing loss is often covered by more striking neurological symptoms. Hearing loss in this syndrome is not specific in terms of gender side, degree, age of onset, and progression. The hearing improvement after posterior fossa decompression is controversial on the basis of literature, while satisfactory result was obtained after cochlear implantation in the patient reported here, who was diagnosed as CMI with hearing loss as the main symptom. Therefore, after ensuring the integrity of the auditory pathway, cochlear implantation may be considered in CMI patients with bilateral severe or profound without other severe neurological symptoms.


Assuntos
Malformação de Arnold-Chiari/complicações , Implante Coclear , Surdez/etiologia , Surdez/cirurgia , Fossa Craniana Posterior/patologia , Audição , Humanos , Resultado do Tratamento
12.
J Craniofac Surg ; 30(8): e755-e757, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31449204

RESUMO

A 78-year-old woman with progressive worsening vertigo and new onset of diplopia. Neurological examination revealed bilateral abducens nerve paralysis and gait disturbance with truncal ataxia. Neuroimaging revealed a mass lesion within the clivus with brain stem compression. The patient was operated with provisional diagnosis of clivus chordoma by neuro-navigation-guided extended endoscopic endonasal approach. Subtotal resection of the tumor was achieved with no intra- or postoperative complications. Histopathologic examination revealed intraosseous meningioma (WHO grade I). To the authors' best knowledge this is the first case reporting clivus site of intraosseous meningioma.


Assuntos
Cordoma/cirurgia , Fossa Craniana Posterior/diagnóstico por imagem , Diagnóstico Diferencial , Neoplasias Meníngeas/diagnóstico , Meningioma/diagnóstico , Neoplasias da Base do Crânio/cirurgia , Idoso , Cordoma/complicações , Cordoma/diagnóstico , Fossa Craniana Posterior/patologia , Diplopia/complicações , Feminino , Neoplasias de Cabeça e Pescoço , Humanos , Neuroimagem , Complicações Pós-Operatórias , Neoplasias da Base do Crânio/complicações , Neoplasias da Base do Crânio/diagnóstico
13.
World Neurosurg ; 130: 512-515, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31279922

RESUMO

BACKGROUND: Hemangiomas are benign blood vessels tumors that represent less than 1% of all the bone neoplasms. Calvarial hemangiomas are mainly solitary lesions commonly located in the frontal and parietal bone; however, they may occur in any skull region. These tumors increase in size over a period of months to years before they start showing their first symptoms such as headache, bone deformity, and pathological fractures. Differential diagnosis with osteosarcoma should be considered. Surgical resection with a safety margin is a standard treatment of the cranial hemangioma. Furthermore, radiotherapy has proven to stop the tumor's growth but not its size. CASE DESCRIPTION: We treated an 11-year-old male who had a rare case of a capillary hemangioma located in the clivus bone. The patient underwent 2 endoscopic endonasal resection because of tumor recurrence. Surgical safety margins are highly recommended, but this procedure could not be performed because of the tumor's location. After the second relapse, the oncology team decided to initiate radiotherapy. At 6-month follow-up, the tumor reduced its size and remained unchanged. CONCLUSIONS: Surgical safety margins are highly important to prevent recurrence in this type of bone tumors. Skull base hemangiomas are a big challenge when you want to achieve these safety margins. We believe that a combined treatment of surgery and radiotherapy should be considered as the main treatment.


Assuntos
Fossa Craniana Posterior/diagnóstico por imagem , Fossa Craniana Posterior/patologia , Hemangioma/diagnóstico , Hemangioma/patologia , Neoplasias da Base do Crânio/diagnóstico , Neoplasias da Base do Crânio/patologia , Criança , Fossa Craniana Posterior/irrigação sanguínea , Diagnóstico Diferencial , Humanos , Masculino , Osteossarcoma/diagnóstico , Osteossarcoma/patologia , Neoplasias da Base do Crânio/irrigação sanguínea
14.
World Neurosurg ; 130: e620-e626, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31265927

