Survey of medications for myelomeningocele patients over their lifetime in Japan.

BACKGROUND: This study aimed to investigate medication prescriptions for patients with myelomeningocele (MMC) across different age groups, particularly in adulthood and after middle age. METHODS: The Japan Medical Data Center (JMDC) database, based on medical claims data, was utilized for this analysis. Patients were divided into 10-year age groups, and prescriptions for analgesics, anticonvulsants, psychotropic drugs, lifestyle disease-related drugs, drugs for urinary incontinence, and laxatives were examined. To compare the differences in the utilization of medications unrelated to lifestyle-related diseases across different age groups, the data was categorized into three age groups: 19 or under, 20-39, and 40 or older. RESULTS: Among the 556 MMC patients, the percentage of those regularly prescribed analgesics increased from 2.8% in patients ≤ 19 to 31.7% in patients 40 or older (p < 0.01). Psychotropic medication use also increased with age, rising significantly from 6.3% in patients ≤ 19 to 34.6% in patients 40 or older (p < 0.01). Patients with MMC showed an increasing trend in prescriptions for lifestyle-related disease medications compared to the normal control group. Notably, the percentage of patients in their 30 s taking hypertension medication was 4.9%, significantly higher than the 0.86% in the control group (p = 0.029). In their 40 s, 22.9% of MMC patients were prescribed hyperlipidemia medication, significantly higher than the 3.9% in the control group (p < 0.01). CONCLUSION: Comprehensive multidisciplinary support and follow-up are crucial to enhance the quality of life for MMC patients, with particular attention to pain management, psychological care, and treatment of lifestyle-related diseases.

Current status and challenges of neurosurgical procedures for patients with myelomeningocele in real-world Japan.

BACKGROUND: Little is known about the real-world status of neurosurgical treatment of myelomeningocele patients. OBJECTIVE: To investigate the real-world status of neurosurgical treatment of myelomeningocele patients, medical claims data provided by the Japan Medical Data Center (JMDC) were analyzed. METHODS: The health claims data of 556 patients with myelomeningoceles from January 2005 to March 2020 were examined. The number of neurosurgical procedures, including myelomeningocele repair, tethered cord release, cerebrospinal fluid (CSF) shunt, CSF drainage, and endoscopic third ventriculostomy (ETV), was determined. RESULTS: A total of 313 neurosurgical procedures were performed for 135 patients in 74 institutions during the study period. The shunt survival rate was most affected by shunts that were revised when the patient was less than 1 year old, which had a significantly lower survival rate than all of the initial shunts performed when the patient was less than on1 year old; the 1-year shunt survival rate was 35 vs 64% (P = 0.0102). The survival rate was significantly lower in patients younger than 1 year who had CSF drainage before shunting compared to those younger than 1 year who did not have CSF drainage before shunting; the 1-year shunt survival rate was 27 vs 59% (P = 0.0196), and 81% of patients remained free of tethered cord release 10 years later. CONCLUSIONS: In this study, a revised shunt of less than 1 year of age and CSF drainage before shunting were the factors that lowered the shunt survival rate in the real world for CSF shunts for hydrocephalus associated with myelomeningocele.

Number of surgeries performed during the lifetime of patients with myelomeningocele.

OBJECTIVE: Patients with myelomeningocele often require multiple surgeries, but no study has clarified the kind of treatment given to these patients throughout their lives. The authors analyzed the type of surgery that was performed and at what age for Japanese patients with myelomeningoceles. METHODS: The Japanese health claims data of 556 patients with myelomeningocele for the period from January 2005 to March 2020 provided by the Japan Medical Data Center Co., Ltd., were examined to investigate the number of surgeries performed and the patient age at surgery for each specialty. The patients were divided into two groups (those ≤ 18 years old [group A] and those > 18 years old [group B]), and the way in which the types of surgery and the percentage of surgeries changed between these two groups was examined. RESULTS: The mean follow-up period was 4.4 years. The mean age at the end of the overall follow-up was 18.6 years (range 0-70.5 years), and 1033 surgeries were performed on 294 patients (0.42 surgeries performed per patient per year) during this period. The number of surgeries for patients in group A was 818 in 192 patients, with 0.62 surgeries per patient per year, and for patients in group B it was 215 in 102 patients, with 0.19 surgeries per patient per year. The number of surgeries and the mean age at the time of surgery were as follows: 313 neurosurgeries, 5.16 years; 280 orthopedic surgeries, 11.36 years; 70 urological surgeries, 14.57 years; and 202 dermatological/plastic surgeries, 16.19 years. In the surgeries related to myelomeningocele, the rates of CSF shunt placement, tethered cord release, muscle and tendon surgery, and other bone and joint surgery decreased significantly in group B, but they continued to undergo these surgeries. In group B, the rates of skin surgery, nephrostomy, ureterostomy, and cystostomy were significantly higher. CONCLUSIONS: A significant number of surgeries in multiple specialties related to myelomeningocele continue to be performed in adulthood, indicating that these patients require continuous care throughout their lives.

