Thoracic diastematomyelia with concurrent intradural epidermoid spinal cord tumor and cervical syrinx in an adult

2002 ◽  
Vol 97 (2) ◽  
pp. 231-234 ◽  
Author(s):  
Jason P. Sheehan ◽  
Jonas M. Sheehan ◽  
M. Beatriz Lopes ◽  
John A. Jane

✓ Diastematomyelia is a rare entity in which some portion of the spinal cord is split into two by a midline septum. Most cases occur in childhood, but some develop in adulthood. A variety of concurrent spinal anomalies may be found in patients with diastematomyelia. The authors describe a 38-year-old right-handed woman who presented with a 7-month history of lower-extremity pain and weakness on the right side. She denied recent trauma or illness. Sensorimotor deficits, hyperreflexia, and a positive Babinski reflex in the right lower extremity were demonstrated on examination. Neuroimaging revealed diastematomyelia extending from T-1 to T-3, an expanded right hemicord from T-2 to T-4, and a C6–7 syrinx. The patient underwent T1–3 total laminectomies, resection of the septum, untethering of the cord, and excision of the hemicord lesion. The hemicord mass was determined to be an intramedullary epidermoid cyst; on microscopic evaluation the diastematomyelia cleft was shown to contain fibroadipose connective tissue with nerve twigs and ganglion cells. Postoperatively, the right lower-extremity pain, weakness, and sensory deficits improved. Diastematomyelia can present after a long, relatively asymptomatic period and should be kept in the differential diagnosis for radiculopathy, myelopathy, tethered cord syndrome, or cauda equina syndrome. Numerous spinal lesions can be found in conjunction with diastematomyelia. To the authors' knowledge, this is the first case in which a thoracic epidermoid cyst and cervical syrinx occurred concurrently with an upper thoracic diastematomyelia. Thorough neuraxis radiographic evaluation and surgical treatment are usually indicated.

1991 ◽  
Vol 75 (3) ◽  
pp. 402-407 ◽  
Author(s):  
Krishna Kumar ◽  
Rahul Nath ◽  
Gordon M. Wyant

✓ Epidural spinal cord stimulation by means of chronically implanted electrodes was carried out on 121 patients with pain of varied benign organic etiology. In 116 patients, the pain was confined to the back and lower extremities and, of these, 56 exhibited the failed-back syndrome. Most patients were referred by a pain management service because of failure of conventional pain treatment modalities. Electrodes were implanted at varying sites, dictated by the location of pain. A total of 140 epidural implants were used: 76 unipolar, 46 Resume electrodes, 12 bipolar, and six quadripolar. Patients were followed for periods ranging from 6 months to 10 years, with a mean follow-up period of 40 months. Forty-eight patients (40%) were able to control their pain by neurostimulation alone. A further 14 patients (12%), in addition to following a regular stimulation program, needed occasional analgesic supplements to achieve 50% or more relief of the prestimulation pain. Pain secondary to arachnoiditis or perineural fibrosis following multiple intervertebral disc operations, when predominantly confined to one lower extremity, seemed to respond favorably to this treatment. Uniformly good results were also obtained in lower-extremity pain secondary to multiple sclerosis. Pain due to advanced peripheral vascular disease of the lower limbs was well controlled, and amputation below the knee was delayed for up to 2 years in some patients. Pain due to cauda equina injury, paraplegic pain, phantom-limb pain, pure midline back pain without radiculopathy, or pain due to primary bone or joint disease seemed to respond less well. Patients who responded to preliminary transcutaneous electrical nerve stimulation generally did well with electrode implants. Notable complications included wound infection, electrode displacement or fracturing, and fibrosis at the stimulating tip of the electrode. Three patients in this series died due to unrelated causes. Epidural spinal cord stimulation has proven to be an effective and safe means of controlling pain on a long-term basis in selected groups of patients. The mechanism of action of stimulation-produced analgesia remains unclear; further studies to elucidate it might allow spinal cord stimulation to be exploited more effectively in disorders that are currently refractory to this treatment modality.


