Temporary Surgical Management of Intraventricular Hemorrhage in Premature Infants

Post-hemorrhagic hydrocephalus (PHH) in preterm infant is common, life-threatening and the main cause of bad developmental outcomes. Ventriculoperitoneal (VP) shunt is used as the ultimate treatment for PHH. Low birth weight and low gestational age are the combination of worse prognostic factors while the single most important prognostic factor of VP shunting is age. Aggressive and early intervention have better effect in intraventricular hemorrhage and intracranial pressures control. It reduces infection rate and brain damage resulted in delayed shunt insertion. It is extremely important to let PHH infants get older and gain weight to have internal organs to be matured before undergoing VP shunt. As premature infants undergo shunt after further growth, shunt-related complications would be reduced. So temporary surgical intervention is critical for PHH infants to have them enough time until permanently shunted.

Medical Applications of 3D Printing and Standardization Issues

The three-dimensional (3D) printing itself is not a novel technology, it is more than 30 years old. Stereolithographic (SLA) technology has been used as the first and popular technology for medical application of 3D printing. Since 1991 Radiology and Plastic Surgery have published articles about SLA for rapid prototyping anatomical 3D models. Medical applications of 3D printing have been popularizing and stabilizing so far. Implantable medical devices such as metal or absorbable implants, surgical guide systems, prosthesis and orthosis, and 3D anatomical models for normal or diseased anatomy have been developing and expanding its markets so far. There are many obstacles, such as insurance, authorization as a medical device, and lack of standards technology for further expansion of medical applications. Many technical specifications and guidelines for authorization as medical device have been published by regulatory bodies from many countries. Even though international standards for 3D printing have been developing more and more, there have been few standards for medical application of 3D printing. In this harsh environment academia, company, research institute, regulatory bodies, and government have been doing good job for the development of 3D printing industry.

Effectiveness of C1 Laminectomy for Chiari Malformation Type 1: Posterior Fossa Volume Expansion and Syrinx-Volume Decrease Rate

Purpose@#This study aimed to analyze the effect of foramen magnum decompression with C1 laminectomy (C1L) for Chiari malformation type 1 (CM-1) in terms of improving clinical symptoms, expanding posterior fossa volume, and decreasing syrinx volume. @*Materials and Methods@#Between January 2007 and June 2019, 107 patients with CM-1 were included. The median patient age was 13±13 years (range: 9 months–60 years), female-to-male ratio was 1:1, and average length of tonsil herniation was 13±5 mm (range: 5–24 mm). Surgical techniques were divided into four groups based on duraplasty or C1L usage. Among the study subjects, 38 patients underwent duraplasty and had their syrinx volumes measured separately on serial magnetic resonance imaging. A three-dimensional visualization software was used to evaluate the syrinx-volume decrease rate. @*Results@#Bony decompression exhibited a mere 20% volume expansion of the lower-half posterior fossa. C1L offered a 3% additional volume expansion, which rose to 5% when duraplasty was added (p=0.029). There were no significant differences in complication rate when C1L was combined with duraplasty (p=0.526). Syrinx volumes were analyzed in 38 patients who had undergone duraplasty. Among them, 28 patients who had undergone duraplasty without C1L demonstrated a 5.9% monthly decrease in syrinx volume, which was 7.5% in the remaining 10 patients with C1L (p=0.040). @*Conclusion@#C1L was effective in increasing posterior fossa volume expansion, both with and without duraplasty. A more rapid decrease in syrinx volume occurred when C1L was combined with duraplasty.

Determining the optimal timing of screening spinal cord ultrasonography to detect filum terminale lipoma in infants

