Congenital pseudarthrosis of the clavicle is a rare condition, which rarely produces functional disabilities except for cosmetic problems. Surgical treatment involves autogenous iliac bone grafts and internal fixation. Few studies have reported long-term results of surgical treatment or morphological changes of the clavicle. We report a patient with a congenital pseudarthrosis of the clavicle, who demonstrated a near normal radiographic appearance of the clavicle and an excellent result 22 years after the operation performed at 4 years of age.
Clavicle ; Cosmetics ; Follow-Up Studies ; Humans ; Pseudarthrosis ; Transplants

Aortoenteric fistula is a rare but potentially fatal condition causing massive gastrointestinal bleeding. In particular, double primary aortoenteric fistulae are vanishingly rare. We encountered a 75-year-old male patient suffering from abdominal pain, hematochezia, hematemesis, and hypotension. His computed tomography images showed abdominal aortic aneurysm and suspected aortoenteric fistulae. During surgery, we found two primary aortoenteric fistulae. The one fistula was detected between the abdominal aorta and the third portion of the duodenum, and the other fistula was detected between the abdominal aorta and the sigmoid colon. We conducted the closure of the fistulae, the exclusion of the aneurysm, and axillo-bifemoral bypass with a polytetrafluoroethylene graft. The patient was discharged with no complications on the 21st postoperative day.
Abdominal Pain ; Aged ; Aneurysm ; Aorta ; Aorta, Abdominal ; Aortic Aneurysm, Abdominal ; Colon, Sigmoid ; Duodenum ; Fistula ; Gastrointestinal Hemorrhage ; Hematemesis ; Hemorrhage ; Humans ; Hypotension ; Male ; Polytetrafluoroethylene ; Stress, Psychological ; Transplants

A 12-year-old female presented with the abnormal findings on the chest PA. The chest CT revealed a retrosternal defect of the diaphragm and a fatty opacity in the pleural cavity, resulting in a diagnosis of Morgagni hernia. It was decided to undergo a laparoscopic surgery. The retrosternal defect of the diaphragm measuring 3.5 cm in diameter was found, through which a portion of the greater omentum and the fatty tissue connected with the falciform ligament were herniated into the pleural cavity. The greater omentum was pushed back into the peritoneal cavity and the fatty tissue connected with falciform ligament was excised. The mediastinal pleura was plicated and the defect of the diaphragm was repaired primarily. Immediately after the operation, the patient developed a right pneumothorax for which a chest tube was inserted. She was discharged at the post-operative third day without any further complications.
Adipose Tissue ; Chest Tubes ; Child ; Diaphragm ; Female ; Hernia ; Hernia, Diaphragmatic ; Humans ; Laparoscopy ; Ligaments ; Omentum ; Peritoneal Cavity ; Pleura ; Pleural Cavity ; Pneumothorax ; Thorax

BACKGROUND: Muscle is a target of immunological injury in several muscle diseases, such as idiopathic inflammatory myopathy. However, it is also a target for gene therapy. Therefore, it is important to understand the immunological capabilities of muscle cells. To assess as to whether muscle cells are actively involved in the inflamed muscle tissue, a human skeletal muscle cell line was tested for the expression of several cytokines and chemokine at the mRNA level. METHODS: A human skeletal muscle cell line (SKM14) had been developed by a retroviral vector encoding v-myc transfection into a 12-week-old human fetal skeletal muscle tissue characterized by the immunostaining of several musclespecific markers. Human skeletal myoblasts of this cell line were tested for their capacity to express different cytokines (IL-1beta, -6, -10, -12, -15, and TNF-alpha) and chemokine (IL-8) mRNA levels at the basal state and in the presence of TNF-alpha(10 ng/ml). RESULTS: The SKM14 cell line was confirmed to be able to express various cytokines constitutively (IL-6, -8, -12, -15, and TNF-alpha) and in the presence of TNF-alpha(IL-1beta, -6, -8, -10, -12, -15, and TNF-alpha). CONCLUSIONS: Our results suggest that muscle cells may play a role as immunocompetent cells.
Cell Line* ; Cytokines* ; Genetic Therapy ; Humans* ; Muscle Cells ; Muscle, Skeletal* ; Myoblasts ; Myoblasts, Skeletal ; Myositis ; RNA, Messenger* ; Transfection ; Zidovudine