GFAP variant p. Tyr366Cys demonstrated widespread brain cavitation in neonatal Alexander disease.

Alexander disease is a rare form of leukodystrophy caused by heterozygous mutations in the gene encoding glial fibrillary acidic protein (GFAP). Brain cavitation in the white matter, predominantly distributed in the frontal periventricular area, has been described in some cases. Here, we present a case of a 1-year-old boy with neonatal Alexander disease caused by the p. Tyr366Cys GFAP variant, with rapid and widespread white matter cavitation. This case broadens the radiological spectrum of Alexander disease and suggests a possible genotype-phenotype correlation between the p. Tyr366Cys variant and cavitation.

Atypical clinical features of children with central nervous system tumor: Delayed diagnosis and switch in handedness.

Herein is described the cases of three children with central nervous system (CNS) tumor, who had switch in handedness occurring before diagnostic confirmation. Although the onset, age, tumor location, and histology were heterogeneous, the diagnosis of CNS tumor was delayed in all three patients. The present experience indicates that switch in handedness should be recognized as a sign of CNS tumor in pediatric patients, and which might prevent delay in diagnosis. Pediatricians should carefully examine such patients who present with some suggestive symptoms of CNS tumor, even when they are unusual, in order to make a timely and appropriate diagnosis.

Hybrid Operating Room for Combined Neuroendovascular and Endoscopic Treatment of Ruptured Cerebral Aneurysms with Intraventricular Hemorrhage.

BACKGROUND: Intraventricular hemorrhages (IVHs) caused by ruptured cerebral aneurysms often have poor outcomes. Treatment challenges include comorbidities, increased intracranial pressure caused by IVH, and risk of rebleeding. CASE DESCRIPTION: Two cases of severe IVH accompanied by acute hydrocephalus caused by ruptured aneurysm were treated with coil embolization followed by endoscopic hematoma evacuation as a single treatment session in a hybrid operating room (OR) equipped with a multipurpose angio biplane system. The first case was an 84-year-old woman with a ruptured basilar top aneurysm, who presented with Hunt and Hess (H&H) grade 5 subarachnoid hemorrhage (SAH) with packed IVH. The second case was a 43-year-old man with a ruptured anterior communicating artery aneurysm who presented with H&H grade 5 SAH with packed IVH. In both cases, endovascular coil embolization was performed first to prevent intraoperative bleeding. The coiled aneurysms suddenly appeared on the screen of the endoscope during the hematoma removal, which could have led to massive rebleeding if not treated previously. Neither patient needed a reinsertion of the ventricular drainage or developed chronic hydrocephalus during hospitalization. The hybrid OR enabled the 2 treatment approaches to be performed without the need to transfer the patient, thereby minimizing the transition time between the modalities. Intraoperative cone-beam computed tomography contributed to the evaluation of residual clots. CONCLUSIONS: A hybrid OR may contribute to a combined neuroendoscopic and endovascular treatment for ruptured cerebral aneurysms with severe intraventricular hemorrhage.

Parallel insertion endoscopic technique for precise catheter placement in cystic craniopharyngiomas.

BACKGROUND: Total removal of craniopharyngioma is the most acceptable therapeutic modality; however, there are cases in which radical excision is not possible. To reduce the cystic component volume, an Ommaya reservoir catheter can be placed endoscopically. However, there are certain complications and risks with this type of maneuver, such as misplacement of the catheter, which may result in leakage of cyst contents or installed fluids such as bleomycin. Thus, accurate placement of intracystic catheter is extremely important. PATIENTS AND METHODS: The authors placed Ommaya reservoir catheters running over the outer surface of a transparent endoscopic sheath in three cases. RESULTS: This neuroendoscopic procedure permits easier manipulation of the catheter and precise placement of the catheter tip. This technique was useful for placement of Ommaya reservoir catheters. CONCLUSIONS: This new technique of catheter placement with neuroendoscopy is more accurate, safer, and less invasive.

Long-term survival following gross total resection of pediatric supratentorial ependymomas without adjuvant therapy.

