How I do it: operative nuances of the Encephalo-Duro-Mio-Synangiosis (EDMS) technique for pediatric moyamoya disease and syndrome.

BACKGROUND: Moyamoya disease, a progressive occlusive arteriopathy mainly affecting the supraclinoid internal carotid artery, leads to abnormal "Moyamoya vessels" and ischemic events in children due to decreased cerebral blood flow. Surgery, especially indirect revascularization, is suggested for pediatric Moyamoya cases. METHOD: We present the Encephalo-Duro-Mio-Synangiosis (EDMS) technique, illustrated with figures and videos, based on 14 years' experience performing 71 surgeries by the senior author (SGJ) and the Moyamoya Interdisciplinary Workteam at "Prof. Dr. J. P. Garrahan" Pediatric Hospital. CONCLUSION: EDMS is a simple and effective treatment for Moyamoya disease, enhancing procedure precision and safety, reducing associated risks, complications, and improving clinical outcomes.

Case report: double dermal sinus tracts of the cervical and thoracic spine associated with congenital intracranial pathology (hydrocephalus and an interhemispheric cyst) in a newborn patient.

INTRODUCTION: A dermal sinus tract (DST) is an uncommon type of spinal dysraphisms characterized by a tract lined with stratified squamous epithelium that extends from the subcutaneous tissue to the underlying thecal sac or neural tube. These developmental anomalies can present asymptomatically with cutaneous abnormalities or with devastating complications. Usually, it is presented as a unique lesion, and there are only a few reports that show multiple sinuses, and none of them associated with midline brain malformations. METHODS: We present the case of a 3-day-old girl with an antenatal diagnosis of hydrocephalus who was diagnosed with double dermal sinus tracts of the cervical and thoracic regions at admission. The patient presented signs of elevated intracranial pressure (ICP), which imposed a challenge in the management of the case. RESULTS: Our patient was successfully treated initially with a lumbar puncture in order to discard a cerebrospinal fluid (CSF) infection. With negative CSF cultures, a ventriculoperitoneal shunt (VPS) was placed. Nine days after the VPS surgery and without signs of infection, the DST was excised in a single procedure, without follow-up complications. CONCLUSION: To our knowledge, this is the first description of a patient with multiple midline neural tube defects (NTDs) associated with congenital intracranial pathology. Although there are no guidelines regarding the best treatment for this complex associated pathology, the patient was treated, without follow-up complications.

Neurosurgical simulation models developed in Latin America and the Caribbean: a scoping review.

Simulation training is an educational tool that provides technical and cognitive proficiency in a risk-free environment. Several models have recently been presented in Latin America and the Caribbean (LAC). However, many of them were presented in non-indexed literature and not included in international reviews. This scoping review aims to describe the simulation models developed in LAC for neurosurgery training. Specifically, it focuses on assessing the models developed in LAC, the simulated neurosurgical procedures, the model's manufacturing costs, and the translational outcomes. Simulation models developed in LAC were considered, with no language or time restriction. Cadaveric, ex vivo, animal, synthetic, and virtual/augmented reality models were included for cranial and spinal procedures. We conducted a review according to the PRISMA-ScR, including international and regional reports from indexed and non-indexed literature. Two independent reviewers screened articles. Conflicts were resolved by a third reviewer using Covidence software. We collected data regarding the country of origin, recreated procedure, type of model, model validity, and manufacturing costs. Upon screening 917 studies, 69 models were developed in LAC. Most of them were developed in Brazil (49.28%). The most common procedures were related to general neurosurgery (20.29%), spine (17.39%), and ventricular neuroendoscopy and cerebrovascular (15.94% both). Synthetic models were the most frequent ones (38.98%). The manufacturing cost ranged from 4.00 to 2005.00 US Dollars. To our knowledge, this is the first scoping review about simulation models in LAC, setting the basis for future research studies. It depicts an increasing number of simulation models in the region, allowing a wide range of neurosurgical training in a resource-limited setting.