BACKGROUND: The inferior vena cava filter (IVCF) is very effective for preventing pulmonary embolism in patients who cannot undergo anticoagulation therapy. However, if a filter is placed in the body permanently, it may lead to other complications. METHODS: A retrospective study was performed of 159 patients who underwent retrievable Cook Celect IVCF implantation between January 2007 and April 2015 at a single center. Baseline characteristics, indications, and complications caused by the filter were investigated. RESULTS: The most common underlying disease of patients receiving the filter was cancer (24.3%). Venous thrombolysis or thrombectomy was the most common indication for IVCF insertion in this study (47.2%). The most common complication was inferior vena cava penetration, the risk of which increased the longer the filter remained in the body (p=0.032, Exp(B)=1.004). CONCLUSION: If the patient is able to retry anticoagulation therapy and the filter is no longer needed, the filter should be removed, even if a long time has elapsed since implantation. If the filter cannot be removed, it is recommended that follow-up computed tomography be performed regularly to monitor the progress of venous thromboembolisms as well as any filter-related complications.
Follow-Up Studies ; Humans ; Pulmonary Embolism ; Retrospective Studies ; Thrombectomy ; Thromboembolism ; Vena Cava Filters* ; Vena Cava, Inferior* ; Venous Thrombosis

Background: When an arteriovenous fistula (AVF) is created using the basilic or deep cephalic vein, it is additionally necessary to transfer the vessels to a position where needling is easy; however, many patients develop wound-related postsurgical complications due to the long surgical wounds resulting from conventional superficialization of a deep AVF or basilic vein transposition. Thus, to address this problem, we performed videoscopic surgery with small surgical incisions. Methods: Data from 16 patients who underwent additional videoscopic radiocephalic superficialization, brachiocephalic superficialization, and brachiobasilic transposition after AVF formation at our institution in 2018 were retrospectively reviewed. Results: Needling was successful in all patients. No wound-related complications occurred. The mean vessel size and blood flow of the AVF just before the first needling were 0.73±0.16 mm and 1,516.25±791.26 mL/min, respectively. The mean vessel depth after surgery was 0.26±0.10 cm. Percutaneous angioplasty was additionally performed in 25% of the patients. Primary patency was observed in 100% of patients during the follow-up period (262.44±73.49 days). Conclusion Videoscopic surgery for AVF dramatically reduced the incidence of postoperative complications without interrupting patency; moreover, such procedures may increase the use of native vessels for vascular access. In addition, dissection using a videoscope compared to blind dissection using only a skip incision dramatically increased the success rate of displacement by reducing damage to the dissected vessels.

BACKGROUND AND OBJECTIVES: The objective of this study was to analyze cardiovascular risk factors in adults with congenital heart disease (ACHD). SUBJECTS AND METHODS: The subjects for this study comprised 135 patients, aged 18 years and above, who visited the ACHD clinic at the Samsung Medical Center and 135 adults with a structurally normal heart who were randomly selected from the Center for Health Promotion during the same period. For the analysis, the ACHD group was further divided into an ACHD group that underwent correction by cardiac surgery and a cyanotic group. RESULTS: The mean (standard diviation) age (years) of patients in the surgically corrected group was 48.4 (10.9) years, while that of patients in the cyanotic group was 43.1 (9.0) years and that of patients in the control group was 47.1 (10.3) years (p=0.042). The adjusted odds ratios (ORs) for past smoking, hypertension, diabetes mellitus, hypercholesterolemia, obesity, and metabolic syndrome were significantly higher in the surgically corrected patients than in the controls. However, the ORs for all variables excluding past smoking were significantly lower in the cyanotic group compared with the control group. After adjustment for age, gender, smoking, alcohol use, and exercise, the ORs for metabolic syndrome were 0.46 (0.35-0.57, p<0.001) and 1.48 (1.14-1.92, p=0.003) in the cyanotic and surgically corrected groups, respectively. CONCLUSION: Cardiovascular risk factors need to be considered in surgically corrected ACHD patients as well as in adults with a structurally normal heart. A further study with a long-term follow-up is needed for developing guidelines for prevention.
Adult* ; Diabetes Mellitus ; Follow-Up Studies ; Health Promotion ; Heart ; Heart Defects, Congenital* ; Humans ; Hypercholesterolemia ; Hypertension ; Metabolic Syndrome X ; Obesity ; Odds Ratio ; Risk Factors* ; Smoke ; Smoking ; Thoracic Surgery

