Assuntos
Caixa Torácica , Humanos , Recém-Nascido , Caixa Torácica/diagnóstico por imagem , Caixa Torácica/anormalidades , Masculino , Siringomielia/diagnóstico por imagem , Siringomielia/complicações , Disostoses/diagnóstico por imagem , Disostoses/genética , Disostoses/complicações , Síndrome Congênita de Insuficiência da Medula Óssea/complicações , Síndrome Congênita de Insuficiência da Medula Óssea/genética , Hérnia Diafragmática/diagnóstico por imagem , Hérnia Diafragmática/complicações , Feminino , Anormalidades MúltiplasRESUMO
BACKGROUND: Scoliosis is a spinal deformity that usually manifests as a structural curve determining a variable deformity of the trunk. According to some published series, 4 to 26% of scoliosis initially classified as idiopathic show neurological alterations when studied with nuclear magnetic resonance, such as syringomyelia and Chiari malformation, among the most frequent. Chiari malformations are characterized by descending herniation of the cerebellar tonsils, brainstem, and IV ventricle into the spinal canal. CASE REPORT: We present the case of a patient whose first symptomatic manifestation was early-onset scoliosis. The overlapping of some physical examination signs, such as postural lateralization and scoliosis, reinforced the active suspicion of neuroaxis alterations. CONCLUSIONS: Early childhood-onset scoliosis should raise a high degree of suspicion for association with neuro-spinal diseases. Although the frequency of Chiari malformation is low, its early approach could reduce the progression of associated comorbidities. An early detection could change the prognosis of the disease.
INTRODUCCIÓN: La escoliosis es una deformidad de la columna que usualmente se manifiesta como una curva estructural que determina una deformidad variable del tronco. Las malformaciones de Chiari se caracterizan por la herniación descendente de las amígdalas cerebelosas, tronco encefálico y IV ventrículo hacia el canal espinal. Según algunas series publicadas, del 4 al 26% de las escoliosis inicialmente catalogadas como idiopáticas muestran alteraciones neurológicas al ser estudiadas con resonancia magnética nuclear, como la siringomielia y malformación de Chiari, dentro de las más frecuentes. CASO CLÍNICO: Se presenta el caso de un paciente cuya primera manifestación sintomática fue escoliosis de comienzo temprano. El solapamiento de algunos signos del examen físico, como la lateralización de la postura y la escoliosis, reforzaron la sospecha activa de alteraciones del neuroeje. CONCLUSIONES: La escoliosis de inicio temprano en la infancia debe despertar un alto grado de sospecha de asociación con enfermedades neuro-espinales. Dentro de este contexto, aunque malformación Chiari es de baja frecuencia, su abordaje precoz permitiría disminuir la progresión de comorbilidades asociadas. La detección temprana podría cambiar el pronóstico de la enfermedad.
Assuntos
Malformação de Arnold-Chiari , Escoliose , Siringomielia , Humanos , Pré-Escolar , Malformação de Arnold-Chiari/complicações , Malformação de Arnold-Chiari/diagnóstico , Malformação de Arnold-Chiari/cirurgia , Siringomielia/complicações , Siringomielia/diagnóstico , Siringomielia/cirurgia , Escoliose/etiologia , Escoliose/complicações , Imageamento por Ressonância Magnética , Descompressão CirúrgicaRESUMO
La siringomielia supone un desafío diagnóstico, ya que es una entidad poco reconocida si no se tiene conciencia de su existencia. Al ser un cuadro progresivo, cuya clínica puede presentarse de forma larvada y ser coincidente con otras patologías neurológicas tales como la esclerosis múltiple, su detección suele realizarse en etapas tardías sobre todo en población adulta y más aún cuando se presenta de forma adquirida. Por lo que el estudio imagenológico con Resonancia Magnética adquiere especial relevancia, permitiendo identificar y clasificar la enfermedad, lo que brindará la base para decidir terapía.
Syringomyelia is a diagnostic challenge, since it is a poorly recognized disease, especially if its existence remains unknown. Being a progressive disease, whose clinic can present in a latent way and be coincident with other neurological pathologies such as multiple sclerosis, its detection is usually conducted in late stages, especially in the adult population and even more when it presents in an acquired way. Therefore, the imaging study with Magnetic Resonance acquires special relevance, allowing to be identified and classified, which will provide the basis for deciding on therapy
Assuntos
Humanos , Feminino , Pessoa de Meia-Idade , Siringomielia/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Siringomielia/terapiaRESUMO
Currarino syndrome (CS) is characterized by a triad of anomalies consisting of: a sacral bone defect, anorectal malformations and a pre-sacral mass. We present the case of an adult patient with a medical history of CS who presented with septic shock and was subjected to an emergency laparotomy due to severe abdominal distension. In this particular case, we underline the importance of immediate surgery on the patient's outcome as well as the considerable role of landiolol in controlling the heart rate with no further deterioration of blood pressure in this patient presenting with atrial fibrillation and sepsis.
