Introduction: Most important feature of exoscopic surgery is that the small optical body improves the operability of surgery. We introduced an exoscope, ORBEYE (OLYMPUS, Japan), in April 2022, and have shifted from microscopic to exoscopic surgery in both adult and pediatric cases. We review our experiences with the use of exoscope and discuss future prospects and issues.

Materials and methods: The usefulness and problems of exoscope were examined in cases between April 2022 and May 2023.

Results: Forty-six pediatric neurosurgical operations were performed during the period, and 21 of them were performed using exoscope. Of these, 7 patients had supratentorial tumors (including choroid plexus hyperplasia), 5 posterior fossa tumors, 3 myelomeningoceles, 1 cephalocele, 1 Chiari malformation, 2 skull tumors, 1 intracerebral hemorrhage, and 1 acute epidural hematoma. No shunts or drainage procedures were performed using exoscope. In all cases, exoscope could be used to complete the operation. Complications included an acute epidural hematoma resulting from cranial pin fixation in a case of medulloblastoma.

Discussions: In a case of craniopharyngioma with cystic components in supra- and infra-tentorial regions, craniotomies in anterior and posterior cranial fossa were performed in the same position, which was particularly useful. In cases of posterior cranial fossa tumor, it was easy to observe the blind spot. The exoscopic approach allowed the surgeon to easily change the visual axis in directions that are difficult to observe with a microscope. In children, the position of the patient may be limited, and the exoscope could be a useful modality. On the other hand, the operability was felt to be poor during fine manipulations because of the directional gap between visual and hand movements. Practice and training are necessary due to the problem of hand-eye coordination.

Background: Helmet therapy for positional cranial deformities in infants has become widespread. Our hospital opened an outpatient clinic for correcting the head shape of infants in August 2022 in collaboration with the Departments of Pediatrics and Neurosurgery. We describe our findings on the differences in cranial measurement using a 3-dimensional scanner and an exclusive vernier micrometer (Craniometer^Ⓡ), as well as ultrasound testing for cranial suture evaluation.

Materials and methods: This study included 137 patients who visited our outpatient clinic for correcting the head shape of infants between August 2022 and May 2023. The purpose of the visit, age in months/days at the time of the visit, and results of the cranial suture ultrasound were all recorded. Craniometer^Ⓡ and 3D scanning were used to quantify cranial asymmetry (CA) and cephalic index (CI). The outcomes of these measurements were compared.

Results: The ages of the patients ranged from 77 to 779 days (median, 152 days). Overall, 115 patients had plagiocephaly (2 with spasmodic torticollis), 17 had brachycephaly, 12 had square shape at crown of head, 6 had long vertical heads, 4 had ear malposition, 3 had cephalhematoma, 1 had a bulge in the front region, 1 had a bulge in the temporal region, 1 had suspected closed anterior fontanelle, 1 had suspected scaphocephaly, 1 had suspected trigonocephaly, and 1 had suspected microcephaly. The reasons for the visits partially overlapped. A total of 130 patients underwent ultrasound examination, with the exception of 7 patients who underwent imaging examinations performed by a previous physician. The age of the 130 patients ranged from 77 to 366 days, with a median age of 146 days. All cranial sutures were visible except the frontal suture. Partial or complete fusion of the frontal suture was observed in 0%, 3.2%, 19.4%, 44.1%, 60%, and 100% at 2, 3, 4, 5, 6, and 7-12 months of age, respectively. At the request of their parents, 86 patients underwent 3-dimensional scanning. When the physicians’ measurements of CA and CI by Craniometer^Ⓡ were compared with the accurate results obtained via 3-dimensional scanning, we found that 10.3% measured the CA as 5 mm milder than it actually was, and 2.3% measured it as 5 mm more severe than it actually was. With regards to brachycephaly, 0% estimated the CI to be >3% milder than it was, indicating an underlying tendency to overestimate severity (−5.7% to 2.8%, median −1.1%).

