Exploring Genetic Determinants: A Comprehensive Analysis of Serpin B Family SNPs and Prognosis in Glioblastoma Multiforme Patients.

Serpins are serine proteinase inhibitors, with several serpins being overexpressed in cancer cells. Thus, we aim to analyze the single-nucleotide polymorphism (SNP) of Serpinb11 and its association with GBM survival. A cohort of 63 GBM patients recruited from King Abdullah University Hospital in Jordan underwent polymorphism analysis and overall survival (OS) assessments. The Cancer Genome Atlas (GBM) cohort was useful for validation. We constructed a risk score using the principal component analysis for the following Serpin genes: Serpinb3, Serpinb5, Serpinb6, Serpinb11, and Serpinb12, and patients were grouped into high- vs. low-risk groups based on the median cutoff. Univariable Cox models were used to study the survival outcomes. We identified a significant association between rs4940595 and survival. In the TCGA cohort, Serpinb3 alterations showed worse OS. Univariable Cox showed worse PFS outcomes with higher SERPINB5 and SERPINB6 expression. A Serpin B 5-gene risk score showed a trend towards worse PFS in the high-risk group. Upregulated DEGs showed GO enrichment in cytokine regulation and production, positive regulation of leukocyte activation, and the MAPK cascade. The high-risk group showed a significantly higher infiltration of M2 macrophages and activated mast cells. Our findings showed a significant role of the Serpin B family in GBM survival in the Jordanian population.

Factors Influencing the Choice of Glucose-Lowering Medications Among Physicians Treating Patients With Type 2 Diabetes.

Background The factors considered by physicians when prescribing a glucose-lowering agent to patients with type 2 diabetes (T2D) in real-world settings are not necessarily consistent with those recommended by clinical practice guidelines. Here, we identify the major factors that drive physicians' selection of glucose-lowering agents in the real world and how these factors may differ by physician's specialty.  Methods A web-based survey was conducted among 135 physicians who manage patients with T2D in Saudi Arabia. Physicians were categorized according to their specialty into "specialists" (endocrinologists and/or diabetologists) and "generalists" (internists, family physicians, and primary care physicians). Physicians were asked about the type of glucose-lowering medication that they would typically prescribe in certain clinical scenarios and what factors drive such a selection. Results Sulfonylurea remains the most frequently prescribed second-line agent, as an add-on to metformin, according to 50% of the physicians surveyed. Most physicians (89%) reported prescribing glucagon-like peptide 1 receptor agonists (GLP-1RA) to less than half of their patients with T2D and ischemic heart disease; over two-thirds reported prescribing sodium-glucose cotransporter 2 inhibitors (SGLT-2i) to less than half of their patients with T2D and heart failure. When prescribing GLP-1RAs, the cost was a "major consideration" by 75% and 65% of the specialists and generalists, respectively. Likewise, when prescribing SGLT-2i, the cost was a major consideration by 57% and 71% of the specialists and generalists, respectively. Several other factors differed between the generalists and specialists when prescribing thiazolidinedione (TZD), sulfonylurea, dipeptidyl peptidase 4 (DPP-4) inhibitors, GLP-1RAs, and SLGT-2i, but not insulin.  Conclusion Our findings highlight several challenges faced by physicians in the real world that may prevent them from adopting the latest evidence-based guidelines when managing patients with T2D. Health policies to increase accessibility to novel glucose-lowering agents, particularly for patients with T2D and cardiovascular/renal diseases, are needed.

Persistent pain and its predictors after starting anti-tumour necrosis factor therapy in psoriatic arthritis: what is the role of inflammation control?