Purpose@#The purpose of this study was to identify the optimal timing for screening spinal cord ultrasonography (US) to detect filum terminale lipoma in infants. @*Methods@#We retrospectively reviewed infants (<12 months old) who underwent repeated spinal cord US between April 2011 and January 2019. We excluded infants if they only had one US examination, or if they had lesions other than filum terminale lipoma. Infants with filum terminale lipoma on magnetic resonance imaging were included in the lipoma group and the others in the control group. A linear mixed model was used to assess differences in the growth pattern of filum terminale thickness by age and group. The cutoff thickness on US and its diagnostic performance were assessed according to age. @*Results@#Among 442 infants with 901 US examinations, 46 were included in the lipoma group and 58 in the control group. Sixty-seven infants had unmeasurable filum terminale thickness on initial US, including 55 neonates (82.1%) before 1 month of age. The lipoma group had significantly greater filum terminale thickness than the control group (P<0.001). Thickness increased with age in the lipoma group (P=0.027). The sensitivity of US was 87.5% and the area under the receiver operating characteristic curve was 0.949 (95% confidence interval, 0.849 to 0.991) with a cutoff value of 1.1 mm in 4- to 6-month-old infants. @*Conclusion@#Screening spinal cord US could effectively diagnose filum terminale lipoma in 4- to 6-month-old infants with a cutoff thickness of 1.1 mm. Spinal cord US can be used to screen young infants with intraspinal abnormalities.

Clinical Features and Outcomes of Intracranial Aneurysm Associated with Moyamoya Disease

Background@#and Purpose: Moyamoya disease (MMD) is a rare form of intracranial stenoocclusive disease that can be associated with intracranial aneurysms. We evaluated the clinical features and outcomes of MMD-associated aneurysms while focusing on their locations. @*Methods@#Between January 1998 and December 2018 there were 1,302 adult and pediatric patients diagnosed as MMD at a single institution. These patients included 38 with 44 MMD-associated aneurysms. The MMD-associated aneurysms were classified into two groups based on their locations: major-artery aneurysms and non-major-artery aneurysms. The clinical and radiological data for patients with MMD-associated aneurysms were reviewed retrospectively. @*Results@#The 44 MMD-associated aneurysms comprised 28 in major arteries and 16 in nonmajor arteries. All of the major-artery aneurysms were initially unruptured lesions, and follow-up angiography showed that 23 (82.1%) had an improved or stable status and 5 (17.9%) had a worse status. The non-major-artery aneurysms comprised 10 ruptured and 6 unruptured lesions, and follow-up angiography showed that 11 (68.8%) had improved or were stable and 5 (31.2%) had worsened. At the latest follow-up, there were four cases of unfavorable outcome: two initial hemorrhagic insults, one treatment-related morbidity, and one repeatedhemorrhage case. @*Conclusions@#MMD-associated aneurysms occurred in 3.3% of the MMD cohort in this study, of which 63.6% were major-artery aneurysms and 36.4% were non-major-artery aneurysms. The major-artery group included 17.9% that became angiographically worse, while 31.2% were growing or hemorrhaging in the non-major-artery group.

Sacral Reconstruction with a 3D-Printed Implant after Hemisacrectomy in a Patient with Sacral Osteosarcoma: 1-Year Follow-Up Result

Pelvic reconstruction after sacral resection is challenging in terms of anatomical complexity, excessive loadbearing, and wide defects. Nevertheless, the technological development of 3D-printed implants enables us to overcome these difficulties. Here, we present a case of sacral osteosarcoma surgically treated with hemisacrectomy and sacral reconstruction using a 3D-printed implant. The implant was printed as a customized titanium prosthesis from a 3D real-sized reconstruction of a patient's CT images. It consisted mostly of a porous mesh and incorporated a dense strut. After 3-months of neoadjuvant chemotherapy, the patient underwent hemisacretomy with preservation of contralateral sacral nerves. The implant was anatomically installed on the defect and fixed with a screw-rod system up to the level of L3. Postoperative pain was significantly low and the patient recovered sufficiently to walk as early as 2 weeks postoperatively. The patient showed left-side foot drop only, without loss of sphincter function. In 1-year follow-up CT, excellent bony fusion was noticed. To our knowledge, this is the first report of a case of hemisacral reconstruction using a custom-made 3D-printed implant. We believe that this technique can be applied to spinal reconstructions after a partial or complete spondylectomy in a wide variety of spinal diseases.

