• Neonatal Medicine
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• 제25권1호
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• pp.49-52
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• 2018

Arterial tortuosity syndrome (ATS) is a very rare autosomal recessive connective tissue disease characterized by generalized elongation and tortuosity of the medium- to large-sized arteries, and other systemic connective tissue manifestations. To date, this disease entity has not been reported in Korea. We report a case of ATS diagnosed in a neonate who presented with severe elongation and tortuosity of the aorta and its major branches, as well as the intracranial arteries. Additionally, the patient presented with a tortuous dilatation of the inferior vena cava, an aneurysmal dilatation of the extra-hepatic bile ducts, and an inguinal and sliding hiatal hernia. The diagnosis was confirmed using DNA sequencing analysis, and the patient demonstrated a compound heterozygosity for two novel mutations (c.738delG [p.Gln247Serfs*33] and c.362T>C [p.Ile121Thr]) in exon 2 of the SLC2A10 gene. Genetic analysis also confirmed that both parents were heterozygous carriers of the responsible mutations. Owing to such clinical manifestations, ATS is often misdiagnosed as other connective tissue diseases including Loeys-Dietz syndrome, Marfan syndrome, and Ehlers-Danlos syndrome. In patients presenting with a high index of suspicion, thorough clinical evaluation and screening for ATS including computed tomography or magnetic resonance angiography and target gene analysis are necessary for early diagnosis and management.

• Journal of Korean Neurosurgical Society
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• 제48권2호
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• pp.139-144
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• 2010

Objective : We report our surgical experience in the treatment of 16 consecutive patients with benign craniovertebral junction (CVJ) tumor, observed from 2003 to 2008 at our department. Methods : We had treated 6 foramen magnum meningiomas, 6 cervicomedullary hemangioblastomas, 1 accessory nerve schwannoma, 1 hypoglossal nerve schwannoma, 1 C2 root schwannoma, and 1 cavernous hemangioma. Clinical results were evaluated by Karnofsky Performance Scale (KPS) and all patients underwent preoperative neuroradiological evaluation with computed tomography (CT) and magnetic resonance image (MRI). Angiography was performed in 15 patients and preoperative embolization was done in 2 patients. Results : Five far-lateral, 1 supracondylar and 10 midline suboccipital approaches were performed. Gross total removal was achieved in 15 cases (94%) and subtotal removal in 1 patient (6%). None of the patients required occipitocervical fusion. Radiological follow-up showed no recurrence in cases totally removed. Postoperative decrease of KPS scores was recorded in only 1 patient. The treatment of cervicomedullary solid hemangioblastoma presented particular issues : by preoperative embolization, we removed tumor totally without an excessive bleeding or brainstem injury. In one of foramen magnum meningioma, we carried out subtotal removal due to hard tumor consistency and encasement of neurovascular structures. Conclusion : The choice of surgical approaches and the extent of bone resection should be defined according to the location and size of individual tumors. Moreover, we emphasize that preoperative neuroradiological evaluations on presumptive tumor type could be helpful to the surgeon in tailoring the technique and providing the required exposure for different lesions, without unnecessary surgical steps.

• Journal of Korean Neurosurgical Society
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• 제30권1호
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• pp.99-104
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• 2001

We present two cases of deep cerebral venous thrombosis(DCVT) with the totally occluded straight sinus. A 42-year-old female received with altered mentality . She has taken antihistamine for six years to treat the paranasal sinusitis. Another 34-year-old female who used the oral contraceptive for 11 months presented with acute behavior change . Both of these patients were diagnosed by computed tomography(CT), magnetic resonance(MR) imaging, and cerebral angiography. They were fully recovered with systemic urokinase thrombolysis followed by heparin therapy. We report that the intravenous thrombolysis was potentially effective management strategy in our cases of DCVT with the totally occluded straight sinus.