OBJECTIVE: While considerable focus has been placed on pain due to inflammation in psoriatic arthritis (PsA), less is reported on pain despite inflammation control. Here, we aimed to investigate the occurrence/predictors of persistent pain, including non-inflammatory components, after starting anti-tumour necrosis factor (anti-TNF) therapy. METHOD: Bionaïve PsA patients starting a first anti-TNF therapy 2004-2010 were identified (South Swedish Arthritis Treatment Group register; N = 351). Outcomes included unacceptable pain [visual analogue scale (VAS) pain > 40 mm], and unacceptable pain despite inflammation control (refractory pain; VAS pain > 40 mm + C-reactive protein < 10 mg/L + ≤ 1 swollen joint of 28), assessed at 0, 3, 6, and 12 months. Baseline predictors were estimated by logistic regression. RESULTS: Upon starting anti-TNF therapy, 85% of patients reported unacceptable pain, falling to 43% at 3 months and then remaining stable. After 12 months, refractory pain constituted 63% of all unacceptable pain. Higher baseline VAS pain/global, worse physical function and lower health-related quality-of-life were associated with a higher risk of unacceptable/refractory pain at 12 months. More swollen joints and higher evaluator's global assessment were associated with a lower risk of 12-month refractory pain. CONCLUSIONS: A substantial proportion of PsA patients reported unacceptable pain throughout the first anti-TNF treatment year. At 12 months, refractory pain constituted about two-thirds of this remaining pain load. More objective signs of inflammation at anti-TNF initiation were associated with less future refractory pain. This highlights insufficient effect of biologics in patients with inflammation-independent pain, warranting alternative treatments.

Prevalence and Risk Factors of Speech Delay in Children Less Than Seven Years Old in Saudi Arabia.

Introduction Verbal communication relies on the ability to speak and understand language. Speech is only one part of language; language can also be expressed through gestures, writing, and other nonverbal means. Speech and language disorders are impairments in the ability to produce and comprehend language, including problems with mouth movements and vocalization. There is a scarcity of literature on this topic in Saudi Arabia; therefore, the aim of this study was to assess the prevalence and risk factors of speech delay in children aged less than seven years in Saudi Arabia. Methods A cross-sectional self-reported descriptive study was conducted from May 2023 to June 2023 among parents of children less than seven years of age in the Kingdom of Saudi Arabia. Simple convenience sampling was implemented. A structured, self-administered questionnaire was designed and presented to the parents of children less than seven years of age. Categorical data were presented as frequencies and percentages. The analysis included a chi-squared test and a Fisher's exact test. Results A total of 617 participants were included in the study. The majority of children were between 1 and 3 years of age (223, 36.1%) and were male (336, 54.5%). Around 45.5% of the respondents reported that their children may have a speech delay. Children aged >3 to 5 years had a significantly higher prevalence of speech delay (112, 53.1%). Additionally, there was a significant difference in speech delay prevalence between male (170, 50.6%) and female (111, 39.5%, p = 0.006) children. A family history of a developmental communication disorder was significantly associated with speech delay (p < 0.001). Children with speech delay were more likely to have hearing issues (19, 70.4%) and motor issues (19, 70.4%). Moreover, autism spectrum disorder in the child was significantly associated with speech delay (p < 0.001). Conclusions The study found that children aged 3 to 5 years had a significantly higher prevalence of speech delay than younger children. There was a significant difference in speech delay prevalence between male and female children. Children with speech delays were more likely to suffer hearing and motor issues. Speech delay was significantly associated with a family history of a developmental communication problem.

Knowledge, Attitude, and Practice of Skin Care Among Elderly Patients in Riyadh, Saudi Arabia.

As people age, the likelihood that they will experience maladies of the skin increases. It is therefore important that older individuals possess the necessary knowledge and proper attitudes and practices regarding their skincare. The purpose of this study was to examine such knowledge, attitudes, and practices among older members of the Saudi Arabian population. The results of the study showed that among the majority of the participants, skincare practices could be considered insufficient and, surprisingly, elderly men undertake better skincare practices than women of this age category. It is recommended that more should be done to educate those within the older Saudi population regarding skin care practices.

The Impact of COVID-19 on Preterm Birth Among Pregnant Women in Al-Qassim, Saudi Arabia.

