RESUMEN

PURPOSE: Neurocysticercosis (NCC) is considered a neglected infectious disease, but the most common parasitic disease of the central nervous system (CNS). Due to oral tendencies in childhood, it is hypothesized that individuals are infected around this age and develop symptoms as lately as during young adulthood. Although it is considered a benign disease, it may cause great impact in the patient's quality of life due to epilepsy, visual symptoms, and hydrocephalus, which eventually requires frequent hospitalizations. The treatment of hydrocephalus is the main challenge for neurosurgeons. METHODS: We performed a concise review on neurocysticercosis in children and the main presentations of NCC in the neurosurgery practice and a systematic review on hydrocephalus secondary to extraparenchymal NCC. RESULTS: Our review showed a rate of complete resolution of hydrocephalus secondary to NCC of around 80% with the first attempt of surgical treatment combined with medication therapy. Endoscopic removal of the intraventricular cysts with third ventriculostomy was the most common treatment modality. Patients previously managed with ventricular shunts are likely to have worse outcomes and complications. CONCLUSION: Endoscopic approach is the gold standard surgical treatment for hydrocephalus secondary to neurocysticercosis.

Asunto(s)

Hidrocefalia , Neurocisticercosis , Neurocirugia , Humanos , Niño , Adulto Joven , Adulto , Neurocisticercosis/complicaciones , Neurocisticercosis/cirugía , Neurocisticercosis/diagnóstico , Calidad de Vida , Ventriculostomía/efectos adversos , Hidrocefalia/etiología , Hidrocefalia/cirugía , Hidrocefalia/diagnóstico

RESUMEN

BACKGROUND: Craniosynostosis are cranial deformities resulting from the early closure of 1 or more sutures. Concomitant facial changes are complex and usually result from the involvement of multiple sutures, which may lead to restriction of cranial growth and brain expansion, ocular compression, and breathing difficulties. Surgical techniques to correct syndromic craniosynostosis have improved over time, considerably reducing the rate of complications of this procedure. OBJECTIVE: To describe in detail (step-by-step) and with pertinent anatomic considerations the technique of monobloc frontofacial advancement using internal distractors. METHODS: We describe the monobloc frontofacial advancement technique with the use of internal distractors, which we use in patients with primary syndromic craniosynostosis (Apert, Crouzon, and Pfeiffer) who have major facial hypoplasia and secondary respiratory repercussions. To illustrate this technique, the procedure was performed in 2 cranial models: an adult artificial acrylic skull of normal morphology for better evidence of anatomic repairs and a 3-dimensional printed infant skull from a tomography file obtained from a child diagnosed with Apert syndrome. RESULTS: The benefits of osteogenic distraction and better surgical timing for each procedure are presented. We presented the changes and details of osteotomies performed during the procedure, as well as anatomic details and care regarding the pterygomaxillary dysjunction. CONCLUSION: Monobloc frontofacial distraction is a procedure with widely demonstrated aesthetic and functional results, and this detailed step-by-step description may improve familiarity with the anatomic landmarks of the procedure and provide a better dynamic understanding of the distraction process.

Asunto(s)

Acrocefalosindactilia , Disostosis Craneofacial , Craneosinostosis , Acrocefalosindactilia/diagnóstico por imagen , Acrocefalosindactilia/cirugía , Adulto , Niño , Disostosis Craneofacial/diagnóstico por imagen , Disostosis Craneofacial/cirugía , Craneosinostosis/diagnóstico por imagen , Craneosinostosis/cirugía , Huesos Faciales/cirugía , Humanos , Lactante , Osteotomía/métodos

