• Journal of Chest Surgery
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• 제21권3호
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• pp.541-546
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• 1988

A 34-year-old female was seen with Budd-Chiari syndrome caused by an obstruction of the inferior vena cava above the hepatic veins. Transatrial dilation or membranotomy was not available due to severe hourglass constriction of the inferior vena cava. Retrohepatic cavoatrial bypass with a low porosity woven Dacron graft[24mm in the diameter] was performed. For increasing the patency of venous graft, we used anticoagulation with warfarin. Postoperative course was uneventful except pleural effusion of the right luring.

• Journal of Yeungnam Medical Science
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• 제34권2호
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• pp.260-264
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• 2017

Klinefelter's syndrome is the most common congenital abnormality that causes primary hypogonadism. It is associated with diseases that predominantly affect women, such as systemic lupus erythematosus (SLE), and it can sometimes cause veno-occlusive disease. We experienced a case of Budd-Chiari syndrome (BCS) in a 33-year-old man with Klinefelter's syndrome presented with hematemesis and edema in both lower extremities. The clinical and laboratory findings were compatible with SLE, antiphospholipid syndrome, and BCS. To the best of our knowledge, this is the first case report to describe a simultaneous presentation of these four clinical syndromes in a single patient.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제12권2호
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• pp.235-239
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• 2009

저자들은 궤양성 대장염으로 진단되어 치료 중이던 15세 소아에서 발생한 간정맥 혈전증(Budd-Chiari 증후군) 1예를 경험하였기에 문헌고찰과 함께 보고한다. 간정맥 혈전증은 소아 궤양성 대장염의 매우 드문 혈관계 합병증으로 이환율과 사망률의 주요한 원인이 될 수 있으므로 진단과 치료에 보다 세심한 주의가 필요하다.

• 대한핵의학회지
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• 제22권1호
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• pp.47-53
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• 1988

Budd-Chiari syndrome (BCS) is a rare clinical entity characterized by post-sinusoidal portal hypertension caused by the obstruction to the hepatic vein outflow The diagnosis is suggested by hepatic scintigraphy and is usually confirmed by hepatic venography, inferior vena cavography and biopsy. The scintigraphic finding of BCS caused by the obstruction of main hepatic vein has been reported to consist typically of hypertrophy of the caudate lobe with increased radionuclide accumulation. Such a typical finding has been accounted for by the fact that the venous outflow from the caudate lobe is preserved when the main hepatic vein is obstructed. But usually, the hepatic venous outflow from the caudate lobe is also obstructed in BCS due to inferior vena caval obstruction. So hepatic scintigraphic findings of BCS due to inferior vena caval obstruction show different findings as compared with the BCS due to hepatic vein obstruction. We evaluate the hepatic scintigrams of the 13 cases of BCS due to inferior vena caval obstruction and review the literatures. The results are as follows : 1) We cannot observe the caudate lobe hypertrophy with increased uptake, which is known as a classic finding in BCS due to hepatic vein obstruction. 2) The most prominent hepatic scintigraphic findings of BCS are nonhomogenous uptake in the liver with extrahepatic uptake in the all cases. 3) We can see cold areas at the superior aspect of right hepatic lobe in 7 cases (54%). This is a useful finding suggesting BCS due to inferior vena caval obstruction.

• 대한핵의학회지
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• 제20권1호
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• pp.93-96
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• 1986

• Journal of Chest Surgery
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• 제26권12호
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• pp.950-954
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• 1993

Two patients with chronic Budd-Chiari syndrome resulting from segmental obstruction of the inferior vena cava underwent operation. There were 1 man and 1 woman. The obstructed segment was directly visualized by a transthoracic, transdiaphragmatic, retroperitoneal approach. In these two cases, severe segmental obstruction of the inferior vena cava was observed just above the right hepatic vein. These patients underwent successful retrohepatic cavoatrial bypass with a polytetrafloroethylene [PTFE] graft [ 16mm plain and 16mm ringed graft ]. There were no operative mortality and postoperative complication. These patients have been followed up for 6months and 36months without evidence of re-obstruction. When there is a severe stricture of the IVC with hepatic veins draining freely into the obstructed segment of the IVC, a dorsal cavoatrial bypass with a PTFE graft, preferably ringed, is the method of choice.