Impact of COVID-19 Infection on Patients with Cancer: Experience in a Latin American Country: The ACHOCC-19 Study.

INTRODUCTION: The ACHOCC-19 study was performed to characterize COVID-19 infection in a Colombian oncological population. METHODOLOGY: Analytical cohort study of patients with cancer and COVID-19 infection in Colombia. From April 1 to October 31, 2020. Demographic and clinical variables related to cancer and COVID-19 infection were collected. The primary outcome was 30-day mortality from all causes. The association between the outcome and the prognostic variables was analyzed using logistic regression models and survival analysis with Cox regression. RESULTS: The study included 742 patients; 72% were >51 years. The most prevalent neoplasms were breast (132, 17.77%), colorectal (92, 12.34%), and prostate (81, 10.9%). Two hundred twenty (29.6%) patients were asymptomatic and 96 (26.3%) died. In the bivariate descriptive analysis, higher mortality occurred in patients who were >70 years, patients with lung cancer, ≥2 comorbidities, former smokers, receiving antibiotics, corticosteroids, and anticoagulants, residents of rural areas, low socioeconomic status, and increased acute-phase reactants. In the logistic regression analysis, higher mortality was associated with Eastern Cooperative Oncology Group performance status (ECOG PS) 3 (odds ratio [OR] 28.67; 95% confidence interval [CI], 8.2-99.6); ECOG PS 4 (OR 20.89; 95% CI, 3.36-129.7); two complications from COVID-19 (OR 5.3; 95% CI, 1.50-18.1); and cancer in progression (OR 2.08; 95% CI, 1.01-4.27). In the Cox regression analysis, the statistically significant hazard ratios (HR) were metastatic disease (HR 1.58; 95% CI, 1.16-2.16), cancer in progression (HR 1.08; 95% CI, 1.24-2.61) cancer in partial response (HR 0.31; 95% CI, 0.11-0.88), use of steroids (HR 1.44; 95% CI, 1.01-2.06), and use of antibiotics (HR 2.11; 95% CI, 1.47-2.95). CONCLUSION: In our study, patients with cancer have higher mortality due to COVID-19 infection if they have active cancer, metastatic or progressive cancer, ECOG PS >2, and low socioeconomic status. IMPLICATIONS FOR PRACTICE: This study's findings raise the need to carefully evaluate patients with metastatic cancer, in progression, and with impaired Eastern Cooperative Oncology Group status to define the relevance of cancer treatment during the pandemic, consider the risk/benefit of the interventions, and establish clear and complete communication with the patients and their families about the risk of complications. There is also the importance of offering additional support to patients with low income and residence in rural areas so that they can have more support during cancer treatment.

[Coma due to syndrome of the trephined]. / Coma secundario a síndrome del trefinado.

The syndrome of the trephined or craniectomized is commonly referred as neurological manifestations associated to skin flap depression and reversible after craneoplasty, which allows its differentiation from post-traumatic syndrome. We present the case of a male patient, 36 years old, with history of decompressive craniectomy. He evolved with sudden neurological worsening associated to syndrome of the trephined and recovery after craneoplasty. Physiopathology of the syndrome involves cerebrovascular, metabolic and cerebrospinal fluid hydrodynamic disturbances as well as parenchymal hyperdynamic mechanisms. Cranioplasty is the gold standard treatment. Still, studies with statistical power are needed to assess correct surgical timing.

Vagus nerve stimulation in pediatric patients with drug-resistant epilepsy: a step-by-step video.

Vagus nerve stimulation (VNS) is a neuromodulatory treatment involving chronic intermittent electrical stimulation of the left vagus nerve, administered through a programmable pulse generator implanted subcutaneously in the chest. This generator connects to a bipolar lead, with electrodes wrapped around the vagus nerve in the neck. Primarily used as an adjunct therapy for patients with refractory epilepsy who cannot undergo or have not benefitted from resective surgery, VNS is generally well tolerated with few severe side effects. Herein is presented an educational surgical video providing a detailed, step-by-step technical description of VNS implantation. The video can be found here: https://stream.cadmore.media/r10.3171/2024.4.FOCVID244.

