• Journal of Korean Neurosurgical Society
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• v.41 no.1
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• pp.57-60
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• 2007

The incidence of diastematomyelia associated with teratoma is extremely rare. We present a case of sacrococcygeal teratoma in a neonate with split spinal cord malformation[SSCM]. Magnetic resonance imaging[MRI] showed a heterogenous mass lesion with cyst in the sacrococcygeal region and multiple spinal anormalies [diastematomyelia, tethered cord, hydromyelia, and hemivertebrae]. The mature teratoma was confirmed on histopathological examination. In SSCMs, the potential for coexisting congenital anomalies at separate levels of the spinal cord must be considered in radiological investigations.

• Journal of Korean Neurosurgical Society
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• v.63 no.3
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• pp.338-341
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• 2020

Cases of syringomyelia associated with spinal dysraphism are distinct from those associated with hindbrain herniation or arachnoiditis in terms of the suspected pathogenetic mechanism. The symptoms of terminal syringomyelia are difficult to differentiate from the symptoms caused by spinal dysraphism. Nonetheless, syringomyelia has important clinical implications, as it is an important sign of cord tethering. The postoperative assessment of syringomyelia should be performed with caution.

• 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.

• Journal of Korean Neurosurgical Society
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• v.63 no.3
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• pp.358-365
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• 2020

To describe how to perform urological evaluation in children with tethered cord syndrome (TCS). Although a common manifestation of TCS is the development of neurogenic bladder in developing children, neurosurgeons often face difficulty in detecting urological problems in patients with TCS. From a urological perspective, diagnosis of TCS in developing children is further complicated due to the differentiation between neurogenic bladder dysfunctions and transient bladder dysfunctions owing to developmental problems. Due to the paucity of evidence regarding evaluation prior to and after untethering, I have shown the purpose and tools for evaluation in my own practice. This may be tailored to the types of neurogenic bladder, developmental status, and risks for deterioration. While the urodynamic study (UDS) is the gold standard test for understanding bladder function, it is not a panacea in revealing the nature of bladder dysfunction. In addition, clinicians should consider the influence of developmental processes on bladder function. Before untethering, UDS should reveal synergic urethral movement, which indicates an intact sacral reflex and lack of TCS. Postoperatively, the measurement of post-void residual urine volume is a key factor for the evaluation of spontaneous voiders. In case of elevation, fecal impaction, which is common in spinal dysraphism, should be addressed. In patients with clean intermittent catheterization, the frequency-volume chart should be monitored to assess the storage function of the bladder. Toilet training is an important sign of maturation, and its achievement should be monitored. Signs of bladder deterioration should be acknowledged, and follow-up schedule should be tailored to prevent upper urinary tract damage and also to determine an adequate timing for intervention. Neurosurgeons should be aware of urological problems related to TCS as well as urologists. Cooperation and regular discussion between the two disciplines could enhance the quality of patient care. Accumulation of experience will improve follow-up strategies.

• Journal of Acupuncture Research
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• v.37 no.4
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• pp.281-284
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• 2020

Filum terminale lipoma is an inherited lumbosacral abnormality that can cause tethered cord syndrome. This report describes an unusual case of lumbago and sciatica, pain suspected to be caused by a filum terminale lipoma where a herniated intervertebral disc had occurred. The patient was hospitalized for 43 days and received integrative Korean medicine treatment, including acupuncture, pharmacopuncture, Chuna therapy, cupping therapy, physiotherapy and herbal medicine. Treatment effectiveness was assessed using the numerical rating scale, Oswestry Disability Index, European Quality of Life 5-Dimensions, and patient symptoms. After inpatient treatment, the pain the patient suffered was significantly reduced, and the evaluation indices scores reflected this. Integrative Korean remedies may be an effective option for lower back pain and lower extremity symptoms which are caused by filum terminale lipoma where a herniated intervertebral disc has occurred. Additional clinical research is required to support this observation.

• 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.

• 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.

• Clinical and Experimental Pediatrics
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• v.61 no.6
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• pp.194-199
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• 2018

Purpose: Sacral dimples are a common cutaneous anomaly in infants. Spine ultrasonography (USG) is an effective and safe screening tool for patients with a sacral dimple. The aim of this study was to determine the clinical manifestations in patients with an isolated sacral dimple and to review the management of spinal cord abnormalities identified with USG. Methods: We reviewed clinical records and collected data on admissions for a sacral dimple from March 2014 through February 2017 that were evaluated with spine USG by a pediatric radiologist. During the same period, patients who were admitted for other complaints, but were found to have a sacral dimple were also included. Results: This study included 230 infants under 6-months-old (130 males and 100 females; mean age $52.8{\pm}42.6days$). Thirty-one infants with a sacral dimple had an echogenic filum terminale, and 57 children had a filar cyst. Twenty-seven patients had a low-lying spinal cord, and only one patient was suspected of having a tethered cord. Follow-up spine USG was performed in 28 patients, which showed normalization or insignificant change. Conclusion: In this study, all but one infant with a sacral dimple had benign imaging findings. USG can be recommended in infants with a sacral dimple for its convenience and safety.

• 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.

• 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.