ABSTRACT
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.
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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.
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Background@#and Purpose A multifactorial antiepileptic mechanism underlies the ketogenic diet (KD), and one of the proposed mechanisms of action is that the KD inhibits the mammalian target of rapamycin (mTOR) pathway. To test this clinically, this study aimed to determine the efficacy of the KD in patients with pathologically confirmed focal cortical dysplasia (FCD) due to genetically identifiable mTOR pathway dysregulation. @*Methods@#A cohort of patients with pathologically confirmed FCD after epilepsy surgery and who were screened for the presence of germline and somatic mutations related to the mTOR pathway in peripheral blood and resected brain tissue was constructed prospectively. A retrospective review of the efficacy of the prior KD in these patients was performed. @*Results@#Twenty-five patients with pathologically confirmed FCD and who were screened for the presence of detectable somatic mTOR pathway mutations had received a sufficient KD. Twelve of these patients (48.0%) had germline or somatic detectable mTOR pathway mutations. A response was defined as a ≥50% reduction in seizure frequency. The efficacy of the KD after 3 months of dietary therapy was superior in patients with detectable mTOR pathway mutations than in patients without detectable mTOR pathway mutations, although the difference was not statistically significant (responder rates of 58.3% vs. 38.5%, p=0.434). @*Conclusions@#A greater proportion of patients with mTOR pathway responded to the KD, but there was no statistically significant difference in efficacy of the KD between patients with and without detectable mTOR pathway mutations. Further study is warranted due to the smallness of the sample and the limited number of mTOR pathway genes tested in this study.
ABSTRACT
Klebsiella pneumoniae is known to cause an invasive syndrome characterized by primary liver abscess associated with metastatic infection. The characteristics of the metastatic infection involving the musculoskeletal system in this invasive syndrome are not well understood. The authors present a case report of a patient who developed abscesses of the lower extremities along with abscesses of multiple organs, such as the liver and eye, caused by K. pneumonia. The patient was diagnosed early, and the infection was successfully controlled after several surgical treatments.
ABSTRACT
A 45-year-old man with a remote history of a left above-the-knee amputation presented to the emergency department with left hip pain after a mechanical fall. This case was an operative challenge because commonly used intraoperative traction methods could not be applied to a patient with an above-the-knee amputation. We describe a rarely utilized surgical technique of applying traction to an amputated extremity via a Steinmann pin during closed reduction and internal fixation of an intertrochanteric fracture.
ABSTRACT
DA-9601 is an extract obtained from Artemisia asiatica, which has been reported to have anti-inflammatory effects on gastrointestinal lesions; however, its possible anti-inflammatory effects on the small intestine have not been studied yet.Therefore, in this study, we investigated the protective effects of DA-9601 against the ACF-induced small intestinal inflammation. Inflammation of the small intestine was confirmed by histological studies and the changes in the CD4 + T cell fraction induced by the inflammation-related cytokines, and the inflammatory reactions were analyzed. Multifocal discrete small necrotic ulcers with intervening normal mucosa were frequently observed after treatment with ACF. The expression of IL-6, IL-17, and TNF-α genes was increased in the ACF group; however, it was found to have been significantly decreased in the DA-9601 treated group. In addition, DA-9601 significantly decreased the levels of proinflammatory mediators such as IL-1β, GMCSF, IFN-γ, and TNF-α; the anti-inflammatory cytokine IL-10, on the other hand, was observed to have increased. It is known that inflammatory mediators related to T cell imbalance and dysfunction continuously activate the inflammatory response, causing chronic tissue damage. The fractions of IFN-γ + Th1 cells, IL-4 + Th2 cells, IL-9 + Th9 cells, IL-17 + Th17 cells, and Foxp3 + Treg cells were significantly decreased upon DA-9601 treatment. These data suggest that the inflammatory response induced by ACF is reduced by DA-9601 via lowering of the expression of genes encoding the inflammatory cytokines and the concentration of inflammatory mediators. Furthermore, DA-9601 inhibited the acute inflammatory response mediated by T cells, resulting in an improvement in ACF-induced enteritis.
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Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.