BACKGROUND: Pregnant women are regarded as a unique group due to the distinct immunological condition that pregnancy produces, which makes pregnant women more susceptible to respiratory infections like coronavirus disease 2019 (COVID-19) and its consequences. During pregnancy, many viral infections have been recognized to increase the risk of adverse obstetrical outcomes such as preterm delivery. The purpose of this study was to investigate the effects of COVID-19 infection on preterm birth in pregnant women in the Al-Qassim region of Saudi Arabia. METHODS:  This retrospective cohort study was conducted in Saudi Arabia between December 2019 to October 2021. The target subjects were pregnant women with live singleton gestations who underwent severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) polymerase chain reaction (PCR) testing for COVID-19 infection during their delivery hospitalization. Data gathered included patient demographic information, clinical characteristics, and pregnancy outcomes. Data were analyzed using R version 4.1.1 (R Core Team (2021); R Foundation for Statistical Computing, Vienna, Austria). RESULTS:  A total of 381 pregnant women were included. The median maternal age of women was 31.0 years (IQR: 27.0, 35.0) and the median BMI value was 30.5 kg/m2 (IQR: 26.9, 34.8). The most common comorbidities were diabetes (7.1%) and asthma (4.5%). A known history of preterm birth was prevalent among 2.9%. Of the participants, 13.6% had a prenatal COVID-19 infection, of whom 57.7% had their infections resolved. The prevalence of positive PCR testing was 13.6%. Preterm birth occurred in 46 women (12.1%, 95%CI 9.1-15.9). Preterm birth was significantly associated with having a maternal age of ≥35 years, having high frequencies of parity, and having a past history of preterm birth, as well as having a history of hypertension and diabetes. Preterm birth was not significantly associated with having a confirmed COVID-19 infection at delivery. CONCLUSION: It was shown that preterm birth is evident among women with COVID-19 infection. Preterm birth is significantly associated with old age, multiparity, and a history of preterm delivery. Preterm birth is not significantly associated with having a confirmed COVID-19 infection at delivery. More research regarding infection-related adverse effects is advised and should be highlighted.

Guillain-Barré Syndrome in Adults in a Decade: The Largest, Single-Center, Cross-Sectional Study From the Kingdom of Saudi Arabia.

BACKGROUND: Guillain-Barré Syndrome (GBS) is the most common cause of acute, usually post-infectious, peripheral neuropathy resulting in a symmetrical, ascending paralysis. We evaluated the clinical and neurophysiological features, treatment, and outcomes of patients with GBS in our center. METHODS: A retrospective chart review on patients with GBS admitted to King Abdulaziz Medical City, Riyadh, Saudi Arabia, from January 2011 to December 2020. Data were analyzed using JMP statistical software version 15 pro. RESULTS: A total of 86 patients who met the criteria were included, 55 (64%) were males, with a mean age of 49.5+/-17.5 years. Antecedent infection was reported in 53 (61.6%), 51 (62.2%) presented within one week of symptoms onset. Ascending weakness was seen in 55 (70.5%), while 70 (81.4%) had areflexia. Acute motor axonal neuropathy (AMAN) was the commonest electrophysiological type of GBS in 41 (51.9%) patients. Albuminocytologic dissociation was seen in 48 (57%) who had lumbar puncture. Nearly half, 41 (47.7%) were admitted to the intensive care unit (ICU). Seventy (81.3%) were treated with intravenous immunoglobulin. There was no significant difference in the clinical presentation, management, ICU requirement, and discharge disposition between males and females. Females were more likely to have a higher disability at discharge (p=0.01). Patients younger than 60 years were more likely to require ICU admission (p=<0.01). CONCLUSION: Our patients with GBS were slightly older than previously reported from the region. AMAN was the commonest type of GBS. Younger patients were more likely to need ICU admission, whereas females were more likely to have a more severe disability.

Prolonged opioid use after distal radius fracture.

