• Asian Pacific Journal of Cancer Prevention
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• v.17 no.7
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• pp.3271-3276
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• 2016

Background: Intracranial nonvestibular schwannomas arising from various cranial nerves excluding CN VIII are uncommon. Recently, stereotactic radiosurgery (SRS) and fractionated stereotactic radiotherapy (SRT) have been widely reported as effective treatment modalities for nonvestibular schwannomas. The purpose of this study was to study the long term clinical outcome for nonvestibular schwannomas treated with both X-Knife and CyberKnife (CK) radiosurgery at one institution. Materials and Methods: From 2004 to 2013, fifty-two nonvestibular schwannoma patients were included in this study, 33 patients (63%) were treated with CK, and 19 (37%) were treated with X-Knife. The majority of the tumors were jugular foramen schwannomas (38%) and trigeminal schwannomas (27%). HSRT was given for 45 patients (86%), whereas CSRT was for 6 (12%) and SRS for 1 (2%). Results: The median pretreatment volume was $9.4cm^3$ (range, $0.57-52cm^3$). With the median follow up time of 36 months (range, 3-135), the 3 and 5 year progression free survival was 94 % and 88%, respectively. Tumor size was decreased in 13 (25%), stable in 29 (56%), and increased in 10 (19%). Among the latter, 3 (30%) required additional treatment because of neurologic deterioration. No patient was found to develop any new cranial nerve deficit after SRS/SRT. Conclusions: These data confirmed that SRS/SRT provide high tumor control rates with low complications. Large volume tumors and cystic expansion after radiation should be carefully followed up with neurological examination and MRI, because it may frequently cause neurological deterioration requiring further surgery.

• 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.53 no.5
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• pp.281-287
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• 2013

Objective : Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor classified as WHO II. It is generally benign, but disease progression and malignant transformation have been reported. Prognostic factors for PXA and optimal therapies are not well known. Methods : The study period was January 2000 to March 2012. Data on MR findings, histology, surgical extents and adjuvant therapies were reviewed in twenty-two patients diagnosed with PXA. Results : The frequent symptoms of PXA included seizures, headaches and neurologic deficits. Tumors were most common in the temporal lobe followed by frontal, parietal and occipital lobes. One patient who died from immediate post-operative complications was excluded from the statistical analysis. Of the remaining 21 patients, 3 (14%) died and 7 (33%) showed disease progression. Atypical tumor location (p<0.001), peritumoral edema (p=0.022) and large tumor size (p=0.048) were correlated with disease progression, however, Ki-67 index and necrosis were not statistically significant. Disease progression occurred in three (21%) of 14 patients who underwent GTR, compared with 4 (57%) of 7 patients who did not undergo GTR, however, it was not statistically significant. Ten patients received adjuvant radiotherapy and the tumors were controlled in 5 of these patients. Conclusion : The prognosis for PXA is good; in our patients overall survival was 84%, and event-free survival was 59% at 3 years. Atypical tumor location, peritumoral edema and large tumor size are significantly correlated with disease progression. GTR may provide prolonged disease control, and adjuvant radiotherapy may be beneficial, but further study is needed.

• The Korean Journal of Pain
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• v.9 no.1
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• pp.63-68
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• 1996

Background; Epidural analgesia for controlling labor pain has recently gained world-wide popularity. However, many patients scheduled for continuous epidural analgesia voice concern over harmful effects to their fetus and other possible complications such as hemodynamic changes, back pain and neurologic sequelae etc. The aim of this study was to evaluate the hemodynamic changes with and without epidural block as a measure to determine the safety of epidural analgesia during labor and delivery. Methods: Twenty healthy subjects were divided equally into two groups(Group 1 without epidural block, and Group 2 with epidural block) and serial hemodynamic measurements were taken in all subjects with transcutaneous impedence cardiography. The epidural catheter was inserted at the level of $L_{3,4}$ in Group 2 and analgesia was maintained using 0.25% bupivacaine mixed with fentanyl. Results: Cardiac output increased slightly with cervical dilatation in both groups, but no significant differences were found between the two groups. Similarly, no significant differences were found in blood pressures between the two groups. Stroke volume and end-diastolic volume indices were slightly decreased in group 1 and slightly increased in group 2. However, there were no significant differences between the two groups. The ejection fraction was nearly constant and ranging 56~59%. Conclusion: We concluded epidural analgesia for labor and delivery is a safe technique for the parturients since results indicated no significant differences in hemodynamic changes, as compared to the control group.

• Journal of Chest Surgery
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• v.39 no.1 s.258
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• pp.68-71
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• 2006

We operated on a 41-year-old man using venous bypass shunt for superior vena cava (SVC) syndrome caused by mediastinal fibrosis. The patient had substantially high venous pressure and high risk of postoperative neurologic deficits. The collateral veins were deemed to be interrupted during the surgical reconstruction of SVC. Treatment included resection of the obstructed SVC and innominate vein and reconstruction with an autologous pericardial tube graft. During the operation, venous drainage from upper body was maintained with an extraluminal bypass shunt. The shunt was effective at prompt relief of venous hypertension, eliminating the time constraints, and preventing the postoperative complications.

