Cranial Repair in Children: Techniques, Materials, and Peculiar Issues.

Cranial repair in children deserves particular attention since many issues are still controversial. Furthermore, literature data offer a confused picture of outcome of cranioplasty, in terms of results and complication rates, with studies showing inadequate follow-up and including populations that are not homogeneous by age of the patients, etiology, and size of the bone defect.Indeed, age has merged in the last years as a risk factor for resorption of autologous bone flap that is still the most frequent complication in cranial repair after decompressive craniectomy.Age-related factors play a role also when alloplastic materials are used. In fact, the implantation of alloplastic materials is limited by skull growth under 7 years of age and is contraindicated in the first years if life. Thus, the absence of an ideal material for cranioplasty is even more evident in children with a steady risk of complications through the entire life of the patient that is usually much longer than surgical follow-up.As a result, specific techniques should be adopted according to the age of the patient and etiology of the defect, aiming to repair the skull and respect its residual growth.Thus, autologous bone still represents the best option for cranial repair, though limitations exist. As an alternative, biomimetic materials should ideally warrant the possibility to overcome the limits of other inert alloplastic materials by favoring osteointegration or osteoinduction or both.On these grounds, this paper aims to offer a thorough overview of techniques, materials, and peculiar issues of cranial repair in children.

Endoscopic lipofilling for velopharyngeal insufficiency after transoral surgery: a technical note.

BACKGROUND: Velopharyngeal insufficiency (VPI) is a known complication of transoral surgery, with a reported incidence of 8.1%. The main factor related to VPI is the split of the soft palate. However, dead space resulting from transoral decompression may play a pivotal role in the pathogenesis of the dysfunction. In our experience, functionally significant dead space is almost constantly present after transoral decompression. This is probably due to malformation in children and postoperative scarring, thus configuring a nosological entity that we could define as "syndrome of the nasopharyngeal dead space." Palatal prosthesis and pharyngoplasty have been proposed, though these surgical procedures are technically tricky and with possible complications, such as OSA symptoms, snoring, and nasopharyngeal stenosis. METHODS: We proposed an effortless and minimally invasive procedure to treat this condition based on lipofilling the nasopharynx posterior wall endoscopically. To test the procedure's functional result, the submucosa of the nasopharynx posterior wall was initially filled with resorbable material, namely fibrin glue and autologous blood. The result was optimal but regressed after one month. Then, we repeated the procedure by lipofilling with autologous abdominal fat, resulting in a more stable anatomical and functional outcome at six months follow-up. RESULTS: The patient had a prompt significant improvement of his complaints (rhinolalia and oronasal regurgitation) and a correct projection of the nasopharynx posterior wall, with correct closure during phonation and absence of oronasal reflux. CONCLUSIONS: The "syndrome of the nasopharyngeal dead space" should be correctly identified after transoral surgery. It can be effectively treated with lipofilling of the posterior nasopharyngeal wall, a simple and minimally invasive procedure.

Coexistence of Trigonocephaly and Sylvian Arachnoid Cysts: A Coincidence?

INTRODUCTION: The association between trigonocephaly and Sylvian fissure arachnoid cysts (ACs) has been occasionally reported in the literature. However, the real incidence of this association and its clinical relevance remain unknown. METHODS: The authors collected and retrospectively reviewed all clinical charts and CT scans of patients surgically treated for trigonocephaly at the Pediatric Neurosurgical Department of Fondazione Policlinico Universitario "Agostino Gemelli" IRCCS from January 2014 to June 2023. RESULTS: During the study period, 136 patients with trigonocephaly underwent surgery. Analysis of the clinical charts revealed that in 39.7% of the cases (54/136), preoperative CT scan depicted the presence of a Sylvian fissure AC. Of these, AC was bilateral in 23 cases and unilateral in the remaining 31. All unilateral ACs were on the left side. The ACs were classified as Galassi grade I in 52 cases (96.3%) and Galassi grade II in 2 cases (3.7%). Interestingly, in 1 case we reported a Galassi grade I AC enlargement during follow-up, thereby necessitating surgical fenestration. CONCLUSION: ACs and trigonocephaly are well-known conditions for pediatric neurosurgeons; however, their association is poorly defined. Despite the lack of reports on the incidence and clinical significance of this association, it is worth knowing that radiological follow-up is essential in monitoring AC evolution.

