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Objective@#: Our objective is to analyze the occurrence, clinical course and risk factors for glioma patients with leptomeningeal metastasis (LM) according to different metastasis patterns and clinical variables. @*Methods@#: We retrospectively reviewed data from 376 World Health Organization (WHO) grade II–IV adult glioma patients who were treated in the National Cancer Center from 2001 to 2020. Patients who underwent surgery at other institutions, those without initial images or those with pathologically unconfirmed cases were excluded. LM was diagnosed based on magnetic resonance imaging (MRI) findings or cerebrospinal fluid (CSF) cytology. The metastasis pattern was categorized as nodular or linear according to the enhancement pattern. Tumor proximity to the CSF space was classified as involved or separated, whereas location of the tumor was dichotomized as midline, for tumors residing in the thalamus, basal ganglia and brainstem, or lateral, for tumors residing in the cerebral and cerebellar hemispheres. @*Results@#: A total of 138 patients were enrolled in the study. A total of 44 patients (38%) were diagnosed with LM during a median follow-up of 9 months (range, 0–60). Among the clinical variables, tumor proximity to CSF space, the location of the tumor and the WHO grade were significant factors for LM development in univariate analysis. In multivariate analysis, the midline location of the tumor and WHO grade IV gliomas were the most significant factor for LM development. The hazard ratio was 2.624 for midline located gliomas (95% confidence interval [CI], 1.384–4.974; p=0.003) and 3.008 for WHO grade IV gliomas (95% CI, 1.379–6.561; p=0.006). @*Conclusion@#: Midline location and histological grading are an important factor for LM in glioma patients. The proximity to the CSF circulation pathway is also an important factor for WHO grade IV glioma LM. Patients carrying high risks should be followed up more thoroughly.
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Purpose@#Controversy exists regarding the role preoperative urodynamic study for asymptomatic closed spinal dysraphism as it has failed to reveal the benefit in surgical decision and expectation of urological outcomes. We explore the relationship between preoperative videourodynamic study and postoperative urological outcomes after toilet training completed, focusing on their capability of spontaneous voiding. @*Methods@#We retrospectively reviewed the data of 181 patients who underwent preventive spinal cord untethering and followed at least till the completion of toilet training. Before untethering, patients underwent preoperative videourodynamic study. Postoperative voiding function was evaluated in 3 phases: (1) till postoperative 6 months, (2) till the completion of toilet training, and (3) 2 years after toilet training. Changing distribution of emptying pattern at each period was described. Also, relevance to preoperative urodynamic parameters on spontaneous voiding and urinary continence after toilet training was assessed. @*Results@#Spinal lipoma and low lying conus were found in 145 (80%) and 128 patients (70.7%), respectively. Spontaneous voiding was found in 125 (69.1%), 164 (90.6%), and 162 patients (89.5%) at postoperative 6 months, till the toilet training, and 2 years after toilet training, respectively. Videourodynamics helped to clarify the presence of vesicourethral synergy. This was correlated with spontaneous voiding at postoperative 6 months and better urinary continence after 2 years of toilet training. @*Conclusions@#Eventual spontaneous voiding was achieved till toilet training in 90% patients following preventive untethering. Those showing preoperative vesicourethral synergy was associated with faster achievement of spontaneous voiding and better urinary continence when they enter elementary school.
