• Korean Journal of Radiology
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• v.20 no.4
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• pp.662-670
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• 2019

Objective: A developmental venous anomaly (DVA) is a vascular malformation of ambiguous clinical significance. We aimed to quantify the susceptibility of draining veins (χ_vein) in DVA and determine its significance with respect to oxygen metabolism using quantitative susceptibility mapping (QSM). Materials and Methods: Brain magnetic resonance imaging of 27 consecutive patients with incidentally detected DVAs were retrospectively reviewed. Based on the presence of abnormal hyperintensity on T2-weighted images (T2WI) in the brain parenchyma adjacent to DVA, the patients were grouped into edema (E+, n = 9) and non-edema (E-, n = 18) groups. A 3T MR scanner was used to obtain fully flow-compensated gradient echo images for susceptibility-weighted imaging with source images used for QSM processing. The χ_vein was measured semi-automatically using QSM. The normalized χ_vein was also estimated. Clinical and MR measurements were compared between the E+ and E- groups using Student's t-test or Mann-Whitney U test. Correlations between the χ_vein and area of hyperintensity on T2WI and between χ_vein and diameter of the collecting veins were assessed. The correlation coefficient was also calculated using normalized veins. Results: The DVAs of the E+ group had significantly higher χ_vein (196.5 ± 27.9 vs. 167.7 ± 33.6, p = 0.036) and larger diameter of the draining veins (p = 0.006), and patients were older (p = 0.006) than those in the E- group. The χ_vein was also linearly correlated with the hyperintense area on T2WI (r = 0.633, 95% confidence interval 0.333-0.817, p < 0.001). Conclusion: DVAs with abnormal hyperintensity on T2WI have higher susceptibility values for draining veins, indicating an increased oxygen extraction fraction that might be associated with venous congestion.

• The Journal of Korea Assosiation for Disability and Oral Health
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• v.6 no.1
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• pp.15-18
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• 2010

Sturge-Weber syndrome is a rare nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation involving the tissue of brain and face. The clinical features are characterized by port wine nevus following one or more divisions of trigeminal nerve, ocular involvement and neurologic involvement such as epilepsy, mental retardation, and contralateral hemiplegia. Oral manifestations include unilateral blood vessel expansion of the oral mucosa, vascular hyperplasia of gingiva, pyogenic granuloma-like massive hemangiomatous proliferation of oral mucosa, macrodontia, ipsilateral macroglossia, blood vessel anomaly of maxilla or mandible and abnormal tooth eruption sequence. This case report is about 11-year-old Sturge-Weber syndrome patient presented port wine nevus on the face, venous malformation on soft plate and buccal mucosa. In this case we performed simple extraction of several deciduous teeth and periodic oral hygiene management. If a patient with Sturge-Weber syndrome has to undergo dental surgery in affected areas of the mouth, great care must be taken to prevent severe hemorrhage.

• Journal of Chest Surgery
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• v.14 no.1
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• pp.40-48
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• 1981

Total anomalous venous return defines a group of congenital heart disease which have in common the entire pulmonary venous drainage returning directly or indirectly to the right atrium instead of to the left atrium. Despite of recent advance in treatment, this severe malformation in its various anatomical forms has a high surgical mortality during early infancy. Because of the high mortality in the untreated infant and the surgical risk in the first year of life, the timing of the operation remains important for optimal result. Three cases of T APV R, two supracardiac types and one mixed type, were treated with extracorporeal circulation during last three years in the Dept. of Thoracic and Cardiovascular Surgery, Seoul National University Hospital. The first one was 10 months old male with supracardiac type which drained through left innominate vein, and he was operated with profound hypothermia and total circulatory arrest but failed. The second case was 7 years old male with supracardiac type drained through left innominate vein, and he was well post operatively, and followed periodically for 12 months. The third case was 24 years old female with mixed type drainage (left upper pulmonary vein drained through left innominate vein, and the others through coronary sinus) was successfully corrected, and she was followed for 4 month without problem. All cases were diagnosed with cardiac catheterization and angiocardiogram, and also with echocardiogram in last two cases. In first two cases of supracardiac type, total circulatory arrest was used in brief period during anastomosis between common pulmonary venous trunk and left atrium. In the last case of mixed type, usual cardiopulmonary bypass with moderate hypothermia was used and total circulatory arrest was not needed.

