Background: Intracranial hemorrhage is rare in children with moyamoya disease. While the Japan Adult Moyamoya (JAM) Trial revealed the preventive effect of direct bypass against rebleeding in hemorrhagic moyamoya disease in adults, direct bypass surgery for children with hemorrhagic moyamoya disease has rarely been addressed.

Methods: Consecutive pediatric patients below 18 years with moyamoya disease was reviewed in our institution. All patients requiring surgery underwent direct bypass and cerebral angiography before and 3 months after surgery. Fragile collateral vessels were assessed with angiography and classified into 3 types (lenticulostriate, thalamic, and choroidal) according to the criteria of the JAM Trial.

Results: Of a total of 104 pediatric patients with moyamoya disease, 5 (4.8%; mean age, 11.2 years) were identified as hemorrhagic disease. Four patients suffered intraventricular hemorrhage at the atrium or the posterior half of the body of the lateral ventricle. Choroidal anastomoses were commonly observed in 9 of 10 hemispheres. Direct bypasses were patent in all 9 hemispheres undergoing surgery. Among collateral subtypes, choroidal anastomosis was most likely to shrink after surgery (87.5% of the positive hemisphere). No rebleeding has occurred as of this writing.

Conclusions: Choroidal anastomosis is a possible characteristic of hemorrhagic moyamoya disease in children. Direct bypass might be effective for not only adult but also pediatric patients with hemorrhagic moyamoya disease in light of its effect at correcting choroidal anastomosis.

Objectives: To describe the clinical and radiographic manifestations of spinal cerebral spinal fluid (CSF) leak in children.

Subjects and methods: This study included patients 15 years of age or younger diagnosed as spinal CSF leak, between December 2015 and April 2019, at Fukuyama Medical Center. We evaluated 38 children (18 boys and 20 girls) with spinal CSF leak (age at onset of symptoms: 10-15 years, mean 13.4 years).

Results: All patients presented with headaches with postural nature, but only one of them presented with a typical orthostatic headache. Unlike adult patients, lightheadedness upon standing was a common symptom, found in 27 patients (71.1%). Among these patients, 18 had been diagnosed as orthostatic dysregulation (OD), and 2 as postural tachycardia syndrome (POTS) before their visit to Fukuyama Medical Center. Sleep arousal disorder was also a common symptom, found in 13 patients (34.2%). Spinal fat-saturated T₂-weighted magnetic resonance imaging (MRI) showed abnormal epidural high intensity in all patients. Fringed epidural space sign (FESS) at the thoracic spine was the universal findings. Three patients were cured with bed rest and/or continuous epidural fluid infusion. Thirty-five patients underwent epidural blood patch (EBP) treatment (1-6 times, mean 2.5) Overall, outcome was excellent in 17 patients, good in 14, fair in 6, and poor in 1. Mild to moderate pain around the injection site for several days was common, but no significant complications were observed.

Conclusions: Spinal CSF leak in childhood may not be so rare as previously considered. Lightheadedness upon standing (including OD, and POTS), and sleep arousal disorder seems to be characteristic to pediatric patients with spinal CSF leak. Most patients can be treated effectively and safely with EBP treatments.

Background: Posterior fossa tumors are frequently associated with non-communicable hydrocephalus in children, but there are no specific guidelines for managing hydrocephalus in these patients.

Objectives: To investigate the implementation rate of postoperative permanent CSF diversion (VP shunt [VPS], endoscopic third ventriculostomy [ETV]) and its predictive factors.

Methods: We retrospectively analyzed 14 patients who underwent posterior fossa tumor resection at our hospital from April 2013 to March 2018. Baseline characteristics, clinical course, imaging data, and tumor pathology were analyzed. Primary outcome was permanent CSF diversion at 6 months after initial surgery.

Results: Management of Hydrocephalus at the time of the first surgery was 8 cases of external ventricular drainage [EVD], 5 cases of excision alone, and 1 case of ETV and EVD. Two patients (14.2%) required permanent CSF diversion 6 months after surgery. Comparison of the two groups by univariate analysis (with permanent CSF diversion vs. without), preoperative diagnosis (medulloblastoma) 100% vs 25% (p = 0.04), less than 2 years old 50.0% vs 0% (p = 0.01), dissemination 50.0% vs 0% (p = 0.01), excision rate (partial removal) 50.0% vs 0% (p = 0.01) were significantly different.

