The persistent left superior vena cava (LSVC) is not rare cardiovascular developmental anomaly occurring both in association with congenital heart disease and as an isolated anomaly of no hemodynamic importance. We have studied 73 cases of the LSCV out of 1,060 cases of congenital heart disease catheterized at Yonsei Cardiovascular Center. We conducted the study with a view point of position of the heart and abdominal organs and segmental analysis of the underlying congenital heart disease. We also analysed the associated extracardiac vascular anomalies. The following results were obtained: 1) The incidence of this anomaly among congenital heart disease was 6.9% and 41 cases(56.2%) had cyanosis. 2) We observed 20 cases(27.3%) with the malposition of the heart and 17 cases(23.3%) with malposition of the abdominal organs. The ventricular loops revealed D-loop in 60 cases, L-loop in 7 cases and in the remaining 6 cases, it was uncertain. 3) With a view point of type of LSVC by Lucas & Krabill, type A was in 50 cases(68.5%), type D in 14 cases(19.2%), type B in 5 cases(6.8%) and type C in 4 cases(5.5%). 4) Associated cardiovascular anomalies were as follows: ventricular septal defect; 42 cases(57.5%), atrial septal defect; 33 cases(45.2%), patent ductus arteriosus; 27 cases(36.9%), and tetralogy of Fallot; 18 cases(24.7%). In conclusion, LSVC usually has no hemodynamic importance, but this cardiac anomaly is frequently combined with complex intracardiac anomalies. Therefore, it is important to making accurate diagnosis and successful management for preventing the risk of it.
Catheters ; Classification* ; Cyanosis ; Diagnosis ; Ductus Arteriosus, Patent ; Heart ; Heart Defects, Congenital ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Hemodynamics ; Incidence ; Tetralogy of Fallot ; Vena Cava, Superior*

Embolization of a carotid cavernous fistula(CCF) by means of a detachable balloon is a well-established method for treating CCFs while preserving a patent parent internal carotid artery(ICA). However, failure to embolize the CCF may occur on a few occasions. Herein we describe a stent-assisted Guglielmi detachable coil embolization that completely occludes the fistulous opening rather than fills the cavernous sinus. By applying this technique, we successfully treated a CCF, without compromise of the parent ICA in patients who has failed with balloon technique previously.
Cavernous Sinus ; Embolization, Therapeutic ; Fistula* ; Humans ; Parents ; Stents*

Protein C exerts anticoagulant effects by inactivating factor Va and VIIIa and stimulating fibrinolysis. The homozygous protein C deficiency is extremely rare and often results in life threatening thrombosis and purpura fulminans with necrotic cutaneous lesions. A child with homozygous protein C deficiency was treated at 6 months by a living-related liver transplantaion. After induction of anesthesia, we started an FFP infusion for protein C replacement and a low molecular weight heparin continuous infusion to prevent thrombosis. A complete reconstitution of protein C activity and resolution of the thrombotic condition occured postoperatively. So we report this case with a brief review of the literature.
Anesthesia* ; Child ; Factor Va ; Fibrinolysis ; Heparin, Low-Molecular-Weight ; Humans ; Liver Transplantation* ; Liver* ; Protein C Deficiency* ; Protein C* ; Purpura Fulminans ; Thrombosis