Giant Congenital Hemangioma of the Skull: Prenatal Diagnosis and Multimodal Endovascular and Surgical Management.

Introduction: calvarial capillary hemangiomas are vascular tumors rarely seen in newborns. Differential diagnosis may be not straightforward on imaging studies and the management depends on patient and lesion characteristics. Case report: we present the case of a large congenital intracranial extra-axial lesion detected by routine prenatal US screening, a giant calvarial congenital hemangioma, treated with a multimodal strategy. Neonatal MR showed a hemorrhagic solid lesion, causing compression of brain tissue. Conservative treatment was attempted, but a one-month follow-up MR showed growth of the lesion with increased mass effect. Pre-operative endovascular embolization and surgical resection were performed. The pathology was consistent with intraosseous capillary hemangioma. The post-operative course was uneventful. At the 8-month follow-up, the patient had no clinical deficits and MR showed complete resection of the lesion. At the 13-month follow-up, the patient was asymptomatic, showing normal neurological examination and psychophysical development. Conclusions: although wait-and-see policy is feasible for small and asymptomatic lesions, radical resection is indicated when the mass is large, thus causing severe mass effect on the brain. Hypervascularization of the tumor may be responsible for hemorrhagic complications and severe anemia. On these grounds, endovascular treatment is feasible and effective to reduce hemorrhagic complications.

Iniencephaly and long-term survival: a possible association-case report and review of the literature.

BACKGROUND: Iniencephaly is a rare, complex, and severe form of neural tube defect (NTD), usually associated with wide systemic involvement and poor prognosis. The malformation involves the occiput and the inion, variably combined with rachischisis of the upper cervical and thoracic spine. Although most patients are either stillborn or perish within a few hours after birth, there are reports linking iniencephaly and long survival. For the neurosurgeon, associated encephalocele and secondary hydrocephalus represent the main challenges in this setting of patients, together with proper prenatal counseling. MATERIAL AND METHODS: Herein, the authors performed a thorough review of the pertinent literature, looking for reports of long-term survivors. RESULTS: To date, only five long-term survivors have been reported, with surgical repair attempted in four of them. Furthermore, the authors added their personal experience about two children with long-term survival who underwent surgery, thus accurately comparing them with the analogous cases previously reported in literature, with the ultimate aim of providing novel information on the pathology and the therapeutic options needed to offer proper treatment to these patients. CONCLUSIONS: Although no distinctive anatomic differences have been prior identified between long-term survivors and other patients, some variations emerged to occur, regarding age of presentation, the extension of the CNS malformation, the systemic involvement, and the surgical procedures offered. Although the authors shed some light on the topic, further studies are warranted to better define this rare and complex disease, and survival.

Chiari I malformation: management evolution and technical innovation.

BACKGROUND AND DEFINITION: In recent years thanks to the growing use of radiological assessment, Chiari I malformation became one of the major diseases for a neurosurgeon to deal with. CIM can be classified according to the extent of cerebellar tonsil tip into the foramen magnum being a protrusion over five mm considered pathological. Such a disease is a heterogeneous condition with a multifactorial pathogenetic mechanism that can subdivided into a primary and secondary form. Regardless of the form, it seems that CIM is the result of an imbalance between the volume of the braincase and its content. Acquired CIMs are secondary to conditions causing intracranial hypertension or hypotension while the pathogenesis of primary forms is still controversial. PATHOGENESIS AND TREATMENT: There are several theories in the literature but the most accepted one implies an overcrowding due to a small posterior cranial fossa. While asymptomatic CIM do not need treatment, symptomatic ones prompt for surgical management. Several techniques are proposed being the dilemma centered in the need for dural opening procedures and bony decompression ones. CONCLUSION: Alongside the paper, the authors will address the novelty presented in the literature on management, diagnosis and pathogenesis in order to offer a better understanding of such a heterogeneous pathology.