BACKGROUND: Prolonged opioid use (more than 90 days) after injury puts the patient at risk for adverse effects. We investigated the patterns of opioid prescription after distal radius fracture and the effect of pre- and post-fracture factors on the risk for prolonged use. METHODS: This register-based cohort study uses routinely collected health care data, including purchases of prescription opioids, in the county of Skåne, Sweden. 9369 adult patients with a radius fracture diagnosed 2015-2018 were followed for 1 year after fracture. We calculated proportions of patients with prolonged opioid use, both in total and according to different exposures. Using modified Poisson regression, we calculated adjusted risk ratios for the following exposures: previous opioid use, mental illness, consultation for pain, surgery for distal radius fracture and occupational/physical therapy after fracture. RESULTS: Prolonged opioid use (4-6 months after fracture) was found in 664 (7.1%) of the patients. A previous, but discontinued, regular use of opioids up to 5 years before fracture increased the risk compared to opioid-naïve patients. Both regular and non-regular opioid use the year before fracture increased the risk. The risk was also higher for patients with mental illness, and those who were treated with surgery, we found no significant effect of pain consultation in previous year. Occupational/physical therapy lowered the risk for prolonged use. CONCLUSION: Considering history of mental illness and previous opioid use while promoting rehabilitation can be important to prevent prolonged opioid use after distal radius fracture. SIGNIFICANCE: We show that a common injury such as distal radius fracture can be a gateway to prolonged opioid use, especially among patients with previous history of opioid use or mental illness. Importantly, previous opioid use as far back as 5 years earlier greatly increases the risk of regular use after the reintroduction of opioids. Considering past use is important when planning treatment with opioids. Occupational or physical therapy after injury is associated with lower risk of prolonged use and should be encouraged.

Surgical Outcomes of Congenital Heart Disease in Down Syndrome: Tertiary Center Experience-Focus on the Electrical Conduction System.

To document outcomes of cardiac surgical repair in Down syndrome (DS) patients with specific focus on the associated electrical conduction morbidities, ultimately leading to a higher incidence of pacemaker implantation (PMI). A retrospective study conducted between 2011 and 2020. A total of 167 DS patients undergoing 204 surgeries were included. The mean gestational age (GA) and mean weight were 37.3 weeks and 5.5 kg, respectively. Complete atrioventricular septal defect (AVSD) was the most common diagnosis. Pre-operative ECG revealed superior axis deviation (SAD) in 92 and 32% of patients with AVSD and isolated perimembranous ventricular septal defect (VSD), respectively (p < 0.01). Postoperative right bundle branch block (RBBB) was observed in 83 and 55% of patients with AVSD and following perimembranous VSD repair, respectively (p = 0.04). Ten patients underwent post-operative pacemaker implantation (PMI). Reintervention rate was around 8.9%. Three mortalities were encountered throughout the study period, 2 of which were in-hospital deaths. Low mortality was observed, however, a higher rate of PMI requirements noted with risk factors including lower age and weight.

The Prevalence of Knee Osteoarthritis and Its Association With Obesity Among Individuals in Saudi Arabia.

Introduction This research, set in 2023 in Saudi Arabia, addresses the rising prevalence of knee osteoarthritis (OA) among obese individuals. It explores associations with age, BMI, and gender, aiming to enhance our understanding of this pressing health issue within the Saudi context and contribute to global research on OA and obesity. Methodology A retrospective cohort study was conducted at King Saud Hospital and Buraydah Central Hospital in the Al-Qassim region from November 2022 to February 2023. It included the patients' records that fit the inclusion criteria during the study period. A data collection sheet was used to collect data, and data was analyzed using SPSS Statistics version 27.0.1 (IBM Corp. Released 2020. IBM SPSS Statistics for Windows, Version 27.0. Armonk, NY: IBM Corp.). Results In 2023, a study on knee OA among 267 obese individuals in Saudi Arabia yielded significant findings. The median age of participants was 60 years, with an average weight of 77.00 kg (IQR:15) and a median BMI of 29.14, categorizing participants as overweight. Ninety percent (n=240) of knee OA cases occurred in individuals classified as "obese" (n=118) or "overweight (n=122)". Furthermore, OA primarily affected both knees in 79.4% (n=212) of cases. Conclusion Study participants, predominantly older and female, reflect OA's age-related and gender-specific prevalence trends. Notably, excess weight and a higher BMI highlight the role of obesity as a significant OA risk factor. Bilateral knee involvement is common, with a strong link between OA and obesity.

