Idiopathic herniation of the thoracic spinal cord: report of three cases

STUDY DESIGN: Cases are reported and the literature is reviewed. OBJECTIVE: To present three cases involving idiopathic herniation of the thoracic cord. SUMMARY OF BACKGROUND DATA: Idiopathic spinal cord herniation is a very rare condition. Only 20 cases have been reported. The radiographic and intraoperative findings concerning this herniation remain insufficient, and its pathophysiology is less understood. METHODS: Idiopathic herniation of the thoracic spine was managed operatively in the three cases. The clinical, radiologic, and intraoperative features in these cases are described, and the pathophysiology of this disorder is discussed from a review of the literature. RESULTS: Two of the three patients had a defect in the inner layer of the duplicated ventral dura mater through which the spinal cord was herniated. The third patient had a ventral epidural cyst into which the spinal cord had protruded. Operative reduction of the spinal cord improved motor power in all three patients, although sensory disturbance remained unchanged. CONCLUSIONS: There should be several types of idiopathic spinal cord herniation. This is the first report of this herniation that focuses the abnormalities of the ventral dura mater, together with image and intraoperative findings.     

Idiopathic spinal cord herniation: report of three cases and review of the literature

STUDY DESIGN: Three case reports and a literature review are presented. OBJECTIVE: To describe characteristic clinical and radiographic findings of idiopathic spinal cord herniation. SUMMARY OF BACKGROUND DATA: Idiopathic spinal cord herniation is a rare disease, with only 26 cases reported before the current study. METHODS: Three cases of idiopathic spinal cord herniation are reported, and previous reports on this subject are reviewed. RESULTS: The responsible regions were in the thoracic spine from T2 to T7. Symptoms were mainly unilateral muscle atrophy in the lower extremity and sensory disturbance below the thoracic level. These symptoms had been progressing gradually. Magnetic resonance imaging demonstrated a unique feature: The spinal cord shifted anteriorly in a few segments. Computed tomographic myelogram showed another distinctive picture: There was no subarachnoid space anterior to the spinal cord. CONCLUSIONS: Because idiopathic spinal cord herniation is out of the concept of "compression myelopathy," this condition may be a pitfall in the diagnosis. Idiopathic spinal cord herniation should be recognized as one of the treatable causes for thoracic myelopathy.     

Idiopathic spinal cord herniation: a new theory of pathogenesis

BACKGROUND: Idiopathic spinal cord herniation is a rare entity that has been described more frequently over the past few years. Its pathophysiology remains obscure, however. METHODS: We report a case of spinal cord herniation and review the literature extensively. In view of our review, we try to determine the clinical features of the condition and the diagnostic measures used, with emphasis on the role of magnetic resonance (MR) phase-contrast CSF study. The factors affecting the outcome of the condition are also studied including time and type of presentation, as well as the surgical procedure performed. The pathophysiological mechanisms behind spontaneous herniation are discussed, and a new hypothesis is proposed. RESULTS: Idiopathic spinal cord herniation occurs in the middle-aged adult, with a preponderance of patients being female. Brown-Séquard syndrome is the most common clinical presentation and usually progresses to spastic paraparesis. MRI typically shows a ventral kink in the thoracic cord, with MR phase-contrast imaging proving an important addition to exclude an arachnoid cyst. Better outcomes were noted in the patients treated earlier, and in those with no spasticity. Widening the dural defect seems to afford better results compared to grafting of the defect. The prognosis is favorable after correction, though a vertebral body herniation variant may be associated with worse outcome. In view of the chronology of events and imaging studies in our patient, we hypothesize that herniation occurs as an acquired phenomenon where an inflammatory process results in adherence between the spinal cord and the dura, with erosion, formation of a dural defect, and then later herniation occurring with cerebral spinal fluid (CSF) pulsations. CONCLUSIONS: Idiopathic herniation of the spinal cord should be recognized and treated early to reach a favorable outcome. It seems to be an acquired condition likely caused by an inflammatory event, the nature of which is to be determined.     

Idiopathic spinal cord herniation: case report and review of the literature

OBJECTIVE AND IMPORTANCE: Idiopathic spinal cord herniation (ISCH) is a rare condition, reported in only 25 patients thus far, in which the thoracic cord is prolapsed through an anterior dural defect. It typically presents in middle age as either Brown-Sequard syndrome or spastic paraparesis. CLINICAL PRESENTATION: A 55-year-old woman initially presented at the age of 41 years with Brown-Sequard syndrome at the T8 disc space level on the left side. Investigations, including primitive magnetic resonance imaging, were deemed negative at that time. After a stepwise deterioration over 14 years, she presented again with spastic paraparesis and double incontinence, in addition to her previous spinothalamic dysfunction. Magnetic resonance imaging at this stage suggested either ISCH or a dorsal arachnoid cyst. INTERVENTION: Through a T7-T8 laminectomy, a left-of-midline ISCH was identified and easily reduced by gentle cord traction. No dorsal arachnoid cyst was identified. The anterior dural defect was repaired with a XenoDerm patch (LifeCell Corp., Woodlands, TX). After surgery, there was improved motor and sphincter function. However, there was continued sensory disturbance. CONCLUSION: ISCH is rare cause of thoracic cord dysfunction. Despite prolonged diagnostic delay, significant clinical improvement may be obtained with ISCH reduction and anterior dural repair.     

