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: 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.     

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.     

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 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.     

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.     

Idiopathic spinal cord herniation in the thoracic spine as a cause of intractable leg pain: case report and review of the literature

We recently experienced a case of idiopathic spinal cord herniation in the upper thoracic spine with development of severe unilateral leg pain: a 57-year-old man who had suffered from severe leg pain for about 15 years with lack of abnormality in his lumbar spine. On MRI of the thoracic spine, however, the spinal cord at T2-T3 was bent forward with dilatation of the posterior subarachnoid space. In combination with the findings of computed tomographic myelography, we diagnosed idiopathic spinal cord herniation and performed a surgical treatment. Immediately after the operation, his leg pain disappeared. Therefore, we concluded that the cause of his leg pain was herniation of the thoracic spinal cord.     

Case of idiopathic thoracic spinal cord herniation with a chronic history: a case report and review of the literature

Idiopathic spinal cord herniation is a rare disease that presents with slowly progressive myelopathy. This article describes the clinical findings of a patient with a chronic history. A 68-year-old woman initially presented at the age of 32 years with left leg weakness. After slowly progressive neurological deterioration over 34 years, she became completely paraplegic. At the age of 66 years, magnetic resonance imaging resulted in a diagnosis of idiopathic spinal cord herniation at the T6/7 level. Surgery was performed to reduce aching of the lower extremities. The spinal cord was released from the dural defect through a T5–T8 laminectomy. The dural defect was enlarged by resecting its periphery to prevent reherniation of the spinal cord. After the surgery, pain in the lower extremities resolved and her motor function slightly improved. Although operative treatment is naturally recommended at the early stage of this disease, our case suggests that some symptoms can be resolved by surgical treatment despite prolonged, severe preoperative symptoms.     

Dorsal thoracic spinal cord herniation: report of an unusual case and review of the literature

Background context

Spinal cord herniation is a rare but well-documented condition that has been associated with tethering through the dural defect. Both spinal cord herniation and cord tethering result in progressive myelopathy that can be improved or stabilized with surgical intervention. Most cases of herniation are caused by dural defects in the ventral or ventrolateral thoracic spine, rarely occurring through the dorsal dura. This is the first reported case of a spontaneous dorsal herniation.

Purpose

To describe a unique case of thoracic tethered cord resulting from a dorsal dural defect through which there is spinal cord herniation.

Study design

A case report and review of the literature.

Methods

A 55-year-old man presented with progressive low back pain, paresthesias, and weakness in his left lower extremity that was exacerbated by walking. Imaging revealed a dorsal dural defect with tethering and herniation of the spinal cord at T7.

Results

The patient underwent a T6–T7 laminoplasty to release the tethered cord and repair the dural defect. At 1-year follow-up, the patient noted improvement in strength and back spasticity.

Conclusions

Spinal cord herniation through a dural defect is an uncommon but important cause of symptomatic tethered cord in adults. Surgical intervention can significantly alter the course and prevent further disability.     

Idiopathic hypertrophic spinal pachymeningitis: a case report and review of literature

Purpose

To report an unusual case of idiopathic hypertrophic spinal pachymeningitis (IHSP) with a review of relevant literature and to discuss the etiology, clinical features, imaging, treatment and prognosis of IHSP.

Methods

The case of a 44-year-old woman is reported. MEDLINE was used to search relevant literatures written in English since 2004.

Results

The patient suffered from progressive mild thoracic backache followed by truncal and lower extremity weakness, numbness and urinary retention. The diagnosis was confirmed by magnetic resonance (MR) imaging and histopathologic examination. Although she received corticosteroid therapy and decompressive surgery, the patient suffered a rapid relapse probably because of the withdrawal of postoperative steroid therapy.

Conclusions

IHSP is a rare disease characterized by inflammatory hypertrophy of the dura mater without identifiable cause and featured clinical progress of radiculalgia to myelopathy. It is a diagnosis of exclusion. In our view, surgical decompression with postoperative steroid therapy may be optimal. Furthermore,we speculated that increased levels of protein and cell count in cerebrospinal fluid (CSF) might be positively related to the disease progression. High inflammatory signs or CSF protein and cell levels before surgery or postoperative residual lesions are possible reasons of poor prognosis in patients with IHSP.

     

Intramedullary spinal cord gangliocytoma: case report and a review of the literature

We present a case of intramedullary spinal gangliocytoma in a 19-year-old woman who presented with a long history of hyperhidrosis of the face and neck areas and intermittent pain of the hands. The tumor involved the cervico-thoracic region of the spinal cord from C7-T3. The patient underwent debulking of the lesion and remains neurologically stable at two years follow-up. A discussion of this case is followed by a review of the literature surrounding this rare clinicopathological entity.     

Traumatic handlebar hernia associated with hepatic herniation: a case report and review of the literature

A traumatic abdominal wall hernia (TAWH) is a rare type of hernia that occurs after blunt trauma to the abdomen. TAWH caused by direct trauma from bicycle handlebars is even more rare with fewer than 30 cases having being reported. Recognition of these hernias is important, because they may be associated with significant intrabdominal injuries. Despite an overall increase in incidence of blunt abdominal trauma, cases of TAWH remain rare, probably because of elasticity of the abdominal wall resists the shear forces generated by a traumatic impact. A high level of clinical suspicion is required for diagnosis of TAWH in patients with handlebar injuries. We present the case of a 20-year-old man with a traumatic handlebar hernia associated with herniation of the liver and hepatic ductal injury, which was managed successfully by a delayed repair of the hernia.     

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.     

Ganglioneurocytoma of the spinal cord: report of a case and review of literature

A case of ganglioneurocytoma of the spinal cord in a 51-year-old man is reported. Patient presented with a progressively worsening back pain and hyposthenia of lower limbs. Magnetic resonance imaging of the spinal cord revealed an intradural lesion in the T10–11 spinal segments, hyperintense on T1-weighted sequences. Complete resection was achieved. The patient did not show evidence of recurrence 12 months after surgery. On histology, the lesion was composed of round to oval cells with fine granular, slightly eosinophilic to clear cytoplasm suggesting a neurocytic differentiation. Some ganglion cells were observed. Neither significant cytologic atypia nor mitoses were present. The neoplastic cells were strongly positive with synaptophysin and negative with glial fibrillary acid protein. Neurocytomas are uncommon tumors typically arising within the lateral ventricles near the foramen of Monro. Extraventricular neurocytomas have been reported at various sites with rare examples in spinal cord. In the present paper, a case of ganglioneurocytoma of the spinal cord is reported with review of literature and discussion of differences compared to neurocytomas of other sites.     

Thoracic fracture-dislocations without spinal cord injury: a case report and literature review

Summary Severe fracture-dislocation of the thoracic spine without neurological deficit is rare. Both translational and rotational deformity of the midthoracic spine makes transection of the cord almost inevitable due to the confined dimensions of the cord and spinal canal. Even though associated fractures of posterior elements are frequently seen, they seldom result in neural sparing. The case of a 24-year-old man who sustained a severe rotational fracture-dislocation of T9/T10 with considerable anterolateral displacement is reported. Due to a fractured left pedicle and a right-sided vertical fracture through the posterior aspect of the vertebral body, alignment of the posterior elements in the spinal canal was maintained and there was no neurological deficit. The patient was operatively treated with posterior segmental instrumentation, and was completely asymptomatic at follow-up 5 years later.