Diastematomyelia presenting as progressive weakness in an adult after spinal fusion for adolescent idiopathic scoliosis.

BACKGROUND CONTEXT: Diastematomyelia is uncommon and rarely presents in adulthood. This report draws attention to the fact that patients who underwent spinal fusion for deformity before the widespread use of computed tomography (CT) and magnetic resonance imaging (MRI) may have unrecognized spinal cord abnormalities. This should be considered if revision surgery is contemplated. PURPOSE: This case report focuses on the late presentation of lower-extremity weakness in a 44-year-old woman with a split cord malformation (diplomyelia), diastematomyelia and tethered cord syndrome. STUDY DESIGN/SETTING: METHODS: The patient underwent instrumented posterior spinal fusion with a Harrington rod as a child for progressive thoracolumbar scoliosis. As an adult, she developed paraparesis after a traumatic event.The patient underwent decompressive laminectomy, subtotal resection of the old fusion mass and resection of the osseous septum. Postoperatively, an anterior spinal fluid leak in the lower thoracic region required repeated fascial grafting, resection of a pseudomeningocele and reverse left latissimus dorsi flap transfer. The leak was controlled, and the patient had near complete resolution of her paraparesis 1 year after her surgery. RESULTS: The case described herein is unusual in that patients with diplomyelia and diastematomyelia rarely are symptomatic in adulthood. However, trauma may precipitate the onset of neurologic symptoms. This patient underwent spinal surgeries to address deformity, pain and progressive lower-extremity weakness. Preoperative CT and MRI studies showed a split cord malformation and diastematomyelia at L1-L2 with spinal stenosis and tethering of both hemicords. CONCLUSIONS: Progressive weakness without any previous neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may develop in the adult with unrecognized diastemotomyelia. This case demonstrates that a thorough preoperative workup of patients with complex spinal deformities is imperative.     

Coexistence of spinal intramedullary teratoma and diastematomyelia in an adult

STUDY DESIGN: A case report of intramedullary spinal cord teratoma with remote diastematomyelia in a female adult. OBJECTIVE: To present a rare case of a dysembryogenic spinal tumor with concurrent split cord malformation and to define the importance of early surgical removal of the tumor. SETTING: A department of neurosurgery in Greece. METHODS: A 44-year old woman, presented with progressive lower limb muscle weakness, gait disturbances and dysesthesias in the trunk and lower extremity. She underwent plain radiographs, CT and MRI scan, which revealed an exophytic intramedullary spinal cord tumor at the level of T8-T10 and distant diastematomyelia in the upper lumbar spine (L2-L3). She underwent surgical intervention. The tumor was subtotally removed. No attempt was made to treat diastematomyelia. RESULTS: Postoperatively, the patient's neurological status started to improve gradually. After 1 year she exhibited better strength in the lower limb muscles and improved sensation. The histological examination demonstrated mature spinal teratoma consisting of ectodermal, mesodermal and endodermal elements. CONCLUSIONS: In adult patients with intramedullary masses of possible dysembryogenic origin, the whole spine must be examined for additional dysraphic lesions. The choice and the timing of a surgical intervention are strongly dependent on the clinical picture.     

Split cord malformation with diastematomyelia presenting as neurogenic claudication in an adult: a case report

STUDY DESIGN: This is a report of a rare presentation of a split cord malformation with diastometamyelia. OBJECTIVES: This report draws attention to the fact that the only manifestation of diastmetamyelia in the adult patient may be neurogenic claudication. SUMMARY OF BACKGROUND DATA: Patients with split cord malformations and diastometamyelia rarely have symptomatic onset in adulthood. When present, a traumatic event leading to an acute neurologic change is the usual presentation. METHODS: An adult patient presented with symptoms of neurogenic claudication in the left leg. Magnetic resonance imaging examination showed a split cord malformation and diastometamylia at L3-L4 with spinal stenosis of the left hemicord. Decompressive laminectomy and subtotal resection of the bony spur were performed. RESULTS: Two years after decompression, the patient has complete resolution of his leg symptoms and is back to work. CONCLUSIONS: Neurogenic claudication without any objective neurologic deficit or neurocutaneous stigmas of an underlying spinal cord abnormality may be the only presentation in the adult with diastometamyelia. Decompression to relieve both clinical and radiologic evidence of spinal stenosis obtained excellent outcome.     

