ABSTRACT
BACKGROUND: Acromegaly is produced by excess growth hormone secreted by a pituitary adenoma of somatotroph cells (ACRO). First-line therapy, surgery and adjuvant therapy with somatostatin analogs, fails in 25% of patients. There is no predictive factor of resistance to therapy. New therapies are investigated using few dispersed tumor cells in acute primary cultures in standard conditions where the cells do not grow, or using rat pituitary cell lines that do not maintain the full somatotroph phenotype. The RET/PIT1/p14ARF/p53 pathway regulates apoptosis in normal pituitary somatotrophs whereas the RET/GDNF pathway regulates survival, controlling PIT1 levels and blocking p14ARF (ARF) and p53 expression. METHODS: We investigated these two RET pathways in a prospective series of 32 ACRO and 63 non-functioning pituitary adenomas (NFPA), studying quantitative RNA and protein gene expression for molecular-clinical correlations and how the RET pathway might be implicated in therapeutic success. Clinical data was collected during post-surgical follow-up. We also established new'humanized' pituitary cultures, allowing 20 repeated passages and maintaining the pituitary secretory phenotype, and tested five multikinase inhibitors (TKI: Vandetanib, Lenvatinib, Sunitinib, Cabozantinib and Sorafenib) potentially able to act on the GDNF-induced RET dimerization/survival pathway. Antibody arrays investigated intracellular molecular pathways. FINDINGS: In ACRO, there was specific enrichment of all genes in both RET pathways, especially GDNF. ARF and GFRA4 gene expression were found to be opposing predictors of response to first-line therapy. ARF cut-off levels, calculated categorizing by GNAS mutation, were predictive of good response (above) or resistance (below) to therapy months later. Sorafenib, through AMPK, blocked the GDNF/AKT survival action without altering the RET apoptotic pathway. INTERPRETATION: Tumor ARF mRNA expression measured at the time of the surgery is a prognosis factor in acromegaly. The RET inhibitor, Sorafenib, is proposed as a potential treatment for resistant ACRO. FUND: This project was supported by national grants from Agencia Estatal de Investigación (AEI) and Instituto Investigación Carlos III, with participation of European FEDER funds, to IB (PI150056) and CVA (BFU2016-76973-R). It was also supported initially by a grant from the Investigator Initiated Research (IIR) Program (WI177773) and by a non-restricted Research Grant from Pfizer Foundation to IB. Some of the pituitary acromegaly samples were collected in the framework of the Spanish National Registry of Acromegaly (REMAH), partially supported by an unrestricted grant from Novartis to the Spanish Endocrine Association (SEEN). CVA is also supported from a grant of Medical Research Council UK MR/M018539/1.
Subject(s)
Acromegaly/diagnosis , Acromegaly/metabolism , Glial Cell Line-Derived Neurotrophic Factor/metabolism , Proto-Oncogene Proteins c-ret/metabolism , Transcription Factor Pit-1/metabolism , Tumor Suppressor Protein p14ARF/metabolism , Tumor Suppressor Protein p53/metabolism , Acromegaly/genetics , Acromegaly/therapy , Animals , Apoptosis/genetics , Biomarkers , Combined Modality Therapy , Gene Expression Profiling , Gene Expression Regulation , Glial Cell Line-Derived Neurotrophic Factor/genetics , Humans , Immunohistochemistry , Models, Biological , Mutation , Pituitary Neoplasms/diagnosis , Pituitary Neoplasms/genetics , Pituitary Neoplasms/metabolism , Prognosis , Proto-Oncogene Proteins c-ret/genetics , Rats , Signal Transduction , Transcription Factor Pit-1/genetics , Treatment Outcome , Tumor Suppressor Protein p14ARF/genetics , Tumor Suppressor Protein p53/geneticsABSTRACT
Los tumores fibrosos solitarios/hemangiopericitomas (TFS/HPC) son tumores mesenquimales fibroblásticos caracterizados por una fusión genética NAB2-STAT6 con una escasa incidencia en el sistema nervioso central (SNC), de los cuales los espinales son una minoría. Presentamos el caso de una mujer de 63 años con paraparesia progresiva y nivel sensitivo D6 que presentaba una lesión intradural extramedular a nivel D9-D10. Realizamos laminectomía con estudio ecográfico intraoperatorio en que visualizamos una lesión heterogénea con patrón infiltrativo afectando a la médula en varios puntos y realizamos resección de la misma. La immunohistoquímica reveló positividad para vimentina, CD34 y STAT6, Ki67 del 2%, diagnosticándose TFS/HPC fenotipo TFS. Los TFS/HPC del SNC son neoplasias raras que deberían tenerse en cuenta en el diagnóstico diferencial de este tipo de lesiones, la clínica y la resonancia magnética suelen llevar a errores en el enfoque diagnóstico. El apoyo de la ecografía intraoperatoria puede ayudar en la toma de decisiones quirúrgicas
