Objective: The mainstay of treatment for pediatric posterior fossa ependymoma (PFE) is maximal cytoreductive surgery followed by radiation therapy. PFE, especially posterior fossa group A ependymoma, which is more common in young children, has a high recurrence rate. The aim of this study was to retrospectively investigate the treatment and outcomes of recurrent PFE in children to identify optimal treatment strategies.

Materials and Methods: From 2006 to 2022, 14 pediatric patients with PFE were treated at Kobe Children’s Hospital. Relapse occurred in eight of these patients, the mean interval between first treatment and first recurrence being 23 months. The children’s mean age at the initial treatment was 4.5 years (range, 2 years to 8 years). We retrospectively reviewed the patients’ medical records for clinical course, imaging findings, treatment details and treatment outcomes.

Results: A total of 25 recurrences had occurred in the study cohort, 12 of which were local, nine metastatic or disseminated, and four both. Five of the eight patients were diagnosed histologically as WHO grade 2 ependymoma and three as WHO grade 3 anaplastic ependymoma at the time of initiating treatment. All three patients whose tumors were subjected to DNA methylation analysis had posterior fossa group A ependymomas. A total of 14 surgical procedures were performed for recurrent lesions in seven patients. One patient underwent surgery four times and one five times. The tumors were grossly totally resected in nine surgeries, sub-totally resected in two surgeries, and partially resected in three surgeries. One patient who had not received radiation during the initial treatment received conventional radiotherapy after recurrence, and two patients with metastatic or disseminated disease underwent proton beam therapy. A total of 13 Gamma Knife surgeries (GKSs) were performed on five patients (five to resected cavities, five to disseminated lesions, and three to growing local lesions). The median duration of progression-free survival after GKS was 22 months. The median progression-free survival was 3.5 months for patients with growing lesions who had not undergone prior resection, or local lesions who had undergone prior partial resection. In contrast, the median progression-free survival was 25 months for patients with small and non-aggressive lesions who had not undergone prior resection, or local lesions who had undergone prior subtotal or more resection. Six of eight patients were treated with chemotherapy for relapse: four with combination chemotherapy, five with temozolomide, and two with oral etoposide. The median durations of progression-free survival after combination chemotherapy and temozolomide treatment were 4.5 and 3 months, respectively. Five of eight patients (62.5%) died during the study period. The median survival after the first relapse for all eight patients was 49 months.

Conclusions: Recurrences of pediatric PFEs are difficult to cure and have a dismal prognosis. Tumor resection followed by GKS has some promise for tumor control after recurrence. Chemotherapy for recurrent PFE in children is unlikely to be effective. A treatment strategy that balances tumor control and maintenance of quality of life is essential.

Backgrounds: Optic pathway hypothalamic gliomas (OPHGs) are low-grade tumors, with the majority being pilocytic astrocytomas. As most cases are unresectable, a “gain time” treatment strategy should be considered until senescence of tumor growing. While the adjuvant chemotherapy may stabilize OPHG, patients occasionally experience tumor regrowth after a while. Among various treatment options for recurrence, we mainly employ repeated chemotherapy. In this study, we report the treatment outcomes of patients with OPHG at our institution.

Methods: In this retrospective study, we evaluated the treatment response of chemotherapy in recurrent OPHG cases diagnosed after 2008. Treatment responses were assessed by the best response during chemotherapy.

Results: Eighteen patients with OPHG were eligible for this study. The median age at onset was 11.5 years (range: 0-34 years). Thirteen of the 18 patients received chemotherapy for the primary tumor at onset. The median event-free survival was 29 months, and 12 of 18 patients experienced tumor recurrence. Of the patients with recurrent OPHG, eight received chemotherapy with Carboplatin (monotherapy or with Vincristine)(N＝4), Temozolomide (N＝3), or Vinblastine (N＝1). Among the four patients who received Carboplatin, three had minor responses (defined as a <50% reduction in tumor size), and one had stable disease. Of the three patients who received Temozolomide, one achieved a minor response, one had stable disease, and one had progressive disease. The patient who received Vinblastine experienced progressive disease. Two patients who showed progressive disease on Temozolomide and Vinblastine subsequently received Carboplatin and achieved minor responses. Overall, a Carboplatin-based regimen led to objective responses in4 of 6 patients with recurrent OPHG. The median follow-up period for the study was 95.7 months (range: 10.4-182 months). To date, 13 cytoreductive surgeries have been performed in eight patients after recurrence, and two patients have received radiotherapy for disease control. One patient died of a seizure 10.4 months post onset, and one patient is currently bedridden with tumoral hemorrhage seven years post onset. The remaining patients live independently, except for two patients who were nearly blind at onset. Renal dysfunction and hearing disturbance were observed as sequelae of repeated chemotherapy in 5 and 2 patients, respectively.

Conclusion: Carboplatin-based chemotherapy demonstrated efficacy in recurrent OPHG, potentially reducing the need for surgery or deferring radiotherapy. However, the sequelae associated with the increased accumulation of chemotherapy, particularly renal dysfunction, are problematic. Effective subsequent therapies, such as molecularly targeted drugs, are desirable for a gain time strategy in patients with OPHG.