Technical note-in situ laminotomy: preserving posterior tension band in surgery of pediatric multilevel spinal tumor.

BACKGROUND: Laminotomy has been introduced in surgical practice to reduce complications of laminectomy after surgery of tumors in the spinal canal. However, the posterior ligament complex, which is routinely interrupted to remove the laminoplasty segment and gain access to the spinal canal, has a tendency not to heal and can lead to progressive kyphosis and collapse. CASE PRESENTATION: A 5-month-old boy affected by a thoracolumbar extradural tumor extending along seven spinal levels was operated on. The tumor was exposed and completely resected by a one-piece laminotomy with preservation of the integrity of the posterior tension band at both extremities. After 1-year radiological examination ruled out spinal deformity. CONCLUSION: The technique herein presented, which we named in situ laminotomy, allows to fully preserve the posterior tension band without reducing the exposure of the spinal canal in multilevel tumors. Additionally, the technique makes also the reconstruction of the spine elements very easy and rapid. However, longer follow-up is necessary to prove the effectiveness of this procedure in preventing long-term deformity and instability.

Adamantinomatous craniopharyngioma: evolution in the management.

BACKGROUND: In spite of the continuous progresses in pediatric neurosurgery, adamantinomatous craniopharyngioma (AC) remains a challenging tumor due to its proximity to optic pathways, pituitary gland, hypothalamus, and Willis' circle, which can result in significant endocrine, cognitive, and neurological morbidity after treatment with subsequent impact on the patient's quality of life (QoL). The relevance that QoL has today explains the changes in the management of AC observed over the time. The goal of the present article is to provide a historical background, to show the milestones in the changes of the AC treatment, and to analyze the current main options to manage such a challenging tumor. MATERIAL AND METHODS: The pertinent literature has been reviewed. Moreover, a comparison between the past and recent personal series is reported. RESULTS: Three main eras have been identified. The first (named Cushing era) was characterized by the need to realize a harmless surgery and to define the best way to approach AC; the second (microscope era) was characterized by a tremendous technical and technological development, with remarkable results in term of safe tumor resection and control but relatively poor QoL outcomes; and the third one (current period) is characterized by an increasing integration between surgery and adjuvant treatments, with relatively minor tumor control but significant improvement of QoL (comparable overall survival). The authors' experience reflects these changes. Two groups of children were compared: 52 cases (mean follow-up: 17.5 years) belong to the historical series (group 1, 1985-2003, aggressive surgical management) and 41 (mean follow-up: 8.5 years) to the current one (Group 2, 2004-2021, integrated management). No significant differences between the two groups were detected about recurrence rate, surgical mortality, and overall survival. However, Group 2 showed significant lower rates of postoperative panhypopituitarism, obesity, and visual deterioration. CONCLUSIONS: Radical surgery allows for a good AC control with a low rate of recurrence but high risk of permanent morbidity. Despite the greater number of recurrences and surgeries, the more conservative policy, based on a combination of treatments, seems to provide the same tumor control with a better QoL. The advances in trans-nasal and trans-ventricular endoscopy, in proton therapy and in the management of the AC cyst are the main factors that allowed such an improvement.

Protocol for the multicentre prospective paediatric craniectomy and cranioplasty registry (pedCCR) under the auspices of the European Society for Paediatric Neurosurgery (ESPN).

PURPOSE: In the paediatric age group, the overall degree of evidence regarding decompressive craniectomy (DC) and cranioplasty is low, whereas in adults, randomised controlled trials and prospective multicentre registries are available. To improve the evidence-based treatment of children, a consensus was reached to establish a prospective registry under the auspices of the European Society for Pediatric Neurosurgery (ESPN). METHODS: This international multicentre prospective registry is aimed at collecting information on the indication, timing, technique and outcome of DC and cranioplasty in children. The registry will enrol patients ≤ 16 years of age at the time of surgery, irrespective of the underlying medical condition. The study design comprises four obligatory entry points as a core dataset, with an unlimited number of further follow-up entry points to allow documentation until adolescence or adulthood. Study centres should commit to complete data entry and long-term follow-up. RESULTS: Data collection will be performed via a web-based portal (homepage: www.pedccr.com ) in a central anonymised database after local ethics board approval. An ESPN steering committee will monitor the project's progress, coordinate analyses of data and presentation of results at conferences and in publications on behalf of the study group. CONCLUSION: The registry aims to define predictors for optimal medical care and patient-centred treatment outcomes. The ultimate goal of the registry is to generate results that are so relevant to be directly transferred into clinical practice to enhance treatment protocols.

