During the pandemic of coronavirus infection (COVID-19), hospital visits were greatly restricted. Even the pediatric ward was no exception. The prohibition of visitation at the end-of-life care of pediatric brain tumor patients had a significant impact on the patients and their families. Therefore, some families of terminally ill pediatric brain tumor children chose to receive home care. We present two cases of terminally ill children with brain tumor and their end-of-life care. Case 1: a 4-year-old boy was diagnosed with a brain tumor following a headache. The rapid growing brain tumor was histologically confirmed as choroid plexus carcinoma. The patient underwent radiochemotherapy after maximum resection. Despite several radiochemotherapeutic interventions for relapses, tumor growth with leptomeningeal dissemination could not be controlled. His parents opted for home palliative care. In close cooperation with a visiting physician and his team in the community, the patient’s palliative care at home was continued for three months and the patient died at home. The overall course of the disease was 23 months. Case 2: a six-year-old boy was radiologically diagnosed diffuse intrinsic pontine glioma following changes in eye position. He underwent radiotherapy. Although his symptoms improved after radiotherapy, the tumor re-grew 6 months later. Despite additional irradiation and intravenous bevacizumab every 2 weeks, his trunk ataxia worsened 10 months after onset. Eleven months after onset, the patient was confined to bed all day and developed dysphagia and was transferred to palliative care. In close cooperation with a visiting physician and his team in the community, the patient’s palliative care at home was continued for one month and his death was finally diagnosed at the hospital. The overall course of the disease was 12 months. The families were generally satisfied with the palliative care provided by us. Due to the pandemic of COVID-19, many hospitalized patients could not receive visitors at the hospital or family members could not visit hospitalized patients in the hospital. Therefore, for terminally ill patients, home palliative care has become an important option than ever before the outbreak of COVID-19, especially in pediatric cases. In close cooperation with a doctor at a clinic with abundant experience in end-of-life care at home for patients with malignant tumors, it is necessary to provide not only pain and suffering relief for the patients, but also care guidance for the family and emergency response so that the family members who support the children are not overwhelmed with anxiety. Cooperation among multiple professions and related departments is essential. Further accumulation of experience is needed for better home palliative care in pediatric cases of brain tumor.

Background: The annual meeting of the Japanese Society of Pediatric Neurosurgery (JSPN) provides pediatric neurosurgeons an opportunity to present and distribute their research. The goal of each project is to get their results published, and the publication rate can highlight the scientific activity of the society. The aim of this study is to clarify the publication rates of presentations from the annual meetings held in the years 2016, 2017, and 2018.

Methods: All presentations from the JSPN annual meeting held in 2016, 2017, and 2018 were identified. The authors name and/or the abstract title were searched on PubMed and Ichushi-Web to verify the published articles. The name of the journal and year of the publication were recorded. Publications published after the year of presentation in peer-reviewed journals were also included. The presentations from educational seminars and invited speakers were excluded from the study.

Results: Overall 173 (27.4%) out of 632 presentations were published in peer-reviewed journals. Sixty-six presentations were written in English and 107 in Japanese. There was no difference in the publication rates based on the types of the presentation. The most common English language journals we encountered along with the percentage of published articles were Child’s Nervous System (31.5%), World Neurosurgery (10.5%), Neurologia Medico Chirurgica (9.2%) and Journal of Neurosurgery; Pediatrics (7.9%), and Nervous System in Children (81.4%) was the journal for Japanese publications. More than 90% of articles were published within 3 years following their presentation. The publication rates of men and women authors were observed to be similar.

Conclusions: The publication rate of abstracts presented at JSPN annual meeting was relatively low compared with previous reports. The results of this study could be a good measure for understanding the academic activities of JSPN.

Purpose: Patients with severe spasticity caused by cerebral palsy suffer from sleep disturbance. Intrathecal baclofen (ITB) therapy releases spasticity; thus, may result to a deep sleep. We measured the sleep pattern of patients with severe spasticity before and after ITB therapy induction.

Methods: Four patients with cerebral palsy (boys aged 10, 13, and 18 years and a woman aged19 years) and two adults with head trauma and stroke participated in the study. All patients presented persistent consciousness disturbance and spastic tetrapalsy with level V in gross motor function classification system. ITB system was implanted in all patients and degrees of pre - and postoperative modified Ashworth scale (mAS) was scored. Total and deep sleep durations were analyzed by motion, heart rate, and heart rate variability using a sports watch mounting acceleration meter and heart rate meter. The measurements were performed overnight 1 day before and 7 days after ITB therapy induction.

