• Clinical and Experimental Pediatrics
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• v.56 no.10
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• pp.431-438
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• 2013

Magnetoencephalography (MEG) records the magnetic field generated by electrical activity of cortical neurons. The signal is not distorted or attenuated, and it is contactless recording that can be performed comfortably even for longer than an hour. It has excellent and decent temporal resolution, especially when it is combined with the patient's own brain magnetic resonance imaging (magnetic source imaging). Data of MEG and electroencephalography are not mutually exclusive and it is recorded simultaneously and interpreted together. MEG has been shown to be useful in detecting the irritative zone in both lesional and nonlesional epilepsy surgery. It has provided valuable and additive information regarding the lesion that should be resected in epilepsy surgery. Better outcomes in epilepsy surgery were related to the localization of the irritative zone with MEG. The value of MEG in epilepsy surgery is recruiting more patients to epilepsy surgery and providing critical information for surgical planning. MEG cortical mapping is helpful in younger pediatric patients, especially when the epileptogenic zone is close to the eloquent cortex. MEG is also used in both basic and clinical research of epilepsy other than surgery. MEG is a valuable diagnostic modality for diagnosis and treatment, as well as research in epilepsy.

• Korean Journal of Radiology
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• v.23 no.6
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• pp.674-687
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• 2022

Patellofemoral instability (PFI) is common in pediatric knee injuries. PFI results from loss of balance in the dynamic relationship of the patella in the femoral trochlear groove. Patellar lateral dislocation, which is at the extreme of the PFI, results from medial stabilizer injury and leads to the patella hitting the lateral femoral condyle. Multiple contributing factors to PFI have been described, including anatomical variants and altered biomechanics. Femoral condyle dysplasia is a major risk factor for PFI. Medial stabilizer injury contributes to PFI by creating an imbalance in dynamic vectors of the patella. Increased Q angle, femoral anteversion, and lateral insertion of the patellar tendon are additional contributing factors that affect dynamic vectors on the patella. An imbalance in the dynamics results in patellofemoral malalignment, which can be recognized by the presence of patella alta, patellar lateral tilt, and lateral subluxation. Dynamic cross-sectional images are useful for in vivo tracking of the patella in patients with PFI. Therapeutic approaches aim to restore normal patellofemoral dynamics and prevent persistent PFI. In this article, the imaging findings of PFI, including risk factors and characteristic findings of acute lateral patellar dislocation, are reviewed. Non-surgical and surgical approaches to PFI in pediatric patients are discussed.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.139-144
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• 2016

Encephalocraniocutaneous lipomatosis (ECCL) is a rare neurocutaneous syndrome that affects ectomesodermal tissues (skin, eyes, adipose tissue, and brain). The neurologic manifestations associated with ECCL are various including seizures. However, ECCL patients very rarely develop brain tumors that originate from the neuroepithelium. This is the first described case of ECCL in combination with dysembryoplastic neuroepithelial tumor (DNET) that presented with intractable seizures. A 7-year-old girl was admitted to our center because of ECCL and associated uncontrolled seizures. She was born with right anophthalmia and lipomatosis in the right temporal area and endured right temporal lipoma excision at 3 years of age. Seizures began when she was 3 years old, but did not respond to multiple antiepileptic drugs. Brain magnetic resonance (MR) imaging performed at 8 and 10 years of age revealed an interval increase of multifocal hyperintense lesions in the basal ganglia, thalamus, cerebellum, periventricular white matter, and, especially, the right temporal area. A nodular mass near the right hippocampus demonstrated the absence of N-acetylaspartate decrease on brain MR spectroscopy and mildly increased methionine uptake on brain positron emission tomography, suggesting low-grade tumor. Twenty-four-hour video electroencephalographic monitoring also indicated seizures originating from the right temporal area. Right temporal lobectomy was performed without complications, and the nodular lesion was pathologically identified as DNET. The patient has been seizure-free for 14 months since surgery. Although ECCL-associated brain tumors are very rare, careful follow-up imaging and surgical resection is recommended for patients with intractable seizures.

