Adult granulosa cell tumors (AGCTs) presenting with massive ascites and elevated serum CA-125 levels have rarely been described in the literature. An ovarian mass, massive ascites, and elevated serum CA-125 levels in postmenopausal women generally suggest a malignant ovarian tumor, particularly advanced epithelial ovarian cancer. AGCT has low 18F-fluorodeoxyglucose uptake on positron emission tomography/computed tomography due to its low metabolic activity. In the present report, we describe a case of an AGCT with massive ascites, elevated serum CA-125 level, and low 18F-fluorodeoxyglucose uptake on positron emission tomography/computed tomography.
Adult* ; Ascites* ; Electrons* ; Female ; Granulosa Cell Tumor* ; Granulosa Cells* ; Humans ; Ovarian Neoplasms

Choledochal cysts are congenital lesions involving cystic dilatation of the bile ducts. The choledochocele, the rarest type of choledochal cyst, is a cystic dilatation of the distal common bile duct that protrudes into the duodenum. There have been few reports concerning malignancy in the biliary tree associated with choledochoceles. We recently experienced a case of a choledochocele with gallbladder cancer.
Bile Ducts ; Biliary Tract ; Choledochal Cyst ; Common Bile Duct ; Dilatation ; Duodenum ; Gallbladder ; Gallbladder Neoplasms

BACKGROUND: The purpose of this study was to determine the optimal dose of remifentanil for minimizing hemodynamic changes during intubation and reducing propofol-induced pain in elderly patients. METHODS: In a randomized prospective study, 60 patients (ASA I-II, elder than 65 years) were enrolled to determine which of two target remifentanil blood concentrations (3 ng/ml, 5 ng/ml) was required to blunt hemodynamic changes during intubation and to reduce propofol-induced pain. After the target effect site concentration of remifentanil had been reached, the target controlled infusion of propofol was started and propofol-induced pain was recorded. Blood pressure and heart rate were recorded at baseline, just before intubation and 1, 3, 5 min after intubation. RESULTS: There were no significant differences in the hemodynamic parameters between two groups, but not in arterial pressure at just before intubation and 1 minute after intubation. However, the group R5 (5 ng/ml) showed significantly less intense pain induced by propofol than in the group R3 (3 ng/ml). CONCLUSIONS: The results suggest that the group R5 provide more relief in propofol induced pain than the group R3, but showed great possibility of hypotension and bradycardia in both groups, which means it should be used with cautions in the elderly patients.
Aged ; Arterial Pressure ; Blood Pressure ; Bradycardia ; Heart Rate ; Hemodynamics ; Humans ; Hypotension ; Intubation ; Piperidines ; Propofol ; Prospective Studies

BACKGROUND: The aim of this study was to investigate the prevalence, clinical and laboratory findings of hereditary hemolytic anemia (HHA) in Korea from 1997 to 2006 and to develop the appropriate diagnostic approach for HHA. METHODS: By the use of questionnaires, information on the clinical and laboratory findings ofHHA diagnosed from 1997 to 2006 in Korea was collected and analyzed retrospectively. A total of 431 cases were enrolled in this study from 46 departments of 35 hospitals. RESULTS: The overall frequency of HHA did not change through the 10-year period for pediatrics but did show an increasing tendency for internal medicine. The overall male to female sex ratio did not show sex predominance (1.17:1), but a significant male predominance with a ratio of 1.49:1 was seen for pediatrics while a significant female predominance with a ratio of 1:1.97 was seen forinternal medicine. Of the total cases, 74.2% (282/431) were diagnosed before the age of 15 years. The etiologies of HHA were classified as red cell membrane defects, hemoglobinopathies, red cell enzyme deficiencies and unknown causes. There were 382 cases (88.6%) of red cell membrane defects with 376 cases (87.2%) of hereditary spherocytosis and 6 cases (1.4%) of hereditary elliptocytosis, 20 cases (4.6%) of hemoglobinopathies with 18 cases (4.2%) of beta-thalassemia, a case (0.2%) of alpha-thalassemia and a case (0.2%) of Hemoglobin Madrid, 7 cases (1.6%) of red cell enzyme deficiencies with 5 cases (1.2%) of glucose-6- phosphate dehydrogenase (G-6-PD) deficiency, a case (0.2%) of pyruvate kinase (PK) deficiency and a case (0.2%) of enolase deficiency, and 22 cases (5.1%) of unknown causes. The most common chief complaint in pediatric patients was pallor and that in adult patients was jaundice. In the red cell membrane defect group of patients, the level of hemoglobin was significantly higher than in adult patients. The mean corpuscular volume, mean corpuscular hemoglobin, corrected reticulocyte count, total and indirect bilirubin level and lactate dehydrogenase levels in the hemoglobinopathy group of patients were significantly lower than the values in the red cell membrane defect group of patients. The mean concentration of G-6-PD was 0.8+/-0.7U/1012RBC in the G-6-PD deficient patients, PK was 1.7U/1010 RBC in the PK deficient patient, and the level of enolase was 0.04U/g of Hb in the enolase deficient patient. CONCLUSION: The most prevalent cause of HHA in Korea during 1997 to 2006 was hereditary spherocytosis, but HHA by other causes such as hemoglobinopathy and red cell enzyme deficiency gradually increased with the development of molecular diagnostic methods and increasing general interest. However, the etiologies of HHA need to be pursued further in 5.1% of the patients. An systematic standard diagnostic approach is needed in a nationwide prospective study for correct diagnoses and appropriate management of HHA.
Adult ; alpha-Thalassemia ; Anemia, Hemolytic, Congenital* ; beta-Thalassemia ; Bilirubin ; Cell Membrane ; Diagnosis ; Elliptocytosis, Hereditary ; Erythrocyte Indices ; Female ; Hemoglobinopathies ; Humans ; Internal Medicine ; Jaundice ; Korea* ; L-Lactate Dehydrogenase ; Male ; Oxidoreductases ; Pallor ; Pathology, Molecular ; Pediatrics ; Phosphopyruvate Hydratase ; Prevalence ; Pyruvate Kinase ; Reticulocyte Count ; Retrospective Studies* ; Sex Ratio ; Surveys and Questionnaires