Conclusions: Ultrasound was useful for confirming suture fusion status in infants <12 months of age. Our findings indicated that measurements by Craniometer^Ⓡ tended to overestimate the severity of CI.

Background: There are no sufficient guidelines to determine the surgical indications for asymptomatic lesions of radiologically suspected low-grade glioma. When the patient is a child, it is sometimes difficult to propose a treatment plan due to the patient’s difficulty in making decisions and the influence of parental concerns. The clinical and imaging biomarkers to estimate the clinical course of the lesions would have high clinical value. We investigated the natural course of asymptomatic lesions of radiologically suspected low-grade glioma.

Materials and Methods: This retrospective study was approved by our institutional review board. In all cases, brain tumors were diagnosed incidentally or found in a medical checkup. Lesions of radiologically suspected low-grade glioma were confirmed by subsequent MR examinations. Patients who chose a follow-up policy until the tumor increased in size and/or became symptomatic were enrolled in this study. We excluded patients with contrast enhanced lesions and insufficient follow-up periods of less than 3 years. Patients with an increase in lesion size within 3 years and who were diagnosed with glioma based on the examination of surgical specimens were included in this study, regardless of whether a tumor or non-neoplastic disease was initially suspected.

Results: Twenty-nine patients were included in this study. The lesion locations were as follows: frontal lobe (n＝13), parietal lobe (n＝3), insular gyrus (n＝3), thalamus (n＝3), cerebellum (n＝4), corpus callosum (n＝1), and the midbrain (n＝2). During the follow-up period, eight patients showed no significant change, four showed a decrease in lesion size, and 17 showed an increase in lesion size. Among the 17 patients, 14 were confirmed to have gliomas after surgical resection. We divided the patients into two groups: patients of <18 years of age and patients of ≥18 years of age. A decrease in the tumor size was only observed in the younger age group; the difference was statistically significant (P<0.0001, Fisher’s exact test). We further analyzed the T2-FLAIR mismatch sign and found that its sensitivity and specificity for the diagnosis of astrocytoma were not high in young patients or for incidental lesions.

Conclusion: Considering that asymptomatic non-contrast-enhanced tumors in patients younger than 18 years might not enlarge, careful observation without surgical intervention proved to be a valid option. On the other hand, the risk of these lesions increasing in size in patients who are 18 years and older should be recognized. Careful follow-up is necessary to avoid missing the opportunity to provide appropriate treatment.

Introduction: The use of intrathecal baclofen (ITB) therapy for pediatric spasticity has increased since its approval in 2007. Our center has performed ITB pump implantation since 2018. In this study, we retrospectively reviewed ITB cases and reported the safe implementation of ITB therapy and its complications at our center.

Purpose and Methods: We reviewed the data of 22 patients who underwent this procedure by 2022 including age at surgery, height, weight, BMI, primary disease, presence of gastrostomy, ventriculoperitoneal shunt (VPS), degree of scoliosis, use of a hybrid operating room, induction time, operation time, blood loss, and complications. Additionally, the induction time, operation time, blood loss, and complications were investigated by dividing the patients into two groups, each with 11 cases, based on the chronological order for the first and second halves.

Results: There was one case of catheter-related complications and one case of cerebrospinal fluid leakage. Furthermore, there was one case of difficult puncture. The induction time was significantly shorter in the second half group (86.1 min in first half group vs. 76.2 min in second half group, P＝0.003). Although there was no significant difference in the operative time, the mean operative time was reduced by 21.5 minutes in second half group. No significant differences were observed in blood loss.

