• Journal of Yeungnam Medical Science
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• v.32 no.1
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• pp.47-49
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• 2015

Sorafenib is indicated for the treatment of advanced hepatocellular carcinoma (HCC), but although rare, tumor lysis syndrome (TLS) can be fatal in HCC patients with a large tumor burden. The authors describe the case of a 55-year-old hepatitis B carrier who visited our clinic with progressive dyspnea for 3 weeks. Chest and abdominal computed tomography revealed a huge HCC in the left lobe of the liver with invasion of the inferior vena cava, right atrium, and pulmonary arteries. After 8 days of sorafenib administration, TLS was diagnosed based on the characteristic findings of hyperuricemia, hyperkalemia, and acute kidney injury with massive tumor necrosis by follow-up imaging. Despite discontinuation of sorafenib and supportive care, the patient's clinical course rapidly deteriorated. The authors describe a rare but fatal complication that occurred soon after sorafenib initiation for HCC. Careful follow-up is required after commencing sorafenib therapy for the early diagnosis and management of TLS.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.331-336
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• 1983

Cor triatriatum is a rare congenital malformation of the heart, in which a septum stretches in a transverse or oblique plane through the left atrium, thus separating it into two compartments. The upper one connects with the pulmonary veins, and the lower one connects with the left ventricle. Due to the rarity of, and great difficulty in-diagnosing, cor trlatrlatum, data On the surgery of this disease are of necessity very limited and so accurate pre-operative diagnosis was very difficult to make. `We experienced a case of the cyanotic congenital heart disease which was diagnosed as a large atrlal septal defect with streaming venous blood from inferior vena cava to left atrium through atrial septal defect in August, 1982. We found that there were transverse septum in the left atrium through atrlal septal defect, the pulmonary venous drainage were located in the upper chamber of the left atrium, and the lower chamber was connected with the left atrial appendage, mitral valve and `left ventricle. But our case had not any opening in this transverse septum and the right atrium was connected with the upper chamber of the left atrium through the upper part of the atrlal septal defect, and was communicated with the lower chamber of the left atrium through the lower part of the atrlal septal defect. We excised the transverse septum and repair this atrial septal defect with Woven Dacron patch accompanying with the drainage of coronary sinus to right atrium. The post-operative course was not eventful and he was discharged with good result on the post-operative 8th day, and has been in good .condition up to now for longer than 10 months.

• Journal of Trauma and Injury
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• v.27 no.3
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• pp.89-93
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• 2014

Bile leaks are complications that are much more frequent after a high-grade liver injury than after a low-grade liver injury. In this report, we describe the management of bile leaks that were encountered after angiographic embolization in a 27-year-old man with a high-grade blunt liver injury. He had undergone an abdominal irrigation and drainage with a laparotomy on post-injury day (PID) 16 due to bile peritonitis and continuous bile leaks from percutaneous abdominal drainage. He required three percutaneous drainage procedures for a biloma and liver abscesses in hepatic segments 4, 5 and 8, as well as endoscopic retrograde cholangiopancreatography with biliary stent placement into the intrahepatic biloma via the common bile duct. We detected communication between the biloma and the bilateral intrahepatic duct by using a tubogram. Follow-up abdominal computed tomography on PID 47 showed partial thrombosis of the inferior vena cava at the suprahepatic level, and the patient received anticoagulation therapy with low molecular weight heparin and rivaroxaban. As symptomatic improvement was achieved by using conservative management, the percutaneous drains were removed and the patient was discharged on PID 82.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.22 no.1
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• pp.80-85
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• 2019

Purpose: Abernethy malformation is a rare condition, which was first described in 1793 as a congenital extrahepatic porto-systemic shunt (CEPS) directing splanchnic blood flow into the inferior vena cava. Eighty cases have been published so far that reported CEPS, while in Korea, very few cases have been reported. Through this study, we present 6 cases of patients diagnosed with CEPS at Samsung Medical Center and compare these with other such cases published in France and China. Methods: We reviewed clinical, laboratory, and imaging data of 6 children with CEPS in our pediatric clinic between 2004 and 2017. Results: A total of 6 children with CEPS was included in this study, namely, one with type 1a, two with type 1b, and three with type 2 CEPS. The most common presenting symptom was gastrointestinal bleeding (50.0%). Therapeutic interventions included shunting vessel ligation (16.7%) in type 2 CEPS and liver transplantation (16.7%) in type 2 CEPS patient with suddenly developed hepatic encephalopathy. Conclusion: There is no consensus guideline for the optimal management of patients with CEPS. Large-sample studies regarding CEPS are needed to evaluate the characteristics of patients with CEPS and determine the treatment guideline for CEPS.

• Journal of Chest Surgery
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• v.30 no.11
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• pp.1128-1131
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• 1997

Injuries to versa cave continue to be associated with a high mortality. Essentials to successful treatment are immediate recognition of the injury and prompt control of the hemorrhage. We have experienced one case of inferior versa java perforation by a chest rainage tube in the patient with post-operative chronic empyema thoracic. The patient was 38-year old male who was taken RLL lobectomy after 6 cycle of chemotherapy due to small cell carcinoma in the RLL & suffered from post-operative chronic empyema thoracis at D hospital. He moved to our hospital for further evaluation with accidental removal of chest drainge tube. We inserted closed drainage tube and dark blood gushed out abruptly just after insertion of the drainage tube. CTscan, MRI, and angiogram were performed and showed the perforation of IVC just below RA. The IVC was repaired using simple interrupted 4-0 Prolene suture through right posterolateral thoracotomy. The patient recovered without event and doing well until now.