Introduction: Although various surgical methods are performed to treat Chiari malformation (CM) type 1 and achondroplasia, foramen magnum decompression (FMD) and C1 laminectomy are common surgical techniques that require sufficient bone resection of the foramen magnum. Since the structure of the craniocervical transition region in both CM type 1 and achondroplasia differs from the normal structure, bone resection in FMD may sometimes be difficult. We verified surgery for CM type 1 and achondroplasia using intraoperative computed tomography (CT) at our hospital.

Materials and methods: Cases of CM type 1 and achondroplasia that were younger than 18 years and underwent surgery using intraoperative CT at our hospital between 2010 and 2022 were analyzed. We examined the number of intraoperative CT scans performed, whether additional bone resection was required after CT scans, and improvements in postoperative images and symptoms.

Results: Six cases of CM type 1 (mean age 11 years) and four of achondroplasia (mean age 7.5 years) underwent FMD and C1 resection using intraoperative CT. Although intraoperative CT was performed only once in all cases of CM type 1, additional bone removal after intraoperative CT was required for one case with an occipital bone anomaly at the foramen magnum. Intraoperative CT was performed once in two cases of achondroplasia; however, additional bone removal after intraoperative CT was required in one case. Intraoperative CT was conducted twice in two cases of achondroplasia to confirm that FMD was sufficient. The decompression width expanded by an average of approximately 4.1 mm after the first CT scans. All cases showed the attenuation of symptoms and/or a reduction in syringomyelia.

Discussion: At the craniocervical junction in achondroplasia, the thickened occipital bone invaginates perpendicularly into the foramen magnum, causing narrowing. Some cases of CM type 1 have bone abnormalities at the craniocervical junction. Due to the above reasons, the bone resection of the foramen magnum may be inadequate in these patients; however, intraoperative CT can visualize the occipital bone of the foramen magnum with easy-to-understand 2D and 3D images, allowing surgeons to confirm the extent of bone resection during surgery. Considering the risk of radiation exposure to children, it is recommended that intraoperative CT be used only in cases of Chiari malformation and achondroplasia in which FMD is difficult.

Conclusion: Although careful consideration must be given to radiation exposure in the case of children, intraoperative CT may be useful for FMD in cases of CM type 1 with abnormalities at the craniocervical junction and achondroplasia.

The number of cases of suspected child abuse that are referred to the Child Guidance Center has been on the increase, exceeding 200,000 cases per year since 2020. In line with this trend, the situation surrounding cases of suspected abusive head trauma has been changing. The existence of Nakamura type 1 subdural hematoma has been reaffirmed, and it is essential to collect not only a simple onset of symptoms (e.g., falls, external force) and medical findings, but also more detailed information on the situation of the injury (e.g., how and where the child was hit, from what height, etc.) and the family situation (potential risks). We believe it is essential to collect this information as early as possible. It is also our mission to ensure that correct information is accurately connected to specialized institutions.

Objective: Conus spinal lipomas are ectopic fat tissue attached to the spinal cord and are derived from the failure at primary neurulation, early secondary neurulation, or their junctional phase. They exhibit some pathomorphological variations between lipoma and neural tissues, requiring on-condition surgical procedures. The aims of this study were to elucidate their treatment outcomes by the types of recent lipoma classification based on embryonic stage and to discuss the relationship between intraoperative findings and outcomes.

Methods: This study enrolled 51 consecutive patients aged 0 month to 20 years (median, 3 months) who were surgically treated for the first time. The rate of neurological complications of surgery, postoperative cord-sac ratio, rate of redo untethering surgery, progression-free survival rate, and Kaplan-Meier curve for freedom from new spontaneous neurological deficits were investigated according to the types of Morota’s classification. The relationship between intraoperative findings and these prognostic criteria was analyzed.

Results: The progression-free survival rates were 100%, 85%, and 61% in Type 1, Type 3, and Type 2 lipoma, respectively. Type 2 lipoma exhibited a higher rate of neurological complications of surgery (17%) and redo untethering surgeries (17%) than the other types. Postoperative cord-sac ratio (81%) was also high in Type 2 lipoma. There was not a defining characteristic time observing new spontaneous neurological deficits.

Conclusion: Type 2 lipoma demonstrated relatively poor outcome compared with the other types. This is because of comparatively high-risk surgical procedures due to complicated pathomorphology. This also implied a wide and intricate cord-lipoma interface and deformed spinal roots, which were further encased by an extensive lipoma.

