Caudal Agenesis: Classification Based on the Pathoembryogenesis of the Spinal Cord.

BACKGROUND AND OBJECTIVES: Caudal agenesis (CA) is a congenital disease characterized by lower vertebral bone defects. Previous classifications for CA were based on the levels of bony defects or the conus medullaris. We created a new pathoembryogenic classification that takes into account the level of conus, considering both its shape and filum. We evaluated its accuracy in reflecting the neurological status and the need for untethering. METHODS: Patients with radiologic studies available on our institute's electronic image view system between 1985 and 2019 were reviewed. Our classification categorized patients with CA into 3 groups: the failure of formation (a conus level > L1 or normal level conus with a blunt shape), the failure of regression (a conus level < L3 or normal level conus with a thickened filum or a filar lipoma), and the normal (a normal level conus without specific abnormalities). We analyzed which of the following 3 classifications better reflects the neurological status and the probability of recommending untethering: classifications based on (1) bony defect level, (2) conus level, and (3) pathoembryogenic mechanism. RESULTS: A total of 89 patients were included in this study. The pathoembryogenic classification revealed more significant differences in motor or sensory deficits compared with other classifications (failure of formation: 42%; failure of regression: 16%; and normal: 0%, P = .039). In addition, the pathoembryogenic classification emerged as a significant or suggestive predictive factor for motor and sensory deficits (motor: odds ratio 11.66, P = .007; sensory: odds ratio 5.44, P = .066). Notably, only the pathoembryogenic classification exhibited a significant difference in the probabilities of recommending untethering between groups (failure of formation: 42%; failure of regression: 81%; and normal 12%, P < .001). CONCLUSION: The correlation between bony classification and spinal cord abnormalities was exaggerated. Our new pathoembryogenic classification was valuable in prognosticating neurological status and identifying the patient group more likely to require untethering among patients with CA.

Novel role of LLGL2 silencing in autophagy: reversing epithelial-mesenchymal transition in prostate cancer.

PURPOSE: Prostate cancer (PCa) is a major urological disease that is associated with significant morbidity and mortality in men. LLGL2 is the mammalian homolog of Lgl. It acts as a tumor suppressor in breast and hepatic cancer. However, the role of LLGL2 and the underlying mechanisms in PCa have not yet been elucidated. Here, we investigate the role of LLGL2 in the regulation of epithelial-mesenchymal transition (EMT) in PCa through autophagy in vitro and in vivo. METHODS: PC3 cells were transfected with siLLGL2 or plasmid LLGL2 and autophagy was examined. Invasion, migration, and wound healing were assessed in PC3 cells under autophagy regulation. Tumor growth was evaluated using a shLLGL2 xenograft mouse model. RESULTS: In patients with PCa, LLGL2 levels were higher with defective autophagy and increased EMT. Our results showed that the knockdown of LLGL2 induced autophagy flux by upregulating Vps34 and ATG14L. LLGL2 knockdown inhibits EMT by upregulating E-cadherin and downregulating fibronectin and α-SMA. The pharmacological activation of autophagy by rapamycin suppressed EMT, and these effects were reversed by 3-methyladenine treatment. Interestingly, in a shLLGL2 xenograft mouse model, tumor size and EMT were decreased, which were improved by autophagy induction and worsened by autophagy inhibition. CONCLUSION: Defective expression of LLGL2 leads to attenuation of EMT due to the upregulation of autophagy flux in PCa. Our results suggest that LLGL2 is a novel target for alleviating PCa via the regulation of autophagy.

Prognostic Factors of Spinal Intramedullary Hemangioblastoma : Analysis of Surgical Outcomes and Tumor Characteristics.

