Cerebral vascular disease is not infrequent disorder in children. Recently, 66 case of cerebral vascular diseases were investigated at Ped. Depart of Yonsei Medical Center. And obtained following result. 1) Age incidence represented 30% was under 1yr. Of age. 40.9% was under 2 years of age. 2) Of 66 cases 1 schemic vascular disease was 54.5% hemorrage vascular disease was 42.4%. 3) Moya disease was 8(12.1%), cases and cases associated with congenital heart disease was 9 cases(13.6%) and A-V malformatin was 8 cases (12.1%). 4) Over all the survival rate was 80.3% from the accident, in ischemic type the survival rate was 91.7% hemorragic type, 64.3%. 5) The occurrence of sequele in ischemic type was 88%, while hemorragic type 67%.
Child* ; Heart Defects, Congenital ; Humans ; Incidence ; Survival Rate ; Vascular Diseases*

The extracardiac conduit Fontan procedure is the last surgical step in the treatment of patients with a functional single ventricle. An acquired pulmonary arteriovenous malformation may appear perioperatively or postoperatively due to an uneven hepatic flow distribution. Here we report a case of a bifurcated Y-graft Fontan operation in a 15-year-old male patient with a unilateral pulmonary arteriovenous malformation after an extracardiac conduit Fontan operation.
Adolescent ; Arteriovenous Malformations* ; Fontan Procedure ; Heart Defects, Congenital ; Humans ; Male ; Vascular Diseases

Adult ; Coronary Vessel Anomalies ; diagnosis ; etiology ; Humans ; Male ; Smoking ; adverse effects ; Vascular Diseases ; congenital ; diagnosis ; etiology

OBJECTIVETo review the efficacy of total anomalous pulmonary venous connection (TAPVC) repair and to conclude the factors impacting the peri-operative death rate.

METHODSThe clinical data of 145 infants under 1 year old who underwent the TAPVC repair from January 2001 to July 2008 was analyzed. There were 94 male and 51 female patients. The mean age when the repair was performed was (7 ± 3) months, and the average weight was (6.3 ± 1.6) kg. As to the pulmonary connection type, 77 patients were supracardiac (53.1%), 47 patients were cardiac (32.4%), 9 patients were intracardiac (6.2%), and the remaining 12 patients were mixed (8.3%). Pre-surgery echocardiography showed that 21 patients had pulmonary venous obstruction (12 patients were supracardiac type, 3 patients were cardiac type, 3 patients were intracardiac type, and 3 patients were mixed type).

RESULTSAll patients underwent two-ventricle anatomy correction (the cases of complex malformations had been excluded). Peri-operative mortality was 11.7% (17/145). Because of the significant improvement in the surgical techniques, anesthesiology, cardiopulmonary bypass and the management of ICU in January 2006, the population was divided into two groups: A (before January 2006) and B (after January 2006). Peri-operative mortality decreased from 19.0% in group A to 6.2% in group B(P = 0.020). After analysis, it was determined that the factors impacting mortality were which group the patient belongs to, whether he/she had preoperative pulmonary vein obstruction and how big the atril septel connection was. The operative technique to keep the anastomotic aperture adequate and prophylaxis pulmonary hypertensive episodes contributed to the improvement on the mortality rate. There had been no case of repeating the surgery because of pulmonary venous obstruction during peri-operative care period.

CONCLUSIONSImprovements of the surgical technique as well as the treatment in preoperative and postoperative have led to the reduction of the mortality. Preoperative pulmonary vein obstruction is still an important factor that contributes to early mortality.

Female ; Humans ; Infant ; Male ; Pulmonary Veins ; abnormalities ; surgery ; Retrospective Studies ; Treatment Outcome ; Vascular Diseases ; congenital ; surgery

After amputation of the extremities, many patients developed problematic sequelae such as loss of function, cosmetic point of view, and psychiatric problems. For period of eleven yesrs from 1976 to 1986, we performed amputation of the fingers in 461 persons, and problematic sequelae were noted in 181 cases of 105 persons. The results were as follows : 1. Causes of the amputation were trauma, infection, burn, frost bite, tumors, and vascular diseases in that order of frequency. 2. Problematic sequelae of the fingers were scar contracutre, scanty soft tissue, neuroma, bony spur formation, deformities, hypersensitivity, ulceration, phantom pain, and depression in that order of frequency. 3. Problematic sequelae in amputation of the fingers could be classified as functional, cosmetic and psychiatric problems. In most instances, the primary concern of a particular patient was amputation itself, and this kind of psychiatric problems should be taken into consideration in the treatment of the amputated fingers.
Amputation ; Burns ; Cicatrix ; Congenital Abnormalities ; Depression ; Extremities ; Fingers ; Humans ; Hypersensitivity ; Neuroma ; Phantom Limb ; Ulcer ; Vascular Diseases

Coronary Aneurysm ; Coronary Angiography ; Coronary Vessel Anomalies ; Humans ; Vascular Diseases/congenital*