• Journal of Korean Neurosurgical Society
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• v.38 no.6
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• pp.413-418
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• 2005

Objective : Although an encephaloduroarteriosynangiosis procedure using the superficial temporal artery [STA-EDAS] is an effective indirect bypass method in children with moyamoya disease[MMD], there is still a need for an additional bypass operation that can cover the area of the posterior circulation. The goal of this study is to evaluate the efficacy of the EDAS procedure using the occipital arteries [OA-EDAS]. Methods : From August 2003 to April 2004, We performed OA-EDAS in sixteen patients with MMD who have a circulatory insufficiency in the territory of the posterior cerebral artery[PCA]. The medical records were reviewed retrospectively. The surgical outcomes, including the changes in neurological status and imaging studies, with the degree of neovascularization on the cerebral angiogram, and the hemodynamic changes on single-photon emission computed tomography[SPECT], were analyzed. Results : These 16 children consisted of 5 boys and 11 girls aged 2 to 9 years. The clinical outcome of their PCA symptoms, such as visual transient ischemic attacks[TIAs] or visual field defect, was favorable in 14 patients of 16. Nine patients of 11 who underwent follow up magnetic resonance imaging[MRI] showed favorable MRI changes. On angiogram most of the patients exhibited good or fair revascularization of the PCA territory [7 of 8]. The hemodynamic changes on SPECT in the PCA territory after surgery showed improved vascular reserve in 13 of the 16 territories. Conclusion : OA-EDAS is a safe and efficacious revascularization procedure in patients with MMD who have compromised cerebral perfusion in PCA territory, or with visual TIAs.

• Clinical and Experimental Pediatrics
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• v.57 no.11
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• pp.500-504
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• 2014

Kaposiform hemangioendothelioma (KHE) is a rare, locally aggressive vascular tumor of intermediate malignancy with resemblance to Kaposi sarcoma. It occurs predominantly in pediatric age groups as a cutaneous lesion with focal infiltration into the adjacent soft tissue and bone. Although visceral involvement is very uncommon, several cases with bone, retroperitoneal, or mediastinal involvement have been described. KHE has been reported to occasionally occur in unusual sites such as the thymus, tonsils, larynx, paranasal sinuses, deltoid muscle, spleen, uterine cervix, thoracic spine, and even the breast. Multifocal KHE is an extremely rare entity with few reports available in the literature, none of which describes pulmonary involvement. Herein, we report a unique case of multifocal KHE in a 13-year-old boy presenting with a huge soft tissue mass in the upper extremity complicated by bilateral pulmonary nodules that developed into large, necrotic tumor masses.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.3
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• pp.304-309
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• 2020

An 18-year-old woman with annular pancreas and duodenal duplication presented with recurrent acute pancreatitis and underwent a resection of duodenal duplication. However, the patient experienced recurrent abdominal pain after resection. Abdominal computed tomography and magnetic resonance imaging showed a dilatation of the peripheral pancreatic duct and stenosis and malformation of both the Wirsung's and Santorini's duct due to multiple stones. The modified puestow procedure was performed. The main pancreatic ducts in the body and tail were opened, and the intrapancreatic common bile duct was preserved. A Roux-en-Y pancreatico-jejunostomy was performed for reconstructing the pancreaticobiliary system after removing the ductal protein plug. The patient experienced no abdominal pain, no significant elevation of the serum amylase and lipase levels, and no stone formation during the 2 years of follow-up. This procedure is considered to be beneficial for pediatric patients with chronic pancreatitis due to annular pancreas and duodenal duplication.

• Journal of Cardiovascular Imaging
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• v.30 no.2
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• pp.140-141
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• 2022

• Journal of Chest Surgery
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• v.50 no.2
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• pp.71-77
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• 2017