Objective: Spinal intramedullary hemangioblastoma is a rare and highly vascularized benign tumor. The characteristics of the tumor, its corresponding location, and surgical outcomes remain unknown. The purpose of this study was to identify risk factors and strategies for neurologic deterioration following hemangioblastoma surgery. Methods: A comprehensive retrospective analysis was undertaken to evaluate patients who underwent surgical intervention for intramedullary hemangioblastoma at our institution from 1993 to 2022. Patients with at least one year of follow-up data were included. The analysis covered patient demographics, pre- and post-operative Modified McCormick Scale (MMCS), tumor location, and tumor size. Results: This study included 25 cases. One-year after surgery, neurological deterioration was observed in 5 (20.0%) cases, and neurological improvement was found in 9 (36.0%) cases. Five cases were ventrally located, and twelve cases were dorsally located. Ventrally located cases were larger in tumor axial size (p = 0.029) than dorsal location tumors, resulting in poorer follow-up MMCS and a higher prevalence of von Hippel-Lindau syndrome (VHL) (p = 0.042). Three of them were confirmed to be supplied by the anterior spinal artery. In the case of dorsally located cases, there was no neurologic deterioration. Conclusion: In intramedullary spinal cord hemangioblastomas, cases located ventrally had a higher incidence of neurological deterioration following surgery than those located dorsally or in intramedullary extramedullary cases. Ventrally located hemangioblastomas were larger than those in other locations. They were mainly supplied by the anterior spinal artery in VHL patients.

Factors Associated With Repeat Surgery in Cervical Ossification of the Posterior Longitudinal Ligaments: Minimum 8-year Follow-up Study.

STUDY DESIGN: Single-center retrospective study. OBJECTIVE: The objective of this study is to identify the factors leading to repeat surgery in patients with cervical ossification of the longitudinal ligament (OPLL) during a minimal 8-year follow-up after the initial surgery. SUMMARY OF BACKGROUND DATA: The long-term effects of cervical OPLL are well known, but it's not always clear how many patients need to have surgery again because their neurological symptoms get worse. METHODS: This study is included 117 patients who underwent surgery for cervical OPLL and had a follow-up of at least 8 years. OPLL type, surgical extent, surgical method, and sagittal radiological parameters were measured, and OPLL characteristics were analyzed. RESULTS: The average age of patients at the time of surgery was 53.2 years, with a male-to-female ratio of 78:39. The median follow-up duration was 122 months (96-170 mo). Out of the total, 20 cases (17.1%) necessitated repeat surgery, among which 8 cases required surgery at the same site as the initial operation. The highest rate of repeat surgery was observed in patients who underwent total laminectomy without fusion (TL), where 6 out of 21 patients (29%) needed a second surgery, and 5 of these (23%) involved the same surgical site. Patients who underwent repeat surgery at the same site exhibited a greater range of motion (ROM) one year postsurgery (16.4 ± 8.5° vs. 23.1 ± 12.7°, P =0.041). In addition, the ROM at 1 year was higher in patients who underwent TL compared with those who had laminoplasty. Furthermore, the recurrence rate for hill-shape OPLL was higher at 30.8% compared to 10% for plateau-shape OPLL ( P = 0.05). CONCLUSION: Larger cervical ROM 1 year after surgery is related to repeat surgery at the same level as previous surgery, especially in laminectomy without fusion surgery.

Neurocognitive outcomes and associated clinical factors 5 years after surgery in children with craniosynostosis.

OBJECTIVE: Craniosynostosis involves early closure of one or more sutures, which is known to limit normal cranium growth and interfere with normal brain development. Various surgical methods are used, ranging from minimally invasive strip craniectomy to more extensive whole-vault cranioplasty. This study aimed to evaluate neurocognitive outcomes 5 years after surgical treatment in children with craniosynostosis and to evaluate relevant clinical factors. METHODS: After exclusion of genetically confirmed syndromic craniosynostosis patients, a retrospective review was conducted on 112 nonsyndromic craniosynostosis patients who underwent surgical treatment and follow-up neurocognitive assessment. Ninety-seven patients underwent strip craniectomy with postoperative orthotic helmet therapy, and 15 received other surgical treatment: 4 with distraction osteotomy and 11 with craniofacial reconstruction. Neurocognitive assessment using the Korean Wechsler Preschool and Primary Scale of Intelligence, Fourth Edition (K-WPPSI-IV), was performed 5 years postoperatively. Clinical factors were assessed regarding neurocognitive outcomes. RESULTS: The mean age at surgery was significantly younger in the strip craniectomy group (strip craniectomy 4.6 months vs other surgical treatment 18.6 months, p < 0.01). Patients with 2 or more sutures involved were more likely to receive more extensive surgical treatment (16.5% in the strip craniectomy group vs 53.8% in the other group, p < 0.01). Four (3.5%) patients who showed evidence of increased intracranial pressure (ICP) also underwent more extensive surgical treatment. Multivariable linear regression revealed a significant correlation of age at neurocognitive testing (-3.18, 95% CI -5.95 to -0.40, p = 0.02), increased ICP (-34.73, 95% CI -51.04 to -18.41, p < 0.01), and the level of maternal education (6.11, 95% CI 1.01-11.20, p = 0.02) with the Full-Scale Intelligence Quotient (FSIQ). Age at surgery, involvement of 2 or more sutures, and type of operation demonstrated no correlation with FSIQ. Among the 97 patients who underwent strip craniectomy, the FSIQ ranged from mean ± SD 100.2 ± 10.2 (bicoronal) to 110.1 ± 12.7 (lambdoid), and there was no significant difference between the suture groups (p = 0.41). The 5 index scores were all within average ranges based on their age norms. CONCLUSIONS: Age at neurocognitive assessment, increased ICP, and maternal education level showed significant correlations with the neurocognitive function of craniosynostosis patients. Although children with craniosynostosis exhibited favorable 5-year postoperative neurocognitive outcomes across various synostosis sutures, longer follow-up is needed to reveal the incidence of neurocognitive dysfunction in these patients.

