In infants, abusive head trauma, especially acute subdural hematomas, is an extremely poor prognosis, wherein the main factor is the widespread low-density changes observed on head computed tomography (CT). We considered this pathological condition when we retrospectively examined our cases: a total of 28 cases which included 18 cases of abuse and 10 cases of accidents. The occurrence of the extensive low-density area was observed in 15 out of 18 patients (83%) in the abuse group and 2 out of 10 patients (20%) in the accident group. This finding was found in a total of 17 cases. Thirteen cases were observed in the bilateral cerebral hemispheres, 4 cases in the unilateral cerebral hemispheres, and 9 cases in the first CT scan, of which 8 cases occurred within a few days. This phenomenon is not caused by a single medical condition, but by the complex effects of various harmful pathophysiological factors on the fragile infant brain. The pattern that occurs within a few days is not appropriate to be considered a simple ischemic change, wherein status epilepticus and hyperperfusion injury are deeply involved. The pattern observed during transport (initial CT) is a hypoxic-ischemic change caused by severe disturbance of consciousness or cardiopulmonary arrest, and it is extremely important to determine the cause of respiratory insufficiency. Deep knowledge of these pathological conditions and extensive clinical experience are essential to appropriate and accurate abuse decisions.

Objective: We describe the turnover method of cranioplasty for management of closed pediatric depressed skull fractures. This technique involves turning the depressed skull bone inside out after craniotomy.

Materials and Methods: We retrospectively investigated the clinical features, surgical complications, and bone fusion in eight patients who underwent surgery using the turnover method for closed depressed skull fractures over the past 15 years. The turnover method involved creation of a burr hole at the edge of the depressed skull bone, and bone was cut along the depressed fracture line. After craniotomy, we turned the flap over and fixed it to the surrounding bone using eight absorbable sutures. Protruding segments of the depressed skull bone, if any, were scraped using a bone rongeur, and a fragile depressed skull bone segment was reinforced with an absorbable suture.

Results: Patients’ mean age at the time of surgery was 6.4 years. The depressed bone was the parietal bone in seven and the frontal bone in one patient. The mean area of bone depression was 7.9 cm² (4.8-13.2 cm²), and the mean depth of the depression was 8.8 mm (7.4-11.9 mm). The mean operation time was 75 min, the estimated blood loss was 55.3 mL, and no patient required blood transfusion or developed infections or surgical complications. The mean follow-up period was 309 days. The largest bone defect observed on the latest computed tomography scan was 4.9 mm (mean), with good bone fusion.

Discussion: Reportedly, depressed fractures are often spontaneously repaired because bones are strongly reconstructed in infants. However, satisfactory spontaneous bone healing may not occur in children aged >1 year, and surgery is required in such cases. The depressed bone elevation method is a simple surgical technique used in such cases; however, this procedure may be associated with instability, and cranioplasty with craniotomy is the preferred approach. In patients who undergo cranioplasty with craniotomy, a plate is used for reconstruction of the depressed bone to achieve its original shape; however, the use of several artificial materials increases the risk of infection. The short operation time and minimal use of artificial materials serve as advantages of the turnover method. In many cases of depressed skull fractures, the inverted depressed skull bone conforms to the natural physiological curvature of the skull. Cranioplasty using the turnover method is useful for effective repair, and we recommend this approach as a simple and highly effective surgical method in children with depressed skull fractures.

Objective: The aim of this study was to evaluate the true state of treatments and outcomes of pediatric patients with diffuse intrinsic pontine glioma (DIPG) in Japan.

Patients and Methods: A retrospective questionnaire survey was performed to evaluate the clinical factors affecting the prognosis of rare pediatric brain tumors by the Study Group of “Nationwide Multicenter Studies to establish Standard Treatment for Childhood Brain Tumors” in the Japan Children’s Cancer Group financed through a grant from the Japan Agency for Medical Research and Development. Ninety-nine pediatric patients with DIPG aged under 15 years at diagnosis were treated from January 1, 2009 to December 31, 2013 in Japan. Patients were included in this study if the magnetic resonance imaging findings were conclusive of DIPG. Approval by the ethical committee of all participating sites was acquired before data collection. Information on the clinical characteristics, tumor- and treatment-related data, and outcome parameters were collected.

Results: Biopsy or partial resection was performed in about 20% of patients. Almost all patients received radiotherapy, and three-fourths received chemotherapy mainly using temozolomide. Median overall survival (OS) was 338 days. OS showed no significant differences between 1) photon and proton radiotherapy, 2) with or without chemotherapy, and 3) chemotherapeutic agents.

Conclusions: This largest retrospective analysis of the true state of treatments and outcomes of pediatric patients with DIPG in Japan showed that the standard therapy of DIPG must be limited to radiation therapy only. A great range of chemotherapy protocols were applied without any survival benefit between 2009 and 2013.

