Evaluation of postoperative fluctuations in plasma sodium concentration and triphasic response after pediatric craniopharyngioma resection: A French cohort study.

INTRODUCTION: Disturbances in plasma sodium levels are a major complication following recent resections of craniopharyngiomas in children. They must be properly managed to avoid neurological sequelae. We aimed to describe the variations and characteristics of postoperative natremia in children who had undergone a first craniopharyngioma resection with a particular focus on the frequency of triphasic syndrome in these patients. METHODS: Paediatric patients with craniopharyngiomas who underwent a first surgical resection in the neurosurgery department of the Hôpital Femme Mère Enfant (Lyon, France) between January 2010 and September 2021 were included in the present study and the medical records were analysed retrospectively. RESULTS: A total of 26 patients were included. Of these, 17 (65.4%) had a postoperative course characterised by the occurrence of both initial diabetes insipidus (DI) and hyponatremia a few days later. Eight patients (30.8%) presented then with isolated and persistent DI. Patients with the triphasic syndrome had a significantly higher grade of Puget classification on MRI (1 and 2), compared to the other patients. CONCLUSION: Dysnatremia is common after craniopharyngioma resections in children. This immediate postoperative complication is particularly difficult to manage and requires rapid diagnosis and prompt initiation of medical treatment to minimize fluctuations in sodium levels and avoid neurological sequelae.

Role of sodium fluorescein in pediatric low-grade glioma surgery: an update.

PURPOSE: Complete surgical resection is still the mainstay in the treatment of central nervous system low-grade tumors, eventually resulting curative. The complete surgical removal of these lesions, however, may be difficult in some cases because of their infiltrative nature. Intraoperative adjuncts may be a game changer. Sodium fluorescein (SF) is among the ideal candidates as intraoperative tools to favor the actual recognition of the tumor extension, since it accumulates in areas of altered blood-brain barrier, a typical characteristic of pediatric gliomas, and has a low rate of adverse events. This work proposes an update of previous works about the evaluation of the feasibility and usefulness of a systematic use of SF in a low-grade lesion group of pediatric patients. METHODS: Pediatric patients operated on for a resection or a biopsy of a low-grade glial or glioneuronal lesion (WHO grade I and II) at our Institution between September 2021 and December 2023, with the intraoperative use of sodium fluorescein (SF), were enrolled in the study. We collected pre-operative and postoperative clinical and radiological data, intraoperative findings, and post-operative pathological diagnoses. RESULTS: No adverse events were registered related to the intraoperative use of SF. SF appeared useful for the localization of boundaries of tumors, especially when characterized by a high degree of infiltration or by a deep-seated location, and for the checking of possible tumor remnants at the end of surgery. A good tumor-to-healthy tissue contrast was registered when tumor visualization was in a range between 1 to 2 h and 30 min after SF injection. Possible "false positives" due to intraoperative vascular wall injury and clearance of SF from both tumor and healthy tissue were observed in some cases and still remain open issues. CONCLUSIONS: SF is a feasible and safe intraoperative adjunct tool in the surgical removal of pediatric low-grade tumors. SF may show its usefulness especially in selected cases, such as deep-seated lesions and infiltrating tumors. Its safety profile, user-friendly management, and potential utility in both tumor resections and neuronavigated biopsies favor its wider use in the surgical treatment of pediatric low-grade tumors.

Pineal cysts in children: a paediatric series treated over the last twenty years in Lyon.

