SURGICAL TREATMENT OF COMPLEX CROUZON SYNDROME CRANIOSYNOSTOSIS

Crouzon syndrome is one of the most common syndromic craniosynostosis. We present complex course of treatment in a child with Crouzon syndrome presented with multisuture craniosynostosis and hydrocephalus. A 3-months-old boy was admitted with clinical signs of hydrocephalus and turribrachycephaly. Firstly, the hydrocephalus was addressed. The ventriculoperitoneal shunt with programmable valve was implanted. Secondly, in May 2017, posterior calvarial remodeling was done. Finally, after the child recovered, anterior calvarial remodeling with fronto-orbital advancement was performed.  This completed the calvarial remodeling in this patient. Child is doing well, so far completing milestones of child development in time. This case presents an extremely complex case of syndromic multisuture craniosynostosis, in which it was necessary to perform the entire calvarial remodeling to correct the deformity and to provide a chance to the brain to grow and develop without constrictions

The Prevalence of Spine Deformities and Flat Feet Among 10-12 Year Old Children Who Train Basketball – Cross-Sectional Study

The aim of this study is to estimate the prevalence of spine and feet deformities among children who are regularly involved in basketball trainings, as well as finding differences in the prevalence of those deformities between children of different gender and age. The study included a total of 64 children, of which 43 were boys and 21 were girls, ages 10-12. All subjects have been regularly participating in basketball trainings for at least one year. Postural disorder is defined as an irregularity in posture of the spine and feet, and it is assessed by visual methods from the front, side and rear side of the body. The prevalence of spinal deformities in our group was 53.13%. The boys had a significantly higher prevalence than girls, 65.1% compared to 28.57% (p=0.006). There was no significant difference in prevalence of spine deformities between children of different ages. The prevalence of feet deformities was 64.06%. There was a statistically significant difference between the sexes, where boys had a significantly greater prevalence of the feet deformities than girls, 83.7% compared to 23.81% (p=0.001). Flat feet were the most common in 10 year old children (85.71%). In conclusion, it can be said that despite regular participation in basketball training, subjects in this study have high prevalence of deformities; especially boys who stand out with the high prevalence of flat feet

Cavernous Malformations of the Central Nervous System: An International Consensus Statement

Introduction: Cavernous malformations (CM) of the central nervous system constitute rare vascular lesions. They are usually asymptomatic, which has allowed their management to become quite debatable. Even when they become symptomatic their optimal mode and timing of treatment remains controversial. Research question: A consensus may navigate neurosurgeons through the decision-making process of selecting the optimal treatment for asymptomatic and symptomatic CMs. Material and methods: A 17-item questionnaire was developed to address controversial issues in relation to aspects of the treatment, surgical planning, optimal surgical strategy for specific age groups, the role of stereotactic radiosurgery, as well as a follow-up pattern. Consequently, a three-stage Delphi process was ran through 19 invited experts with the goal of reaching a consensus. The agreement rate for reaching a consensus was set at 70%. Results: A consensus for surgical intervention was reached on the importance of the patient’s age, symptomatology, and hemorrhagic recurrence; and the CM’s location and size. The employment of advanced MRI techniques is considered of value for surgical planning. Observation for asymptomatic eloquent or deep-seated CMs represents the commonest practice among our panel. Surgical resection is considered when a deep-seated CM becomes symptomatic or after a second bleeding episode. Asymptomatic, image-proven hemorrhages constituted no indication for surgical resection for our panelists. Consensus was also reached on not resecting any developmental venous anomalies, and on resecting the associated hemosiderin rim only in epilepsy cases. Discussion and conclusion: Our Delphi consensus provides an expert common practice for specific controversial issues of CM patient management

In Memoriam: A Memoir for Our Fallen "Heroes"

This article is made available for unrestricted research re-use and secondary analysis in any form or be any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.Even though neurosurgeons exercise these enormous and versatile skills, the COVID-19 pandemic has shaken the fabrics of the global neurosurgical family, jeopardizing human lives, and forcing the entire world to be locked down. We stand on the shoulders of the giants and will not forget their examples and their teachings. We will work to the best of our ability to honor their memory. Professor Harvey Cushing said: “When to take great risks; when to withdraw in the face of unexpected difficulties; whether to force an attempted enucleation of a pathologically favorable tumor to its completion with the prospect of an operative fatality, or to abandon the procedure short of completeness with the certainty that after months or years even greater risks may have to be faced at a subsequent session—all these require surgical judgment which is a matter of long experience.” It is up to us, therefore, to keep on the noble path that we have decided to undertake, to accumulate the surgical experience that these icons have shown us, the fruit of sacrifice and obstinacy. Our tribute goes to them; we will always remember their excellent work and their brilliant careers that will continue to enlighten all of us. This memorial is intended to commemorate our colleagues who succumbed during the first 4 months

Familial hypercholesterolaemia in children and adolescents from 48 countries: a cross-sectional study