RESUMEN

PURPOSE: Myelomeningocele (MMC) is an open neural tube defect that causes great morbidity. Prenatal open repair is the standard treatment; however, there are many complications related to the procedure. This study reports preliminary findings of open in utero repair of MMC in a public tertiary hospital in Brazil and describes factors that could be associated with increased surgical morbidity. METHODS: Thirty-nine patients underwent open in utero repair of MMC from October 2015 to August 2019. The Clavien-Dindo classification of surgical complications and a classification system with the preterm definitions of the World Health Organization were used, respectively, for maternal and fetal complications. RESULTS: A total of 28 mothers (71.8%) and 31 fetuses (79.5%) experienced at least one minor to major complication. Three mothers (7.7%) had a severe grade 4 complication. Fetal complications grades 3 to 5 occurred in 13 fetuses (33.3%). Gestational age at surgery and at birth were 24.88 ± 1.16 weeks and 33.23 ± 3.68 weeks, respectively. Preterm delivery occurred in 30 patients (76.9%), membrane rupture in 18 patients (46.2%) and chorioamnionitis in 13 patients (33.3%). CONCLUSION: Open fetal surgery for MMC was performed at a Brazilian public tertiary care center, resulting in three grade 4 maternal complications. Relevant fetal complications were also present. The use of a standard classification system for complications renders studies more comparable and data more useful for counseling patients. Adjustments of perioperative procedures and long-term follow-up are needed to determine the real benefit of open in utero repair of MMC at our hospital.

Asunto(s)

Feto/cirugía , Hidrocefalia/cirugía , Meningomielocele/cirugía , Defectos del Tubo Neural/cirugía , Disrafia Espinal/cirugía , Estudios Transversales , Femenino , Hospitales , Humanos , Recién Nacido , Embarazo , Resultado del Embarazo , Atención Prenatal , Resultado del Tratamiento

RESUMEN

PURPOSE: Studies have examined the impact of fusion of the sagittal suture in the skull base while others have evaluated the growth of the skull base before and after surgery. This study aims to perform the anthropometric measures of the skull base in children with scaphocephaly to evaluate the influence of surgical repair in the remodeling of the skull base and anthropometric measures. METHODS: Twenty-one children with diagnosis of scaphocephaly were operated between April 2007 and October 2008, and anthropometric measures at the skull base were performed before and after a year of surgery. The measures were the cranial index (CI), distance between the crista galli and tuberculum sellar (CG-TS), distance between the crista galli and the internal auditory meatus (CG-IAM), distance between the oval foramen (OF-OF), distance between the internal auditory meatus (IAM-IAM), the angle of the skull base (Â1), and the angle between the nasion, center of sella, and basion (Â2). RESULTS: There was a normalization of the CI in all children, confirming an appropriate cranial remodeling. The CG-TS measure evaluated the anterior skull base, with proportional growth of 12.5%. The mediolateral growth was observed by the increase of OF-OF measures by 8.5% and IAM-IAM by 9.5%. The CG-TS measure grew by 7.2%. There was no statistical difference in the angles analyzed. CONCLUSION: Surgical treatment of scaphocephaly led to remodeling of the skull base, confirmed by the changes of anthropometric measures taken before and after a year of surgery.

Asunto(s)

Craneosinostosis , Niño , Suturas Craneales/cirugía , Craneosinostosis/cirugía , Hueso Etmoides , Huesos Faciales , Humanos , Lactante , Cráneo , Base del Cráneo/cirugía

RESUMEN

BACKGROUND AND AIM: Cleidocranial dysplasia is a rare disorder of skeletal development that mainly promotes, among other malformations, inadequate development of clavicles and failure in cranial closure. In this affection, the role of neurosurgery in addressing cranial defects is rarely discussed. MATERIAL AND METHODS: We conducted an extensive review of the literature using the PubMed database, giving a greater focus to publications in the field of neurosurgery. Additionally, we report a case of a 2-year-old female child with cleidocranial dysplasia. RESULTS: In our review, we encountered several cases of orthodontic implications but a few cases on cranial defect approach. CONCLUSION: The articles present literature that is unanimous on the recommendation of expectant conduct in children since the cranial block can occur spontaneously, even if the delayed form. In our approach, we opted for an expected strategy concerning the cranial defect, using a helmet made for brain protection. We also made the referral for multidisciplinary monitoring of pediatrics, neuropediatrics, ophthalmology, dentistry, and orthopedics.