Surgical Anatomy and Technique of Peri-Insular Hemispherotomy in Pediatric Epilepsy.

BACKGROUND AND OBJECTIVES: Hemispherotomy is a highly complex procedure that demands a steep learning curve. An incomplete brain disconnection often results in failure of seizure control. The purpose of this article was to present a step-by-step guide to the surgical anatomy of this procedure. It is composed of a 7-stage approach, enhancing access to and improving visualization of deep structures. METHODS: A retrospective analysis of 39 pediatric patients with refractory epilepsy who underwent this technique was conducted. Engel scores were assessed 1 year postsurgery. Cadaveric dissections were performed to illustrate the procedure. RESULTS: Between 2015 and 2022, 39 patients were surgically treated using the peri-insular technique. The technique involved 7 stages: patient positioning, operative approach, opercular resection, transventricular callosotomy, fronto-orbital disconnection, anterior temporal disconnection, and posterior temporal disconnection. Most of the patients (92.30%) were seizure-free (Engel class I) at 1 year postoperative, 5.13% were nearly seizure-free (Engel II), and 2.56% showed significant improvement (Engel III). Complications occurred in 8% of cases, including 1 infection, 2 cases of aseptic meningitis, and 1 non-shunt-requiring acute hydrocephalus. CONCLUSION: The peri-insular hemispherotomy technique offers excellent seizure control with a low complication rate. Our visual documentation of surgical anatomy, complemented by detailed descriptions of surgical nuances, significantly contributes to a comprehensive understanding of this technique.

Results of training with a low-cost simulation model for endoscope-assisted scaphocephaly repair.

OBJECTIVE: Endoscope-assisted repair of sagittal craniosynostosis is an effective technique that requires a learning curve. Surgical simulation models can be applied to acquire the necessary skills for this procedure. Several models with a wide range of costs have been described for training in this technique. The aim of this work was to present the results of training with a low-cost simulation model for endoscope-assisted sagittal craniosynostosis repair. METHODS: A simulation model for sagittal craniosynostosis was developed using low-cost materials. The model is easily assembled and allows successive uses. Three neurosurgery residents, 3 fellows, and 2 neurosurgeons performed a 4-session training program in sagittal craniosynostosis repair. The Global Rating Scale (GRS) score, number of errors, and the time required to perform the task were reported by 2 independent evaluators using a checklist. Measurements were compared between the first and last training using the Wilcoxon signed-rank test. All participants completed a questionnaire (5-point Likert scale) regarding the realism of the simulation model. RESULTS: A model was developed to recreate the steps required to perform an endoscope-assisted scaphocephaly repair with the patient in a simulated sphinx position. All participants improved their GRS performance between the first and final training. The median time needed to perform the initial training was 47.5 minutes (interquartile range [IQR] 44.5-48 minutes, interrater difference [IRD] p = 0.77), and for the last training was 40.5 minutes (IQR 35.5-43 minutes, IRD p > 0.99). The median number of errors reported in the initial training was 5.5 (IQR 3-7.75 errors, IRD p = 0.8), and in the last training was 1 (IQR 0.75-2.25 errors, IRD p = 0.35). There was a statistically significant difference regarding the time and number of errors between the initial and final training (p < 0.001). More than 85% of the participants found that the surface anatomy, skull and anterior fontanel, fused sagittal suture, and epidural space of the model were realistic and had appropriate detail required to perform the surgery. All respondents agreed or strongly agreed that the endoscope handling was realistic, and that the steps and skills required to complete the task were representative of those required for the real procedure. CONCLUSIONS: A low-cost sagittal craniosynostosis simulation model was developed, allowing successive uses. The acquisition of skills within the simulation was demonstrated for all participants regarding the GRS score and the number of errors and time needed to perform the task. In addition, the model was found to be realistic in terms of anatomical references and the procedural steps required for this minimally invasive technique.