Roseibium algicola sp. nov. and Roseibium porphyridii sp. nov., isolated from marine red algae.

Two Gram-stain-negative, strictly aerobic rods, designated as RMAR6-6T and KMA01T, exhibiting catalase- and oxidase-positive activities, were isolated from marine red algae in the Republic of Korea. Cells of strain RMAR6-6T exhibited flagellar motility, while those of strain KMA01T were non-motile. Strain RMAR6-6T exhibited optimal growth at 30-35°C and pH 7.0-8.0 with 4.0-6.0 % (w/v) NaCl, while strain KMA01T grew optimally at 30-35 °C, pH 7.0-8.0 and 2.0-5.0% NaCl. Both strains shared common major respiratory isoprenoid quinone (ubiquinone-10), cellular fatty acids (C18 : 0, C18: 1 ω7c 11-methyl, C20 : 1 ω7c and summed feature 8) and polar lipids (phosphatidylglycerol, phosphatidylmonomethylethanolamine, diphosphatidylglycerol, phosphatidylethanolamine, phosphatidylcholine and sulphoquinovosyldiacylglycerol). The genomic DNA G+C contents were 59.0 and 55.0 mol% for strains RMAR6-6T and KMA01T, respectively. With 98.5 % 16S rRNA gene similarity, 75.2 % average nucleotide identity (ANI) and 19.8 % digital DNA-DNA hybridization (dDDH) values, strains RMAR6-6T and KMA01T were identified as representing distinct species. Phylogenetic analyses based on both 16S rRNA gene and genome sequences revealed that strains RMAR6-6T and KMA01T formed distinct phylogenic lineages within the genus Roseibium, most closely related to Roseibium aggregatum IAM 12614T and Roseibium album CECT 5094T, respectively. The ANI and dDDH values between strain RMAR6-6T and R. aggregatum IAM 12614T were 87.5 and 33.3 %, respectively. Similarly, the values between KMA01T and R. album CECT 5094T were 74.2 % (ANI) and 19.3 % (dDDH). Based on phenotypic, chemotaxonomic and molecular characteristics, strains RMAR6-6T and KMA01T represent two novel species of the genus Roseibium, for which the names R. algicola sp. nov. (RMAR6-6T=KACC 22482T=JCM 34977T) and R. porphyridii sp. nov. (KMA01T=KACC 22479T=JCM 34597T) are proposed, respectively.

Parendozoicomonas callyspongiae sp. nov. Isolated from a Marine Sponge, Callyspongia elongate, and Reclassification of Sansalvadorimonas verongulae as Parendozoicomonas verongulae comb. nov.