The recent trends and future prospects of neuroendoscopic surgery in our hospital were examined. From January 2015 to December 2021, 61 cases and 70 surgeries of children under the age of 18 years who underwent surgery using a neuroendoscope at our hospital were included. The most common surgical procedure was endoscopic third ventriculostomy (ETV) in 39 cases, of which 3 were re-ETV surgery. Of the 39 cases, 12 simultaneously underwent shunt removal and 11 simultaneously underwent tumor biopsy. The surgical procedure that has been increasing in recent years is ETV for shunt removal, and shunt withdrawal was possible in 7 of 12 (58.3%) cases. As new surgical procedures, which have been performed in recent years, 6 cases underwent endoscopic-assisted suturectomy for craniosynostosis, and 2 cases underwent choroid plexus cauterization (CPC). The role of neuroendoscope in the field of pediatric neurosurgery is vital, which will possibly increase in the future. Shunt withdrawal and avoidance by ETV and CPC also reduce the burden on neurosurgeons, such as shunt troubles and follow-up after carryover. In recent years, ETV, as the initial treatment for hydrocephalus, is targeted at children aged 6 months or older in principle from the viewpoint of success rate, and the policy is to try ETV with shunt removal when shunt malfunction occurs.

Peripheral primitive neuroectodermal tumors/Ewing’s sarcoma family tumor (pPNET/ESFT) are small, round cell tumors that have a decided predilection for young patients and commonly arise from bone and soft tissue. In this study, we present a rare case of pPNET arising from cranial dura matter in a 6-year-old girl. She presented with headache, vomiting, and growing mass in the left temporal region. Neuroimaging revealed a occupied lesion in the left temporal region which expanded to the subcutaneous and subdural spaces. The patient underwent subtotal resection of the tumor because it was hemorrhagic, and the diagnosis of a pPNET was made based on histological, immunohistochemical, and molecular genetics (EWS-FLI1 fusion gene) findings. PET scan and bone marrow aspiration showed no evidence of systemic involvement. The patient underwent adjuvant treatment with radiotherapy and chemotherapy with VDC-IE regimen. After 4.5 years, the neuroimaging showed no recurrent lesions. In case that the tumor is very hemorrhagic and the degree of adhesion to the brain parenchyma is very strong, leaving to postoperative adjuvant chemoradiotherapy instead of sticking to the complete removal through a single step craniotomy might be an option to consider.

Scurvy due to Vit.C deficiency was widely prevalent in the world during the Age of Discovery. It reduces strength of vessel wall and causes bleeding. Nowadays, it is a rare and uncommon disease. We present a case of epidural hematoma associated with scurvy.

A 4-year-old girl admitted to our hospital with symptoms of difficulty in walking, leg pain, and bleeding from the swelled gums lasting 2.5 months. The blood cell count, hemogram and coagulation factors in laboratory data were normal. She had a headache without obvious trauma. CT scanning revealed an epidural hematoma in the right frontal region. A removal of epidural hematoma with hemostasis was performed. Re-bleeding did not occur after surgery, but the anemia was progressed. Other symptoms had also gotten worse. An interview about her daily meal revealed she was extremely picky eater. As the serum Vit.C concentration was quite low, she was finally diagnosed with scurvy. After starting oral administration of Vit.C, her symptoms quickly improved.

In modern times, it is extremely rare to experience nutritional disorders in developed country. However, mal-nutrition can be occur due to some reason like this case. Intracranial hemorrhage without history of underlying disease or trauma and accompanied with multi-organ findings may be caused by nutritional disorders.

Pial arteriovenous fistula (AVF) is a rare vascular lesion which has been recently established as a distinct clinical entity from other vascular malformations. Herein, we report a rare case of spontaneous regression of hemorrhagic multiple pial AVFs.

A 4-year-old girl presented with a simple partial seizure of the right upper extremity. Computed tomography scan showed a subcortical hematoma in the left parietal lobe. Digital subtraction angiography (DSA) demonstrated a single feeder-single drainer pial AVF in the left parietal lobe, and a single feeder-dual drainer pial AVF in the left Sylvian fissure. Subsequent DSAs showed occlusion of left parietal pial AVF and significant flow reduction of left Sylvian fissure pial AVF.

Pial AVF is a rare disease and its natural history has not been elucidated. We present a rare case of spontaneous occlusion and significant flow reduction after hemorrhage.