Discussion: In this study, only 14.2% of cases required persistent CSF diversion, which were similar to previous reports. There are some reports that recommend pre-resectional ETV, however, in most cases, many of them would be underwent unnecessary procedures for hydrocephalus that can be resolved only with excision with or without temporary EVD.

Conclusion: Permanent CSF diversion (VPS or ETV) was not essential at the time of initial surgery for posterior fossa tumors in children, and it could be managed by resection with or without EVD.

Background: Central nervous system (CNS) germinomas have favorable cure rates. However, long-term follow-up data are limited because of the rarity of these tumors. A recent report based on the SEER database investigated CNS-germ cell tumors (GCT) and showed that the 5-year survival outcomes of patients who developed germinomas were worse compared to that for patients with nongerminomatous germ cell tumors (NGGCT). In this study, we evaluated the long-term follow-up outcomes of germinoma and NGGCT survivors.

Materials and Methods: Our institutional review board approved this retrospective study. Between 1985 and 2018, 66 patients with germinomas (male: female = 55: 11, ages: 9-39, mean: 18, median: 15) were treated at Hiroshima University Hospital. In the same period, 20 patients with NGGCTs (male: female = 20: 0, ages: 5-51, mean: 18, median: 11) were treated in our institution. Long-term follow-up data of all patients was obtained from the medical records.

Results: The long-term follow-up on patients with germinomas was performed up to 28 years (median: 12 years) with a 10-year survival rate of 96% and a 20-year survival rate of 87%. The follow-up data for patients with NGGCT was up to 25 years (median: 6 years) with a 10-year survival rate of 85% and a 20-year survival rate of 85%. Factors that affected the course of treatment included progressive disease and associated teratoma syndrome, which resulted in decreased treatment intensity. The factor affecting the long-term outcome and quality of life in patients with GCTs was the site of tumor development. Those with multiple lesions/dissemination at the onset and neurohypophyseal location tended to have poor prognoses, and those with basal ganglia involvement tended to have a poor quality of life owing to cognitive impairment. Overall, four patients had secondary cancers and six patients experienced recurrence following germinoma treatment; one patient died from an adrenal crisis.

Conclusion: Long-term prognosis and the quality of life of patients are affected by tumor site. It is important to select an appropriate treatment intensity based on an accurate diagnosis. Multidisciplinary treatment that includes appropriate hormone replacement, obesity prevention and treatment for lifestyle-related diseases should be considered for patients with germinomas and NGGCT survivors.

Background: This study investigates the relationship between final height and treatment interventions, such as replacement therapy for growth hormone deficiency (GHD), in pediatric brain tumor survivors.

Materials and Methods: This retrospective study was approved by the institutional review board of our institute. Among the patients admitted between 1989 and 2018, we enrolled 36 with germinoma, 12 with nongerminomatous germ cell tumors (NGGCTs), and 32 with embryonal tumors; other inclusion criteria were patient age at onset < 18 years at the onset and the patients were alive and event-free for 10 years or alive without recurrence. We evaluated craniospinal irradiation (CSI), presence of pituitary lesions, the presence or absence of GHD and growth hormone (GH) replacement therapy, and the standard deviation score (SDS) of patient heights.

Results: In malignant brain tumor survivors, the final hieght SDS of female patients was significantly lower than that of male patients (P < 0.05). Additionally, younger age at onset was a risk factor for lower height SDS (P < 0.01). In total, 68% of patients with neurohypophyseal germinomas were required to undergo GH replacement therapy. The median height SDS of survivors with neurohypophyseal involvement increased from -2.293 to -0.27 after GH replacement therapy and that of survivors without neurohypophyseal involvement showed similar improvements. The median height SDS of survivors who underwent high-dose chemotherapy with peripheral blood stem cell transplantation rescue was significantly lower than that of those who did not undergo such treatment (-2.59 vs. -0.46, P ＜ 0.05, respectively). GH replacement therapy was effective in treating the short stature of malignant brain tumor survivors without CSI, as their height SDS improved from -2.069 to 0.4889 (P ＜ 0.05). However, the height SDS of survivors with CSI worsened from -1.269 to -2.608.

Conclusions: CSI, female gender, onset at a younger age, and high-dose chemotherapy with bone marrow transplantation rescue were risk factors of short stature in malignant brain tumor survivors. Early and appropriate GH replacement therapy should be considered for those patients.

In cases of recurrent ependymoma with dissemination, the efficacy of adjuvant therapies is still being investigated.

