A procedure descried by Fontan and Baudet in 1971 successfully bypassed the right ventricle in Tricuspid Atresia patients, after then many modification of the Fontan operation had been described and applied to many cyanotic complex heart patients. Forty patients with a variety of cardiac malformation underwent the Fontan operation at Seoul National University hospital (September 1978 to June 1986). The age at operation ranged 2 months to 18 years. Each number of cases according to basic cardiac anomaly was as follows; 17 in Tricuspid Atresia, 17 in Univertricular Heart, 2 in Double outlet of Right Ventricle, 2 in Transposition of the Great Arteries and 2 in Criss-cross heart. Total mortality rate after the Fontan operation was 50%. There was only one late death (>30 days). Mortality rate under 4 years of age (67%) was higher than that between 4 and 18 years of age (40%). we observed a significantly higher mortality for patients who, in the immediate postoperative period, had central venous pressure greater than 25cm H2O. 45% among survivals did not require further medication. Although mortality rate after the Fontan operation is much higher than that in the foreign literature, operative mortality will decline with the increased expirence of surgeon and the effective patients selection.
Arteries ; Central Venous Pressure ; Crisscross Heart ; Fontan Procedure* ; Heart ; Heart Ventricles ; Humans ; Mortality ; Postoperative Period ; Seoul ; Tricuspid Atresia

From February 1987 through July 1990, the seventeen cases of inoperabelintracranial arteriovenous malformation (AVM) were treated using 6 MV linear accelerator at the Division of Therapeutic Radiology, Kang Nam St. Mary's Hospital. Of seventeen cases, fourteen were male and three were female. Ages ranged from 10 to 51 years (median age of 26 years). The main symptoms were headache, epilepsy and hemiparesis in decreasing order of frequency. The middle cerebral artery is the most common origin of the feeding vessel (41.2%). Four were treated by conventionally fractionated radiation therapy (CRT), thirteen were treated by stereotactic radiosurgery (RS). Duration of follow-up study, two of four CRT group showed minimal response. Of thirteen cases of RS group, two (15.4%) showed complete response, five (38%) partial response, two (15.4%) minimal response and four (30.7%) no response by the same assessment. There was no statistical significance in terms of follow-up period (p=0.22), size of lesion (p=0.82) and treated dose (p=0.65). Further accumulation of experience s recommended with proper case selection and sufficient follow-up period.
Arteriovenous Malformations ; Epilepsy ; Female ; Follow-Up Studies ; Headache ; Humans ; Intracranial Arteriovenous Malformations* ; Male ; Middle Cerebral Artery ; Paresis ; Particle Accelerators ; Radiation Oncology ; Radiosurgery*

Pigmented mammary Paget disease is a very rare clinicopathologic variant of mammary Paget disease. Diagnosis is often difficult because its clinical and histological features are very similar to those of malignant melanoma. Herein, we report a case of pigmented mammary Paget disease misdiagnosed as malignant melanoma.
Diagnosis ; Melanoma* ; Paget's Disease, Mammary*

Interstitial granulomatous dermatitis (IGD) is a rare disease that has been associated with multiple systemic diseases, particularly autoimmune conditions like rheumatoid arthritis and systemic lupus erythematosus. IGD has a variable clinical presentation and highly characteristic histological features of interstitial infiltrate of histiocytes between the degenerated collagen bundles. Here we report the case of a 63-year-old woman who presented with a 3-month history of multiple asymptomatic erythematous papules on the bilateral aspects of the trunk. A histopathological examination of the lesion showed an interstitial lymphohistiocytic infiltrate in the dermis. There were also foci of palisading histiocytes along with degenerating collagen bundles.
Arthritis, Rheumatoid ; Collagen ; Dermatitis* ; Dermis ; Female ; Histiocytes ; Humans ; Immunoglobulin D ; Lupus Erythematosus, Systemic ; Middle Aged ; Rare Diseases