A strictly aerobic Gram-negative bacterium, designated 2012CJ34-2T, was isolated from marine sponge to Chuja-do in Jeju-island, Republic of Korea and taxonomically characterized. Cells were catalase- and oxidase-positive, and non-motile rods (without flagella). Growth was observed at 15-42 °C (optimum, 30 °C), pH 6-9 (optimum, pH 7), and in the presence of 0.5-10% (w/v) NaCl (optimum, 2-3%). The major cellular fatty acid and respiratory quinones were identified summed feature 3 (C16:1 ω7c/C16:1 ω6c), and Q-8 and Q-9, respectively. The polar lipids comprised diphosphatidylglycerol, phosphatidylglycerol, phosphatidylethanolamine, an unidentified aminophospholipid, two unidentified phospholipids, and three unidentified lipids. The DNA G+C content was 48.0 mol%. Phylogenetic analyses based on 16S rRNA gene and whole genome sequences showed that strain 2012CJ34-2T formed a clade with Parendozoicomonas haliclonae S-B4-1UT and Sansalvadorimonas verongulae LMG 29871T within the family Endozoicomodaceae. Genome relatedness values, including dDDH, ANI and AF, and AAI and POCP, among strain 2012CJ34-2T, P. haliclonae S-B4-1UT, and S. verongulae LMG 29871T were within the range of the bacterial genus cut-off values. Based on the phylogenetic, chemotaxonomic, and genomic analyses, strain 2012CJ34-2T represents a novel bacterial species of the family Endozoicomodaceae, for which the name Parendozoicomonas callyspongiae sp. nov. is proposed. The type strain is 2012CJ34-2T (= KACC 22641T = LMG 32581T). Additionally, we proposed the reclassification of Sansalvadorimonas verongulae of the family Hahellaceae as Parendozoicomonas verongulae of the family Endozoicomonadaceae.

Intramedullary spinal capillary hemangioma with secondary neurulation defect in children.

Intramedullary spinal capillary hemangioma is a rare occurrence in pediatric patients, and only limited cases have been reported. This study presents the first two cases of spinal capillary hemangioma co-present with retained medullary cord and one case of spinal capillary hemangioma with lumbosacral lipomatous malformation. Previous literature on ten patients with this pathology was reviewed. We speculated pathogenesis, imaging features, and histopathologic findings of the disease.

Fibrous hamartoma of infancy of the spinal cord resembling conus and filum, with a coexisting sacral dimple.

Fibrous hamartoma of infancy (FHI) is a rare benign soft tissue lesion of infants and young children. It usually occurs within the first 2 years of life at the superficial layer of the axilla, trunk, upper arm, and external genitalia. FHI in the central nervous system (CNS) is extremely rare. So far, only two spinal cord FHI cases have been reported. We present a case of a 1-month-old girl who presented with a skin dimple in the coccygeal area. Her MRI showed a substantial intramedullary mass in the thoracolumbar area with a sacral soft tissue mass and a track between the skin lesion to the coccygeal tip. Her normal neurological status halted immediate surgical resection. A skin lesion biopsy was first performed, revealing limited information with no malignant cells. A short-term follow-up was performed until the intramedullary mass had enlarged on the 5-month follow-up MRI. Based on the frozen biopsy result of benign to low-grade spindle cell mesenchymal tumor, subtotal resection of the mass was done, minimizing damage to the functioning neural tissue. Both the skin lesion and the intramedullary mass were diagnosed as FHI. Postoperative 5.5-year follow-up MRI revealed minimal size change of the residual mass. Despite being diagnosed with a neurogenic bladder, the patient maintained her ability to void spontaneously, managed infrequent UTIs, and continued toilet training, all while demonstrating good mobility and no motor weakness. This case is unique because the lesion resembled the secondary neurulation structures, such as the conus and the filum, along with a related congenital anomaly of the dimple.

Surgical treatment of interhemispheric arachnoid cysts.

OBJECTIVE: In children, interhemispheric arachnoid cysts (IHACs) are rare lesions often associated with corpus callosum dysgenesis. It is still controversial about surgical treatments for IHACs. We aim to report our experience with pediatric IHAC patients and evaluate surgical courses and neurological developments. METHODS: Pediatric IHACs treated between 2001 and 2021 were reviewed retrospectively. IHAC was observed until they represented rapid cyst enlargement or neurological symptoms. Cyst fenestration was done by microscope or endoscope, depending on the IHAC's location. Cyst size and corpus callosum dysgenesis were evaluated with neuroimaging. Neurological development was assessed from medical records at the last follow-up. RESULTS: Fifteen children received cyst fenestration surgery (mean age 11.4 months). Eleven patients (73.3%) under observation showed rapid cyst enlargement in a short period (median 5 months). Cysto-ventriculostomy (CVS) and cysto-cisternostomy (CCS) regressed the cyst size significantly (p = 0.003). The median follow-up duration was 51 months (range 14-178 months). Corpus callosum dysgenesis was observed in eleven patients (73.3%, complete = 5, partial = 6). Among eight patients (53.3%) having developmental delay, five patients (33.3%) showed speech delay, including one patient with intractable seizures. CONCLUSION: Pediatric IHACs frequently present within 1 year after birth, with rapid cyst enlargement. CVS and CCS were effective in regressing the cyst size. Corpus callosum dysgenesis accompanied by IHAC might have a risk of language achievement; however, development delay could rely on multifactorial features, such as epilepsy or other brain anomalies.

