Tethered cord syndrome in children with anorectal malformations

2000 ◽  
Vol 92 (4) ◽  
pp. 626-630 ◽  
Author(s):  
Natarajan Muthukumar ◽  
Bhuvaneswari Subramaniam ◽  
Thangaraj Gnanaseelan ◽  
Ramesh Rathinam ◽  
Appaswamy Thiruthavadoss

Object. Anorectal malformations are known to be associated with neurological deficits, which may contribute to the disability suffered by patients with these malformations. This study was undertaken to determine the incidence and pattern of sacral abnormalities in children with anorectal malformations, the incidence and nature of the neurological deficits, and the incidence and nature of operable intraspinal abnormalities in patients with this condition.Methods. Neurological evaluation was performed in 81 children with anorectal malformations. Plain x-ray films were obtained to identify the presence of sacral abnormalities. The patients with neurological deficits were evaluated for the presence of operable intraspinal anomalies, and when such anomalies were identified, correction of the same was undertaken. In 21% of these children radiographic evidence of sacral abnormalities was shown. Fifteen percent of patients harbored neurological deficits, and 10% harbored operable intraspinal anomalies. In addition, one patient had split notochord syndrome. Patients with operable intraspinal anomalies underwent surgical correction, with resultant neurological improvement.Conclusions. Bone abnormalities of the sacrum, neurological deficits, and operable intraspinal lesions are not uncommon in children with anorectal malformations. Because the neurological deficits can contribute to the disability suffered by these individuals, we recommend routine screening of patients with anorectal malformations and neurological deficits and/or sacral abnormalities for the early identification and treatment of potentially correctable intraspinal lesions.

1984 ◽  
Vol 61 (3) ◽  
pp. 531-538 ◽  
Author(s):  
Nicholas M. Barbaro ◽  
Charles B. Wilson ◽  
Philip H. Gutin ◽  
Michael S. B. Edwards

✓ The authors reviewed the clinical findings, radiological evaluation, and operative therapy of 39 patients with syringomyelia. Syringoperitoneal (SP) shunting was used in 15 patients and other procedures were used in 24 patients. Follow-up periods ranged from 1½ to 12 years. During the period of this study, metrizamide myelography in conjunction with early and delayed computerized tomography scanning replaced all other diagnostic procedures in patients with syringomyelia. Preoperative accuracy for the two procedures was 87%. The most common symptoms were weakness (79%), sensory loss (67%), pain (38%), and leg stiffness (28%). Surgery was most effective in stabilizing or alleviating pain (100%), sensory loss (81%), and weakness (74%); spasticity, headache, and bowel or bladder dysfunction were less likely to be reversed. Approximately 80% of patients with idiopathic and posttraumatic syringomyelia and 70% of those with arachnoiditis improved or stabilized. Better results were obtained in patients with less severe neurological deficits, suggesting the need for early operative intervention. A higher percentage of patients had neurological improvement with SP shunting than with any other procedure, especially when SP shunting was the first operation performed. Patients treated with SP shunts also had the highest complication rate, most often shunt malfunction. These results indicate that SP shunting is effective in reversing or arresting neurological deterioration in patients with syringomyelia.


1991 ◽  
Vol 75 (1) ◽  
pp. 40-44 ◽  
Author(s):  
Michael B. Sisti ◽  
Robert A. Solomon ◽  
Bennett M. Stein