BACKGROUND: Pincer nail deformity is a transverse overcurvature of the nail. This study aimed to define the anatomical characteristics of pincer nail deformity and to evaluate the surgical outcomes. METHODS: A retrospective review was conducted on 20 cases of pincer nail deformity of the great toe. Thirty subjects without pincer nail deformity or history of trauma of the feet were selected as the control group. Width and height indices were calculated, and interphalangeal angles and base widths of the distal phalanx were measured with radiography. We chose the surgical treatment methods considering perfusion-related factors such as age, diabetes mellitus, kidney disease, and peripheral vascular disease. The zigzag nail bed flap method (n=9) and the inverted T incision method (n=11) were used to repair deformities. The outcomes were evaluated 6 months after surgery. RESULTS: The interphalangeal angle was significantly greater in the preoperative patient group (14.0degrees+/-3.6degrees) than in the control group (7.9degrees+/-3.0degrees) (P<0.05). The postoperative width and height indices were very close to the measurements in the control group, and most patients were satisfied with the outcomes. CONCLUSIONS: We believe that the width and height indices are useful for evaluating the deformity and outcomes of surgical treatments. We used two different surgical methods for the two patient groups with respect to the perfusion-related factors and found that the outcomes were all satisfactory. Consequently, we recommend taking into consideration the circulatory condition of the foot when deciding upon the surgical method for pincer nail deformity.
Congenital Abnormalities* ; Diabetes Mellitus ; Foot ; Humans ; Kidney Diseases ; Nails, Ingrown ; Onychomycosis ; Osteophyte ; Peripheral Vascular Diseases ; Radiography ; Retrospective Studies ; Toes

Hypertensive pulmonary vascular disease can develop in those cases of congenital cardiac shunt in which critical levels of pulmonary artery pressure and flow are reached and exceeded. We have experienced two cases of plexogenic arteriopathy in complex congenital heart disease and tried to evaluate of distribution of arterial lesions by total mapping of the explanted lung. Case 1 and 2 were 12-year-old boy and 36 year-old man. They were treated with combined heart-lung transplantation. Mapping of the both lungs was done, and graded according to Heath and Edward's grading scheme. The elastic pulmonary artery was tortuous, dilated and aortic configuration. Both lungs showed mostly grade 3. Plexiform lesion or veinlike branches of hypertrophied muscular arteries arosed in a lateral branch of a muscular artery that might be proximal to an area of occlusion. Comprising the right and left lung, the right was more severe than the left. By getting closer to the distal part, the grade tended to increase to 4 to 5. By analyzing the pulmonary lobe, severe pulmonary hypertension of grade 4 or 5 was comparatively disseminated throughout the right lung. On the other hand, in the left lung, the grade of the lower lobe was higher than that of the upper lobe, and within the upper lobe, there was a tendency for the grade of inferior segment to be higher than that of the corresponding apical segment.
Adult ; Arteries ; Child ; Hand ; Heart Defects, Congenital* ; Heart-Lung Transplantation ; Humans ; Hypertension ; Hypertension, Pulmonary ; Lung ; Male ; Pulmonary Artery ; Vascular Diseases

Peyronie's disease(PD) is an inflammatory condition characterized by the formation of fibrous, noncompliant nodules in the tunica albuginea leading to penile deformity and bending. Repetitive microtrauma of the penis during sexual intercourse is thought to incite a local autoimmune reaction in genetically susceptible individuals. The fibrotic plaques are most likely produced by tunical fibroblasts in response to cytokine stimulation, such as by transforming growth factor-beta1. In the acute inflammatory phase, pain during erection and penile deviation are the main symptom. In later stages, PD is often associated with erectile dysfunction(ED), distal flaccidity, or both. The causes of ED include psychological factors (performance anxiety), penile pain and deformity, flail penis, and vascular disease. Conservative treatment is useful during the early inflammatory stage. Despite myriad proposed medical therapies, including numerous oral agents and local injection regimens, there have been limited advances. Surgery is typically reserved for patients who fail to respond to conservative treatment and have had no disease progression for at least several months. Penile lengthening procedures with different grafting materials have, to a great extent, replaced conventional procedures with penile plication or its modifications. Prosthesis insertion is reserved for patients with severe ED that does not respond to medical management. As the molecular mechanism of inflammation and wound healing is elucidated, new approaches for medical intervention, such as modification of fibroblast function, growth factor activity, and cytokine action, will no doubt be available for therapy of PD.
Coitus ; Congenital Abnormalities ; Disease Progression ; Fibroblasts ; Humans ; Inflammation ; Male ; Penile Induration* ; Penis ; Prostheses and Implants ; Psychology ; Transplants ; Vascular Diseases ; Wound Healing

We experienced a case of eisenmenger syndrome treated by simple surgical intervention. Eisenmenger syndrome refers to patients with congenital heart defects who have a systemic level of pulmonary arterial pressure and high pulmonary vascular resistance, with right-to-left or bidirectional shunting. Because a progressive rise in pulmonary vascular resistance may be better tolerated with an intracardiac communication, surgical repair in such patients may shorthen their life. But, urgent surgical intervention should be undertaken to reverse process if the pulmonary vascular disease is of recent origin. Patients who respond to vasoilator therapy, such as oxygen, are subjected to surgery. We reported a case of PDA with eisenmenger syndrome whose pulmonary vascular resistance fall following vasodilator, 100% oxygen, and closure of defect during cardiac catheterization, preoperatively, and who may faborable after simple surgical repair.
Arterial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Ductus Arteriosus, Patent* ; Eisenmenger Complex* ; Heart Defects, Congenital ; Humans ; Ligation* ; Oxygen ; Vascular Diseases ; Vascular Resistance