BACKGROUND: Pineal cysts are a rare lesion of the pineal gland. Pineal cysts are benign lesions, generally asymptomatic, and are usually an incidental discovery on MRI performed for other problems. The management of pineal cysts in children remains a matter for debate. Here, we report our own retrospective paediatric cases that have been surgically treated and review the paediatric literature on this topic. METHODS: This is a retrospective monocentric study. All patients operated by the senior author (CM) for a benign pineal cyst from 2000 to 2021 were included. All other pineal region cystic lesions were excluded. Medical and surgical data were extracted from the hospital medical database. RESULTS: Twelve patients were included. The clinical symptomatology was characterized by headaches in seven patients, visual troubles in two patients, precocious puberty in one patient, signs of intracranial hypertension in two patients, seizures associated with headache in one patient, and headaches associated with behavioural troubles in another patient. No major post-operative complications were observed in this series. It is to noted that surgery was performed because a suspicion of a true pineal parenchymal tumour has been made. Histopathological study came back with the diagnosis of pineal cyst. CONCLUSIONS: Pineal cyst is rare. If the radiological diagnosis is clear, no surgery is advocated except in cases associated with hydrocephalus and rapid growth. In case of a suspicion of a true pineal parenchymal tumour, a surgery may be needed to confirm the diagnosis. Lastly, we stress that only cystic lesions of the pineal gland itself should be considered as pineal cyst.

Sodium fluorescein in pediatric oncological neurosurgery: a pilot study on 50 children.

BACKGROUND: Sodium fluorescein (SF) is currently considered a valid intraoperative adjunct in the resection of high-grade brain lesions in adults. Experiences in pediatric groups and in low-grade gliomas and other low-grade lesions are still limited in literature, and subjective evaluation of fluorescence is still a limitation. MATERIAL AND METHODS: This study retrospectively reviewed all patients with brain or spine lesions operated on from September 2021 to July 2022 in the Pediatric Neurosurgery Unit of Hôpital Femme Mère Enfant, Lyon, who had received 5 mg/kg of 10%. Surgery was performed using a YELLOW560 filter at crucial times. At the end of surgery, the first operator completed a questionnaire, including his opinion on whether SF had been useful in tumor resection, recorded as a binary variable. Post hoc, surgical images were reviewed using ImageJ, an open-source Java image processing platform. In order to compare independent discrete variables, we applied the Student's t test, and we applied the Chi-square or Fisher exact test for binary variables. A threshold of p < 0.05 was set for statistical significance. RESULTS: We included 50 pediatric patients (0.2-17.6 years old). Forty/50 lesions showed SF uptake (80%). The differentiation between healthy and affected tissue, thanks to SF, subjectively evaluated by the surgeon, had as objective counterpart the statistically significant higher brightness of green in lesions, registered by the software (p < 0.001). SF overall allowed a good differentiation in 33/50 lesions, and overall utility of SF has been noted in 67% of them. When specifically considering gliomas, overall utility reached 75%. CONCLUSION: SF is a feasible, safe, and useful intraoperative adjunct in pediatric neurosurgery. In particular, it seems to have a promising role in some low-grade infiltrating glial tumors. The subjective evaluation of fluorescence seems to be reliable with respect to image analyses software.

Sodium fluorescein in pediatric neurosurgery: a systematic review with technical considerations and future perspectives.

BACKGROUND: Sodium fluorescein (SF) is routinely used in several centers as a valid intraoperative adjunct in adult oncological neurosurgery. Its use in pediatric neurosurgery is increasing, although its role is not yet well-defined in children. We reviewed the current literature in order to evaluate the use of SF in children with CNS and PNS lesions. METHODS: For this systematic review, we searched PubMed, Scopus, and Embase databases, and forward and backward citations for studies published between database inception and July 31st, 2022. We included any article type or congress abstract adding at least a new case, without restrictions of language or publication status, concerning the use of SF in neurosurgical procedures in patients under 18 years of age. We excluded studies concerning purely vascular cases and cerebrospinal fluid leaks. RESULTS: Of 4094 records identified, 19 articles were eligible and included for further analysis. As per July 31st, 2022, at least 119 patients aged from 11 months to 17.9 years underwent surgery with SF. No serious adverse events were reported. A large variety of tumor types was operated, in most cases resected under the specific YELLOW 560 nm filter after a low-dose SF injection (2-5 mg/kg) at the end of anesthesia induction. SF was reported particularly useful in gangliogliomas and pilocytic astrocytomas. DISCUSSION/CONCLUSION: Given its easy-to-use profile, low cost, and safety, SF seems to be a feasible and valid adjunct in the pediatric population when aiming at individuating a biopsy target or maximizing extent of resection, particularly in some tumor types. Further studies are required to strengthen the evidence on its impact on outcomes.