ALK-rearranged histiocytosis: Report of two cases with involvement of the central nervous system.

AIMS: Histiocytoses are a heterogeneous group of localized or disseminated diseases. Clinical presentation and patients' outcome vary greatly, ranging from mild to life-threatening disorders. Rare cases of systemic or localized histiocytosis harboring ALK rearrangement have been reported. METHODS: Two cases of CNS histiocytosis were thoroughly investigated by implementing multiple molecular tests, i.e. FISH, RT-qPCR, NGS analysis. RESULTS: In a 10-month old girl (patient #1), MRI showed two left hemispheric lesions and a right fronto-mesial lesion histologically consisting of a moderately cellular infiltrative proliferation, composed by CD68(PGM1)+/CD163+ spindle cells. ALK 5'/3'-imbalance and a KIF5B(exon 24)-ALK(exon 20) fusion were documented by RT-qPCR and NGS analysis, respectively. A subsequent CT scan showed multiple hepatic and pulmonary lesions. The patient was started on chemotherapy (vinblastine) associated to an ALK-inhibitor (Alectinib) with remarkable response. In a 11-year-old girl (patient #2), MRI showed a right frontal 1.5 cm lesion. Neuropathological examination revealed a histiocytic proliferation composed by medium sized CD68(PGM1)+/HLA-DR+ cells, showing moderate ALK1 positivity. ALK rearrangement and a KIF5B(exon 24)-ALK(exon 20) fusion were demonstrated also in this case. Subsequent CT, 18F-FDG-PET and MRI scans showed the presence of a single right femoral lesion, proved to be a fibrous cortical defect. CONCLUSIONS: In ALK-histiocytoses, CNS involvement may occur as part of a systemic disease or, rarely, as its only primary disease localization, which could remain otherwise asymptomatic. The diagnosis often relies on neuropathological examination of brain biopsy, which may pose a diagnostic challenge due to the variable histopathological features. An integrated histological and molecular approach in such cases is recommended.

Craniosynostosis and hydrocephalus: relevance and treatment modalities.

INTRODUCTION: Hydrocephalus is variously associated to syndromic craniosynostosis (CS), while it is randomly encountered in monosutural CS. Pathogenesis is still debated and reliable criteria for the diagnosis of overt hydrocephalus are lacking. Additionally, optimal treatment is controversial since it should balance the need to relieve intracranial hypertension and the risk of recurrence favored by lowering intracranial pressure. METHODS: A thorough review of the literature has been performed. Accordingly, pathogenic theories, diagnostic issues, and treatment options on hydrocephalus presenting in the context of CS are discussed. RESULTS: The association of hydrocephalus to simple CS is considered a fortuitous event. Its treatment is usually driven by the etiology and clinical relevance of hydrocephalus, favoring treatment before surgical correction to reduce CSF-related complications. On the other side, pathogenesis of hydrocephalus in the context of syndromic CS has been mainly related to factors that are secondary to the synostostic process, such as craniocerebral disproportion and venous hypertension. Hydrocephalus complicates 12-15% of syndromic CS, though its incidence is more relevant in FGFR2-related CS and raises up to 88% in Pfeiffer syndrome. Overt hydrocephalus should be properly differentiated by non-tense ventriculomegaly that is more frequent in Apert syndrome. Since intracranial hypertension is constant in syndromic CS even in the absence of active hydrocephalus, radiological monitoring of ventricular size along with intracranial pressure monitoring is essential. Active hydrocephalus occurs more frequently in infants, though stable ventriculomegaly may evolve into overt hydrocephalus after cranial expansion. If hydrocephalus is not clinically prominent, cranial expansion should be favored as first surgical step. Although posterior cranial expansion may address posterior cranial fossa constriction and stabilize ventricular dilation, effectiveness in long-term control of hydrocephalus is not clear. ETV is an effective treatment option, though success rate is affected by the presence of brain malformations and patient age. Extrathecal CSF shunting should be used as last resource due to the increased risk of complications in this context. CONCLUSIONS: The pathogenesis of hydrocephalus complicating syndromic CS should be further investigated. Concomitantly, the definition of reliable diagnostic criteria is advocated in order to promptly and properly identify active hydrocephalus. Finally, treatment algorithm should refine the best timing and treatment options aiming to relieve intracranial hypertension on one side and reduce the risk of restenosis on the other side.