• Journal of Korean Neurosurgical Society
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• 제56권2호
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• pp.86-90
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• 2014

Objective : The spot sign is related with the risk of hematoma expansion in spontaneous intracerebral hemorrhage (ICH). However, not all spot sign positive patients undergo hematoma expansion. Thus, the present study investigates the specific factors enhancing the spot sign positivity in predicting hematoma expansion. Methods : We retrospectively studied 316 consecutive patients who presented between March 2009 to March 2011 with primary ICH and whose initial computed tomography brain angiography (CTA) was performed at our Emergency Department. Of these patients, 47 primary ICH patients presented spot signs in their CTA. We classified these 47 patients into two groups based on the presence of hematoma expansion then analyzed them with the following factors : gender, age, initial systolic blood pressure, history of anti-platelet therapy, volume and location of hematoma, time interval from symptom onset to initial CTA, spot sign number, axial dimension, and Hounsfield Unit (HU) of spot signs. Results : Of the 47 spot sign positive patients, hematoma expansion occurred in 26 patients (55.3%) while the remaining 21 (44.7%) showed no expansion. The time intervals from symptom onset to initial CTA were $2.42{\pm}1.24$ hours and $3.69{\pm}2.57$ hours for expansion and no expansion, respectively (p=0.031). The HU of spot signs were $192.12{\pm}45.97$ and $151.10{\pm}25.14$ for expansion and no expansion, respectively (p=0.001). Conclusions : The conditions of shorter time from symptom onset to initial CTA and higher HU of spot signs are the emphasizing factors for predicting hematoma expansion in spot sign positive patients.

• Journal of Chest Surgery
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• 제41권5호
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• pp.651-654
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• 2008

외상에 의해 발생하는 쇄골하동맥의 가성동맥류는 매우 드물다. 10년전 교통사고를 당한 49세 여자환자는 외부병원에서 직장검진시 우연히 발견한 종격동 종양을 진단받고 본원에서 진단 및 치료 목적으로 개흉술을 시행 받았다. 개흉술하에 종괴의 일부분을 박리하던 중 대량 출혈 소견 보여 출혈 부위를 봉합한 후 혈관촬영을 추가로 시행하였다. 좌측 쇄골하동맥 가성동맥류 진단하에 재수술을 시행하여 동맥류를 절제하고 단단문합하였다. 환자는 술 후 특별한 문제없이 술 후 11일째 퇴원하였다.

• Journal of Yeungnam Medical Science
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• 제5권2호
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• pp.205-211
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• 1988

본 저자들은 44세의 여자환자로 3년전부터 발생한 경련성 발작후 의식소실로 간질로 진단 받은 후 항경련제를 투여받았으나 효과가 없었던, 금식검사에서 혈청 insulin, C-peptide, glucose, 24시간 뇨 c-peptide 및 선택적 췌장동맥 촬영술로 인술린종을 진단, 수술로 종물을 적출후 경련성 발작이 사라진 인술린종 1례를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Korean Neurosurgical Society
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• 제30권3호
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• pp.389-394
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• 2001

경막정맥동 혈전증은 다양한 원인을 가진 비교적 드문 질환으로 알려져왔으며 임상양상 및 발현형태가 매우 다양하여 임상적 진단이 쉽지 않았다. 그러나 최근에는 여러 영상진단 방법, 특히 자기공명영상 혈관 촬영등을 통해 조기 진단이 가능하게되었다. 치료 방법을 결정할 때, 환자의 임상증상과 정맥순환계의 개인간 차이가 매우 중요한 인자중 하나이다. 저자들은 보존적 치료로 좋은 결과를 얻은 2례의 경막정맥동 환자의 특성을 문헌고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제43권4호
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• pp.458-461
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• 2010

53세 된 여자 환자가 우측 대동맥 궁, Kommerell 게실 및 비정상 좌측 쇄골하동맥 등의 병변으로 내원하였으며 증상은 없었다. 6 mm ringed Gore-Tex 인조혈관을 이용하여 비정상 좌측 쇄골하동맥과 좌측 총경동맥을 연결하여 우회하고, 대동맥 궁의 Kommerell 게실을 막고자 stent graft를 삽입하는 hybrid 수술을 시행 하였다. 수술 직후 시행한 혈관 조영술과 수술 후 첫날 시행한 전산화 단층 촬영상 성공적인 대동맥류 차단 및 좌측 쇄골하동맥의 우회소견을 보여주었다. 수술 및 시술에 동반된 합병증은 없었다.