Solitary fibrous tumours/haemangiopericytomas (SFT/HPC) are fibroblastic mesenchymal neoplasms that harbour a genetic fusion of NAB2/STAT6. There are few reported cases in the central nervous system (CNS), of which spinal tumours are minority. We present a case of a 63-year-old woman with progressive paraparesis and a sensory level of T6. On the MRI we detected an intradural extramedullary lesion on T9-T10. We performed a laminectomy with an intraoperative ultrasound study in which we observed a heterogeneous lesion with an infiltrating pattern affecting the medulla at several points, and resection of the lesion. Immunohistological findings revealed positivity for vimentin, CD34 and STAT6; Ki-67 was 2%. A final diagnosis of SFT/HPC with SFT phenotype was made. SFT/HPC of the CNS is a rare neoplasm that should be taken into account in the differential diagnosis of these kinds of lesions, as clinical and MRI findings often lead to errors in diagnostic approach. The support of intraoperative ultrasound can assist in surgical decision-making
Subject(s)
Humans , Female , Middle Aged , Spinal Cord/diagnostic imaging , Hemangiopericytoma/diagnostic imaging , Hemangiopericytoma/surgery , Spinal Cord/pathology , Magnetic Resonance Spectroscopy , ImmunohistochemistryABSTRACT
Solitary fibrous tumours/haemangiopericytomas (SFT/HPC) are fibroblastic mesenchymal neoplasms that harbour a genetic fusion of NAB2/STAT6. There are few reported cases in the central nervous system (CNS), of which spinal tumours are minority. We present a case of a 63-year-old woman with progressive paraparesis and a sensory level of T6. On the MRI we detected an intradural extramedullary lesion on T9-T10. We performed a laminectomy with an intraoperative ultrasound study in which we observed a heterogeneous lesion with an infiltrating pattern affecting the medulla at several points, and resection of the lesion. Immunohistological findings revealed positivity for vimentin, CD34 and STAT6; Ki-67 was 2%. A final diagnosis of SFT/HPC with SFT phenotype was made. SFT/HPC of the CNS is a rare neoplasm that should be taken into account in the differential diagnosis of these kinds of lesions, as clinical and MRI findings often lead to errors in diagnostic approach. The support of intraoperative ultrasound can assist in surgical decision-making.
Subject(s)
Hemangiopericytoma/surgery , Solitary Fibrous Tumors/surgery , Spinal Cord Neoplasms/surgery , Biomarkers, Tumor/analysis , Decompression, Surgical , Diagnostic Errors , Female , Hemangiopericytoma/chemistry , Hemangiopericytoma/complications , Hemangiopericytoma/diagnostic imaging , Humans , Laminectomy , Lumbar Vertebrae/surgery , Magnetic Resonance Imaging , Meningeal Neoplasms/diagnosis , Meningioma/diagnosis , Middle Aged , Neuroimaging , Paraplegia/etiology , Solitary Fibrous Tumors/chemistry , Solitary Fibrous Tumors/complications , Solitary Fibrous Tumors/diagnostic imaging , Somatosensory Disorders/etiology , Spinal Cord Neoplasms/chemistry , Spinal Cord Neoplasms/complications , Spinal Cord Neoplasms/diagnostic imaging , Spinal Fusion , Spinal Stenosis/surgery , Surgery, Computer-Assisted , Thoracic Vertebrae , Ultrasonography, InterventionalABSTRACT
Spontaneous cerebrospinal fluid (CSF) leaks represent a clinical entity in which CSF rhinorrhea occurs in the absence of any inciting event. Spontaneous CSF leaks are associated with elevated intracranial pressure (ICP) or have underlying idiopathic intracranial hypertension (IIH). We report a cohort of patients who have undergone nasal endoscopic repair for spontaneous CSF leaks. We review our perioperative complications and the effectiveness of the nasal endoscopic approach to repair spontaneous CSF leaks. Also, we examine the evidence correlating spontaneous CSF leaks and IIH and the role of decreasing ICP in the treatment of nasal spontaneous CSF leaks. A retrospective analysis of patients with nasal spontaneous cerebrospinal fluid leaks was performed. Data on the nature of presentation, patient body mass index, defect location and size, ICP, clinical follow-up, and complications were collected. Thirty-five patients had nasal spontaneous cerebrospinal fluid leaks with evidence of IIH's symptoms. The most common sites were the cribriform plate, the ethmoid roof, and sphenoid lateral pterygoid recess. All patients underwent endonasal endoscopic surgery to repair the defect. Postoperatively, all patients underwent lumbar drainage and acetazolamide therapy. Nasal spontaneous cerebrospinal fluid leaks represent a surgical challenge because of their high recurrence rates. The most important factor for obtaining a successful repair in these patients is reducing their intracranial pressure through nutritional, medical, or surgical means.
