• Title/Summary/Keyword: Congenital defect

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Right Bochdalek hernia with pneumothorax in adult (성인에서 기흉을 동반한 우측의 Bochdalek Hernia 1)

  • 백광제
    • Journal of Chest Surgery
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    • v.17 no.4
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    • pp.729-734
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    • 1984
  • Bochdalek hernia is a type of congenital diaphragmatic defect in the posterolateral portion of the diaphragm. The defect is usually Lt. sided due to protective effect of liver on right. Sex distribution is male preponderance [2:1] and it is diagnosed during neonate, mostly first 24 hours, due to severe respiratory distress. We experienced a rare case of old aged female patient with congenital Bochdalek hernia on Rt. side which was found incidentally during treatment of spontaneous pneumothorax of Rt. side. 17 year old female patient was admitted to CS department for chest discomfort on right and mild dyspnea with duration of 20 days. Under the diagnosis of spontaneous pneumothorax, Rt. closed thoracostomy and underwater sealed drainage with continuous suction was applied. On follow-up chest x-ray, poorly defined hazy increased density with multiple air-fluid levels in Rt. lower lung field and Lt. subphrenic free air were noted. So, Barium enema was done under the impression of Rt. diaphragmatic hernia, and nearly entire colon proximal to sigmoid was demonstrated in the Rt. hemithorax. Operation was done-for surgical repair of defected diaphragm through Rt. posterolateral thoracotomy. Operative findings were as follows; 1.Hypoplastic Rt. lung, esp. RML & RLL. 2.Nearly entirely intestines were herniated. 3.Diaphragmatic defect was located on posterolateral portion of the diaphragm, about 10x3cm in size with blunt smooth margin. 4.A large bleb on apex of RUL of lung. Herniated intestines were repaired into abdominal cavity manually and defect of diaphragm was repaired with No. I black silk interrupted sutures directly, and bleb was resected. Postoperative courses were uneventful and the patient was discharged with good condition on POD 14th.

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A Case Report of Congenital Eyelid Defect without Any Other Deformities (다른 기형을 동반하지 않은 선천성 상안검 결손증 1례 보고)

  • Moon, Jae Won;Yang, Jeong Yeol
    • Archives of Craniofacial Surgery
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    • v.9 no.2
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    • pp.90-92
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    • 2008
  • Purpose: Congenital upper eyelid defect is rare anomaly whether it is compared with syndromic anomaly or not. It has many clinical manifestation in the extent, location. Many operation procedures such as simple closure, semicircular rotation flap, Cutler Beard procedure, lower lid rotation flap, etc can be used to reconstruct eyelid defects. We intend to introduce a simple, congenital eyelid defect which was not compared with syndromical anomaly, ophthalmic complication. Methods: Our experience is a case of 19 years old female who had a upper eyelid coloboma without any other anomalies. we could not find any skeletal deformity in orbital CT scan. she had no ophthalmic problem. we reconstructed the defect with bilateral marginal flap after deepithelization of supramarginal area and tarsal reposition. Results: There were no visible deformity of lid lining. postoperative scar was favorable. satisfactory results were obtained in cosmetic and functional aspects. Conclusion: Upper lid coloboma without other anomalies is rare. we obtained satisfactory outcome as treated this rare case with marginal flap advancement.

Congenital Cardiovascular Anomalies in Adults : A report 266 surgical cases (성인 심장기형 266예 수술 예)

  • Lee, Yung-Kyoon;Yang, Gi-Min
    • Journal of Chest Surgery
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    • v.13 no.4
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    • pp.414-417
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    • 1980
  • During the period from 1958 to July 31, 1980, there were 1764 Cardiovascular Surgical cases consisting of 1166 congenital and 598 acquired lesions in the Department of Cardiothoracic Surgery, Seoul National University Hospital. For 1764 cases 1162 open heart surgery and 602 conventional operations were performed. Among 1166, congenital lesions 266 adult cases were found. Adult cases occupy 15.1% of the total cases consisting of 24.5% of acyanotic and 19.9% of cyanotic group. Over all operative mortality of adult cases was 6.8% compared with 12.4% of total cases. Left over congenital cardiovascular anomalies in Korea can be safely operated at the present time. The oldest operated patient among adult congenital cardiovascular anomalies was 57 years old female atrial septal defect case. For 1162 cases open heart surgery bubble oxygenators were utilized except 2 membrane oxygenators. Various kinds of bubble oxygenators have been used but recently Shiley**** oxygenators are being used exclusively with satisfactory results.