Pediatric supratentorial ependymoma is very rare. In pediatric patients with supratentorial ependymoma, surgery alone may be an acceptable treatment when postoperative imaging confirms a gross total resection. Surgical resection is the standard and the most important treatment for ependymoma. The role of radiation therapy and/or chemotherapy following a gross total resection of supratentorial ependymoma has been uncertain. We report 2 cases of pediatric supratentorial ependymomas treated by gross total resection without postoperative adjuvant therapy. The first patient was a 7-year-old girl who presented with motor weakness and a hemiconvulsion of the right leg. Magnetic resonance imaging (MRI) revealed a large heterogeneously enhanced tumor in the left frontal lobe. The second patient was an 8-year-old girl who presented with headache. MRI revealed a huge heterogeneously enhanced tumor in the left frontal lobe. Gross total resection was achieved in both patients. Postoperative radiotherapy and chemotherapy were avoided following gross total resection. Histologically, the lesions demonstrated grade II ependymoma and anaplastic ependymoma, respectively. After follow-up of 120 months, neither patient had recurrence or dissemination. These results suggest that patients with pediatric supratentorial ependymoma treated by gross total resection alone have a favorable outcome, and postoperative radiotherapy and chemotherapy may be avoided.

Aggressive large cell medulloblastoma extending to the extracranial region in brain-dead state.

INTRODUCTION: The authors describe the case of a 29-month-old boy who presented with acute non-communicating hydrocephalus caused by a small tumor in the fourth ventricle. He became brain-dead immediately and remained stable in that condition. MATERIALS AND METHODS: Six months later, despite being in a brain-dead state, a rapid direct tumor extension from the intracranial to extracranial region was observed, and chemoradiotherapy was performed following tumor biopsy. The histopathological diagnosis was large cell medulloblastoma. Although treatment was initially effective, the tumor again aggressively invaded the cervical muscles via the spinal canal. Comparative genomic hybridization (metaphase) analysis revealed a pattern of aberrations predictive of a poor prognosis (+1q, ?17p, +17q, and probable amplification of c-myc gene), and he eventually died 11 months after onset. RESULTS: Direct invasion of medulloblastoma from the intracranial to extracranial region is extremely rare, and, to our knowledge, this is the first report of medulloblastoma exhibiting rapid extension to the extracranial region in brain-dead state. CONCLUSIONS: For patients with medulloblastomas, careful observation is needed even in brain-dead state. The etiology of this rare condition as well as the genetic characteristics responsible for aggressive tumor behavior are discussed.

[Clinical prognosis and therapeutic aspects in management of pediatric moyamoya disease].

We reviewed the clinical prognosis and therapeutic aspects in the management of pediatric moyamoya disease from our data base for the last 8 years since establishment of our institution. We found 99 cerebrovascular disease cases among the total of 1,159 cases, and 20 out of them were moyamoya disease. Only 13 cases were analyzed as the rest were not available for follow up. Nine girls and 4 boys were admitted with an average age of 5 years-old 4 months. Initial symptom were TIA in 5, cerebral infarction in 5, migraine in 2, and epilepsy in one. The type of moyamoya disease was bilateral type in 10 cases, and unilateral type in 3 cases. Clinical condition and investigations, as well as the effect of conservative management, required surgery in 9 cases (8 indirect revascularization and one direct-bypass surgery), and the rest 4 cases were managed conservatively. Only 10 cases were followed-up, 9 operated cases and one conservatively managed case. In 6 of 9 of the operated cases, there was recurrence: cerebral infarction in 3, TIA recurrence in 2 and epileptic seizure in one. On MRA a steno-occlusive lesion of PCA was found in 4 of the 6 recurrence patients, and improvement was achieved by additional surgery in one of them. All of the cases developed good postoperative collateral circulation. The conservatively treated patient did not show neither steno-occlusive lesion of PCA, nor a clinical recurrence for 7 years observation. Therefore, posterior circulation system in moyamoya disease is important as a collateral circulation in addition to the revascularization procedures, and we believe that the progression of the steno-occlusive lesion of PCA is an important indicator, predicting recurrence.

Ventricular anatomy of hydrocephalus associated with myeloschisis and endoscopic third ventriculostomy.

OBJECTS: To characterize the anatomical features of the ventricular regions in patients with myeloschisis and related to successful performance of endoscopic third ventriculostomy (ETV). METHODS: Radiological and endoscopic findings of 21 myeloschisis patients were retrospectively reviewed. Radiological features that could interfere with endoscopic procedures were (1) a huge massa intermedia (12/19), (2) sloping of the third ventricular floor (3/10), (3) narrow anteroposterior length of the third ventricular floor (2/10), and (4) narrow prepontine cistern (8/21). Endoscopic findings were (a) a narrow foramen of Monro (0/3), (b) hypertrophy of the anterior commissure (1/3), (c) sloping of the third ventricle floor (1/3), (d) a huge massa intermedia (3/3), and (e) opaque third ventricular floor (3/3). These endoscopic findings did not interfere with endoscopic procedures by using the Oi-HandyPro neuroendoscope without the above-mentioned radiological features 3 or 4. CONCLUSION: Narrow anteroposterior length of the third ventricular floor and narrow prepontine cistern are not infrequently observed. Preoperative evaluation and intraoperative inspection of these findings are very important in successful performance of ETV.