RESUMO

OBJECTIVE: The aim of this study was to discuss the relationship between Ki-67 values and the degree to which chordoma invade the clivus and to certify that the prognosis of chordoma is worse when it invades the middle and lower clivus than when it does not. METHODS: We collected 56 cases of first-time chordoma illness in which patients received no treatment before surgery. Patients underwent craniocerebral magnetic resonance imaging and skull-base 3-dimensional computed tomography scans before the operation. We divided patients into 2 groups depending on the extent to which the middle and lower clivus were invaded. We classified patients with chordoma that did not significantly invade the middle and lower clivuses into a "noninvasive" group and the others into an "invasive" group. Ki-67 values were extracted from the pathological report after surgery. We use an independent χ2 test to indicate that Ki-67 values for the invasive group were higher than those for the noninvasive group. RESULTS: We grouped the data and did a statistical analysis. We found that the Ki-67 values are >5% for most patients in whom chordoma have eroded the middle-lower clivus, whereas it is ≤5% for patients in whom the middle-lower clivus region has not been invaded. Therefore, there is a correlation between Ki-67 value and the region of chordoma invading the clivus. CONCLUSIONS: Statistical analysis revealed that Ki-67 values when the chordoma invaded the middle and lower clivus were significantly higher than when it did not. Thus, we can conclude that the prognosis is worse when chordoma invade the middle and lower clivus.


Assuntos
Cordoma/patologia , Fossa Craniana Posterior/patologia , Invasividade Neoplásica/patologia , Neoplasias da Base do Crânio/patologia , Adolescente , Adulto , Idoso , Biomarcadores Tumorais/análise , Cordoma/diagnóstico por imagem , Fossa Craniana Posterior/diagnóstico por imagem , Feminino , Humanos , Antígeno Ki-67/análise , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Invasividade Neoplásica/diagnóstico por imagem , Prognóstico , Neoplasias da Base do Crânio/diagnóstico por imagem , Tomografia Computadorizada por Raios X , Adulto Jovem
15.
J Med Case Rep ; 13(1): 222, 2019 Jul 21.
Artigo em Inglês | MEDLINE | ID: mdl-31325957

RESUMO

BACKGROUND: Management of the disproportionately large communicating fourth ventricle is still problematic. CASE PRESENTATION: Two cases of disproportionately large communicating fourth ventricle were treated successfully. One was a case of a 51-year-old Han Chinese woman with a complaint of headache and dizziness of 1 year's duration. Magnetic resonance imaging (MRI) demonstrated hydrocephalus with a disproportionately large fourth ventricle. She underwent a ventriculo-peritoneal shunt of the right lateral ventricle. Her symptoms were relieved totally. Five years later, magnetic resonance imaging showed she had a normal ventricular system. The other case was a 24-year-old Han Chinese man with a 2-month history of headache and dizziness accompanied by progressive loss of bilateral vision. Magnetic resonance imaging revealed hydrocephalus with a disproportionately large fourth ventricle, crowded posterior cranial fossa, and syringomyelia extending from C1 to C5. He underwent suboccipital and C1 decompression and duraplasty. Shortly after the surgery, his symptoms were relieved completely, the syringomyelia completely disappeared, and the fourth ventricle became significantly smaller. CONCLUSIONS: The management of the disproportionately large communicating fourth ventricle should be individualized. If it coexists with crowded posterior cranial fossa or syringomyelia, posterior fossa decompression could be an option for initial management. If there is no sign of crowded posterior cranial fossa or syringomyelia, shunt of the lateral ventricles might be the first choice.


Assuntos
Quarto Ventrículo/patologia , Hidrocefalia/etiologia , Fossa Craniana Posterior/diagnóstico por imagem , Fossa Craniana Posterior/patologia , Tontura/etiologia , Feminino , Quarto Ventrículo/diagnóstico por imagem , Quarto Ventrículo/cirurgia , Cefaleia/etiologia , Humanos , Hidrocefalia/diagnóstico por imagem , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Siringomielia/complicações , Siringomielia/diagnóstico por imagem , Siringomielia/patologia , Resultado do Tratamento , Derivação Ventriculoperitoneal , Adulto Jovem
16.
J Pediatr Endocrinol Metab ; 32(8): 797-802, 2019 08 27.
Artigo em Inglês | MEDLINE | ID: mdl-31323007

RESUMO

PHACE syndrome is an uncommon disorder of posterior fossa anomalies, cervicofacial infantile hemangiomas, arterial anomalies, cardiac defects, eye anomalies, and midline/ventral defects. Endocrine abnormalities including hypopituitarism and ectopic thyroid were rarely described. In this article we review occurrence, onset, presenting symptoms, hormonal treatments and outcomes of all endocrine abnormalities in PHACE syndrome. Eleven of 20 (55%) had hypothalamic-pituitary dysfunction and 10 of 20 (50%) had thyroid dysgenesis. A thorough understanding of the endocrine manifestations is important for clinicians to early identify endocrine involvement in PHACE and develop plans for monitoring and treatment of its complications.