Assuntos
Anormalidades do Sistema Digestório , Sepse , Siringomielia , Adulto , Canal Anal/anormalidades , Humanos , Reto/anormalidades , Sacro/anormalidadesRESUMO
Multiple congenital malformations can occur concomitantly in several species since the anomaly in one organ may lead directly to the malformation of another. Additionally, the etiology is not always clarified. Choristoma refers to an ectopic tissue that is histologically normal in an abnormal location. A case of pulmonary and nodal choristoma associated with cranioschisis, hydrocephalus, and syringomyelia in a new-born calf is reported here. Clinically, the calf had a mass in the frontal region of the head associated with local bone deformation. At necropsy, there was cranioschisis in the junction of the frontal bones and a 14 × 10 × 7 cm mass, grossly consistent with well-differentiated pulmonary tissue covered by skin, covering the opening between the frontal bones, and extending into the cranial cavity, leading to local cerebral compression. With the pulmonary choristoma, there was a well-differentiated lymphoid tissue. Additionally, in the central nervous system, there was severe hydrocephalus involving lateral ventricles and multiple areas of syringomyelia in the spinal cord.
Múltiplas malformações congênitas podem ocorrer concomitantemente em diversas espécies, uma vez que a anomalia em um órgão pode diretamente acarretar a malformação de outro. Ainda, a etiologia envolvida nem sempre é identificada. Coristomas são caracterizados por tecido ectópico histologicamente normal em uma localização anômala. Um caso de coristoma pulmonar e nodal associado a craniosquise, hidrocefalia e siringomielia em um bezerro recém-nascido é descrito neste trabalho. Clinicamente, o bezerro apresentava uma massa na região frontal da cabeça associada a deformação óssea local. No exame de necropsia, havia craniosquise na junção dos ossos frontais e tecido pulmonar bem diferenciado recoberto por pele, medindo 14 x 10 x 7 cm, recobrindo a abertura entre os ossos frontais e adentrando a cavidade craniana, acarretando o achatamento do córtex cerebral adjacente. Juntamente com o tecido pulmonar, havia um nódulo composto por tecido linfoide bem diferenciado. Adicionalmente, no sistema nervoso central, havia hidrocefalia severa envolvendo os ventrículos laterais e múltiplas áreas de siringomielia na medula espinhal.
Assuntos
Animais , Feminino , Bovinos , Siringomielia/veterinária , Anormalidades Congênitas/veterinária , Bovinos/anormalidades , Coristoma/veterinária , Hidrocefalia/veterinária , PulmãoRESUMO
Objetivo: describir y analizar los casos de siringomielia por malformación de Chiari tipo 1 con resolución espontánea. Método: se buscó en Pubmed bajo las palabras clave malformación de Chiari tipo 1, siringomielia y resolución espontánea todos los casos relacionados. Se registraron las características poblacionales de cada caso, evolución y mecanismos propuestos. Resultados: En la población adulta se encontraron 22 casos (edad media: 35,81) y en la población pediátrica se encontraron 37 casos (edad media: 9,82). Las hipótesis sobre los mecanismos que produjeron la resolución espontánea fueron: cambios en la posición de las amígdalas relacionadas con la edad, atrofia del cerebelo, fisura medular, reducción del esfuerzo físico, variaciones de la presión intraespinal, crecimiento del cráneo y disminución de la presión intracraneana. Conclusión: La resolución espontánea es posible en pocos casos. Si bien este hecho invita a realizar controles periódicos y evitar la cirugía, la espera conlleva riesgos ante la posibilidad de un agravamiento agudo o de que surjan secuelas permanentes. La información disponible hoy en día no permite predecir con seguridad la evolución de cada caso
Objective: describe and analyze the cases of syringomyelia caused by Chiari type 1 malformation with spontaneous resolution. Method: we searched in Pubmed with the key words Chiari malformation type 1, syringomyelia and spontaneous resolution all related cases. The population characteristics of each case were recorded along with its outcome and proposed mechanisms. Results: in the adult population there were 22 cases (medium age: 35.81) and in the pediatric population there were 37 cases (medium age: 9.82). The hypothesis about the mechanisms that produce the spontaneous resolution were: changes in tonsils position related to age, cerebellar atrophy, cord fissures, reduction of physical effort, variations in intraspinal pressure, skull growth and decrease in intracranial pressure. Conclusion: the spontaneous resolution of syringomyelia invites to carry out regular check-ups and avoid surgery. However, waiting carries risks with the possibility of acute worsening or permanent sequelae. The information available today does not allow to predict with certainty the evolution of each case.
