Venous Thromboembolism Risk and Thromboprophylaxis in Pediatric Neurosurgery and Spinal Injury: Current Trends and Literature Review.

Although the entities of venous thromboembolism (VTE), deep venous thrombosis, pulmonary embolus, and thromboprophylaxis in adult patients undergoing brain tumor and spine surgery, traumatic brain injury and elective neurosurgical procedures are widely elucidated, the same is not valid when pediatric patients are under consideration. An attempt to review the peculiarities of these patients through a comprehensive bibliographic review is undertaken. We performed a narrative summary of the relevant literature dedicated to pediatric patients, centered on traumatic brain injury, the general incidence of thromboembolic disease in this patient population, the role of low molecular weight heparin (LMWH) in the treatment and prophylaxis of VTE, and its role in elective neurosurgical procedures, including spinal operations. Additionally, the risk of deep venous thrombosis in elective neurosurgical procedures is reviewed. Due to inherent limitations of the current studies, particularly a restricted number of patients, our data are underpowered to give a definitive protocol and guidelines for all the affected patients. Our current conclusions, based only on pediatric patients, argue that there is limited risk of VTE in pediatric patients suffering from brain tumors and that the possibility of VTE is very low in children undergoing elective neurosurgical procedures. There is no consensus regarding the exact incidence of VTE in traumatic brain injury patients. LMWH seems to be a safe and effective choice for the "at risk" pediatric patient population defined as being older than 15 years, venous catheterization, nonaccidental trauma, increased length of hospital stays, orthopaedic (including spinal) surgery, and cranial surgery.

The Entity of Cerebellar Mutism Syndrome: A Narrative Review Centered on the Etiology, Diagnostics, Prevention, and Therapeutic Options.

Cerebellar mutism syndrome (CMS), also known as posterior fossa syndrome, is an entity that entails a constellation of signs and symptoms which are recorded in a limited number of pediatric patients who have been operated on mainly for tumors involving the posterior cranial fossa, and more precisely, the region of the vermis. Medulloblastoma seems to constitute the most commonly recognized pathological substrate, associated with this entity. The most prevalent constituents of this syndrome are noted to be a, often transient, although protracted, language impairment, emotional lability, along with cerebellar and brainstem dysfunction. Apart from that, a definite proportion of involved individuals are affected by irreversible neurological defects and long-lasting neurocognitive impairment. A bulk of literature and evidence based on clinical trials exist, which reflect the continuous effort of the scientific community to highlight all perspectives of this complex phenomenon. There are several circumstances that intervene in our effort to delineate the divergent parameters that constitute the spectrum of this syndrome. In summary, this is implicated by the fact that inconsistent nomenclature, poorly defined diagnostic criteria, and uncertainty regarding risk factors and etiology are all constituents of a non-well-investigated syndrome. Currently, a preliminary consensus exists about the identification of a group of diagnostic prerequisites that are managed as sine qua non, in our aim to document the diagnosis of CMS. These include language impairment and emotional lability, as proposed by the international Board of the Posterior Fossa Society in their consensus statement. It is common concept that midline tumor location, diagnosis of medulloblastoma, younger age at diagnosis, and preoperatively established language impairment should be accepted as the most determinant predisposing conditions for the establishment of this syndrome. A well-recognized pathophysiological explanation of CMS includes disruption of the cerebellar outflow tracts, the cerebellar nuclei, and their efferent projections through the superior cerebellar peduncle. Despite the relative advancement that is recorded regarding the diagnostic section of this disease, no corresponding encouraging results are reported, regarding the available treatment options. On the contrary, it is mainly targeted toward the symptomatic relief of the affected individuals. The basic tenet of our review is centered on the presentation of a report that is dedicated to the definition of CMS etiology, diagnosis, risk factors, clinical presentation, and clinical management. Apart from that, an effort is made that attempts to elucidate the paramount priorities of the scientific forum, which are directed toward the expansion our knowledge in the era of diagnostics, prevention, and therapeutic options for patients suffering from CM, or who are at risk for development of this syndrome.