Posterior vault "free-floating" bone flap: indications, technique, advantages, and drawbacks.

BACKGROUND: The enlargement of the posterior cranial fossa volume is considered one of the main steps of the surgical management of children with multiple sutures craniosynostosis. Different management options have been proposed including fixed expansive craniotomy, free bone flap craniotomy, and distraction osteogenesis. OBJECTIVES: To review indications to "free bone flap" craniotomy for the posterior fossa expansion, detailing advantages, disadvantages, and complications related to the technique. RESULTS AND CONCLUSIONS: A review of the literature shows that "free bone flap" posterior expansion cranioplasty still has a role, particularly in infants with thin and "honeycomb" structure of the bone, allowing to gain adequate intracranial volume increases and to postpone to a more adequate time surgery aimed at anterior cranial fossa expansion.

Functional and morphological changes in hypoplasic posterior fossa.

BACKGROUND: The knowledge of the development and the anatomy of the posterior cranial fossa (PCF) is crucial to define the occurrence and the prognosis of diseases where the surface and/or the volume of PCF is reduced, as several forms of craniosynostosis or Chiari type I malformation (CIM). To understand the functional and morphological changes resulting from such a hypoplasia is mandatory for their correct management. The purpose of this article is to review the pertinent literature to provide an update on this topic. METHODS: The related and most recent literature addressing the issue of the changes in hypoplasic PCF has been reviewed with particular interest in the studies focusing on the PCF characteristics in craniosynostosis, CIM, and achondroplasia. RESULTS AND CONCLUSIONS: In craniosynostoses, namely, the syndromic ones, PCF shows different degrees of hypoplasia, according to the different pattern and timing of early suture fusion. Several factors concur to PCF hypoplasia and contribute to the resulting problems (CIM, hydrocephalus), as the fusion of the major and minor sutures of the lambdoid arch, the involvement of the basal synchondroses, and the occlusion of the jugular foramina. The combination of these factors explains the variety of the clinical and radiological phenotypes. In primary CIM, the matter is complicated by the evidence that, in spite of impaired PCF 2D measurements and theories on the mesodermal defect, the PCF volumetry is often comparable to healthy subjects. CIM is revealed by the overcrowding of the foramen magnum that is the result of a cranio-cerebral disproportion (altered PCF brain volume/PCF total volume). Sometimes, this disproportion is evident and can be demonstrated (basilar invagination, real PCF hypoplasia); sometimes, it is not. Some recent genetic observations would suggest that CIM is the result of an excessive growth of the neural tissue rather than a reduced growth of PCF bones. Finally, in achondroplasia, both macrocephaly and reduced 2D and 3D values of PCF occur. Some aspects of this disease remain partially obscure, as the rare incidence of hydrocephalus and syringomyelia and the common occurrence of asymptomatic upper cervical spinal cord damage. On the other hand, the low rate of CIM could be explained on the basis of the reduced area of the foramen magnum, which would prevent the hindbrain herniation.

Ventriculosubgaleal shunt and neuroendoscopic lavage: refining the treatment algorithm of neonatal post-hemorrhagic hydrocephalus.