Traumatic subacute subdural effusion in the posterior fossa associated with secondary acute hydrocephalus in a neonate. Case report.

The authors report on a neonatal patient with traumatic subacute subdural effusion in the posterior fossa associated with secondary acute hydrocephalus. The infant fell from his mother's hand onto the floor, injuring his left parietal region. Computed tomography (CT) scans of the patient's head revealed a linear fracture of the left parietal bone, a small contusion in the right temporal lobe, and a small subdural hematoma in the right posterior fossa with thin subdural effusion. Serial CT scans revealed a progressive increase in subdural effusion bilaterally in the posterior fossa. On Day 7 the anterior fontanelle was tense and CT scans revealed marked hydrocephalus associated with thick subdural effusion in the posterior fossa. External drainage of both the subdural effusion and dilated lateral ventricles improved the patient's condition, and no reaccumulation of subdural effusion has been observed. The origin and treatment of this rare clinical entity is discussed.

Huge arachnoid cyst incorporating choroid plexus.

BACKGROUND: We report a rare case of arachnoid cyst incorporating choroid plexus. This 7-month-old girl presented with macrocrania. Magnetic resonance (MR) imaging disclosed a cystic lesion arising from the left prepontine cistern extending to the left middle cranial fossa. METHODS: First, we performed resection of the membrane microscopically and obtained an adequate reduction of the cystic size. However, 4 months after the first operation, the cyst was enlarged again, and bulging of the portion of the craniotomy was noted. Thus, we performed neuroendoscopic transcortical ventriculo-cyst-cysternostomy and confirmed the choroid plexus inside of the arachnoid cyst. DISCUSSION: Postoperative course was uneventful. This is the first reported case of choroid plexus within an arachnoid cyst, determined neuroendoscopically.

Neuronavigational neuroendoscopic surgery. Frameless free-hand maneuvering of a handy rigid-rod neuroendoscope on visualized three-dimensional computerized image guidance: trajectory to the prepontine cistern in cadaver study.

BACKGROUND: Considering the separate benefits of neuronavigation and neuroendoscopy, neuroendoscopic surgery with the aid of neuronavigation systems will play an increasingly important role in the future. Bearing this in mind, the present research project was conducted to facilitate neuronavigational neuroendoscopic surgery along the pathway to the prepontine cistern using cadaver heads. MATERIALS AND METHODS: A computer-aided, frameless image-guided stereotactic navigation system and a new type of handy rigid-rod neuroendoscope were used. The ideal entry point and the safest trajectory to the prepontine cistern through the foramen of Monro were defined in two formalin-fixed cadaver heads and clinical brain MRI data. Then, maneuvering of the neuroendoscope with the aid of the neuronavigation system was performed. RESULTS: Straight trajectories from the entry point to the prepontine cistern could be designed. For the registration accuracy of the tip of the neuroendoscope, the virtual image registered a mean error distance of 5.42 mm away from the reference point along the axis of vertical line. However, free-hand maneuvering enabled the neuroendoscope to be finely manipulated without damaging brain tissues. Neuroendoscopic anatomical views of the interpeduncular and prepontine cistern were also acquired. CONCLUSION: Interactive use of free-hand maneuvering of the handy rigid-rod neuroendoscope together with frameless neuronavigation systems plot the way to true neuronavigational neuroendoscopic surgery in a safe and reliable manner. This pairing of the most recent technological neurosurgical options with better understanding of neuroendoscopic anatomy enables the neurosurgeon to acquire broader treatment options for central nervous system diseases.

Endoscopic biopsy interpretation difficulties in a congenital diffuse intracranial teratoma.