Results of the treatment of pineal tumors in children: the Lyon experience.

INTRODUCTION: Pineal tumors are rare and their incidence is of 1% among all pediatric tumors of the central nervous system. Patient survival depends on the histology, the extension of the surgical removal, and the efficacy of the complementary treatment (chemotherapy and cranio-spinal irradiation), as well as the age of the patient. MATERIALS AND METHODS: In this study, we analyzed 151 pediatric patients treated for pineal tumors from 1997 to 2020 in Lyon, France. All patients were recorded in the French Register of Pineal tumors, which has been centralized and maintained in Lyon since 2010. RESULTS: Our analysis shows that benign tumors have an overall positive prognosis with total surgical removal. Concerning pineal parenchymal tumors, pinealoblastomas have a poor prognosis , especially in children less than three years old. A new pathological classification system allows for a better stratification of patient risk within different groups of patients with pineal tumors. It is also important to note that the identification of DICER 1 syndrome in families with pinealoblastomas warrant further medical investigation. Patients with Germ Cell Tumors have more favorable outcomes, with a global survival rate of 87 % and a pure germinoma survival rate of almost 97%. When analyzing the prognosis of pineal gland gliomas, otherwise known as tectal plate gliomas, pilocytic astrocytomas had a promising prognosis. Otherwise, prognosis of other tectal plate gliomas are related to the grade of malignancy and the efficacy of complementary treatment. Lastly, papillary tumors need a complete removal for the best chance of survival, and Atypical teratoid/ rabdoid tumors (AT/TR) still have a bad prognosis, regardless of surgical resection. CONCLUSIONS: Our results show that, with regards to pediatric pineal region tumors, there are still areas in prognostic indicators that need to be improved. Similarly, these pathologies need to be treated via a multidisciplinary approach to improve a patient's survival rate and their quality of life.

The sub-occipital transtentorial approach for pineal region tumors: how I do it.

BACKGROUND: Two major approaches exist for the surgical removal of pineal region tumors: the supracebellar infratentorial and the sub-occipital transtentorial. METHODS: We present the Lyon's technique of the sub-occipital transtentorial approach for pineal region tumors and our tricks to avoid complications. The principle is to expose the pineal region under the occipital lobe and not through the interhemispheric fissure. CONCLUSIONS: The sub-occipital transtentorial approach is a direct, extra cerebral, safe, and effective way to access tumors of the pineal region.

New insights in craniovertebral junction MR changes leading to stenosis in children with achondroplasia.

PURPOSE: To characterize natural history and early changes of craniovertebral junction stenosis in achondroplasia correlating with clinical and radiological outcome. METHODS: Retrospective measures on craniovertebral junction were performed blindly, on sagittal T2-weighted images, in 21 patients with achondroplasia referred from 2008 to 2020. Clinical and polysomnography data were retrospectively collected. Each patient was paired for age and gender with four controls. Wilcoxon means comparison or Student's t-tests were applied. RESULTS: Twenty-one patients (11 females, from 0.1 to 39 years of age) were analyzed and paired with 84 controls. A craniovertebral junction stenosis was found in 11/21 patients (52.4%), all before the age of 2 years. Despite a significant reduction of the foramen magnum diameter (mean ± SD: patients 13.6 ± 6.2 mm, controls 28.5 ± 4.7 mm, p < .001), craniovertebral junction stenosis resulted from the narrowing of C2 dens-opisthion antero-posterior diameter (8.7 ± 3.9 mm vs 24.6 ± 5.1 mm, p < .001). Other significant changes were opisthion anterior placement (-0.4 ± 2.8 mm vs 9.4 ± 2.3 mm, p < .001), posterior tilt of C2 (46.2 ± 13.7° vs 31.6 ± 7.9°, p < .001) and of C1 (15.1 ± 4.3° vs 11.9 ± 5.0°, p = 0.01), and dens thickening (9.4 ± 2.2 mm vs 8.5 ± 2.1 mm, p = 0.03), allowing to define three distinguishable early craniovertebral junction patterns in achondroplasia. All children with C2-opisthion antero-posterior diameter of more than 6 mm had a better clinical and radiological outcome. CONCLUSION: Craniovertebral junction in achondroplasia results from narrowing between C2 dens and opisthion related to anterior placement of opisthion, thickening of C2 dens, and posterior tilt of C1-C2. A threshold of 6 mm for dens-opisthion sagittal diameter seems to correlate with clinical and radiological outcome.