1970 ◽  
Vol 33 (6) ◽  
pp. 676-681 ◽  
Author(s):  
Ian C. Bailey

✓ This is an analysis of 10 cases of dermoid tumor occurring in the spinal canal (8 lumbar and 2 thoracic). Low-back pain was the commonest presenting symptom, especially if the tumor was adherent to the conus medullaris. Other complaints included urinary dysfunction and motor and sensory disturbances of the legs. Clinical and radiological evidence of spina bifida was found in about half of the cases and suggested the diagnosis of a developmental type of tumor when patients presented with progressive spinal cord compression. At operation, the tumors were often found embedded in the conus medullaris or firmly adherent to the cauda equina, thus precluding complete removal. Evacuation of the cystic contents, however, gave lasting relief of the low-back pain and did not cause any deterioration in neurological function. In a follow-up study, ranging from 1 to 15 years, virtually no improvement in the neurological signs was observed. On the other hand, only one case has deteriorated due to recurrence of tumor growth.


1982 ◽  
Vol 57 (1) ◽  
pp. 48-56 ◽  
Author(s):  
Bjørn Magnaes

✓ When an intraspinal expanding lesion causes a spinal block, a segment of the spinal cord or cauda equina will be subjected to general pressure from the surrounding tissue. This spinal block pressure, the spinal equivalent to intracranial pressure, was measured by lumbar infusion of fluid and simultaneous recording of the volume-pressure curve caudal to the block. The point of deviation from or breakthrough of the exponential volume-pressure curve indicated the spinal block pressure. Spinal block pressure of about 500 mm H2O and more could be determined by this method, and, when it was combined with Queckenstedt's test, lower pressures could be assessed as well. In the static (thoracic) part of the spine, spinal block pressure up to the level of arterial blood pressure was recorded. In the dynamic part of the spine, however, spinal block pressure could exceed arterial blood pressure due to external compressive forces during extension of the spine. There was a general tendency for more severe neurological deficits in patients with high spinal block pressure; but the duration of the pressure, additional focal pressure, and spinal cord compared with nerve root compression seemed equally important factors. The recording has implications for diagnosis, positioning of patients for myelography and surgery, selection of high-risk patients for the most appropriate surgical procedure, and detection of postoperative hematoma. There were no complications associated with the recordings.


1976 ◽  
Vol 44 (6) ◽  
pp. 744-747 ◽  
Author(s):  
Eric T. Yuhl ◽  
John R. Bentson

✓ A case of ependymoma of the conus medullaris and cauda equina is described in which spinal angiography demonstrated rapid arteriovenous shunting, an angiographic sign which is typical of arteriovenous malformations and which has not been previously reported to occur with ependymomas.


1976 ◽  
Vol 44 (5) ◽  
pp. 613-616 ◽  
Author(s):  
Glen S. Merry ◽  
D. Barry Appleton

✓ A case is reported of spinal aneurysm in a child with a family history of hereditary hemorrhagic telangiectasia causing spinal cord and cauda equina compression. The operative approach is discussed.


1974 ◽  
Vol 40 (2) ◽  
pp. 252-254 ◽  
Author(s):  
John B. Runnels ◽  
John W. Hanbery

✓ A rare case of spontaneous subarachnoid hemorrhage from an extramedullary upper thoracic astrocytoma is described. The differential diagnosis between cranial and spinal spontaneous subarachnoid hemorrhage is discussed.


1973 ◽  
Vol 39 (5) ◽  
pp. 662-665 ◽  
Author(s):  
Nettleton S. Payne ◽  
Joseph V. McDonald

✓ The rupture of an ependymoma of the cauda equina associated with trauma and subarachnoid hemorrhage is described. The clinical course of the patient is discussed, and the mechanism and significance of the rupture postulated.


1991 ◽  
Vol 74 (5) ◽  
pp. 720-728 ◽  
Author(s):  
Stephen J. Whitaker ◽  
Eric M. Bessell ◽  
Sue E. Ashley ◽  
H. J. G. Bloom ◽  
B. Anthony Bell ◽  
...  