INTRODUCTION: Congenital brain tumours are a rare entity that is nowadays often already recognised during pregnancy by ultrasound (US) and magnetic resonance (MR). Even though the definitive diagnosis is usually achieved by means of histological studies, in some cases the diagnosis may remain uncertain because of the malformative origin of this type of tumour. CASE REPORT: We describe a patient with a diffuse congenital intracranial mass diagnosed by intrauterine US and foetal MR performed to further evaluate the lesion and the associated foetal hydrocephalus. After delivery by caesarean section, an endoscopic biopsy was performed. Several specimens were obtained and resulted in the diagnosis of primitive neuroectodermal tumour (PNET). Despite polychemotherapy, the tumour continued to enlarge and the patient died at 2 months of age. Post-mortem histological examination of the intracranial mass showed a diffuse intracranial teratoma. CONCLUSION: Endoscopic biopsy specimen examination resulted in a diagnosis that was not confirmed by post-mortem findings. The scarce differentiation of teratoma components and their high variability in histomorphology as well as the huge size of the tumoral mass may limit the interpretation of endoscopic biopsy specimens, even when multiple and obtained from different areas.

Biomechanical and phenotypic changes in the vasospastic canine basilar artery after subarachnoid hemorrhage.

Because it has been argued that active myogenic tone prolongs cerebral vasospasm for >2 wk after subarachnoid hemorrhage (SAH), we attempted to identify the mechanism that plays the main role in sustaining the prolonged cerebral vasospasm. We especially focused on the roles of biomechanical and phenotypic changes in the cerebral arteries in the mechanisms of prolonged vasospasm after SAH. We used the basilar arteries from a "two-hemorrhage" canine model to make serial measurements of maximal contraction capacity and arterial stiffness (papaverine-insensitive tone) until day 28. We also examined hematoxylin-eosin-stained vasospastic canine basilar arteries for histological changes and immunohistochemically examined them for expression of myosin heavy chain isoforms (SMemb, SM1, and SM2), which are markers of smooth muscle phenotypic changes. Changes in collagen concentration in canine basilar arteries were also measured. Angiographic cerebral vasospasm persisted until day 14 and then gradually diminished; artery diameter returned to the control diameters on day 28. Maximal contraction capacity decreased until day 21 and showed some recovery by day 28. Arterial stiffness, on the other hand, progressed until day 28. Histological examination revealed medial thickening and increased connective tissue until day 21 and a return to control findings by day 28. The increased connective tissue was not accompanied by changes in collagen concentration, suggesting a role of some other protein in the increase in connective tissue. Immunohistochemical studies with anti-SMemb, anti-SM1, and anti-SM2 antibodies showed enhanced expression of SMemb from day 7 to day 21 and disappearance of SM1 and SM2 on days 14 and 21. The changes in myosin heavy chain isoform expression returned to normal on day 28. The above results indicate that biomechanical and phenotypic changes may play a pivotal role in sustaining cerebral vasospasm for >2 wk after SAH, with minimal changes in active myogenic arterial tone.

Indication for and surgical outcomes of the distraction method in various types of craniosynostosis. Advantages, disadvantages, and current concepts for surgical strategy in the treatment of craniosynostosis.

BACKGROUND: Various surgical techniques for the treatment of craniosynostosis using distraction devices have been described over the last few years and we have applied these procedures in seven patients with varying types of craniosynostosis. The aim of this report is to clarify the advantages and disadvantages of these surgical methods and to discuss current concepts for the surgical strategy in the treatment of craniosynostosis. MATERIAL AND METHODS: From January 2001 to March 2003, 25 patients with craniosynostosis were examined. Among them, 7 patients, 5 with Apert syndrome, 1 with Crouzon disease, and 1 with multiple-synostosis, underwent surgical treatment using the distraction method with internal distraction devices, according to our treatment strategy for craniosynostosis. All patients underwent preoperative and postoperative evaluations, which included the patient's neurological state, developmental quotient (DQ), and three-dimensional CT (3D-CT). RESULTS: The timing of the procedures undertaken was between the ages of 1 year 5 months and 12 years 6 months (mean age 4 years 11 months). Five patients had received previous treatment and this procedure was used as a secondary operation. Postoperative distraction distances varied from 7 to 20.5 mm (mean distraction distance: 14 mm). Satisfactory cranial volume expansion and aesthetically pleasing morphological states were achieved in all cases. Regarding complications, one patient required re-operation because of dislocation of the device and skin erosion caused by infection around the penetrated wound. Finally, in a second patient a distortion of the device occurred, but no re-operation was needed. CONCLUSION: The advantage of the distraction method is its applicability for Toddler or Elder Children Calvarial Reconstruction to correct cosmetic and functional problems. One disadvantage is the difficulty in using it for Infantile Calvarial Normalization because of thin calvarial bones and the necessity for re-operation to remove the device, which may result in it becoming a "fixation procedure," essentially contraindicated for the fast-developing brain and calvarias. However, the efficacy of this procedure is that the many advantages outweigh the disadvantages as sufficient calvarial expansion and good results using the distraction method, especially in toddler and elder children age groups, can be achieved.