Long-term surgical outcomes of idiopathic spinal cord herniation

Background  

Because of the lack of long-term postoperative follow-up studies of idiopathic spinal cord herniation (ISCH), there is little information about the long-term effectiveness and complications of the dural defect enlargement in patients with ISCH. The purpose of this study is to determine the long-term effectiveness of this procedure.     

特发性脊髓疝1例报告并文献复习

<正>脊髓疝是一种罕见的疾病,以脊髓疝出硬膜外及继发的脊髓功能障碍为特点。可分为创伤后性、医源性和特发性三类[1]。创伤后性及医源性脊髓疝有其特殊的病史,容易明确诊断;特发性脊髓疝临床罕见,临床表现与很多脊髓疾病相似,影像检查有时鉴别困难,加之对其认识不足,术前确诊困难。2011年我院收治1例特发性脊髓疝,报告如下。患者女,24岁,因双下肢麻木无力进行性加重2年5个月于2011年3月9日入院。患者2008年10月始无明     

Idiopathic spinal cord herniation associated with calcified thoracic disc extrusion--case report

A 48-year-old man presented with idiopathic spinal cord herniation associated with calcified thoracic disc extrusion at the T7-8 intervertebral level, manifesting as Brown-Sequard syndrome at the thoracic level persisting for 20 years. Preoperative magnetic resonance imaging and computed tomography myelography revealed ventral displacement of the spinal cord and extrusion of a calcified disc at the T7-8 intervertebral level. At surgery, the spinal cord herniation at this level was released from the dura mater and carefully returned to the dural sac. An extruded calcified thoracic disc was found just below the dural defect at the same level. The development of idiopathic spinal cord herniation is associated closely with a defect in the ventral dura mater of unknown etiology. In our case, the etiology of the ventral dural defect was probably associated with the calcified thoracic disc extrusion.     

Surgical management of aggressive vertebral hemangiomas causing spinal cord compression: long-term clinical follow-up of five cases

Background  

Aggressive vertebral hemangiomas causing spinal compression are rare, and there is controversy with regard to treatment. The purpose of this study was to evaluate the clinical results of patients with aggressive vertebral hemangiomas at a mean follow-up of more than 10 years after total excision and discuss the treatment options for the tumors.     

Idiopathic spinal cord herniation which extended remarkably up- and downward from dural defect: case report

A case of idiopathic spinal cord herniation which extended remarkably up- and downward from a dural defect is described. A 53-year-old woman presented with numbness and pain of the right lower limb. Magnetic resonance (MR) imaging revealed ventral displacement of the spinal cord and dilation of the dorsal subarachnoid space at T4-5. CT Myelography showed ventral deviation of the spinal cord at the T4/5 level. A laminectomy of T3-T5 was performed, and the herniated spinal cord was untethered and wrapped by Goretex membrane. Postoperative MR image revealed normal location of the spinal cord. Among many cases of spinal cord herniations, this one is considered to be a rare case of idiopathic spinal cord herniation which showed marked protrusion up- and downward from a dural defect.     

Transplantation of autologous activated Schwann cells in the treatment of spinal cord injury: six cases, more than five years of follow-up

Schwann cells (SCs) are the main glial cells of the peripheral nervous system, which can promote neural regeneration. Grafting of autologous SCs is one of the well-established and commonly performed procedures for peripheral nerve repair. With the aim to improve the clinical condition of patients with spinal cord injury (SCI), a program of grafting autologous activated Schwann cells (AASCs), as well as a series of appropriate neurorehabilitation programs, was employed to achieve the best therapeutic effects. We selected six patients who had a history of SCI before transplantation. At first, AASCs were obtained by prior ligation of sural nerve and subsequently isolated, cultured, and purified in vitro. Then the patients accepted an operation of laminectomy and cell transplantation, and no severe adverse event was observed in any of these patients. Motor and sensitive improvements were evaluated by means of American Spinal Injury Association (ASIA) grading and Functional Independence Measure (FIM); bladder and urethral function were determined by clinical and urodynamic examination; somatosensory evoked potentials (SSEPs) and motor evoked potentials (MEPs) were used to further confirm the functional recovery following transplantation. The patients were followed up for more than 5 years. All of the patients showed some signs of improvement in autonomic, motor, and sensory function. So we concluded that AASC transplantation might be feasible, safe, and effective to promote neurorestoration of SCI patients.     

Modified surgical technique for the treatment of idiopathic spinal cord herniation.