✓ Surgical resection of 10 obscure arteriovenous malformations (AVM's) was accomplished with craniotomy guided by computerized tomography (CT) or angiography and the use of the Brown-Roberts-Wells stereotactic frame. Stereotactic craniotomy was invaluable for resection of the following types of AVM's: 1) AVM's with a nidus less than 2 cm in diameter, 2) AVM's located in an eloquent area of the brain, and 3) AVM's located deep in the brain. Stereotactic localization of these AVM's on preoperative radiological studies provides a precise route to the nidus, often avoiding important areas of the brain. This series included six male and four female patients with a mean age of 32 years. All patients presented with an intracerebral hemorrhage, from which eight made a complete neurological recovery prior to surgery. Two AVM's were located on the cortex, three were found subcortically, and five were situated near the ventricles or in the deep white matter. As a guide, angiography was used in six cases and CT in four cases. In each instance, the study providing the best image of the AVM nidus was employed. Postoperatively, no neurological deficits were found in eight patients and, in the two patients with preoperative deficits, neurological improvement was observed after recovery from surgery. Postoperative studies revealed complete removal of the AVM in all patients, and all lesions were confirmed histologically. The authors conclude that stereotactic craniotomy provides the optimum operative approach for the localization and microsurgical resection of AVM's that are either obscure or located deep in the brain.


1986 ◽  
Vol 65 (5) ◽  
pp. 608-610 ◽  
Author(s):  
Joong-Uhn Choi ◽  
Harold J. Hoffman ◽  
E. Bruce Hendrick ◽  
Robin P. Humphreys ◽  
William S. Keith

✓ Infarction of the spinal cord in childhood is rarely due to trauma. During a 15-year period (1971 to 1985), eight children were admitted to The Hospital for Sick Children, Toronto, with a diagnosis of traumatic infarction of the spinal cord. All of these patients had delayed onset of neurological signs varying between 2 hours and 4 days after their initial trauma. No bone abnormalities were seen on plain spine x-ray films. Myelography was carried out in seven of these children and found to be normal in all seven. Six patients who were paraplegic at the time of admission remained permanently paraplegic, but two with incomplete cord signs did show some improvement.


2001 ◽  
Vol 95 (2) ◽  
pp. 173-178 ◽  
Author(s):  
Sabine Hüttmann ◽  
Juergen Krauss ◽  
Hartmut Collmann ◽  
Niels Sörensen ◽  
Klaus Roosen

Object. The clinical features specific to tethered cord syndrome (TCS) in adults as well as factors determining outcome and prognosis have rarely been addressed systematically. The authors studied 56 patients, 54 of whom were treated surgically over the last 16 years. Methods. In 17 patients who had been asymptomatic during childhood, TCS was diagnosed 8 years after onset of symptoms. Tethered cord syndrome was diagnosed 4 years after worsening in 39 patients with neurological signs or symptoms since childhood. The patients were followed for an average of 8 years. Features specific to adult-age presentation included nondermatomal pain aggravated by movement in 34 patients and conditions such as pregnancy and childbirth (in five of 11 pregnant patients). The most frequent tethering lesions were lipoma in 32, tight terminal filum in 28, and split cord malformation and secondary adhesions in 12 patients, respectively. Improvement or stabilization of symptoms at 6 months after surgery was noted in 46 (85%) of 54 patients. Improvement in pain status was most frequent (86%) followed by improvements in spasticity (71%), bladder dysfunction (44%), and sensorimotor deficits (35%). Factors associated with adverse outcome included preoperative duration of neurological deficits more than 5 years and incomplete untethering. On average, 8 (80%) of 10 patients with incomplete untethering developed recurrent symptoms 5 years after surgery compared with only seven (16%) of 44 patients in whom complete untethering was achieved. Seven patients underwent reoperation and in five of them stabilization of symptoms was attained. At a mean follow up of 8 years, 46 (85%) of the 54 surgically treated patients were in stable neurological condition, including those in whom reoperation was performed. Conclusions. Surgery for TCS is as beneficial in adults as it is in children. Its success depends on early diagnosis and complete untethering of the spinal cord.