ABSTRACT
DA-9601 is an extract obtained from Artemisia asiatica, which has been reported to have anti-inflammatory effects on gastrointestinal lesions; however, its possible anti-inflammatory effects on the small intestine have not been studied yet.Therefore, in this study, we investigated the protective effects of DA-9601 against the ACF-induced small intestinal inflammation. Inflammation of the small intestine was confirmed by histological studies and the changes in the CD4 + T cell fraction induced by the inflammation-related cytokines, and the inflammatory reactions were analyzed. Multifocal discrete small necrotic ulcers with intervening normal mucosa were frequently observed after treatment with ACF. The expression of IL-6, IL-17, and TNF-α genes was increased in the ACF group; however, it was found to have been significantly decreased in the DA-9601 treated group. In addition, DA-9601 significantly decreased the levels of proinflammatory mediators such as IL-1β, GMCSF, IFN-γ, and TNF-α; the anti-inflammatory cytokine IL-10, on the other hand, was observed to have increased. It is known that inflammatory mediators related to T cell imbalance and dysfunction continuously activate the inflammatory response, causing chronic tissue damage. The fractions of IFN-γ + Th1 cells, IL-4 + Th2 cells, IL-9 + Th9 cells, IL-17 + Th17 cells, and Foxp3 + Treg cells were significantly decreased upon DA-9601 treatment. These data suggest that the inflammatory response induced by ACF is reduced by DA-9601 via lowering of the expression of genes encoding the inflammatory cytokines and the concentration of inflammatory mediators. Furthermore, DA-9601 inhibited the acute inflammatory response mediated by T cells, resulting in an improvement in ACF-induced enteritis.
ABSTRACT
Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.
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Purpose@#To determine how the location, displacement, intra-articular involvement, comminution of a 5th metatarsal base fracture affect results of early weight-bearing treatment. @*Materials and Methods@#From January 2013 to July 2017, 34 cases of 34 patients diagnosed with a fracture of the zone I and II 5th metatarsal base were enrolled. The mean follow-up period was 13 months (6–15 months). One patient was excluded as a refracture during the follow-up period, and 33 patients underwent conservative treatment. Anteroposterior, lateral, and simple oblique radiography and computed tomography of the foot were performed to evaluate the location and displacement of the fracture, the degree of joint involvement, and comminution. In all 33 patients, a short leg cast or boot brace was selected immediately after the injury, tolerable weight bearing was allowed. If the pain disappeared, full weight bearing was performed after wearing a plain shoe or postoperative shoe. As a clinical result, the American Orthopedic Foot and Ankle Society (AOFAS) score was evaluated at the final follow-up. During outpatient follow-up, a simple radiograph of the foot was taken to confirm the time of radiological bone union and return to work. @*Results@#Nine males and 24 females, with an average age of 48.7 years, were enrolled in the study. Twenty-four patients had zone I fractures, and nine patients had zone II fractures. Twenty-two out of 33 patients had a fracture displacement of 2 mm or more. Nine and five patients had joint involvement and comminution, respectively. There was a statistically significant return to work from zone I to zone II. The AOFAS score was excellent at the final follow-up and there was no significant difference. When classifying and comparing the degree of fracture displacement, joint involvement, and comminution, there were no significant differences in the radiological union time and return to work. In all cases, satisfactory results were obtained at the final follow-up. @*Conclusion@#Satisfactory clinical results can be obtained by allowing early weight-bearing regardless of the fracture location, displacement, joint involvement, or comminution in zone I and II 5th metatarsal base fractures.
