• Title/Summary/Keyword: Congenital defect

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Serial Reconstruction Considerating the Aesthetic Unit on Congenital Giant Nevus in Periorbital Area (미용단위를 고려한 안와 주변부의 선천성 거대모반의 단계적 치료)

  • Jo, Sung-Hyun;Kim, Jin-Woo;Jung, Jae-Hak;Kim, Young-Hwan;Sun, Hook
    • Archives of Plastic Surgery
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    • v.37 no.4
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    • pp.465-468
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    • 2010
  • Purpose: Soft tissue deformity and skin defect after tumor resection in the periorbital area can cause trouble in the function of eyelid as well as in the aspect of external appearance. Therefore, as cosidering reconstruction in periorbital area, detailed assessment of both functional and aesthetic property are required. thus, the purpose of this study is to examine an appropriate reconstruction through clinical cases. Methods: A 14-year-old girl with congenital giant hairy nevus on right periorbital area was selected. Her first visit to our plastic surgery outpatient clinic was on July 2006. Since then, she has undergone staged removal of lesions and reconstruction by various flap technique such as pedicled island flap, forehead galeal flap, paramedian forehead flap, cheek rotation & advancement flap. Results: In the case of this girl, most lesions were removed and replaced by normal skins. Although there was the difference of skin color after skin graft, such difference was not noticeable and section scar by skin flaps was slight. There was no obvious dysfunction in the eyelids and the girl and her parents were satisfied with results after the surgery. Conclusions: In the reconstruction of soft tissue defect or soft tissue deformity and contracture, it is required to choose appropriate reconstruction method, considering aesthetic and functional aspects depending on aesthetic unit sufficiently.

Usefulness of using Tissue Expander in Pediatric Patient (소아 환자에서 조직확장기 사용의 유용성)

  • Lee, Jun-Woo;Park, Chul-Gyoo;Park, Jong-Lim;Kim, Yong-Kyu
    • Archives of Plastic Surgery
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    • v.37 no.6
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    • pp.763-768
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    • 2010
  • Purpose: Reconstruction of soft tissue defect using tissue expander can provide better flap which is more similar to surrounding tissue in color, skin texture and hair compared to other methods. Many pediatric patients need reconstruction of soft tissue defect because of giant congenital nevi, congenital or acquired malformations and burn scars. Reconstruction using tissue expander is adequate to minimize dysmorphism in these patients. We intended to assess outcomes of using tissue expander in pediatric patients by retrospective study. Methods: Total cases were 168 of pediatric patients who received soft tissue reconstruction using tissue expander by the same surgeon from February, 1982 to May, 2009. All patients who received soft tissue reconstruction were under 10 years old. Mean age was 4.3 years old, the youngest 13 months, the oldest 8 years. Eightynine cases were male and 79 cases were female. Most common cause was giant hairy nevi (67 cases, 39.9%), secondary cause was burn scar/scar contracture (61 cases, 36.3%). Trunk (38 cases, 22.6%) was most common anatomical location. Results: Soft tissue defects were successfully covered using tissue expander in 149 cases (88.7%) without major complications. There was infection on 8 cases (4.7%) and we treated by adequate antibiotics in these cases. There were tissue expander folding or valve displacement on 5 cases (3%). Conclusion: Usage of tissue expander is useful on pediatric patients because tissue expansion is rapid on children and there are less secondary contractures on operation site than full thickness skin graft. Because of psychological stress due to tissue expander, operation should be performed before school age.