In this study, we present a case of a 6-year-old Japanese girl with ependymoma in the posterior fossa. The 45-mm tumor is located mainly at the fourth ventricle and extended to the right foramen of Luschka, the jugular foramen, and the space between the basilar artery and clivus, which induced obstructive hydrocephalus. Because the patient underwent a three-stage surgery for 3 months, a gross total resection was almost achieved. During the third operation, the pathological diagnosis was anaplastic ependymoma. Therefore, she underwent fractionated radiation therapy of 54 Gy for the posterior fossa after 4 months since the initial surgery. No disseminated regions were observed at that time. A magnetic resonance imaging is performed every 3 months to show the progress of the recurrent tumor in a span of 21 months and the dissemination in the cerebrospinal cavity at 35 months since the first operation. Overall, she underwent 14 intracranial tumor removal procedures and 11 stereotactic radiosurgeries for all the 34 targets.

The recurrent but irradiated tumors had been under controlled for about 1 year. She maintained a good quality of life, with a Karnofsky Performance Status (KPS) score of 90, and had no major neurological deficits. In the course of another year, the tumor recurred, and she died six and half years later from the onset. Moreover, the combination of surgery and radiosurgery can achieve local control of anaplastic ependymoma even in a disseminated case for about 1 year keeping a good KPS score. Palliative therapy as multiple surgery or radiation therapy for recurrent tumor might also increase the patient's burden. Furthermore, stereotactic radiotherapy will be the preferred treatment option to decrease the frequency of surgery, maintaining a good quality of life.

Craniopharyngiomas are histopathologically benign tumors that arise from Rathke's pouch. They can recur after treatment and cause various complications such as hypopituitarism, cognitive dysfunction, and visual disturbance. Total removal is the gold standard treatment, but it is difficult to remove totally due to the proximity of the tumor to the optic nerves, chiasm, pituitary stalk, hypothalamus, and internal carotid artery. There are two peak age ranges of incidence, in children aged < 15 years and in adults aged 45-60 years. Craniopharyngiomas are rare in newborns and infants, and the prognosis is generally unfavorable. Patients with childhood-onset craniopharyngiomas often endure multiple complications and limited activity of daily life for a long time. Herein, we report a case of infantile craniopharyngiomas with intractable postoperative complications and review the literature.

A 14-month-old boy exhibiting lethargy was referred to our hospital. His anterior fontanel was swollen, and his head circumference was enlarged. Head computed tomography depicted an approximately 7.5-cm tumor with calcification and multiple cysts, which had reached the third ventricle and caused obstructive hydrocephalus. Head magnetic resonance imaging depicted a solid tumor with isointense signal in T1 and T2-weighted images, surrounded by multiple cysts.

After ventricular drainage, the patient underwent total resection of the tumor via an interhemispheric approach. The optic nerves and optic tracts were both preserved, but the pituitary stalk-which was the origin of tumor-was resected. The pathology results indicated adamantinomas craniopharyngioma. After surgery, hormone replacement therapy for hypopituitarism and diabetes insipidus were required. On postoperative day 19, the patient underwent a subdural peritoneal shunt due to subgaleal and subdural effusion, and shunt infection with methicillin-resistant Staphylococcus aureus (MRSA) developed. Repeated subdural drainages and irrigations were performed, and the original shunt was ultimately replaced with an antibiotic-impregnated shunt system.

The patient still has subdural hygroma, hypopituitarism requiring hormone replacement therapy, and severe developmental retardation. These complications are potentially fatal, so follow-up by a multidisciplinary team including a neurosurgeon, pediatrician, and endocrinologist will be required throughout his life.

In cases of traumatic skull fracture with laceration of the dura mater, complete closure and cosmetic cranioplasty are necessary to prevent delayed cerebrospinal fluid (CSF) leaks and infection. This is the case of a 6-year-old boy who was hospitalized after a motor vehicle accident. Brain computed tomography (CT) showed traumatic intracranial hemorrhage and multiple skull fractures. He was referred to our hospital for surgical intervention. Bone fragments were removed from the right frontal lobe and the orbit. Complete closure of the lacerated dura mater was performed using transplants from the pericranium, fibrin glue, and absorbable polyglycolic acid sheets. Furthermore, cosmetic cranioplasty was performed using hydroxyapatite ceramic implants. His postoperative prognosis was favorable without surgical complications including delayed CSF leaks or infection. This case was presented to highlight the importance of complete closure of the lacerated dura mater and to demonstrate the efficacy of hydroxyapatite ceramic implants during cosmetic cranioplasty among pediatric patients.