The Effect of Sociocultural Attitudes on Developing Eating Disorders Among Young Females in Almadinah Almunawarah, Saudi Arabia.

BACKGROUND: Eating disorders (ED) are believed to be more susceptible in women due to varied factors involving dissatisfaction with their body and appearance. The exact cause of ED isn't known. But it may be triggered by biological, psychological, environmental, and social factors. OBJECTIVES: The current literature aims to explore the body dissatisfaction of women from Almadinah Almunawarah and factors that may contribute to developing risk of ED and assess the discrepancies between desired and healthy BMI. METHODS: The Sociocultural Attitudes Toward Appearance Questionnaire-4 (SATAQ-4) questionnaire surveyed 384 females to explore family, peer, and media pressure, followed by the Eating Attitudes Test-26 (EAT-26) questionnaire to recognize those at risk of developing ED. The body dissatisfaction of the sample was measured by the difference between the healthy BMI and the desired BMI. RESULTS: A total of 127 of the participants, who were reported to have a high probability of developing an ED, had the highest factor scored in the SATAQ-4 questionnaire being media exposure with a p-value less than 0.001. The study showed a difference in the ideal body and what is considered a healthy BMI. Results showed no correlation between BMI and developing ED. DISCUSSION: Women of younger age groups are more vulnerable to being under the influence of sociocultural attitudes, thus they are more susceptible to developing risky eating behaviors. This can be affected by family, peers, and media factors. CONCLUSION: The findings of this study show a high prevalence of risky eating behaviors, particularly among those who experience family and media pressure toward body shape and weight. Peer pressure was also identified as a significant risk factor. These findings emphasize the need for interventions that target sociocultural attitudes and provide support for vulnerable individuals.

Dorsal rhizotomy in cerebral palsy: How root sectioning is influenced by intraoperative neuromonitoring?

INTRODUCTION AND OBJECTIVE: Dorsal rhizotomy is a controversial procedure for treating spasticity in children with cerebral palsy, particularly regarding the influence of intraoperative neuromonitoring (ION). The objective of this study was to evaluate the influence of ION in adjusting root sectioning compared the preoperative program established by the multidisciplinary team. MATERIAL AND METHODS: Twenty-four consecutive children with spastic diplegia or quadriplegia, operated on between 2017 and 2020 in the University Hospital of Nancy, France, were studied. All underwent the same procedure: Keyhole Intralaminar Dorsal rhizotomy (KIDr) with enlarged multilevel interlaminar openings to access all roots from L2 to S2. The Ventral Root (VR) was stimulated to map radicular myotomes, and the Dorsal Root (DR) to test excitability of the segmental circuitry. Muscle responses were observed independently by the physiotherapist and by EMG-recordings. The study compared final root sectioning per radicular level and per side after ION versus the preoperative program determined by the multidisciplinary team. RESULTS: ION resulted in significant differences in final percentage root sectioning (P<0.05), with a decrease for L2 and L3 and an increase for L5. ION modified the symmetry of sectioning, with 32% instead of 5% in preoperative program. Only 5 children showed change in GMFC score 6 months after surgery. CONCLUSION: The use of ION during dorsal rhizotomy led to important modifications of root sectioning during surgery, which justifies individual control of each root, level by level and side by side, to optimize the therapeutic effect.