Asunto(s)

Displasia Cleidocraneal , Preescolar , Displasia Cleidocraneal/diagnóstico por imagen , Femenino , Dispositivos de Protección de la Cabeza , Humanos , Cráneo

RESUMEN

PURPOSE: Brainstem lesions comprise 10-20% of all pediatric brain tumors. Over the past years, the benefits of stereotactic biopsy versus the use alone of MRI features to guide treatment have been controversial. METHODS: Retrospective study with pediatric patients submitted to stereotactic brainstem biopsies between 2008 and 2018. Demographic, clinical, imaging, and surgical characteristics were recorded, as well as the histological diagnosis, complications, and survival. Predictors of survival were evaluated through Cox regression models after multivariate adjustment. RESULTS: Twenty-six patients (mean age of 8.8 ± 4.3 years and 14 female). Diagnosis was reached on 84.6% (95% CI 65.1-95.6%) of the patients. Glioma was diagnosed on 20 cases (11 high-grade and 9 low-grade lesions). There was no association between age and gender and the dichotomized histological diagnosis. Contrast enhancement, diffuse distribution, invasion of adjacent structures, and remote injury were present on 62.5%, 75.0%, 62.5%, and 25.0% of the cases. Hydrocephalus at admission was present on almost half of the patients (46.2%). Only radiological invasion of adjacent structures had a possible association with high-grade lesions (p = 0.057). Surgical trajectory was trans-cerebellar in most of the cases (79.9%). There were no major complications and only two minor/transitory complications. Poorer survival was independently associated with high-grade lesions (HR 32.14, 95% CI 1.40-735.98, p = 0.030) and contrast enhancement at MRI (HR 36.54, 95% CI 1.40-952.26, p = 0.031). CONCLUSIONS: Stereotactic biopsy was safe and allows successful tissue sampling for a definite diagnosis. Poorer survival was independently associated with high-grade and contrast-enhancing lesions.

Asunto(s)

Neoplasias Encefálicas , Neoplasias del Tronco Encefálico , Glioma , Adolescente , Biopsia , Neoplasias Encefálicas/diagnóstico por imagen , Neoplasias del Tronco Encefálico/diagnóstico por imagen , Neoplasias del Tronco Encefálico/cirugía , Niño , Preescolar , Femenino , Glioma/diagnóstico por imagen , Glioma/cirugía , Humanos , Imagen por Resonancia Magnética , Estudios Retrospectivos , Técnicas Estereotáxicas

RESUMEN

BACKGROUND: Myelomeningocele (MMC) is often related to hydrocephalus and Chiari malformation (CM) type 2; however, other brain abnormalities have been reported in this population. In order to better understand and quantify other forebrain abnormalities, we analyzed magnetic resonance imaging (MRI) of MMC patients treated in utero or postnatal. METHODS: Between January 2014 and March 2017, 59 MMC were treated in our hospital. Thirty-seven patients (32 postnatal and 5 intrautero repair) had brain MRI and were enrolled at the study. MRI was analyzed by two experienced neuroradiologists to identify the supra and infratentorial brain abnormalities. RESULTS: A wide range of brain abnormalities was consistently identified in MMC patients. As expected, the most common were hydrocephalus (94.5%) and CM type II (89.1%). Of note, we found high incidence of corpus callosum abnormalities (86.4%), mostly represented by dysplasia (46%). CONCLUSIONS: The data are consistent with the concept that brain abnormalities related to MMC can be both infratentorial and supratentorial, cortical, and subcortical. More studies are needed to correlate these forebrain abnormalities to long-term functional outcome and their prognostic value for these patients.

