Hérnia medular idiopática atípica ­ relato de caso / Atypical Idiopathic Spine Cord Herniation (ISCH) ­ Case Report

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.

Idiopathic Spinal Cord Herniation

STUDY DESIGN: Literature review. OBJECTIVES: The aim of this study was to provide insight into idiopathic spinal cord herniation (ISCH) in terms of clinical presentation, pathophysiology, diagnosis, classification, and treatment. SUMMARY OF LITERATURE REVIEW: ISCH is a rare disorder characterized by anterior displacement of the spinal cord through a ventral dural defect. It has increasingly been recognized and described over the past 10 years. MATERIALS AND METHODS: Review of the English-language literature on ISCH. RESULTS: ISCH occurs in middle-aged adults with a female preponderance. The most common clinical presentation is Brown-Sequard syndrome, which can progress to spastic paraparesis. Its pathophysiology is unknown. However, some authors proposed that inflammation may play an important role in the emergence of a dural defect. Magnetic resonance imaging typically shows an anterior kink of the thoracic spinal cord with an obliteration of the ventral subarachnoid space and the widened dorsal subarachnoid space. Surgery is generally recommended for patients with motor deficits or progressive neurological symptoms. The posterior approach has been used because it allows wide exposure of the spinal cord. The surgical treatment of ISCH consists of spinal cord reduction from the ventral dural defect, which can be managed with enlargement, direct repair, or duraplasty (dural repair with a patch). In recent years, duraplasty has been used more frequently than enlargement of the dural defect. CONCLUSIONS: ISCH causing thoracic myelopathy could be safely treated with surgical management. The possibility of this disease should be kept in mind when treating patients with progressive myelopathy.

Posterior Trans-Dural Repair of Iatrogenic Spinal Cord Herniation after Resection of Ossification of Posterior Longitudinal Ligament

Iatrogenic spinal cord herniation is a rare complication following spinal surgery. We introduce a posterior trans-dural repair technique used in a case of thoracic spinal cord herniation through a ventral dural defect following resection of ossification of the posterior longitudinal ligament (OPLL) in the cervicothoracic spine. A 51-year-old female was suffering from paraplegia after laminectomy alone for cervicothoracic OPLL. Magnetic resonance imaging revealed a severely compressed spinal cord with pseudomeningocele identified postoperatively. Cerebrospinal fluid leak and iatrogenic spinal cord herniation persisted despite several operations with duroplasty and sealing agent. Finally, the problems were treated by repair of the ventral dural defect with posterior trans-dural duroplasty. Several months after surgery, the patient could walk independently. This surgical technique can be applied to treat ventral dural defect and spinal cord herniation.

Idiopathic Spinal Cord Herniation Presented as Brown-Sequard Syndrome: A Case Report and Surgical Outcome

Spinal cord herniation is a rare condition that has become increasingly recognised in the last few years. The authors report a case of idiopathic spinal cord herniation in a 33 year old woman performed with progressive Brown-Sequard syndrome. The diagnosis was made on MR imaging. After repairing the herniation, the patient made a gradual improvement. Potential causes are discussed, including a possible role of dural defect. In conclusion, idiopathic spinal cord herniation is a potentially treatable condition that should be more readily diagnosed that increasing awareness and improved imaging techniques.

Abordaje posterolateral extracavitario a la columna torácica y hernia medular transdural idiopática: reporte de caso y revisión de la bibliografía / Posterolateral approach to the thoracic spine and idiopathic transdural medullary hernia: case report and literature review

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

Idiopathic spinal cord herniation syndrome: a case report.

Idiopathic spinal cord herniation syndrome is a rare entity. Only 17 cases have been reported in literature so far. They typically occur in the dorsal region of the spinal cord, spontaneously, through a defect/rent in the anterior Dura. Majority present in middle age women, with progressive paraparesis/asymmetrical motor weakness in lower limbs.

Unilateral Paramedian Transpedicular Approach for Repair of Anterior Transdural Spinal Cord Herniation: Report of a Case and Literature Review

Idiopathic spinal cord herniation is a rare but potentially treatable cause of thoracic myelopathy. The rarity and variable clinical presentation often results in missed diagnosis and delay in treatment. Posterior midline approach with laminectomy has been the most common approach performed for spinal cord herniation in cases described in the literature. A posterior approach is limited by the danger of retracting the spinal cord and difficulty visualizing the dural defect. Considering the anterior location of the dural defect, a posterolateral allows for a more ventral view without spinal cord manipulation. We report a rare case of idiopathic spinal cord herniation which was managed by unilateral paramedian transpedicular approach with an excellent clinical outcome.

Herniación medular transdural idiopática: informe de dos casos y revisión de la literatura / Idiopathic transdural spinal cord herniation: report of two cases and literature review

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%.

A Case Suggesting Spontaneous Spinal Cord Herniation Presented as Brown-Sequard Syndrome

No abstract available.

Idiopathic Spinal Cord Herniation as a Treatable Cause of Progressive Brown-Sequard Syndrome

Idiopathic spinal cord herniation is a rare spinal cord disorder caused by spinal cord prolapse through a adural defect. It is a curable disease, so early detection is of particular importance. We report a 38-year-old woman with Brown-Sequard syndrome which was caused by the thoracic spinal cord herniation. Her weakness was almost completely resolved after surgical management, which emphasizes the importance of early diagnosis and surgical management in this rare disease entity.

Spontaneous Thoracic Spinal Cord Herniation

Spontaneous herniation of the spinal cord is a very rare. It's clinical symptom presents with progressive myelopathy. A 42-year old male patient who presented the progressive left leg weakness and Brown-Seqaurd syndrome is presented. MRI showed a typical finding of dural defect and herniation of the cord on the level of T3-4. Repair of dural defect using an artificial dura and reposition of cord herniation were undertaken after three level laminectomies with SSEP monitoring. Postoperatively, symptoms were improved rapidly. In our knowledgement,this is first case being reported in Korea. This entity, although rare, should be considered in the differential diagnosis of myelopathy in the absence of a mass lesion.