Congenital toxoplasmosis, although uncommon, is a condition grouped under the toxoplasmosis, rubella cytomegalovirus, herpes simplex, and HIV infections. We report a case of congenital toxoplasmosis manifesting as fetal hydrocephalus. A female neonate, weighing 2,224 g, was delivered through Caesarean section at 37 weeks of gestation. At 27 weeks of gestation, the patient was referred to our department owing to the detection of fetal ventriculomegaly by ultrasonography. Computed tomography and magnetic resonance imaging performed following birth revealed severe lateral ventricular dilatation and periventricular calcification. The results for Toxoplasma gondii-specific IgM antibody were in the serum were positive, and Toxoplasma gondii DNA was detected in the cerebrospinal fluid (CSF) by polymerase chain reaction. A diagnosis of congenital toxoplasmosis was established based on these findings. Oral administration of pyrimethamine, sulfadoxine, folic acid, and prednisolone was started at the age of 4 days. This treatment regimen was continued for 1 year. Furthermore, for treating hydrocephalus, an Ommaya reservoir was implanted when the patient was 3 days old, and a ventriculoperitoneal shunt was later performed around 1 month of age. At 4 months, a shunt revision was performed because of shunt obstruction caused by elevated protein level in the CSF. Early diagnosis and prompt treatment are crucial for improved growth and neurological development in such cases.

Hypoplasia of the posterior arch of the atlas (C1) is an uncommon cause of spinal canal stenosis, leading to myelopathy. We present a 9-year-old boy who started falling down frequently at the age of 5. He was born prematurely with low birth weight and exhibited intellectual disability. At the age of 8, the bilateral spasticity of his lower extremities worsened, and he was referred to our hospital. MR imaging demonstrated severe stenosis of the spinal canal at the level of the atlas, with spinal cord compression and the intensity change on T2-weighted imaging. CT revealed a bifid posterior arch of the C1 with an associated anomaly, including fused C2-3 vertebral bodies and dens hyperplasia. Dynamic cervical spine X-ray showed no instability. Due to the progression of the patient’s symptoms, he underwent a C1 laminectomy. There were no perioperative complications, and the spasticity of both lower extremities decreased postoperatively. Typically, spinal canal stenosis caused by hypoplasia of the C1 posterior arch responds well to surgery. C1 posterior arch excision alone can be effective without leading to subsequent instability.

Cerebral amyloid angiopathy (CAA) is a degenerative disease that affects the walls of the arteries in the central nervous system. According to the Boston Criteria, the diagnostic criterion for CAA is being aged 55 years or older. Further, CAA is more common in older individuals and rarely occurs at younger ages. Amyloid precursor protein, preseniline-1, and preseniline-2 gene abnormalities are known to be the cause of juvenile CAA. In recent years, there have been reports of juvenile CAA without family history. It has been reported that amyloid-β (Aβ) may be transmitted between individuals similarly to the abnormal prion protein in prion disease, as observed in cases of childhood surgical history and cadaveric dura grafts. A history of head injury is also suspected as a potential cause of the disease; however, besides head injury surgery, there are also instances where surgery for meningioma, myelomeningocele, and syringomyelia appears to have triggered the onset of the disease. There have been reports indicating that Aβ may have spread through surgical instruments during neurosurgery. Further, a report exists of the disease having been caused by the agent used for embolization of the external carotid artery; in this case, there was no history of craniotomy. In Japan, the import of freeze-dried human dura mater commenced in 1973; however, in response to the identification of iatrogenic Creutzfeldt-Jakob Disease as a potential consequence, the Ministry of Health and Welfare implemented emergency measures to stop its use in March 1997. It takes approximately 30 years from the initial surgery to the onset of symptoms of CAA, and there is a possibility that the incidence of CAA will continue to increase in Japan. In case 1, the patient, who had a history of surgery for head injury at the age of 16 years, developed right occipitotemporal lobe subcortical hemorrhage at the age of 49 years, and pathological examination led to the diagnosis of CAA. In case 2, the patient, who underwent craniotomy for chronic infantile subdural hematoma due to vitamin K deficiency, developed 11 cerebral hemorrhages starting at the age of 32 years, and pathological examination led to the diagnosis of CAA. There was no family history, and the patient was thought to have had a history of surgery in childhood. In cases of young-onset CAA with no family history, it is necessary to differentiate between CAA caused by childhood surgery.

Introduction: The prevalence of arachnoid cysts has been reported to range from 0.23-2.6%, and most commonly presents with subdural hematoma or intracapsular hemorrhage after head injury. However, epidural hematomas are rarely associated with arachnoid cysts. In this report, we describe the case of a 2-year-old boy with a giant arachnoid cyst in the middle fossa who presented with an epidural hematoma after a minor head injury.

Case: The patient was a two-year-old boy originally born at 41 weeks, with an uneventful perinatal period. Although 2-year checkup revealed delayed speech development and enlarged head circumference, no imaging examination was performed. On the day of admission, the patient fell down from a height of 50 cm. Four hours after the injury, vomiting and tendency to somnolence were observed, and the child was transported to the emergency room. A CT scan of the head revealed a linear fracture of the left parietal and temporal bone and a left acute epidural hematoma. Emergency craniotomy was performed to remove the hematoma, achieving an immediate improvement in the patient’s level of consciousness. Two months after the first operation, an endoscopic fenestration of the cyst wall was performed because the cyst remained unchanged.

Conclusion: Bruising of the thinning area caused by the arachnoid cyst may have resulted in a skull fracture, and the acute epidural hematoma may have developed significantly because of the lack of brain parenchyma directly beneath the fracture.