Subject(s)
Cerebrospinal Fluid Leak/surgery , Natural Orifice Endoscopic Surgery/methods , Pseudotumor Cerebri/complications , Cerebrospinal Fluid Leak/diagnostic imaging , Cerebrospinal Fluid Leak/etiology , Cerebrospinal Fluid Rhinorrhea/etiology , Drainage , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Nose , Retrospective StudiesABSTRACT
La apoplejía pituitaria es normalmente el resultado de un infarto hemorrágico que acontece a un adenoma hipofisario. La presentación clínica comprende un espectro muy amplio e incluye desde casos asintomáticos, el cuadro clínico clásico e incluso muerte súbita. La isquemia cerebral tras una apoplejía pituitaria ocurre muy raras veces. Se postulan como mecanismos etiopatogénicos el vasoespasmo y/o la compresión vascular arterial provocada por el propio tumor. En el presente artículo describimos un caso de apoplejía pituitaria asociada a infarto cerebral y discutimos la relación entre ambos eventos
Pituitary apoplexy is usually the result of haemorrhagic infarction in a pituitary adenoma. The clinical presentation varies widely and includes asymptomatic cases, classical pituitary apoplexy and even sudden death. Cerebral ischemia due to pituitary apoplexy is very rare. It may be caused by vasospasm or direct compression of cerebral vessels by the tumor. We report a case of pituitary apoplexy associated with cerebral infarction and discuss the relationship between the two events
Subject(s)
Humans , Female , Middle Aged , Pituitary Apoplexy/complications , Cerebral Infarction/etiology , Vasospasm, Intracranial/etiology , Headache/etiology , Hyperprolactinemia/complications , Adrenal Cortex Hormones/therapeutic useABSTRACT
Pituitary apoplexy is usually the result of hemorrhagic infarction in a pituitary adenoma. The clinical presentation varies widely and includes asymptomatic cases, classical pituitary apoplexy and even sudden death. Cerebral ischemia due to pituitary apoplexy is very rare. It may be caused by vasospasm or direct compression of cerebral vessels by the tumor. We report a case of pituitary apoplexy associated with cerebral infarction and discuss the relationship between the two events.
Subject(s)
Cerebral Infarction/etiology , Pituitary Apoplexy/complications , Adenoma , Adult , Brain Ischemia , Female , Humans , Pituitary NeoplasmsABSTRACT
Objetivos: Analizar las características clínicas, los factores de recidiva y el resultado quirúrgico de un grupo de pacientes con hematoma subdural crónico bilateral (HSCB) y compararlos con una serie de hematomas subdurales crónicos unilaterales (HSCU). Pacientes y métodos: Estudio retrospectivo que incluye a 1.523 pacientes, tratados quirúrgicamente durante un período de 30 años, de un hematoma subdural crónico. Los pacientes se dividieron en 2 grupos: el de estudio formado por 190 pacientes que presentaban un HSCB y el control formado por 1.333 pacientes que presentaban un HSCB. Resultados: La serie de estudio está formada por 126 varones (66,3%) y 64 mujeres (33,7%) con una edad media de 74,8±10,2 años. En el grupo control hay 870 varones (65,2%) y 463 mujeres (34,8%) con una edad media de 73,2% ± 12,1. El síntoma de inicio más frecuente fue el deterioro cognitivo en 63 pacientes (33,2%) en los bilaterales y en 416 (29,5%) en los unilaterales. Se produjeron 18 casos de recidiva (9,4%) en los bilaterales y 77 (5,7%) en los unilaterales (p = 0,027). La mortalidad fue de 10 casos (5,2%) en los bilaterales y de 55 (4%) en el grupo control. Se encontraron como factores significativos de recidiva: sexo masculino (p = 0,022), la ingesta de fármacos anticoagulantes/antiagregantes (p = 0,032) y la mala situación clínica al ingreso (p = 0,039). Conclusiones: El HSCB afecta de manera importante a los varones y la forma de presentación más habitual es la cefalea. Los factores más importantes que influyen en las recidivas son el sexo masculino, la ingesta de fármacos anticoagulantes/antiagregantes y la peor situación clínica al ingreso. Los índices de recidiva fueron significativamente superiores a los unilaterales
Objective: The aim of this study is to analyse the clinical findings and surgical results in a series of patients with bilateral chronic subdural haematoma (BCSDH), and compare the results with a series of patients treated for unilateral chronic subdural haematoma (UCSDH). Patients and methods: A retrospective study was performed on 1523 patients diagnosed and surgically treated for chronic subdural haematoma over a period of 30 years. Patients were divided into 2 groups: The study group consisting of 190 patients operated on for a BCSDH and the control group consisting of patients operated on for an UCSDH (1333 cases). Results: The patient series included 126 males (66.3%) and 64 females (33.7%), with a mean age at diagnosis of 74.8 ± 10.2. The control group consisted of 870 males (65.2%) and 463 women (34.8%), with a mean age of 73.2 ± 12.1. The most common presenting symptoms was cognitive impairment in 63 patients (33.2%) with BCSDH and 416 (29.5%) with UCSDH. Recurrence rates were 9.4% (18 patients) and 5.7% (77 patients) in unilateral and bilateral haematomas, respectively. The mortality was 10 patients (5.2%) with BCSDH and 55(4%) with UCSDH. Factors significantly related to recurrence in the univariate analysis were being male (P = .040), anticoagulant/antiplatelet therapy (P = .032), and poor neurological status at admission (P = .039). Conclusions: This study indicates that BCSDH is more frequent in males, and the most common presentation is headache. The most important factors influencing recurrences are being male, intake of anticoagulant-antiaggregant drugs, and worse clinical status at admission