Asunto(s)

Malformación de Arnold-Chiari , Hidrocefalia , Meningomielocele , Malformación de Arnold-Chiari/complicaciones , Malformación de Arnold-Chiari/diagnóstico por imagen , Encéfalo/diagnóstico por imagen , Humanos , Hidrocefalia/diagnóstico por imagen , Hidrocefalia/etiología , Meningomielocele/complicaciones , Meningomielocele/diagnóstico por imagen , Estudios Retrospectivos

RESUMEN

BACKGROUND: Caudal duplication syndrome (CDS) is associated with complete/partial duplication of the spine, spinal cord, duplication/malformations of other caudal structures, and often neurological dysfunction. CASE DESCRIPTION: A 9-month-old with the prenatal diagnosis of multiple fetal malformations was born through cesarean section at 39 weeks. Computed tomography and magnetic resonance imaging revealed a series of abnormalities: intramedullary cyst at the L4 level with tethering of the conus with a lipoma, a duplication of the sacrum, duplication of the intestines, a ventricular septal defect, bladder duplication, four kidneys, two fully formed functional penises, two anuses (imperforate on the left), and a paramedian ossified appendage suggestive of a malformed leg. The child, at an 8 months of age, underwent a laminectomy at the L4 level for resection of the conus lipoma, release of the tethered cord, and resection of the intramedullary cyst. CONCLUSION: Spine/spinal cord duplication syndromes are typically associated with spine/spinal cord abnormalities and are often associated with moderate/severe neurological deficits. Additional malformations associated with the CDS include duplication of vascular structures/organs (e.g., bladder and distal gastrointestinal tract). Here, we focused on both the patient's quality of life and the multidisciplinary approach to managing the duplication syndrome associated with multiple system malformations (e.g., neurological/spinal, bladder, and gastrointestinal, others).

RESUMEN

OBJECTIVE: To study the prevalence of OTX1 and OTX2 gene expression in 60 medulloblastoma specimen samples and to establish correlations between gene expression and clinical and histopathological aspects. METHODS: We performed a retrospective analysis of 60 patients with a diagnosis of medulloblastoma at the Clinicas Hospital of the School of Medicine, University of São Paulo, and the Cancer Hospital of Barretos. We created a database of the 60 patients containing information on the gene expression of OTX1 and OTX2 (obtained using real-time polymerase chain reaction) and clinical and epidemiological data. Statistical tests were performed to verify potential correlations of clinicopathological data and follow-up aspects with gene expression. RESULTS: The OTX1 gene was expressed in 52% of the study population. Expression varied with age (higher in adults), location (predominantly by hemisphere), and histological type (desmoplastic). The OTX2 gene was expressed in 62% of the study population. Expression varied with age (higher in younger age groups), location (predominantly vermis), and histological type (classic and anaplastic). A statistical correlation between OTX2 gene expression and the development of leptomeningeal metastases was observed. CONCLUSIONS: The relative expression of OTX1 and OTX2 was dependent on patient age, tumor location, and histological variant. In addition, OTX2 expression might be a predictive factor for leptomeningeal metastases of medulloblastoma. The OTX pathway should be consider as an important venue for medulloblastomas development.

Asunto(s)

Neoplasias Cerebelosas/genética , Meduloblastoma/genética , Proteínas de Neoplasias/genética , Factores de Transcripción Otx/genética , Adolescente , Adulto , Factores de Edad , Brasil , Neoplasias Cerebelosas/epidemiología , Neoplasias Cerebelosas/terapia , Niño , Preescolar , Femenino , Regulación Neoplásica de la Expresión Génica , Humanos , Lactante , Estimación de Kaplan-Meier , Masculino , Meduloblastoma/epidemiología , Meduloblastoma/secundario , Meduloblastoma/terapia , Neoplasias Meníngeas/secundario , Persona de Mediana Edad , Reacción en Cadena en Tiempo Real de la Polimerasa , Estudios Retrospectivos , Adulto Joven

RESUMEN

BACKGROUND: Apert syndrome is a rare form of syndromic craniosynostosis, also known as acrocephalosyndactyly, which is a disorder characterized by a unique set of craniofacial, hand, and foot abnormalities. Diagnosis is made through a genetic analysis, where the mutation of FGFR2, Ser252Trp, and Pro253Arg confirms the diagnosis. CASE PRESENTATION: Although craniosynostosis is the most common characteristic in clinical presentation, we present an atypical case of a one-and-a-half-year-old girl with Apert syndrome confirmed by genetic testing but without craniosynostosis.

