There are still few reports about radical surgical resection for midbrain gliomas. Considering the surgery of midbrain gliomas, it is reasonable to refer the classification reported by Cavalheiro, et al. They classified midbrain gliomas into anterior (ventral), central, and posterior (dorsal) type on the basis of surgical approaches. It is possible to achieve total removal of non-infiltrating posterior type midbrain gliomas because they tend to be exophytic and extend backward. In such cases, the supracerebellar infratentorial approach or the occipital transtentorial approach has been used without requiring brainstem incision. We experienced two central-posterior type rosette-forming glioneuronal tumors and one posterior recurrent extention of an anterior-central-posterior type pilocytic astrocytoma and achieve radical resection via occipital transtentorial approach in combination with intraoperative motor-evoked potential monitoring and mapping without damage of pyramid tract. In this report, we described these surgical methods and reviewed reports emphasizing the radical surgery of midline gliomas.

Objective: Intraventricular hemorrhage (IVH) after proximal ventriculoperitoneal (VP) shunt catheter removal is a well-known complication. IVH is usually caused by disruption of the choroid plexus surrounding the catheter with inappropriate surgical traction. The blood clots may cause an early occlusion of the ventricular catheter. Inserting a stylet into the catheter and using monopolar cautery may reduce this complication and increase the success rate of ventricular catheter removal. Here, we report the treatment results of this technique at our institution.

Materials and Methods: A retrospective study was conducted between January 1, 2008, and December 31, 2020, at the Osaka Women’s and Children’s Hospital. Ventricular catheter removal was performed 177 times. Except for removals due to infection, corruption of shunt system, abnormal location of ventricular catheter, vulve or peritoneal catheter and so on, Ventricular catheter removal for ventricular catheter occlusion was performed 71 times. 3 of 71 revisions were excluded because of occlusion immediately after surgery due to blood clot or debris, so 68 revisions were examined in the end. First, we attempted to remove the catheter using gentle traction. If the surgeon felt resistance, a stylet was passed down through the ventricular catheter, and electrocautery was applied by the monopolar coagulator. The ventricular catheter was then removed again. Postoperative computed tomography (CT) was performed within 3 days to observe IVH and other complications. The patient’s age at ventricular catheter placement, rate of applying the technique, success rate of removing the ventricular catheter by this technique, periods from ventricular catheter placement to subsequent removal, and presence of IVH, complications and shunt survival by the technique were reported.

Results: Of the 68 catheters, 39 were removed by gentle traction (traction group), while the electrocoagulation method was applied to 29 catheters (electrocoagulation group). Twenty-four of the 29 revisions were achieved using monopolar coagulation. In contrast, ventricular catheter removal was not possible in 5 of the 29 revisions due to hard adherence. In the traction group, IVH was recognized on postoperative CT scans in 2 revisions. In contrast, IVH was reported in 7 revisions in the electrocoagulation group, only one of which required re-revision owing to obstruction of the catheter.

Discussion: Leaving the ventricular catheter is undesirable because it can cause infections. Once infection due to VP shunt occurs, removal of shunt system as well as antibiotic administration should be done. In our institution, removal of ventricular catheter could be achieved using monopolar electrocoagulation in most cases, and the incidence rate of IVH is lower than that in previous reports. Monopolar electrocoagulation is useful for easy removal of ventricular catheters and can prevent IVH.

Objective: The ribbon procedure, which was established at Okayama University, is the indirect revascularization of the anterior cerebral artery (ACA) territory in patients with moyamoya disease (MMD). The aim of this study is to evaluate the surgical and long-term outcomes of the ribbon procedure for pediatric patients with MMD.

Materials and methods: The records of 36 consecutive pediatric patients (<16 years of age) who were surgically treated were reviewed. Operation time, bleeding volume, perioperative complication, angiographical collateral formation 6 months after the procedure, change in frequency of lower limb weakness, and most recent modified Rankin Scale (mRS) score were evaluated.

Results: Seven patients (19%) underwent the ribbon procedure. The mean age was 6.6 ± 3.0 years and the mean follow-up period was 55 ± 29 months. Four patients underwent the ribbon procedure as an initial treatment and three underwent it as a secondary treatment. The mean operation time was 160 min and the mean bleeding volume was 16.6 ml. Perioperative complications occurred in 2 patients: one had wound infection, and the other had asymptomatic cerebral infarction in the ACA territory and chronic subdural hematoma. The transient lower limb weakness disappeared in six patients postoperatively. Six patients exhibited excellent or good collateral formation in the ACA territory on angiography 6 months after the ribbon procedure. No patients experienced stroke during the follow-up period. Most recent mRS scores was 0 in 5 patients, 2 in one patient, and 4 in one patient.