Symptomatic Chiari malformations. An analysis of presentation, management, and long-term outcome

The Chiari malformation is a condition characterized by herniation of the posterior fossa contents below the level of the foramen magnum, and is categorized into three types based on the degree of herniation. The authors review their surgical experience between 1975 and 1985 with 50 patients afflicted with symptomatic Chiari malformations. Any patient with associated myelomeningocele, tethered spinal cord, lipoma, or diastematomyelia was excluded from this series. Forty-one patients had Chiari I malformations, seven were classified as having Chiari II, and two as having Chiari III. The presentation of pediatric and adult patients was identical. Treatment was directed at the posterior fossa pathology. Seven patients with accompanying ventral bone compression underwent transoral decompression of the cervicomedullary junction, 42 had posterior decompressive procedures, and six received ventriculoperitoneal shunts. The posterior decompression included opening the outlet foramina of the fourth ventricle, occluding any communication between the spinal cord central canal and the obex, shunting the fourth ventricle, and placing a dural graft. Postoperatively, 20% of the patients are asymptomatic, 66% improved, and 8% stabilized; in 6% the disease has progressed in spite of multiple procedures. Preoperative signs that are predictive of a less favorable outcome include muscle atrophy, symptoms lasting longer than 24 months, ataxia, nystagmus, trigeminal hypesthesia, and dorsal column dysfunction (p less than 0.05, chi-square test). A model based on the presence or absence of atrophy, ataxia, and scoliosis at the time of the preoperative examination has been generated that allows prediction of long-term outcome at the 95% confidence level.     

Lumbar teratoma presenting intradural and extramedullary extension in a neonate.

BACKGROUND CONTEXT: The association of teratomas and spinal malformations such as spina bifida, partial sacral agenesis, hemivertebrae, and diastematomyelia has been described in the literature. Reported cases, however, are mainly presacral or sacrococcygeal with an extremely rare presentation of intradural extension. PURPOSE: A case of lumbar teratoma with an intradural extension and extramedullary component and the clinical outcome following surgical treatment are reported. STUDY DESIGN/SETTING: To our knowledge, among the reported teratoma cases with an intradural extension and extramedullary component, our case has a distinguishing feature regarding the involvement of the lumbar spine. It is also the first case, showing no neurological deficit during the postoperative period. METHODS: A full-term, female infant presented with a 30 x 30 x 10 mm lumbar mass covered with normal skin. The mass contained an irregular, bone-like, hard and mobile material accompanying cystic components. Magnetic resonance imaging revealed a total closure defect of the first and second lumbar laminae and a subcutaneous mass with intradural extension. The lesion was found to penetrate the dural sac through an extended exposure from T12 to L3. There was no firm attachment of the intradural, extramedullary component of the lesion. Total removal of the tumor was achieved. RESULTS: The patient was discharged on day 7 without any neurological deficit or sign of hydrocephalus. The pathological examination showed a benign teratoma containing mature cartilage, muscle, adipose tissue, and glandular tissue. Follow-up at 2 years showed no recurrence or neurological deficit and a normal sphincter tone. Urodynamic evaluation was within normal limits. CONCLUSION: Accompanying a spinal dysraphic state, the mature teratoma in our case may support the idea of a tumor actually arising from a dysraphism and growing outward to produce the mass.     

Adult onset of tethered spinal cord syndrome due to fibrous diastematomyelia: case report

Adult onset of the symptoms of tethered spinal cord is a rare entity that is occasionally associated with diastematomyelia. Only one case of fibrous diastematomyelia in an adult has been reported. The fibrous nature of this disease may present a diagnostic difficulty. A 32-year-old man with the adult onset of impairment of sacral functions with lumbar fibrous diastematomyelia is reported. Surgical release of the spinal cord was followed by improvement of the patient's function.     

Adult onset tethered cord syndrome associated with intradural dermoid cyst. A case report

STUDY DESIGN: A case report and a review of literature. OBJECTIVES: To describe a rare case of adult onset tethered cord syndrome associated with intradural dermoid cyst. SETTING: General Orthopedics, Japan. METHODS: A 50-year-old woman was referred to us because of right leg pain and pollakiuria. Neurological examinations and radiological assessments including myelography, computerized tomography scan and Magnetic resonance image were carried out. We diagnosed it as the adult onset tethered cord syndrome associated with an intradural cystic lesion. RESULTS: The cystic lesion was totally removed following laminectomy from L5 through S4. Histologically, the tumor was diagnosed as a dermoid cyst. CONCLUSIONS: Intradural dermoid could produce adult onset tethered cord syndrome, but it was not reported in the English literatures to our knowledge.     