Asunto(s)

Acrocefalosindactilia/patología , Craneosinostosis , Femenino , Humanos , Lactante

RESUMEN

The ventriculoperitoneal shunt (VPS) is an established treatment for hydrocephalus. The functioning of the system requires a pressure difference between the cranial and abdominal cavities. The VPS can be particularly problematic in patients with increased intra-abdominal pressure (IAP). We report the case of a 16-year-old girl with VPS since she was 2 months old due to hydrocephalus secondary to myelomeningocele. The patient had been asymptomatic ever since, but she sought the emergency service with intermittent headache and vomiting. A non-enhanced brain tomography, a shunt trajectory X-ray and an abdominal ultrasound revealed no cause of system malfunction. In view of the persistent clinical picture, a revision of the shunt was performed, which revealed adequate intraoperative functioning. She returned with the same symptoms two weeks after surgery. The patient was obese (body mass index [BMI]: 48). We hypothesized intermittent valve malfunction due to increased intra-abdominal pressure. She underwent a ventriculoatrial shunt, without intercurrences. In the postoperative period, the patient presented transient tachycardia and was asymptomatic at the 6-month follow-up. Obesity should be considered an important variable for the inadequate functioning of the VPS due to increased IAP and catheter dystocia to the extraperitoneal cavity. Studies have already correlated the IAP with the BMI, which reaches between 8 mm Hg and 12 mm Hg in obese individuals. Therefore, the BMI can be considered during the selection of valve pressure in systems with non-adjustable valves to prevent insufficient drainage. The recognition of obesity as a cause of VPS malfunction is fundamental to avoid unnecessary surgeries and intermittent malfunction of the system.

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A derivação ventriculoperitoneal (DVP) é um tratamento estabelecido para a hidrocefalia; contudo, algumas variáveis podem influenciar na eficácia desta modalidade. O funcionamento do sistema requer uma diferença de pressão entre as cavidades craniana e abdominal. A DVP pode ser particularmente problemática em pacientes com aumento da pressão intra-abdominal (PIA). Neste artigo, relatamos o caso de uma paciente do sexo feminino, de 16 anos, portadora de DVP desde os 2 meses de idade por hidrocefalia secundária a mielomeningocele. Desde então assintomática, procurou o pronto-socorro com queixa de cefaleia e vômitos intermitentes. Uma tomografia de crânio sem contraste, um raio X (RX) do trajeto do cateter distal, e uma ultrassonografia (USG) abdominal não evidenciaram a causa do mau funcionamento do sistema. Diante do quadro persistente, realizou-se uma revisão da derivação, que mostrou funcionamento adequado no período intraoperatório. A paciente retornou com os mesmos sintomas duas semanas após a cirurgia. A paciente era obesa (índice de massa corporal [IMC]: 48). Aventou-se possível funcionamento intermitente da válvula pelo aumento da PIA. A paciente foi submetida a uma derivação ventrículo-atrial, que foi realizada sem intercorrências. No pós-operatório, ela apresentou quadro transitório de taquicardia, e não apresentou sintomas no acompanhamento feito depois de 6 meses. A obesidade deve ser considerada uma variável importante para o funcionamento inadequado da DVP, pelo aumento da PIA e pela associação com distocia do cateter para a cavidade extraperitoneal. Estudos já correlacionaram a PIA com o IMC, que pode atingir entre8 mm Hg e 12 mm Hg em obesos. Logo, o IMC pode ser considerado na seleção da pressão da válvula em sistemas com válvulas não ajustáveis, para prevenir a drenagem insuficiente. O reconhecimento da obesidade de risco para o mau funcionamento da DVP é fundamental para evitar cirurgias desnecessárias e o mau funcionamento intermitente do sistema.