Assuntos
Anormalidades Múltiplas/etiologia , Coartação Aórtica/etiologia , Doenças do Sistema Endócrino/complicações , Anormalidades do Olho/etiologia , Síndromes Neurocutâneas/etiologia , Anormalidades Múltiplas/patologia , Coartação Aórtica/patologia , Fossa Craniana Posterior/patologia , Anormalidades do Olho/patologia , Neoplasias Faciais/etiologia , Neoplasias Faciais/patologia , Cardiopatias Congênitas/etiologia , Cardiopatias Congênitas/patologia , Hemangioma/etiologia , Hemangioma/patologia , Humanos , Síndromes Neurocutâneas/patologia , Síndrome
17.
Medicine (Baltimore) ; 98(23): e15980, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31169734

RESUMO

Owing to the special growth pattern of chordomas and the limited treatment options currently available, the treatment of chordoma still remains difficult. In this study, we hope to further clarify the relationship between surgical treatment and radiotherapy of chordoma and disease progression.All patients with a primary histopathological diagnosis of clival or spinal chordomas recorded in our institution between 1976 and 2017 were examined.A total of 60 patients (location: skull base/clival, n = 24; vertebral column, n = 5; sacrum, n = 31) had a mean follow-up time of 7.7 years (range 12 months-35 years). Compared with patients who received subtotal resection (n = 5, 5-year and 10-year survival = 61% and 39%, respectively), the annual survival rate of patients who received total resection (n = 55, 5-year and 10-year survival = 67%, respectively) was significantly higher. The overall 10-year survival rate (58%) of patients treated with surgery alone was significantly different from those treated with a combination of surgery and radiation (73%). The long-term prognosis of sacral chordoma was the worst (10-year survival rate = 48%).The best treatment strategy for improved long-term survival in chordoma was a combination of surgical resection and radiation therapy. Adjuvant radiotherapy for chordoma significantly improves disease-free survival, although the long-term survival benefit remains to be determined. A worse prognosis and poor long-term survival are seen in sacral chordomas.


Assuntos
Cordoma/mortalidade , Neoplasias da Base do Crânio/mortalidade , Neoplasias da Coluna Vertebral/mortalidade , Adulto , Cordoma/terapia , Fossa Craniana Posterior/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prognóstico , Radioterapia Adjuvante , Sacro/patologia , Base do Crânio/patologia , Neoplasias da Base do Crânio/terapia , Neoplasias da Coluna Vertebral/terapia , Taxa de Sobrevida , Resultado do Tratamento
18.
Medicine (Baltimore) ; 98(19): e15533, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-31083202

RESUMO

Posterior fossa decompression (PFD) is the standard procedure for the treatment of Chiari malformation type I (CMI). Although most patients have satisfactory surgical outcomes, some show no improvement or even a worsening of symptoms. Patient selection is thought to account for these different surgical outcomes. Our aim was to evaluate the predictive value of the preoperative posterior cranial fossa (PCF) morphology on the efficacy of PFD.Data from 39 CMI patients with CMI-related symptoms who underwent occipital foramen enlargement + C-1 laminectomy + enlarged duraplasty were retrospectively collected from January 2011 to May 2018. The patients were divided into improved and unimproved groups according to the modified Chicago Chiari Outcome Scale. Demographic information and clinical history, including preoperative comorbidities and clinical manifestations, were recorded for the 2 groups and compared. PCF morphology was assessed based on 13 linear, 8 angular, 4 areal parameters and 4 ratios related to these liner and areal parameters. The data were then analyzed statistically.Of the 39 patients with CMI, 24 showed improvement after PFD (61.5%), whereas the remaining 15 patients showed no improvement (38.5%). The preoperative symptoms lasted 1 to 240 months, with a median of 24 months. The follow-up period ranged from 2 to 82 months, with a median of 27 months. The improved and unimproved groups differed significantly with regard to upper limb numbness (OR = 10, P = .02) and upper limb weakness (OR = 4.86, P = .02). The 2 groups did not differ significantly with regard to any morphological parameters such as tonsillar descent, syrinx size.Preoperative upper limb numbness and upper limb weakness are unfavorable factors that influence the outcome of PFD in patients with CMI. However, the morphology of PCF cannot predict the response to PFD in patients with CMI.


