• Title/Summary/Keyword: 선천성 기형

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Congenital Cystic Adenomatoid Malformation(CCAM) (선천성 낭포성 유선종 기형)

  • 김수원
    • Journal of Chest Surgery
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    • v.21 no.6
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    • pp.1084-1094
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    • 1988
  • Conservative management of 3 iatrogenic perforations of intrathoracic esophagus was reviewed. The primary disorders were achalasia in 2 patients and congenital tracheoesophageal fistula in 1 patient. Perforation occurred after treatment of the primary disorders in the distal esophagus in 2 patients and mid-thoracic esophagus in 1 patient. All the perforations appeared late after the previous treatments and the inflammation spread to mediastinum and pleural cavity in all the 3 patients. Conservative management of esophageal perforation was carried out with intraluminal drainage from the perforated site of esophagus[insertion of Levin`s tube and continuous suction], pleural drainage and feeding of liquid diet through gastrostomy tube with Fowler`s position. The patients revealed spontaneous closure of perforated sites about 3 to 4 weeks after this conservative management without open thoracotomy. This result suggests that this conservative management may be accepted as therapeutic method in the thoracic esophageal perforations regardless of cause and time of the perforation.

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Congenital cystic adenoid malformation: report of 2 cases (선천성 낭종성 선종양 기형 2례 보)

  • Jin, Seong-Hun;Kim, Ju-Hyeon
    • Journal of Chest Surgery
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    • v.17 no.2
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    • pp.326-330
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    • 1984
  • Congenital Cystic Adenomatoid Malformation [C.C.A.M], one of lung bud anomalies, is an unusual lesion, only about 200 cases baring been reported by 1980, and characterized by marked proliferation of terminal respiratory structures. Recently we experienced two infants with C.C.A,M., whose clinical courses were quite different. On case 1, the patient was 25-day-old female, and suffered from progressive respiratory distress for 10 days duration. A right middle lobectomy was performed, with a satisfactory postoperative course. On case II, the patient was 7omonth-old male, and admitted for evaluation of known pulmonary anomaly, which was detected for the first time during hospitalization for treatment of pneumonia at 1 month of his age. He underwent a lingular segmentectomy, but died of respiratory insufficiency on postop 10th day. We believe that awareness of the presence of C.C.A.M. is important in making the differential diagnosis of progressive respiratory disease or of recurrent pulmonary infection in infants.

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Surgical Result of Congenital Mitral Regurgitation in Children (선천성 승모판폐쇄부전증 교정수술의 단기성적)

  • 홍유선;박영환
    • Journal of Chest Surgery
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    • v.30 no.4
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    • pp.373-377
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    • 1997
  • Between January, 1991 and May 1995, mitral valve repair was undertaken on 32 patients under 15 years for congenital mitral regurgitation. Mean age was 24.0$\pm$26.1 months(range 3 months to 15 years), 16 patients were male and 16 patients were female associated cardiac anomalies were found in 26 patients (81%), and ventricular septal defects were noted in 18 patients(56%). In regards to pathologic findings, there were annular dilatation(n:7), leaflet prolapse(n=18), cleft leaflet(n=5) and restricted valve motion (n=2). The method of repair consisted of annuloplasty(Modif ed Devega type) in 14, repair of redundunt leaflet in 6, closure of cleft in 5, triangular resection in 2 and splitting of papillary muscle in one. There was no operative mortality and two late deaths occurred as a result of heart failure and sepsis. Tro patients required replacement of the mitral valve after 3 months and 7 months respectivehy because of recurrent mitral regurgitation. Actuarial survival was 92.5% at 46 months and actuarial freedom from reoperation was 95% at 12 months and 92.5% at 46 months. Actuarial freedom from valve repair failure was 68% at 12 months and 61.8% at 46 months. Although valve repair failure rate was high, we believe that mitral valve repair should be of rcrred to children because of low mortality and low reoperation rate.