Background: Approximately 450 000 children are born with familial hypercholesterolaemia worldwide every year, yet only 2·1% of adults with familial hypercholesterolaemia were diagnosed before age 18 years via current diagnostic approaches, which are derived from observations in adults. We aimed to characterise children and adolescents with heterozygous familial hypercholesterolaemia (HeFH) and understand current approaches to the identification and management of familial hypercholesterolaemia to inform future public health strategies. Methods: For this cross-sectional study, we assessed children and adolescents younger than 18 years with a clinical or genetic diagnosis of HeFH at the time of entry into the Familial Hypercholesterolaemia Studies Collaboration (FHSC) registry between Oct 1, 2015, and Jan 31, 2021. Data in the registry were collected from 55 regional or national registries in 48 countries. Diagnoses relying on self-reported history of familial hypercholesterolaemia and suspected secondary hypercholesterolaemia were excluded from the registry; people with untreated LDL cholesterol (LDL-C) of at least 13·0 mmol/L were excluded from this study. Data were assessed overall and by WHO region, World Bank country income status, age, diagnostic criteria, and index-case status. The main outcome of this study was to assess current identification and management of children and adolescents with familial hypercholesterolaemia. Findings: Of 63 093 individuals in the FHSC registry, 11 848 (18·8%) were children or adolescents younger than 18 years with HeFH and were included in this study; 5756 (50·2%) of 11 476 included individuals were female and 5720 (49·8%) were male. Sex data were missing for 372 (3·1%) of 11 848 individuals. Median age at registry entry was 9·6 years (IQR 5·8-13·2). 10 099 (89·9%) of 11 235 included individuals had a final genetically confirmed diagnosis of familial hypercholesterolaemia and 1136 (10·1%) had a clinical diagnosis. Genetically confirmed diagnosis data or clinical diagnosis data were missing for 613 (5·2%) of 11 848 individuals. Genetic diagnosis was more common in children and adolescents from high-income countries (9427 [92·4%] of 10 202) than in children and adolescents from non-high-income countries (199 [48·0%] of 415). 3414 (31·6%) of 10 804 children or adolescents were index cases. Familial-hypercholesterolaemia-related physical signs, cardiovascular risk factors, and cardiovascular disease were uncommon, but were more common in non-high-income countries. 7557 (72·4%) of 10 428 included children or adolescents were not taking lipid-lowering medication (LLM) and had a median LDL-C of 5·00 mmol/L (IQR 4·05-6·08). Compared with genetic diagnosis, the use of unadapted clinical criteria intended for use in adults and reliant on more extreme phenotypes could result in 50-75% of children and adolescents with familial hypercholesterolaemia not being identified. Interpretation: Clinical characteristics observed in adults with familial hypercholesterolaemia are uncommon in children and adolescents with familial hypercholesterolaemia, hence detection in this age group relies on measurement of LDL-C and genetic confirmation. Where genetic testing is unavailable, increased availability and use of LDL-C measurements in the first few years of life could help reduce the current gap between prevalence and detection, enabling increased use of combination LLM to reach recommended LDL-C targets early in life

Sitting position awake craniostomy with drainage for chronic subdural hematoma: a viable alternative?

Background/Aim. Chronic subdural hematoma (CSDH) is one of the most frequent neurosurgical conditions with an overall incidence ranging from 1.72 to 20.6 per 100,000 persons per year. The surgical procedure for CSDH is relatively simple and usually performed in the supine position. Reported reoccurrence rates range from 11.7% to 28%. Postperative pneumocephalus was previously identified as a sole predictor of reoccurrence. The aim of this study was to assess the advantage of the procedure in the sitting position in patients with CSDH and a possible impact on the reoccurrence rate. Methods. The study included 31 patients who underwent awake craniostomy with closed system drainage for CSDH (16 in supine and 15 in sitting position) in our department from December 2016 to March 2018. Results. A total of 22 males and 9 females were included in the study. The overall reoccurrence rate was 19% (22% and 18% in females and males, respectively). The reoccurrence was noted in 5 patients who had undergone surgery in the supine position, and in one case in the sitting position. Our results revealed a lower reoccurrence rate in patients undergoing surgery in the sitting position, although not reaching statistical significance [ odds ratio (OR): 0.18, 95% confidence interval: 0.01-1.42, p = 0.172]. Conclusion. Craniostomy in the sitting position under local anesthesia is a safe, simple, and reliable procedure for CSDH treatment. Besides being very comfortable for the patient, according to our initial results, it might also lead to a lower reoccurrence rate, probably due to the better management of the air inflow, and consequent pneumocephalus

Global neurosurgery amongst the EANS community: where are we at?

Antibody-drug conjugates (ADC) are antineoplastic agents recently introduced into the antitumor arsenal. T-DM1, a trastuzumab-based ADC that relies on lysosomal processing to release the payload, is approved for HER2-positive breast cancer. Next-generation ADCs targeting HER2, such as [vic-]trastuzumab duocarmazine (SYD985), bear linkers cleavable by lysosomal proteases and membrane-permeable drugs, mediating a bystander effect by which neighboring antigen-negative cells are eliminated. Many antitumor therapies, like DNA-damaging agents or CDK4/6 inhibitors, can induce senescence, a cellular state characterized by stable cell-cycle arrest. Another hallmark of cellular senescence is the enlargement of the lysosomal compartment. Given the relevance of the lysosome to the mechanism of action of ADCs, we hypothesized that therapies that induce senescence would potentiate the efficacy of HER2-targeting ADCs. Treatment with the DNA-damaging agent doxorubicin and CDK4/6 inhibitor induced lysosomal enlargement and senescence in several breast cancer cell lines. While senescence-inducing drugs did not increase the cytotoxic effect of ADCs on target cells, the bystander effect was enhanced when HER2-negative cells were cocultured with HER2-low cells. Knockdown experiments demonstrated the importance of cathepsin B in the enhanced bystander effect, suggesting that cathepsin B mediates linker cleavage. In breast cancer patient-derived xenografts, a combination treatment of CDK4/6 inhibitor and SYD985 showed improved antitumor effects over either treatment alone. These data support the strategy of combining next-generation ADCs targeting HER2 with senescence-inducing therapies for tumors with heterogenous and low HER2 expression. Significance: combining ADCs against HER2-positive breast cancers with therapies that induce cellular senescence may improve their therapeutic efficacy by facilitating a bystander effect against antigen-negative tumor cells