A Low-Cost Simulation Model for Endoscopic-Assisted Sagittal Craniosynostosis Repair.

OBJECTIVE: The objective is to introduce a low-cost simulation model for endoscopic-assisted sagittal craniosynostosis repair in which bleedings can be simulated and to present the initial experience using the model as a learning tool. METHODS: A 3-dimensional synthetic skull was printed using polylactic acid. The brain, dura mater, sagittal sinus, and skin were constructed using low-cost materials. The simulation was performed at Garrahan Pediatric Hospital's simulation center employing a rigid endoscope (Pes Pilot HD 0° 4 mm). RESULTS: A low-cost simulation model for sagittal craniosynostosis was built in order to recreate the steps needed to perform a strip craniectomy with lateral extensions. The initial estimated cost is 10 U.S. dollars, and subsequent uses cost approximately 2 U.S. dollars. Four training sessions were performed, and a reduction in surgical time was recorded from 125 to 80 minutes. CONCLUSIONS: We introduce a low-cost simulation model for scaphocephaly endoscopic-assisted surgery in which bleeding can be reproduced. The initial experience shows the possibility of using the model as a learning tool.

Low-Cost Stereotactic Brain Biopsy Simulation Model.

OBJECTIVE: Simulation training improves technical skills in a safe environment. Stereotactic techniques are widely used in neurosurgery for different kinds of procedures. The objective of the study was to describe a combined cadaveric and synthetic low-cost stereotactic simulation model and its validation by neurosurgeons. METHODS: The brain was made using self-supporting gel with solid and cystic lesions. We used imaging scans to calculate x, y, and z target coordinates. A standard frame needle biopsy was performed. We calculated the number of mistakes and time needed to accomplish the task, and we evaluated the frame assembly and biopsy performance. Wilcoxon signed rank was used to analyzed the data; we considered a P value <0.05 as statistically significant. RESULTS: The median initial number of mistakes was 32 (interquartile range [IQR]: 27.5-37) and after repeated training and feedback the final median number was 3.5 (IQR: 2-6) (P < 0.001). The median time needed to finish the exercises before training was 1020.5 seconds (IQR: 908-1125.5) and after using the model the final median time was 479 seconds (IQR: 423-503) (P < 0.0001). CONCLUSIONS: We presented a stereotactic simulation model with immediate haptic feedback. The model can be easily handmade in any neurosurgical laboratory. This model allows neurosurgeons in training to acquire and improve stereotactic techniques, reducing the number of surgical mistakes and time needed to finish the task.

Factores de riesgo asociados a la persistencia de hidrocefalia posterior a la resección de tumores de fosa posterior / Risk factors associated with hydrocephalus persistence after posterior fossa tumors resection

Introducción. Las neoplasias de fosa posterior son los tumores de sistema nervioso central más frecuentes en la población pediátrica y una causa frecuente de hidrocefalia. El objetivo del presente trabajo es analizar los factores de riesgo asociados a hidrocefalia luego de las cirugías de resección de tumores de fosa posterior en una población pediátrica. Material y métodos. Se realizó un estudio observacional retrospectivo de pacientes pediátricos operados de tumores de fosa posterior en un único hospital. Se analizaron potenciales factores de riesgo pre y post quirúrgicos. Se consideró como variable respuesta la necesidad de derivación definitiva para tratar la hidrocefalia a los 6 meses de la resección tumoral. Resultados. En el análisis univariado se detectaron múltiples factores de riesgo significativos. Sin embargo, solamente 3 se mantuvieron en el modelo multivariado: grado de resección (Subtotal: OR 7.86; Parcial: OR 20.42), infección postoperatoria (OR 17.31) y ausencia de flujo de salida postoperatorio en IV ventrículo (OR 4.29). Éste modelo presentó una buena capacidad predictiva (AUC: 0.80, Sensibilidad 80.5%, Especificidad 76.3%). Conclusión. La realización de tercer ventriculostomía endoscópica preoperatoria no redujo la incidencia de hidrocefalia postoperatoria. El grado de resección tumoral, la presencia de infección postoperatoria y la obstrucción de salida del IV ventrículo fueron los factores de riesgo más importantes para el requerimiento de sistema derivativo definitivo luego de la resección de un tumor de fosa posterior. Ésto podría influir en la toma de decisiones respecto al tratamiento en este grupo de pacientes pediátricos