BACKGROUND: The optimal management of neonatal post-hemorrhagic hydrocephalus (PHH) is still debated, though several treatment options have been proposed. In the last years, ventriculosubgaleal shunt (VSgS) and neuroendosdcopic lavage (NEL) have been proposed to overcome the drawbacks of more traditional options, such as external ventricular drainage and ventricular access device. METHODS: We retrospectively reviewed neonates affected by PHH treated at our institution since September 2012 to September 2020. Until 2017 patients received VSgS as initial treatment. After the introduction of NEL, this treatment option was offered to patients with large intraventricular clots. After NEL, VSgS was always placed. Primary VSgS was reserved to patients without significant intraventricular clots and critically ill patients that could not be transferred to the operating room and undergo a longer surgery. RESULTS: We collected 63 babies (38 males and 25 females) with mean gestational age of 27.8 ± 3.8SD weeks (range 23-38.5 weeks) and mean birthweight of 1199.7 ± 690.6 SD grams (range 500-3320 g). In 6 patients, hemorrhage occurred in the third trimester of gestation, while in the remaining cases hemorrhage complicated prematurity. This group included 37 inborn and 26 outborn babies. Intraventricular hemorrhage was classified as low grade (I-II according to modified Papile grading scale) in 7 cases, while in the remaining cases the grade of hemorrhage was III to IV. Mean age at first neurosurgical procedure was 32.2 ± 3.6SD weeks (range 25.4-40 weeks). Death due to prematurity occurred in 5 patients. First-line treatment was VSgS in 49 patients and NEL in the remaining 14 cases. Mean longevity of VSgS was 30.3 days (range 10-97 days) in patients finally requiring an additional treatment of hydrocephalus. Thirty-two patients required one to three redo VSgS. Interval from initial treatment to permanent shunt ranged from 14 to 312 days (mean 70.9 days). CSF infection was observed in 5 patients (7.9%). Shunt dependency was observed in 51 out of 58 surviving patients, while 7 cases remained shunt-free at the last follow-up. Multiloculated hydrocephalus was observed in 14 cases. Among these, only one patient initially received NEL and was complicated by isolated trapped temporal horn. CONCLUSIONS: VSgS and NEL are two effective treatment options in the management of PHH. Both procedures should be part of the neurosurgical armamentarium to deal with PHH, since they offer specific advantages in selected patients. A treatment algorithm combining these two options may reduce the infectious risk and the risk of multiloculated hydrocephalus.

Diagnosis and management of cerebral venous sinus thrombosis in children: a single-center retrospective analysis.

PURPOSE: Cerebral venous thrombosis (CVT) is a rare disease in children, characterized by partial or total occlusion of blood flow in the cerebral venous system. The aim of this study is to describe clinical presentation, neuroimaging features, therapeutic management, and outcome of children with CVT. METHODS: We retrospectively analyzed the data, including clinical manifestations, laboratory data, neurological findings, and treatment of children with radiologically confirmed CVT, admitted between January 2010 and March 2020 to our hospital. Cases of CVT complicating brain surgery were excluded. RESULTS: We enrolled 24 children with CVT. Infection was the main etiology (58.3%), followed by trauma in 16.7% of cases. In the remaining 25% of cases, the cause was identified only in one patient presenting a thrombophilic factor. The most frequent site of thrombosis was the superficial venous system (86.8%), with multiple localizations disclosed in 79% of patients. All children received anticoagulant therapy with low molecular weight heparin (LMWH). One patient died for systemic complications of an underlying disease. No patient developed hemorrhagic events during the therapy, lasting from 35 to 360 days (mean 86 days). In all but one surviving patients (22 out of 24), recanalization of the sinus was observed at AngioMRI performed during follow-up. No neurological complications of CVT were recorded (mean follow-up: 1.5 year). CONCLUSIONS: CVT may present with subtle and unspecific clinical manifestations in children. High level of suspicion should be kept in trauma and sinusitis. Anticoagulation treatment is safe and effective and should be promptly started to improve outcome.

Role of susceptibility-weighted imaging and intratumoral susceptibility signals in grading and differentiating pediatric brain tumors at 1.5 T: a preliminary study.

