ABSTRACT
A hérnia medular idiopática (HMI) é uma causa rara de mielopatia progressiva que afeta principalmente mulheres de meia idade com apresentação clínica típica com a Síndrome de Brown-Sequard. Possui etiologia incerta, sendo a teoria mais aceita a de ser um defeito congênito na dura-máter que leva a uma herniação lenta e progressiva da medula que ocasiona uma lesão evolutiva, podendo levar a um déficit irreversível quando subdiagnosticado e não tratado da forma ideal. A realização da ressonância magnética é fundamental para o diagnóstico, e a cirurgia é o tratamento de escolha para reverter e cessar os sintomas mielopáticos. O presente artigo mostra um caso de uma apresentação atípica da localização do defeito dural e da herniação, não descrita ainda na literatura, levando a uma apresentação neurológica e anatômica incomum para esta patologia, obrigando a realizar um planejamento cirúrgico específico para tal caso.
Idiopathic medullary hernia is a rare cause of progressive myelopathy, primarily affecting middle-aged women, typical clinical presentation with Brown-Sequárd Syndrome. Its etiology is uncertain, but the most accepted theory is that a congenital defect in the dura mater leads to a slow and progressive spinal cord herniation, causing an evolutionary spinal cord injury, which can lead to an irreversible deficit when underdiagnosed and not treated adequately. Magnetic resonance imaging is essential for the diagnosis, and surgery is the treatment of choice to reverse and stop myelopathy symptoms. The present article shows a case of an atypical presentation of the location of the dural defect and herniation, not yet described in the literature, leading to an unusual neurologic and anatomical presentation for this pathology, requiring a specific planning for this case.
Subject(s)
Humans , Female , Adult , Spinal Cord Diseases , Herniorrhaphy , HerniaABSTRACT
OBJETIVO: describir la resolución quirúrgica de un caso de hernia medular transdural. Descripción: Paciente de 33 años de edad con antecedentes de historia progresiva de debilidad motora de 6 meses de evolución. Al examen se constató paraparesia asimétrica con anestesia por debajo del dermatoma T8, constituyendo un síndrome de Brown Sequard. En las imágenes de resonancia magnética se observó herniación ventral de la medula espinal a nivel T7-8. INTERVENCIÓN: se realizó abordaje extrapleural posterolateral a la columna torácica. Con técnica microquirúrgica se encontró el defecto dural en la duramadre ventral, y la herniación medular correspondiente en el nivel T7-8. Se encontraron, asimismo, cuerpos osteocartilaginosos en dicho nivel. Se procedió a disecar la interface médula espinal del defecto dural, ampliando el mismo y liberando la médula espinal, sin necesidad de retracción medular por esta vía. Luego de reponer en su correcta posición a la médula se procedió al cierre dural primario con microsutura. CONCLUSIÓN: las hernias medulares transdurales torácicas el abordaje posterolateral extracavitario es una opción válida, permitiendo tener una mejor visión microquirúrgica de la duramadre ventral, con la ventaja de no requerir tracción ni rotación neural como se requiere en el caso de la laminectomía tradicional
OBJECTIVE: to describe the successful surgical repair of idiopathic transdural spinal cord herniation DESCRIPTION: the patient was a 33-year-old female with a 6-month history of progressive motor weakness. Neurologic examination revealed asymmetric paraparesis with anesthesia below the T8 dermatome, consistent with Brown-Séquard Syndrome. Magnetic resonance imaging (MRI) of the thoracic spine demonstrated a spinal cord that was displaced and herniated ventrally, with loss of the normal overlying cerebrospinal fluid space between T7 and T8. INTERVENTION: a lateral extra-pleural approach was used to access the thoracic spine. With microsurgery, we identified a defect in the ventral dura, with transdural spinal cord herniation. Osteocartilaginous bodies also were observed. Microsurgical un-tethering of the spinal cord herniation was performed along with primary repair of ventral dural defect. To un-tether the herniation without spinal cord manipulation, an incision was made on one side of the defect; with this maneuver, the diameter of the defect was increased, avoiding any traction on neural structures. Once the herniated spinal cord was freed, the ventral dura was repaired via non-interrupted closure. CONCLUSION: the posterolateral extra-pleural approach is a good option to treat spinal cord herniation in the thoracic spine, providing a better optical corridor to detect ventral dural defects than with a thoracic laminectomy, and avoiding any neural traction or rotation
Subject(s)
Humans , Spinal Cord Diseases , Brown-Sequard Syndrome , HerniaABSTRACT
Introducción: La herniación medular transdural idiopática es una entidad rara cuyas formas son la postraumática y posquirúrgica; es omitida en la valoración preoperatoria y con frecuencia afecta al segmento torácico. Clínicamente puede causar mielopatía progresiva o síndrome de Brown-Séquard, cuyo diagnóstico se establece por resonancia magnética. La finalidad de informar esta entidad es su dificultad diagnóstica y, por lo tanto, para establecer un manejo óptimo. Casos clínicos: Dos pacientes mal diagnosticados al inicio e intervenidos en otros segmentos del raquis. Finalmente fueron valorados por sospecha clínica de herniación medular transdural idiopática y por exclusión de otras patologías. Se les realizó laminectomía en los niveles afectados, reducción de la hernia medular y colocación de parche sintético en duramadre. Conclusiones: La herniación medular transdural idiopática se ha atribuido a debilidad congénita de la duramadre o duplicación dural ventral con herniación a través de la capa interna debido a la presión continua del líquido cefalorraquídeo que empuja la médula fuera del espacio subdural. Se estima que el diagnóstico preoperatorio se realiza en una tercera parte de los casos, confirmándose con resonancia magnética. El tratamiento quirúrgico se efectúa en pacientes con progresión de los síntomas; los pacientes cuyos síntomas son leves o ausentes se mantienen bajo monitoreo. El tratamiento oportuno puede permitir la recuperación del déficit neurológico, mejorando la afección motora en 80% y la afección sensitiva en 35%.
BACKGROUND: Idiopathic transdural spinal cord herniation (ISCH) is a rare entity with postsurgical and post-trauma forms. ISCH is often omited in the preoperative evaluation. It often affects the thoracic segment and presents clinically as a rare cause of progressive myelopathy or Brown-Séquard syndrome, whose diagnosis is established by magnetic resonance imaging (MRI). We report on this rare entity due to its difficult diagnosis, making optimal management difficult. CLINICAL CASE: We present the cases of two patients with ISCH who were misdiagnosed and operated on in other spinal segments without reaching an accurate diagnosis. In our institution, patients with clinical suspicion were evaluated by imaging studies in order to rule out other pathologies. Laminectomy was performed on the involved levels, reducing herniation and with the placement of a synthetic spinal patch to the duramater. CONCLUSIONS: ISCH has been attributed to congenital weakness of the duramater or the dural ventral duplication with herniation through the inner layer due to continuous pressure from cerebrospinal fluid that pushes the marrow out of the subdural space. It is estimated that presurgical diagnosis is done only in one third of the cases, confirmed by MRI. Surgery is performed on patients with symptom progression. Surveillance in those patients with mild symptoms is recommended. Treatment may allow recovery of the neurological deficit, improving motor affection in 80% of patients and sensory affection in 35%.