Fulvestrant alleviates cisplatin-induced acute kidney injury via repression of BNIP3-mediated apoptosis and autophagy.

Cisplatin-induced acute kidney injury (AKI) is a clinical disease characterized by a sudden loss of renal function within a few hours or days, due to cisplatin uptake. Fulvestrant is an oestrogen receptor alpha (ERα) antagonist used for endocrine therapy. However, the role of fulvestrant in cisplatin-induced AKI remains unclear. In this study, we investigated the effects of fulvestrant on the regulation of apoptotic cell death and autophagic response in cisplatin-induced AKI. The human kidney proximal tubule epithelial cell line (HK-2) was co-treated with fulvestrant and cisplatin. C57BL/6 mice were subcutaneously injected with fulvestrant and cisplatin was administered via intraperitoneal injection. First, cisplatin treatment increased ERα expression, apoptosis, and autophagy in HK-2 cells. Fulvestrant treatment decreased apoptosis and autophagy, which were accompanied by cisplatin treatment in HK-2 cells. Consistent with in vitro results, cisplatin treatment significantly increased ERα expression in vivo. Additionally, cisplatin treatment increased renal injury, apoptosis, and autophagy. Surprisingly, compared to that in the cisplatin-treated mice group, reduced cisplatin-induced renal injury, apoptosis, and autophagy was observed in the cisplatin+fulvestrant-treated mice group. In summary, these results suggest that fulvestrant plays an important role in cisplatin-induced AKI by decreasing apoptosis and autophagy.

The Outcomes of Endoscopic Suturectomy in Syndromic Craniosynostosis.

OBJECTIVE: Endoscopic suturectomy is a widely practiced surgical option for infants with craniosynostosis. But the efficacy and safety of the procedure remain unclear in syndromic patients. This study aims to evaluate the efficacy and safety of endoscopic suturectomy for patients with syndromic craniosynostosis. METHODS: From January 2013 to December 2020, 242 patients underwent endoscopic suturectomy at our institution. The surgical outcome was determined to be favorable or unfavorable based upon the necessity of an additional cranial surgery upon the last follow-up. First, we analyzed the outcomes of 26 syndromic craniosynostosis patients who have followed up for over a year. Second, we compared the outcomes between the syndromic (N=12) and nonsyndromic (N=11) patients with bilateral coronal synostosis who have followed up for over a year. RESULTS: Twenty-three out of 26 syndromic craniosynostosis patients (88%) showed favorable outcomes without significant complications. In the analysis for bilateral coronal synostosis patients, 11 of 12 syndromic patients (92%) presented favorable outcomes, and all nonsyndromic patients showed favorable outcomes. No significant differences were observed in various anthropometric indices (cranial index, intracranial volume, anterior cranial height, anterior cranial base length, and cranial height-length index) and surgical outcomes between syndromic and nonsyndromic groups. CONCLUSIONS: Endoscopic suturectomy has the potential to be a surgical option for syndromic craniosynostosis. Even for patients with unfavorable outcomes, endoscopic suturectomy could serve as a bridge treatment for infants to counter cranial deformation before additional extensive surgery.

Muricauda myxillae sp. nov., isolated from a marine sponge (Myxilla rosacea), and reclassification of Flagellimonas hymeniacidonis as Muricauda symbiotica nom. nov.