Conclusion: The ribbon procedure is a safe and effective treatment for pediatric patients with MMD.

The number of long-term survivors of pediatric medulloblastoma has been increasing due to improved treatment options. However, secondary cancer can be an issue for long-term survivors. Of the 27 patients with cerebellar medulloblastoma treated at our institution, two developed cerebellar glioblastomas after the initial treatment at five years and 11 months and 22 years and eight months, respectively. These two cerebellar glioblastomas were clinically diagnosed as radiation-induced secondary cancer.

Both of the two cases we clinically diagnosed as radiation-induced glioblastoma had negative IDH-1, EGFR expression and positive p53 expression in immunohistochemistry, and the molecular evaluation was consistent with radiation-induced secondary cancer.

Management of central nervous system (CNS) germ cell tumors (GCTs) is one of major issues in pediatric and young adult patients. Germinoma has come to have a relatively good prognosis, yet recurrence occurs infrequently. Non-germinomatous GCTs (NGGCTs) are still challenging and require intensive chemotherapy with minimized radiotherapy. GCTs arise preferentially in midline structures, including pineal gland and neurohypophysis. Accordingly, patients suffer from hydrocephalus, ophthalmological and neuroendocrinological symptoms. Histopathological components of GCTs are classified into 5 subtypes: germinoma, teratoma, choriocarcinoma, yolk sac tumor, and embryonal carcinoma. GCTs often consist of more than one of these components. Diagnosis and decision making on treatment strategy depend both on biopsy specimens and on tumor markers including alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG).

We report here our series of 24 (male: female = 20: 4, ages: 9-32, mean: 19, median: 19) patients with CNS-GCTs treated from 2000 to 2020. Eighteen of them suffered from germinoma and others from NGGCTs (2 yolk sac tumor, 2 choriocarcinoma, 1 embryonal carcinoma and 1 immature teratoma). Ten germinoma patients and 2 yolk sac tumor patients were treated with ICE regimen (IFO/CDDP/VP-16). Eight germinoma patients were treated with CARE regimen (CBDCA/VP-16). Three NGGCTs patients (1 choriocarcinoma, 1 embryonal carcinoma and 1 immature teratoma) diagnosed after 2008 were treated with Japanese Pediatric Brain Tumor Consortium (JPBTC) regimen (CPA/CDDP/VP-16+intrathecal MTX) by the dedicated pediatric oncologist. Radiotherapy was basically 24 Gy whole ventricular irradiation with optional local boost for germinoma, while 47.6∼55 Gy involved-field radiotherapy sometimes including 22.4∼25.6 Gy craniospinal irradiation for NGGCTs. One of NGGCTs (yolk sac tumor) treated with ICE regimen before 2008 died 17 months due to dissemination, while other one of NGGCTs (choriocarcinoma) with family history of schwannoma experienced lumber spinal root schwannoma 10 years after initial treatment. One of germinoma experienced local recurrence 15 years after treatment, while other one died 19 years after onset unrelated to primary disease.

All types of GCTs are treated according to the best contemporary protocol and dedicated specialists obtained. Patients must be followed up for long-term, not only due to their neuroendocrinological sequelae, but also due to potential late complications including secondary cancer and recurrence.

The effect of surgical resection on recurrent pediatric malignant brain tumors is still controversial. We analyzed cases of seven patients who underwent surgical resection of recurrent lesions.

In all patients, the Karnofsky performance status was not aggravated after multiple surgeries. The progression free survival periods in four patients with posterior infratentorial ependymomas were 10, 18, 24, and 35 months. Three of the four patients survived about 100 months following multiple repeat surgeries and re-radiation. However, one patient died because of disseminated lesions 21 months after the initial therapy. These results suggested that repeat surgeries for recurrent ependymomas were effective unless disseminated lesions were detected. However, the effects of repeat surgeries were modest in our patients with glioblastoma. Based on the World Health Organization’s Classification of Central Nervous System tumors, 5^th Edition, these tumors were diagnosed as diffuse astrocytoma, IDH-mutant, grade 4, and diffuse hemispheric tumor, H3 G34-mutant. Although these tumors did not recur as leptomeningeal dissemination, local tumor control was very poor by repeat surgeries. Finally, one infant case with SHH, TP53-wild type medulloblastoma survived more than 100 months following repeat surgeries and chemotherapy alone. The indication of repeat surgeries for medulloblastoma might be limited; thus, precise molecular and histological information was useful to determine therapy.