Ophthalmological lesions in shaken baby syndrome: A retrospective analysis of 133 consecutive cases (1992-2018).

INTRODUCTION: Shaken Baby Syndrome (SBS) is a non-accidental head trauma in which shaking causes cranio-cerebral lesions. Shaking can lead to ophthalmologic lesions such as retinal hemorrhage (RH). The aim of the present study was to compare our long-term results in to the literature data. PATIENTS AND METHODS: This study was a single-center retrospective descriptive analysis of 133 consecutive SBS cases (1992-2018). Only seniors in ophthalmology were authorized to perform these examinations. We studied type of lesion (retinal, intra-vitreal, papilledema), location (uni- or bi-lateral), and correlation with gender and age. Infants with a traumatic context without suspicion of child abuse were excluded. RESULTS: Mean age at diagnosis was 131days (range, 14days-10months). Boys accounted for 72.2% of the population. The prevalence of ophthalmologic lesions was 70.3%. 94.4% were RH; intra-vitreous hemorrhage (6.7%) and papilledema (11.1%) were less frequent. Lesions were bilateral in 81.1% of cases. Retinal lesions were classified in terms of location. Macular involvement was diagnosed in 8.2% of cases. 18.8% of retinal lesions could not be classified because of lack of precision in the ophthalmology report. The prevalence of ophthalmic lesions was higher for children aged over 6months: 80%. CONCLUSION: This series highlighted a high rate of ophthalmic lesions in SBS, with a high rate of bilateral involvement. RH was the most frequent lesion. RH in a context of subdural hematoma is a strong argument in favor of SBS. The forensic implications are that rigorous ophthalmologic examination by a senior practitioner is mandatory.

Prenatal neurosurgical counseling for conditions affecting the fetal nervous system.

OBJECTIVE: The aim of this study was to share a single center's experience of prenatal neurosurgical counseling and explore pregnant women's experiences with counseling. MATERIAL AND METHODS: This retrospective study analyzed data for 81 women who received prenatal counseling in a single institution (same senior pediatric neurosurgeon) over a 6-year period. Additionally, a retrospective questionnaire study was conducted with 33 women who chose to continue their pregnancy, to assess the strengths and weaknesses of counseling and analyze the reasons for their decision. RESULTS: Spinal dysraphism was the most frequent condition leading to prenatal counseling, followed by conditions affecting the cerebrospinal fluid. 57.6% of the women did not follow the French national recommendations on folic acid supplementation in the periconceptional period, and 38.3% underwent termination of pregnancy (TOP). One-third of the 33 women who answered our questionnaire changed their mind about TOP after counseling, and 50% reported that the information provided influenced their decision. CONCLUSION: Prenatal neurosurgical counseling is nowadays an important part of a pediatric neurosurgeon's practice. It provides specific information to the woman to decide whether to continue the pregnancy. Urological concerns are frequent among the malformations encountered. Hence, we conclude that these women should be offered the possibility of seeing a urologist. Areas for improvement include greater awareness regarding folic acid supplementation and improved psychological care. The advantage for a woman of consulting a neurosurgeon consists in receiving information that is as accurate as possible about the level of disability of the future child and about surgery and follow-up.

Pediatric dental treatments with pharmacological and non-pharmacological interventions: a cross-sectional study.