Subject(s)
Humans , Hematoma, Subdural, Chronic/epidemiology , Platelet Aggregation Inhibitors/therapeutic use , Anticoagulants/therapeutic use , Retrospective Studies , Risk Factors , Case-Control Studies , Tomography, X-Ray Computed , Recurrence , Severity of Illness IndexABSTRACT
OBJECTIVE: The aim of this study is to analyse the clinical findings and surgical results in a series of patients with bilateral chronic subdural haematoma (BCSDH), and compare the results with a series of patients treated for unilateral chronic subdural haematoma (UCSDH). PATIENTS AND METHODS: A retrospective study was performed on 1523 patients diagnosed and surgically treated for chronic subdural haematoma over a period of 30 years. Patients were divided into 2 groups: The study group consisting of 190 patients operated on for a BCSDH and the control group consisting of patients operated on for an UCSDH (1333 cases). RESULTS: The patient series included 126 males (66.3%) and 64 females (33.7%), with a mean age at diagnosis of 74.8±10.2. The control group consisted of 870 males (65.2%) and 463 women (34.8%), with a mean age of 73.2±12.1. The most common presenting symptoms was cognitive impairment in 63 patients (33.2%) with BCSDH and 416 (29.5%) with UCSDH. Recurrence rates were 9.4% (18 patients) and 5.7% (77 patients) in unilateral and bilateral haematomas, respectively. The mortality was 10 patients (5.2%) with BCSDH and 55(4%) with UCSDH. Factors significantly related to recurrence in the univariate analysis were being male (P=.040), anticoagulant/antiplatelet therapy (P=.032), and poor neurological status at admission (P=.039). CONCLUSIONS: This study indicates that BCSDH is more frequent in males, and the most common presentation is headache. The most important factors influencing recurrences are being male, intake of anticoagulant-antiaggregant drugs, and worse clinical status at admission.
Subject(s)
Hematoma, Subdural, Chronic/surgery , Adolescent , Adult , Aged , Aged, 80 and over , Female , Hematoma, Subdural, Chronic/pathology , Humans , Male , Middle Aged , Retrospective Studies , Young AdultABSTRACT
Los quistes aracnoideos son lesiones quísticas malformativas que contienen en su interior un líquido de características similares al líquido cefalorraquídeo. Constituyen alrededor del 1% de todos los procesos ocupantes de espacio a nivel intracraneal y, en los últimos años, su incidencia se ha incrementado ligeramente debido a la mejora en las técnicas de diagnóstico radiológico. Aunque la mayoría de los quistes aracnoideos se sitúan en la cavidad craneal, especialmente en la región de la cisura de Silvio, un menor número de casos pueden localizarse a nivel espinal. Realizamos una revisión sobre las diferentes técnicas de diagnóstico por imagen de los quistes aracnoideos, analizando la indicación de cada una de ellas en función de la localización y edad de los pacientes
Arachnoid cysts are malformed lesions that contain a fluid similar to the cerebrospinal fluid, and are usually located within the arachnoidal membrane. They represent 1% of all intracranial lesions, and in recent years, with the development of radiological techniques, the clinical detectability of arachnoid cysts seems to have increased. Although the majority of diagnosed arachnoid cysts are located in the cranial cavity and especially in the Sylvian fissure, a small number are located at spinal level and they can occur extra- or intra-spinally. An analysis is carried out, detailing the various tests used for the diagnosis of both intracranial and spinal arachnoids cysts, analysing the indications of each one depending on the location of the cysts and patient age
Subject(s)
Female , Humans , Male , Diagnostic Imaging/instrumentation , Diagnostic Imaging/methods , Arachnoid Cysts , Ultrasonography, Doppler, Transcranial/methods , Ultrasonography, Doppler, Transcranial , Magnetic Resonance Imaging/methods , Skull/pathology , Skull , Cerebral Aqueduct , Prenatal Diagnosis/methods , Diagnosis, DifferentialABSTRACT
Arachnoid cysts are malformed lesions that contain a fluid similar to the cerebrospinal fluid, and are usually located within the arachnoidal membrane. They represent 1% of all intracranial lesions, and in recent years, with the development of radiological techniques, the clinical detectability of arachnoid cysts seems to have increased. Although the majority of diagnosed arachnoid cysts are located in the cranial cavity and especially in the Sylvian fissure, a small number are located at spinal level and they can occur extra- or intra-spinally. An analysis is carried out, detailing the various tests used for the diagnosis of both intracranial and spinal arachnoids cysts, analysing the indications of each one depending on the location of the cysts and patient age.