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Purpose@#Intensity-modulated radiotherapy (IMRT) allows for more precise treatment, reducing unwanted radiation to nearby structures. We investigated the safety and feasibility of IMRT for anaplastic ependymoma patients below 3 years of age. @*Materials and Methods@#A total of 9 anaplastic ependymoma patients below 3 years of age, who received IMRT between October 2011 and December 2017 were retrospectively reviewed. The median equivalent dose in 2 Gy fractions was 52.0 Gy (range, 48.0 to 60.0 Gy). Treatment outcomes and neurologic morbidities were reviewed in detail. @*Results@#The median patient age was 20.9 months (range, 12.1 to 31.2 months). All patients underwent surgery. The rates of 5-year overall survival, freedom from local recurrence, and progression-free survival were 40.6%, 53.3%, and 26.7%, respectively. Of the 9 patients, 5 experienced recurrences (3 had local recurrence, 1 had both local recurrence and cerebrospinal fluid [CSF] seeding, and 1 had CSF seeding alone). Five patients died because of disease progression. Assessment of neurologic morbidity revealed motor dysfunction in 3 patients, all of whom presented with hydrocephalus at initial diagnosis because of the location of the tumor and already had neurologic deficits before radiotherapy (RT). @*Conclusion@#Neurologic morbidity is not caused by RT alone but may result from mass effects of the tumor and surgical sequelae. Administration of IMRT to anaplastic ependymoma patients below 3 years of age yielded encouraging local control and tolerable morbidities. High-precision modern RT such as IMRT can be considered for very young patients with anaplastic ependymoma.
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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.
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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.
ABSTRACT
Epilepsy surgery revealed dramatically improved seizure outcomes over medical therapy in drug-resistant epilepsy patients. Children with epilepsy, however, have multiple epileptic focuses which require multilobar resection for better seizure outcome. Multilobar resection has not only the several severe surgical complications, such as hydrocephalus and shunt-related craniosynostosis, due to intracranial volume reduction. Isolation method (disconnection surgery) was progressively studied over epileptic focus removal (resective surgery) for seizure control. This concept was first introduced for functional hemispherotomy, and its primary principle is to preserve the vital vascularized brain that is functionally disconnected from the contralateral healthy brain. Currently in most epilepsy centers, the predominant disconnection surgical methods, including functional hemispherotomy, are continually being refined and are showing excellent results. They allow the functional isolation of the hemisphere or multi-lobe, affected by severe epilepsy. This review describes recent findings concerning the indication, surgical technique, seizure outcome and complications in several disconnection surgeries including the functional hemispherotomy for refractory pediatric epilepsy.
Subject(s)
Child , Humans , Brain , Cerebral Decortication , Craniosynostoses , Epilepsy , Hydrocephalus , Methods , SeizuresABSTRACT
Epilepsy surgery revealed dramatically improved seizure outcomes over medical therapy in drug-resistant epilepsy patients. Children with epilepsy, however, have multiple epileptic focuses which require multilobar resection for better seizure outcome. Multilobar resection has not only the several severe surgical complications, such as hydrocephalus and shunt-related craniosynostosis, due to intracranial volume reduction. Isolation method (disconnection surgery) was progressively studied over epileptic focus removal (resective surgery) for seizure control. This concept was first introduced for functional hemispherotomy, and its primary principle is to preserve the vital vascularized brain that is functionally disconnected from the contralateral healthy brain. Currently in most epilepsy centers, the predominant disconnection surgical methods, including functional hemispherotomy, are continually being refined and are showing excellent results. They allow the functional isolation of the hemisphere or multi-lobe, affected by severe epilepsy. This review describes recent findings concerning the indication, surgical technique, seizure outcome and complications in several disconnection surgeries including the functional hemispherotomy for refractory pediatric epilepsy.
Subject(s)
Child , Humans , Brain , Cerebral Decortication , Craniosynostoses , Epilepsy , Hydrocephalus , Methods , SeizuresABSTRACT
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.
Subject(s)
Craniotomy , Endoscopes , Endoscopy , Epilepsy , Hamartoma , Laser Therapy , Patient Selection , RadiosurgeryABSTRACT
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.
Subject(s)
Brain , Craniosynostoses , Endoscopes , Hamartoma , Hydrocephalus , Neuroendoscopy , Skull Base , Spine , Surgeons , VentriculostomyABSTRACT
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.
Subject(s)
Axis, Cervical Vertebra , Basal Ganglia , Brain Neoplasms , Central Nervous System , Clinical Protocols , Craniospinal Irradiation , Drug Therapy , Germ Cells , Germinoma , Neoplasms, Germ Cell and Embryonal , Quality of Life , Radiotherapy , Specialization , Survival RateABSTRACT
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.
Subject(s)
Child , Humans , Brain , Congenital Abnormalities , Craniosynostoses , Prognosis , Skull , SuturesABSTRACT
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.