• Journal of Yeungnam Medical Science
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• v.33 no.2
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• pp.166-169
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• 2016

Afferent loop obstruction following gastrectomy is a rare but fatal complication. Clinical features of afferent loop obstruction are mainly gastrointestinal symptoms. A 56-year-old female underwent radical total gastrectomy with Roux-en-Y esophagojejunostomy for treatment of advanced gastric cancer. After fourteen months postoperatively, she showed gradual development of edema of both legs. Computed tomography (CT) scan showed disease progression at the jejunojejunostomy site and consequent dilated afferent loop, which resulted in inferior vena cava (IVC) compression. A drainage catheter was placed percutaneously into the afferent loop through the intrahepatic duct and an IVC filter was placed at the suprarenal IVC, and self-expanding metal stents were inserted into bilateral common iliac veins. With these procedures, sympotms related with afferent loop obstruction and deep vein thrombosis were improved dramatically. The follow-up abdominal CT scan was taken 3 weeks later and revealed the completely decompressed afferent loop and improved IVC patency. Surgical treatment should be considered as the first choice for afferent loop obstruction; however, because it is more immediate and less invasive, non-surgical modalities, such as percutaneous catheter drainage or stent placement, can be effective alternatives for inoperable cases or risky patients who have severe medical comorbidities.

• Neonatal Medicine
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• v.18 no.2
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• pp.404-408
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• 2011

Scimitar syndrome (SS) is a rare congenital anomaly characterized by hypoplasia of the right lung and partially anomalous pulmonary venous drainage to the inferior vena cava. The term scimitar derives from the shadow created by the anomalous pulmonary vein on chest X-ray that closely resembles that of a curved Turkish sword. It rarely presents as an isolated abnormality. Various cardiac and non-cardiac anomalies have been association with SS, such as right lung hypoplasia, dextroposition of the heart, hypoplasia of the right pulmonary artery, systemic arterial blood supply to the right lower lung from the infra-diaphragmatic aorta, and a secundum type of atrial septal defect. However, an imperforate anus has not been reported previously in association with SS. We describe the first case of infantile scimitar syndrome accompanied with an imperforate anus in a newborn who presented with tachypnea and right pulmonary atelectasis.

• Neonatal Medicine
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• v.25 no.1
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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 Chest Surgery
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• v.46 no.1
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• pp.41-48
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• 2013

Background: We present our 12-year experience of pulmonary thromboendarterectomy in patients with chronic thromboembolic pulmonary hypertension. Materials and Methods: Between January 1999 and March 2011, 16 patients underwent pulmonary thromboendarterectomy. Eleven patients (69%) were classified as functional class III or IV based on the New York Heart Association (NYHA) classification. Seven patients had a history of inferior vena cava filter insertion, and 5 patients showed coagulation disorders. Pulmonary thromboendarterectomy was performed during total circulatory arrest with deep hypothermia in 14 patients. Results: In-hospital mortality and late death occurred in 2 patients (12.5%) and 1 patient (6.3%), respectively. Extracorporeal membrane oxygenation support was required in 4 patients who developed severe hypoxemia after surgery. Thirteen of the 14 survivors have been followed up for 54 months (range, 2 to 141 months). The pulmonary arterial systolic pressure and cardiothoracic ratio on chest radiography was significantly decreased after surgery ($76{\pm}26$ mmHg vs. $41{\pm}17$ mmHg, p=0.001; $55%{\pm}8%$ vs. $48%{\pm}3%$, p=0.003). Tricuspid regurgitation was reduced from $2.1{\pm}1.1$ to $0.7{\pm}0.6$ (p=0.007), and the NYHA functional class was also improved to I or II in 13 patients (81%). These symptomatic and hemodynamic improvements maintained during the late follow-up period. Conclusion: Pulmonary thromboendarterectomy for chronic thromboembolic pulmonary hypertension shows good clinical outcomes with acceptable early and long term mortality.

• The Korean Journal of Physiology
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• v.2 no.1
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• pp.9-15
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• 1968

Changes in gastrointestinal tissue blood volume induced by variations of venous pressure between 6 and 40 mmHg were studied in 32 rabbits. Venous pressure lowering was produced by withdrawal of appropriate volume of blood and venous pressure elevation was obtained by partial occlusion of intra-thoracic vena cava inferior. Estimation of regional tissue blood volume was performed by means of regional distribution of injected $Cr^{51}-labeled$ red blood cells. The following results were obtained. 1. At the normal control venous pressure value of 18 mmHg, spleen showed the highest value of tissue blood volume expressed on weight basis, namely, $111{\mu}l/gm$, Liver tissue blood volume was $95\;{\mu}l/gm$, small intestine 24 and stomach $21\;{\mu}l/gm$, respectively. 2. Linear relationships were observed between venous pressure change and gastrointestinal tissue blood volume. The coefficients of correlation were: in spleen r=0.723; in liver r=0.791; in stomach r=0.704, respectively. In small intestine the relationship was less clear and r=0.358. Tissue blood volume of extrabdominal tissue, such as M. gastrocnemius was not influenced by venous pressure change. 3. The highest change in tissue blood volume expressed on weight basis was observed in spleen. The liver tissue showed the next highest change. Change in total tissue blood volume, however, was greatest in liver and next greatest in small intestine. This was interpreted by the fact that total weight of these two organs was much greater than that of spleen. 4. The mechanism that the change in tissue blood volume lies in the venous system which has a great compliance was discussed.