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One Stage Total Repair of the Aortic Arch Anomaly using the Regional Perfusion (대동맥궁 이상이 동반된 선천성 심장병에서 국소 순환을 이용한 일차 완전 교정)

  • Jang Woo-Sung;Lim Cheong;Lim Hong-Kook;Min Sun-Kyung;Kwak Jae-Kun;Chung Eui-Seuk;Kim Dong-Jin;Kim Woong-Han
    • Journal of Chest Surgery
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    • v.39 no.6 s.263
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    • pp.434-439
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    • 2006
  • Background: Deep hypothermic circulatory arrest during repair of aortic arch anomalies may induce neurological complications or myocardial injury. So we surveyed if the regional cerebral and myocardial perfusion might eliminate those potential side effects. Material and Method: From March 2000 to December 2004, 62 neonates or infants with aortic arch anomaly underwent one stage biventricular repair using the regional perfusion technique by single surgeon. Preoperative diagnosis of the arch anomaly consisted of coarctation (n=46), interruption of the aorta (n=12), hypoplastic left heart syndrome (n=2) and truncus areteriosus (n=2). Combined anomalies were ventricular septal defect (n=51), TAPVR (n=1), PAPVR (n=1) and atrioventricular septal defect (n=2). Arterial cannula was inserted at the innominate artery. Result: The mean regional perfusion time of brain was $28{\pm}10min$. Operative mortality rates was 0 (0/62). Late death was 1 (1/62) during $11{\pm}7$ months of follow-up. Neurologic complications consisted of transient chorea in 1 case. There was no reoperation associated with arch anolamy. Pulmonary complication associated with arch repair occurred in f case which was managed by aortopexy. Conclusion: One-tage rch repair using the regional profusion is safe and effective in minimizing the neurologic and myocardial complications.

A Case of Late Presentation of Congenital Cystic Adenomatoid Malformation of the Lung (14세 여아에서 발견된 선천성 낭종성 선종성 기형 1예)

  • Lee, Myung In;Sohn, So Hee;Lee, Dae Joon;Ha, Dong Yul;Jee, Young Koo;Lee, Kye Young;Kim, Keun Youl;Choi, Young Hi;Cho, Jeong Hi;Seo, Pil Weon;Kim, Sam Hyun
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.5
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    • pp.805-811
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    • 1996
  • Congenital cystic adenomatoid malformation of the Lung(CCAM) is characterized by anomalous fetal development of terminal respiratory structures, resulting in an adenomatoid proliferation of bronchiolar elements and cystic formation. CCAM was first described and differentiated from other cystic lung disease in the English literature by Ch'in and Tang in 1949. CCAN is a rare, potentially lethal form of congenital pulmonary cystic disease and the salient features of lesion are an irregular network of terminal respiratory bronchiole-like structures and macrocysts variably lined by pseudostratified ciliated columnar epithelium and simple cuboidal epithelium. Adult presentation of CCAM of the lung is so rare that only 9 cases have been reported in the literature of date. The pathogenesis of CCAM remains disputed and reseachers have variously proposed that the lesion represents a developmental anomaly, hamartoma, or a fonn of pulmonary dysplasia. Van Dijk and Wagenvoort divided CCAM into three subtypes : cystic, intermediated, and solid. These correspond to types I, II, and III of Stocker. In adults, the evaluation of cystic or multi cystic lung disease requires consideration of a differential diagnosis including the acquired lesions of lung abscess, cavitary neoplasm or inflammatory mass, bullous disease, bronchiectasis, and postionflammatory pneumatocele. Congenital lesions such as sequestration, bronchopulmonary-foregut anomalies, and bronchogenic cyst are also encounted. The definitive treatment for CCAM is complele removal of the involved lobe. Panial lobectomy leads to multiple complications, including severe post-operative infection. We report a case of CCAM in a 14-year-old female presentated with a pneumothorax and large bullae, who was treated by surgical remove of the involved lobe.

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Simultaneous Repair of Secondary Anterior Chest Wall Deformity and Secundum Atrial Septal Defect -1 Case Report- (심방중격결손을 동반한 이차성 전흉벽기형의 동시교정 -1예 보고-)

  • 김용희;정종필
    • Journal of Chest Surgery
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    • v.30 no.12
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    • pp.1247-1250
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    • 1997
  • A 13-year-old boy presented with anterior chest wall depression and dyspnea on exertion(NYHA II). He underwent Ravitch operation for pectus excavatum 7 years ago. A preoperative echocardiographic study revealed secundum atrial septal defect. He had no other abnormality of laboratory test, except FVC and FEVI were decreased into 2.03 L(7 %) and 1.82 L(71 %). He underwent repair of secondary anterior chest wall deformity and secundum atrial septal defect. We used unique method, raising sternum at right angle to secure good operative field for open heart surgery. Acute respiratory insufficiency was developed on postoperative day 1. Mechanical ventilation was applied which could be weaned on postoperative day 6 and thereafter hospital course was uneventful without any other sequale. He was discharged on postoperative day 19.