Asunto(s)

Humanos , Femenino , Adolescente , Derivación Ventriculoperitoneal , Obesidad Infantil , Hidrocefalia , Obesidad/complicaciones

RESUMEN

BACKGROUND: Carotid-cavernous fistula (CCF) is a shunt between the carotid artery and the cavernous sinus. Traumatic CCFs are diagnosed in 0.2% of head traumas being only 4.6% of the pediatric population. Classified by Barrow in 1985, type A CCF is the most frequent, occurring in 75% of cases. Type A is characterized by direct and high-flow CCF that generally can occur as a result of traumatic injury or rupture of an intracavernous aneurysm. CASE PRESENTATION: The subject was an 8-year-old boy with penetrating trauma to his left eye. During the initial evaluation, a computed tomography (CT) scan was unremarkable, and after relief of symptoms, the patient was discharged. Seven days later, he developed grade I proptosis, conjunctival chemosis, ophthalmoplegia (III, IV, and VI cranial nerve palsies), and left-sided ptosis and mydriasis. Arteriography confirmed a post-traumatic CCF, and the patient was treated with an endovascular detachable balloon. CONCLUSION: CCF should be suspected in craniofacial traumas with ocular symptoms. The presence of a skull base fracture on CT is a poor predictor of CCF associated with head trauma. Early diagnosis and treatment can prevent permanent neurological deficits and unfavorable outcomes.

Asunto(s)

Fístula del Seno Cavernoso de la Carótida/cirugía , Traumatismos Craneocerebrales/cirugía , Lesiones Oculares/cirugía , Heridas Penetrantes/cirugía , Fístula del Seno Cavernoso de la Carótida/diagnóstico por imagen , Fístula del Seno Cavernoso de la Carótida/etiología , Niño , Traumatismos Craneocerebrales/complicaciones , Traumatismos Craneocerebrales/diagnóstico por imagen , Procedimientos Endovasculares/métodos , Lesiones Oculares/complicaciones , Lesiones Oculares/diagnóstico por imagen , Humanos , Masculino , Heridas Penetrantes/complicaciones , Heridas Penetrantes/diagnóstico por imagen

RESUMEN

CASE REPORT: The authors report two cases of arachnoid cysts (ACs) neither detected during pregnancy nor shortly after birth when newborns underwent CT scan evaluation after birth head trauma. ACs were diagnosed at 10 months and 6 years, respectively. The first one becomes symptomatic, and the other one was incidentally found during a head trauma investigation. DISCUSSION: These cases give support for the postnatal pathogenesis for some of the assumed congenital ACs. We collected data from the literature that supports the acquired hypothesis for ACs.

Asunto(s)

Quistes Aracnoideos/diagnóstico por imagen , Quistes Aracnoideos/etiología , Traumatismos Craneocerebrales/complicaciones , Traumatismos Craneocerebrales/diagnóstico por imagen , Quistes Aracnoideos/cirugía , Niño , Traumatismos Craneocerebrales/cirugía , Humanos , Lactante , Masculino

RESUMEN

BACKGROUND: Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus. CASE PRESENTATION: A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up. CONCLUSION: Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.

Asunto(s)

Hemangioblastoma/complicaciones , Hidrocefalia/etiología , Hidrocefalia/cirugía , Neoplasias de la Médula Espinal/complicaciones , Antígenos CD34/metabolismo , Femenino , Hemangioblastoma/diagnóstico por imagen , Humanos , Hidrocefalia/diagnóstico por imagen , Lactante , Molécula-1 de Adhesión Celular Endotelial de Plaqueta/metabolismo , Neoplasias de la Médula Espinal/diagnóstico por imagen , Tomógrafos Computarizados por Rayos X

RESUMEN

METHODS: A literature review using the PubMed data base, MEDLINE, EMBASE, Science Direct, The Cochrane Database, Google Scholar, and clinical trials. Selected papers from 1922 to 2016 were studied. We selected 109 papers, through key-words, with inclusion and exclusion criteria. DISCUSSION: This paper discusses the risk factors for birth trauma, the anatomy of the occipito-anterior and vertex presentation, and traumatic brain lesions. CONCLUSION: Birth-related traumatic brain injury may cause serious complications in newborn infants. Its successful management includes special training, teamwork, and an individual approach.