Subject(s)
Arachnoid Cysts/diagnosis , Diagnostic Imaging , Diagnosis, Differential , HumansABSTRACT
Los quistes aracnoideos (QA) intracraneales son lesiones quísticas formados a partir de las aracnoides y que tienen una prevalencia entre el 1-2% en la población pediátrica. Presentamos el caso clínico de un niño de 2 años que presentó inestabilidad para la marcha y episodios de desviación ocular; al nacimiento se le había realizado una ecografía transfontanelar que fue normal. Se le practicó una tomografía computarizada (TC) y resonancia magnética (RM) que evidenciaron un quiste aracnoideo supraselar acompañado de importante hidrocefalia. Se realizó una fenestración endoscópica del quiste con remisión completa de la sintomatología. En la revisión de la literatura únicamente hemos encontrado 6 casos previos de QA sin un origen congénito o traumático, y exclusivamente uno de ellos de localización supraselar. Analizamos las características clínicas, radiológicas y el tratamiento en los casos publicados con anterioridad
Intracranial arachnoid cysts are fluid-filled cavities that arise within the cranial arachnoid, representing approximately 1%-2% of all intracranial lesions among the paediatric population. We present the case of a 2-year-old boy who presented with instability and episodes of ocular deviation. A computed tomography scan (CT scan) and magnetic resonance imaging (MRI) of the brain revealed a suprasellar cyst and obstructive hydrocephalus. At birth a transfontanellar ultrasound was normal. The cyst underwent endoscopic fenestration with complete remission of symptoms. In the review of the literature, we found only 6 previous cases of an intracranial arachnoid cyst whose origin was not clearly congenital or traumatic, and ours is the second case of a suprasellar arachnoid cyst to arise de novo. The clinical features, imaging characteristics and treatment of the previously reported cases are discussed
Subject(s)
Humans , Male , Infant , Arachnoid Cysts/surgery , Central Nervous System Cysts/surgery , Endoscopy/methods , CraniotomyABSTRACT
Intracranial arachnoid cysts are fluid-filled cavities that arise within the cranial arachnoid, representing approximately 1%-2% of all intracranial lesions among the paediatric population. We present the case of a 2-year-old boy who presented with instability and episodes of ocular deviation. A computed tomography scan (CT scan) and magnetic resonance imaging (MRI) of the brain revealed a suprasellar cyst and obstructive hydrocephalus. At birth a transfontanellar ultrasound was normal. The cyst underwent endoscopic fenestration with complete remission of symptoms. In the review of the literature, we found only 6 previous cases of an intracranial arachnoid cyst whose origin was not clearly congenital or traumatic, and ours is the second case of a suprasellar arachnoid cyst to arise de novo. The clinical features, imaging characteristics and treatment of the previously reported cases are discussed.
