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Purpose: To gain insight into the patient characteristics of surgically treated hydrocephalus in the Netherlands, we report the first data from the Dutch Quality Registry NeuroSurgery (QNRS) database for infants with hydrocephalus requiring surgical intervention. Methods: We used the prospectively gathered database concerning infants ≤ 2 years of age surgically treated for hydrocephalus. We report data from start of registry, concerning etiology, age, and treatment of patients registered. We compared data with the Hydrocephalus Clinical Research Network (HCRN), a multicenter network of pediatric neurosurgical institutions in North America. Results: A total of 359 operated infants was registered in the period from 2010 to 2017. A drop in patients registered was seen in 2015, possibly due to revisions of the database. Most infants were operated on between 1 and 6 months of age. Cause of hydrocephalus was predominantly intracranial hemorrhage, followed by congenital causes. The proportion of infants with aqueduct stenosis and myelomeningocele as cause of hydrocephalus stayed relatively stable during this period of registration. Initial shunting was performed in 40% and reservoir/ETV as initial treatment was done in 60%. In both groups, 50% needed revision surgery. Conclusions: The first data concerning surgically treated pediatric hydrocephalus from a prospectively collected Dutch register are presented, showing similar results when comparing to the HCRN database. [ABSTRACT FROM AUTHOR]

Background The pathogenesis of hydrocephalus in human neonates with spina bifida (SB) is incompletely understood. In HTx rats, a model for congenital hydrocephalus, initial cerebrospinal fluid (CSF) malabsorption and CSF obstruction seemingly interplay in the pathogenesis. In human hydrocephalic SB neonates, assessment of CSF biomarkers may help to distinct between the relative contribution of CSF malabsorption and obstruction in the development of hydrocephalus. In this respect, concentrations of aminoterminal propeptide of type 1 collagen (PINP), a biomarker for arachnoideal fibrosis, has been shown to relate to CSF malabsorption in human hydrocephalic neonates. Additionally, high concentrations of transforming growth factor ß1 (TGFß1, involved in cellular proliferation and migration) are associated with CSF malabsorption (mice), whereas low TGFß1 concentrations are associated with congenital CSF obstruction (HT-x rats). In human hydrocephalic SB neonates, it is unclear how PINP and TGFß1 relate to the development of hydrocephalus. Elucidation of the pathogenesis of hydrocephalus in SB neonates may be important for the timing and optimalization of therapeutical strategies. Objective To determine the relative contribution of CSF malabsorption and obstruction during the development of hydrocephalus in SB neonates Materials and methods CSF concentrations of PINP (radio isotope assay) and TGFß1 (ELISA) were assessed in hydrocephalic SB neonates (n = 10), and compared with PINP and TGFß1 concentrations in hydrocephalic neonates with aqueduct stenosis (CSF obstruction (n = 4)), and with fetal intraventricular haemorrhages (CSF malabsorption (n = 4)). Interleukin-6 concentrations (IL-6; ELISA) in CSF were determined in all samples to estimate the proinflammatory state. Results In CSF from SB-hydrocephalus patients, concentrations of PINP and TGF ß1 were ∼80% and ∼50% lower than in malabsorption hydrocephalus (median values: PINP 181 ng/ml vs. 1074 ng/ml, resp., p = 0.002; TGF ß1 104 vs. 277 pg/ml, resp., p = 0.03). CSF PINP and TGF ß1 concentrations did not significantly differ between SB-hydrocephalus and obstruction hydrocephalus. Median IL-6 CSF concentrations did not differ between the groups. Conclusion Present data on CSF biomarkers strongly indicate that CSF obstruction contributes more to the development of hydrocephalus in SB neonates than arachnoideal CSF malabsorption. These data in hydrocephalic SB neonates are compatible with the concept that low CSF growth factor concentrations and CSF obstruction to the cortex may relate to cortical impairment [ABSTRACT FROM AUTHOR]