• Journal of Chest Surgery
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• 제43권5호
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• pp.525-528
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• 2010

선천성 하대정맥의 중단은 간울혈과 간변성을 유발하며, 비장비대와 복수와 복통, 하지부종이 동반된다. 49세 여자환자가 자궁근종과 월경과다, 그리고 철결핍성빈혈로 본원 산부인과에 입원하여 수술 전 복부컴퓨터단층촬영검사 및 복부 및 흉부 대정맥조영술을 시행하여 하대정맥의 중단의 소견이 관찰되었다. 환자는 우측 후측방 개흉술을 시행하여 흉강 내로 접근하였고, 횡격막을 절개하여 후복막 접근을 이용하여 하대정맥을 박리하여 16 mm PTFE graft를 이용하여 우회수술을 시행하였다. 수술 후 graft의 꺾임 현상이 관찰되어 혈관성형술 및 스텐트 삽입을 시행받았으며, 이후 환자는 특별한 문제 없이 회복되었다. 하대정맥의 선천성 중단이 관찰되는 환자에게서는 PTFE 인조혈관을 이용한 후방 대정맥-우심방 우회수술이 적절한 선택이며, 후측방 개흉술을 통한 복막뒤공간 접근법이 적절한 시야를 제공할 수 있을 것이라 생각된다.

• Journal of Chest Surgery
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• 제24권7호
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• pp.732-738
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• 1991

Membranous obstruction of the inferior vena cava[IVC] is a rare congenital anomaly that may present clinical features of Budd-Chiari syndrome caused by chronic obstruction of hepatic drain. We have experienced a case of IVC obstruction caused by hour-glass constriction and membrane in its center. Operative correction was accomplished using profound hypothermia [20%] and total circulatory arrest of 26 minutes. This technique permitted resection of membrane with direct vision and removal of thrombus of IVC and hepatic vein. After then constricted IVC was repaired with autologous pericardial patch. Total circulatory arrest was used intermittently for good visual field. Postoperative course was smooth and postoperative angiography showed unobstructed flow through the IVC in spite of slight constriction of cavoatrial junction and nearly complete disappearance of collateral vessels.

• 대한핵의학회지
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• 제19권1호
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• pp.145-148
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• 1985

A small number of pathologic entities such as Budd-Chiari Syndrome, cirrhosis, focal nodular hyperplasia, and superior and inferior vena cava obstruction has been reported to result in focal areas of increased uptake of radiocolloid on the hepatoscintigram. We recently studied a patient with focal accumulation of $^{99m}Tc-phytate$ at the inferior aspect of the liver, at the junction of the right and left lobe. The superior vena cava scintiangiogram was taken for the evaluation of the superior vena cava obstruction and collateral circulations. As a result of superior vena caval obstruction a considerable amount of blood flowed to the liver through the anterior parietal and periumblical venous channels. A certain fraction of radiocolloid delivered by the rete mirabile perfused to a localized area of the liver. This would explain the hot spot around the porta hepatis in this case.

• Journal of Chest Surgery
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• 제10권2호
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• pp.315-326
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• 1977

From 1958 to October 1977, 294 cases of acquired heart disease were operated. There were 68 cases of pericardium, 3 trauma, 2 foreign body, one cardiac thrombus, 3 atrial myxoma, 2 left atrial and 1 right atrial, 2 Budd-Chiari syndrome, and 214 valvular heart disease. Out of 214 cases of valve operation 73 valves were replaced in 64 patients. Male to female ratio was 1.46: 1. The youngest age was 14 years in male and 18 in female. The oldest was 54 years in male and 52 in female. Fifty-five cases of single valve were replaced, consisting of 47 mitral and 8 aortic valves. There were 9 double valve replacement cases which consist of 7 mitral and aortic and 2 mitral .and tricuspid valves. Six varieties of prosthetic valves, 3 ball and 3 disc types and 3 kinds of xenograft tissue valves were utilized. Beall, BjSrk-Shiley and Starr-Edwards prosthetic valves and Hancock valves were used mainly. For single valve 34. 5% and for double valve replacement 44% mortality were noted. There were 23 operative deaths out of 64 patients, over all mortality rate of 36.9%. Mortality for mitral valve replacement was 29.5%. But in recent 12 consecutive cases one death occurred, showing 8.3% mortality. In earlier days thrombocyte anti-adhesive drug dipyramidole-persantin-aspirin and/or SP 54 were adminstered. But in recent cases after heparinization, coumadin and Persantin were prescribed routinely.