Background. Posterior fossa tumors are the most frequent central nervous system neoplasms in the pediatric population and a frequent cause of hydrocephalus. The objective of this study is to analyze the risk factors associated with hydrocephalus after posterior fossa tumors resection in a pediatric population. Methods. A retrospective observational study was conducted on pediatric patients who underwent posterior fossa tumor resection in a single hospital. Potential pre- and post-operative risk factors were analyzed. The need for definitive shunt placement to treat hydrocephalus at 6 months after tumor resection was considered as the outcome variable. Results. Univariate analysis identified multiple significant risk factors. However, only 3 factors remained in the multivariate model: extent of resection (subtotal: OR 7.86; partial: OR 20.42), postoperative infection (OR 17.31), and absence of postoperative outflow of the fourth ventricle (OR 4.29). This model showed good predictive capacity (AUC: 0.80, Sensitivity 80.5%, Specificity 76.3%). Conclusion. Preoperative endoscopic third ventriculostomy did not reduce the incidence of postoperative hydrocephalus. The extent of tumor resection, presence of postoperative infection, and obstruction of fourth ventricle outflow were the most important risk factors for the requirement of a definitive shunt system after posterior fossa tumor resection. This could influence treatment decisions in this group of pediatric patients

Factores de riesgo asociados a la persistencia de hidrocefalia posterior a la resección de tumores de fosa posterior / Risk factors associated with hydrocephalus persistence after posterior fossa tumors resection

Introducción: Las neoplasias de fosa posterior son los tumores de sistema nervioso central más frecuentes en la población pediátrica y una causa frecuente de hidrocefalia. Objetivo: Analizar los factores de riesgo asociados a hidrocefalia luego de las cirugías de resección de tumores de fosa posterior en una población pediátrica. Material y Métodos: Se realizó un estudio observacional retrospectivo de pacientes pediátricos operados de tumores de fosa posterior en un único hospital. Se analizaron potenciales factores de riesgo pre y post quirúrgicos. Se consideró como variable respuesta la necesidad de derivación definitiva para tratar la hidrocefalia a los 6 meses de la resección tumoral. Resultados: En el análisis univariado se detectaron múltiples factores de riesgo significativos. Sin embargo, solamente 3 se mantuvieron en el modelo multivariado: grado de resección (Subtotal: OR 7.86; Parcial: OR 20.42), infección postoperatoria (OR 17.31) y ausencia de flujo de salida postoperatorio en IV ventrículo (OR 4.29). Éste modelo presentó una buena capacidad predictiva (AUC: 0.80, Sensibilidad 80.5%, Especificidad 76.3%). Conclusión: La realización de tercer ventriculostomía endoscópica preoperatoria no redujo la incidencia de hidrocefalia postoperatoria. El grado de resección tumoral, la presencia de infección postoperatoria y la obstrucción de salida del IV ventrículo fueron los factores de riesgo más importantes para el requerimiento de sistema derivativo definitivo luego de la resección de un tumor de fosa posterior. Ésto podría influir en la toma de decisiones respecto al tratamiento en este grupo de pacientes pediátricos(AU)