Background and Purpose: T2-FLAIR mismatch is a highly specific imaging biomarker of IDH -mutant diffuse astrocytoma in adults. It has however also been described in MYB/MYBL1 -altered low grade tumors. Our aim was to assess the diagnostic power of the T2-FLAIR mismatch in IDH -mutant astrocytoma and MYB/MYBL1 -altered low-grade tumors in children and correlate this mismatch with histology., Materials and Methods: We evaluated MR imaging examinations of all pediatric patients, performed at the Princess Máxima Center for Pediatric Oncology and the University Medical Center Utrecht between January 2012 and January 2023, with the histomolecular diagnosis of IDH -mutant astrocytoma, diffuse astrocytoma MYB/MYBL1 -altered, or angiocentric glioma, and the presence of T2-FLAIR mismatch was assessed. Histologically, the presence of microcysts in the tumor (a phenomenon suggested to be correlated with T2-FLAIR mismatch in IDH -mutant astrocytomas in adults) was evaluated., Results: Nineteen pediatric patients were diagnosed with either IDH -mutant astrocytoma ( n  = 8) or MYB/MYBL1 -altered tumor ( n  = 11: diffuse astrocytoma , MYB- or MYBL1 -altered n  = 8; or angiocentric glioma n  = 3). T2-FLAIR mismatch was present in 11 patients, 3 (38%) in the IDH -mutant group and 8 (73%) in the MYB/MYBL1 group. No correlation was found between T2-FLAIR mismatch and the presence of microcysts or an enlarged intercellular space in either IDH -mutant astrocytoma ( P  = .38 and P  = .56, respectively) or MYB/MYBL1 -altered tumors ( P  = .36 and P  = .90, respectively)., Conclusions: In our pediatric population, T2-FLAIR mismatch was more often found in MYB/MYBL1 -altered tumors than in IDH -mutant astrocytomas. In contrast to what has been reported for IDH -mutant astrocytomas in adults, no correlation was found with microcystic changes in the tumor tissue. This finding challenges the hypothesis that such microcystic changes and/or enlarged intercellular spaces in the tissue of these tumors are an important part of explaining the occurrence of the T2-FLAIR mismatch., (© 2024 by American Journal of Neuroradiology.)

Background and Purpose: Diffuse midline glioma H3K27-altered (DMG) is an aggressive, inoperable, predominantly paediatric brain tumour. Treatment strategies are limited, resulting in a median survival of only 11 months. Currently, radiotherapy (RT), often combined with temozolomide, is considered the standard of care but remains palliative, highlighting the urgency for new therapies. Radiosensitisation by olaparib, an inhibitor of PARP1 and subsequently PAR-synthesis, is a promising treatment option. We assessed whether PARP1 inhibition enhances radiosensitivity in vitro and in vivo following focused ultrasound mediated blood-brain barrier opening (FUS-BBBO)., Methods: Effects of PARP1 inhibition were evaluated in vitro using viability, clonogenic, and neurosphere assays. In vivo olaparib extravasation and pharmacokinetic profiling following FUS-BBBO was measured by LC-MS/MS. Survival benefit of FUS-BBBO combined with olaparib and RT was assessed using a patient-derived xenograft (PDX) DMG mouse model., Results: Treatment with olaparib in combination with radiation delayed tumour cell proliferation in vitro through the reduction of PAR. Prolonged exposure of low olaparib concentration was more efficient in delaying cell growth than short exposure of high concentration. FUS-BBBO increased olaparib bioavailability in the pons by 5.36-fold without observable adverse effects. A Cmax of 54.09 μM in blood and 1.39 μM in the pontine region was achieved following administration of 100 mg/kg olaparib. Although RT combined with FUS-BBBO mediated olaparib extravasation delayed local tumour growth, survival benefits were not observed in an in vivo DMG PDX model., Conclusions: Olaparib effectively radiosensitises DMG cells in vitro and reduces primary tumour growth in vivo when combined with RT. Further studies are needed to investigate the therapeutic benefit of olaparib in suitable preclinical PDX models., (Copyright © 2023 The Authors. Published by Elsevier B.V. All rights reserved.)

