• Title/Summary/Keyword: tethered cord syndrome

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Features of the Filum Terminale in Tethered Cord Syndrome with Focus on Pathology

  • Sim, Jungbo;Shim, Youngbo;Kim, Kyung Hyun;Kim, Seung-Ki;Lee, Ji Yeoun
    • Journal of Korean Neurosurgical Society
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    • v.64 no.4
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    • pp.585-591
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    • 2021
  • Objective : Filum transection is one of the most commonly performed operative procedure in pediatric neurosurgery. However, the clinical and pathological features as well as the surgical indication are not well-established. This study aimed to analyze the characteristics of patients who underwent transection of the filum during the last 10 years in a single institute. Methods : A total of 82 patients underwent transection of the filum during the period. As a general rule, we performed the transection in patients who are symptomatic or have abnormality in the urologic or neuromuscular evaluations. There were exceptions as asymptomatic patients who only fit the definition of thickened filum (width greater than 2.0 mm or conus level below L3 vertebral body) were operated by parent's wish or surgeon's preference according to radiological findings, etc. Results : Seventy-six out of 82 patients had fibrous tissue in the pathologic specimen of filum. Interestingly, patients who had glial cells were more correlated with no preoperative syrinx, and no progression of syrinx even for those who did have syrinx initially. Also, larger percentage of symptomatic patients had peripheral nerve twigs than asymptomatic patients. No difference in conus level or thickness of filum was found between patients with or without preoperative syrinx. Significantly more patients with syrinx (56%) were chosen to be operated without any symptom or abnormality in study i.e., solely based on radiological findings than those without syrinx (21%). The surgical outcome for syrinx was favorable, as all but one patient had either improved or static syrinx. The exceptional case had increase in size due to the upward displacement of the proximal end of the cut filum. Conclusion : This study evaluated the pathological, clinical, radiological features of patients who underwent transection of the filum. Interesting correlations between pathological findings and clinical features were found. Excellent outcome regarding preoperative syrinx was also shown.

Spinal Dysraphism in the Last Two Decades : What I Have Seen during the Era of Dynamic Advancement

  • Wang, Kyu-Chang
    • Journal of Korean Neurosurgical Society
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    • v.63 no.3
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    • pp.272-278
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    • 2020
  • Compared to any other decade, the last two decades have been the most dynamic period in terms of advances in the knowledge on spinal dysraphism. Among the several factors of rapid advancement, such as embryology during secondary neurulation and intraoperative neurophysiological monitoring, there is no doubt that Professor Dachling Pang stood high amidst the period. I review here the last two decades from my personal point of view on what has been achieved in the field of spinal dysraphism, focusing on occult tethered cord syndrome, lumbosacral lipomatous malformation, terminal myelocystocele, retained medullary cord, limited dorsal myeloschisis and junctional neural tube defect. There are still many issues to revise, add and extend. Profound knowledge of basic science is critical, as well as refined clinical analysis. I expect that young scholars who follow the footsteps of precedent giants will shed bright light on this topic in the future.

A case of congenital cloacal exstrophy/omphalocele-exstrophy-imperforate anus-spinal defects syndrome and a successful pregnancy

  • Seat, Mara;Boxwalla, Munira;Hough, Arielle;Goodwin, Glenn
    • Clinical and Experimental Reproductive Medicine
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    • v.49 no.3
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    • pp.215-218
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    • 2022
  • Herein, we report an exceptionally rare case of a 25-year-old woman with cloacal exstrophy/omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) syndrome achieving a viable pregnancy despite many gastrointestinal and genitourinary malformations and multiple respective corrective operations. The patient was born with two vaginas, two uteruses, four ovaries, an imperforate anus, a large omphalocele including bowel and bladder exstrophy, and diaphysis of the pubic rami. This patient is the only documented OEIS patient not to have tethered spinal cord as an anomaly, perhaps contributing to her successful pregnancy. After experiencing preeclampsia with severe features at 35 weeks, the baby was born via cesarean section.