Assuntos
Malformação de Arnold-Chiari/cirurgia , Fossa Craniana Posterior/patologia , Adulto , Idoso , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/patologia , Fossa Craniana Posterior/diagnóstico por imagem , Feminino , Humanos , Imagem por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Valor Preditivo dos Testes , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
19.
Neuroradiology ; 61(9): 1011-1022, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31119343

RESUMO

PURPOSE: While 84% of patients surgically treated for Chiari malformation type 1 (CM1) demonstrate improved quality of life after posterior fossa decompression surgery, there are many risks associated with this surgery. Surgical planning to identify candidates likely to improve postoperatively may benefit from an improved understanding of morphological changes after decompression surgery. To evaluate these changes, we quantified 59 morphological parameters on 42 CM1 adult female patients before and after CM1 decompression surgery. METHODS: Fifty-nine morphological parameters in the posterior cranial fossa, cranio-cervical, and intracranial regions in the midsagittal plane were evaluated using 42 T1-weighted magnetic resonance images of female CM1 patients before and after surgery, and 42 healthy female controls. Morphological differences before and after surgery were compared through the development of a technique to establish the opisthion location, a key reference point not present after surgery. RESULTS: In addition to the expected reduction of the cranio-caudal dimension of the cerebellum, objective analyses showed a significant increase in the area of the cerebrospinal fluid spaces, posterior (6×) and inferior (2.6×) to the cerebellum (+ 112 ± 102 and + 140 ± 127 mm2, respectively). This increased area was primarily impacted by an average reduction in the occipital bone length of 24.5 ± 7.3 mm following surgery. Based on multiple angles, results demonstrated a 2°-4° anterior rotation of the cerebellum after surgery. CONCLUSION: Our results show that decompression surgery results in significant changes in the cerebellum and cerebrospinal fluid spaces. Further investigation should determine how these morphological changes impact clinical outcomes.


Assuntos
Malformação de Arnold-Chiari/cirurgia , Fossa Craniana Posterior/cirurgia , Descompressão Cirúrgica , Adulto , Malformação de Arnold-Chiari/diagnóstico por imagem , Malformação de Arnold-Chiari/patologia , Estudos de Casos e Controles , Fossa Craniana Posterior/diagnóstico por imagem , Fossa Craniana Posterior/patologia , Feminino , Humanos , Imagem por Ressonância Magnética , Resultado do Tratamento
20.
World Neurosurg ; 129: e171-e176, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31121374

RESUMO

BACKGROUND: Cerebrospinal fluid (CSF) leak remains a significant complication of posterior fossa tumor surgery. The goal of this study was to evaluate the wound CSF leakage rate in pediatric patients who underwent fourth ventricle tumor resection in a single-site setting and to explore the association of CSF leakage with the length of stay in the intensive care unit (ICU) and with dural sealant application. METHODS: Two hundred and eleven patients who underwent fourth ventricle tumor surgery between 2008 and 2016 were included in this study. Patient data were evaluated retrospectively. RESULTS: Postoperative wound CSF leakage was observed in 6 patients (2.8%). One hundred and seventy-six patients (83.4%) stayed in the ICU for ≤1 day, and CSF leakage developed in 4 of these patients (2.3%). Thirty-five patients (16.6%) stayed in the ICU for >1 day, and CSF leakage was observed in 2 of these patients (5.7%). The observed difference was not statistically significant (P = 0.260). There were no statistically significant differences in the CSF leakage rates in groups with and without dural sealing before (n = 2 [3%] and n = 4 [2.8%], respectively; P = 0.99) and after (n = 2 [3.0%] and n = 4 [6.0%], respectively; P = 0.68) application of the propensity score matching algorithm. CONCLUSIONS: The low CSF leakage rate (2.8%) identified in this study was associated with a certain algorithm of patient management. There was no strong evidence that the CSF leakage rate was associated with the length of stay in the ICU or with dural sealant application.


Assuntos
Neoplasias do Ventrículo Cerebral/cirurgia , Vazamento de Líquido Cefalorraquidiano/etiologia , Fossa Craniana Posterior/cirurgia , Quarto Ventrículo/cirurgia , Glioma/cirurgia , Neoplasias Infratentoriais/cirurgia , Procedimentos Neurocirúrgicos/efeitos adversos , Adolescente , Neoplasias do Ventrículo Cerebral/patologia , Criança , Pré-Escolar , Fossa Craniana Posterior/patologia , Craniotomia/efeitos adversos , Feminino , Quarto Ventrículo/patologia , Glioma/patologia , Humanos , Lactente , Recém-Nascido , Neoplasias Infratentoriais/patologia , Masculino , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos
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