Pulmonary atresia with major aorto-pulmonart collateral arteries(abbreviated as MAPCA in the following)as a route for pulmonary blood flow presents many problem in diagnosis and management and recently new approach to surgical management (unifocalization)was suggested and few reported it's application. Nineteen cases who had pulmonary with MAPCA, diagnosed at Seoul National University Children's Hospital from October 1987 to May 1989, were studied to lool at the relationship between MAPCA, central pulmonary artery and segmental pulmonary arteries. Also was observed the clinical course and operative management. The average number of MACPA in each patient was 4, two toward right lung and the other two toward left lung. MACPA arose most frequently from descending aorta and innominate artery contralateral to the side of aortic arch. Central pulmonary artery was identified in 86.7%. The number of bronchopulmonary segment connected to central pulmonary artery directly or indrectly was very variable so was the number of bronchopulmonary segment connected to MAPCA only. Three had a total correction and 14 had a various kinds of palliative operation once or twice. Seven had a called "unifocalization"(connection of MAPCA to central pulmonary artery) to correct arborization abnormality but the result was not satisfactory for technical reason.
Aorta, Thoracic ; Arteries* ; Brachiocephalic Trunk ; Diagnosis* ; Humans ; Lung ; Pulmonary Artery ; Pulmonary Atresia* ; Seoul

No abstract available.
Central Venous Catheters* ; Fibrin*

No abstract available.

No abstract available.
Leukemia*

No abstract available.
Child* ; Humans ; Infant, Newborn*

STUDY DESIGN: This study analyzed the efficacy of the reduction and its maintenance using the posterior pedicular screw system in adult patients undergoing surgical treatment for symptomatic, isthmic spondylolisthesis. OBJECTIVE: To evaluate the effect of the reduction for lumbar isthmic spondylolisthesis in adult. SUMMARY OF LITERATURE REVIEW: If the increase of the shear force is approved as a main biomechanical feature of spondylolisthesis, reduction of the shear forces to the physiologic level must be regarded as the goal of surgical treatment. MATERIALS AND METHODS: 11 cases were treated with posterior monosegmental two point f;cation and posterolateral fusion (Group 1), 8 cases with a combined surgery of posterior monosegmental two point fixation, posterolateral fusion and anterior interbody fusion (Group 2), and 20 cases with posterior two segmental three point fixation and posterolateral fusion (Group 3). We assessed the clinical results and the radiologic objective parameters. RESULTS: The patients were followed up for more than 2 years (mean : 64 months). The satisfactory clinical results were 9 cases (81.8%) in Group 1 ,7 cases (87.5%) in Group 2 and 17 cases (85%) in Group 3. Radiologically, the efficacy of reduction and its maintenance were satisfactory results in Group 2, and the changes of the slip angle and the disc height showed that the loss of correction was greater than postoperative correction, although there were no statistical significant differences in the three groups. CONCLUSION: According to the our results, there was no significant relationship between the clinical result and the rdiological one. However, we think that the correcton of the deformity using the posterior pedicular screw system have some biomechanical advantages and seem to be recommendable for the treatment of isthmic spondylolisthesis in adults.
Adult* ; Congenital Abnormalities ; Humans ; Spondylolisthesis*

BACKGROUND: To get the information about the clinical characteristics of the paroxysmal junctional tachycardia in children and to get the general principle in managing these children. METHOD: Analysis of the medical records of the 43 patients(male 30, female 13) with paroxysmal junctional tachycardia(JT) who had been followed-up in this hospital for a mean of 4.6 years(range 1 month up to 12 years) was done. RESULT: In 19 patients, JT started before 1 years of age : in 2, during gestational period, in 15, within 4 months of age, in 2, after 4 months of age. The next peak was 7 in the age of 5 years. The associated cardiac abnormalities were complex congenital defects in 2, tumor in 1, dilated and hypertrophic cardiomyopathy in 1 each. The significant hemodynamic disturbances during JT were noticed in 25. Among those whose surface electrocardiogram during JT were available, mean heart rate during JT was 232rpm(range 160-310) ; narrow QRS complex in 33 and wide in 1 ; P` wave in ST segment or T wave in 22. The delta waves were noticed after stopping JT and during followe up in 20. The types of delta waves were A in 7, B in 9, and indeterminate in 4. The different forms of delta waves unrelated to the degree of fusion were noticed in 6 ; disappearance or intermittent form of delta wave in 4. The efficacy of stopping JT was as follows : ATP 84.4%(38/45), diving reflex 50%(7/14), other vagal stimulation 71.4%(5/7), digoxin 72.7%(8/11), verapamil 54.5%(12/22), D/C cardioversion 62.5%(5/8), neosynephrine 100%(2/2). There were 2 deaths due to associated cardiac defects and 2 elective catheter ablations during the followe up period. The preventive medication with digoxin, beta blocker, and/or verapamil was succesful in 14, partially succesful in 11, failed in 14. The 7 persistent JT were treated with amiodarone in 3, with amiodarone and beta blocker in 1, with flecainide and digoxin in 1. In 1, surgical ablation of accessory pathway was done due to persistent JT. At present, JT do not recur or occur transiently without drugs in 29 ; with drugs, JT become controlled without recurrence in 4, with transient episodes in 4 and with intermittent episodes in 1. CONCLUSION: Althouh the JT in children is benign in most cases spite of the severity during the early period, JT is persistent in cases and needs potent drugs to control JT. Ablation of the foci may be necessary in these cases. Even in patients whose long-term results are benign, it is necessary to choose the optimal drugs to terminate and prevent the JT during the intervening period.
Adenosine Triphosphate ; Amiodarone ; Cardiomyopathy, Hypertrophic ; Catheters ; Child* ; Congenital Abnormalities ; Digoxin ; Diving ; Electric Countershock ; Electrocardiography ; Female ; Flecainide ; Heart Rate ; Hemodynamics ; Humans ; Medical Records ; Phenylephrine ; Recurrence ; Reflex ; Tachycardia* ; Verapamil

No abstract available.
Coronary Artery Disease* ; Coronary Vessels* ; Dobutamine* ; Echocardiography, Stress* ; Humans ; Myocardium* ; Tomography, Emission-Computed, Single-Photon*

Spinal dysraphism, first described by Lichtenstein in 1940, is a congenital anom- aly due to incomplete fusion or malformation of the midline dorsal embryonic structures including the ectoderm, mesoderm, and neuroectoderm. Spina bifida occulta is a spinal dystraphism and a manifestation of a midline defect of osseous spine and related structures without cyst formation. Spinal dysraphism may be associated with hypertrichosis, lipoma, dimpling, pigmentation, hemangioma, congenital scar, sinus, cyst, or skin defect in the midline dorsal area. We observed a 23-year-old female patient with spina bifida occulta who had a blue scar surrounded by circumscribed hair growth of the lumbosacral area since 1-montb-old age. Histopathologic finding of the blue scar revealed common blue nevus showing intradermal melanocytes and melanophages. Spine X-ray showed hemivertebra of L5 and spina bifida occulta of Sl. There were no abnormal neurologic signs.
Cicatrix ; Ectoderm ; Embryonic Structures ; Female ; Hair ; Hemangioma ; Humans ; Hypertrichosis* ; Lipoma ; Melanocytes ; Mesoderm ; Neural Plate ; Neurologic Manifestations ; Nevus, Blue* ; Pigmentation ; Skin ; Spina Bifida Occulta* ; Spinal Dysraphism* ; Spine ; Young Adult

No abstract available.
Heterotaxy Syndrome*