The Unnecessity of Positron Emission Tomography Computed Tomography in the Etiologic Evaluation of Neurodevelopmental Delay in Craniosynostosis Patients

BACKGROUND: In evaluation of craniosynostosis patients in terms of neurodevelopmental delay, positron emission tomography computed tomography (PET-CT) scan can be used to assess brain abnormalities through glucose metabolism. We aimed to determine the unnecessity of PET-CT in this study. METHODS: Thirty-eight patients diagnosed with craniosynostosis who underwent distraction osteogenesis from October, 2010 to November, 2013 were reviewed. Magnetic resonance imaging (MRI) and PET-CT scan were carried out for evaluation of the brain structure and function, whereas X-ray and CT scan were taken for evaluation of the skull. RESULTS: Nine patients reported abnormal MRI findings which were not significant, and five patients showed local problem on brain on PET-CT scan. No correlation was found among them. CONCLUSION: PET-CT evaluation of possible abnormal brain findings do not affect surgical planning or require additional therapy. Preoperative PET-CT scan is not the essential study to get any etiologic information of the disease consequences or to establish the treatment plan.

Endoscopic Treatment of Hypothalamic Hamartomas

Hypothalamic hamartoma (HH) is a benign indolent lesion despite the presentation of refractory epilepsy. Behavioral disturbances and endocrine problems are additional critical symptoms that arise along with HHs. Due to its nature of generating epileptiform discharge and spreading to cortical region, various management strategies have been proposed and combined. Surgical approaches with open craniotomy or endoscopy, stereotactic approaches with radiosurgery and gamma knife surgery or radiofrequency thermos-coagulation, and laser ablation have been introduced. Topographical dimension and the surgeon’s preference are key factors for treatment modalities. Endoscopic disconnection has been one of the most favorable options performed in treating HHs. Here we discuss presurgical evaluation, patient selection, surgical procedures, and complications.

Neuroendoscopy: Current and Future Perspectives

Neuroendoscopic surgery is performed because it causes minimal damage to normal structures, carries a lower rate of complications, and achieves excellent outcomes. Surgeons using an endoscope and related instruments can perform complex operations through very small incisions, which is especially useful for minimally invasive procedures for the brain and spine. Neuroendoscopic surgery is now performed in cases of obstructive hydrocephalus, various intraventricular lesions, hypothalamic hamartomas, craniosynostosis, skull base tumors, and spinal lesions. This review discusses the brief history of neuroendoscopy and the current state and future perspectives of endoscopic surgery.

Current Trends in Management for Central Nervous System Germ Cell Tumor / 임상소아혈액종양

Central nervous system germ cell tumor is a rare but important tumor in childhood brain tumors. It requires a multidisciplinary approach to increase survival and promote quality of life, and all three treatment modalities including surgery, radiotherapy and chemotherapy has its own distinct role for germ cell tumor. For germinoma, radiotherapy alone can cure the disease but, the effort to limit the long term toxicity and the proper combination of chemotherapy and radiotherapy are under investigation. Craniospinal irradiation is reserved only for the disseminated germinoma or nongerminomatous germ cell tumor (NGGCT). For germinoma, craniospinal irradiation of 20 to 24 Gy is sufficient to control microscopic disease in the spinal axis. Chemotherapy and radiotherapy composed of 30 to 40 Gy of local field radiotherapy and 20 to 24 Gy of whole ventricular irradiation are required for localized germinoma, but the proper combination of two modalities has yet to be defined. For NGGCT, both the chemotherapy and radiotherapy should be performed, and survival rate is substantially increasing with modern treatment protocols. The omission of craniospinal irradiation is being tried for the localized NGGCT in international cooperative group trials. Surgery has its role for the resection of residual disease after the treatment, and the extent of resection in NGGCT has the prognostic implication. Bifocal germ cell tumors and basal ganglia germ cell tumor have distinctive clinical course and mandate special attention. To advance clinical and biological perspectives in central nervous germ cell tumor, the cooperation and communication of the multidisciplinary specialists are essential.