BACKGROUND: The long-term results of combined mitral valve repair and aortic valve replacement (AVR) have not been well evaluated. This study was performed to investigate the early and long-term results of mitral valve repair with AVR. MATERIAL AND METHOD: We retrospectively reviewed 45 patients who underwent mitral valve repair and AVR between September 1990 and April 2002. The average age was 47 years; 28 were men and 17 women. Twelve patients had atrial fibrillation and three had a previous cardiac operation. The mitral valve disease consisted of pure insufficiency (MR) in 34 patients, mitral stenosis (MS) in 3, and mixed lesion in 8. Mitral valve disease was due to rheumatic origin in 24 patients, degenerative in 11, annular dilatation in 8, and ischemia or endocarditis in 2. The functional anatomy of mitral valve was annular dilatation in 31 patients, chordal elongation in 19, leaflet thickening in 19, commissural fusion in 13, chordal fusion in 10, chordal rupture in 6, and so on. Aortic prostheses used included mechanical valve in 32 patients, tissue valve in 12, and pulmonary autograft in one. The techniques of mitral valve repair included annuloplasty in 32 patients and various valvuloplasty of 54 techniques in 29 patients. Total cardiopulmonary bypass and aortic cross clamp time were 204+/-62 minute and 153+/-57 minutes, respectively. RESULT: Early death was in one patient due to low output syndrome (2.2%). After follow up of 57+/-37 months, late death was in one patient and the actuarial survival at 10 years was 96+/-4%. Recurrent MR developed grade II or III in 11 patients and moderate MS in 3. Three patients required reoperation for valve-related complications. The actuarial freedom from recurrent MR, MS, and reoperation were 64+/-11%, 86+/-8%, and 89+/-7% respectively. CONCLUSION: Combined mitral valve repair with AVR offers good early and long-term survival, and adequate techniques and selection of indication of mitral valve repair, especially in rheumatic disease, are prerequisites for better long-term results.
Aortic Valve* ; Atrial Fibrillation ; Autografts ; Cardiopulmonary Bypass ; Dilatation ; Endocarditis ; Female ; Follow-Up Studies ; Freedom ; Humans ; Ischemia ; Male ; Mitral Valve Stenosis ; Mitral Valve* ; Prostheses and Implants ; Reoperation ; Retrospective Studies ; Rheumatic Diseases ; Rupture

BACKGROUND: We analysed the surgical outcomes of immediate reoperations after mitral valve repair. MATERIAL AND METHOD: Eighteen patients who underwent immediate reoperation for failed mitral valve repair from April 1995 through July 2001 were reviewed retrospectively. There were 13 female patients. The mitral valve disease was regurgitation (MR) in 12 patients, stenosis (MS) in 3, and mixed lesion in 3. The etiologies of the valve disease were rheumatic in 9 patients, degenerative in 8, and endocarditis in 1. The causes of reoperation was residual MR in 13 patients, residual MS in 4, and rupture of left ventricle in 1. Fourteen patients had rerepair for residual mitral lesions (77.8%) and four underwent replacement. RESULT: There was no early death. After mean follow-up of 33 months, there was one late death. Echocardiography revealed no or grade I of MR (64.3%) in 9 patients and no or mild MS in 11 patients (78.6%). Reoperation was done in one patient. The cumulative survival and freedom from valve-related reoperation at 6 years were 94% and 90%, respectively. The cumulative freedom from recurrent MR and MS at 4 years were 56% and 44%, respectively. CONCLUSION: This study suggests that immediate reoperation for failed mitral valve repair offers good early and intermediate survival, and mitral valve rerepair can be successfully performed in most of patients. However, because mitral rerepair have high failure rate, especially in rheumatic valve disease, adequate selections of valvuloplasty technique and indication are important to reduce the failure rate of mitral rerepair.
Constriction, Pathologic ; Echocardiography ; Endocarditis ; Female ; Follow-Up Studies ; Freedom ; Heart Ventricles ; Humans ; Mitral Valve* ; Reoperation* ; Retrospective Studies ; Rupture