Results: Doses of baclofen were adjusted at 70-200 µg/day. Spasticity scored by mAS significantly decreased from 2.85 ± 0.12 to 2.31 ± 0.36 (p ＝ 0.014). Average total and deep sleep durations preoperatively were 7 h 26 m ± 3 h 3 m and 30 m ± 34 m, which were shorter than the sleep durations of the same age group of healthy individuals. Total and deep sleep durations under the ITB therapy significantly increased to 8 h 48 m ± 2 h 17 m (p ＝ 0.04) and 1 h 8 m ± 2 h 47 m (p ＝ 0.03).

Conclusion: In patients with severe spasticity, sleep was a disturbance that may be improved by ITB therapy. Sleep duration and depth, as well as mAS, could serve as good indicators for adjusting the optimal ITB dose.

Objective: Helmet therapy has been demonstrated as effective in the treatment of moderate and severe positional cranial deformation (PCD) in infants. Our objective was to investigate the relationship between the degree of deformation, helmet therapy prescriptions, and the actual application of helmet therapy.

Materials and methods: In this retrospective study, we enrolled infants who were evaluated in our department for cranial deformation between April 2022 and December 2022. We recorded their age in months and gender, measured head dimensions, and calculated cephalic index (CI) and cranial vault asymmetry index (CVAI). The CI and CVAI values were categorized into five grades, ranging from grade 1 (mild) to grade 5 (profound). Statistical analysis included the assessment of differences in CI and CVAI grades between groups with helmet therapy prescriptions and those without, as well as between groups with helmet therapy application and those without, using the Wilcoxon rank-sum test.

Results: Our study comprised sixty-four infants, including 40 males (62.5%) and 24 females (37.5%). The median age, with 1st-3rd quartiles, was five months (4-7 months). Among these, fifty-four infants exhibited PCD, with 45 having positional plagiocephaly (70.3%) and 9 having positional brachycephaly (14.1%). Of the 54 infants with PCD, 31 (57.4%) sought helmet therapy prescriptions, and 13 of them (41.9%) proceeded with helmet therapy. The prescription group exhibited significantly higher CVAI grades than the non-prescription group (p＝0.77). However, there were no statistical differences observed between the prescription and non-prescription groups or between the helmet therapy and non-helmet therapy groups regarding CI. Similarly, there were no statistical differences between the helmet therapy and non-helmet therapy groups for CVAI.

Conclusion: Our study highlights that infants seeking helmet therapy prescriptions for PCD tend to have higher CVAI grades than those who do not seek prescriptions. These findings could provide valuable insights for clinicians considering PCD therapy options.

Objective: In surgical resection of pediatric craniopharyngioma, reducing the recurrence rate by improving the resection rate, preserving visual function, and minimizing damage to surrounding vital structures is necessary. Endoscopic endonasal surgery has recently become the primary surgical approach for craniopharyngiomas. Hereby, we report on the resection of these lesions via endoscopic endonasal surgery for pediatric cases at our hospital.

Materials and Methods: Between 2014 and 2022, 22 cases of pediatric craniopharyngioma underwent endoscopic endonasal surgery. Eleven cases were primary; another 11 were recurrences. The average age for this group was 11.4 years. Nine cases were retrochiasmatic type, and 4 cases were of third ventricle type lesions; the tumors were resected by thoroughly drilling the dorsum Sella, upper clivus, and the posterior clinoid process. Furthermore, one case of a large multilobulated lesion with significant lateral extension was resected using an endoscope endonasal approach combined with a microscopic transcranial approach.

Results: Based on the degree of tumor resection, total resection was achieved in 18 cases (initial 11 cases, recurrence 7 cases) and subtotal resection in 4 cases (initial 0 cases, recurrence 4 cases). Visual and cognitive function improved or at least preserved in all cases. Postoperative cerebrospinal fluid leakage was observed in 4 cases. The pituitary stalk was preserved during surgery in 6 cases, postoperative anterior pituitary function was maintained in 2 cases, and posterior lobe function was preserved in 3 cases. At an average follow-up period of 50 months, no recurrence was observed in all 11 of the 11 initially diagnosed cases. However, tumor recurrence was encountered in 2 of 7 total resection cases and 2 of 4 subtotal resection cases in 11 recurrent diagnosed cases.