Incidentally Detected Pericardial Defect in a Patient with Pneumothorax as Confirmed on Video-Assisted Thoracoscopic Surgery (흉강경 수술로 확인한 우연히 발견된 기흉을 동반한 심막결손)

  • Hyunwoo Cho;Eun-Ju Kang;Moon Sung Kim;Sangseok Jeong;Ki-Nam Lee
    • Journal of the Korean Society of Radiology
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    • v.82 no.3
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    • pp.749-755
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    • 2021
  • Congenital defects of the pericardium, which are generally asymptomatic, are rare disorders characterized by complete or partial absence of the pericardium. Here, we report a rare case of a 19-year-old male who was incidentally diagnosed with congenital absence of the left pericardium during examination for symptoms of pneumothorax. Chest radiography and CT revealed a collapsed left lung without any evidence of trauma, no unusual findings of free air spaces along the right side of the ascending aorta, heart shifted toward the left side of the thorax, and a shallow chest. Subsequent thoracoscopy confirmed the absence of the left pericardium and displacement of the heart toward the left thoracic cavity. We further discuss the correlation between radiologic images and surgical findings of a congenital pericardial defect associated with spontaneous pneumothorax.

Outcomes after repair of complete atrioventricular canal with a modified single-patch technique: a retrospective study

  • George Samanidis;Konstantinos Kostopanagiotou;Meletios Kanakis;Georgios Kourelis;Kyriaki Kolovou;Georgios Vagenakis;Dimitrios Bobos;Nicholas Giannopoulos
    • Journal of Yeungnam Medical Science
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    • v.40 no.2
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    • pp.187-192
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    • 2023
  • Background: This study aimed to present the short- and midterm outcomes after complete atrioventricular canal defect (CAVC) repair using a single-patch technique. Methods: This study included 30 children who underwent surgical correction of the CAVC using a single-patch technique. Results: The median age of the patients was 5.7 months (interquartile range [IQR], 5.0-7.5 months), and 23 patients (76.7%) had type A CAVC. Fourteen patients (46.7%) were female and 17 (56.7%) had been diagnosed with Down syndrome. The in-hospital mortality rate was 0%. No deaths were observed during a median follow-up of 4 years (IQR, 3.5-5.0 years). Patients without Down syndrome were associated with late moderate mitral regurgitation (MR) (p=0.02). Late MR less than moderate degree was observed in 96.6%, 78.5%, and 50% of patients after 2, 4, and 5 years of follow-up, respectively, while late tricuspid valve regurgitation less than moderate degree was observed in 96.7%, 85.9%, and 59.0% of patients after 2, 4, and 6 years of follow-up, respectively. After a median follow-up of 4 years, only one patient had required surgical repair of a left ventricular outflow tract obstruction, which occurred 26 months after the first operation. Multivariable logistic regression analysis adjusted for the type of CAVC, sex, Down syndrome, age, and weight revealed that the absence of Down syndrome was a risk factor for late moderate MR (MR-2) (odds ratio, 0.05; 95% confidence interval, 0.006-0.50; p=0.01). Conclusion: A single-patch technique for CAVC surgical repair is a safe method with acceptable short- and midterm results.

Truncus Arteriosus, Type IV -one case report- (동맥간증 제 IV형 -1예 보고-)

  • 이종태
    • Journal of Chest Surgery
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    • v.13 no.3
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    • pp.243-249
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    • 1980
  • Truncus ateriosus is one of the cyanotic congenital heart disease. The incidence is relatively uncommon, as 0.4% of totoal congenital heart disease. Embryologically the defect is due to a lack of partitioning of the embryonic truncus and conus during the first few weeks of fetal life. The ventricular septal defect is invariable present. A single arterial vessel arises from the heart and supplies blood to the aorta, the lung, and the coronary arteries. In 1949, collett and Edwards classified this defect according to anatomic variation to four major types, such as type I, II, III, and IV. Type IV is defined that pulmonary arteries are absent, and the pulmonary arterial supply arises from the descending thoracic aorta. This patients often have a continuous murmur head particularly well in the interscapular area. No effective surgical treatment is available. We have experienced one case of truncus arteriosus, type IV of Collett and Edwards in the Department of Thoracic and Cardiovascular Surgery, Kyungbook National University Hospital. This patient was 10 year-old girl. The chief complaints were cyanosis and dyspnea on exertion since birth. She was admitted at this hospital on April 16, 1980. The continous machinery murmur was heard loudest at the interscapular area. The chest X-ray films revealed cardiomegaly with an increase in pulmonaryvascular markings. The pulmonary secotr was significantly concave. No filling of pulmonary arteries noticed by the right ventriculogram. There was possible biventricular hypertrophy in EKG. The echocardiogram showed that the demension of the aortic root was larger than normal and minimal increase of the left ventricular internal dimension. The cardiac catheterization data was obtained by use of the great saphenus vein approach. The systolic pressure of the right ventricular outflow tract was 80 mmHg and was similar to that of the aorta. The oxygen saturation data revealed the evidence of the left to right shunt at the level of ventricular septum. The patient was operated and the diagnosis was confirmed as trucus arteriosus, type IV. No effective surgical interventins were performed.