Assuntos
Malformação de Arnold-Chiari , Siringomielia , Fossa Craniana PosteriorRESUMO
BACKGROUND: Patients with adolescent idiopathic scoliosis (AIS) are commonly monitored for curve progression with spinal radiographs; however, the utility of magnetic resonance imaging (MRI) screening is unclear. The purpose of this study was to assess the findings of screening MRI for patients with a nonsurgical curve size ordered during routine clinical care and compare them with MRI ordered for patients with large curves as part of preoperative screening. METHODS: All consecutive patients with presumed AIS who underwent entire-spine MRI with a presumed diagnosis of idiopathic scoliosis at a single institution between 2017 and 2019 were retrospectively reviewed. Patients were stratified based on MRI indication into the following groups: preoperative evaluation, pain, neurological symptoms, abnormal radiographic curve appearance, rapidly progressive curve, and other. Neural axis abnormalities recorded included concern for tethered spinal cord, syringomyelia, and Chiari malformation. The MRI findings of preoperative patients with large curves were compared with all other patients. The number needed to diagnose (NND) a neurological finding was calculated in patients whose MRIs were ordered during routine clinical care. The amount charged for each patient undergoing entire-spine MRI was determined by review of our institution's Financial Decision Support system. RESULTS: There were 344 patients included in this study with 214 (62%) MRIs performed for preoperative evaluation. Although MRI abnormalities were found in 49% of patients, only 7.0% (24/344) demonstrated neural axis abnormalities with no difference between preoperative and other indications (P=0.37). For patients with nonsurgical curves undergoing MRI due to a complaint of back pain (n=28), there were no neural axis abnormalities, and a lower rate of disk herniation/degenerative changes detected compared with preoperative MRI (3.6% vs. 18%, P=0.06). Among the 15 patients undergoing MRI for a neurological concern, 1 had a neural axis abnormality that required surgical detethering. The NND for MRI to detect a neural axis abnormality that potentially required neurosurgical intervention in nonpreoperative patients with a neurological concern was 34.4. The average cost for MRI was $17,816 (range: $2601 to $22,411) with a total cost of $2,368,439 for nonsurgical curves. CONCLUSIONS: Entire-spine MRI for nonpreoperative indications including pain, abnormal radiographic curve appearance, and rapid curve progression has minimal utility for patients with AIS. For patients with neurological complaints, the NND a potentially treatment-altering finding with MRI is 34.4. LEVEL OF EVIDENCE: Level II-diagnostic.
Assuntos
Escoliose , Siringomielia , Adolescente , Criança , Humanos , Imageamento por Ressonância Magnética , Estudos Retrospectivos , Escoliose/diagnóstico por imagem , Escoliose/cirurgia , Coluna VertebralAssuntos
Artropatia Neurogênica , Doenças Musculoesqueléticas , Doenças do Sistema Nervoso Periférico , Articulação do Ombro , Siringomielia , Artropatia Neurogênica/diagnóstico por imagem , Artropatia Neurogênica/etiologia , Humanos , Ombro , Articulação do Ombro/diagnóstico por imagem , Siringomielia/complicações , Siringomielia/diagnóstico por imagemRESUMO
TITLE: Meningioma sincitial intramedular asociado a siringomielia cervical. Réplica.
Assuntos
Neoplasias Meníngeas , Meningioma , Neoplasias da Medula Espinal , Siringomielia , Humanos , PescoçoRESUMO
Much controversy remains on the current management of Chiari I deformity (CID) in children, with many clinical, surgical and ethic-legal implications. The Brazilian Society of Pediatric Neurosurgery (SBNPed, in the Portuguese acronym) has put together a panel of experts to analyze updated published data on the medical literature about this matter and come up with several recommendations for pediatric neurosurgeons and allied health professionals when dealing with CID. Their conclusions are reported herein, along with the respective scientific background.