• Journal of Chest Surgery
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• 제33권1호
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• pp.7-19
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• 2000

Background :To assess the accuracy of Electron-Beam Tomography(EBT) in following evaluation of the pulmonary vascular system after a shunt operation in the cyanotic con-genital heart disease with pulmonary stenosis or pulmonary atresia. Material and Method : Sixteen patients(M:F=11:5) who received Blalock-Taussig(n=8) bidirectional cavo-pulmonary shunt(n=10) and unifocalization (n=2) were ncluded in the study. We evaluated the patency of the shunt the morphology of intrapericardial and hilar pulmonary arteries(PA) peripheral pulmonary vascularity by background lung attenuation and the abundance of arterial & venous collateral. Angiography(n=12) and echocardiography(n=20) were used as the gold standard for the comparison of EBT results. Result: EBT was consistent with angiogram/ echo in 100% of the evaluation for the patency of the shunt and in 12(by angiogram 100%) and 19(by echo 95%) for the detection the hypoplasia stenosis or interruption of central PA In measuring of PA EBT and angiogram corrlated(r=0.91) better than EBT-echo(r=0.88) or echo-angiogram(r=0.72) Abundant systemic arterial collateral were noted in 4 and venous collateral in 3 cases. In evaluating the peripheral pulmonary vascularity the homogenous and normal-ranged lung attenuation(m=6) decreased but homo-genous attenuation(n=1) segment-by-sgment heterogeneous attenuation(n=3) homogenous but asymmetrical attenuation(n=3) segment-by-segment heterogeneous attenuation(n=3) homogenous but asymmetrical attenuation(n=3) and venous congestion(n=2) were observed nd 12 of them were compatible with the blood flow pattern revealed by cardiac catheterization. Conclusion: EBT was accurate in the integrated evaluation of the pulmonary vascular system after the shunt including the patency of the shunt operaion the morphology and dimension of the central and hilar PAs and the loco-regional pulmonary flow in the lung parenchyma. It suggests the useful information about the need of secondary shunt operation the proper timing time for total repair and the need of interventional procedure prior to total repair.

• Tuberculosis and Respiratory Diseases
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• 제79권3호
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• pp.179-183
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• 2016

A 59-year-old man presented with acute dyspnea following sudden productive cough and expectoration of a full cup of "blood-tinged" sputum. He had been diagnosed with hepatitis B virus-related hepatocellular carcinoma and had received transarterial chemoembolization 5 years ago for a 20-cm hepatic mass; he denied any history of hematemesis and the last esophagogastroduodenoscopy from a year ago showed absence of varix. Chest computed tomography (CT) with angiography showed new appearance of right basal lung consolidation but no bleeding focus. Despite the use of systemic antibiotics, the patient developed respiratory failure on day 7 of hospitalization. After intubation, a massive amount of brown sputum with anchovy-paste-like consistency was suctioned via the endotracheal tube. Bronchoscopic toileting was performed and the patient was extubated. In the ward, he continued to expectorate the brown sputum. On day 25 of hospitalization, a repeat CT scan showed simultaneous disappearance of the pneumonic consolidation and the necrotic fluid within the hepatic mass, suggesting the presence of a fistula. He has continued to receive systemic antibiotics, sorafenib, and entecavir, and follow up by respiratory and hepato-oncology specialists.