• Archives of Plastic Surgery
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• v.48 no.6
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• pp.622-629
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• 2021

Background Venous malformations (VMs) are the most common type of vascular malformations. Intramuscular venous malformations (IMVMs) are lesions involving the muscles, excluding intramuscular hemangiomas. The purpose of this study was to compare clinical outcomes between patients with IMVMs who were treated with sclerotherapy and those who were treated with surgical excision. Methods Of 492 patients with VMs treated between July 2011 and August 2020 at a single medical center for vascular anomalies, 63 patients diagnosed with IMVM were retrospectively reviewed. Pain, movement limitations, swelling, and quality of life (QOL) were evaluated subjectively, while radiological outcomes were assessed by qualified radiologists at the center. Complication rates were also evaluated, and radiological and clinical examinations were used to determine which treatment group (sclerotherapy or surgical excision) exhibited greater improvement. Results Although there were no significant differences in pain (P=0.471), swelling (P=0.322), or the occurrence of complications (P=0.206) between the two treatment groups, the surgical treatment group exhibited significantly better outcomes with regard to movement limitations (P=0.010), QOL (P=0.013), and radiological outcomes (P=0.017). Moreover, both duplex ultrasonography and magnetic resonance imaging showed greater improvements in clinical outcomes in the surgical excision group than in the sclerotherapy group. Conclusions Although several studies have examined IMVM treatment methods, no clear guidelines for treatment selection have been developed. Based on the results of this study, surgical excision is strongly encouraged for the treatment of IMVMs.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.331-336
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• 1983

Cor triatriatum is a rare congenital malformation of the heart, in which a septum stretches in a transverse or oblique plane through the left atrium, thus separating it into two compartments. The upper one connects with the pulmonary veins, and the lower one connects with the left ventricle. Due to the rarity of, and great difficulty in-diagnosing, cor trlatrlatum, data On the surgery of this disease are of necessity very limited and so accurate pre-operative diagnosis was very difficult to make. `We experienced a case of the cyanotic congenital heart disease which was diagnosed as a large atrlal septal defect with streaming venous blood from inferior vena cava to left atrium through atrial septal defect in August, 1982. We found that there were transverse septum in the left atrium through atrlal septal defect, the pulmonary venous drainage were located in the upper chamber of the left atrium, and the lower chamber was connected with the left atrial appendage, mitral valve and `left ventricle. But our case had not any opening in this transverse septum and the right atrium was connected with the upper chamber of the left atrium through the upper part of the atrlal septal defect, and was communicated with the lower chamber of the left atrium through the lower part of the atrlal septal defect. We excised the transverse septum and repair this atrial septal defect with Woven Dacron patch accompanying with the drainage of coronary sinus to right atrium. The post-operative course was not eventful and he was discharged with good result on the post-operative 8th day, and has been in good .condition up to now for longer than 10 months.

• Journal of Chest Surgery
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• v.16 no.3
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• pp.362-367
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• 1983

Pulmonary arteriovenous fistula is a congenital malformation resulting from errant capillary development, with incomplete formation or disintegration of the vascular septa that normally divide the primitive connections between the venous and arterial plexuses. It generally occurs as part of the disorder known as hereditary hemorrhagic telangiectasia [Rendu-Osler-Weber disease]. The hereditary lesion is transmitted as a simple non-sex-linked dominant trait. It may be single or multiple, too small to see on plain chest films or large and easily recognized. One third of the lesions are multiple on plain chest film. The pathogenesis of its symptoms is that unoxygenated, desaturated arterial blood enters into the pulmonary venous system, directly. Recently we have experienced a case of the pulmonary arteriovenous fistula in 26 years old male soldier, which was confirmed by pulmonary angiography preoperatively. 2 thumb-tip sized, well circumscribed cystic masses filled with bright red colored blood were seen in subpleural and anterolateral portion of the right upper lobe. Right upper Iobectomy was performed due to close approximation of the fistula with pulmonary vein. Microscopically, it shows angiomatous dilatation of the abnormal vessels embedding in the parenchyma. Postoperative physiologic studies show nearly normal arterial oxygen saturation, hemoglobin and RBC count. There was good, uneventful postoperative course.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.34 no.6
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• pp.488-493
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• 2012

Hemangioma and vascular malformation are the most common benign tumors that are caused by congenitally or traumatic events. Theses tumors represent approximately 1/3~1/4 of all hemangiomas and vascular malformations in the head and neck. There are many forms of treatment for hemangioma and vascular malformation including closed observation, surgery, radiotherapy, laser therapy, steroid therapy, compression, embolization, and sclerotherapy. Ethanolamine oleate is an unsaturated fatty acid salt that has been used as a sclerosing agent because of its excellent thrombosing properties. This paper presents 1 case of intraoral multiple venous malformations treatment with 1.25% ethanolamine oleate (3.6~9.6 mg dose) intralesionally injected for 6 to 14 weeks over 2 week intervals. After the sclerotherapy, lesions almost completely disappeared without side effects. In conclusion, sclerotherapy using ethanolamine oleate is very effective against venous malformations, and sufficiently provides alternative support for surgical and other methods.