OBJECTIVE AND IMPORTANCE: Spinal cord herniation is not a common disease and only 80 cases have been reported to the best of our knowledge. The treatment of this lesion is thought to be difficult and a better surgical technique has not been established. CLINICAL PRESENTATION: A 57-year-old man presented with gradually worsening spastic gait and hypoesthesia in the bilateral lower extremities suspected to be due to Brown-Séquard syndrome. Magnetic resonance imaging showed a thoracic spinal cord herniation into the anterior hiatus at T2 - 3. INTERVENTION: A modified technique of closure of the hiatus in front of the herniation was performed. A surgical artificial dural membrane was introduced between the herniated portion of the thoracic cord and the dural hiatus and was placed to cover the whole dural defect in order to minimize the operative procedure at the ventral side of the spinal cord. On subsequent magnetic resonance imaging, the herniation was remarkably improved and patient's symptoms were abolished. CONCLUSION: The direct widening of the dural defect has been reported to be the only treatment providing a good outcome. We suggest that our modified technique for its closure can be as safe and effective as the direct widening method.     

Idiopathic spinal cord herniation associated with intervertebral disc extrusion: a case report and review of the literature

STUDY DESIGN: A case of idiopathic spinal cord herniation is reported, and the literature is reviewed. OBJECTIVE: To report a case of thoracic spinal cord herniation with a ventral dural defect, probably caused by thoracic disc extrusion. SUMMARY OF BACKGROUND DATA: Recently, reports of spinal cord herniation have been increasing. This increase can be attributed to the development of magnetic resonance imaging and increased awareness of this entity. However, the cause of the ventral dural defect remains unknown. METHODS: A 54-year-old woman had experienced Brown-Séquard syndrome for 2 years. Magnetic resonance imaging demonstrated an S-shaped anterior kinking of the spinal cord, with dilation of the dorsal subarachnoid space. RESULTS: After incision of the dural sac and gentle retraction of the spinal cord, a dural defect was recognized into which the spinal cord had herniated. An extruded disc was visualized through the defect at T3-T4. The ventral dural defect and the dorsal incision of the dural sac were repaired with a fascial graft from the thigh. CONCLUSIONS: Intraoperative findings suggest that the thoracic disc herniation in the current case was the probable cause of the ventral dural defect. Surgical reconstruction using double fascial graft under careful spinal cord monitoring resulted in a satisfactory neurologic recovery.     

Management of intramedullary spinal cord tumors: surgical considerations and results in 45 cases

Background and purpose

Intramedullary spinal cord tumors (IMSCT) are relatively rare neoplasms, accounting for less than 5% of all central nervous system tumors. The optimum management of these tumors still remains controversial. Many decades ago, partial surgical resection followed by radiotherapy was the conventional management for IMSCT. Nowadays, maximal surgical resection of IMSCT without adjuvant therapy is the rule. We discuss the management of our cohort of 45 patients and review retrospectively the surgical outcome and survival.

Methods and results

We reviewed the charts of 45 patients who underwent surgery for IMSCT in our institution since 1990. The study included 23 female and 22 male with a mean age of 28.7 years (range: 18 months-64 years). In 40 patients, the final diagnosis was based on the results of MR imaging. The cervical location of the tumor was the most common (20 cases). Surgical procedures included a gross-total resection in 31 cases, subtotal resection in six cases, partial resection in five cases and a biopsy in three cases. The large majority of patients had histologically-proven low-grade tumors composed essentially of astrocytomas (44,4%) and ependymomas (28,8%). There was no mortality related to surgery. Concerning the functional outcome at six months, we noted that 22.2% of our patients deteriorated, 47.3% stayed the same and 30.5% improved. We found that patients with mild or no preoperative deficits were exceptionally damaged by the surgical procedure.

Conclusions

The gold-standard treatment of IMSCT remains maximal microsurgical resection without adjuvant therapy. For malignant or rapidly recurrent IMSCT, the optimum management is still controversial. Determinant predictors for a good outcome after surgery of IMSCT are histological type of lesion, total removal of the tumor and a satisfactory neurological status before surgery.     

Clinico-radiological correlation and surgical outcome of idiopathic spinal cord herniation: A single centre retrospective case series

Context: Idiopathic spinal cord herniation (ISCH) remains an obscure, under recognised but potentially treatable entity among the etiologies of compressive myelopathy.Findings: Here, we present a retrospective case series of eight patients of ISCH with their clinical features, imaging findings and long-term follow up data along with a relevant review of the literature. Mean age at presentation was 36 years with the duration of symptoms ranging from two months to more than thirty years. Contrary to existing data, only two patients in our series had the classical presentation of Brown-Sequard syndrome (n = 2). The clinical spectrum of the presentation was broad with pure spastic quadriparesis (n = 3), girdle sensation (n = 1) and radiculopathy (n = 1), the latter presentation has been unreported for ISCH. The typical radiological picture was seen in all patients. All except one patient was managed conservatively. In the one patient who underwent bilateral dentate ligament excision, there was mild functional improvement on long-term follow-up despite a definite worsening of the radiological picture. Five of the patients had a long-term follow-up with no clinically or functionally significant worsening.Clinical relevance: The current study highlights the wide clinical spectrum and relatively stable natural course of ISCH and reports on hitherto undescribed presentation as radiculopathy.