Subject(s)
Arachnoid Cysts , Arachnoid Cysts/diagnosis , Arachnoid Cysts/surgery , Child, Preschool , Humans , MaleABSTRACT
Los quistes aracnoideos son lesiones quísticas con un contenido similar al líquido cefalorraquídeo. Representan alrededor del 1% de todas las lesiones expansivas intracraneales y son característicos de las dos primeras décadas de la vida, aunque en ocasiones se diagnostican en adultos. Presentamos una revisión sobre nuestra experiencia en el tratamiento quirúrgico de quistes aracnoideos intracraneales. Material y métodos: Realizamos una revisión de 103 niños menores de 16 años intervenidos quirúrgicamente por un quiste aracnoideo intracraneal en los que se empleó una derivación cistoperitoneal en 53 casos; una craneotomía con fenestración del quiste, en 44 niños, y 11 casos con fenestración por vía endoscópica. Resultados: En todos los pacientes se consiguió alivio de la sintomatología y reducción o desaparición del quiste. Se presentaron 21 complicaciones quirúrgicas: 12 en los pacientes tratados con válvula (22.6%) y 9 en los sometidos a craneotomía (20.4%). La mortalidad de la serie fue de dos casos (1.95%). Discusión y conclusiones: El tratamiento de los quistes aracnoideos intracraneales debe reservarse exclusivamente para los casos sintomáticos. La craneotomía con fenestración de las membranas y la derivación cistoperitoneal son buenas opciones de tratamiento, ya que consiguen un buen control, tanto del tamaño del quiste como en la resolución de la sintomatología. La mejor opción terapéutica es en la actualidad la fenestración endoscópica, ya que se trata de una técnica poco invasiva, que no requiere la implantación de materiales extraños y cuyo índice de complicaciones y recidivas es relativamente bajo...
Subject(s)
Humans , Child , Arachnoid Cysts , Therapeutics , Craniotomy , Hematoma, Subdural , Neuroendoscopy , Tomography, X-Ray ComputedABSTRACT
Los quistes aracnoideos intracraneales constituyen una patología frecuente en la edad infantil y su tratamiento quirúrgico se realizará en función de su sintomatología y su localización. Habitualmente se trata de lesiones únicas, aunque pueden asociarse a otras patologías tanto malformativas como tumorales. Presentamos el caso clínico de un varón recién nacido diagnosticado de un quiste aracnoideo de la cisterna cuadrigeminal que fue tratado mediante endoscopia. En el postoperatorio, ante un empeoramiento neurológico se diagnosticó un meduloblastoma de cerebelo que no se evidenciaba en los estudios neurorradiológicos previos y del que se realizó una extirpación parcial. Dada la edad del paciente y el pobre pronóstico, no se realizó tratamiento oncológico. La asociación de un meduloblastoma de cerebelo y un quiste aracnoideo es excepcional, y hasta donde podemos conocer únicamente existe en la bibliografía un caso similar
Arachnoid cysts are very common lesions in paediatric patients, with treatment dependingon their location and symptomatology. They are usually solitary cysts but may be associatedwith other central nervous system diseases such as tumours and congenital deformities.We describe the case of a neonate diagnosed with an arachnoid cyst of the quadrigeminalcistern treated by endoscopy. After the operation, the childs condition worsened; a CT scanrevealed a midline posterior fossa tumour not visible in the preoperative neuroradiologicaltests. The tumour, a medulloblastoma, was partially removed. Given the childs age andthe poor prognosis, oncological treatment was not undertaken. The association between medulloblastoma and arachnoid cyst is very rare, and we could find only one such case inthe literatura
Subject(s)
Humans , Male , Infant, Newborn , Medulloblastoma/congenital , Arachnoid Cysts/congenital , Brain Neoplasms/congenital , Cisterna Magna/pathology , Hydrocephalus/etiology , Brain Neoplasms/surgeryABSTRACT
Tension pneumocephalus (TP) is a clinical entity characterized by continued build-up of air within the cranial cavity, leading to abnormal pressure exerted upon the brain and subsequent neurologic deterioration, due to development of a mass effect and potentially a herniation syndrome. Intracranial complications of endoscopic sinus surgery (ESS) and other endonasal procedures are fortunately very rare, occurring in less than 3% of cases. We report 4 cases of small bone defects (<3 mm) in the anterior cranial base accompanied by TP, caused by ESS and other endonasal procedures. The pathophysiology and management of this clinical entity is discussed with a pertinent literature. Four patients with small (<3 mm) skull base defects were identified. All patients presented with active cerebrospinal fluid leaks. CT scans showed intracranial tension pneumocephalus. Using image-guided endoscopic techniques, all defects were addressed with multi-layer repair. Closure was achieved in all patients on the first attempt, with an average follow-up of 36 months. Tension pneumocephalus is a rare event that can occur as a result of traumatic or iatrogenic violation of the dura and should be considered in all patients presenting with altered mental status after endoscopic sinus surgery or other surgical and diagnostic procedures that violate either the cranial or spinal dura. Because of the potential for rapid clinical deterioration and death, prompt brain imaging is warranted to rule out the diagnosis, and urgent neurosurgical consultation is indicated for definitive management.