A Gram-stain-negative, aerobic and non-motile rods strain, designated as strain 2012CJ39-3T, was isolated from a marine sponge, Myxilla rosacea, collected on Chuja Island, Republic of Korea. Optimal growth of strain 2012CJ39-3T was observed at 25 °C, pH 7.0 and in the presence of 2.0-3.0 % (w/v) NaCl. Strain 2012CJ39-3T contained menaquinone-6 as the respiratory quinone, iso-C15 : 1 G, iso-C15 : 0, and iso-C17 : 0 3-OH as the predominant fatty acids, and phosphatidylethanolamine, an unidentified phospholipid, an unidentified glycolipid, three unidentified aminolipids and nine unidentified lipids as major polar lipids. The genomic DNA G+C content was 38.4 mol%. Results of phylogenetic analyses based on the 16S rRNA gene and whole-genome sequences revealed that strain 2012CJ39-3T formed a distinct phyletic lineage in the genus Muricauda. Strain 2012CJ39-3T was most closely related to Flagellimonas hymeniacidonis 176CP5-101T, Muricauda spongiicola 2012CJ35-5T, Muricauda algicola AsT0115T, Muricauda flava DSM 22638T and Muricauda parva SW169T with 96.5, 96.4, 96.3, 95.8 and 95.6 % 16S rRNA gene sequence similarity, respectively. The average nucleotide identity and digital DNA-DNA hybridization values between strain 2012CJ39-3T and M. spongiicola 2012CJ35-5T, F. hymeniacidonis 176CP5-101T, M. algicola AsT0115T, M. flava DSM 22638T and M. parva SW169T were 75.6, 74.2, 78.6, 75.3 and 74.8 % and 27.4, 19.9, 36.3, 24.2 and 18.9 %, respectively. Based on these results, strain 2012CJ39-3T represents a novel species of the genus Muricauda, for which the name Muricauda myxillae sp. nov. is proposed. The type strain is 2012CJ39-3T (=KACC 22644T= LMG 32582T). In addition, Flagellimonas hymeniacidonis is reclassified as Muricauda symbiotica nom. nov.

Alishewanella maricola sp. nov., isolated from seawater of the Yellow Sea.

A Gram-stain-negative and facultative aerobic bacterium, strain 16-MAT, was isolated from seawater of Yellow Sea in South Korea. Cells were catalase- and oxidase-positive and non-motile rods. Growth occurred at 4-37 °C (optimum, 30 °C) and pH 6.0-11.0 (optimum, 8.0), and in the presence of 0-7.0% NaCl (optimum, 3 %). Strain 16-MAT contained ubiquinone-8 as the sole isoprenoid quinone, C16 : 0 and summed feature three as the major fatty acids (>10 %), and phosphatidylglycerol, phosphatidylethanolamine, an unidentified phospholipid, an unidentified aminophospholipid, and an unidentified polar lipid as the major polar lipids. The genome size and DNA G+C content of strain 16-MAT were 3.69 Mb and 46.0 mol%, respectively. Strain 16-MAT was most closely related to Alishewanella alkalitolerans LNK-7.1T with a 97.9 % 16S rRNA gene sequence similarity. A phylogenomic tree based on whole genome sequences showed that strain 16-MAT formed a phylogenetic lineage within the genus Alishewanella. Based on the phenotypic, chemotaxonomic, and molecular analyses, strain 16-MAT represents a novel species of the genus Alishewanella, for which the name Alishewanella maricola is proposed. The type strain is 16-MAT (=KACC 22238T =JCM 34596T).

Correlation Between the Severity of Multifidus Fatty Degeneration and the Size of Ossification of Posterior Longitudinal Ligament at Each Spinal Level.

OBJECTIVE: This study aimed to investigate the correlation between ossification of the posterior longitudinal ligament (OPLL) size and multifidus fatty degeneration (MFD), hypothesizing that larger OPLL sizes are associated with worse MFD. METHODS: One hundred four patients with cervical OPLL who underwent surgery were screened. OPLL occupying diameter and area ratios, the severity of MFD using the Goutallier classification, and range of motion (ROM) of cervical flexion-extension (ΔCobb) were measured. Correlation analyses between OPLL size, MFD severity, and ΔCobb were conducted. MFD severity was compared for each OPLL type using one-way analysis of variance. RESULTS: The final clinical data from 100 patients were analyzed. The average Goutallier grade of C2-7 significantly correlated with the average OPLL diameter and area occupying ratios, and OPLL involved vertebral level (r = 0.58, p < 0.01; r = 0.40, p < 0.01; r = 0.47, p < 0.01, respectively). The OPLL size at each cervical level significantly correlated with MFD of the same or 1-3 adjacent levels. ΔCobb angle was negatively correlated with the average Goutallier grade (r = -0.31, p < 0.01) and average OPLL occupying diameter and area ratios (r = -0.31, p < 0.01; r = -0.35, p < 0.01, respectively). Patients with continuous OPLL exhibited worse MFD than those with segmental OPLL (p < 0.01). CONCLUSION: OPLL size is clinically correlated with MFD and cervical ROM. OPLL at one spinal level affects MFD at the same and 1-3 adjacent spinal levels. The worsening severity of MFD is associated with the longitudinal continuity of OPLL.