✓ Fifty-eight patients with histologically verified spinal cord ependymomas were treated at the Royal Marsden Hospital and Atkinson Morley's Hospital between 1950 and 1987. The median age in this series was 40 years (range 1 to 79 years) and the male:female ratio was 1.8:1. Ten patients had tumors in the cervical cord and 10 in the thoracic cord; 14 tumors involved the conus medullaris and 24 the cauda equina. Forty ependymomas were grade 1 and 13 were grades II to IV (in five patients there was insufficient material for grading). Eleven patients underwent biopsy only, 33 had partial or subtotal resection, and 14 had complete resection. Forty-three patients received postoperative radiotherapy. The median follow-up period was 70 months (range 3 to 408 months). Cause-specific survival rates were 74% and 68% at 5 and 10 years, respectively. On univariate analysis, age, histological grade, postoperative neurological function, and era of treatment were significant prognostic factors for survival. The histological grade was the only significant independent prognostic factor. The relative risk of death from ependymoma was 9.0 for patients with tumor grades II to IV compared to grade I (p < 0.005, 95% confidence interval 2.7 to 30). The survival rates of patients following complete excision were significantly better compared to those after incomplete surgery (p < 0.025). The majority of completely resected neoplasms were low-grade cauda equina tumors. Despite incomplete surgery. 5- and 10-year progression-free survival rates following radical radiotherapy were both 59%, and cause-specific survival rates were 69% at 5 years and 62% at 10 years. This suggests that radiotherapy may achieve long-term tumor control in over half of those patients with residual spinal ependymoma.


1983 ◽  
Vol 58 (6) ◽  
pp. 954-958 ◽  
Author(s):  
Román Garza-Mercado

✓ Diastematomyelia is rarely diagnosed in the adult. Only a few such cases have been reported in the literature. A 26-year-old Mexican man with lumbar diastematomyelia is reported who also harbored a T-12 intramedullary epidermoid tumor and an extradural teratoma located in the dorsal aspect of the dural sac opposite L-4. These three rare coincidental lesions were removed at surgery. The patient's condition improved.


1996 ◽  
Vol 85 (6) ◽  
pp. 1036-1043 ◽  
Author(s):  
Shlomo Constantini ◽  
John Houten ◽  
Douglas C. Miller ◽  
Diana Freed ◽  
Memet M. Ozek ◽  
...  

✓ Over a 13-year period extending from 1980 to 1993, 27 children less than 3 years of age underwent operation for removal of an intramedullary spinal cord tumor (IMSCT). The majority (18 of 27) of children had undergone surgery before being referred to New York University (NYU) Medical Center. The most common reasons for radiological investigation were pain (42%), motor regression (36%), gait abnormalities (27%), torticollis (27%), and progressive kyphoscoliosis (24%). Forty procedures were performed in 27 children. Nine children underwent two operations and two children underwent three procedures. A gross-total resection was achieved in 72% of the procedures. There was no surgical mortality. A comparison of the preoperative and 3-month postoperative functional grades for the first NYU procedure (NYU-1) yielded the following findings: 20 patients' conditions remained the same, five patients improved, and two patients deteriorated. The functional outcomes of a second operation (NYU-2) were similar. The majority of the children (24 of 27, 89%) had histologically determined low-grade lesions. There were 12 patients with low-grade astrocytomas (Grades I-III), eight with gangliogliomas, two with ganglioglioneurocytomas, one with a glioneurofibroma, and one child with a mixed astro/oligodendroglioma. Two children had anaplastic astrocytomas (Grades II–III) and one child had a glioblastoma multiforme. In a median follow-up review of 76 months, two patients died and two patients were lost to follow up. The 3- and 5-year progression-free survival (PFS) rates were 81.7% (standard error of the mean (SEM) 0.083) and 76.2% (SEM 0.094), respectively. Eight of 24 patients suffered a recurrence within a mean time of 45.4 ± 28.9 months. All were treated with surgery (NYU-2). Lesions recurred in three of 12 children with low-grade astrocytomas, two of eight children with gangliogliomas, one child with an anaplastic astrocytoma, one child with a ganglioglioneurocytoma, and one child with a glioblastoma multiforme. At follow-up review, most of these children were doing well. Sixteen are in functional Grades I or II and 18 children attend a normal school system. The authors conclude that surgery for the removal of IMSCTs in children less than 3 years of age can be performed radically and safely. The postoperative functional performance is determined by the degree of the preoperative deficit. It is, therefore, of utmost importance to diagnose and treat these children as early as possible. Spinal cord tumors should be recognized as potentially excisable lesions on their initial presentation and when they recur. The optimum treatment for malignant lesions is still to be determined.


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