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The purpose of treatment for unilocular intracranial cysts (UICs) is to release elevated intracranial pressure. Neuroendoscopic fenestration (NF) is one of the most effective and minimally invasive options for treating UICs, especially in young children; however, the optimal location and number of fenestrations, the necessity of using endoscopic third ventriculostomy (ETV) in combination with fenestration, and the course of treatment are not well known. We retrospectively reviewed the hospital records between 2012 and 2019. The patients were studied in terms of sex, age at surgery, preoperative symptoms, cyst localization and size, course of treatment, ventricular diameter, developmental assessment, anatomical location, and the number of fenestrations. There were four eligible patients in the relevant period: two boys and two girls. The median age at the time of surgery was 16 months. With regard to the location of the cysts, there were two cases of cavum velum interpositum (CVI), one case of quadrigeminal cistern, and one case of an isolated lateral ventricle. The most common preoperative finding was an enlarged head circumference. All the patients were treated with NF, including one case of reoperation after open head surgery. Postoperatively, we used the frontal and occipital horn ratio (FOHR) to evaluate the ventricular size. The average reduction in the FOHR was 0.003. In the most recent developmental assessment or examination during the follow-up period, two patients showed normal development, and two patients showed developmental delay. Based on our past experience and reports, we believe that it is recommended to perform two fenestrations for a single cyst. This is because it creates a flow of cerebrospinal fluid (CSF) within the cyst into normal CSF reflux. For lesions with obstruction of the aqueduct, such as cysts in the quadrigeminal cistern, ETV should be considered if it can be performed safely, in preparation for the worsening of hydrocephalus due to obstruction by enlargement of the cyst.

Introduction: Moyamoya syndrome associated with Williams syndrome is very rare but has been reported to have severe outcomes. Here, we reported a case of Williams syndrome with moyamoya syndrome that was confirmed by the presence of an RNF213 mutation. Case Presentation: A 6-year-old boy with Williams syndrome presented with right hemiparesis induced by hyperventilation. Magnetic resonance angiography and cerebral angiography showed severe stenosis of the bilateral internal carotid arteries and development of moyamoya vessels. Genetic analysis identified a heterozygous c.14576G>A (p.R4859K) mutation in RNF213. Moyamoya syndrome was diagnosed, and bilateral indirect revascularization surgery was conducted without complications and with a good postoperative course. In moyamoya syndrome associated with Williams syndrome, adequate perioperative management of both the moyamoya arteries and the cardiovascular abnormalities is important to prevent complications. Conclusion: This was the first report on a case in which moyamoya syndrome associated with Williams syndrome was confirmed by the presence of a heterozygous RNF213 mutation. Similar to the workup of moyamoya disease, confirmation of RNF213 mutation in Williams syndrome may be useful in predicting the development of moyamoya syndrome that can lead to severe complications.

Amniotic band syndrome (ABS) is a congenital abnormality that can cause a variety of deformities. Here, we report a case of ABS in which the amniotic band adhered to the skull, causing a partial cranial defect that was difficult to differentiate from an occipital encephalocele. The mother was a 24-year-old with an unremarkable medical and family history. Ultrasonography performed at 16 weeks showed that the fetus had a membranous structure in the occipital region. Occipital encephalocele was suspected, and she underwent cesarean section at 38 weeks. A male newborn was delivered, and his left occipital skin had a defect measuring 2 cm, from which a cystic structure had prolapsed. Cranial magnetic resonance imaging showed that the cystic structure had homogeneous high signal intensity on T2-weighted images. The neonate then underwent repair of the occipital encephalocele. During the operation, the membranous structures and dura were not continuous. Histological examination revealed that the membranous structures were composed of amnion, suggesting that this was a case of ABS. ABS may present with an encephalocele-like morphology without affecting the brain tissue and meninges. If an atypical encephalocele is found after birth, examination of the placenta may be helpful for a definitive diagnosis.

Unilateral lambdoid synostosis (ULS) is a rare suture anomaly and its diagnosis and treatment remain controversial. We investigated how to improve their function and morphology through the review of two cases of ULS with improved function and morphology by the same unreported operative procedure. Moreover, we discussed the diagnosis and treatment of ULS. Case 1 involved a 15-month-old female, brought for consultation because of suspected right ULS. She was diagnosed with right lambdoid and squamous synostoses and approximately 15 mm descent of the cerebellar tonsil. Thus, we performed foramen magnum decompression, first cervical vertebra laminectomy, and skull lengthening. Case 2 involved an 18-month-old female. She also consulted for suspected right ULS. Computed tomography revealed right ULS and squamous synostosis and magnetic resonance imaging showed approximately 9 mm descent of the cerebellar tonsil. We performed foramen magnum decompression and skull lengthening. Both procedures included decompression of the foramen magnum. We enforced osteotomy of the affected side of the lambdoid suture by crossing the superior sagittal sinus and added horizontal osteotomy from the superior right squamous suture to the coronal suture. Three extension devices were placed, two and one in the lambdoid and squamous sutures, respectively. After skull extension and descent of the cerebellar tonsil, exertion of the left frontal bone was improved. In cases of ULS, the squamous suture fusion and the drooping of the cerebellar tonsils can be complicated. Extending the lambdoid suture upward and downward and decompressing the posterior cranial fossa may improve the morphological and functional prognoses.