Non-accidental head injury of infants: legal implications for the pediatric neurosurgeon.

BACKGROUND: Though the neurosurgeon's role in non-accidental head injury (NAHT) is the prompt recognition and care of the inflicted injuries, he/she should be aware of the possible legal implications related to this particular neurosurgical condition. MATERIAL AND METHODS: Based on published data and their own clinical and medico-legal practice, the authors review the role of the neurosurgeon in NHAT. RESULTS: Besides the contribution that can be given by the neurosurgeon to a correct differential diagnosis, he/she is likely to be involved in the interpretations of the pathological findings in the case where the judge may request for a medical opinion concerning etiology, circumstances, severity, and consequences. As a member of a multidisciplinary team, usually the neurosurgeon is only requested for information regarding the lesions he was called to recognize and treat. Nevertheless, such information may have a pivotal part in the evaluation process. Consequently, the neurosurgeon should be able to reach a correct differential diagnosis of NAHT among all the events that may share similar clinical and anatomo-pathological characteristics and be aware of the ongoing scientific controversies related to the diagnosis and pathophysiology. CONCLUSIONS: In practical terms, the pediatric neurosurgeon is called to individuate and record all the precise details of the condition to be eventually offered to the judge in case of controversy. Whereas the diagnosis of NAHT should be evoked at the initial phase in order not to leave the child unprotected, all possible alternative hypotheses should be ruled out "beyond reasonable doubt" for the court. The medical file should be maintained in the record carefully and be accessible even after a long time.

Anatomical bases of fast parietal grasp control in humans: A diffusion-MRI tractography study.

The dorso-posterior parietal cortex (DPPC) is a major node of the grasp/manipulation control network. It is assumed to act as an optimal forward estimator that continuously integrates efferent outflows and afferent inflows to modulate the ongoing motor command. In agreement with this view, a recent per-operative study, in humans, identified functional sites within DPPC that: (i) instantly disrupt hand movements when electrically stimulated; (ii) receive short-latency somatosensory afferences from intrinsic hand muscles. Based on these results, it was speculated that DPPC is part of a rapid grasp control loop that receives direct inputs from the hand-territory of the primary somatosensory cortex (S1) and sends direct projections to the hand-territory of the primary motor cortex (M1). However, evidence supporting this hypothesis is weak and partial. To date, projections from DPPC to M1 grasp zone have been identified in monkeys and have been postulated to exist in humans based on clinical and transcranial magnetic studies. This work uses diffusion-MRI tractography in two samples of right- (n = 50) and left-handed (n = 25) subjects randomly selected from the Human Connectome Project. It aims to determine whether direct connections exist between DPPC and the hand control sectors of the primary sensorimotor regions. The parietal region of interest, related to hand control (hereafter designated DPPChand), was defined permissively as the 95% confidence area of the parietal sites that were found to disrupt hand movements in the previously evoked per-operative study. In both hemispheres, irrespective of handedness, we found dense ipsilateral connections between a restricted part of DPPChand and focal sectors within the pre and postcentral gyrus. These sectors, corresponding to the hand territories of M1 and S1, targeted the same parietal zone (spatial overlap > 92%). As a sensitivity control, we searched for potential connections between the angular gyrus (AG) and the pre and postcentral regions. No robust pathways were found. Streamline densities identified using AG as the starting seed represented less than 5 % of the streamline densities identified from DPPChand. Together, these results support the existence of a direct sensory-parietal-motor loop suited for fast manual control and more generally, for any task requiring rapid integration of distal sensorimotor signals.