Neurodevelopmental Problems in Non-Syndromic Craniosynostosis

Craniosynostosis is the premature fusion of calvarial sutures, resulting in deformed craniofacial appearance. Hence, for a long time, it has been considered an aesthetic disorder. Fused sutures restrict growth adjacent to the suture, but compensatory skull growth occurs to accommodate the growing brain. The primary goal for the management of this craniofacial deformity has been to release the constricted skull and reform the distorted shape of the skull vault. However, the intellectual and behavioral prognosis of affected children has also been taken into consideration since the beginning of the modern era of surgical management of craniosynostosis. A growing body of literature indicates that extensive surgery, such as the whole-vault cranioplasty approach, would result in better outcomes. In addition, the age at treatment is becoming a major concern for optimal outcome in terms of cosmetic results as well as neurodevelopment. This review will discuss major concerns regarding neurodevelopmental issues and related factors.

Multiple Spontaneous Intracerebral Hematoma without Presenting Risk Factors

The incidence of intracerebral hemorrhage in those aged 45–84 years is 0.3-0.5%. In people over 80 years of age, this incidence increases 25-fold compared with that of the total population. The most common causes of spontaneous intracerebral hemorrhage in the younger population are vascular malformation, aneurysm, and overuse of drugs. In contrast, common causes in the elderly include hypertension, tumors, and coagulation disorders. Here, we present a case involving a 72-year-old male patient who, without any of these predisposing conditions, was admitted to the hospital with spontaneous intracerebral hemorrhage and showed signs of multifocal intracerebral hemorrhage during his stay. We conclude that spontaneous intracerebral hemorrhage can occur without any predisposing factors, and can lead to a patient's death. Therefore, the possibility of recurrent spontaneous intracerebral hemorrhage must be considered in patients with primary spontaneous intracerebral hemorrhage.

Current Trends in Management for Central Nervous System Germ Cell Tumor / 임상소아혈액종양

Central nervous system germ cell tumor is a rare but important tumor in childhood brain tumors. It requires a multidisciplinary approach to increase survival and promote quality of life, and all three treatment modalities including surgery, radiotherapy and chemotherapy has its own distinct role for germ cell tumor. For germinoma, radiotherapy alone can cure the disease but, the effort to limit the long term toxicity and the proper combination of chemotherapy and radiotherapy are under investigation. Craniospinal irradiation is reserved only for the disseminated germinoma or nongerminomatous germ cell tumor (NGGCT). For germinoma, craniospinal irradiation of 20 to 24 Gy is sufficient to control microscopic disease in the spinal axis. Chemotherapy and radiotherapy composed of 30 to 40 Gy of local field radiotherapy and 20 to 24 Gy of whole ventricular irradiation are required for localized germinoma, but the proper combination of two modalities has yet to be defined. For NGGCT, both the chemotherapy and radiotherapy should be performed, and survival rate is substantially increasing with modern treatment protocols. The omission of craniospinal irradiation is being tried for the localized NGGCT in international cooperative group trials. Surgery has its role for the resection of residual disease after the treatment, and the extent of resection in NGGCT has the prognostic implication. Bifocal germ cell tumors and basal ganglia germ cell tumor have distinctive clinical course and mandate special attention. To advance clinical and biological perspectives in central nervous germ cell tumor, the cooperation and communication of the multidisciplinary specialists are essential.

Patterns of Failure Following Multimodal Treatment for Medulloblastoma: Long-Term Follow-up Results at a Single Institution / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: The purpose of this study is to investigate the long-term results and appropriateness of radiation therapy (RT) for medulloblastoma (MB) at a single institution. MATERIALS AND METHODS: We analyzed the clinical outcomes of 106 patients with MB who received RT between January 1992 and October 2009. The median age was 7 years (range, 0 to 50 years), and the proportion of M0, M1, M2, and M3 stages was 60.4%, 8.5%, 4.7%, and 22.6%, respectively. The median total craniospinal irradiation (CSI) and posterior fossa tumor bed dose in 102 patients (96.2%) treated with CSI was 36 Gy and 54 Gy, respectively. RESULTS: The median follow-up period in survivors was 132 months (range, 31 to 248 months). A gradual improvement in survival outcomes was observed, with 5-year overall survival rates of 61.5% in 1990s increasing to 73.6% in 2000s. A total of 29 recurrences (27.4%) developed at the following sites: five (17.2%) in the tumor bed; five (17.2%) in the posterior fossa other than the tumor bed; nine (31%) in the supratentorium; and six (20.7%) in the spinal subarachnoid space only. The four remaining patients showed multiple site recurrences. Among 12 supratentorial recurrences, five cases recurred in the subfrontal areas. Although the frequency of posterior fossa/tumor bed recurrences was significantly high among patients treated with subtotal resection, other site (other intracranial/spinal) recurrences were more common among patients treated with gross tumor removal (p=0.016). There was no case of spinal subarachnoid space relapse from desmoplastic/extensive nodular histological subtypes. CONCLUSION: Long-term follow-up results and patterns of failure confirmed the importance of optimal RT dose and field arrangement. More tailored multimodal strategies and proper CSI technique may be the cornerstones for improving treatment outcomes in MB patients.