Surgical indication of pediatric Rathke's cleft cyst based on a 20-year retrospective cohort.

OBJECTIVE: Rathke's cleft cyst (RCC) is the most commonly encountered pituitary incidentaloma in children. Because RCC is not frequently diagnosed in children, there are few reports on pediatric RCCs. The natural course of the disease and appropriate treatments are still obscure. The present study aimed to elucidate the natural history and surgical indications of RCCs in children. METHODS: The authors retrospectively reviewed the clinical presentations, imaging features, ophthalmological evaluations, endocrine evaluations, and surgical outcomes of pediatric RCCs at a single institution from January 2000 to October 2022. Clinical outcomes between the surgery and observation groups were compared. RESULTS: Among 93 patients, there were 41 patients in the surgery group and 52 patients in the observation group. The mean age at diagnosis was 10.9 years, and the mean follow-up period was 5.6 years. Headache fully or partially improved after surgery (86.2%), but the rate of improvement was not different from that of the observation group (70.0%). Ophthalmological abnormalities were effectively improved by surgical treatment (93.3%). Both the improvement and deterioration rates of endocrine abnormalities were significantly higher in the surgery group (p = 0.026 and p < 0.001, respectively), but the deterioration rate (43.9%) was higher than the improvement rate (14.6%). In the surgery group, the recurrence rate was 17.1% and the reoperation rate was 4.9%. Compared with total cyst wall resection, cyst fenestration with partial wall resection was associated with a higher recurrence rate (26.9%, p = 0.035) but a lower rate of endocrine abnormalities (30.8%, p = 0.049). CONCLUSIONS: Pediatric RCCs of ≥ 10 mm in size were analyzed. Ophthalmological abnormalities are the major surgical indications for pediatric RCCs. Headache and partial endocrine abnormalities may be improved with surgery, but they are not absolute indications for surgery. Cyst fenestration with partial wall resection via an endoscopic endonasal approach is the most recommended surgical method. Follow-up is essential to monitor for the occurrence of visual field defects and the recurrence of cysts.

Spontaneous recovery of underlying bony erosion following surgical removal of craniofacial dermoid cysts in periatric patients: A prospective study.

This study aimed to evaluate the spontaneous recovery of bone deformity after surgical excision of craniofacial dermoid cysts in pediatrics. Pediatric patients who underwent excision of a dermoid cyst were included in the study. A prospective analysis was conducted to evaluate the amount of bone recovery by comparing the depth of bony concavity in the preoperative and postoperative (6 months) ultrasonography. In 145 of 187 patients with preoperative imaging available, the mean size of dermoid cysts was 1.4 cm3 (range, 0.1 to 9.5), and 41.4% (60/145 cases) showed cranial bone depression. In the comparison of preoperative and postoperative ultrasonography of 30 patients, the mean depth of bony cavity decreased significantly from 4.0 to 0.9 mm (p<0.001) after a mean of 6.7 months postoperatively. There was 13.3% (4/30) of mild (≤2.0 mm), 40.0% (12/30) of moderate (>2.0 to ≤4.0 mm), and 46.7% (14/30) of severe (>4.0 mm) depression, and the concavity depth significantly decreased in all groups (p = 0.028, mild; p<0.001, moderate; p<0.001 severe). Within the limitations of the study it seems that significant recovery of cranial bone depression does take place within 6 months after excision of craniofacial dermoid cysts in pediatric patients, saving the need for immediate reconstruction.