Slit ventricle syndrome: Historical considerations, diagnosis, pathophysiology, and treatment review.

After the introduction of shunt treatment for the management of childhood hydrocephalus, a wide variety of complications related to this treatment modality have been recognized. The entity of slit ventricle syndrome (alternatively, symptomatic ventricular coaptation) is one of them, is frequently encountered in the pediatric population and its symptom complex resembles that of shunt failure. We conducted research on PubMed®, MEDLINE®, and Web of Science®, using the keywords: "slit ventricles," "slit ventricle syndrome," "SVS" and "ventricular coaptation." The aim of our review was to trace the advances made through the past decades, concerning our knowledge about the clinical characteristics, pathophysiology, and treatment options of this entity. The discrepancy among researchers about the offending etiology and the optimum treatment algorithm of this entity, as well as the necessity of an updated concept regarding shunt over drainage is analyzed. The multiple treatment modalities proposed and pathophysiologic mechanisms implicated for the treatment of slit ventricle syndrome illustrate the complexity of this entity. Consequently, the issue requires more detailed evaluation. In this review, we comment on all the main facets related to shunt over drainage and the resultant slit ventricle syndrome.

The entity of the trapped fourth ventricle: A review of its history, pathophysiology, and treatment options.

An isolated or trapped fourth ventricle is a relatively rare, although serious, adverse effect of hemorrhagic, infectious, or inflammatory processes that involve the central nervous system. This entity usually occurs after successful shunting of the lateral ventricles and may become clinically evident with the development of delayed clinical deterioration. This decline of the neurological status of the patient is evident after an initial period of improvement of the relevant symptoms. Surgical treatment options include cerebrospinal fluid shunting procedures, along with open surgical and endoscopic approaches. Complications related to its management are common and are related with obstruction of the fourth ventricular catheter, along with cranial nerve or brainstem dysfunction. We used the keywords: "isolated fourth ventricle," and "trapped fourth ventricle," in PubMed® and Web of Science®. Treatment of the trapped fourth ventricle remains a surgical challenge, although the neurosurgical treatment armamentarium has broadened. However, prompt recognition of the clinical and neurological findings that accompany any individual patient, in conjunction with the relevant imaging findings, is mandatory to organize our treatment plan on an individual basis. The current experience suggests that any individual intervention plan should be mainly based on the underlying pathological substrate of hydrocephalus. This could help us to preserve the patient's life, on an emergent basis, as well as to ensure an uneventful neurological outcome, maintaining at least the preexisting level of neurological function.

Fetal subependymal giant cell astrocytoma: A case report and review of the literature.

BACKGROUND: Subependymal giant cell astrocytomas (SEGAs) appear approximately in 10% of patients with tuberous sclerosis. These tumors are most commonly diagnosed in childhood and adolescence, with in utero diagnosed SEGAs being an extremely rare entity. CASE DESCRIPTION: We present the case of a congenital SEGA detected in an antenatal ultrasound and further investigated with fetal magnetic resonance imaging (MRI) scans at 22 and 32 weeks of gestational age. At 9 days of age, the child underwent craniotomy and partial excision of the tumor, followed by a second more extensive operation 13 days later. The patient was subsequently administered mammalian target of rapamycin inhibitor (everolimus). CONCLUSION: In the latest follow-up MRI, at the age of two, the SEGA remained unchanged. Management of these tumors in neonates is challenging, mainly due to high morbidity and mortality of surgical treatment in these ages.

The 100 Most Cited Papers About Brain Metastases.