Asunto(s)

Lesiones Encefálicas/etiología , Complicaciones del Trabajo de Parto , Femenino , Humanos , Recién Nacido , Embarazo , Factores de Riesgo

RESUMEN

ABSTRACT The neonatal period is a highly vulnerable time for an infant. The high neonatal morbidity and mortality rates attest to the fragility of life during this period. The incidence of birth trauma is 0.8%, varying from 0.2-2 per 1,000 births. The aim of this study is to describe brain traumas, and their mechanism, anatomy considerations, and physiopathology of the newborn traumatic brain injury. Methods A literature review using the PubMed data base, MEDLINE, EMBASE, Science Direct, The Cochrane Database, Google Scholar, and clinical trials. Selected papers from 1922 to 2016 were studied. We selected 109 papers, through key-words, with inclusion and exclusion criteria. Discussion This paper discusses the risk factors for birth trauma, the anatomy of the occipito-anterior and vertex presentation, and traumatic brain lesions. Conclusion Birth-related traumatic brain injury may cause serious complications in newborn infants. Its successful management includes special training, teamwork, and an individual approach.

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RESUMO O período neonatal é um período vulnerável para o recém nascido. As altas taxas de morbidade e mortalidade neonatal atestam a fragilidade da vida durante esta fase. Trauma durante o nascimento é de 0,8%, variando de 0,2 a 2 por 1000 nascimentos. O objetivo deste estudo é descrever o tocotraumatismo, seu mecanismo, considerações anatômicas e fisiopatologia da lesão em recém nascido. Métodos Revisão da literatura utilizando base de dados PubMed, MEDLINE, EMBASE, Science Direct, The Cochrane Detabase, Google Scolar, ensaios clínicos. Os trabalhos selecionados foram de 1922 a 2016. Foram selecionados 109 trabalhos, através de palavras-chave, inclusão e critérios de exclusão. Discussão Este artigo discute os fatores de risco para o trauma do nascimento, a anatomia da apresentação do vértex occipto-anterior e as lesões traumáticas cerebrais. Conclusão Lesão cerebral traumática no nascimento pode causar complicações graves nos recém-nascidos. O tratamento desta condição deve ser especializado, envolvendo trabalho, equipe e abordagem individualizado

Asunto(s)

Humanos , Femenino , Embarazo , Recién Nacido , Lesiones Encefálicas/etiología , Complicaciones del Trabajo de Parto , Factores de Riesgo

RESUMEN

BACKGROUND/AIMS: Although craniopharyngioma (CP) is histologically benign, it is a pituitary tumour that grows rapidly and often recurs. Adamantinomatous CP (ACP) was associated with an activating mutation in ß-catenin, and it has been postulated that pituitary stem cells might play a role in oncogenesis in human ACP. Stem cells have also been identified in pituitary adenoma. Our aim was to characterize the expression pattern of ABCG2, CD44, DLL4, NANOG, NOTCH2, POU5F1/OCT4, SOX2, and SOX9 stem cell markers in human ACP and pituitary adenoma. METHODS AND RESULTS: We studied 33 patients (9 ACP and 24 adenoma) using real-time quantitative PCR (RT-qPCR) and immunohistochemistry. SOX9 was up-regulated in ACP, exhibiting positive immunostaining in the epithelium and stroma, with the highest expression in patients with recurrence. CD44 was overexpressed in ACP as confirmed by immunohistochemistry. SOX2 did not significantly differ among the tumour types. The RT-qPCR array showed an increased expression of MKI67,OCT4/POU5F1, and DLL4 in all tumours. NANOG was decreased in ACP. ABCG2 was down-regulated in most of the tumours. NOTCH2 was significantly decreased in the adenomas. CONCLUSION: Our results confirm the presence of stem cell markers in human pituitary tumours as well as the different expression patterns of ACP and adenoma. These findings suggest that ACP may originate from a more undifferentiated cell cluster. Additionally, SOX9 immunodetection in the stroma and the highest expression levels related to the relapse of patients suggest a contribution to the aggressive behaviour and high recurrence of this tumour type.