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One-Stage Repair of an Interrupted Aortic Arch with an Aortopulmonary Window in a Premature Neonate

  • Bobos, Dimitrios;Kanakis, Meletios A.;Koulouri, Sofia;Giannopoulos, Nicholas M.
    • Journal of Chest Surgery
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    • v.48 no.6
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    • pp.411-414
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    • 2015
  • Interrupted aortic arch with an aortopulmonary window is a rare congenital entity that is associated with high morbidity and mortality, especially in premature low-birth-weight infants, and the proper timing of surgical correction remains a matter of debate. We present the case of a premature infant weighing 1.6 kg who successfully underwent one stage surgical repair to treat interrupted aortic arch with an aortopulmonary window. The therapeutic management of this patient is described below, and a review of the literature is presented.

Congenitally Corrected Transposition of the Great Arteries associated with Ventricular Septal Defect and Patent Ductus Arteriosus - One case report - (심실중격결손증 및 동맥관개존증을 동반한 선천성 교정형 대혈관전위증 1례 보고)

  • 장동철
    • Journal of Chest Surgery
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    • v.20 no.4
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    • pp.786-792
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    • 1987
  • Congenitally corrected transposition of the great arteries is a congenital cardiac anomaly with ventriculoarterial discordant connection and atrioventricular discordant connection. This report describes a 17 year old male patient who had congenitally corrected transposition of the great arteries associated with ventricular septal defect and patent ductus arteriosus, underwent patch closure of the ventricular septal defect and suture closure of patent ductus arteriosus, and was discharged on 9th day after surgery with good condition.

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Open Heart Surgery with Pulmonary Resection (개심술과 폐엽절제술의 동시 수술 경험)

  • 이용재
    • Journal of Chest Surgery
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    • v.26 no.3
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    • pp.234-235
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    • 1993
  • We have experienced a case of congenital heart disease who developed pulmonaryaspergilloma and then had open heart surgery associated with pulmonary resection. A 53 year old female patient was admitted of fever and chill without cyanosis and hemoptysis. Chest CT showed cavitary lesion with enhanced wall in right midle lung and huge pulmonary artery. Secundum atrial septal defect was identified by echocardiography and catheterization, preoperatively. The patient was identified finally as atrial septal defect associated with pulmonary aspergilloma, in operation and pathology.

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Transaortic Closure of Ventricular Septal Defect in Corrected Transposition of the Great Arteries (심실중격 결손 및 폐동맥협착이 동반된 선천성 교정형 대혈관 전위증 - 대동맥 절개를 통한 심실중격 결손의 봉합 치험 1례 -)

  • 전예지
    • Journal of Chest Surgery
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    • v.23 no.4
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    • pp.764-768
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    • 1990
  • Congenitally corrected transposition of great arteries is a congenital cardiac anomaly with ventriculoarterial discordant connection and atrioventricular discordant connection. A 8-year-old girl had congenitally corrected transposition of the great arteries with ventricular septal defect and pulmonary valvular stenosis. By transaortic approach, ventricular septal defect was closed and pulmonary valvotomy was performed by transpulmonary approach. No heart block or aortic insufficiency developed postoperatively.