BACKGROUND: A vast amount of articles centered on brain metastases have been published. OBJECTIVE: To present the 100 most-cited articles dedicated to brain metastasis and to accomplish a broad literature review. METHODS: In December 2019, we performed a title-focused search using the Thomson Reuters Web of Science database to identify the most cited articles centered on brain metastatic disease. Our search query term was based on using the following algorithm: "brain metastases" OR "brain metastasis" OR "brain metastatic disease" OR "cerebral metastases" OR "cerebral metastasis" OR "cerebral metastatic disease." Afterward, we reviewed the results to certify that they were relevant to the purposes of our research protocol. The 100 most cited papers were chosen and further analyzed. RESULTS: Our search resulted in 11,579 articles, published from 1975 until the completion of our survey. The most cited article, by Patchell et al., was published in 1990, with 1862 citations, and an average of 62.07 citations per year, whereas the last in our list, by Gaspar et al., was published in 2010, with 195 total citations, and an average of 19.50 citations per year. Countries with the highest-cited articles included the United States (75 records), followed by Canada (16 records). CONCLUSIONS: We discovered the top 100 most-cited articles centered on brain metastasis, all of which show a potentially increased level of interest, because they are meaningful scientific reports. In addition, we reviewed the historical development and advances in brain metastasis research and relevant points of interest, alongside the relevant contributions of different authors, fields of special interest, and countries. Many of the most cited articles were written by authors whose specialty was not neurosurgery or by neurosurgeons who were supported by colleagues from other medical fields. As a consequence, many of these articles were not published in neurosurgery-dedicated journals.

Postoperative Tetraplegia to a Child after Cerebellar Pilocytic Astrocytoma Excision at Prone Position: Case Report and Literature Review.

BACKGROUND Various factors have been implicated in the pathogenesis of infarction after posterior fossa surgery such as venous air embolism, patient's position (seated or prone), hyperflexion of the neck, excessive spinal cord traction, cervical canal stenosis, and systemic arterial hypotension. The main aim of this case report was to elucidate a case in which hydrogen peroxide was implicated in a major and systemic complication after a neurosurgical procedure. CASE REPORT We describe the case of a 5-year-old female patient who was admitted to our hospital because of a cerebellar hemispheric astrocytoma associated with obstructive hydrocephalus and accompanied by 2 syringomyelic cavities in the cervicothoracic portion of the spinal cord. Immediately after gross total resection of the lesion, impaired mobility of the upper and lower extremities was observed, a finding that was not consistent with intraoperative neurophysiologic monitoring data. Hydrogen peroxide had been judiciously used to irrigate the resection tumor cavity. In the next few postoperative days, the patient suffered from transient diabetes insipidus and hyperpyrexia, indicative of hypothalamic injury. CONCLUSIONS Neurological evaluation of the patient, after stabilization of her medical condition, revealed residual spasticity of upper and lower extremities, rendering her able to mobilize via the aid of wheelchair only. The most possible pathophysiologic explanation of her neurological deterioration, including hypothalamic dysfunction, was analyzed. The role of hydrogen peroxide as a source of free radical formation, and its co-responsibility for vascular platelet aggregation and vasoconstriction was considered, upon case review, the main responsible etiologic factor.

The 100 most cited papers about pediatric traumatic brain injury: a bibliometric analysis.

BACKGROUND: The high incidence of traumatic brain injury (TBI) in children, combined with the challenges in diagnosis and treatment options, the difficulty of predicting the outcome of each case, and also the wide variety of possibly lifelong complications, has led to an extraordinary number of published papers regarding this topic. This bibliometric analysis is aimed at identifying and reviewing the 100 most cited papers in the most challenging and trending aspects of pediatric traumatic brain injury. METHODS: A search was performed using the Web of Science database in October 2018. Results were organized by citation number, and the 100 most cited papers were further reviewed and analyzed. RESULTS: Our search resulted in 2754 published papers from 1975 until October 2018, of which 1783 (64.74%) had been published in the last decade (2010-2018). The 100 most cited papers about traumatic brain injury in children have an average citation of 140.59 and have been published in 44 different journals. Four hundred thirty-five authors have contributed to these prominent articles, most of them from the USA. CONCLUSIONS: By reviewing those highly cited papers, we sought to offer significant help not only for studying this challenging field but also for designing new studies.

Fibrous dysplasia of occipital and temporal bone. A case report.