Fixed posterior cranial vault expansion technique.

BACKGROUND: Different techniques to reshape the posterior skull vault have been developed in the last decades, all sharing the same goals of increasing the skull volume, decreasing the intracranial pressure (ICP), correcting the cranial dysmorphy, and contributing to a better growth of the skull. Though over the last years most refinements in these techniques have focused on the use of hardware as distractors or springs, the fixed posterior vault expansion remains a valuable procedure for cranial remodeling. METHODS: We describe in details the technique used for fixed posterior vault expansion in children that is applied at the French Referral center for Craniosynostosis of Lyon, France. DISCUSSION: The fixed posterior vault expansion increases the risk of perioperative complications due to the elevation of the posterior bone flap from the dura but allows an immediate decompression and correction of the shape, simplifying the postoperative course.

CSF shunting in myelomeningocele-related hydrocephalus and the role of prenatal imaging.

OBJECTIVE: Hydrocephalus is commonly associated with myelomeningocele (MMC). Indication and timing of cerebrospinal fluid (CSF) shunting are still a topic of discussion. The aim of this study was to investigate whether the analysis of prenatal cerebral imaging studies could provide information that is predictive of the necessity of CSF shunting in the postnatal period. MATERIAL AND METHODS: Among 73 infants operated on because of MMC between January 2003 and June 2020, 50 had undergone prenatal and postnatal MRI studies and were considered for analysis. For each patient, frontal horn width, atrial ventricle diameter, third ventricle diameter, and subarachnoid spaces (sinocortical width, craniocortical width, and the interhemispheric width) have been measured on prenatal, postnatal, and a follow-up MRI study. The need of CSF shunting device placement in relation to prenatal and early postnatal MRI data was investigated. RESULTS: Of the 50 infants, 31 (62%) developed a progressive hydrocephalus. Of these, 30 needed a CSF shunt and the majority of them (n=29) was operated on within 28 days after birth. One patient needed CSF shunt implantation at 45 days after birth and one child developed a late progressive hydrocephalus, successfully treated by ETV alone, at 14.2 months of age. All patients with an atrial ventricle diameter greater than 1.9 cm and a 3rd ventricle diameter larger than 0.3 cm on antenatal third trimester imaging have undergone CSF shunting within 1 month after birth. Conversely, all the children that did not undergo a CSF shunt placement showed an atrial cerebral ventricle diameter inferior to 1.2 cm and a 3rd ventricle width < 0.3 cm on antenatal imaging. Frontal horn width and subarachnoid CSF spaces' evolution did not seem to play a role. CONCLUSION: The prenatal MRI assessment of the associated prenatal ventriculomegaly in MMC provides parameters that have a predictive value heralding the probability of a CSF diversion procedure after birth. In the same way, the analysis of intrauterine MRI studies may identify those subjects that are less at risk of developing a progressive hydrocephalus after birth, therefore encouraging a more cautious attitude towards the early implantation of CSF shunting devices in the current clinical practice.

Superiority of thoracolumbar injury classification and severity score (TLICS) over AOSpine thoracolumbar spine injury classification for the surgical management decision of traumatic spine injury in the pediatric population.