Conclusion: Even significantly large lesions that have extended to the anterior skull base or the anterior brainstem/third ventricle can be treated with endoscopic endonasal surgery alone, provided the lesion is located in the midline. Nevertheless, combining endoscopic endonasal and transcranial surgery may be necessary if the tumor has extended laterally beyond the internal carotid artery, oculomotor nerve, or posterior communicating artery.

We studied the efficacy of endoscopic third ventriculostomy (ETV) and the possibility of shunt removal in five pediatric patients with hydrocephalus, with implanted cerebrospinal fluid shunts. Shunt removal was successfully archived in one patient with myelomeningocele (MMC) and two patients with aqueductal stenosis (AQS). However, shunt removal was unsuccessful in one patient after intraventricular hemorrhage and in one patient with an interhemispheric fissure cyst.

ETV is effective in the pathogenesis of hydrocephalus in which there is an occlusive mechanism in the ventricles and the subarachnoid space. Therefore, ETV is effective for AQS, with a high probability of shunt removal, especially in cases with a ballooning of the third ventricle floor. Patients with MMC often have concomitant hydrocephalus in infancy, and shunt implantation is often necessary because ETV is ineffective during this period. Owing to changes in cerebrospinal fluid dynamics with growth, patients become less dependent on their shunt, making shunt removal possible. However, it is necessary to consider whether ETV will be required before shunt removal. The indications for ETV and the possibility of shunt removal should be considered for each patient according to the pathophysiology of their disease.

The intrinsic lesions of the brainstem in children lead to a variety of diseases. Although their differentiation is important; however, their diagnosis is difficult via neuroradiological imaging alone. Herein, we report a pediatric case of a developmental venous anomaly (DVA) sited at the brain stem. The patient’s initial head MRI revealed an intrinsic lesion with ill-defined and brush-like contrast enhancements, which was primarily suspected to be a brain tumor. However, the atypical clinical course of the patient and neuroimaging findings such as the rapid exacerbations and remissions in a short time period, and rack of lesion volume effect observed in MRI, refrain us from proceeding with highly invasive examination and initiating any type of treatment including chemotherapy, radiotherapy and/or administration of steroids. Consequently, a complete recovery of symptoms and improvement in neuroimaging findings were achieved during the natural course of the disease. Retrospectively, MRI with contrast mater at the initial visit revealed a DVA traversing across the medulla oblongata, suggesting that the this case’s pathophysiology was based on a venous malformation of the medulla oblongata with stagnant outflow and stenosis, causing increased venous pressure and contributing to the clinical symptoms and findings on neuroradiological imaging. In this study, we report this extremely rare case of DVA with a literature review.

Complications of ventriculo-peritoneal shunts (VP shunts) in children are not rare. A few of these complications include infection, malfunction, excessive spinal fluid drainage, intraperitoneal cysts, and skin abnormalities. Herein, we report a case in which a patient with hydrocele due to peritoneal malabsorption following repeated VP shunt procedures was successfully weaned off the shunt via endoscopic third ventriculostomy (ETV).

He was born at 22 weeks 6 days gestation as one of the twins, weighing 614 grams. He was in a state of shock due to sepsis from the time of his birth, and intensive care procedures like intubation and ventilation was immediately started. Bilateral intraventricular hemorrhage was observed at 1 day of age. Simultaneously, he developed severe necrotizing enterocolitis, requiring long-term central intravenous feeding and antimicrobial therapy. When his condition was stable, a cerebrospinal fluid reservoir (CSF reservoir) was placed, and intermittent CSF elimination was started at the age of 179 days. Results of ventriculography confirmed that there was an obstruction of the midbrain aqueduct, and a VP shunt was performed at 209 days of age. Moreover, the patient required 7 shunt reconstructions (1 ventricular catheter occlusion, 1 extension by growth, 3 abdominal catheter malfunctions, and 2 shunt valve occlusions) till the age of 16 years. A shunt with siphon guard was used but slit like ventricle persisted. Adhesions after necrotizing enterocolitis caused peritoneal pseudocyst and caused a dysfunction on the peritoneal side. He had psychomotor retardation, eye movement disorder, visual impairment, and gait disorder due to spasticity in both the lower limbs. He also had a hydrocele at around 10 years of age, but the amount was small, and was considered acceptable to follow-up.