After untethering surgery of a tethered spinal cord of a tight filum terminale, patients are usually kept in the horizontal decubitus position to prevent cerebrospinal fluid (CSF) leakage. However, the optimal period for keeping these patients in this position has not been established yet. Surgical results in two groups of pediatric patients with a tight filum terminale were retrospectively analyzed. Group A was maintained in the horizontal decubitus position for 72 h and group B was managed without being kept in this position postoperatively. A total of 313 patients underwent sectioning of a tight filum terminale. Of these patients, 144 were maintained horizontally for 72 h postoperatively (group A) and 169 were managed without this position (group B). Among the patients who were maintained horizontally for 72 h, one (0.7%) developed pseudomeningocele. No patients experienced CSF leakage in this group. Among the patients who were not horizontal, one (0.6%) developed CSF leakage and one (0.6%) developed pseudomeningocele. Maintaining patients without restriction of their position does not appear to change the rate of postoperative CSF leakage or pseudomeningocele. This suggests that maintaining patients horizontally after transection of a tight filum terminale is not necessary for preventing CSF leakage.

Cohort study. Long-term urological outcomes in patients with spinal lipoma after prophylactic tethered cord release (TCR) in infancy were investigated. Children’s hospital in Yokohama, Japan. Children under one year of age with spinal lipoma who underwent TCR between 1990 and 2010 were investigated. According to Arai’s classification, lipomas other than filar lipoma were classified into four types: caudal, dorsal, transitional, and lipomyelomeningocele. The level of the conus medullaris was divided into three categories: L3-5, L5/S1, and sacral. Urological outcomes, including the need for clean intermittent catheterization (CIC), urinary incontinence, presence of renal deterioration, and the need for bladder augmentation, were investigated by both lipoma type and level of the conus medullaris. Fifty-three patients met the inclusion criteria. The median follow-up period was 14.2 years (interquartile range 9.6–17.6 years). Of the 53 patients, ten (19%) were on CIC, and six (11%) were incontinent. Overall, two patients (4%) had renal deterioration detected by DMSA renal scan, and two (4%) needed augmentation cystoplasty. Of the lipoma types, transitional type showed the worst outcomes with respect to need for CIC (54%) and urinary incontinence (38%). There were no significant differences in renal deterioration and the rate of bladder augmentation by lipoma type. No urological outcomes were significantly associated with conus level. Even after prophylactic TCR in infancy in children with spinal lipoma, 19% of patients needed CIC in long-term follow-up. Of the lipoma types, transitional type showed the worst outcomes with respect to need for CIC and urinary incontinence.

Pediatric schwannomas are rare, and most of them are associated with neurofibromatosis type 2 (NF2) and usually located in the vestibular nerve. Herein, we present the first pediatric case of intracranial schwannoma derived from the IX/X nerve complex unrelated to NF2. The patient was a 9-year-old boy who presented with a 3-month history of headache and nausea. There was no family history of NF2. Imaging studies revealed a cystic lesion with enhanced wall in the left cerebellomedullary fissure. During the operation, the IX/X nerve complex was strongly adhered to the tumor at the jugular foramen. The tumor was totally excised, and the postoperative MRI demonstrated no residual tumor. Histopathological diagnosis was schwannoma. Genetic analysis revealed no mutation associated with NF2 and schwannomatosis. We reported the first case of pediatric lower cranial nerve schwannoma which was not associated with NF2. The schwannoma should be included as differential diagnosis of pediatric posterior fossa tumors.

BACKGROUND In intracranial ependymoma, the effectiveness of chemotherapy and radiation therapy is unclear, and the degree of tumor removal contributes to the improvement of life prognosis. METHODS We examined ependymoma cases treated in our institution from July 1998 to March 2017. RESULTS There were 18 boys and 7 girls. The average age at the time of surgery is 5.3±3.6 years. The pathological diagnosis was Grade II for 8 cases and Grade III for 17 cases. Genetic analysis was performed in 16/25 cases (64%). Of the infratentorial cases, 10/11 cases (90.1%) were PFA and PFB were one case. Of the supratentorial cases, 3/5 cases (60%) were positive for RELA fusion. As chemotherapy, 19 patients were VCR + VP-16 + CDDP + CPA. Irradiation was performed in all cases, local irradiation (50.4–55.8Gy) in 22 cases (88%), and craniospinal irradiation in 2 cases (8%). The 7-year OS was 74.6±9% and the 7-year PFS was 59.7±10.5%. Grade III showed a short OS (p = 0.053). GTR and NTR were obtained in the first excision in 14 cases (56%), and OS and PFS were not significantly different from those in the STR group (p = 0.219, p = 0.248). GTR and NTR including 2nd-look surgery were obtained in 18 cases (72%), and significant improvement of OS was observed compared with STR group (p = 0.02). CONCLUSION Even if it is not GTR or NTR at the first operation, improvement of OS is expected by total excision after chemotherapy.