Skull Reconstruction with Custom Made Three-Dimensional Titanium Implant

BACKGROUND: Source material used to fill calvarial defects includes autologous bones and synthetic alternatives. While autologous bone is preferable to synthetic material, autologous reconstruction is not always feasible due to defect size, unacceptable donor-site morbidity, and other issues. Today, advanced three-dimensional (3D) printing techniques allow for fabrication of titanium implants customized to the exact need of individual patients with calvarial defects. In this report, we present three cases of calvarial reconstructions using 3D-printed porous titanium implants. METHODS: From 2013 through 2014, three calvarial defects were repaired using custommade 3D porous titanium implants. The defects were due either to traumatic subdural hematoma or to meningioma and were located in parieto-occipital, fronto-temporo-parietal, and parieto-temporal areas. The implants were prepared using individual 3D computed tomography (CT) data, Mimics software, and an electron beam melting machine. For each patient, several designs of the implant were evaluated against 3D-printed skull models. All three cases had a custom-made 3D porous titanium implant laid on the defect and rigid fixation was done with 8 mm screws. RESULTS: The custom-made 3D implants fit each patient's skull defect precisely without any dead space. The operative site healed without any specific complications. Postoperative CTs revealed the implants to be in correct position. CONCLUSION: An autologous graft is not a feasible option in the reconstruction of large calvarial defects. Ideally, synthetic materials for calvarial reconstruction should be easily applicable, durable, and strong. In these aspects, a 3D titanium implant can be an optimal source material in calvarial reconstruction.

Cognitive Outcome of Pediatric Moyamoya Disease

Quality of life is the current trend and issue for the most of human diseases. In moyamoya disease (MMD), surgical revascularization has been recognized as the possible assistance to reduce the neurological insult. However, the progressive nature of the disease has been invincible so far. To improve the quality of life of MMD patients not only the protection from the neurological insult but also the maintenance or improvement of cognitive function is inevitable. For pediatric MMD patients, younger age or longer duration of disease is the key factor among the prognostic factors for bad neurological outcomes. Hence, 'the earlier, the better' is the most precious rule for treatment. Protection from neurological insult is very critical and foremost important to improve cognitive outcome. Clinicians need to know the neuropsychological profile of MMD patients for the care of whole person and make an effort to protect the patients from neurological insults to maintain or improve it.

Exploring the Subtypes of Intellectual and Psychological Characteristics in Children with Brain Tumors and Their Mothers' Parenting Stress

PURPOSE: Parenting stress could be different among mothers of children with brain tumors. The aim of this study was to explore subtypes of children with brain tumor based on their intellectual and psychological characteristics and to compare the parenting stress of their mothers among the subtypes. METHODS: Latent profile analysis was conducted on the Verbal IQ, Performance IQ, and the scores of Social Problem, Internalization Behavior Problem, and Externalization Behavior Problem in a total of 90 children with brain tumors. Furthermore, the mothers' parenting stress was compared among the explored subtypes. RESULTS: Four-categorization appeared the best way to describe the intellectual and psychological presentation of the children with brain tumors and they are as follows; (1) Borderline Intelligence-Socially Maladjusted (B-SM: 24.4%), (2) Average Intelligence-Internalization Behavior Problem (A-I: 44.4%), (3) Average Intelligence-Normal Behavior (A-N: 18.9%), and (4) Retarded Intelligence-Global Maladjusted (R-GM: 12.2%). Mothers of R-GM group reported higher levels of stress on total domain and the subfactors of distractibility and adaptability of Korean Parenting Stress Index compared to mothers of A-N group, and the highest level of stress on spouse subfactor among the mothers of all subtypes. Mothers of subtypes with either intellectual or psychological problems showed higher level of stress on demandingness subfactor compared to mothers of A-N group. CONCLUSION: Eighty percents of children with brain tumor showed intellectual or psychological problems and the mothers of the children with both problems reported the highest parenting stress. The tailored services are needed to manage the parenting stress of mothers of children with brain tumors.