At the age of 16, he presented headache and vomiting. Head CT revealed the enlargement of the slit like ventricle. Shunt tube contrast examination showed that the ventricular catheter was patent. A post-contrast CT scan showed a large cyst formation from the abdominal cavity to the right scrotum and contrast medium inflow into the cyst. The shunt was not obstructed, and it was concluded that the hydrocephalus was aggravated due to intraperitoneal malabsorption. On the third day, the catheter was replaced with an external drainage, and the symptoms improved. MRI showed obstruction of the midbrain aqueduct and ballooning of the third ventricular floor. Based on the history, the risk of shunt occlusion was considered high. ETV was selected due to its high success rate. The first ETV was performed without complications, but the stoma was closed nine days after operation. The second operation was performed after 20 days, where we perforated two stomas on the tuber cinereum, and connected them, made a large stoma, and opened the Lilliquist's membrane, which ensured that there was no deterioration in his condition for the next ten years. His hydrocele was caused by extension of a peritoneal pseudocyst into the scrotum, which is not previously reported. Additionally, ETV should be considered for weaning from shunt dependence.

The optimal timing of ventriculoperitoneal shunt insertion (VPS) for treating post-meningitis hydrocephalus is often decided after confirming normalized cerebrospinal fluid (CSF) parameters and negative CSF cultures without any antibiotics. Herein, we present a case in which VPS insertion timing was delayed due to lack of any improvement in CSF glucose levels.

A premature female infant, weighing 1828 g and born at 33 weeks gestational age, developed Group B streptococcus meningitis on the 36th day post-birth and received ampicillin treatment. Concurrently, cerebral infarction in the right parietal and occipital lobes was confirmed via magnetic resonance imaging (MRI). A follow-up MRI on day 56 revealed hydrocephalus, necessitating extraventricular drainage. Subsequent CSF samples were obtained through ventricular drainage. CSF analysis on day 75 showed improvements, except those in glucose levels (25 mg/dl). Gadolinium-enhanced fluid-attenuated inversion recovery (FLAIR) MRI showed abnormal meningeal enhancement. Further, the possibility of bacterial overgrowth in capsular polysaccharide necessitated rifampicin treatment. Although meningeal contrast gradually improved, CSF glucose levels remained low (27 mg/dl), leading to a switch to linezolid. Ultimately, although glucose level did not normalize (30 mg/dl), abnormal meningeal enhancement on FLAIR MRI disappeared, facilitating VPS surgery on day 154. One year post-surgery, no infections have been noted.

It has been shown that factors contributing to decreased CSF glucose levels include depletion caused by bacteria, severe inflammation, potential blood-brain barrier dysfunction, and the site of CSF collection. Given that low CSF glucose levels may arise from causes other than infection, early VPS may be acceptable even if CSF glucose level does not recover post normal findings of other examinations, including negative CSF cultures.

We present a surgical case of non-traumatic acute subdural hematoma resulting from lupus anticoagulant-hypoprothrombinemia syndrome (LAHPS), a rare blood coagulation disorder. A 9-year-old girl presented to our hospital with complaints of headache. A head MRI revealed a small acute subdural hematoma, leading to her admission. The following day, the patient underwent an emergency craniotomy for hematoma evacuation due to impaired consciousness caused by an enlarged hematoma. The patient exhibited a pronounced tendency to bleed during the procedure, which presented significant challenges in surgical hemostasis. We successfully performed the surgery with a hinge craniotomy (HC) for cerebral decompression. Corticosteroids were not administered to avoid thrombosis. However, an acute subdural hematoma recurred one week later, and a second craniotomy was performed for hematoma removal. Surgical hemostasis was once again challenging during this procedure. HC was again performed, and the patient was treated with corticosteroids for two months after surgery. The postoperative course was uneventful, with no rebleeding.

Melanotic neuroectodermal tumors of infancy (MNTI) are rare, pigmented craniofacial tumors. Herein, we report the case of a melanotic neuroectodermal tumor of infancy that arose in the skull. The mass was first observed at 2 months of age and grew rapidly. Computed tomography revealed bony destruction around the left frontotemporal area, and magnetic resonance imaging revealed hyperperfusion of the tumor area. A biopsy led to the diagnosis of MNTI. The tumor grew after two courses of Vincristine, Doxorubicin, Cyclophosphamide, Ifosfamide, Etoposide (VDC-IE) chemotherapy. At 7 months of age, tumor-feeding embolization was performed, followed by craniotomy, and the bone defect was reconstructed using an absorbable plate. MNTI is a benign tumor with a low recurrence rate after total resection. However, it is necessary to perform reliable tumor resection occasionally with chemotherapy and embolization.