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Purpose@#We aimed to refine the radiotherapy (RT) volume and dose for intracranial germinoma considering recurrences and long-term toxicities. @*Materials and Methods@#Total 189 patients with intracranial germinoma were treated with RT alone (n=50) and RT with upfront chemotherapy (CRT) (n=139). All cases were confirmed histologically. RT fields comprised the extended-field and involved-field only for primary site. The extended-field, including craniospinal, whole brain (WB), and whole ventricle (WV) for cranial field, is followed by involved-field boost. The median follow-up duration was 115 months. @*Results@#The relapses developed in 13 patients (6.9%). For the extended-field, cranial RT dose down to 18 Gy exhibited no cranial recurrence in 34 patients. In CRT, 74 patients (56.5%) showed complete response to chemotherapy and no involved-field recurrence with low-dose RT of 30 Gy. WV RT with chemotherapy for the basal ganglia or thalamus germinoma showed no recurrence. Secondary malignancy developed in 10 patients (5.3%) with a latency of 20 years (range, 4 to 26 years) and caused mortalities in six. WB or craniospinal field rather than WV or involved-field significantly increased the rate of hormone deficiencies, and secondary malignancy. RT dose for extended-field correlated significantly with the rate of hormone deficiencies, secondary malignancy, and neurocognitive dysfunction. @*Conclusion@#De-intensifying extended-field rather than involved-field or total scheme of RT will be critical to decrease the late toxicities. Upfront chemotherapy could be beneficial for the patients with complete response to minimize the RT dose down to 30 Gy. Prospective trials focused on de-intensification of the extended-field RT are warranted.
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During the last two decades, there have been remarkable advances in knowledge regarding secondary neurulation. An increased number of cases of occult spinal dysraphism and progress in basic embryology research have provoked the continuous discovery of new disease entities and the reclassification of occult spinal dysraphic lesions. Examples of such changes are described. The characteristics of secondary neurulation compared with those of primary neurulation are listed and discussed. Our fundamental questions include what the evolutionary significance of secondary neurulation is and what the advantages of having secondary neurulation are. However, our current data and speculations are insufficient to support scientific inference. The direction of future progress of research in this field is predicted. The role of clinicians in this progress is emphasized.
ABSTRACT
Caudal agenesis refers to the congenital malformation with the essential feature of the agenesis of the sacrococcygeal bone. It is associated with various types of spinal cord anomaly as well as with complex anomalies of genitourinary or gastrointestinal system. The wide spectrum of the disease can be attributed to its pathoembryological origin, the secondary neurulation. This review presents the definition, etiology, classification, and clinical features of the disease.
ABSTRACT
Recent advancements in basic research on the process of secondary neurulation and increased clinical experience with caudal spinal anomalies with associated abnormalities in the surrounding and distal structures shed light on further understanding of the pathoembryogenesis of the lesions and led to the new classification of these dysraphic entities. We summarized the changing concepts of lesions developed from the disordered secondary neurulation shown during the last decade. In addition, we suggested our new pathoembryogenetic explanations for a few entities based on the literature and the data from our previous animal research. Disordered secondary neurulation at each phase of development may cause corresponding lesions, such as failed junction with the primary neural tube (junctional neural tube defect and segmental spinal dysgenesis), dysgenesis or duplication of the caudal cell mass associated with disturbed activity of caudal mesenchymal tissue (caudal agenesis and caudal duplication syndrome), failed ingression of the primitive streak to the caudal cell mass (myelomeningocele), focal limited dorsal neuro-cutaneous nondisjunction (limited dorsal myeloschisis and congenital dermal sinus), neuro-mesenchymal adhesion (lumbosacral lipomatous malformation), and regression failure spectrum of the medullary cord (thickened filum and filar cyst, low-lying conus, retained medullary cord, terminal myelocele and terminal myelocystocele). It seems that almost every anomalous entity of the primary neural tube may occur in the area of secondary neurulation. Furthermore, the close association with the activity of caudal mesenchymal tissue in secondary neurulation involves a wider range of surrounding structures than in primary neurulation. Although the majority of the data are from animals, not from humans and many theories are still conjectural, these changing concepts of normal and disordered secondary neurulation will provoke further advancements in our management strategies as well as in the pathoembryogenetic understanding of anomalous lesions in this area.