Cervical diastematomyelia presenting in adulthood. Case report

A case of cervical diastematomyelia in an adult is reported. The patient first noted sensory and motor symptoms at 34 years of age after two episodes of cervical trauma. Metrizamide computerized tomography myelography of the cervical spine and cord showed the region of diastematomyelia and revealed a spur containing both bone and fat tissue projecting into the spinal canal and cord. The spur arose from the laminae and spinous processes of C-2 and C-3, and was successfully excised. Postoperatively, the patient's deficits gradually improved. The literature concerning adult cervical diastematomyelia is reviewed.     

Spinal cord arteriovenous malformation presenting as chest pain in a child

STUDY DESIGN: A case report of spinal arteriovenous malformation presented as chest pain in a child. OBJECTIVES: To illustrate the atypical clinical presentation as right chest pain in a child with spinal cord arteriovenous malformation. SETTING: Chang Gung Memorial Hospital and Chang Gung University, Taiwan. CASE REPORT: An 8-year-old boy was diagnosed with spinal cord arteriovenous malformation after paraplegia. However, early diagnosis was delayed for the atypical initial clinical presentation as chest pain. After surgery and rehabilitation for 6 months, the patient could walk without assistance. CONCLUSIONS: Correct diagnosis of spinal cord arteriovenous malformations is sometimes difficult in children with atypical presentations. Early diagnosis relies on detailed examination and high index of suspicion.     

Computed tomographic metrizamide myelography in Chiari malformation in adult--special reference to the diagnostic values of reconstruction CT

Three adult patients with a clinical diagnosis of Chiari malformation were examined by computed tomography after intrathecal injection of metrizamide via the lumbar route. In all patients Chiari malformation was demonstrated as a soft tissue oval mass at the level of C1-C2 vertebral body that was intradural and posterior to the medulla and the upper cervical spinal cord. These findings were more accurately visualized in saggital and in frontal reconstructed CTs. In one patient the spinal cord was measurably enlarged at the upper thoracic level. A delayed scan was obtained in another case at 24 hours after the injection and the syrinx was remained opacified whereas the density of cerebrospinal fluid and parenchyma decreased. In the third patient no abnormality of the cord was demonstrated either in CT or myelography. High resolution CT, especially reconstruction-CT appears to be more reliable than myelography and should be the examination of choice in the diagnosis of Chiari malformation.     

Metastatic hemangiopericytoma to the cauda equina: a case report.

BACKGROUND CONTEXT: Hemangiopericytoma is an aggressive tumor associated with high recurrence and metastasis. Metastases are usually delayed, long after diagnosis of the primary lesion. Metastatic hemangiopericytoma to the spinal cord is especially rare. PURPOSE: To report a rare clinical presentation of a metastatic intradural, intramedullary hemangiopericytoma to the cauda equina from a cerebellar hemangiopericytoma. STUDY DESIGN: Case report with a review of the literature. METHODS: Clinical history, physical findings, and magnetic resonance imaging studies of a patient with a metastatic intradural, intramedullary hemangiopericytoma to the cauda equina are reported. RESULTS: A case report is presented of a female with an intradural, intramedullary lesion at the L4-S1 level, presenting initially with progressive pain and motor weakness affecting the left lower extremity. She had a history of a cerebellar hemangiopericytoma, which had been treated with total resection and radiotherapy 4 years earlier. This patient developed urinary urgency and frequency. Pathological analysis revealed a hemangiopericytoma, which had a similar character to a cerebellar lesion. After radiotherapy, the tumor was mostly diminished and her symptoms totally resolved. CONCLUSIONS: Hemangiopericytomas have a strong tendency to both local recurrence and metastasis. Common metastatic sites are the skeletal system, lung, liver, and abdominal cavity. To the authors' knowledge, there have been no reports of spinal intradural, intramedullary metastasis of hemangiopericytoma.     