2005 ◽  
Vol 2 (2) ◽  
pp. 128-136 ◽  
Author(s):  
Rudolf Beisse ◽  
Thomas Mückley ◽  
Meic H. Schmidt ◽  
Matthias Hauschild ◽  
Volker Büren

Object. Decompression of the spinal canal in the management of thoracolumbar trauma is controversial, but many authors have advocated decompression in patients with severe canal compromise and neurological deficits. Anterior decompression, corpectomy, and fusion have been shown to be more reliable for spinal canal reconstruction than posterior procedures; however, traditional anterior-access procedures, thoracotomy, and thoracoabdominal approaches are associated with significant complications. Endoscopy-guided spinal access avoids causing these morbidities, but it has not been shown to yield equivalent results in spinal canal clearance. This study was conducted to demonstrate the effectiveness of endoscopic spinal canal decompression and reconstruction quantitatively by using pre- and postoperative computerized tomography (CT) scanning. Methods. Thirty patients with thoracolumbar canal compromise underwent endoscopic anterior spinal canal decompression, interbody reconstruction, and stabilization for fractures (27 cases), and tumor, infection, and severe degenerative disc disease (one case each). The mean follow-up period was 42 months (range 24 months–6 years). Neurological examinations, Frankel grades, radiological studies, and intraoperative findings were prospectively collected. Spinal canal clearance quantified on pre- and postoperative CT scans improved from 55 to 110%. A total of 25% of patients with complete paraplegia and 65% of those with incomplete neurological deficit improved neurologically. The complication rate was 16.7% and included one reintubation, two pleural effusions, one intercostal neuralgia, and one persistent lesion of the sympathetic chain. Conclusions. The authors describe the endoscopic technique of anterior spinal canal decompression in the thoracolumbar spine. The morbidities associated with an open procedure were avoided, and excellent spinal canal clearance was accomplished as was associated neurological improvement.


1970 ◽  
Vol 33 (6) ◽  
pp. 646-653 ◽  
Author(s):  
Warren P. Sights ◽  
Robert J. Bye

✓ An experimental study is presented of the histopathological reaction of the brain to shotgun pellets surgically implanted in cats. Hardened lead shot produced the least reaction, copper-coated pellets the most, and nickel-coated shot an intermediate reaction. Seven histological changes and neurological deficits were apparent in cats with implanted copper-coated pellets; a marked migration of copper-coated shot was revealed by x-ray and autopsy examination. Three clinical cases are presented to illustrate the clinical significance of the laboratory data.


2002 ◽  
Vol 97 (2) ◽  
pp. 218-222 ◽  
Author(s):  
Patrick W. Hitchon ◽  
Aaron M. From ◽  
Matthew D. Brenton ◽  
John A. Glaser ◽  
James C. Torner

Object. In this clinical review of ankylosing spondylitis, the authors emphasize the fragility of the spine and the degree of neurological deficit that can result from minor injury. The management of thoracolumbar fractures complicating ankylosing spondylitis is discussed. Methods. Six thoracic and five lumbar fractures were identified in a review of patients in whom ankylosing spondylitis had been diagnosed. Nine fractures were associated with an extension deformity. The mechanism of injury was regarded as major in three and minor in eight. The mean age in patients suffering fractures after minor traumatic injury was 59.6 ± 14.5 years (± standard deviation), whereas the mean age in patients suffering fractures after major traumatic injury was 49.3 ± 10.1 years. Two patients were treated with bedrest and brace therapy, and nine underwent instrument-assisted fusion. Postinjury neurological deficits were demonstrated in six patients, in three of whom neurological improvement was observed. Conclusions. Patients with ankylosing spondylitis are at risk of injury, particularly extension fractures of the thoracic and lumbar spine. The incidence of trauma-induced neurological deficit is by no means negligible. Management consists of surgery in patients with vertebral instability and neural compression, and recumbency in those with stable fractures.