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Objective@#: Few studies exist on primary spinal cord tumors (PSCTs) in pediatric patients. The purpose of this study was to perform descriptive analysis and detailed survival analysis for PSCTs. @*Methods@#: Between 1985 and 2017, 126 pediatric patients (male : female, 56 : 70) with PSCTs underwent surgery in a single institution. We retrospectively analyzed data regarding demographics, tumor characteristics, outcomes, and survival statistics. Subgroup analysis was performed for the intramedullary (IM) tumors and extradural (ED) tumors separately. @*Results@#: The mean age of the participants was 6.4±5.04 years, and the mean follow-up time was 69.5±46.30 months. The most common compartment was the ED compartment (n=57, 45.2%), followed by the IM (n=43, 34.1%) and intradural extramedullary (IDEM; n=16, 12.7%) compartments. Approximately half of PSCTs were malignant (n=69, 54.8%). The most common pathologies were schwannomas (n=14) and neuroblastomas (n=14). Twenty-two patients (17.5%) died from the disease, with a mean disease duration of 15.8±15.85 months. Thirty-six patients (28.6%) suffered from progression, with a mean period of 22.6±30.81 months. The 10-year overall survival (OS) rates and progression-free survival (PFS) rates were 81% and 66%, respectively. Regarding IM tumors, the 10-year OS rates and PFS rates were 79% and 57%, respectively. In ED tumors, the 10-year OS rates and PFS rates were 80% and 81%, respectively. Pathology and the extent of resection showed beneficial effects on OS for total PSCTs, IM tumors, and ED tumors. PFS was affected by both the extent of removal and pathology in total PSCTs and ED tumors; however, pathology was a main determinant of PFS rather than the extent of removal in IM tumors. The degree of improvement in the modified McCormick scale showed a trend towards improvement in patients with IM tumors who achieved gross total removal (p=0.447). @*Conclusion@#: Approximately half of PSCTs were malignant, and ED tumors were most common. The most common pathologies were schwannomas and neuroblastomas. Both the pathology and extent of resection had a decisive effect on OS. For IM tumors, pathology was a main determinant of PFS rather than the extent of removal. Radical excision of IM tumors could be a viable option for better survival without an increased risk of worse functional outcomes.
ABSTRACT
During the last two decades, there have been remarkable advances in knowledge regarding secondary neurulation. An increased number of cases of occult spinal dysraphism and progress in basic embryology research have provoked the continuous discovery of new disease entities and the reclassification of occult spinal dysraphic lesions. Examples of such changes are described. The characteristics of secondary neurulation compared with those of primary neurulation are listed and discussed. Our fundamental questions include what the evolutionary significance of secondary neurulation is and what the advantages of having secondary neurulation are. However, our current data and speculations are insufficient to support scientific inference. The direction of future progress of research in this field is predicted. The role of clinicians in this progress is emphasized.
ABSTRACT
Caudal agenesis refers to the congenital malformation with the essential feature of the agenesis of the sacrococcygeal bone. It is associated with various types of spinal cord anomaly as well as with complex anomalies of genitourinary or gastrointestinal system. The wide spectrum of the disease can be attributed to its pathoembryological origin, the secondary neurulation. This review presents the definition, etiology, classification, and clinical features of the disease.
ABSTRACT
Recent advancements in basic research on the process of secondary neurulation and increased clinical experience with caudal spinal anomalies with associated abnormalities in the surrounding and distal structures shed light on further understanding of the pathoembryogenesis of the lesions and led to the new classification of these dysraphic entities. We summarized the changing concepts of lesions developed from the disordered secondary neurulation shown during the last decade. In addition, we suggested our new pathoembryogenetic explanations for a few entities based on the literature and the data from our previous animal research. Disordered secondary neurulation at each phase of development may cause corresponding lesions, such as failed junction with the primary neural tube (junctional neural tube defect and segmental spinal dysgenesis), dysgenesis or duplication of the caudal cell mass associated with disturbed activity of caudal mesenchymal tissue (caudal agenesis and caudal duplication syndrome), failed ingression of the primitive streak to the caudal cell mass (myelomeningocele), focal limited dorsal neuro-cutaneous nondisjunction (limited dorsal myeloschisis and congenital dermal sinus), neuro-mesenchymal adhesion (lumbosacral lipomatous malformation), and regression failure spectrum of the medullary cord (thickened filum and filar cyst, low-lying conus, retained medullary cord, terminal myelocele and terminal myelocystocele). It seems that almost every anomalous entity of the primary neural tube may occur in the area of secondary neurulation. Furthermore, the close association with the activity of caudal mesenchymal tissue in secondary neurulation involves a wider range of surrounding structures than in primary neurulation. Although the majority of the data are from animals, not from humans and many theories are still conjectural, these changing concepts of normal and disordered secondary neurulation will provoke further advancements in our management strategies as well as in the pathoembryogenetic understanding of anomalous lesions in this area.