"特发性"胸椎左侧凸患者并发脊髓病变及其临床意义

目的 探讨对临床上无明显神经损害的"特发性"胸椎左侧凸患者存在的脊髓病变情况,并分析其临床意义.方法 1997年10月至2003年10月共诊治"特发性"胸椎左侧凸59例,其中男性31例,女性28例;年龄7～44岁,平均15岁;Cobb角15°～108°,平均56°.所有病例经临床及影像学检查,排除神经纤维瘤及先天性脊椎发育异常等病因.对临床诊断为"特发性"胸椎左侧凸的患者行全脊髓磁共振成像(MRI)检查,对脊髓病变的发生率进行统计.结果 59例"特发性"胸椎左侧凸患者经全脊髓MRI检查后,共有33例发现髓内病变,髓内病变发生率为56%,其中Chiari畸形合并脊髓空洞24例,脊髓空洞5例,Chiari畸形、脊髓空洞、脊髓裂1例,Chiari畸形、脊髓空洞、脊髓栓系1例,脊髓空洞合并栓系1例,Dandy-Walker畸形1例.其余26例未发现脊髓病变.分析伴有脊髓病变的胸椎左侧凸患者中男性比例及平均Cobb角均显著大于无脊髓病变的胸椎左侧凸(P＜0.05).结论 对于胸椎左侧凸,尤其是男性、Cobb角较大的患者需高度怀疑伴有脊髓病变.对于此类患者术前常规全脊髓MRI检查具有重要意义.     

Diastematomyelia of the cervical spinal cord with tethering in an adult

A case of cervical diastematomyelia associated with spinal cord tethering in an adult is presented. The differences between cervicodorsal and lumbar region spinal dysraphic states are emphasized with a review of the embryological factors involved. Possible causes and subsequent treatment are outlined.     

Intramedullary neurenteric cysts of the spine. Report of three cases

Neurenteric spinal cysts are infrequently occurring dysraphic lesions that are caused by persistent or abnormal communication among neuroectoderm, notochord, and endoderm. They are generally located at the intradural, extramedullary compartment of the low cervical or upper spinal canal. They occur primarily in infants and in young adults in combination with other congenital abnormalities. The authors report on three cases of lower-thoracic and thoracolumbar intramedullary neurenteric cysts that atypically presented in adult patients in whom there was no concomitant malformation. These lesions appear notable for location, clinical presentation, intraoperative findings, and imaging features.     

Intradural tumor and concomitant disc herniation of cervical spine

We report a rare patient of a simultaneous extradural and intradural compression of the cervical spinal cord due to co-existent intervertebral disc herniation and an intradural schwannoma at the same level. The intradural lesion was missed resulting in recurrence of myelopathy after a surprisingly complete functional recovery following anterior cervical discectomy. Retrospectively, it was noted that the initial cord swelling noticed was tumor being masked by the compression produced by the herniated disc. A contrast magnetic resonance imaging scan is important in differentiating intradural tumors of the spinal cord. A high index of suspicion is often successful in unmasking both the pathologies.     

Intradural arachnoid cyst associated with thoracic spinal compression fracture: 7-year follow up after surgery

STUDY DESIGN: A case report with long-term follow after a surgical procedure. OBJECTIVES: To describe a case of intradural arachnoid cyst secondary to a compression fracture in the thoracic spine and to report long-term results after surgical treatment with hemilaminectomy. SETTING: Osaka, Japan. METHODS: A 68-year-old man who had a traumatic intradural arachnoid cyst following an adjacent compression fracture of T5 underwent surgery. Intraoperatively, after recognition of intradural arachnoid cyst with an echogram following hemilaminectomy, the dural sac was incised and the arachnoid cyst was resected under microscopic observation. RESULTS: At 7 years after the operation, the low intensity within the vertebral body of the compression fracture had resolved and the spinal cord remained in its normal shape and position. No progression of kyphotic deformity was detected. CONCLUSION: A compression fracture of the thoracic spine can be associated with an intradural arachnoid cyst. Microscopic resection via hemilaminectomy for the cyst showed a good result in a 7-year follow up.     