Background: Cardiac computed tomography (CT) has emerged as an alternative to magnetic resonance imaging (MRI) for ventricular volumetry. However, the clinical use of cardiac CT requires external validation. Methods: Both cardiac CT and MRI were performed prior to pulmonary valve implantation (PVI) in 11 patients (median age, 19 years) who had undergone total correction of tetralogy of Fallot during infancy. The simplified contouring method (MRI) and semiautomatic 3-dimensional region-growing method (CT) were used to measure ventricular volumes. Results: All volumetric indices measured by CT and MRI generally correlated well with each other, except for the left ventricular end-systolic volume index (LV-ESVI), which showed the following correlations with the other indices: the right ventricular end-diastolic volume index (RV-EDVI) (r=0.88, p<0.001), the right ventricular end-systolic volume index (RV-ESVI) (r=0.84, p=0.001), the left ventricular end-diastolic volume index (LV-EDVI) (r=0.90, p=0.001), and the LV-ESVI (r=0.55, p=0.079). While the EDVIs measured by CT were significantly larger than those measured by MRI (median RV-EDVI: $197mL/m^2$ vs. $175mL/m^2$, p=0.008; median LV-EDVI: $94mL/m^2$ vs. $92mL/m^2$, p=0.026), no significant differences were found for the RV-ESVI or LV-ESVI. Conclusion: The EDVIs measured by cardiac CT were greater than those measured by MRI, whereas the ESVIs measured by CT and MRI were comparable. The volumetric characteristics of these 2 diagnostic modalities should be taken into account when indications for late PVI after tetralogy of Fallot repair are assessed.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.6
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• pp.555-563
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• 2021

Purpose: The aim of this study was to evaluate the pancreatic fat fraction (PFF) using magnetic resonance imaging (MRI) in children with and without obesity and to correlate PFF with body mass index (BMI) z-score, hepatic fat fraction (HFF), and ultrasonography-derived pancreato-perihepatic fat index (PPHFI). Methods: This prospective study included 45 children with obesity and 19 without obesity (control group). PFF and HFF were quantitatively assessed using the abdominal multi-echo Dixon method for MRI. The PPHFI was assessed using transabdominal ultrasonography. Anthropometric, MRI, and ultrasonographic characteristics were compared between the two groups. Correlations between PFF, HFF, PPHFI, and BMI z-scores in each group were also analyzed. Results: The PFF, HFF, PPHFI, and BMI z-score were higher in the group with obesity than in the control group (PFF: 6.65±3.42 vs. 1.78±0.55, HFF: 19.5±13.0 vs. 2.31±1, PPHFI: 3.65 ±1.63 vs. 0.94±0.31, BMI z-score: 2.27±0.56 vs. 0.42±0.54, p<0.01, respectively). PFF was correlated with BMI z-scores, PPHFI, and HFF in the obesity group, and multivariate analysis showed that PFF was strongly correlated with BMI z-score and PPHFI (p<0.05). The BMI z-score was strongly correlated with PFF in the control group (p<0.01). Conclusion: These results suggest that MRI-derived PFF measures are associated with childhood obesity. PFF and PPHFI were also highly correlated in the obesity group. Therefore, PFF may be an objective index of pancreatic fat content and has the potential for clinical utility as a non-invasive biomarker for the assessment of childhood obesity.

• Korean Circulation Journal
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• v.54 no.2
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• pp.78-90
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• 2024

Background and Objective: We aimed to investigate long-term clinical and echocardiographic outcomes, including tricuspid valve durability, annular growth, and left ventricular reverse remodeling, after modified cone reconstruction in patients with Ebstein's anomaly. Methods: This was a retrospective analysis of all pediatric patients who underwent modified cone reconstruction for Ebstein's anomaly at a single tertiary center between January 2005 and June 2021. Results: A total of 14 pediatric patients underwent modified cone reconstruction for Ebstein's anomaly; the median age was 5.8 years (range, 0.01-16.6). There were three patients (21.4%) with Carpentier type B, ten patients with Carpentier type C (71.4%), and one patient with Carpentier type D (7.1%). There was no early or late mortality, arrhythmia, or readmission for heart failure at a 10-year follow-up. There were no cases of more than mild tricuspid stenosis or more than moderate tricuspid regurgitation during the study period, except for one patient with severe tricuspid regurgitation who underwent reoperation. The z value for tricuspid valve annular size significantly decreased immediately after the operation (2.46 vs. -1.15, p<0.001). However, from 1 year to 7 years after surgery, the z values were maintained between -1 and +1. Left ventricular end-systolic volume, end-diastolic volume, and stroke volume increased after surgery and remained elevated until seven years postoperatively. Conclusions: Ebstein's anomaly in children can be repaired by modified cone reconstruction with low mortality and morbidity, good tricuspid valve durability, and annular growth relative to somatic growth.