• Journal of Chest Surgery
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• v.14 no.4
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• pp.381-387
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• 1981

Fourteen Infants with congenital cardiac anomalies underwent primary surgical Intervention within the first 12 months of life. There were eight patients with ventricular septal defect, two with total anomalous pulmonary venous return [TAPVR], and the remainders with tetralogy of Fallot, transposition of great arteries [d-TGA], Taussing-Bing malformation, and coronary A-V fistula. The age of the patients ranged from 5 to 12 months, with a mean age of 9.9 months. The mean weight was 6.7 Kg [3.8 to 9.5 KS]. Congestive heart failure persisting despite intensive medical treatment was present In 8 patients [56%], and was the most common indication for operation. Early operation was necessary in 5 of these patients [35%], because of failure to thrive and recurrent pulmonary infection. In one patient with TOF, frequent hypoxic spell prompted the necessity for early operation. In cases of VSD, TAP. VR, TOF, and coronary A-V fistula, Intracardiac repair was done with conventional cardiopulmonary bypass, chemical cold cardioplegia, and topical myocardial cooling. Deep hypothermic circulatory arrest with surface induced cooling, followed by core cooling and core rewarming, was employed .for better exposure in the cases of d-TGA and Taussing-Bing malformation. The results were however, not satisfactory. The overall mortality was 28 per cent. There were no deaths in the eight patients with VSD. The one with coronary A-V fistula survived. The other 5 cases all expired either on the table or immediately after operation. The non-fatal post-operative complications included low cardiac output, respiratory insufficiency, bleeding, and temporary A-V block. The causes of death were prolonged circulatory arrest time in d-TGA, complete A-V block and low cardiac output in TOF and Taussing-Bing malformation and prolonged bypass time and Inadequate correction in TAPVR.

• Journal of Chest Surgery
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• v.14 no.4
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• pp.388-398
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• 1981

Fourteen Infants with congenital cardiac anomalies underwent primary surgical Intervention within the first 12 months of life. There were eight patients with ventricular septal defect, two with total anomalous pulmonary venous return [TAPVR], and the remainders with tetralogy of Fallot, transposition of great arteries [d-TGA], Taussing-Bing malformation, and coronary A-V fistula. The age of the patients ranged from 5 to 12 months, with a mean age of 9.9 months. The mean weight was 6.7 Kg [3.8 to 9.5 KS]. Congestive heart failure persisting despite intensive medical treatment was present In 8 patients [56%], and was the most common indication for operation. Early operation was necessary in 5 of these patients [35%], because of failure to thrive and recurrent pulmonary infection. In one patient with TOF, frequent hypoxic spell prompted the necessity for early operation. In cases of VSD, TAP. VR, TOF, and coronary A-V fistula, Intracardiac repair was done with conventional cardiopulmonary bypass, chemical cold cardioplegia, and topical myocardial cooling. Deep hypothermic circulatory arrest with surface induced cooling, followed by core cooling and core rewarming, was employed .for better exposure in the cases of d-TGA and Taussing-Bing malformation. The results were however, not satisfactory. The overall mortality was 28 per cent. There were no deaths in the eight patients with VSD. The one with coronary A-V fistula survived. The other 5 cases all expired either on the table or immediately after operation. The non-fatal post-operative complications included low cardiac output, respiratory insufficiency, bleeding, and temporary A-V block. The causes of death were prolonged circulatory arrest time in d-TGA, complete A-V block and low cardiac output in TOF and Taussing-Bing malformation and prolonged bypass time and Inadequate correction in TAPVR.

• Journal of The Korean Society of Integrative Medicine
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• v.9 no.4
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• pp.85-89
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• 2021

Background : Klippel-Trenaunay-Weber syndrome (KTS) is a rare congenital medical condition characterized by complex vascular malformation. KTS consists of a classic triad of capillary malformation (hemangioma), venous malformations and bone or soft tissue hypertrophy causing limb asymmetry. The aim of this report is to describe management for gait disturbance and foot pain in a Patient with KTS using custom-made total contact insole. Case presentation : A 32-year-old man with KTS presented with a 3-year history of gait disturbance on hard surface due to right first toe pain and Achilles tendon tightness. The patient had soft tissue hypertrophy, varicose veins and port-wine stains over the right lower limb associated with KTS. True leg length discrepancy was 2 cm. We prescribed custom-made total contact insole to protect his deformed foot and correct leg length discrepancy. The insole of right side included wedge shaped heel lift and the insole of left side included full length lift to add extra support on unaffected side. Also, we provided compression stocking and physiotherapy including manual lymphatic drainage for lymphedema and stretching exercise for tightness in right lower extremity. At 3 years follow-up, postural alignment including pelvic obliquity was improved using a custom-made total contact insole. The degree of scoliosis and foot pain were also reduced. Conclusion : An individualized and multidisciplinary approach is essential regarding the complexity of comorbidities in patients with KTS. For patients with KTS, orthotic management should be considered to prevent and correct deformities related to KTS. Active orthotic management, compression stocking and physiotherapy can enhance the quality of life and function in patients.