BACKGROUND: Pulmonary artery banding (PAB)in the functional univentricular heart (UVH)is a palliative procedure for staging toward the Fontan procedure;however,it is known to be a risk factor. MATERIALS AND METHOD: The records of all 37 patients with functional UVHs who underwent surgical palliation using PAB between September 1989 and August 1999 were reviewed retrospectively.We investigated the aortic arch obstruction,the development and progression of subaortic stenosis after PAB,and risk factor of mortality according to surgical method. RESULT: In 37 neonates and infants with single ventricular physiology,aortic arch obstruction was combined in 7.There were 6 early deaths (16.2%)after PAB and 3 late deaths (8.1%)after Fontan operation.The actuarial overall survival including early mortality at 3 and 5 years were 8 0 .7+/-6.6%,72.2 +/-8.2% respectively. Among 31 patients who survived PAB,27 patients (87.1%)could become candidates for Fontan operation;22 patients(71.0%)completed Fontan operation with 3 deaths and 5 were waiting bidirectional cavopulmonary shunt(BCPS)or Fontan operation (follow-up mean 4.5 year,minimal 2 year). Subaortic stenosis developed in 8 patients after PAB (8/29,27.6%);3 cases in the patients without arch anomaly (3/22,13.6%)and 5 in those with arch anomal y (5/7,71.4%).The subaortic stenosis was managed with Damus-Kaye-Stansel procedure (DKS)in 6 patients without operative mortality and conal septum resection in 2 without long-term survivor. Analysis of risk factors established that aortic arch obstruction was strongly associated with subaortic stenosis (p<0.001).The only risk factor of late mortality was Fontan procedure without staged palliation by BCPS (p=0.001). CONCLUSION: PAB is effective as an initial palliative step in functional UVH.And the high risk group of patients with aortic obstruction can undergo effective short-term PAB as an initial palliative step,with subsequent DKS for subaortic stenosis.This strategy,initial PAB and careful surveillance,and early relief of subaortic stenosis can maintain acceptable anatomy and hemodynamics for later Fontan procedures.
Aorta, Thoracic ; Constriction, Pathologic ; Fontan Procedure ; Heart* ; Hemodynamics ; Humans ; Infant ; Infant, Newborn ; Mortality ; Pulmonary Artery* ; Risk Factors ; Survivors

BACKGROUND: Reoperations on the aortic root or the ascending aorta are being performed with increasing frequency and remain a challenging problem.This study was performed to analyze the results of reoperations on the ascending aorta and aortic root. MATERIAL AND METHOD: Between May 1995 and April 2001,30 patients had reoperations on the ascending aorta and aortic root and were reviewed retrospectively.The mean interval between the previous repair and the actual reoperation was 56 months(range 3 to 142 months).Seven patients(23.3%)had two or more previous operations.The indications for reoperations were true aneurysm in 7 patients(23.3%),prosthetic valve endocarditis in 6(20%),false aneurysm in 5(16.7%),paravalvular leak associated with Behcet's disease in 4(13.3%),malfunction of prosthetic aortic valve in 4(13.3%),aortic dissection in 3(10%),and annuloaortic ectasia in 1(3.3%).The principal reoperations performed were aortic root replacement in 17 patients(56.7%),replacement of the ascending aorta in 8(26.7%),aortic and mitral valve replacement with reconstruction of fibrous trigone in 2(6.6%),patch aortoplasty in 2(6.6%), and aortic valve replacement after Bentall operation in 1(3.3%).The cardiopulmonary bypass was started before sternotomy in 7 patients and the hypothermic circulatory arrest was used in 16(53.3%).The mean time of circulatory arrest,total bypass,and aortic crossclamp were 20 +/- 12 minutes,228 +/- 56 minutes,and 143 +/- 62 minutes,respectively. RESULT: There were three early deaths(10%).The postoperative complications were reoperation for bleeding in 7 patients(23.3%),cardiac complications in 5(16.7%),transient acute renal failure in 2(6.6%), transient focal seizure in 2(6.6%),and the others in 5.The mean follow-up was 22.8 +/- 20.5 months.There were two late deaths(7.4%).The actuarial survival was 92.6 +/- 5.0%at 6 years. One patient required reoperation for complication of reoperation on the ascending aorta and aortic root(3.7%).The 1-and 6-year actuarial freedom from reoperation was 100%and 83.3 +/- 15.2%,respectively.One patient with Behcet's disease are waiting for reoperation due to false aneurysm,which developed after aortic root replacement with homograft.There were no thromboembolisms or anticoagulant related complications. CONCLUSIONS: This study suggests that reoperations on the ascending aorta and aortic root can be performed with acceptable early mortality and morbidity,and adequate surgical strategies according to the pathologic conditions are critical to the prevention of the reoperation.
Acute Kidney Injury ; Aneurysm ; Aorta* ; Aortic Valve ; Cardiopulmonary Bypass ; Dilatation, Pathologic ; Endocarditis ; Follow-Up Studies ; Freedom ; Hemorrhage ; Humans ; Mitral Valve ; Mortality ; Postoperative Complications ; Reoperation ; Seizures ; Sternotomy ; Thromboembolism