BACKGROUND: The standard operative treatment of primary spontaneous pneumothorax (PSP) is thoracoscopic wedge resection, but necessity of pleurodesis still remains controversial. Nevertheless, pleural procedure after wedge resection such as pleurodesis has been performed in some patients who need an extremely low recurrence rate. MATERIALS AND METHODS: From January 2000 to July 2010, 207 patients who had undergone thoracoscopic wedge resection and pleurodesis were enrolled in this study. All patients were divided into two groups according to the methods of pleurodesis; apical parietal pleurectomy (group A) and pleural abrasion (group B). The recurrence after surgery had been checked by reviewing medical record through follow-up in ambulatory care clinic or calling to the patients, directly until January 2011. RESULTS: Of the 207 patients, the recurrence rate of group A and B was 9.1% and 12.8%, respectively and there was a significant difference (p=0.01, Cox's proportional hazard model). There was no significant difference in age, gender, smoking status, and body mass index between two groups. CONCLUSION: This study suggests that the risk of recurrence after surgery in PSP is significantly low in patients who underwent thoracoscopic wedge resection with parietal pleurectomy than pleural abrasion.
Ambulatory Care ; Body Mass Index ; Follow-Up Studies ; Humans ; Medical Records ; Pleurodesis ; Pneumothorax ; Recurrence ; Smoke ; Smoking ; Thoracoscopy

BACKGROUND: The tetralogy of Fallot (TOF) with pulmonary atresia (PA) and a ductus-dependent pulmonary circulation (no major aorto-pulmonary collateral arteries (MAPCAs)) has been treated with staged repair or primary repair depending on the preference of surgeons or institutions. We evaluated the 19-year outcome of staged repair for this anomaly to find out whether our surgical strategy should be changed. MATERIALS AND METHODS: Forty-four patients with TOF/PA with patent ductus arteriosus (PDA) who underwent staged repair from June 1991 to October 2010 were included in this retrospective study. The patients with MAPCAs were excluded. The average age at the first palliative shunt surgery was 40.8+/-67.5 days (range: 0~332 days). Thirty-one patients (31/44, 70%) were neonates. The average weight was 3.5+/-1.6 kg (range: 1.6~8.7 kg). A modified Blalock-Taussig (BT) shunt was performed in 38 patients, classic BT shunt in 4 patients, and central shunt in 2 patients. Six patients required concomitant procedures: pulmonary artery angioplasty was performed in 4 patients, pulmonary artery reconstruction in one patient, and re-implantation of the left pulmonary artery to the main pulmonary artery in one patient. Four patients required a second shunt operation before the definitive repair was performed. Thirty-three patients underwent definitive repair at 24.2+/-13.3 months (range: 7.3~68 months) after the first palliative operation. The average age at the time of definitive repair was 25.4+/-13.5 months (range: 7.6~68.6 months) and their average weight was 11.0+/-2.1 kg. For definitive repair, 3 types of right ventricular outflow procedures were used: extra-cardiac conduit was performed in 30 patients, trans-annular patch in 2 patients, and REV operation in 1 patient. One patient was lost to follow-up after hospital discharge. The mean follow-up duration for the rest of the patients was 72+/-37 months (range: 4~160 months). RESULTS: Ten patients (10/44, 22.7%) died before the definitive repair was performed. Four of them died during hospitalization after the shunt operation. Six deaths were thought to be shunt-related. The average time of shunt-related deaths after shunt procedures was 8.7 months (range: 2 days~25.3 months). There was no operative mortality after the definitive repair, but one patient died from dilated cardiomyopathy caused by myocarditis 8 years and 3 months after the definitive repair. Five-year and 10-year survival rates after the first palliative operation were 76.8% and 69.1%, respectively. CONCLUSION: There was a high overall mortality rate in staged repair for the patients with TOF/PA with PDA. Majority of deaths occurred before the definitive repair was performed. Therefore, primary repair or early second stage definitive repair should be considered to enhance the survival rate for patients with TOF/PA with PDA.
Angioplasty ; Arteries ; Cardiomyopathy, Dilated ; Ductus Arteriosus, Patent ; Follow-Up Studies ; Hospitalization ; Humans ; Infant, Newborn ; Lost to Follow-Up ; Myocarditis ; Pulmonary Artery ; Pulmonary Atresia ; Pulmonary Circulation ; Retrospective Studies ; Survival Rate ; Tetralogy of Fallot

Scleroderma is a connective tissue disorder characterized by fibrosis of the skin, vessels and internal organs (gastrointestinal tract, lung, heart, and kidney etc.). Renal involvement in scleroderma has a clinical manifestation from subnephrotic proteinuria to renal crisis accompanied by hypertension, and shows a typical histology of intimal proliferation and fibrinoid necrosis of blood vessel without primary glomerular pathology. We experienced a case of nephrotic syndrome in a 31-old-female patient with sclerodema. But the patient has not showed a clinical feuture of hypertension or renal crisis, moreover, renal biopsy of the patient showed global sclerosis and crescent formation in the glomeruli without vessel involvement. We report this case with a brief review of literature.
Biopsy ; Blood Vessels ; Connective Tissue ; Fibrosis ; Glomerulonephritis ; Heart ; Humans ; Hypertension ; Kidney ; Lung ; Necrosis ; Nephrotic Syndrome* ; Pathology ; Proteinuria ; Sclerosis ; Skin