PURPOSE: Thoracolumbar fractures are the most common kind of spine injury in children. Several types of spine injury can occur, and for this reason, treatment algorithms have been put in place for the management of these patients. At present, the thoracolumbar injury classification and severity score system (TLICS) and the thoracolumbar AOSpine injury score (AOSpine score) aimed at providing treatment recommendations. We aimed to assess the reliability, in children, of the TLICS scoring and AOSpine scoring systems, and to define the superiority of one of the methods of scoring, to spread its use in routine clinical management in the pediatric spine trauma. METHODS: A retrospective chart review of consecutive children admitted to a Level 1 trauma center for traumatic thoracolumbar fractures, between 2006 and 2019, was performed. We compared the management we performed in clinical practice in children with spine trauma, to the decisional algorithms based on the TLICS and AOSpine scores. According to these scores, surgical treatment should be performed when the TLICS score ≥ 5 and the AOSpine score > 5; and surgical or conservative treatment was considered reasonable when the TLICS score = 4 and the AOSpine score = 4 or 5. Surgical indications were based on the clinical status, the anatomy of the fracture, and the risk of sagittal imbalance of the growing spine. RESULTS: Fifty-four patients met the inclusion criteria. We demonstrated that both the AOSpine score and the TLICS scores had a significant correlation for surgical management decision of spine trauma (p < 0.0001). We found a high concordance between surgical decision making in the pediatric clinical practice and the TLICS score. In our pediatric cohort, there were significantly more patients with TLICS ≥ 5 (n = 47, 87%) than with AOSpine score > 5 (n = 26, 46%, p < 0.0001). There were significantly more patients with TLICS ≥ 4 (n = 53, 98%), than with AOSpine score ≥ 4 (n = 42, 77%, p = 0.001). ConclusionsThe TLICS score was significantly more appropriate than the AOSpine score, for the surgical treatment decision in children, especially when considering the future risk of sagittal imbalance.

Coronal and lambdoid suture evolution following total vault remodeling for scaphocephaly.

OBJECTIVE: Different types of surgical procedures are utilized to treat craniosynostosis. In most procedures, the fused suture is removed. There are only a few reports on the evolution of sutures after surgical correction of craniosynostosis. To date, no published study describes neosuture formation after total cranial vault remodeling. The objective of this study was to understand the evolution of the cranial bones in the area of coronal and lambdoid sutures that were removed for complete vault remodeling in patients with sagittal craniosynostosis. In particular, the investigation aimed to confirm the possibility of neosuture formation. METHODS: CT images of the skulls of children who underwent operations for scaphocephaly at the Hôpital Femme Mère Enfant, Lyon University Hospital, Lyon, France, from 2004 to 2014 were retrospectively reviewed. Inclusion criteria were diagnosis of isolated sagittal synostosis, age between 4 and 18 months at surgery, and availability of reliable postoperative CT images obtained at a minimum of 1 year after surgical correction. Twenty-six boys and 11 girls were included, with a mean age at surgery of 231.6 days (range 126-449 days). The mean interval between total vault reconstruction and CT scanning was 5.3 years (range 1.1-12.2 years). RESULTS: Despite the removal of both the coronal and lambdoid sutures, neosutures were detected on the 3D reconstructions. All combinations of neosuture formation were seen: visible lambdoid and coronal neosutures (n = 20); visible lambdoid neosutures with frontoparietal bony fusion (n = 12); frontoparietal and parietooccipital bony fusion (n = 3); and visible coronal neosutures with parietooccipital bony fusion (n = 2). CONCLUSIONS: This is the first study to report the postoperative skull response after the removal of normal patent sutures following total vault remodeling in patients with isolated sagittal synostosis. The reappearance of a neosuture is rather common, but its incidence depends on the type of suture. The outcome of the suture differs with the incidence of neosuture formation between these transverse sutures. This might imply genetic and functional differences among cranial sutures, which still have to be elucidated.

Management of Chiari type I malformation: a retrospective analysis of a series of 91 children treated surgically.