Background: Posterior fossa tumors are the most frequent central nervous system neoplasms in the pediatric population and a frequent cause of hydrocephalus. Objective: Analyze the risk factors associated with hydrocephalus after posterior fossa tumors resection in a pediatric population. Methods: A retrospective observational study was conducted on pediatric patients who underwent posterior fossa tumor resection in a single hospital. Potential pre- and post-operative risk factors were analyzed. The need for definitive shunt placement to treat hydrocephalus at 6 months after tumor resection was considered as the outcome variable. Results: Univariate analysis identified multiple significant risk factors. However, only 3 factors remained in the multivariate model: extent of resection (subtotal: OR 7.86; partial: OR 20.42), postoperative infection (OR 17.31), and absence of postoperative outflow of the fourth ventricle (OR 4.29). This model showed good predictive capacity (AUC: 0.80, Sensitivity 80.5%, Specificity 76.3%). Conclusion: Preoperative endoscopic third ventriculostomy did not reduce the incidence of postoperative hydrocephalus. The extent of tumor resection, presence of postoperative infection, and obstruction of fourth ventricle outflow were the most important risk factors for the requirement of a definitive shunt system after posterior fossa tumor resection. This could influence treatment decisions in this group of pediatric patients

Sagitectomía Endoscópica: Planeamiento Quirúrgico con Modelos de Simulación de Bajo Costo / Endoscopic Sagitectomy: Surgical Planning with Low-Cost Simulation Models

Objetivos: El objetivo es presentar un caso de sagitectomía asistida por endoscopía y su planificación y entrenamiento prequirúrgico con modelos de simulación de bajo costo. Métodos: Se procesó una tomografía de un paciente con escafocefalia, a partir de la cual se realizó una impresión 3D de un cráneo sin huesos parietales. Se completó el modelo de simulación con materiales de bajo costo para simular los tejidos blandos y hueso. Se realizó el entrenamiento con un endoscopio de 30º. Luego del entrenamiento, se realizó una cirugía en un paciente de 2 meses de edad que presentaba una escafocefalia. Resultados: Se creó un modelo de simulación de bajo costo (aproximadamente 20 US$) para el entrenamiento de sagitectomía asistida por endoscopía. Se realizó la cirugía en un paciente, sin complicaciones quirúrgicas ni posoperatorias. Conclusión: El entrenamiento con simuladores de bajo costo permite planificar una sagitectomía asistida por endoscopía, disminuyendo los errores y los tiempos de la curva de aprendizaje.

Factores pronósticos en el traumatismo craneoencefálico grave en pediatría / Prognostic factors in severe head injury in pediatrics

Objetivos: Analizar y establecer una asociación entre las características del Traumatismo Craneoencefálico (TEC) grave en pediatría (edad, etiología, características clínicas, lesiones intracraneales y cirugía) y los resultados a largo plazo. Material y Método: Revisión de los pacientes con TEC grave ingresados al Hospital Garrahan desde enero 2013 hasta enero 2019. Se analizaron las características al ingreso y el tratamiento instaurado. Se utilizó la escala Glasgow Outcome Scale (GOS) para evaluar los resultados a 12 meses. Se realizó un análisis estadístico mediante las pruebas de Chi2 y Fisher. Se consideró como significativo a un valor de p menor a 0.05. Resultados: Se registraron 54 pacientes con TEC grave y seguimiento posterior de 12 meses. La mediana de edad fue de 6 años (3-12). La mayoría fue de sexo masculino 62.96% (34). La etiología más frecuente fue la caída de altura (42.59%) mientras que la lesión intracraneal que más se observó fue el hematoma extradural (25.93%). Los factores que se asociaron a mal pronóstico fueron las lesiones no accidentales (100% vs 0%; p=0.02), la midriasis bilateral (100% vs 0%; p= 0.001) y el hematoma subdural (70% vs 30%; p= 0.002). Los factores asociados a buen pronóstico fueron las caídas de altura (54.84% vs 45.16%; p=0.01) y un examen oftalmológico normal al ingreso (90% vs 10%; p=0.006). Conclusión: Los factores que se asociaron a mal pronóstico fueron el trauma no accidental, la midriasis y el hematoma subdural agudo. Por otro lado, las caídas y el examen oftalmológico normal se asociaron a mejor pronóstico.