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Surgical Treatment of Atrial Septal Defect in Adult - Clinical Review of 31 Cases - (성인의 선천성 심방중격결손증의 외과적 치료)

  • 장운하;오태윤;배상일
    • Journal of Chest Surgery
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    • v.31 no.8
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    • pp.770-775
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    • 1998
  • Background: Atrial septal defect (ASD) is the most common congenital cardiac anomaly, accounting for 30 percent of congenital heart disease detected in the adult. Many patients with ASD are well tolerated and reach adult without significant symptoms. The patients with ASD die 4th and 5th decades, but prolonged survival is not uncommon. In general, the survival depends on whether pulmonary hypertension develops during adulthood or not. The most common cause of death in the patients with ASD is right ventricular failure or arrhythmias. Materials and methods: From January 1988 to June 1997, 33 cases of ASD underwent open heart surgeries in our hospital. Among them, 31 cases were adult ASD, and 2 tricuspid regurgitation, 1 pulmonic stenosis, 1 mitral regurgitation, 1 tricuspid regurgitation, and 1 coronary artery disease were combinded. All of the patients underwent surgical repair using autologus pericardial patch or direct closure. Results: The postoperative course was smooth and uneventful. Most of the patients showed significant improvement in ECG finding, hemodynamic profile, radiologic finding, and echocardiography, after surgery. Conclusions: Conclusively, most of the ASD should be closed even in patients over the age of 60 years, and early surgical repair must be done to prevent pulmonary hypertension, right ventricular failure, and arrythmias.

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Identification of 1p36 deletion syndrome in patients with facial dysmorphism and developmental delay

  • Seo, Go Hun;Kim, Ja Hye;Cho, Ja Hyang;Kim, Gu-Hwan;Seo, Eul-Ju;Lee, Beom Hee;Choi, Jin-Ho;Yoo, Han-Wook
    • Clinical and Experimental Pediatrics
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    • v.59 no.1
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    • pp.16-23
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    • 2016
  • Purpose: The 1p36 deletion syndrome is a microdeletion syndrome characterized by developmental delays/intellectual disability, craniofacial dysmorphism, and other congenital anomalies. To date, many cases of this syndrome have been reported worldwide. However, cases with this syndrome have not been reported in Korean populations anywhere. This study was performed to report the clinical and molecular characteristics of five Korean patients with the 1p36 deletion syndrome. Methods: The clinical characteristics of the 5 patients were reviewed. Karyotyping and multiplex ligation-dependent probe amplification (MLPA) analyses were performed for genetic diagnoses. Results: All 5 patients had typical dysmorphic features including frontal bossing, flat right parietal bone, low-set ears, straight eyebrows, down-slanting palpebral fissure, hypotelorism, flat nasal roots, midface hypoplasia, pointed chins, small lips, and variable degrees of developmental delay. Each patient had multiple and variable anomalies such as a congenital heart defect including ventricular septal defect, atrial septal defect, and patent duct arteriosus, ventriculomegaly, cryptorchism, or hearing loss. Karyotyping revealed the 1p36 deletion in only 1 patient, although it was confirmed in all 5 patients by MLPA analyses. Conclusion: All the patients had the typical features of 1p36 deletion. These hallmarks can be used to identify other patients with this condition in their early years in order to provide more appropriate care.