Intraoperative Neurophysiology Monitoring for Spinal Dysraphism

  • Kim, Keewon
    • Journal of Korean Neurosurgical Society
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    • v.64 no.2
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    • pp.143-150
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    • 2021
  • Spinal dysraphism often causes neurological impairment from direct involvement of lesions or from cord tethering. The conus medullaris and lumbosacral roots are most vulnerable. Surgical intervention such as untethering surgery is indicated to minimize or prevent further neurological deficits. Because untethering surgery itself imposes risk of neural injury, intraoperative neurophysiological monitoring (IONM) is indicated to help surgeons to be guided during surgery and to improve functional outcome. Monitoring of electromyography (EMG), motor evoked potential, and bulbocavernosus reflex (BCR) is essential modalities in IONM for untethering. Sensory evoked potential can be also employed to further interpretation. In specific, free-running EMG and triggered EMG is of most utility to identify lumbosacral roots within the field of surgery and filum terminale or non-functioning cord can be also confirmed by absence of responses at higher intensity of stimulation. The sacral nervous system should be vigilantly monitored as pathophysiology of tethered cord syndrome affects the sacral function most and earliest. BCR monitoring can be readily applicable for sacral monitoring and has been shown to be useful for prediction of postoperative sacral dysfunction. Further research is guaranteed because current IONM methodology in spinal dysraphism is still deficient of quantitative and objective evaluation and fails to directly measure the sacral autonomic nervous system.

Imaging Diagnosis of Sacrocaudal Dysgenesis in a Shih-tzu Dog

  • Choi, Soo-Young;Lee, In;Cho, Na-Young;Shin, Bong-Hun;Lee, Ki-Ja;Choi, Ho-Jung;Lee, Young-Won
    • Journal of Veterinary Clinics
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    • v.33 no.6
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    • pp.389-391
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    • 2016
  • A 10-month-old, intact female Shih-tzu dog with a pelvic limb ataxic gait, and urinary and fecal incontinence since birth, was examined by computed tomography and magnetic resonance imaging. The butterfly hemivertebra of the fourth lumbar vertebra, agenesis of the third sacral and coccygeal vertebrae, and spina bifida of the remaining sacral vertebra were observed on the computed tomography images. Magnetic resonance imaging revealed traction of the conus medullaris. The dog was diagnosed with sacrocaudal dysgenesis with presumptive tethered cord syndrome, and concurrent infectious cystitis was found by ultrasonography and urinary analysis. The Shih-tzu recovered from the cystitis, but still has the ataxic gait and urinary and fecal incontinence. This report summarizes the case of a dog with sacrocaudal dysgenesis that was identified by imaging diagnosis.

Analysis of Risk Factors and Management of Cerebrospinal Fluid Morbidity in the Treatment of Spinal Dysraphism

  • Lee, Byung-Jou;Sohn, Moon-Jun;Han, Seong-Rok;Choi, Chan-Young;Lee, Dong-Joon;Kang, Jae Heon
    • Journal of Korean Neurosurgical Society
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    • v.54 no.3
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    • pp.225-231
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    • 2013
  • Objective : Spinal dysraphism defects span wide spectrum. Wound dehiscence is a common postoperative complication, and is a challenge in the current management of cerebrospinal fluid (CSF) leaks and wound healing. The purpose of this study is to evaluate the risks of CSF-related morbidity in the surgical treatment of spinal dysraphism. Methods : Ten patients with spinal dysraphism were included in this retrospective study. The median age of the cohort was 4.8 months. To assess the risk of CSF morbidity, we measured the skin lesion area and the percentage of the skin lesion area relative to the back surface for each patient. We then analyzed the relationship between morbidity and the measured skin lesion area or related factors. Results : The overall median skin lesion area was 36.2 $cm^2$ (n=10). The percentage of the skin lesion area relative to the back surface ranged from 0.6% to 18.1%. During surgical reconstruction, 4 patients required subsequent operations to repair CSF morbidity. The comparison of the mean area of skin lesions between the CSF morbidity group and the non-CSF morbidity group was statistically significant (average volume skin lesion of $64.4{\pm}32.5cm^2$ versus $27.7{\pm}27.8cm^2$, p<0.05). CSF morbidity tended to occur either when the skin lesion area was up to 44.2 $cm^2$ or there was preexisting fibrosis before revision with an accompanying broad-based dural defect. Conclusion : Measuring the lesion area, including the skin, dura, and related surgical parameters, offers useful information for predicting wound challenges and selecting appropriate reconstructive surgery methods.