1996 ◽  
Vol 84 (6) ◽  
pp. 962-971 ◽  
Author(s):  
Tohru Mizutani

✓ A long-term follow-up study (minimum duration 2 years) was made of 13 patients with tortuous dilated basilar arteries. Of these, five patients had symptoms related to the presence of such arteries. Symptoms present at a very early stage included vertebrobasilar insufficiency in two patients, brainstem infarction in two patients, and left hemifacial spasm in one patient. Initial magnetic resonance (MR) imaging in serial slices of basilar arteries obtained from the five symptomatic patients showed an intimal flap or a subadventitial hematoma, both of which are characteristic of a dissecting aneurysm. In contrast, the basilar arteries in the eight asymptomatic patients did not show particular findings and they remained clinically and radiologically silent during the follow-up period. All of the lesions in the five symptomatic patients gradually grew to fantastic sizes, with progressive deterioration of the related clinical symptoms. Dilation of the basilar artery was consistent with hemorrhage into the “pseudolumen” within the laminated thrombus, which was confirmed by MR imaging studies. Of the five symptomatic patients studied, two died of fatal subarachnoid hemorrhage (SAH) and two of brainstem compression; the fifth patient remains alive without neurological deficits. In the three patients who underwent autopsy, a definite macroscopic double lumen was observed in both the proximal and distal ends of the aneurysms within the layer of the thickening intima. Microscopically, multiple mural dissections, fragmentation of internal elastic lamina (IEL), and degeneration of media were diffusely observed in the remarkably extended wall of the aneurysms. The substantial mechanism of pathogenesis and enlargement in the symptomatic, highly tortuous dilated artery might initially be macroscopic dissection within a thickening intima and subsequent repetitive hemorrhaging within a laminated thrombus in the pseudolumen combined with microscopic multiple mural dissections on the basis of a weakened IEL. The authors note and caution that symptomatic, tortuous dilated basilar arteries cannot be overlooked because they include a group of malignant arteries that may grow rapidly, resulting in a fatal course.


1986 ◽  
Vol 65 (5) ◽  
pp. 706-709 ◽  
Author(s):  
Yoko Nakasu ◽  
Jyoji Handa ◽  
Kazuyoshi Watanabe

✓ Two patients with benign intracerebral cysts are reported and a brief review of the literature is given. Although computerized tomography (CT) scanning is useful in detecting a variety of intracerebral cysts, the CT findings are not specific for any lesion. An exploratory operation with establishment of an adequate route of drainage and a histological examination of the cyst wall are mandatory in the management of patients with a progressive but benign lesion.


1998 ◽  
Vol 88 (1) ◽  
pp. 57-65 ◽  
Author(s):  
Yusuf Ersşahin ◽  
Saffet Mutluer ◽  
Sevgül Kocaman ◽  
Eren Demirtasş

Object. The authors reviewed and analyzed information on 74 patients with split spinal cord malformations (SSCMs) treated between January 1, 1980 and December 31, 1996 at their institution with the aim of defining and classifying the malformations according to the method of Pang, et al. Methods. Computerized tomography myelography was superior to other radiological tools in defining the type of SSCM. There were 46 girls (62%) and 28 boys (38%) ranging in age from less than 1 day to 12 years (mean 33.08 months). The mean age (43.2 months) of the patients who exhibited neurological deficits and orthopedic deformities was significantly older than those (8.2 months) without deficits (p = 0.003). Fifty-two patients had a single Type I and 18 patients a single Type II SSCM; four patients had composite SSCMs. Sixty-two patients had at least one associated spinal lesion that could lead to spinal cord tethering. After surgery, the majority of the patients remained stable and clinical improvement was observed in 18 patients. Conclusions. The classification of SSCMs proposed by Pang, et al., will eliminate the current chaos in terminology. In all SSCMs, either a rigid or a fibrous septum was found to transfix the spinal cord. There was at least one unrelated lesion that caused tethering of the spinal cord in 85% of the patients. The risk of neurological deficits resulting from SSCMs increases with the age of the patient; therefore, all patients should be surgically treated when diagnosed, especially before the development of orthopedic and neurological manifestations.


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