Background: In recent years, medical professionals have begun to acknowledge the significance of fertility preservation in the treatment of young people and children who have been diagnosed with cancer. We opened the AYA cancer board in 2018 with the intention of actively providing information to patients as well as family members. We report on our experience with fertility preservation as well as its current state for the treatment of malignant brain tumors.

Methods: From 2015 to 2021, we retrospectively reviewed the medical records of children and adolescent patients with malignant brain tumors who were treated at our institution. We collected data on the diagnosis, the presence of children or partners, informed consent for fertility preservation, the desire for fertility preservation, and the modalities of fertility preservation.

Results: A total of 68 individuals with malignant brain tumors were treated, with 22 receiving informational counseling about fertility-preserving informed consent and 10 undergoing fertility preservation (M:F=9:1). Only 3/32 patients had an informed consent about fertility preservation before the AYA cancer board opened, but 19/36 patients did. According to the classification of the diseases, informed consent of fertility preservation was carried out in 12/19 cases of germ cell tumor, 5/9 cases of embryonal tumor, 3/9 case of low grade glioma, 2/29 cases of high grade gliomas, and 0/2 cases of primary central nervous system lymphoma. Fertility preservation can be hindered by a number of factors, including severe neurological symptoms, the requirement of immediate treatment, pituitary malfunction, not yet having had spermarche in boys, and the avoidance of surgical intervention in girls.

Discussion and conclusion: By developing an in-hospital system and simplifying the flow of patient referral, it has been confirmed that the medical staff’s awareness of fertility preservation has grown. On the other hand, it was discovered that younger children with malignant brain tumors and/or individuals with pituitary malfunction had difficulty with fertility preservation. Future research is required to resolve these issues.

We experienced a case of very low birth weight infant with intraventricular hemorrhage and necrotizing enterocolitis (NEC) repeated shunt malfunction due to the abdominal cerebrospinal pseudocyst (APC) after ventriculoperitoneal shunt (VPS). Ventriculoatrial shunt was useful in patients with recurrent APC, and reconstruction by VAS should be considered early in high-risk patients. As the progress of neonatal care, there would be more opportunities to treat infants with more complicated and serious conditions such as post-hemorrhagic hydrocephalus with NEC.

The treatment strategy for traumatic optic neuropathy is mainly high-dose steroid therapy and optic nerve decompression, but no standard treatment has been established. The main symptom of traumatic optic neuropathy is rapid loss of visual acuity, but visual acuity examination can be difficult in cases of impaired consciousness due to head trauma or in children. We report a case of traumatic optic neuropathy with improved visual acuity after transcranial optic nerve decompression.

A 14-year-old boy fell into a gutter while jogging and bruised the outer part of his right eyebrows. Immediately after the injury, his right visual acuity was maintained, but the next day, his right visual acuity deteriorated to hand motion. Computed tomography showed a fracture of the lateral wall of the right orbit and of the optic canal. Twenty-seven hours after the injury, a craniotomy was performed to remove a fractured bone fragment. Visual acuity and the visual field improved after the operation.

Helpful points for diagnosing traumatic optic neuropathy and transcranial optic nerve decompression surgery are discussed. To make an appropriate diagnosis, it is important to suspect traumatic optic neuropathy if the lateral eyebrows are bruised and to confirm a relative afferent pupillary defect (RAPD) in patients who cannot complain of visual loss. In optic nerve decompression surgery, it is advisable to select the target decompression site based on the fracture and determine the approach route and method according to the surgical experience at each facility.

Traumatic intracranial aneurysms (TICAs) are rare, occurring in fewer than 1% of all patients with cerebral aneurysms. TICAs can result from a mild closed head injury and are more common in the pediatric population than in adults. TICAs are formed usually in the anterior circulation, and less than 10% of them occur in the posterior circulation. There are few reports of TICAs arising from the posterior inferior cerebellar artery (PICA). Herein, we describe a pediatric case of a TICA developed on the origin of a PICA after a blunt head injury.

A 9-year-old boy lost consciousness after hitting his left lower jaw on the ground after being thrown down. On arrival in the emergency department, his Glasgow Coma Scale (GCS) was 12 (E3V4M5) and a computed tomography (CT) brain scan demonstrated subarachnoid hemorrhage with acute hydrocephalus. A CT angiography revealed no vascular abnormalities or aneurysms. After the emergency placement of an external ventricular drain, his consciousness was noticeably improved. On day 7, a digital subtraction angiography (DSA) revealed a small, newly developed saccular aneurysm at the origin of the left PICA and suggestive of a TICA. A follow-up DSA was conducted 3 months later, which showed a remarkable growth of the aneurysm, and we performed intra-aneurysmal coil embolization intending to preserve the PICA. Six months after coil embolization, some filling of the aneurysm due to coil compaction was confirmed. The patient was re-treated successfully with coil embolization to the small part of the residual aneurysm. For 10 years after the second embolization, the complete obliteration of the aneurysm has been confirmed on DSA and magnetic resonance angiography. The patient has remained free of symptoms.