McCune-Albright syndrome (MAS) is a disorder characterized by the triad of fibrous dysplasia (FD), café-au-lait spots (CAS), and endocrine abnormalities. Somatic mutations in the GNAS (guanine nucleotide binding protein, alpha stimulating) gene have been implicated in the etiology of MAS. We report the case of a 12-year-old male adolescent who was diagnosed with MAS following evaluation of an incidentally detected mass at the convexity of the skull. Computed tomography of the head performed at the time of a head injury revealed hypodensity in the right parietal bone, and the patient was referred to our hospital for further evaluation. Physical examination revealed that in addition to a bulge in the right parietal bone, the patient had CAS on the right shoulder. The lesions did not cross the midline and showed irregular margins. Gadolinium-enhanced T1- and T2-weighted magnetic resonance imaging of the head revealed a homogeneously enhanced mass with signal intensity equal to that of the cerebral cortex, and bone scintigraphy revealed tracer accumulation in the same region. In view of the possibility of neoplastic lesions, such as Langerhans cell histiocytosis, we removed the skull tumor 3 months after the head trauma. Based on histopathological evaluation of the resected tumor, and FD and CAS over the right shoulder, we clinically diagnosed the patient with MAS. Subsequent genetic analysis of the bone tumor tissue revealed a GNAS gene mutation, and the diagnosis of MAS was genetically confirmed. Cells with GNAS mutations can be distributed in a mosaic pattern throughout the body, including the bones, skin, and endocrine tissues. The detection rate of mutations in the peripheral blood is low, and genetic analysis of tissue samples obtained from lesions is useful for diagnostic confirmation of MAS. Diagnosis of FD based exclusively on imaging is challenging and may be inaccurate; therefore, biopsy-based pathologic and genetic analyses should be considered to confirm the diagnosis of MAS.

Chiari malformation type I (CM I) causes various morphological malformations in the posterior fossa. Here, we present two cases of CM I with internal occipital crest (IOC) duplication wherein the patients underwent surgery.

Case 1: A 3-year-old girl was diagnosed with CM I due to enlarged head circumference and developmental retardation. Computed tomography (CT) revealed IOC duplication. The patient was diagnosed with symptomatic CM I, which became a bony decompression. Foramen magnum decompression and duplicated IOC ostectomy were performed with the primary goal of IOC duplication removal. The patient's postoperative course was good.

Case 2: An 18-month-old girl was diagnosed with CM I due to developmental retardation. A head CT revealed IOC duplication. Foramen magnum decompression was performed, and the patient's postoperative course was good.

Wide foramen magnum ostectomy is important for decompression; however, in case with duplicated IOC, while the cerebellar tonsils are compressed, its ostectomy is also necessary for the decompression.

Suprasellar arachnoid cyst treatment includes endoscopic ventricle-cystostomy (VC) and ventriculo-cysto-cisternostomy (VCC). Endoscopic VCC was performed on a 15-month-old boy with suprasellar and bilateral middle cranial fossa arachnoid cysts. Several perforating branches were seen from the posterior traffic artery in the septum between the cysts, which could be damaged if the window was opened. Additionally, a small hole was found in the septum between the cysts on the left side, and communication between the cysts was considered. Hence, the septum was not opened. Postoperative MRI showed the decreased size of both suprasellar arachnoid and the bilateral middle cranial fossa arachnoid cysts. In cases where other arachnoid cysts are in contact with the suprasellar arachnoid cyst, VCC alone may be effective because of the communication between the cysts, as in this case.

The use of skin sutures using an absorbable thread (Vicryl Rapide), often used in orthopedics and plastic surgery, was discussed from the viewpoint of pediatric neurosurgery. The galea aponeurotica, not the dermis, is usually sutured to protect hair follicles. In contrast, nylon threads or skin staplers are generally used as skin sutures. These require that the threads and staples must be removed after healing. During this process, considering both pain-control and the movement of the child is necessary. The use of skin stapling tapes, such as Steri-Strip, has the advantage of not requiring suture removal. However, because of their lack of strength, they are limited to shallow, tension-free wounds. At our hospital, except for a part of the implantation of cerebral spinal fluid shunt systems and foreign bodies, such as an Ommaya reservoir, which is expected to cause spinal fluid leakage, we have experienced that the use of Vicryl Rapide for skin sutures after suturing the galea aponeurotica (in the back, among others, for skin suture after dermal suture) reduces stress on pediatric patients at the time of suture removal. Furthermore, there were no cosmetic problems or infection.

Pediatric meningioma is extremely rare and differs in characteristics from that of adult one in certain aspects. Herein, we report a case of pediatric meningioma in the lateral ventricle. The patient was a 12-year-old girl presenting epilepsy, vomiting, and right homonymous hemianopsia. Radiological examination displayed a large, enhancing tumor in the left lateral ventricle. The tumor was totally removed via craniotomy. Pathological examination revealed a fibrous meningioma. Further, the postoperative course was complicated due to progressive subdural fluid collection, which was treated using a cerebrospinal fluid shunt. A follow-up after 2 years presented no evidence of recurrence. A review of literature focusing on pediatric meningiomas in the lateral ventricle indicates the following characteristics: no difference in the sex ratio, left-sided predominance, high prevalence of hydrocephalus, and a low frequency of the meningothelial type in the histology.