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Open heart surgery in the first two years of life (2 세 이하 유아기의 개심술)

  • Park, Lee-Tae;Seo, Gyeong-Pil
    • Journal of Chest Surgery
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    • v.16 no.4
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    • pp.431-443
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    • 1983
  • A hundred and fifty-one patients with congenital heart disease less than 24 months old underwent intracardiac repairs from January 1982 to July 1983, which consists 24.2% of all the patients with congenital heart diseases operated during the same period. There were 98 patients[64.9%] with acyanotic congenital heart disease and 53 patients[35.1%] with cyanotic congenital heart disease, and 55 patients[36.4%] were less than 1 year of age. Twenty-two patients died within 30 days after surgery and 3 patients died after postoperative 30th day: Ventricular septal defect, four of 90 patients; Tetralogy of Fallot, five of 23 patients; Transposition of great arteries, nine of 17 patients; Tricuspid atresia, four of 5 patients; Pulmonary atresia, all of 2 patients; Single ventricle, one of single patients. Over all mortality was 16.6% and mortality of acyanotic congenital heart disease, cyanotic congenital heart disease and patients less then 1 year of age was 4.1%, 39.6% and 20.0% respectively. Still the mortality of cyanotic congenital heart disease is high. Careful preoperative evaluation of the detailed intracardiac anatomy and hemodynamics of the patients and proper selection of surgical treatment yield better clinical results.

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A Case of Congenital Intrahepatic Portosystemic Shunt Associated with VSD Detected by Antenatal Sonography and Treated with Four Coil Embolizations and Open Heart Surgery after Birth (산전 초음파로 발견되고 출생 후 4회의 코일 색전술과 심 교정술로 치료된 심실 중격 결손을 동반한 선천성 간내 문맥전신성 단락 1례)

  • Na, Ji-Youn;Kim, Eun-Sun;Kim, Sang-Duk;Kim, Ee-Kyung;Kim, Han-Suk;Choi, Jung-Hwan;Chung, Jeong-Eun;Joung, Jin-Uuk
    • Neonatal Medicine
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    • v.15 no.2
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    • pp.176-182
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    • 2008
  • A congenital portosystemic shunt is a very rare portosystemic vascular anomaly which leads to jaundice, hypoglycemia, hyperammonemia, liver cirrhosis, hepatic coma, and pulmonary hypertension. Anatomically, portosystemic shunts are divided into intra- and extrahepatic shunts. Congenital intrahepatic portosystemic shunts are rare anomalies, and the early diagnosis is important to prevent hepatic encephalopathy and hypoglycemia. We report a case of an infant with symptoms of heart failure due to a congenital intrahepatic portosystemic shunt and a ventricular septal defect (VSD), which were treated successfully with four coil embolizations and open heart surgery for the VSD.

Congenital Bochdalek hernia: report of 4 cases (선천성 Bochdalek hernia4례 보고)

  • Jin, Jae-Gwon;Park, Ju-Cheol;Yu, Se-Yeong
    • Journal of Chest Surgery
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    • v.15 no.4
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    • pp.432-439
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    • 1982
  • Congenital posterolateral diaphragmatic hernia [Bochdalek hernia] is the result of a congenital diaphragmatic defect in the posterior costal part of the diaphragm in the region of the tenth and eleventh ribs. There is usually free communication between the thoracic and abdominal cavities. The defect is most commonly found on the left [90%], but may occurs on the right, where the liver often prevents detection. The male to female ratio is 2:1. Owing to the negative intrathoracic pressure, herniation of abdominal contents through the defects occurs, with resultant collapse of the lung. Shifting of mediastinum to the opposite side and compression of the opposite lung occurs. Most often these hernias are manifestated by acute respiratory distress in the newborn. A second, but less well recognized, group of patient with Bochdalek hernia survive beyond the neonatal period, usually present at a later time with "failure of thrive, intermittent vomiting, or progressive respiratory difficulty. " The diagnosis can often be made on clinical ground from the presence of respiratory distress, absence of breath sounds on the chest presence of bowel sounds over the chest . Roentgenogram of the chest confirm the diagnosis. Obstruction and strangulation have been reported but are rare. Treatment consists of early reliable identification of these congenital diaphragmatic hernia with high risk and surgical repairment. and postoperative pharmacological management with extracorporeal membranous oxygenation [=ECMO] support in the period of intensive care. On the surgical approach, for defects on left side, an abdominal incision is preferred, because of the high incidence of malrotation and obstructing duodenal bands. In the neonate, the operative mortality may be appreciable, but, later repair almost always is successful. During the period from 1972 to 1982, 4 cases of congenital Bochdalek hernia were experienced at the Kyung-Hee University Hospital.

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