BACKGROUND: Pulmonary hypertension (PH) associated with end stage liver disease is rare but the risk of hemodynamic deterioration during liver transplantation may be high. This study was done to characterize the pulmonary hemodynamics during liver transplantation and to seek the relationship between pulmonary artery pressure (PAP) and other hemodynamic variables. METHODS: One hundred patients undergoing liver transplantation were chosen and we divided patients into normal and PH groups (mean pulmonary artery pressure [MPAP] > 25 mmHg). Hemodynamic data was collected throughout the surgery. Studied variables between groups were analyzed with an unpaired t-test. The relationship between MPAP and other hemodynamic variables was analyzed with a linear regression test. Survival analysis was performed by cumulative survival analysis (Logrank test). RESULTS: Incidence of PH during liver transplantation was 34%, and true PH (pulmonary vascular resistance index [PVRI] > 150 dyne.sec/cm5/m2, MPAP > 25 mmHg) was 7%. MPAP, systemic vascular resistance index, cardiac index, right ventricular ejection fraction, maximum elastance, central venous pressure (CVP), pulmonary artery occlusion pressure (PAOP), and right ventricular end-diastolic volume index were significantly higher in the PH group. In the PH group, right ventricular function curve was abnormal. MPAP correlated significantly with PAOP, and CVP (P < 0.01). One year survival rate showed no significant difference between groups (Logrank test P = 0.49). CONCLUSIONS: Episodes of increased pulmonary artery pressure during liver transplantation was not infrequent. PAP was more dependent on preloads. In patients with high PAP, RV diastolic dysfunction was usually observed. Early mortality rate after liver transplantation was not associated with PH.
Central Venous Pressure ; End Stage Liver Disease ; Hemodynamics ; Humans ; Hydrogen-Ion Concentration ; Hypertension, Pulmonary ; Incidence ; Linear Models ; Liver Transplantation* ; Liver* ; Mortality ; Pulmonary Artery* ; Stroke Volume ; Survival Rate ; Vascular Resistance ; Ventricular Function, Right

Meningiomas causing intracranial hemorrhage are rare, and hemorrhage from a lateral ventricular meningioma seems to be even rarer. We report a case of trigonal meningioma in a 43-year-old woman who presented with intraventricular hemorrhage, and describe the CT, MRI and angiographic findings.
Adult ; Case Report ; Female ; Human ; Intracranial Hemorrhages/*etiology/radiography ; Lateral Ventricles/*radiography ; Meningeal Neoplasms/*complications/*radiography ; Meningioma/*complications/*radiography

No abstract available.
Mycobacterium tuberculosis* ; Mycobacterium* ; Polymerase Chain Reaction* ; Sputum*

No abstract available.
Humans* ; Lymphokines* ; Monocytes* ; Mycobacterium tuberculosis* ; Mycobacterium*

Leukemia is a rare disease in the newborn infant. We have presented an autopsy case of congenital acute myelocytic leukemia in a female neonate and discussecd with review of literature. At birth, she was relatively in good health with 4.2 kg in body weight except a large cephalhematoma on left parietal scalp and multiple subcutaneous nodules with ecchymosis on entire body surface. Hemoglobin concentration was 12.0 gm/, Hct 34.6% and erythrocyte count was 2.24 millions. Of 212,400 leukocytes/mm2, 47% were myeloblast. Biopsy of skin nodules reveal leukemia cutis, which disappear dramatically with anticancer drug. The infant was expired 12 days after admission due to intracerebral hemorrhage and acute renal failure.
Infant ; Male ; Female ; Infant, Newborn ; Humans ; Biopsy

Leukemia is a rare disease in the newborn infant. We have presented an autopsy case of congenital acute myelocytic leukemia in a female neonate and discussecd with review of literature. At birth, she was relatively in good health with 4.2 kg in body weight except a large cephalhematoma on left parietal scalp and multiple subcutaneous nodules with ecchymosis on entire body surface. Hemoglobin concentration was 12.0 gm/, Hct 34.6% and erythrocyte count was 2.24 millions. Of 212,400 leukocytes/mm2, 47% were myeloblast. Biopsy of skin nodules reveal leukemia cutis, which disappear dramatically with anticancer drug. The infant was expired 12 days after admission due to intracerebral hemorrhage and acute renal failure.
Infant ; Male ; Female ; Infant, Newborn ; Humans ; Biopsy

We report a case of epidermal nevus syndrome showing various skin manifestations improved with CO₂ laser and chemical peeling in a 11-year-old girl. Skin lesions were composed of linear verrucous plaques and numerous papillomatous papules on the face, neck, scalp and trunk, multiple congenital nevocellular nevi on the face, and extensive cafe au lait spots on the trunk. The associated findings of skeletal involvement were gingival hemihypertrophy and benign bone lesion of the 7th rib. CO₂ laser and chemical peeling MCA, 50% TCA) were applied to remove these skin lesions which improved considerably.
Cafe-au-Lait Spots ; Child ; Female ; Humans ; Neck ; Nevus* ; Ribs ; Scalp ; Skin Manifestations* ; Skin*