Objective: To analyze and establish an association between the characteristics of severe traumatic brain injury (TBI) in pediatric patients (age, etiology, clinical characteristics, intracranial injuries and surgery) and long-term results. Material and Method: Review of patients with severe TBI admitted to Garrahan Pediatric Hospital from January 2013 to January 2019. The characteristics of admission and treatment instituted were analyzed. The Glasgow Outcome Scale (GOS) was used to evaluate results at 12 months. A statistical analysis was performed using Chi2 and Fisher tests. A p value less than 0.05 was considered significant. Results: A total of 54 patients with severe TBI and subsequent follow-up of 12 month were reported. The median age was 6 years (3-12). Most were male 62.96% (34). The most common etiology was height falls (42.59%) while the most observed intracranial lesion was extradural hematoma (25.93%). Non-accidental injuries (100% vs 0%; p=0.02), bilateral mydriasis (100% vs 0%; p=0.001) and subdural hematoma (70% vs 30%; p=0.002) were associated with poor prognosis. Height drops (54.84% vs 45.16%; p=0.01) and a normal ophthalmological examination at income (90% vs 10%; p=0.006) were associated with good prognosis. Conclusion: Non-accidental trauma, mydriasis and acute subdural hematoma were associated with poor prognosis, whereas falls and normal eye exam were associated with better prognosis

Malignant solitary fibrous tumor of the kidney: report of the first case managed with interferon.

Solitary fibrous tumors of the kidney are extremely rare tumors with unpredictable behavior. We describe a case of a patient with a solitary fibrous tumor of kidney with malignant findings with distant metastasis and nephrectomy managed with subcutaneous interferon achieving 23 months of progression-free survival. To date there is no prospective evaluation of any specific modality of treatment, but the surgical management and long-term followup are the only ones so far recommended strategies in the management of these patients. Studies are awaited with more patients to evaluate the different strategies of systemic therapy reported so far to allow adding survival benefit.

Coma secundario a síndrome del trefinado / Coma due to syndrome of the trephined

El síndrome del trefinado o craniectomizado abarca manifestaciones neurológicas asociadas a la depresión del flap cutáneo y se distingue del síndrome postraumático por su reversibilidad con el tratamiento reparador del defecto craneano. El coma no es una forma habitual de presentación. Comunicamos un caso de presentación atípica en un hombre de 36 años de edad con antecedente de craniectomía descompresiva, que presentó un cuadro de deterioro neurológico profundo atribuible al síndrome del trefinado, el cual revirtió tras la craneoplastía. En la fisiopatología del síndrome intervienen trastornos cerebrovasculares, metabólicos, hidrodinámicos del líquido cefalorraquídeo e hiperdinamismo de las estructuras encefálicas. El gold standard terapéutico es la craneoplastía. Se requieren estudios de mayor peso estadístico para determinar el tiempo quirúrgico apropiado.

The syndrome of the trephined or craniectomized is commonly referred as neurological manifestations associated to skin flap depression and reversible after craneoplasty, which allows its differentiation from post-traumatic syndrome. We present the case of a male patient, 36 years old, with history of decompressive craniectomy. He evolved with sudden neurological worsening associated to syndrome of the trephined and recovery after craneoplasty. Physiopathology of the syndrome involves cerebrovascular, metabolic and cerebrospinal fluid hydrodynamic disturbances as well as parenchymal hyperdynamic mechanisms. Cranioplasty is the gold standard treatment. Still, studies with statistical power are needed to assess correct surgical timing.

Use of erlotinib throughout pregnancy: a case-report of a patient with metastatic lung adenocarcinoma.

