Chiari Malformation with Surgically Induced Open Neural Tube Defect in Late Chick Embryos : Characterization by Magnetic Resonance Imaging and Histopathological Analysis

Objective@#: Chiari II malformation (CM II) is still the main cause of severe morbidity and mortality in children with open neural tube defects (ONTDs). The goal of this study was to validate a CM II model in late-stage chick embryos with surgically induced ONTDs. @*Methods@#: To make the chick embryo model of ONTD, their neural tubes were opened for a length of 5–6 somites at the thoracic level in Hamburger and Hamilton stage 18 chick embryos (n=150). They were reincubated in ovo. up to a total age of 17–21 days. A total of 19 embryos survived and were assigned to either the postoperative day (POD) 14–15 group (n=6) or the POD 17–18 group (n=13). Magnetic resonance imaging (MRI) and histopathologic findings of embryo heads with spinal ONTDs were compared with age-matched normal chick embryos. @*Results@#: The chick embryos with ONTDs demonstrated definite and constant structural changes, such as downward displacement of the cerebellum to just above the foramen magnum and narrow and small cerebrospinal fluid spaces in the crowded small posterior fossa. These morphologic features were more prominent in the POD 17–18 group than in the POD 14–15 group. @*Conclusion@#: This is the first description of CM II with spinal ONTD in a late-stage chick embryo model with MRI and histopathological analysis. The morphological changes of the posterior fossa in this study mimic those of CM II associated with spinal ONTD in humans. This model will facilitate investigation of the pathogenesis of CM II.

Clinical Outcome of Endoscopic Procedure in Patients with Shunt Malfunction

Objective@#: The goal of this study was to analyze the clinical outcomes of endoscopic third ventriculostomy (ETV) and endoscopic septostomy when shunt malfunction occurs in a patient who has previously undergone placement of a ventriculoperitoneal shunt. @*Methods@#: From 2001 to 2020 at Seoul National University Children's Hospital, patients who underwent ETV or endoscopic septostomy for shunt malfunction were retrospectively analyzed. Initial diagnosis (etiology of hydrocephalus), age at first shunt insertion, age at endoscopic procedure, magnetic resonance or computed tomography image, subsequent shunting data, and follow-up period were included. @*Results@#: Thirty-six patients were included in this retrospective study. Twenty-nine patients, 18 males and 11 females, with shunt malfunction underwent ETV. At the time of shunting, the age ranged from 1 day to 15.4 years (mean, 2.4 years). The mean age at the time of ETV was 13.1 years (range, 0.7 to 29.6 years). Nineteen patients remained shunt revision free. The 5-year shunt revisionfree survival rate was 69% (95% confidence interval [CI], 0.54–0.88). Seven patients, three males and four females, with shunt malfunction underwent endoscopic septostomy. At the time of shunting, the age ranged from 0.2 to 12 years (mean, 3.9 years). The mean age at the time of endoscopic septostomy was 11.9 years (range, 0.5 to 29.5 years). Four patients remained free of shunt revision or addition. The 5-year shunt revision-free survival rate was 57% (95% CI, 0.3–1.0). There were no complications associated with the endoscopic procedures. @*Conclusion@#: The results of our study demonstrate that ETV or endoscopic septostomy can be effective and safe in patients with shunt malfunction.

Clinical Effect of Transverse Process Hook with K-Means Clustering-Based Stratification of Computed Tomography Hounsfield Unit at Upper Instrumented Vertebra Level in Adult Spinal Deformity Patients