Fibrous dysplasia is a rare non-malignant condition where fibrous tissue replaces the normal bone architecture. Involvement of temporal and occipital bones is exceptionally rare and is associated with unique complications. A 10-year-old boy presented with right retroauricular enlargement and pain. Imaging studies and biopsy revealed fibrous dysplasia of the temporal and occipital bones. There was no hearing loss or sequelae arising from posterior fossa compression. The patient was discharged with follow-up instructions. Only 10 cases of occipital bone fibrous dysplasia have been reported in the medical literature. Occipital bone fibrous dysplasia can be complicated with Chiari malformation and syringomyelia while temporal bone involvement is associated with hearing loss. These potential developments require close follow-up that includes detailed neurologic examination, imaging and audiology.

Intraparenchymal Pericatheter Cyst after Cerebrospinal Fluid Shunt: A Rare Complication with Challenging Diagnosis - Case Presentation and Review of the Literature.

An intraparenchymal pericatheter cyst is a rare complication of ventriculoperitoneal shunt, which is not well described yet. Due to its rarity, lack of characteristic symptoms and radiological features that often mimic brain tumors or abscesses, especially in head computed tomography without contrast can be easily misdiagnosed. We report the case of a 9-year-old girl who was admitted to a peripheral hospital due to severe headaches and vomiting. The child had a history of craniotomy and ventriculoperitoneal shunt for posterior fossa tumor, performed in our department, 4 years earlier. The patient underwent a brain magnetic resonance imaging (MRI) scan and transmitted to our hospital with the diagnosis of brain tumor. However, a closer look at the MRI established the diagnosis of intraparenchymal pericatheter cerebrospinal fluid cyst; hence, the patient underwent shunt revision and cyst drainage. We researched the literature and described 20 reported cases, discussing the pathophysiological mechanisms, the radiological features, and the optimal treatment of this interesting, yet a challenging complication.

Primitive synovial sarcoma of suboccipital region in child.

Synovial sarcoma (SS) most commonly affects the lower limbs of males in the third to fifth decades of life, with masses of the head and neck accounting for 3-10% of all cases, mainly as a metastatic lesion. The lack of specific symptoms and radiological features in addition to the diversity of their microscopic aspects may cause confusion in the diagnosis; hence, knowledge of the unusual locations of SSs is very important. The immunohistochemistry, and more recently the cytogenetic studies, contribute to the differential diagnosis. We report the case of a 12-year-old girl with a rare primary SS in the suboccipital region, which underwent complete surgical resection.

Paediatric gliomas: diagnosis, molecular biology and management.

Paediatric gliomas represent the most common brain tumour in children. Early diagnosis and treatment greatly improve survival. Histological grade is the most significant classification system affecting treatment planning and prognosis. Paediatric gliomas depend on pathways and genes responsible for mitotic activity and cell proliferation as well as angiogenesis (MAPK, VEGF, EFGR pathways). Symptoms such as focal neurologic deficit or seizures can facilitate diagnosis, but they are not always present and therefore diagnosis is occasionally delayed. Imaging has adequate diagnostic accuracy (surpassing 90%), and novel imaging techniques such as MR spectroscopy and PET increase only slightly this percentage. Low grade gliomas (LGG) can be approached conservatively but most authors suggest surgical excision. High grade gliomas (HGG) are always operated with exception of specific contradictions including butterfly or extensive dominant hemisphere gliomas. Surgical excision is universally followed by radiotherapy and chemotherapy, which slightly increase survival. Inoperable cases can be managed with or without radiosurgery depending on location and size, with adjunctive use of radiotherapy and chemotherapy. Surgical excision must be aggressive and gross total resection (GTR) should be attempted, if possible, since it can triple survival. Radiosurgery is effective on smaller tumours of <2 cm2. Surgical excision is always the treatment of choice, but glioma recurrences, and residual tumours in non-critical locations are candidates for radiosurgery especially if tumour volume is low. Management of recurrences includes surgery, radiosurgery and chemoradiotherapy and it should be individualized according to location and size. In combination with molecular targeted therapeutic schemes, glioma management will be immensely improved in the next years.