ABSTRACT
Objective@#: Few studies exist on primary spinal cord tumors (PSCTs) in pediatric patients. The purpose of this study was to perform descriptive analysis and detailed survival analysis for PSCTs. @*Methods@#: Between 1985 and 2017, 126 pediatric patients (male : female, 56 : 70) with PSCTs underwent surgery in a single institution. We retrospectively analyzed data regarding demographics, tumor characteristics, outcomes, and survival statistics. Subgroup analysis was performed for the intramedullary (IM) tumors and extradural (ED) tumors separately. @*Results@#: The mean age of the participants was 6.4±5.04 years, and the mean follow-up time was 69.5±46.30 months. The most common compartment was the ED compartment (n=57, 45.2%), followed by the IM (n=43, 34.1%) and intradural extramedullary (IDEM; n=16, 12.7%) compartments. Approximately half of PSCTs were malignant (n=69, 54.8%). The most common pathologies were schwannomas (n=14) and neuroblastomas (n=14). Twenty-two patients (17.5%) died from the disease, with a mean disease duration of 15.8±15.85 months. Thirty-six patients (28.6%) suffered from progression, with a mean period of 22.6±30.81 months. The 10-year overall survival (OS) rates and progression-free survival (PFS) rates were 81% and 66%, respectively. Regarding IM tumors, the 10-year OS rates and PFS rates were 79% and 57%, respectively. In ED tumors, the 10-year OS rates and PFS rates were 80% and 81%, respectively. Pathology and the extent of resection showed beneficial effects on OS for total PSCTs, IM tumors, and ED tumors. PFS was affected by both the extent of removal and pathology in total PSCTs and ED tumors; however, pathology was a main determinant of PFS rather than the extent of removal in IM tumors. The degree of improvement in the modified McCormick scale showed a trend towards improvement in patients with IM tumors who achieved gross total removal (p=0.447). @*Conclusion@#: Approximately half of PSCTs were malignant, and ED tumors were most common. The most common pathologies were schwannomas and neuroblastomas. Both the pathology and extent of resection had a decisive effect on OS. For IM tumors, pathology was a main determinant of PFS rather than the extent of removal. Radical excision of IM tumors could be a viable option for better survival without an increased risk of worse functional outcomes.
ABSTRACT
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.
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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.
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There has been confusion in the classification of terminal myelocystocele (TMCC) due to its diverse morphology and vague pathoembryogenesis. TMCC could be summarized as having the essential features of an elongated caudal spinal cord extruding out of the dorsal extraspinal space that fuses with the subcutaneous fat, which is in the shape of a trumpet-shaped cerebrospinal fluid-filled cyst. The extraspinal portion of the extruded spinal cord is nonfunctional. The morphological features suggest that TMCC is formed during secondary neurulation, specifically the failure of the degeneration of the secondary neural tube near the time of the terminal balloon. This review discusses the definition, as well as the clinical and surgical features, of TMCC with special emphasis on its pathoembryogenesis.
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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.
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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.