Surgical treatment of hypothalamic hamartoma

Hypothalamic hamartomas are often associated with early onset gelastic seizures, precocious puberty, behavioral problem and suboptimal response to antiepiletptic drugs. Until now, four surgical options have been reported to reduce seizure by >50%. Surgical excision have good seizure outcome but postoperative complications were not infrequent, whether by pterional or transcallosal interforniceal approach. Radiosurgery is noninvasive alternative to resective surgery but the effect usually does not appear until several months later. Radiofrequency ablation is less invasive than surgical resection and its effect is immediate, but lacks long term follow-up data. It also requires three dimensional analysis of the lesion to enhance effi cacy and safety. As hypothalamic hamartoma is intrinsically epileptogenic and epileptogenic discharges spread from the lesion, blocking the seizure propagation through endoscopic disconnection is regarded as an effective and safer option. Surgical choice for a particular patient should take into account the hamartoma’s size, location, surgeon’s preferences, possible complication as well as the effect and risk of the various surgical methods. In the present review, open surgery, endoscopic disconnection, radiosurgery and radiofrequency ablation are discussed.

The Emotional Distress Prior to the Surgery and the Parenting Stress in Elementary School Children with Neurosurgical Diseases: The Preliminary Study

OBJECTIVES: This study was conducted to compare emotional problems in children with neurosurgical diseases prior to surgery as well as levels of parenting stress experienced by mothers of children treated with surgery and those without surgery. The goals was to provide a basis on which comprehensive treatment interventions could be established. METHODS: Subjects included 78 elementary school children who visited the Department of Pediatric Neurosurgery as well as their mothers. The Children's Depression Inventory (CDI) and the Revised Children Manifest Anxiety Scale (RCMAS) were administered to children. The Parenting Stress Indexs (PSI) and Beck Depression Inventory (BDI) were administered to their mothers. RESULTS: The children with neurosurgical diseases showed higher levels of depression prior to surgery. In their mothers, parenting stress was relatively higher and parenting competence was lower. In particular, for those children who underwent surgery, depression was significantly higher. Specifically, they exhibited more negative expectations, and negative self-image. In mothers of children who underwent surgery, depression was significantly higher. CONCLUSION: Our results suggest higher levels of emotional distress and parental stress in children who undergo neurosurgical operations and their mothers, respectively. Based on this, the necessity for surgical and comprehensive psychological intervention is suggested.

Long Term Magnetic Resonance Angiography Follow-up in Moyamoya Disease / 대한뇌혈관외과학회지

OBJECTIVE: Revascularization is an effective treatment for the ischemic symptom of moyamoya disease. Indirect revascularization is also effective. Magnetic resonance angiography (MRA) has the ability for collateral formation that is equivalent to conventional angiography. This study analyzed the results of indirect revascularization by MRA. METHODS: A total of 25 patients underwent bilateral EDAS for the management of moyamoya disease. All patients underwent MRA after surgery more than 24 months later. The collateral formation was graded as Good, Fair, and Poor. The clinical outcome was assessed as Excellent, Good, Fair, and Poor. RESULTS: Good collateral formation was 32 sides of the EDAS, and fair was 18. An excellent clinical outcome was obtained in 15 patients, Good in 8, Fair in 1, and Poor in 1. There was a significant correlation between the preoperative symptom, gender, and the clinical outcome. CONCLUSION: In the management of ischemic moyamoya disease, indirect revascularization has been the golden standard with remarkably low morbidity and mortality. Moreover, and MRA can replace conventional angiography in the follow-up of moyamoya patients.