BACKGROUND: This study was undertaken to investigate the outcome of composite graft aortic root replacement using coronary button reimplantation technique for the treatment of aneurysms of the ascending aorta involving the aortic root. MATERIAL AND METHOD: Between April 1995 and September 2001, 54 patients having aortic root replacement with a composite valve graft using direct coronary button reimplantation were reviewed retrospectively. Left ventricular dysfunction was present in 14 patients(25.9%), aortic regurgitation in 48(89%), and Marfan's syndrome in 17(31.5%). The indications for operation were annuloaortic ectasia in 29 patients(53.7%), aortic dissection in 11(20.4%), aneurysms of the ascending aorta involving aortic root in 12(22.2%), and aortitis in 2(3.7%). Six patients(11.1%) had previous cardiac or ascending aortic operations. Concomitant procedures were arch replacement in 21 patients(38.9%), coronary artery bypass graft in 7(13%), mitral valve repair or replacement in 4(7.4%), and others in 6. The mean time of circulatory arrest, total bypass, and aortic crossclamp were 18+/-9 minutes, 177+/-42 minutes, and 127+/-31 minutes, respectively. RESULT: There was 1 early death(1.9%). Mean follow-up was 24.6+/-19.5 months. There were two late deaths(3.8%) including one death due to the traumatic cerebral hemorrhage. The Kaplan-Meier survival rate was 98.0+/-2.0% and 93.1+/-5.1% at 1 and 6 years, respectively. Two patients required reoperation owing to a false aneurysm at the root anastomosis site anda malfunction of prosthetic aortic valve(3.8%). Staged operation for dissection of the remaining thoracoabdominal aorta was performed in 1 patient. The freedom rate from reoperation was 97.8+/-2.0% and 65.3+/-26.7% at 1 and 6 years, respectively. CONCLUSION: This study suggests that composite graft aortic root replacement using open button technique is a safe and effective therapy for a variety of aneurysms of the aortic root, resulting in good early and mid-term results. Careful follow-up of all patients following composite graft root replacement is important for the good long-term results.
Aneurysm ; Aneurysm, False ; Aorta ; Aortic Valve Insufficiency ; Aortitis ; Cerebral Hemorrhage, Traumatic ; Coronary Artery Bypass ; Dilatation, Pathologic ; Follow-Up Studies ; Freedom ; Humans ; Marfan Syndrome ; Mitral Valve ; Reoperation ; Replantation* ; Retrospective Studies ; Survival Rate ; Transplants* ; Ventricular Dysfunction, Left

We report one case of an 18-day-old female patient, weighing 3.4 kg, with severe cyanosis. The diagnosis was made with only transthoracic echocardiography, which revealed cor triatriatum with an atretic small opening of fibromuscular membrane, obstructive infracardiac total anomalous pulmonary venous drainage(TAPVD), severely restrictive interatrial communication, and scanty mitral inflow and aortic forward flow. The preoperative decision-making for biventricular repair was not easy due to collapsed left heart system caused by remarkably reduced blood flow. An emergent operation was performed due to severe cyanosis. All left heart structures were somewhat hypoplastic but thought to be adequate for systemic circulation. Biventricular repair was done without specific intraoperative problems. The postoperative course was uneventful. The patient has been doing well with no evidence of pulmonary vein stenosis or mitral regurgitation for 4 months after operation.
Constriction, Pathologic ; Cor Triatriatum* ; Cyanosis ; Diagnosis ; Drainage* ; Echocardiography ; Female ; Heart ; Humans ; Membranes ; Mitral Valve Insufficiency ; Pulmonary Veins