The use of erlotinib throughout pregnancy has not been previously reported. We present the case of a 40 year-old female patient with stage IV lung adenocarcinoma, mediastinal, bone and cerebral metastasis, a EGFR mutation and no smoking history, who had begun first line treatment with erlotinib 150 mg once daily. After two and a half months of treatment a fourteen-week pregnancy was documented, and after informing on fetal risks secondary to erlotinib use and maternal risks secondary to treatment withholding, she decided to continue with treatment under clinical surveillance by both the oncology and obstetrics clinics. At thirty-three weeks gestation a live born 1600 g female was born by caesarean section without evidence of congenital malformations. Imaging assessment after eight months of treatment showed complete bone and central nervous system response and partial lung and mediastinal response. The patient is currently undergoing the 11th month of treatment and is asymptomatic, the baby is 4 months old and is in good health.

Tratamiento oncológico del angiosarcoma cutáneo: revisión de la literatura / Oncologic treatment of cutaneous angiosarcoma: review of the literature

El hemangiosarcoma cutáneo es una enfermedad maligna rara de origen vascular, y corresponde a menos del 1% de todas las malignidades y al 2% de todos los sarcomas de tejidos blandos. Su presentación usual es en el rostro y en la región del cuero cabelludo; al momento de diagnosticarse ya es una enfermedad avanzada. Afecta a menudo al anciano del género masculino y de raza blanca. El tratamiento oncológico se basa en la resección quirúrgica, la radioterapia y la quimioterapia, dado el alto riesgo tanto de recaída local como de diseminación hematológica con intención paliativa. Las tasas de control locorregional a 5 años son, aproximadamente, del 40% al 50%, las tasas de supervivencia libre de metástasis a distancia a 5 años están en el rango del 20% al 40%, y las tasas de supervivencia a 5 años se encuentran entre el 10% y el 30%.

Cutaneous hemangiosarcoma is a rare malignant disease of vascular origin which accounts for less than 1% of all malignancies and 2% of all soft tissue sarcomas. It most frequently affects elderly white males, and is usually found on the face and scalp; at diagnosis it tends to be advanced. Oncologic treatment is based upon surgical resection, radiotherapy and chemotherapy due to the high risk of local relapse as well as to hematologic dissemination with palliative intention. Loco-regional control rates at 5 years range from 40% to 50%, metastasis-free survival rates at 5 years are from 20% to 40%, and survival rates at 5 years from 10% to 30%.

Carcinoma córtico-adrenal: revisión de la literatura / Adrenal-cortical carcinoma: a review of the literature

El carcinoma córtico-adrenal es una entidad que se presenta raras veces; su evolución es agresiva, con una alta probabilidad de recaída y una supervivencia a 5 años que no supera el 60%. El único tratamiento curativo es la cirugía, siempre y cuando esta sea completa y a los pacientes se los diagnostique en estadios tempranos. Otras intervenciones que se pueden brindar son la radioterapia, la quimioterapia y el control de secreción hormonal en el contexto adyuvante o paliativo. En algunos casos (síndrome de Cushing) el bloqueo hormonal previo a la cirugía es imperativo. En esta revisión se describen la patogénesis, el diagnóstico, los factores pronósticos y el tratamiento del carcinoma córtico-adrenal, con el propósito de guiar el enfoque diagnóstico y el tratamiento.

Adrenal-cortical carcinoma is a rarely occurring entity; it evolves aggressively, has a high probability of relapse and survival at 5 years does not surpass 60%. Surgery provides the only curative treatment, but only when it is complete and carried out on patients with early-stage diagnosis. Additional treatments that may be used include radiotherapy, chemotherapy and control of hormonal secretion in an adjuvant or palliative context. In some cases (Cushing´s syndrome), it is imperative to provide hormonal block before surgery. The pathogenesis, diagnosis, prognostic factors and treatment of adrenal-cortical carcinoma are described in this review in order to sharpen the focus on diagnosis and treatment.