Congenital Partial Pericardial Defect Presenting as Chest Pain (가슴 통증을 동반한 선천성 부분 심낭 결손증)

  • Kim, Yong-Ho;Kang, Min-Woong;Lim, Seung-Pyung;Lee, Young;Gil, Hong-Ryang;Yu, Jae-Hyeon
    • Journal of Chest Surgery
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    • v.40 no.10
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    • pp.719-721
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    • 2007
  • Patients with congenital pericardial defect display few symptoms and this is an unusual disease, so it is generally found incidentally in most of the cases. In some cases, symptoms such as chest pain are found due to partial cardiac herniation. We report here on a 14-year-old girl with dyspnea and chest pain that started during physical activity 3 months before the hospital visit. She was diagnosed with herniation of the left atrial appendage with a partial pericardium defect and she was treated with an operation.

Various Methods of Reconstruction in Nasal Defect (코 결손 부위에 따른 다양한 재건)

  • Kim, Seok Kwun;Yang, Jin Il;Kwon, Yong Seok;Lee, Keun Cheol
    • Archives of Craniofacial Surgery
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    • v.11 no.1
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    • pp.13-18
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    • 2010
  • Purpose: Nasal defect can be caused by excision of tumor, trauma, inflammation from foreign body reaction. Nose is located in the middle of face and protruded, reconstruction should be done in harmony with size, shape, color, and textures. We report various methods of nasal reconstruction using local flaps. Methods: From March 1998 to July 2008, 36 patients were operated to reconstruct the nasal defects. Causes of the nasal defects were tumor (18 cases), trauma (11 cases), inflammation from foreign body reaction (5 cases) and congenital malformation (2 cases). The sites of the defects were ala (22 cases), nasal tip (8 cases) and dorsum (6 cases). The thickness of the defects was skin only (5 cases), dermis and cartilagenous layer (7 cases) and full-thickness (24 cases). According to the sites and thickness of the defects, various local flaps were used. Most of alar defects were covered by nasolabial flaps or bilobed flaps and the majority of dorsal and tip defects were covered by paramedian forehead flaps. Small defects below $0.25 cm^2$ were covered with composite graft or full-thickness skin graft. Results: The follow-up period was 14 months. Partial flap necrosis was observed in a case, and one case of infection was reported, it was improved by wound revision and antibiotics. Nasal reconstruction with various local flaps could provide satisfactory results in terms of color and texture match. Conclusion: The important factors of nasal reconstruction are the shape of reconstructed nose, color, and texture. Nasolabial flap is appropriate method for alar or columellar reconstruction and nasolabial island flap is suitable for tip defect. The defect located lateral wall could be reconstructed with bilobed flap for natural color and texture. Skin graft should be considered when the defect could not afford to be covered by adjacent local flap. And entire nasal defect or large defect could be reconstructed by paramedian forehead flap.

Cor triatriatum with left superior vena cava[Report of a case] (좌측 상공정맥을 동반한 삼심방증 1례 보고)

  • 박병순
    • Journal of Chest Surgery
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    • v.18 no.2
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    • pp.293-298
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    • 1985
  • Cor triatriatum is a rare congenital malformation of the heart in which a septum stretches in a transverse plane through the left atrium, thus creates two left atrial subchambers. The upper one connects with the pulmonary veins, and the lower connects with the left ventricles. Due to the rarity of, and difficulty in diagnosing car triatriatum, data on the surgery of the disease are of necessity and very limited. A case of cor triatriatum combined with atrial septal defect and persistent left superior vena cava was experienced in November, 1984 in Chonnam University Medical School. There was a transverse septum in the left atrium below atrial septal defect, all pulmonary veins were drained into the upper chamber of the left atrium which connected with the right atrium via atrial septal defect and the lower chamber via an oval opening[8mm] in the abnormal septum and the lower chamber was connected with the left atrial appendage, and the left ventricle via mitral valve. There was persistent left superior vena cava drained to left atrium and coronary sinus. The abnormal transverse septum within the left atrium was completely excised and the atrial septal defect was repaired with Woven Dacron patch. The post-operative course was not eventful and the patient was discharged to home with good result on the 15th postoperative day, and has been in good condition upto now.

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