PURPOSE: Susceptibility-weighted imaging (SWI) is useful for glioma grading and discriminating between brain tumor categories in adults, but its diagnostic value for pediatric brain tumors is unclear. Here we evaluated the usefulness of SWI for pediatric tumor grading and differentiation by assessing intratumoral susceptibility signal intensity (ITSS). METHODS: We retrospectively enrolled 96 children with histopathologically diagnosed brain tumors, who underwent routine brain MRI exam with SWI (1.5 T scanner). Each tumor was assigned an ITSS score by a radiology resident and an experienced neuroradiologist, and subsequently by consensus. Statistical analyses were performed to differentiate between low-grade (LG) and high-grade (HG) tumors, histological categories, and tumor locations. Inter-reader agreement was assessed using Cohen's kappa (κ). RESULTS: The interobserver agreement was 0.844 (0.953 between first reader and consensus, and 0.890 between second reader and consensus). Among all tumors, we found a statistically significant difference between LG and HG for ITSS scores of 0 and 2 (p = 0.002). This correlation was weaker among astrocytomas alone, and became significant when considering only off-midline astrocytomas (p = 0.05). Scores of 0 and 2 were a strong discriminating factor (p = 0.001) for astrocytomas (score 0) and for embryonal, choroid plexus, germ-cell, pineal, and ependymoma tumors (score 2). No medulloblastoma showed a score of 0. CONCLUSIONS: Our preliminary ITTS results in pediatric brain tumors somewhat differed from those obtained in adult populations. These findings highlight the potential valuable role of ITSS for tumor grading and discriminating between some tumor categories in the pediatric population.

Current trends in the management of subependymal giant cell astrocytomas in tuberous sclerosis.

INTRODUCTION: The management of subependymal giant cells astrocytomas (SEGAs) has been traditionally represented by surgical treatment through an open craniotomic approach. Though open surgery still represents a major option in the management of this kind of tumors, the introduction of mTOR inhibitors in the clinical practice, technological advances in neuroendoscopy and the more recent use of laser interstitial therapy have significantly enlarged the range of available management opportunities. METHODS: A thorough review of the literature has been performed. Accordingly, current views in open surgical treatment, medical therapy, endoscopic tumor removal and new trends (such as laser interstitial thermal therapy) are discussed. RESULTS: The risk of significant neurological morbidity (5-50%) complicating open surgery has been for a long time representing a main drawback in the management of SEGAs. More recent series report a significant reduction of morbidity and mortality. The mTOR inhibitors have demonstrated efficacy in both warranting a tumor reduction by up to 60% of the tumor size and helping the control of seizures. However, the reported rate of side effects is as high as 30% and tumor recurrence is a documented occurrence at the time of mTOR inhibitor discontinuation. Endoscopic tumor removal has been more extensively considered an option due to the acquisition of new tools. Limits are still represented by tumor size (< 3 cm) and broad attachment of the tumor to the basal ganglia. Laser interstitial thermal therapy (LITT) is the more recently considered option. Though promising, only short follow-up is available so far, while data on medium- and long-term results of this treatment are completely lacking to date. CONCLUSIONS: Surgical treatment remains a mainstay of the management of SEGAs. The indication for an open craniotomic approach should be balanced with an endoscopic tumor removal or LITT according to patient conditions, presence or not of an active hydrocephalus and extension of the attachment of the tumor to the basal ganglia. The mTOR inhibitors do have a definite role both as primary and as adjuvant treatment, but consistent limitations are represented up to now by a not negligible rate of complications and the uncertainties related to the possibility of tumor recurrence once the medical treatment is discontinued.

The scalp block for postoperative pain control in craniosynostosis surgery: a case control study.