The purpose of this paper is to provide novel insight into the rare pediatric meningiomas. We retrospectively analyzed pediatric surgical cases of meningioma during 2002 to 2017 in our institution. The preoperative, intraoperative, and the postoperative status were collected to find any unique features that has not reported in the past. Nine surgeries out of 5 patients were identified. The mean age was 7 years old (range 1–14 years old). Four patients were females. The mean tumor diameter was 52 mm (range 23–81 mm). The tumor locations were optic nerve sheath, Sylvian fissure, parasagittal, trigone of the lateral ventricle, and cerebellopontine angle. The Sylvian fissure meningioma without dural attachment (MWODA) was found in a 15-month-old female. A relapsed parasagittal meningioma showed regression in histological grade and residual tumor demonstrated spontaneous regression. In the initial surgeries, Simpson grade 1 resection was achieved in 2 cases. The pathological diagnoses were 1 meningothelial, 1 metaplastic, 2 atypical, and 1 clear cell meningiomas. The mean postoperative follow-up period was 71 months. Three patients experienced recurrence of the tumor. At the latest follow-up, all patients were free of radiological tumor recurrence or regrowth with a mean follow-up of 4 years (range 1–6.9 years). All patients were in the modified Rankin scale of 0–1. MWODA is not considered to be rare in pediatric meningioma and should be included in the differential diagnosis. We presented a histologically regressed relapsed meningioma, which spontaneously regressed after subtotal resection. In the case of recurrent meningioma, surgical resection and adjuvant radiation therapy could be effective for long-term control of the tumor.

OBJECTIVEThe goal of this study was to clarify the long-term outcome of endoscopic third ventriculostomy (ETV) in pediatric hydrocephalus in light of the ETV Success Score (ETVSS), shunt dependency, and intellectual development.METHODSThe authors retrospectively analyzed pediatric patients with hydrocephalus who underwent ETV between 2002 and 2012 and who were followed for longer than 5 years as a single-center cohort. The data of the patients’ pre- and postoperative status were collected. The relationships between ETVSS and the full-scale IQ as well as shunt dependency were analyzed. The usefulness of ETVSS for repeat ETV and the change of radiological parameters of ventricle size before and after ETV were also analyzed. The success of ETV was defined as no requirement for further CSF diversion procedures.RESULTSFifty ETVs were performed in 40 patients. The average ETVSS was 61 and the success rate at 6 months was 64%. The mean follow-up was 9.9 years (5.2–15.3 years), and the long-term success rate of ETV was 50%. The Kaplan-Meier survival curve continued to show a statistically significant difference among patients with a low, moderate, and high ETVSS, even after 6 months (p = 0.002). After 15 months from the initial ETV, no patients required additional CSF diversion surgery. There was no statistical significance between ETVSS and the long-term full-scale IQ or shunt dependency (p = 0.34 and 0.12, respectively). The radiological improvement in ventricle size was not associated with better future educational outcome.CONCLUSIONSThe ETVSS was correlated with the long-term success rate. After 15 months from the initial ETV, no patients required an additional CSF diversion procedure. The ETVSS was not considered to be correlated with long-term intellectual status.

BACKGROUND In intracranial ependymoma, the effectiveness of chemotherapy and radiation therapy is unclear, and the degree of tumor removal contributes to the improvement of life prognosis. Methods: We examined ependymoma cases treated in our institution from July 1998 to March 2017. RESULTS There were 18 boys and 7 girls. The average age at the time of surgery is 5.3 ± 3.6 years. The pathological diagnosis was Grade II for 8 cases and Grade III for 17 cases. Genetic analysis was performed in 16/25 cases (64%). Of the infratentorial cases, 10/11 cases (90.1%) were PFA and PFB were one case. Of the supratentorial cases, 3/5 cases (60%) were positive for RELA fusion. As chemotherapy, 19 patients were VCR + VP-16 + CDDP + CPA. Irradiation was performed in all cases, local irradiation (50.4–55.8Gy) in 22 cases (88%), and craniospinal irradiation in 2 cases (8%). The 7-year OS was 74.6 ± 9% and the 7-year PFS was 59.7 ± 10.5%. Grade III showed a short OS (p = 0.053). GTR and NTR were obtained in the first excision in 14 cases (56%), and OS and PFS were not significantly different from those in the STR group (p = 0.219, p = 0.248). GTR and NTR including 2nd-look surgery were obtained in 18 cases (72%), and significant improvement of OS was observed compared with STR group (p = 0.02). In patients with hydrocephalus preoperatively, OS tended to be short (p = 0.057), especially in cases requiring VP shunt placement, OS was significantly shortened (p = 0.017). CONCLUSION Even if it is not GTR or NTR at the first operation, improvement of OS is expected by total excision after chemotherapy. The importance of chemotherapy was suggested to be suppression of tumor growth until reoperation and reduction of blood loss during surgery.