Alkalicoccobacillus porphyridii sp. nov., isolated from a marine red alga, reclassification of Shouchella plakortidis and Shouchella gibsonii as Alkalicoccobacillus plakortidis comb. nov. and Alkalicoccobacillus gibsonii comb. nov., and emended description of the genus Alkalicoccobacillus Joshi et al. 2022.

A Gram-stain-positive alkali-tolerant and strictly aerobic bacterium, designated strain P16T, was isolated from a marine red alga, Porphyridium cruentum, in the Yellow Sea, Republic of Korea. Cells were motile rods with peritrichous flagella and exhibited catalase and oxidase activities. The optimal growth of strain P16T was observed to occur at 30 °C and pH 8.0 and in the presence of 2.0 % (w/v) NaCl. Menaquinone-7 was identified as the sole respiratory quinone. Strain P16T contained anteiso-C15 : 0, iso-C15 : 0, iso-C14 : 0 and iso-C16 : 0, and diphosphatidylglycerol, phosphatidylglycerol and phosphatidylethanolamine as major cellular fatty acids and polar lipids, respectively. The G+C content of strain P16T was 40.8 mol%. Strain P16T was most closely related to Shouchella plakortidis P203T, Shouchella gibsonii DSM 8722T and Alkalicoccobacillus murimartini LMG 21005T with 98.1, 98.1 and 98.0 % 16S rRNA gene sequence similarities, respectively. Phylogenetic analyses based on 16S rRNA gene and genome sequences revealed that strain P16T, S, plakortidis, S. gibsonii and A. murimartini formed a single phylogenetic lineage cluster, and genomic relatedness analyses showed that they are different species. Based on phylogenetic, phenotypic, chemotaxonomic and molecular features, strain P16T represents a novel species of the genus Alkalicoccobacillus, for which the name Alkalicoccobacillus porphyridii sp. nov. is proposed. The type strain is P16T (=KACC 19520T=JCM 32931T). In addition, S. plakortidis and S. gibsonii are reclassified as Alkalicoccobacillus plakortidis comb. nov. (type strain P203T=DSM 19153T=NCIMB 14288T) and Alkalicoccobacillus gibsonii comb. nov. (type strain PN-109T=ATCC 700164T=DSM 8722T=KCCM 41407T), respectively.

Long-Term Surgical Outcome of Indirect Bypass Surgery in Young Children With Moyamoya Disease.

BACKGROUND: The prognosis of moyamoya disease (MMD) in young children (younger than 4 years) is worse than that of older adults. The effectiveness of surgery is still inconclusive. OBJECTIVE: To evaluate long-term outcomes after indirect bypass in young children with MMD. METHODS: A total of 1417 MMD children underwent indirect bypass from August 1988 to October 2020. This study included 135 patients who were younger than 4 years at the time of surgery. The clinical features and surgical outcomes of these patients were assessed. We analyzed the long-term outcome of 102 children who were followed up for more than 5 years (mean: 18.8 years, range: 5-27.3 years). Cross-sectional analysis was performed to evaluate overall outcomes based on the Lansky Play Performance Scale (LPS). The annual risk of symptomatic stroke after surgery was calculated with a person-year method, and the event-free survival rate was evaluated using the Kaplan-Meier method. RESULTS: The overall clinical outcome was favorable (LPS ≥ 80) in 88% of the patients. The overall postoperative adverse event rate was 15%, including 1 death. At the last follow-up, 86% of patients who had seizures at diagnosis were seizure-free. During the follow-up, there were 3 symptomatic infarctions on the operated hemisphere (postoperative 3, 3, and 10 months each). There was no hemorrhagic event. The annual infarction rate was 0.16% per person-year. The 20-year event-free survival rates for symptomatic infarction were 97%. CONCLUSION: Indirect bypass could provide a satisfactory long-term outcome and prevent recurrent stroke in young children with MMD.

Secondary Neurulation Defects: Retained Medullary Cord.

Retained medullary cord (RMC) is a defect resulting from impaired secondary neurulation. Intraoperatively, RMC is recognizable as an elongated cord-like structure caudal to the conus, that contains histologically confirmed neuroglial components and a lumen with an ependymal lining. It characteristically does not possess neurological function. This chapter aims to summarize (1) the mechanisms that lead to the occurrence of RMC; (2) the various forms of RMC, such as cystic RMC and 'possible RMC', and (3) the treatment strategies, especially untethering through limited exposure.