Background: Holographic neuronavigation has several potential advantages compared to conventional neuronavigation systems. We present the first report of a holographic neuronavigation system with patient-to-image registration and patient tracking with a reference array using an augmented reality head-mounted display (AR-HMD)., Methods: Three patients undergoing an intracranial neurosurgical procedure were included in this pilot study. The relevant anatomy was first segmented in 3D and then uploaded as holographic scene in our custom neuronavigation software. Registration was performed using point-based matching using anatomical landmarks. We measured the fiducial registration error (FRE) as the outcome measure for registration accuracy. A custom-made reference array with QR codes was integrated in the neurosurgical setup and used for patient tracking after bed movement., Results: Six registrations were performed with a mean FRE of 8.5 mm. Patient tracking was achieved with no visual difference between the registration before and after movement., Conclusions: This first report shows a proof of principle of intraoperative patient tracking using a standalone holographic neuronavigation system. The navigation accuracy should be further optimized to be clinically applicable. However, it is likely that this technology will be incorporated in future neurosurgical workflows because the system improves spatial anatomical understanding for the surgeon.

3 patients presented with non-functioning pituitary adenomas: a 50-year-old woman who had an adenoma that had not increased in size for 3 years; a 68-year-old man with an adenoma that was undiagnosed for 5 years and led to pituitary insufficiency and bitemporal hemianopsia; and a 64-year-old woman, who had refused therapy and follow-up after diagnosis of the adenoma 20 years earlier. She was admitted with a hydrocephalus, pituitary insufficiency, and severe visual loss. The clinical symptoms of pituitary adenomas are caused by the mass effects of the tumour and may vary considerably between patients. Transsphenoidal surgery is indicated in cases of suprasellar extension with compression or impending compressing of the optic chiasm. A 'wait-and-see' approach can be used for patients with smaller tumours and no visual field defects. The natural course of these adenomas is such that lifelong follow-up is necessary. Postoperative radiotherapy can be effective in reducing recurrence rates without negative effects on quality of life.

A 9-year-old boy who since 3 years had been treated with a brace for presumed idiopathic thoracic scoliosis was referred because of severe syringomyelia on MRI of the spinal cord. Neuroimaging of the spinal cord had been performed because of back pain, although the patient had no neurological signs or symptoms. Besides extensive syringomyelia, the MRI revealed a type I Chiari's malformation which we assumed to be the primary cause of the syringomyelia and the subsequent scoliosis. Surgical decompression of the Chiari's malformation led to a remarkable reduction of the syringomyelia and to stabilisation of the scoliosis. In children with structural scoliosis, ancillary investigation in the way of neuroimaging of the spinal cord should be considered to exclude underlying spinal cord pathology, even in the absence of neurological abnormalities.

A 32-year-old woman, a 73-year-old man and a 26-year-old pregnant woman presented with headache, vomiting, and variable presence of visual disturbances, impaired consciousness, and circulatory shock. All three had pituitary apoplexy. In the first patient lymphocytic hypophysitis was diagnosed, the second had a nonfunctional adenoma and the last patient probably also. All three patients were treated conservatively with full recovery of signs and symptoms. However pituitary insufficiency remained in all three. Pituitary apoplexy is an acute event with significant morbidity and mortality. The cause is a rapid expansion of, usually, a pre-existing, often not yet known, adenoma by massive haemorrhage or infarction. Pressure, among other things, causes hypopituitarism, meningism, compression of the chiasma opticum and visual disturbances. Although many predisposing factors have been identified, pituitary apoplexy is often an unpredictable event. Diagnosis is made by the typical clinical presentation, eye examination, MRI and by measuring pituitary hormones. Treatment is with suppletion of the deficient hormones and in selected patients by transsphenoidal decompression surgery.