Discussion: In second half group, in which hybrid operating rooms were used more frequently, the time required for induction was reduced by discussing and sharing the positional settings with operating room nurses during preoperative evaluation. Furthermore, the fluoroscopy tube angle at the time of the preoperative evaluation puncture was recorded and reproduced, which was thought to have reduced the operating time. Preoperative evaluation of patients with severe scoliosis is important not only to determine the efficacy of ITB, but also to plan pump implantation. Aspiration from the catheter access port and computed tomography myelography are considered useful for the diagnosis of catheter obstruction. In addition, a compromise between catheter placement height and catheter insertion under direct vision may be useful for avoiding complications in patients with severe scoliosis. difficult catheterization and perioperative shunt dysfunction is necessary. In severely emaciated patients, it is necessary to pay attention to spinal fluid leakage and delayed wound healing.

Purpose: In recent years, helmet therapy for cranial deformities has become possible in Japan, and treatment often requires outpatient treatment. The most important thing is to differentiate between craniosynostosis: CS and physiological deformities such as positional head deformity: PHD, however in outpatient settings, a high proportion of the family’s concerns about future disadvantage of cranial deformity are addressed. There are concerns that patients may be transitioned to helmet therapy without proper diagnosis. In this study, we examined the patients who visited our department’s outpatient department with the chief complaint of cranial deformity.

Methods: We investigated the reasons for consultation and cranial morphology of patients who visited our outpatient department with cranial deformity as their chief complaint over a 2-year period starting January 2020. Cases referred for treatment of CS were excluded. Differentiation from CS was made based on physical findings, and head XP and CT were performed when necessary.

Results: There were 291 cases (190 males, 101 females, age at presentation: mean 5.7±5.3 months, median 5 months, mode 4 months), 37 cases were found to have deformities during infant check-up, and 254 cases were diagnosed by their parents. Four patients (1.4%) were diagnosed with CS (lambda synostosis 1, sagittal suture synostosis 2, and coronal suture synostosis 1). Three cases were diagnosed at infant check-up (at 2 months, 8 months, and 1.5 years old), and one case (at 3.5 years old) was diagnosed after being examined by a family member. Cases other than CS included 258 cases of positional head deformity: PHD, 11 cases of metopic ridge, 3 cases of ossifying cephalhematoma, and 16 other cases.

Discussions: As interest in PHD increases in pediatric neurosurgery outpatient clinics, it is thought that patients will become polarized into those seeking cosmetic improvements and those seeking to alleviate concerns about other disadvantages. For the former, we believe it is desirable to respond to the wishes of the patient’s family in order to meet the needs of aesthetic medicine. In the latter case, the response may vary depending on what is considered disadvantageous, so it is desirable to provide accurate information for PHD. In outpatient treatment of PHD, the first step is to differentiate it from pathological diseases such as CS, and before the stage of neck stabilization is reached, we should provide guidance on appropriate physical countermeasures. If helmet therapy is to be performed after neck stabilization, obtaining sufficient understanding from the patient’s family is considered to be essential.

A 4-year-old girl was referred to our institute for recurrent transient left-sided weakness and abnormal findings on magnetic resonance angiography. She had a family history of neurofibromatosis type 1 (NF1) and multiple pigmented skin lesions from the birth. Cerebral angiography showed severe stenosis of the terminal portions of the right internal carotid artery and abnormal plexiform vascular networks similar to the twig-like middle cerebral artery (MCA) that connected the proximal part of internal carotid artery stenosis to the proximal middle cerebral artery. Ethmoidal moyamoya vessels were also noted. A small plexiform vascular network was also observed around the stenosis of the left posterior cerebral artery, Although the plexiform vascular networks was similar to twig-like MCA that is regarded to be congenital, the location of these structures is different from where twig-like MCA exists, and recent onset of ischemic symptoms and hemodynamic disturbance suggested acquired disease. Therefore, we diagnosed her as NF1-related moyamoya syndrome (Suzuki staging of the right/left was stage III/IV). Whole-exome sequencing identified a missense variant of NF1 gene (c.2509T>C, p.W837R, rs587781747) with intact RNF213 gene. This NF1 missense gene variant may induce neurofibromin dysfunction and disruption of the Ras-MAPK pathway, leads to smooth muscle cell proliferation and thickening of the tunica intima, and causes arterial stenosis and these unique plexiform networks that is rarely observed in sporadic moyamoya disease.