Subject(s)
Nasal Septum/surgery , Paranasal Sinuses/surgery , Pneumocephalus/diagnosis , Pneumocephalus/physiopathology , Postoperative Complications/diagnosis , Postoperative Complications/physiopathology , Rhinoplasty , Sinusitis/surgery , Turbinates/surgery , Adult , Cerebrospinal Fluid Rhinorrhea/diagnosis , Cerebrospinal Fluid Rhinorrhea/physiopathology , Cerebrospinal Fluid Rhinorrhea/surgery , Chronic Disease , Dura Mater/injuries , Encephalocele/diagnosis , Encephalocele/physiopathology , Encephalocele/surgery , Female , Humans , Iatrogenic Disease , Male , Middle Aged , Neurologic Examination , Neuronavigation , Pneumocephalus/surgery , Postoperative Complications/surgery , Skull Base/physiopathology , Skull Base/surgery , Tomography, X-Ray ComputedABSTRACT
Arachnoid cysts are very common lesions in paediatric patients, with treatment depending on their location and symptomatology. They are usually solitary cysts but may be associated with other central nervous system diseases such as tumours and congenital deformities. We describe the case of a neonate diagnosed with an arachnoid cyst of the quadrigeminal cistern treated by endoscopy. After the operation, the child's condition worsened; a CT scan revealed a midline posterior fossa tumour not visible in the preoperative neuroradiological tests. The tumour, a medulloblastoma, was partially removed. Given the child's age and the poor prognosis, oncological treatment was not undertaken. The association between medulloblastoma and arachnoid cyst is very rare, and we could find only one such case in the literature.
Subject(s)
Arachnoid Cysts/congenital , Cerebellar Neoplasms/congenital , Infant, Premature, Diseases/surgery , Medulloblastoma/congenital , Arachnoid Cysts/diagnostic imaging , Arachnoid Cysts/surgery , Cerebellar Neoplasms/diagnostic imaging , Cerebellar Neoplasms/pathology , Cerebellar Neoplasms/surgery , Cesarean Section , Cranial Fontanelles , Craniotomy , Decompression, Surgical/methods , Fatal Outcome , Fourth Ventricle/pathology , Humans , Hydrocephalus/congenital , Hydrocephalus/diagnostic imaging , Hydrocephalus/etiology , Hydrocephalus/surgery , Infant, Newborn , Infant, Premature , Male , Medulloblastoma/diagnostic imaging , Medulloblastoma/pathology , Medulloblastoma/surgery , Neoplasm Invasiveness , Tectum Mesencephali/diagnostic imaging , Ultrasonography , VentriculostomyABSTRACT
Objetivos Analizar las características demográficas, clínicas, radiológicas y los resultados quirúrgicos de una serie de pacientes jóvenes con hematoma subdural crónico. Pacientes y métodos Estudio retrospectivo que incluye 42 pacientes con edad inferior a 40 años diagnosticados y tratados quirúrgicamente de un hematoma subdural crónico durante un período de 30 años (1982-2011).Resultados La serie consta de 32 varones y 10 mujeres con una edad media de 29,3±8,9 años (intervalo 4-39). El período transcurrido entre el traumatismo y el inicio de la sintomatología fue de 33,4±9,7 días (intervalo 19-95). Los principales síntomas de presentación fueron la cefalea (59,5%) y las crisis comiciales (21,4%), y los factores predisponentes más frecuentes fueron tener implantada una derivación ventrículo-peritoneal en 5 (11,9%) casos y los trastornos hematológicos en otros 5 (11,9%) pacientes. El hematoma fue derecho en 21 pacientes (50%), izquierdo en 19 (45,3%) y bilateral en los restantes 2 (4,7%). Se produjeron 2 complicaciones postoperatorias: un caso de recidiva y un hematoma subdural agudo sobre la cavidad del hematoma. No se produjeron muertes relacionadas con el tratamiento. Conclusiones El hematoma subdural crónico es una patología poco frecuente en las primeras décadas de la vida. Afecta con mayor frecuencia a los varones, y la forma de presentación más habitual es la cefalea. Los índices de recidiva y las complicaciones postoperatorias son significativamente inferiores que las presentes en otros rangos de mayor edad (AU)
Objective: The objective of this study was to analyse demographic, clinical and radiological findings and surgical results in a series of chronic subdural haematomas (CSDH) in young adult patients. Patients and methods: This retrospective study included 42 patients under 40 years of age who were diagnosed and surgically treated for a CSDH during a 30-year period (1982-2011).Results: Of the 42 cases analysed, 32 were males and 10 (..) (AU)
Subject(s)
Humans , Hematoma, Subdural, Chronic/surgery , Craniocerebral Trauma/complications , Headache/etiology , Retrospective Studies , Risk Factors , Arachnoid Cysts/surgery , Tomography, X-Ray ComputedABSTRACT
OBJECTIVE: The objective of this study was to analyse demographic, clinical and radiological findings and surgical results in a series of chronic subdural haematomas (CSDH) in young adult patients. PATIENTS AND METHODS: This retrospective study included 42 patients under 40 years of age who were diagnosed and surgically treated for a CSDH during a 30-year period (1982-2011). RESULTS: Of the 42 cases analysed, 32 were males and 10 were females, and the mean age at diagnosis was 29.3±8.9 years (range: 4 to 39 years). The mean interval from trauma to appearance of clinical symptoms was 33.4±9.7 days (range: 19 to 95 days). The main symptoms were headache (59.5%) and seizures (21.4%), and the most frequent predisposing factors were ventriculoperitoneal shunting in 5 (11.9%) patients and haematological disorders in another 5 (11.9%) cases. CSDH was right-sided in 21 cases (50%), left-sided in 19 cases (45.3%) and bilateral in the remaining 2 patients (4.7%). Postoperative complications occurred in 2 patients (1 recurrence and 1 acute subdural haematoma). CONCLUSIONS: CSDH is a rare pathology during the first decades of life. It mainly affects males and headache is usually the first symptom. Prognosis is good in young patients, since postoperative complications and recurrences are less frequent at this age than in older populations.