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During the follow-up period after surgery for spinal dysraphism, a certain portion of patients show neurological deterioration and its secondary phenomena, such as motor, sensory or sphincter changes, foot and spinal deformities, pain, and spasticity. These clinical manifestations are caused by tethering effects on the neural structures at the site of previous operation. The widespread recognition of retethering drew the attention of medical professionals of various specialties because of its incidence, which is not low when surveillance is adequate, and its progressive nature. This article reviews the literature on the incidence and timing of deterioration, predisposing factors for retethering, clinical manifestations, diagnosis, surgical treatment and its complications, clinical outcomes, prognostic factors after retethering surgery and preventive measures of retethering. Current practice and opinions of Seoul National University Children’s Hospital team were added in some parts. The literature shows a wide range of data regarding the incidence, rate and degree of surgical complications and long-term outcomes. The method of prevention is still one of the main topics of this entity. Although alternatives such as spinal column shortening were introduced, re-untethering by conventional surgical methods remains the current main management tool. Re-untethering surgery is a much more difficult task than primary untethering surgery. Updated publications include strong skepticism on re-untethering surgery in a certain group of patients, though it is from a minority of research groups. For all of the abovementioned reasons, new information and ideas on the early diagnosis, treatment and prevention of retethering are critically necessary in this era.
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BACKGROUND AND PURPOSE: This study investigated the seizure recurrence rate and potential predictors of seizure recurrence following antiepileptic drug (AED) withdrawal after resective epilepsy surgery in children with focal cortical dysplasia (FCD). METHODS: We retrospectively analyzed the records of 70 children and adolescents with FCD types I, II, and IIIa who underwent resective epilepsy surgery between 2004 and 2015 and were followed for at least 2 years after surgery. RESULTS: We attempted AED withdrawal in 40 patients. The median time of starting the AED reduction was 10.8 months after surgery. Of these 40 patients, 14 patients (35%) experienced seizure recurrence during AED reduction or after AED withdrawal. Half of the 14 patients who experienced recurrence regained seizure freedom after AED reintroduction and optimization. Compared with their preoperative status, the AED dose or number was decreased in 57.1% of patients, and remained unchanged in 14.3% after surgery. A multivariate analysis found that incomplete resection (p=0.004) and epileptic discharges on the postoperative EEG (p=0.025) were important predictors of seizure recurrence after AED withdrawal. Over the mean follow-up duration of 4.5 years after surgery, 34 patients (48.6% of the entire cohort) were seizure-free with and without AEDs. CONCLUSIONS: Children with incomplete resection and epileptic discharges on postoperative EEG are at a high risk of seizure recurrence after drug withdrawal. Complete resection of FCD may lead to a favorable surgical outcome and successful AED withdrawal after surgery.
Subject(s)
Adolescent , Child , Humans , Anticonvulsants , Electroencephalography , Epilepsy , Follow-Up Studies , Freedom , Malformations of Cortical Development , Multivariate Analysis , Recurrence , Retrospective Studies , SeizuresABSTRACT
OBJECTIVE: Intracranial aneurysms are not common in young age patients. We sought to find the characteristics of the intracranial aneurysms in patients under 20 years of age.METHODS: We reviewed 23 consecutive patients ≤20 years of age treated for their intracranial aneurysms during the period from 1995 to 2017. From medical records and imaging studies, we gathered data on age, sex, presentation, associated medical condition, location and characteristics of aneurysms, treatment and clinical outcomes.RESULTS: The patients’ ages ranged from 13 months to 20 years (median, 14 years). There were 16 males and seven females (male to female ratio, 2.3 : 1) with 31 aneurysms. Clinical presentations included sudden severe headache in 61%, followed by altered mentality in 17% and seizure in 17%. More than one-fourth patients had specific medical conditions related to the development of the cerebral aneurysms. The majority of aneurysms occurred in the anterior circulation (71%), and were saccular (71%). There were each three patients with false aneurysms (13%) and giant aneurysms (13%), and only one patient with multiple aneurysms (4%). We treated 22 patients : 21 aneurysms with the endovascular methods, three with open surgery, and one with combined treatment. Good functional outcome could be achieved in 86% during the follow-up period.CONCLUSION: In this series, the young-age patients with intracranial aneurysms were characterized by male predominance, related specific medical conditions, low incidence of multiple aneurysms, high incidence of giant aneurysms and good functional outcome after treatment.