Objective@#: This study aimed to investigate the efficacy of transverse process (TP) hook system at the upper instrumented vertebra (UIV) for preventing screw pullout in adult spinal deformity surgery using the pedicle Hounsfield unit (HU) stratification based on K-means clustering. @*Methods@#: We retrospectively reviewed 74 patients who underwent deformity correction surgery between 2011 and 2020 and were followed up for >12 months. Pre- and post-operative data were used to determine the incidence of screw pullout, UIV TP hook implementation, vertebral body HU, pedicle HU, and patient outcomes. Data was then statistically analyzed for assessment of efficacy and risk prediction using stratified HU at UIV level alongside the effect of the TP hook system. @*Results@#: The screw pullout rate was 36.4% (27/74). Perioperative radiographic parameters were not significantly different between the pullout and non-pullout groups. The vertebral body HU and pedicle HU were significantly lower in the pullout group. K-means clustering stratified the vertebral body HU ≥205.3, <137.2, and pedicle HU ≥243.43, <156.03. The pullout rate significantly decreases in patients receiving the hook system when the pedicle HU was from ≥156.03 to < 243.43 (p<0.05), but the difference was not statistically significant in the vertebra HU stratified groups and when pedicle HU was ≥243.43 or <156.03. The postoperative clinical outcomes improved significantly with the implementation of the hook system. @*Conclusion@#: The UIV hook provides better clinical outcomes and can be considered a preventative strategy for screw-pullout in the certain pedicle HU range.

Therapeutic Potentiality of Celtis choseniana Nakai on Androgenic Alopecia through Repression of Androgen Action and Modulation of Wnt/β-catenin Signaling

In this study, we investigated the efficacy of Celtis choseniana Nakai (C. choseniana) as complementary herbal medicine to ameliorate androgenic alopecia (AGA). The effects of C. choseniana on AGA were evaluated using testosterone propionate-induced AGA mouse model and dihydrotestosterone-treated human hair follicle dermal papilla cells. In vivo, C. choseniana treatment deactivated androgen signaling by reducing the concentration of serum dihydrotestosterone level and expressions of 5α-reductase 2 and androgen receptor. Next, C. choseniana treatment increased the hair regrowth rate. Histological studies demonstrated that C. choseniana induced the anagen phase in testosterone propionate-induced AGA mouse model. Cellular proliferation was promoted by C. choseniana treatment via increasing the expression of proliferation factors, such as proliferating cell nuclear antigen and cyclin D1. Furthermore, C. choseniana treatment increased the expression of proteins related to the Wnt/β-catenin signaling pathway. In addition, dickkopf-1, a Wnt inhibitor, was downregulated with C. choseniana treatment. Likewise, C. choseniana treatment promoted cellular proliferation in vitro. This study demonstrated the inhibitory effect of C. choseniana on androgen-induced AGA. Moreover, C. choseniana induced activation of Wnt/β-catenin signaling, resulting in prolonged anagen and cellular proliferation. Therefore, we suggest that C. choseniana can be used as a therapeutic agent to alleviate AGA.

Cyclin-Dependent Kinase Inhibitor 2A is a Key Regulator of Cell Cycle Arrest and Senescence in Endothelial Colony-Forming Cells in Moyamoya Disease

Objective@#: Endothelial colony-forming cells (ECFCs) have been reported to play an important role in the pathogenesis of moyamoya disease (MMD). We have previously observed stagnant growth in MMD ECFCs with functional impairment of tubule formation. We aimed to verify the key regulators and related signaling pathways involved in the functional defects of MMD ECFCs. @*Methods@#: ECFCs were cultured from peripheral blood mononuclear cells of healthy volunteers (normal) and MMD patients. Low-density lipoproteins uptake, flow cytometry, high content screening, senescence-associated β-galactosidase, immunofluorescence, cell cycle, tubule formation, microarray, real-time quantitative polymerase chain reaction, small interfering RNA transfection, and western blot analyses were performed. @*Results@#: The acquisition of cells that can be cultured for a long time with the characteristics of late ECFCs was significantly lower in the MMD patients than the normal. Importantly, the MMD ECFCs showed decreased cellular proliferation with G1 cell cycle arrest and cellular senescence compared to the normal ECFCs. A pathway enrichment analysis demonstrated that the cell cycle pathway was the major enriched pathway, which is consistent with the results of the functional analysis of ECFCs. Among the genes associated with the cell cycle, cyclin-dependent kinase inhibitor 2A (CDKN2A) showed the highest expression in MMD ECFCs. Knockdown of CDKN2A in MMD ECFCs enhanced proliferation by reducing G1 cell cycle arrest and inhibiting senescence through the regulation of CDK4 and phospho retinoblastoma protein. @*Conclusion@#: Our study suggests that CDKN2A plays an important role in the growth retardation of MMD ECFCs by inducing cell cycle arrest and senescence.