OBJECTIVES: Behaviour management strategies involving pharmacological or non-pharmacological interventions during dental procedures should be considered to attain safe and successful treatment outcomes. This study compared the frequencies of use and the completeness of treatment with these interventions. METHODS: A total of 1725 dental records of patients up to 18 years old, who were treated in the King Abdulaziz Medical City in Jeddah City from October 2018 to June 2019, were used in this retrospective, cross-sectional study. Inferential analysis, Chi-square test, Kruskal-Wallis test, and regression model were used in the data analysis. RESULTS: About two-thirds of the patients were treated with attendant non-pharmacological interventions, while one-third, with pharmacological interventions. The application of General Anesthesia (GA) was the most frequently used intervention. Restorative procedures and extractions were done in higher frequencies with pharmacological interventions. Treatments with space maintainers and orthodontic appliances were carried out in higher frequencies with non-pharmacological strategies. The choice of intervention was significantly influenced by the systemic conditions of the patients. Patients treated with non-pharmacological intervention comprised the dominant type of patients, because they required treatments with less pain. Those treated with GA needed restorative treatments and extractions, or treatments that involve pain, but these treatments had higher frequencies of being completed. CONCLUSIONS: The treatments with pharmacological intervention through GA have higher frequencies of being completed, compared to those with non-pharmacological interventions. Factors, such as age, potential to complete the treatment, and the type of dental treatment applied, influence the choice of treatment intervention.

Surgical approach to the posterior fossa in children, including anesthetic considerations and complications: The prone and the sitting position. Technical note.

INTRODUCTION: Medulloblastoma (MB) in children is, in most cases, a midline lesion located in the posterior fossa, with a high tendency to invade the fourth ventricle (and sometimes its floor). METHODS: In this technical note, we present both the sitting and the prone position for children with medulloblastoma. These positions will be described together by deliberately deciding not to oppose one against the other. The park bench position, seldom used in pediatric neurosurgery is not described here. The latter procedure can be useful, however, for MB located more laterally or within the cerebellopontine angle. RESULTS: The aim of this technical note is to provide the reader with a step by step procedure (from installation to closure), at least from the author's point of view. A special focus regarding anesthetic considerations is also provided. This anesthesia requires perfect coordination and communication between both the anesthetic and surgical teams. Complications of posterior fossa surgery are mentioned as well. Some of the advantages and disadvantages of each positions are mentioned. CONCLUSION: Only careful installation, respect of certain surgical principles (careful attention of the vermis and dentate nuclei for example), knowledge of anatomy, closure and perioperative monitoring, screening and immediate correction of potentially devastating complications will enable surgeons to obtain the best results from their surgery.

Cerebellar swelling after surgery for medulloblastoma with leptomeningeal dissemination in children. A case based-update.

OBJECT: Despite the improvement in the overall management of medulloblastomas in recent years, certain phenomena and in particular postoperative cerebellar swelling remain an enigma. This rare complication, little described in the literature, is nonetheless life threatening for the patients. CASE REPORTS: We report our experience about two children who developed severe cerebellar swelling with hydrocephalus and upward herniation soon after a gross total resection of a fourth ventricle medulloblastoma by a telo-velar approach. Despite rapid management of ventricular dilation and optimal medical intensive treatment of intracranial hypertension, both children died quickly after the surgery. Pathological examination analyses were in favour of anaplastic/large cell medulloblastoma. DISCUSSION: Diffuse cerebellar swelling with upward herniation may occur postoperatively in young children with anaplastic/large cell medulloblastoma with leptomeningeal spread. In the literature, only 4 cases have been so far described with delayed onset of symptoms. Two children survived with an aggressive management (decompressive surgery and early radio-chemotherapy). CONCLUSION: Cerebellar swelling is an unrecognised and sudden complication of posterior fossa surgery for metastatic anaplastic medulloblastoma with leptomeningeal dissemination in young children. An initial less invasive surgical approach could be considered in such cases, in order to prevent this complication with potentially tragic issue, and which cannot be managed with a CSF shunt alone.

Optic pathway tumor in children: Toward a new classification for neurosurgical use.