No abstract available.
Hamartoma*

PURPOSE: To determine the usefulness of MR imaging for differentiation between infectious and non-infectiousbursitis. MATERIALS AND METHODS: MR images of 16 patients (18 lesions) in whom bursitis around the hip had beendiagnosed were analyzed for homogeneity of the bursa, the presence of septation, the enhancement pattern, andassociated findings. Clinical data (symptoms and signs, laboratory data, aspiration of the bursa, and surgicalfindings) were available for correlation. The location of bursitis was trochanteric (n=9), ischiogluteal (n=5),iliopsoas (n=3), or ischiotrochanteric (n=1). RESULTS: Etiologies included infection in seven cases (3 pyogenic; 4tuberculous) and noninfecti-on in 11 (6 inflammation; 3 hemorrhage; 2 metabolic disease). In seven patients withinfectious bursitis, T1-weighted enhanced image revealed thick rim enhancement of the bursa (n=7) association withchanges in bone marrow signal intensity (n=2), bone erosion (n=2), and cellulitis (n=1). Of 11 cases ofnoninfectious bursitis, three demonstrated typical signal characte-ristics of hematoma within the distended bursa.In six of seven patients who underwent contrast-enh ancement, thick and thin peripheral enhancement of the bursawas noted. Bone erosion was found in one case of tuberculous bursitis and two of metab-olic disease. Internalseptation (n=4) and internal debris (n=3) were found in both infected and noninfected patients. CONCLUSION: MRimaging plays an important role in the diagnosis of bursitis around the hip. MR findings of thick rim enhancement,associated cellulitis, and changes in bone marrow signal intensity are suggestive of infectious bursitis.
Bone Marrow ; Bursitis* ; Cellulitis ; Diagnosis ; Femur ; Hematoma ; Hemorrhage ; Hip* ; Humans ; Inflammation ; Magnetic Resonance Imaging

Sirenomelia, also known as the mermaid syndrome, is a rare and usually lethal congenital malformation of uncertain etiology. It is characterized by fusion of the lower limbs and commonly associated with severe urogenital and gastrointestinal malformations. Early antenatal sonogram allows for earlier, and less traumatic termination of pregnancy, but usually it is impared by severe oligohydramnios related to bilateral renal agenesis. We diagnosed a sirenomelia with severe oligohydramnios at early second trimester after amnioinfusion and terminated. We report a case with a brief review of the literature.
Ectromelia* ; Female ; Humans ; Lower Extremity ; Oligohydramnios ; Pregnancy ; Pregnancy Trimester, Second

PURPOSE: Genexol-PM is a Cremophor EL–free formulation of low-molecular-weight, non-toxic, and biodegradable polymeric micelle-bound paclitaxel. We conducted a phase III study comparing the clinical efficacy and toxicity of Genexol-PM with conventional paclitaxel (Genexol). MATERIALS AND METHODS: Patients were randomly assigned (1:1) to receive Genexol-PM 260 mg/m² or Genexol 175 mg/m² intravenously every 3 weeks. The primary outcome was the objective response rate (ORR). RESULTS: The study enrolled 212 patients, of whom 105 were allocated to receive Genexol-PM. The mean received dose intensity of Genexol-PM was 246.8±21.3 mg/m² (95.0%), and that of Genexol was 168.3±10.6 mg/m² (96.2%). After a median follow-up of 24.5 months (range, 0.0 to 48.7 months), the ORR of Genexol-PM was 39.1% (95% confidence interval [CI], 31.2 to 46.9) and the ORR of Genexol was 24.3% (95% CI, 17.5 to 31.1) (p(non-inferiority)=0.021, p(superiority)=0.016). The two groups did not differ significantly in overall survival (28.8 months for Genexol-PM vs. 23.8 months for Genexol; p=0.52) or progression-free survival (8.0 months for Genexol-PM vs. 6.7 months for Genexol; p=0.26). In both groups, the most common toxicities were neutropenia, with 68.6% occurrence in the Genexol-PM group versus 40.2% in the Genexol group (p < 0.01). The incidences of peripheral neuropathy of greater than grade 2 did not differ significantly between study treatments. CONCLUSION: Compared with standard paclitaxel, Genexol-PM demonstrated non-inferior and even superior clinical efficacy with a manageable safety profile in patients with metastatic breast cancer.
Breast Neoplasms* ; Breast* ; Disease-Free Survival ; Follow-Up Studies ; Humans ; Incidence ; Neutropenia ; Paclitaxel* ; Peripheral Nervous System Diseases ; Polymers* ; Treatment Outcome