• Journal of Genetic Medicine
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• v.15 no.1
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• pp.28-33
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• 2018

Fabry disease (FD) is a rare X-linked lysosomal storage disorder caused by the deficiency of ${\alpha}$-galactosidase A. Patients with classical FD present acroparesthesia, hypohidrosis, cornea verticillata, disseminated angiokeratoma, and microalbuminuria in childhood, and develop life-threatening renal, cardiac, and cerebrovascular complications typically after the fourth decade of life. To date, more than 700 mutations responsible for FD have been identified in the human GLA gene. Herein, we report a novel GLA mutation, c.1117_1141del25 (p.Gly373Profs*10), identified in an 11-year-old Korean boy with FD presenting early cardiac and neurologic manifestation and in other affected family members. The boy had acroparesthesia, hypohidrosis, cornea verticillata, and left ventricular hypertrophy. His mother and sister also had acroparesthesia. Two males on the mother's side had similar pain and died of unknown causes. The plasma ${\alpha}$-galactosidase A activity (4.1 nmol/hr/mg protein) of the patient was markedly lower than the mean value of the controls. The plasma level of globotriaosylsphingosine was elevated in the patient and all the carriers. We concluded the novel GLA mutation c.1117_1141del25 is a pathogenic mutation for FD, probably related to the early cardiac manifestation of FD.

• Archives of Plastic Surgery
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• v.38 no.4
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• pp.516-518
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• 2011

Purpose: The prevalence of antibiotic-resistant Streptococcus pneumoniae meningitis has increased worldwide. There are some reports about postoperative antibiotic-resistant Streptococcus pneumoniae infection after craniofacial surgery, but, there is no report in Korea. We present a report on the treatment of postoperative multidrug-resistant Streptococcus pneumoniae (MRSP) meningitis and sepsis after craniofacial surgery based on our experience. Methods: The patient was a 7-year-old boy with Crouzon's disease who was treated by fronto-orbital bar advancement. Intraoperatively, frontal sinus opening was seen during osteotomy which was covered with forehead galeopericranial flap. MRSP meningitis was diagnosed after the surgery, he was treated with intravenous vancomycin, meropenem, and levofloxacin. Results: The patient was treated successfully after 3 weeks of intravenous antibiotics treatment. During the 8 month follow-up period, there was no neurologic sequelae. Conclusion: Postoperative infection after craniofacial surgery is an important phenomenon that needs immediate recognition. Prevention, early diagnosis, and treatment immediate after onset are important as countermeasures against postoperative drug-resistant bacterial infection. To prevent adverse outcome and reoperation, proper antibiotics treatment should be performed.

• Archives of Plastic Surgery
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• v.37 no.6
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• pp.827-830
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• 2010

Purpose: Congenital spinal dermal sinus tract is a rare lesion connecting skin to deeper structures including neural tissue. It results from the failure of the neuroectoderm to separate from the cutaneous ectoderm in the third to fifth week of gestation. The common locations are the lumbosacral and occipital regions. Sometimes it extends to spinal canal. In this paper we report a case of congenital spinal dermal sinus tract in the coccyx. Methods: A 21-month-old male child born after an uncomplicated full-term pregnancy was admitted to our institute with a midline dermal sinus and a cartilaginous protrusion in the coccygeal region. There were no signs of infection. Neurologic examination showed no functional deficit in both lower limbs. He was treated with complete excision of the tract and an underlying accessory cartilage. Results: The spinal dermal sinus tract was extended from the skin to the coccyx. The stalk was loosely attached to the accessory cartilage of coccyx. At that point, it was dissected from the accessory cartilage and resected. The accessory cartilage was also resected at the bone and cartilage junction. During the follow-up period of 6 months, the wound healed well without any complication nor recurrence. Conclusion: Congenital spinal dermal sinus tract is known as a form of spinal dysraphism. In order to prevent complications, timely surgical intervention including complete resection of sinus tract with correction of associated abnormalities is of utmost importance.

• Pediatric Infection and Vaccine
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• v.25 no.1
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• pp.45-49
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• 2018

Group B streptococcus (GBS) is the leading cause of neonatal morbidity and mortality. Late-onset GBS disease commonly manifests as occult bacteremia or meningitis. Approximately 50% of survivors of late-onset meningitis have long-term neurologic sequelae. Cerebrovascular complications are often associated with unfavorable clinical outcomes of GBS meningitis. There have been a few reports of cerebral infarction accompanied by GBS meningitis. We report a 29-day-old girl with severe, widespread cerebral infarction due to late-onset GBS meningitis. Isolated GBS strain from this patient was serotype III, ST-19. Currently, she has cortical blindness and significant developmental delay.

• Journal of Korean Neurosurgical Society
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• v.39 no.3
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• pp.188-191
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• 2006

Objective : The purpose of this study is to evaluate the efficacy and necessity of combined anterior approach [discectomy and fusion] and posterior approach[open-door laminoplasty] in the treatment of cervical spondylotic myelopathy. Methods : The authors reviewed 14 cases in whom combined anterior and posterior approach performed for cervical myelopathy due to simultaneous anterior and posterior pathology such as huge central disc herniation with narrow spinal canal between January 2000 and December 2002. Clinical symptoms were evaluated by Japanese Orthopaedic Association [JOA] score and then the cervical curvature, change of spinal canal to vertebral body[SC/VB] ratio and canal widening were measured and compared to the clinical symptoms. Results : The mean JOA score increased from $10.4{\pm}3.1$ preoperatively to $14.8{\pm}1.2$ at the final follow up with a mean recovery rate 66.4%. In all cases, there were not neurologic deterioration. Mild postoperative complications developed in two cases. One patient had a limitation of range of neck motion and the other one showed kyphotic change. Postoperative radiography showed an improvement of body to canal ratios [average $0.70{\pm}0.08$ before surgery to $1.05{\pm}0.12$ after surgery and mainte nance or recovery of cervical lordosis. Canal widening of antero-posterior diameter and dimension after operation is 6.8mm. $116.61mm^2$. Conclusion : Combined anterior and posterior procedure could be helpful in decompression of the spinal cord and good functional recovery in spondylotic myelopathy patients with combined anterior and posterior pathology such as huge disc herniation accompanying narrow spinal canal.