OBJECT: Optic pathway tumors (OPT) represent a challenge for pediatric neurosurgeons. Role of surgery is debated due to the high risk of iatrogenic damage, and in lasts decades it lost its importance in favor of chemotherapy. However, in some cases surgery is necessary to make biomolecular and histological diagnosis, to manage intracranial hypertension (IH) and to cooperate with medical therapies in controlling tumor relapse. With the aim to standardize selection of surgical OPT cases, we propose a simple, practical and reproducible classification. METHODS: We retrospectively analyzed data of 38 patients with OPT treated at our institution (1990-2018). After careful analysis of MRI images, we describe a new classification system. Group 1: lesion limited to one or both optic nerve(s). Group 2: chiasmatic lesions extending minimally to hypothalamus. Group 3: hypothalamo-chiasmatic exophitic lesions invading the third ventricle; they can be further divided on the base of concomitant hydrocephalus. Group 4: hypothalamo-chiasmatic lesions extending widely in lateral direction, toward the temporal or the frontal lobes. Patients' data and adopted treatment are reported and analyzed, also depending on this classification. RESULTS: Twenty children were operated on for treatment of OPT during the study period. Permanent clinical impairment was noted in 5 (25%) of operated patients, while visual improvement was noted in 1 patient. OS rate was 100% at 5 years, with a median follow up of 9 years (ranging from 2 to 23). Prevalence of intracranial hypertension and proportion of first-line surgical treatment decision were significantly higher in groups 3-4 compared to groups 1-2 (P<0.001 for both tests). CONCLUSION: Surgery can offer a valuable therapeutic complement for OPT without major risk of iatrogenic damage. Surgery is indispensable in cases presenting with IH, as in groups 3 and 4 lesions. Eligibility of patients to surgery can be based on this new classification system.

Diffuse infiltrative pontine glioma biopsy in children with neuronavigation, frameless procedure: A single center experience of 10 cases.

INTRODUCTION: This study presented pediatric DIPG 's biopsy with frameless Neuronavigation. PATIENTS AND METHODS: We report our experience about 10 patients who had Diffuse Intrinsic Pontine Glioma between 2014 and 2018. All patients were biopsied with BrainLab Varioguide Neuronavigation®. We always used fusion between specific CT Scan and MRI to selected target, made planning and biopsies. All patients were included in BIOMEDE after scientific and ethic discussions. We always selected a trans-cerebellar trajectory and made same procedure (lot of biopsies at one level). All patients have MRI at J1 to verify site of biopsy and to eliminate complication. RESULTS: The average age was 8.1 years. Symptoms were common with principally headaches and nystagmus. All biopsies were contributive for histopathological diagnosis and establish molecular profile for molecular study. We have no definitive morbidity and procedure duration was 93minutes in average. All MRI didn't showed intracranial complication after procedure and showed great precision of biopsy compared with the selected target. DISCUSSION: We reviewed the literature and compare our results with series of DIPG biopsies using stereotactic frame or robotic assisted frameless. It was a safe, accuracy and easiness procedure. We always have histopathological and molecular result to proceed next step of treatment. This modality is an alternative possibility to biopsy very young patients with low morbidity.

Neurophysiological mechanisms of hypertonia and hypotonia in children with spastic cerebral palsy: surgical implications.

Mechanism of hypertonia in cerebral palsy children is dual: a neural component due to spasticity (velocity dependent) and a biomechanical component linked to soft tissue changes. Their differentiation-which might be clinically difficult-is however crucial, as only the first component will respond to anti-spastic treatments, the second to physiotherapy. Furthermore, spasticity is frequently associated with dystonia, which is a sustained hypertonic state induced by attempts at voluntary motion. Spasticity and dystonia have to be carefully distinguished as dorsal rhizotomy will not significantly influence the dystonic component. Spasticity, which by definition opposes to muscle stretching and lengthening, has two important consequences. First, the muscles tend to remain in a shortened position, which in turn results in soft tissue changes and contracture. The second is that movements are restricted. Thus, both hypertonia and lack of mobilization create a vicious circle leading to severe locomotor disability linked to irreducible musculotendinous retraction and joint ankylosis/bone deformities. These evolving consequences should be highly considered during the child's assessment for decision-making. The hypotonic effects of lumbosacral dorsal rhizotomy, which are not only segmental on the lower limbs but also supra-segmental through the reticular formation, are finally discussed.