Asunto(s)

Adenoma/metabolismo , Biomarcadores de Tumor/metabolismo , Craneofaringioma/metabolismo , Células-Madre Neurales/metabolismo , Neoplasias Hipofisarias/metabolismo , Adenoma/patología , Adolescente , Adulto , Anciano , Niño , Preescolar , Craneofaringioma/patología , Femenino , Expresión Génica , Humanos , Receptores de Hialuranos/metabolismo , Masculino , Persona de Mediana Edad , Neoplasias Hipofisarias/patología , Factor de Transcripción SOX9/metabolismo , Factores de Transcripción SOXB1/metabolismo , Adulto Joven

RESUMEN

PURPOSE: Patients with myelomeningocele have a high mortality and neurological disabilities that are correlated with the anatomical characteristics of the defect and with the development of acquired complications. The challenge in the postnatal management of myelomeningocele (MMC) is the early recognition of cases at risk for complications in order to establish individualized treatment strategies. This study aims to identify short-term prognostic markers for newborns with MMC. Anatomical characteristics of the spinal defect and technical aspects of the neurosurgical correction were analyzed for this purpose. METHODS: A retrospective cohort study was conducted in 70 patients with MMC born between January 2007 and December 2013. Features of MMC anatomy and neurosurgical treatment were analyzed for the following outcomes: neonatal resuscitation, length of hospital stay, need for ventricular shunt, wound dehiscence, wound infection, central nervous system infection, and sepsis. RESULTS: Large MMC was associated with central nervous system (CNS) infection, wound complications, and longer hospital stay. Patients with thoracic MMC required longer hospital stay. Surgical repair performed after 48 h of life increased in 5.72 times the risk of CNS infection. Absence of antenatal hydrocephalus was a favorable prognostic marker. CONCLUSION: Extent of the spinal cord defect and the time of surgical correction influenced the short-term outcomes of patients with myelomeningocele. Extensive lesions were associated with higher rates of CNS infections, surgical wound complications, and prolonged hospital stay. Interventions performed within 48 h after birth significantly reduced occurrence of CNS infections. Absence of antenatal hydrocephalus was associated with fewer complications in the first days of life.

Asunto(s)

Hidrocefalia/etiología , Meningomielocele/diagnóstico , Procedimientos Neuroquirúrgicos/métodos , Niño , Preescolar , Estudios de Cohortes , Femenino , Edad Gestacional , Humanos , Hidrocefalia/terapia , Lactante , Masculino , Meningomielocele/complicaciones , Meningomielocele/terapia , Diagnóstico Prenatal , Médula Espinal/patología , Factores de Tiempo

RESUMEN

Medulloblastoma is a highly aggressive brain tumor and one of the leading causes of morbidity and mortality related to childhood cancer. These tumors display differential ability to metastasize and respond to treatment, which reflects their high degree of heterogeneity at the genetic and molecular levels. Such heterogeneity of medulloblastoma brings an additional challenge to the understanding of its physiopathology and impacts the development of new therapeutic strategies. This translational effort has been the focus of most pre-clinical studies which invariably employ experimental models using human tumor cell lines. Nonetheless, compared to other cancers, relatively few cell lines of human medulloblastoma are available in central repositories, partly due to the rarity of these tumors and to the intrinsic difficulties in establishing continuous cell lines from pediatric brain tumors. Here, we report the establishment of a new human medulloblastoma cell line which, in comparison with the commonly used and well-established cell line Daoy, is characterized by enhanced proliferation and invasion capabilities, stem cell properties, increased chemoresistance, tumorigenicity in an orthotopic metastatic model, replication of original medulloblastoma behavior in vivo, strong chromosome structural instability and deregulation of genes involved in neural development. These features are advantageous for designing biologically relevant experimental models in clinically oriented studies, making this novel cell line, named USP-13-Med, instrumental for the study of medulloblastoma biology and treatment.