Effects of Albizia julibrissin Durazz through Suppression of Mitochondrial Fission and Apoptosis in Cisplatin-induced Acute Kidney Injury

Albizia julibrissin Durazz. (AJ; family Minosaceae) is widely distributed worldwide, and its stem bark has been used as a traditional herbal medicine. Acute kidney injury (AKI) is a clinical syndrome that results in sudden loss of renal function. This study aimed to investigate the effects of AJ against cisplatin-induced AKI using a human kidney proximal tubule epithelial cell line (HK-2) and cisplatin-treated mice. In vitro, cisplatin treatment increased apoptosis in HK-2 cells. However, AJ treatment decreased apoptosis of cisplatin-treated HK-2 cells. In vivo, cisplatin treatment accelerated renal injury by increasing the levels of renal injury markers, such as blood urea nitrogen, creatinine, kidney injury molecule 1, and neutrophil gelatinase-associated lipocalin, which were reversed by AJ treatment. Histopathologically, AJ treatment resulted in decreased renal damage with less tubular necrosis and brush border desquamation compared with the AKI group. Additionally, cisplatin treatment upregulated mitochondrial fission, a pathological characteristic of AKI, which was downregulated by AJ treatment. Along with increased mitochondrial fission, AJ treatment also reduced cisplatin-induced apoptosis.These results suggest that AJ may be a potential therapeutic agent for cisplatin-induced AKI.

The extract of Celtis choseniana Nakai alleviates testosterone-induced benign prostatic hyperplasia through inhibiting 5α reductase type 2 and the Akt/NF-κB/AR pathway / 中国天然药物

Benign prostatic hyperplasia (BPH) is a chronic male disease characterized by the enlarged prostate. Celtis chosenianaNakai (C. choseniana) is medicinally used to alleviate pain, gastric disease, and lung abscess. In this study, the effect of C. choseniana extract on BPH was investigated using testosterone-induced rats. Sprague Dawley rats were divided into five groups: control, BPH (testosterone 5 mg·kg-1), Fina (finasteride 2 mg·kg-1), and C. choseniana (50 and 100 mg·kg-1). After four weeks of TP treatment with finasteride or C. choseniana, prostate weights and DHT levels were measured. In addition, the prostates were histopathologically examined and measured for protein kinase B (Akt)/nuclear factor-κB (NF-κB)/AR signaling, proliferation, apoptosis, and autophagy. Prostate weight and epithelial thickness were reduced in the C. choseniana groups compared with that in the BPH group. The extract of C. choseniana acted as a 5α reductase inhibitor, reducing DHT levels in the prostate. Furthermore, the extract of C. choseniana blocked the activation of p-Akt, nuclear NF-κB activation and reduced the expression of AR and PSA compared with BPH. Moreover, the expression of Bax, PARP-1, and p53 increased, while the expression of bcl-2 decreased. The present study demonstrated that C. choseniana extract alleviated testosterone-induced BPH by suppressing 5α reductase and Akt/NF-κB activation, reducing AR signaling and inducing apoptosis and autophagy in the prostate. These results suggested that C. choseniana probably contain potential herbal agents to alleviate BPH.