A female neonate was born at term with congenital large skin-covered occipital encephalocele. Magnetic resonance (MR) imaging at 3 days of age showed brain parenchyma protruding extracranially from a bony defect in the parieto-occipital region, and the size of the mass was 70 mm in diameter. She underwent resection of encephalocele and cranioplasty. Postoperative computed tomography (CT) showed no hemorrhage or hydrocephalus. MRI 6 months after resection of encephalocele revealed enlarged ventricular structures and multiple cysts, and she underwent cysto-peritoneal (CP) shunt, fenestration of cysts and cranioplasty. Preoperative imaging studies are important to evaluate for the presence of protrusion of lateral ventricular structures and vascular inflows into the encephalocele. A large encephalocele must be amputated at the base, as a brain protruding outside the cranium is considered to have lost significant neurological function. If the bone defect is not covered with an absorbable plate or other material during the initial surgery, there is a risk that the brain will protrude again postoperatively. In addition, hydrocephalus is likely to occur in the chronic phase and careful follow-up is essential. Since cranial defects never form spontaneously, it is advisable to perform cranioplasty with an autogenous bone fragment as a rotational flap with periosteum during the secondary surgery, if possible. The key points of our surgical technique are described in detail.

The patient is a 1year 7months old girl. A right eyelid subcutaneous hematoma (raccoon eye) and an epidural hematoma-like findings were found on head CT. Intraoperative findings revealed a hematoma as well as bone abnormalities and an epidural soft tissue tumor. Postoperative whole-body CT revealed a right adrenal tumor, and the pathological findings of the tissue specimen removed at the time of surgery led to the diagnosis of neuroblastoma. Neuroblastoma is known to have an improved prognosis with early intervention. However, neurosurgeons are unfamiliar with this disease, and knowledge of this disease can contribute to early intervention without assuming that the combination of eyelid hematoma and acute epidural hematoma-like findings was caused by trauma

Extra-axial fluid collection (EAFC) can occur following surgery or trauma. Here, we report a case of EAFC which developed after the endoscopic removal of a large atypical teratoid/rhabdoid tumor (AT/RT) in the left temporal lobe. A girl, aged 1 year 9 months, with a left temporal AT/RT measuring 71 mm in maximum diameter underwent endoscopic surgery through a small craniotomy. Subtotal resection of the tumor with a small-diameter cavitron ultrasonical surgical aspirator was performed through an approximately 2 cm cortical incision. The inferior horn of the left lateral ventricle was opened. Two weeks after surgery, she developed an EAFC accompanied by vomiting and right hemiparesis, requiring emergency drainage. The symptoms disappeared after approximately two months, with improvement in the EAFC. If the ventricle is opening during tumor removal through a small cortical incision, and cerebrospinal fluid flow is impaired, the possibility of symptomatic EAFC should be considered.

This case report presents a rare occurrence of a 14-year-old boy who was initially admitted with convulsion and subsequently diagnosed with acute epidural hematoma (AEDH) on the right side. He was born preterm, with low birth weight, and as the second twin of a dichorionic diamniotic pair. He had a history of surgery at the age of one for a diagnosis of hypospadias and bifid scrotum; however, intraoperative bleeding findings were normal. This AEDH was unrelated to any fractures, and intraoperative findings ruled out bleeding associated with the middle meningeal artery or other fractures. These peculiarities prompted speculation regarding the etiology of the bleeding. During surgery, approximately 1,500 ml of blood was observed. Subsequent postoperative investigations, prolonged low platelet counts indicated a high likelihood of immune thrombocytopenia (ITP). Platelet count was elevated after helicobacter pylori eradication. ITP-associated non-traumatic AEDH is under-reported. Therefore, with or without a history of trauma, hematological diseases, including ITP, should be considered as a course of AEDH.