• Journal of Korean Neurosurgical Society
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• v.42 no.1
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• pp.42-45
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• 2007

Spinal cord lipomas are commonly associated with spinal dysraphism. The lipomas without spinal dysraphism have been reported to be only 1% of all spinal cord lipomas. We report two cases of patients with spinal intramedullary lipomas without dysraphism. One patient was a 57-year-old man who had lower back pain and right-sided sciatica for 20 years. Magnetic resonance imaging (MRI) of the spine demonstrated an intradural mass occupying L1 to L2. The other patient was a 27-year-old woman who had back pain and gait disturbance for 5 months. MRI of the spine showed an elongated intradural mass occupying T7 to T9. Total laminectomy with partial resection of the lesions was performed on both patients. Pathological studies confirmed the diagnosis of spinal cord lipoma. They exhibited no dysraphism. Postoperatively, neurological symptoms improved in both patients.

• Journal of Korean Neurosurgical Society
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• v.63 no.3
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• pp.342-345
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• 2020

Some types of spinal dysraphism can be accompanied by extraspinal cysts, including myelomeningocele, myelocystocele, myelocele, meningocele, limited dorsal myeloschisis, lipomyelomeningocele, and terminal myelocystocele. Each disease is classified according to the developmental mechanism, embryologic process, site of occurrence, or internal structure of the extraspinal cyst. In most cystic spinal dysraphisms except meningocele, part of the spinal cord is attached to the cyst dome. Most open spinal dysraphisms pose a risk of infection and require urgent surgical intervention, but when the cyst is accompanied by closed spinal dysraphism, the timing of surgery may vary. However, if the extraspinal cyst grows, it aggravates tethering by pulling the tip of the cord, which is attached to the dome of the cyst. This causes neurological deficits, so urgent surgery is required to release the tethered cord.

• 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.39 no.3
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• pp.224-227
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• 2006

Extradural lipomas have been frequently reported in the literature, but intramedullary lipomas are far rarer, constituting only approximately 2% of total intramedullary tumors. Intramedullary lipomas are also commonly associated with spinal dysraphism. Lipomas which are not associated with spinal dysraphism are present in only about 1% of spinal lipoma patients. Here, we report a rare case of a patient suffering from an isolated intramedullary lipoma without evidence of spinal dysraphism.

• Journal of Korean Neurosurgical Society
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• v.63 no.3
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• pp.366-372
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• 2020

This article recounts the author's personal views and recollections of the history, conception of embryogenetic theories, application of intraoperative electrophysiology, and development of prospective treatment recommendations of several important spinal dysraphic malformations, including limited dorsal myeloschisis, dermal sinus tract, retained medullary cord, terminal myelocystocoele, and complex spinal cord lipomas.

• 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.

• 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.

• 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.

• Clinical and Experimental Pediatrics
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• v.62 no.2
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• pp.68-74
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• 2019

Purpose: To investigate the prevalence of occult spinal dysraphism (OSD) and subsequent neurosurgery in pediatric patients with isolated or combined dorsal midline cutaneous stigmata with or without other congenital malformations. Methods: We carried out a retrospective review of patients who underwent sonography or magnetic resonance imaging (MRI) for OSD because of suspicion of dorsal midline cutaneous stigmata (presumed to be a marker for OSD) between January 2012 and June 2017. Information about patient characteristics, physical examination findings, spinal ultrasound and MRI results, neurosurgical notes, and accompanying congenital anomalies was collected. Results: Totally 250 patients (249 ultrasound and one MRI screening) were enrolled for analysis. Eleven patients underwent secondary MRI examinations. The prevalence of OSD confirmed by an MRI was 2.4% (6 patients including one MRI screening). Five patients (2%) had tethered cord and underwent prophylactic neurosurgery, 3 of whom had a sacrococcygeal dimple and a fibrofatty mass. Prevalence of tethered cord increased as markers associated with a sacrococcygeal dimple increased (0.5% of the isolated marker group, 8.1% of the 2-marker group, and 50% of the 3-marker group). Incidence of OSD with surgical detethering in 17 other congenital anomaly patients was 11.8%, which was higher than the 1.3% in 233 patients without other congenital anomalies. Conclusion: Our results suggest that the presence of dorsal midline cutaneous stigmata, particularly fibrofatty masses, along with a sacrococcygeal dimple is associated with OSD or cord tethering requiring surgery. OSD should be suspected in patients with concurrent occurrence of other congenital anomalies.

• Journal of Korean Neurosurgical Society
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• v.63 no.3
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• pp.314-320
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• 2020

Retained medullary cord (RMC) is a relatively recent term. Pang et al. newly defined the RMC as a late arrest of secondary neurulation leaving a non-functional vestigial portion at the tip of the conus medullaris. RMC, which belongs to the category of closed spinal dysraphism, is a cord-like structure that is elongated from the conus toward the cul-de-sac. Because intraoperative electrophysiological confirmation of a non-functional conus is essential for the diagnosis of RMC, only a tentative or an assumptive diagnosis is possible before surgery or in cases of limited surgical exposure. We suggest the term 'possible RMC' for these cases. An RMC may cause tethered cord syndrome and thus requires surgery. This article reviews the literature to elucidate the pathoembryogenesis, clinical significance and treatment of RMCs.