Influence of Measurement Procedure on the Korean Matrix Sentence-in-Noise Intelligibility for Normal-Hearing Listeners / 대한이비인후과학회지

Background and Objectives@#A Korean Matrix Sentence Test is one of the adaptive tests used to evaluate speech intelligibility in noise. The present study aimed to investigate the influence of measurement procedure (presentation level and mode) on the Korean matrix sentence-in-noise intelligibility of normal-hearing listeners.Subjects and Method Twenty-seven normal-hearing adults participated in the study. The speech reception threshold (SRT) was determined using a software-based adaptive procedure, which converges on the signal-to-noise ratio (SNR), corresponding to 50% intelligibility. The speech or speech-shaped noise was given at three presentation levels based on the previous categorical loudness judgments (between soft and medium: 55 dB SPL, medium: 65 dB SPL, and loud level: 80 dB SPL). The effect of the presentation mode was explored by fixing either the sentence level or noise level, while the noise or sentence level was adaptively adjusted according to the subject’s response. @*Results@#The presentation level significantly affected the SRTs, whereas the SRTs did not depend on the presentation mode. The sentence-in-noise intelligibility was similar between 55 and 65 dB SPL, yet the sentence-in-noise intelligibility significantly decreased at 80 dB SPL regardless of the presentation mode. Correlation analyses showed moderate to high positive correlations among the results of two presentation modes. @*Conclusion@#A presentation level of 55 or 65 dB SPL yielded comparable adaptive measurement results for normal-hearing subjects, yet the sentence-in-nose intelligibility became worse at 80 dB SPL. Clinicians should be careful with the influence of the presentation level when identifying speech-in-noise intelligibility.

The Fate of Proximal Junctional Vertebral Fractures after Long-Segment Spinal Fixation : Are There Predictable Radiologic Characteristics for Revision surgery?

Objective@#: To investigate the radiographic characteristics of the uppermost instrumented vertebrae (UIV) and UIV+1 compression fractures that are predictive of revision surgery following long-segment spinal fixation. @*Methods@#: A total 27 patients who presented newly developed compression fracture at UIV, UIV+1 after long segment spinal fixation (minimum 5 vertebral bodies, lowest instrumented vertebra of L5 or distal) were reviewed retrospectively. Patients were divided into two groups according to following management : revisional surgery (group A, n=13) and conservative care (group B, n=14). Pre- and postoperative images, and images taken shortly before and after the occurrence of fracture were evaluated for radiologic characteristics @*Results@#: Despite similar degrees of surgical correction of deformity, the fate of the two groups with proximal junctional compression fractures differed. Immediately after the fracture, the decrement of adjacent disc height in group A (32.3±7.6 mm to 23.7±8.4 mm, Δ=8.5±6.9 mm) was greater than group B (31.0±13.9 mm to 30.1±15.5 mm, Δ=0.9±2.9 mm, p=0.003). Pre-operative magnetic resonance imaging indicated that group A patients have a higher grade of disc degeneration adjacent to fractured vertebrae compared to group B (modified Pfirrmann grade, group A : 6.10±0.99, group B : 4.08±0.90, p=0.004). Binary logistic regression analysis indicated that decrement of disc height was the only associated risk factor for future revision surgery (odds ratio, 1.891; 95% confidence interval, 1.121–3.190; p=0.017). @*Conclusion@#: Proximal junctional vertebral compression fractures with greater early-stage decrement of adjacent disc height were associated with increased risk of future neurological deterioration and necessity of revision. The condition of adjacent disc degeneration should be considered regarding severity and revision rate of proximal junctional kyphosis/proximal junction failures.

Junctional Neurulation : A Junction between Primary and Secondary Neural Tubes

Recent case reports of junctional neural tube defect (JNTD) which is a peculiar type of spinal anomaly showing the functional disconnection of the primary and secondary neural tubes has risen interest in the process of junctional neurulation (the connection between the two neural tubes) during development. This article summarizes the clinical features of the JNTD and reviews the literature on the basic research on junctional neurulation.

Disorders of Secondary Neurulation : Mainly Focused on Pathoembryogenesis

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.

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

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.

Clinicopathological Features of Primary Solitary Spinal Cord Tumors in Pediatric Patients : A 32-Year Single Institution Experience

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.

The Fate of Proximal Junctional Vertebral Fractures after Long-Segment Spinal Fixation : Are There Predictable Radiologic Characteristics for Revision surgery?