PURPOSE: Postoperative analgesia after corrective surgery of pediatric craniosynostosis is crucial in terms of short- and long-term outcomes. The objective of this observational study was to evaluate the effectiveness of an analgesic technique based on the scalp block versus traditional pharmacological approach. METHODS: Thirteen patients, aged between 3 months and 2 years, undergoing surgical correction of craniosynostosis, received scalp nerve block before awakening (scalp block group). This group of patients was compared to a second group of 13 patients, retrieved from our database, treated with the traditional pharmacological approach (control group). Pain scores, need of rescue therapy, resumption of oral nutrition, degree of parent satisfaction at discharge, and length of stay in the Pediatric Intensive Care Unit (PICU) and in the hospital were compared between the two groups. RESULTS: Objective Pain Score values were significantly lower in patients treated with scalp block at 30 min and at 8 h after extubation. The number of pharmacological interventions for the treatment of pain in PICU was significantly higher in Control group. Children in Scalp block group started earlier oral feeding than in Control group, both for clear fluids and milk. Length of stay in PICU was longer in Scalp block group than in Control group, though the difference between the mean data in the two groups is about 3 . This difference does not seem significant from a clinical standpoint, since it seems more related to logistic issues (e.g., availability of bed in the Pediatric Neurosurgery Unit, presence of a parent), rather than clinical problems contraindicating the discharge from PICU. Indeed, the overall hospital length of stay was similar between the two groups. CONCLUSION: Scalp nerve block was effective for immediate postoperative pain control in patients younger than 2 years who underwent cranioplasty for craniosynostosis. The best pain control compared to conventional therapy allowed to limit the need for rescue analgesics in PICU and an earlier recovery time. ClinicalTrials.gov Identification: NCT04133467.

Securing CSF catheters to the skin: from sutures and bolt system to subcutaneous anchoring device towards zero complications.

INTRODUCTION: Securing the catheter to the skin either with sutures or staples and to the skull with bolt system still represents the most common options in the management of CSF external drainage. However, these options bear an unavoidable risk of complications. This problem is common to vascular accesses and has been successfully overcome with the introduction of device for subcutaneous anchoring (SecurAcath®, Interrad Medical, Inc., Plymouth, Minnesota), which has rapidly become the standard of care in this field. METHODS: We report our experience with the use of SecurAcath® to secure CSF drainage, either ventricular or spinal. Results were compared with literature data. RESULTS: Since 2015, SecurAcath® was used in 209 patients (mean age 7 years) to secure 195 external cranial catheters (either ventricular or subdural or intralesional) and 16 spinal drainages. Indwell time ranged from 5 to 30 days. No complication related to the use of the device was observed. In particular, there was no case of dislocation or accidental pullout of the catheter. Rate of infection, or superinfection in case of ventricular catheter implanted for CSF infection, was null. CONCLUSIONS: SecurAcath® is a safe and effective device to secure CSF external catheters, with several relevant advantages, including easy placement and maintenance. Moreover, it may stay in place for the whole duration of the catheter without any skin tissue trauma and allows a complete antisepsis of the exit site, thus reducing local skin complications. This factor has significant impact on the reduction of infection rate of external CSF catheters.

Cerebellar mutism: the predictive role of preoperative language evaluation.

BACKGROUND: The association between preoperative language impairment and development of cerebellar mutism after surgical treatment of posterior fossa tumors has gained increasing interest in recent years based on the concept that both local compression/infiltration of the infratentorial periventricular anatomical structures involved in speech and language, as well as an increased distraction of supratentorial periventricular anatomical structures, due to an associated hydrocephalus, involved in the coordination of speech circuits, might lead to the subclinical presence of language disturbances already at diagnosis, predicting the development of a cerebellar mutism syndrome after tumor removal. METHODS: A thorough review of the literature on the subject has been performed, together with a review of our institutional experience reporting the related long-term (10 years) results. RESULTS AND CONCLUSIONS: According to our institutional experience, 20/70 (28.5%) children presented preoperative language impairment and developed cerebellar mutism after surgery. A residual impairment persists in 75% of the cases at a follow-up of 2 years, and in 50% of the cases at 10 years, a finding which actually depicts cerebellar mutism no more as a transient deficit but among long-term sequelae. The appearance of complex dysarthria in the postoperative period is a negative prognostic factor for the long-term persistence of speech disturbances.