Neurogenic disorders of bladder emptying in closed spinal dysraphism]

Closed (occult) spinal dysraphism, e.g. lipomyelomeningocele, intraspinal lipoma, diastematomyelia, the tethered spinal cord in its various forms and dysgenesis of the sacrum, is often diagnosed late and only symptoms of neurogenic bladder dysfunction are present. A lipomyelomeningocele mostly causes detrusor and sphincter dysfunction, as was the case in five of six children among our patients. However, improvement of neurological and urological symptoms after the operation can only be achieved in about 40%. Four of eight children with diastematomyelia suffered from neurogenic bladder dysfunction; three have meanwhile undergone surgery with complete recovery in one, no relevant change in the second, and worsening in the third. Originally a specific term, the "tethered spinal cord" when associated with spinal dysraphism has taken on a more general meaning. Nowadays this term is not only used for a short, thickened and tight filum terminale, but comprises any pathology, which prevents the spinal cord from ascending. MRI examination of the craniovertebral junction and spinal cord of patients with treated myelomeningocele often reveals secondary pathologic changes: these may be areas of cord atrophy, hydromyelic cavitation or ventral compression from arachnoid cysts with clinical symptoms mostly after the age of 5 years. In these children a changing urodynamic pattern may therefore be caused by such a pathology and is an indication for a thorough neurological examination including MRI. Of all the dysrhaphic states mentioned above, sacral dysgenesis is the most frequent. The sacral osteological anomaly, as a numerical and as a structural anomaly, also determines the neuro-urological deficit.(ABSTRACT TRUNCATED AT 250 WORDS)     

Infantile spinal cord tumor: diagnostic difficulties. A case report

We report an infant with a thoracic intradural extramedullary tumor to illustrate the presentation of an infantile spinal cord tumor and the difficulty in establishing a diagnosis. An infant presented with edema of the legs, motor loss in the lower extremities, and anuria at 3 months of age. Magnetic resonance imaging of the lumbar region did not reveal any abnormalities. At 1 year of age, the patient underwent surgery to correct urinary incontinence of unknown etiology. The patient developed gait disturbance and had abdominal pain at 17 months of age. Magnetic resonance imaging of the thoracic spine revealed an intradural extramedullary tumor at T2-T5. The spinal cord tumor was completely resected and pathologic findings indicated an endodermal cyst. All symptoms improved after the operation. Infants who present with weakness of the lower legs, urinary incontinence and unknown abdominal pain should be evaluated for a spinal cord tumor.     

Extramedullary Hemangioblastoma of the Conus Medullaris

Summary ? Background. We report the case of an extramedullary pathologically proven hemangioblastoma of the conus medullaris. As spinal dural arteriovenous fistulas most commonly present with a conus medullaris syndrome, our presentation of the MRI, myelographic, and angiographic findings of this unique lesion may be useful in differentiating these two entities.  Clinical Material. We report the case of a 57 year old woman with a two year history of progressive low back and right lower extremity pain and weakness. Spinal MRI and myelography demonstrated serpiginous vasculature on the dorsum of the spinal cord consistent with either a vascular tumor or malformation. Selective spinal angiography was thus undertaken by the neuroendovascular team which revealed a tumor nodule consistent with vascular tumor. T12-L1 laminectomy was performed and a 6 mm vascularized tumor was found in the intradural extramedullary compartment adjacent to the conus medullaris. The tumor was completely removed and pathological analysis was consistent with hemangioblastoma.  Conclusion. This report documents a unique location for extramedullary spinal hemangioblastomas. Although both MRI and myelography are helpful in studying these lesions, angiography remains the gold standard in differentiating between vascular tumor and malformation. We suggest that the angiography be performed by a neurointerventional team to facilitate embolization, should this be warranted.     

The anterior approach to anterior cervical meningiomas: review illustrated by a case

Summary Anterior cervical meningiomas have traditionally been operated on by a posterior approach. However, several reports of an anterior approach to anterior cervical meningiomas and other anterior cervical intradural lesions have documented important advantages: large bony window of access, extradural coagulation of anterior blood supply to meningiomas, visualization of the intradural pathology in front of the spinal cord, and absence of manipulation of the spinal cord during resection of the lesion.In this study, the literature about anterior approaches to anterior cervical meningiomas is systematically reviewed and the advantages and disadvantages of the technique are discussed. Furthermore, a case of complete resection of a large anterior cervical intradural extramedullary meningioma from C5–7 by a three-level corpectomy with progressive postoperative neurological recovery is illustrated, confirming the advantages of the anterior approach.