Objective@#: To investigate the radiographic characteristics of the uppermost instrumented vertebrae (UIV) and UIV+1 compression fractures that are predictive of revision surgery following long-segment spinal fixation. @*Methods@#: A total 27 patients who presented newly developed compression fracture at UIV, UIV+1 after long segment spinal fixation (minimum 5 vertebral bodies, lowest instrumented vertebra of L5 or distal) were reviewed retrospectively. Patients were divided into two groups according to following management : revisional surgery (group A, n=13) and conservative care (group B, n=14). Pre- and postoperative images, and images taken shortly before and after the occurrence of fracture were evaluated for radiologic characteristics @*Results@#: Despite similar degrees of surgical correction of deformity, the fate of the two groups with proximal junctional compression fractures differed. Immediately after the fracture, the decrement of adjacent disc height in group A (32.3±7.6 mm to 23.7±8.4 mm, Δ=8.5±6.9 mm) was greater than group B (31.0±13.9 mm to 30.1±15.5 mm, Δ=0.9±2.9 mm, p=0.003). Pre-operative magnetic resonance imaging indicated that group A patients have a higher grade of disc degeneration adjacent to fractured vertebrae compared to group B (modified Pfirrmann grade, group A : 6.10±0.99, group B : 4.08±0.90, p=0.004). Binary logistic regression analysis indicated that decrement of disc height was the only associated risk factor for future revision surgery (odds ratio, 1.891; 95% confidence interval, 1.121–3.190; p=0.017). @*Conclusion@#: Proximal junctional vertebral compression fractures with greater early-stage decrement of adjacent disc height were associated with increased risk of future neurological deterioration and necessity of revision. The condition of adjacent disc degeneration should be considered regarding severity and revision rate of proximal junctional kyphosis/proximal junction failures.

Junctional Neurulation : A Junction between Primary and Secondary Neural Tubes

Recent case reports of junctional neural tube defect (JNTD) which is a peculiar type of spinal anomaly showing the functional disconnection of the primary and secondary neural tubes has risen interest in the process of junctional neurulation (the connection between the two neural tubes) during development. This article summarizes the clinical features of the JNTD and reviews the literature on the basic research on junctional neurulation.

Disorders of Secondary Neurulation : Mainly Focused on Pathoembryogenesis

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.

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

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.

Clinicopathological Features of Primary Solitary Spinal Cord Tumors in Pediatric Patients : A 32-Year Single Institution Experience

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.

Surgical Strategy for Sacral Tumor Resection

Purpose@#This study aimed to present our experiences with a precise surgical strategy for sacrectomy. @*Materials and Methods@#This study comprised a retrospective review of 16 patients (6 males and 10 females) who underwent sacrectomy from 2011 to 2019. The average age was 42.4 years old, and the mean follow-up period was 40.8 months. Clinical data, including age, sex, history, pathology, radiographs, surgical approaches, onset of recurrence, and prognosis, were analyzed. @*Results@#The main preoperative symptom was non-specific local pain. Nine patients (56%) complained of bladder and bowel symptoms. All patients required spinopelvic reconstruction after sacrectomy. Three patients, one high, one middle, and one hemisacrectomy, underwent spinopelvic reconstruction. The pathology findings of tumors varied (chordoma, n=7; nerve sheath tumor, n=4; giant cell tumor, n=3, etc.). Adjuvant radiotherapy was performed for 5 patients, chemotherapy for three, and combined chemoradiotherapy for another three. Six patients (38%) reported postoperative motor weakness, and newly postoperative bladder and bowel symptoms occurred in 5 patients. Three patients (12%) experienced recurrence and expired. @*Conclusion@#In surgical resection of sacral tumors, the surgical approach depends on the size, location, extension, and pathology of the tumors. The recommended treatment option for sacral tumors is to remove as much of the tumor as possible. The level of root sacrifice is a predicting factor for postoperative neurologic functional impairment and the potential for morbidity. Pre-operative angiography and embolization are recommended to prevent excessive bleeding during surgery. Spinopelvic reconstruction must be considered following a total or high sacrectomy or sacroiliac joint removal.

Secondary Neurulation Defects-1 : Retained Medullary Cord

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.