INTRODUCTION: The diagnosis of Chiari I malformation, its symptomatology, and the results of its surgical management are still discussed. We report a pediatric series of CMI without associated skull base malformations or cerebellar growth anomalies operated between 2001 and 2018. MATERIAL AND METHODS: Ninety-one children out of 146 surgically treated cases have been included in the study. Age at surgery ranged from 5 months to 17 years clinical data, and complementary examinations leading to the surgical indication have been analyzed together with the surgical outcomes. The average follow-up duration was of 4 years. The occipito-cervical decompression with duraplasty without opening the arachnoid was the procedure of election. Three quarters of patients presented with headaches, 12% with cerebellar syndrome, 13% with vertigo, 26% with nausea or vomiting, 24% with sensorimotor deficits, 11% with cranial nerve deficits, and 29% with other symptoms. Eighteen percent of patients suffered from scoliosis, 47% had an associated syrinx and 16% a ventricular dilation. RESULTS: After the treatment, the clinical symptomatology improved in about three-quarters of the patients: headache (69.4%), nausea or vomiting (66.7%), sensorimotor deficits (55.6%), and other symptoms (78.3%). Syringomyelic cavities diminished partially in size or disappeared in 58.3% of patients, remained stable in 29.2%, and worsened in 12.5%. Only one-third of children with preoperative scoliosis benefited from the surgical treatment. No clinical signs or symptoms were found to be reliable predictors of surgical success, neither the extent of the cerebellar tonsil descent. CONCLUSION: Occipito-cervical decompression allows to improve the clinical condition in the majority of children with symptomatic CMI in the absence of associated cervico-spinal junction alterations, craniosynostosis, or cerebellar growth anomalies. No clinical signs or symptoms neither radiological criterion appear to be a specific finding for the surgical indication.

Reduced-dose craniospinal irradiation is feasible for standard-risk adult medulloblastoma patients.

INTRODUCTION: Medulloblastoma is the most common malignant brain tumor in children, but accounts for only 1% of brain cancers in adults. For standard-risk pediatric medulloblastoma, current therapy includes craniospinal irradiation (CSI) at reduced doses (23.4 Gy) associated with chemotherapy. Whereas most same-stage adult patients are still given CSI at 36 Gy, with or without chemotherapy, we report here on our use of reduced-dose CSI associated with chemotherapy for older patients. METHODS: We gathered non-metastatic patients over 18 years old (median age 28 years, range 18-48) with minimal or no residual disease after surgery, no negative histological subtypes, treated between 1996-2018 at the Centre Léon Bérard (Lyon) and the INT (Milano). A series of 54 children with similar tumors treated in Milano was used for comparison. RESULTS: Forty-four adults were considered (median follow-up 101 months): 36 had 23.4 Gy of CSI, and 8 had 30.6 Gy, plus a boost to the posterior fossa/tumor bed; 43 had chemotherapy as all 54 children, who had a median 83-month follow-up. The PFS and OS were 82.2 ± 6.1% and 89 ± 5.2% at 5 years, and 78.5 ± 6.9% and 75.2 ± 7.8% at ten, not significantly different from those of the children. CSI doses higher than 23.4 Gy did not influence PFS. Female adult patients tended to have a better outcome than males. CONCLUSION: The results obtained in our combined series are comparable with, or even better than those obtained after high CSI doses, underscoring the need to reconsider this treatment in adults.

Awake brain surgery in children-a single-center experience.

INTRODUCTION: Awake brain surgery (ABS) represents a rare surgical procedure in children as age and psychological aspects, which are considered to interfere with its feasibility and psychological outcome and limit its application. Only few pediatric case series have been reported so far, indicating a more complex translation of this surgical approach to children. However, the advances in neuropsychological testing and monitoring may have a substantial impact on ameliorating the eligibility of children undergoing awake procedures. This study addresses the condition of ABS in a pediatric cohort, focusing on its practicability and diversified outcome aspects. METHODS: We performed a retrospective review and prospective outcome analysis of pediatric patients with CNS lesions undergoing ABS between 2005 and 2018, completed at the University of Lyon, France. RESULTS: Eighteen children were considered for ABS with respect to the eloquent location of their CNS lesions documented in their pre-operative MRI. Seventeen of them underwent asleep-awake-asleep brain surgery. The cohort included 5 males and 12 females. The median age at surgery was 14.8 years, (range 9.4 to 17.6 years). Intraoperative testing included electrocortical stimulation while pursuing speech or motor activity. Most of the lesions were intrinsic tumors of glial origin. A complete tumor removal was achieved in 11 patients (65%). Post-operative neurological deficits were transiently observed in 2 patients, whereas severe psychological reactions occurred in 1 child. Persistent attention deficits were found in 2 patients. One patient experienced an infectious complication requiring antibiotic treatment. Two patients died during follow-up due to tumor progression. The mean duration of follow up was 22.2 months (range 3.4 to 46.8 months). CONCLUSIONS: ABS was shown to be beneficial in terms of efficient tumor resection besides simultaneous preservation of neurological functions. Psychological preparation of the families and the children is essential to increase the number and age range of patients, who can benefit from this technique. Neuropsychological testing before and after surgery is essential to determine cognitive outcome, which can be altered in a minority of patients.