Subject(s)
Hematoma, Subdural, Chronic/epidemiology , Adolescent , Adult , Child , Child, Preschool , Craniotomy , Dominance, Cerebral , Female , Headache/etiology , Hematoma, Subdural, Chronic/complications , Hematoma, Subdural, Chronic/diagnostic imaging , Hematoma, Subdural, Chronic/surgery , Humans , Male , Postoperative Complications/etiology , Retrospective Studies , Seizures/etiology , Tomography, X-Ray Computed , Trephining , Ventriculoperitoneal Shunt/adverse effects , Young AdultABSTRACT
Our review of the literature is basically focused on the primary prophylaxis of early seizures after surgery of cerebral supratentorial tumors, with the aim of suggesting several recommendations in medical antiepileptic treatment to avoid this kind of seizures which occur immediately after surgery. In conclusion, it is recommended to provide criteria for prophylaxis of early seizures after surgery of cerebral supratentorial tumors. ItÌs recommended a one week treatment with antiepileptic drugs in patients who didnt have seizures jet, starting immediately after the surgical treatment. If seizures appear during progress of the disease, a large period treatment will be needed. Preferred antiepileptic treatment is intravenous and with a low interactions profile. Levetiracetam, followed by valproic acid seem to be most appropriated drugs due to their properties and protective effects, particularly for our patients requirements. These recommendations are considered a general proposal to effective clinical management of early seizures after surgery, not taking into account the single circumstances of our patients. Always, clinical features of the patients could modify even significantly these guides in the benefit of each patient.
Subject(s)
Neurosurgery , Seizures , Anticonvulsants/therapeutic use , Humans , Spain , Supratentorial NeoplasmsABSTRACT
Con la finalidad de proponer una serie de recomendaciones del tratamiento médico antiepiléptico, en el perioperatorio de los tumores cerebrales supratentoriales, se realiza una revisión de la literatura enfocada sobre todo a la profilaxis primaria de las crisis epilépticas precoces acaecidas en el postoperatorio inmediato. Se concluye que es recomendable pautar profilaxis primaria antiepiléptica poscirugía durante una semana en los pacientes con tumor cerebral supratentorial que no han presentado crisis epilépticas. Si las crisis aparecen durante la evolución de la enfermedad, es necesario pautar un tratamiento a largo plazo. Dadas las características de estos pacientes, se recomienda usar un fármaco antiepiléptico con presentación por vía intravenosa y un perfil bajo de interacciones. El levetiracetam, seguido del valproato, parecen ser los más adecuados. Dichas recomendaciones deben considerarse como una guía general de manejo, pudiendo ser modificadas, incluso de manera significativa, por las circunstancias propias de cada caso clínico (AU)
Our review of the literature is basically focused on the primary prophylaxis of early seizures after surgery of cerebral supratentorial tumors, with the aim of suggesting several recommendations in medical antiepileptic treatment to avoid this kind of seizures which occur immediately after surgery. In conclusion, it is recommended to provide criteria for prophylaxis of early seizures after surgery of cerebral supratentorial tumors. It's recommended a one week treatment with antiepileptic drugs in patients who didn't have seizures jet, starting immediately after the surgical treatment. If seizures appear during progress of the disease, a large period treatment will be needed. Preferred antiepileptic treatment is intravenous and with a low interactions profile. Levetiracetam, followed by valproic acid seem to be most appropriated drugs due to their properties and protective effects, particularly for our patients requirements. These recommendations are considered a general proposal to effective clinical management of early seizures after surgery, not taking into account the single circumstances of our patients. Always, clinical features of the patients could modify even significantly these guides in the benefit of each patient (AU)