The possible mechanism of TICAs in children may be due to the pediatric cervical spine and craniocervical junction being relatively mobile, exposing the vertebral artery (VA) to stretching and shearing forces at the head and face trauma. Especially, when the PICA starts immediately after the dural penetration of the VA-as in the present case, where the PICA fixed to the dura mater is subject to rotational acceleration-a pseudoaneurysm may form at its origin. We affirm that repeat vascular imaging is imperative for prompt diagnosis and management of a TICA, even if the initial imaging was negative. Endovascular coil embolization with parental vessel preservation should be considered as the first treatment option for a traumatic PICA pseudoaneurysm in children, from a less-invasive perspective.

This study investigated CSF leak that occurred in five adolescents (3 males, 2 females) following COVID-19 vaccination. Mean patient age was 15.6 years (range, 14-17 years) at onset. CSF leakage was diagnosed based on the findings of CT myelography and radioisotope (RI) cisternography in one case, and by the effectiveness of epidural physiological saline injection in the remaining four cases.

All patients had received the Comirnaty vaccine (BNT162b2, BioNTech/Pfizer) and experienced one or more symptoms such as intractable headache, vertigo, fatigue, or visual disturbance. All patients underwent neurological examinations but a diagnosis was not reached in any case.Some cases were diagnosed as mental problem. Three patients were completely disabled following vaccination and unable to carry out any self-care, one was capable of only limited self-care, and one was unable to perform any work activities.

Subsequent treatment with an epidural blood patch (EBP) was effective in all cases. Three patients (60.0%) completely recovered to full unrestricted activity at pre-disease levels, and two patients (40.0%) achieved partial recovery. In patients who experience various intractable symptoms such as headache after COVID-19 vaccination and in whom treatment is ineffective, CSF leakage should be considered in the differential diagnosis.

Retinal hemorrhage (RH) is frequently associated with abusive head trauma (AHT): however, little is known about the relationship between preretinal hemorrhage (PRH) and visual impairment. Here we report the case of an AHT, who had RH and PRH on a long-term basis. The infant was a seven-month-old female and presented bruises on the left side of her face. Her father reported that she had hit her face on a baby seat because of a loosened seat-belt. No one but her father was in the car at the time. As she showed generalized convulsions the next day, she was transferred to our hospital by an ambulance. At the first examination, she showed status epilepticus, and was therefore intubated and controlled using a respirator under sedation. Computed tomography revealed an acute subdural hematoma in both the left cerebral hemisphere and interhemispheric fissure. On ophthalmologic examination on day 2, massive RHs were extensively observed in both eyes. In addition, a small right PRH and large left PRH were observed. The left PRH extended over the entire macular region. Diffusion-weighted magnetic resonance imaging (MRI) performed on day 6 after status epilepticus stabilization revealed extensive hyper-intense lesions in the subcortical white matter bilaterally. At follow-up on day 39, the right RH had disappeared. A small amount of the right PRH and left residual RH disappeared on day 55. However, a large left PRH was still observed in the macular region, and left internal strabismus was observed on day 104. All ocular fundus hemorrhage disappeared on day 167. MRI performed on day 319 revealed mild brain atrophy in the left occipital lobe, without any evidence of parenchymal lesions. No macular or optic disc lesions were observed and the patient was diagnosed with sensory strabismus on day 331. Her development appeared normal except for left internal strabismus on day 382. We presumed that a large PRH persistently covering the entire macular region in the high visual sensitivity period may have affected the visual function due to form deprivation amblyopia. This amblyopia may have caused the sensory strabismus.

A boy aged 12 years consulted a local doctor due to rhinolalia aperta and dysphagia and was found to exhibit paralysis of the right soft palate. A head MRI scan revealed the possibility of an intracranial tumor, and he was referred to our department on day 14 after the onset of symptoms. The symptoms had resolved by day 8, and at the time of examination by our department, the rhinolalia aperta and dysphagia had disappeared. Although the head MRI FLAIR images displayed signs of a tumor-like artifact in the right ventral medulla, it was not visible in the sagittal view and was thus determined to be an artifact. Contrast-enhanced MRI revealed swelling of right cranial nerves IX and X, which were enhanced. Since no antecedent infection and rapid improvement in neurological symptoms was observed, a diagnosis of idiopathic soft palate paralysis was made.