Craniofacial bone alterations in patients with neurofibromatosis type 1.

Osseous manifestations of neurofibromatosis 1 (NF-1) occur in a minority of the affected subjects but may be because of significant clinical impairment. Typically, they involve the long bones, commonly the tibia and the fibula, the vertebrae, and the sphenoid wing. The pathogenesis of NF-1 focal osseous lesions and its possible relationships with other osseous NF-1 anomalies leading to short stature are still unknown, though it is likely that they depend on a common mechanism acting in a specific subgroup of NF-1 patients. Indeed, NF-1 gene product, neurofibromin, is expressed in all the cells that participate to bone growth: osteoblasts, osteoclasts, chondrocytes, fibroblasts, and vascular endothelial cells. Absent or low content of neurofibromin may be responsible for the osseous manifestations associated to NF-1. Among the focal NF-1 osseous anomalies, the agenesis of the sphenoid wing is of a particular interest to the neurosurgeon because of its progressive course that can be counteracted only by a surgical intervention. The sphenoid wing agenesis is regarded as a dysplasia, which is a primary bone pathology. However, its clinical progression is related to a variety of causes, commonly the development of an intraorbital plexiform neurofibroma or the extracranial protrusion of temporal lobe parenchyma and its coverings. Thus, the cranial bone defect resulting by the primary bone dysplasia is progressively accentuated by the orbit remodeling caused by the necessity of accommodating the mass effect exerted by the growing tumor or the progression of the herniated intracranial content. The aim of this paper is to review the neurosurgical and craniofacial surgical modalities to prevent the further progression of the disease by "reconstructing" the normal relationship of the orbit and the skull.

Psychological aspects of awake brain surgery in children-interests and risks.

Awake brain surgery (ABS) in children remains a subject of controversial debate for the potential psychological limitations that are related to this type of procedure. However, the tolerance and benefits of ABS in adults advocate for increased application of ABS in children. In this study, we report the psychological assessment, evaluation algorithm, and outcome of pediatric patients, who underwent ABS for surgical treatment of lesions in eloquent areas. Psychological selection criteria and the specifications of psychological support are described. A retrospective review and analysis of psychological assessment and psychological outcome of pediatric patients, who underwent ABS between 2005 and 2018 at the Department of pediatric neurosurgery, University of Lyon, France, was performed. Long-term psychological outcomes are reported. ABS was proposed to 18 children aged between 9 and 17 years and their families. After psychological evaluation of the individual patient and their familial surrounding, five boys and 12 girls (n = 17) were accounted eligible for ABS. They underwent asleep-awake-asleep brain surgery with intraoperative testing. In 16 cases, ABS could be performed as planned. Psychological alterations were postoperatively observed in 3 patients, symptoms of a post-traumatic stress disorder in 1 patient. The precise preoperative evaluation of the risk-benefit ratio in children plays a crucial role in anticipating a good psychological outcome. Professional psychological preparation and support of the child and his or her family are the key elements for successful completion of ABS.

The first case report of medulloblastoma associated with Tatton-Brown-Rahman syndrome.

DNMT3A codes for a DNA methyl transferase enzyme that plays a central role embryogenesis. Somatic mutations in this gene have been associated with tumorigenesis and are associated with a number of cancers. The recently described Tatton-Brown-Rahman syndrome (TBRS) is due to heterozygous germline mutations in the DNMT3A gene. So far, only one case of hematological malignancy associated with TBRS have been reported. Here, we describe the first case presenting with TBRS and medulloblastoma. We also discuss the associations between mutations in DNMT3A found in TBRS, AML, and medulloblastoma.