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Surgical Role of the Treatment of the Patients with Infective Endocarditis in Childhood (아동기 전염성 심내막염 환자치료의 외과적 역할)

  • 김경환;서경필
    • Journal of Chest Surgery
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    • v.30 no.12
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    • pp.1175-1183
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    • 1997
  • Background. Infective endocarditis s an uncommon but serious disease in children. Optimal treatment strategy, especially surgical indications, continues to evolve. Method. In this report, we retrospectively reviewed 19 patients who underwent operations in Seoul National University Children's Hospital for infective endocarditis between September 1986 and February 1996. There were 8 male and 11 female patients(mean age 70.6 months) and their main symptoms were fever and dyspnea. Preoperative echocardiography detected vegetation in 14 cases out of 19. Congenital anomalies were in 15 cases and acquired forms were in 4 cases. Causative organisms were identified in 12 cases and Gram-positive cocci were in 7 cases. Adequate preoperative antibiotic management was done. Result. With cardiopulmonary bypass in all cases, surgical corrections including vegetation removal were performed. 4 patients died after operations(21.1%). All the mortality cases were having underlying complex cardiac anomalies. There were no late deaths. Act arial survival rate was 79.0% at 1 year and 79.0% at 5 year and reoperationfree survival rate was 73.7% at 1 year and 73.7% at 5 year. Collclusion. Despite advances in antibiotic therapy, early surgical treatment is imperative in a significant subset, and concurrent intracardiac repair should be appropriate.

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Modified Starnes Operation for Neonatal Stenotic Ebstein Anomaly (신생아 폐쇄성 엡스타인 기형에 대한 변형 Starnes 술식)

  • Lee Seung Hyun;Yun Tae-Jin
    • Journal of Chest Surgery
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    • v.38 no.9 s.254
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    • pp.633-636
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    • 2005
  • Sixteen-day-old baby with severe Ebstein anomaly underwent emergency operation to relieve progressive hypoxia and congestive heart failure. Operative findings showed huge right atrium and atrialized right ventricle (aRV) with very small functional RV by distal displacemcent of tricuspid valve mechanism. We elected to perform modified Starness operation because biventricular repair was deemed unattainable. After pulmonary and tricuspid valves were primarily closed, aRV was obliterated with multiple sutures from RV apex to the base. Then a PTFE (Gore-Tex, USA) vascular graft was interposed between innominate artery and main pulmonary artery for systemic to pulmonary shunt. The patient was discharged uneventfully, and received bi-directional cavopulmonary shunt 6 months later.

A CASE OF DERMIS-FAT AUTOTRANSPLANTATION FOR CORRECTION OF SOFT TISSUE DEFICIT IN HEMIFACIAL MICROSOMIA (반안면왜소증환자에 있어서 자가지방이식을 이용한 연부조직결손의 수복예)

  • Park, Young-Wook;Lee, Jin-Gew;Min, Byoung-Il
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.13 no.1
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    • pp.82-87
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    • 1991
  • Hemifacial microsomia is a term used to describe a facial anomalies caused by the defect of anatomic structures originated from the first and the second branchial arches. The defect area encompasses some facial areas including mandible, facial muscles, masticatory muscles, cranial nerves, auricles, etc., and the degree of manifestations of the anomalies is extmely diverse. A 20-year-old man complaining of facial asymmetry and malocclusion visited our hospital. An orthognathic surgery was performed for the correction of hard tissue anomalies and then autogenous dermis-fat autotransplantation was done for the improvement of remaining soft tissue defect. The result was esthetically good and the case was presented here.

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Treatment of Facial Arteriovenous Malformations by Embolization: A Case Report (색전술에 의한 안면부 동정맥 기형의 치료 : 증례 보고)

  • Jeongyeon, Kim;Hyunsoo, Lim;Okhyung, Nam;Hyo-seol, Lee;Sungchul, Choi;Misun, Kim
    • Journal of the korean academy of Pediatric Dentistry
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    • v.49 no.2
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    • pp.228-233
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    • 2022
  • Arteriovenous malformations (AVMs) are rare congenital anomalies characterized by direct communication between arteries and veins that bypass the capillary bed. AVMs may not manifest clinically until late infancy or childhood. In particular, facial AVMs can cause urgent life-threatening dental events. A 5-year-old girl without a medical history visited the hospital because of spontaneous gingival bleeding around the posterior gingival area of the lower left 2nd primary molar. Angiography through the femoral approach under general anesthesia was performed for differential diagnosis and therapeutic option. The blood flow was effectively reduced after arterial embolization alone, and there was no evidence of recurrence at the 5-month follow-up. The present study reports that embolization of the affected vessels can be a more effective and safe method than surgical resection for the treatment of AVM during the growth period.