• Title/Summary/Keyword: Congenital Anomalies

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Branchial Cleft Anomalies (새성기형)

  • Jeoung Dong-Won;Kim Dong-Yi;Yoon Jung-Han;JeGal Young-Jong
    • Korean Journal of Head & Neck Oncology
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    • v.12 no.1
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    • pp.22-25
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    • 1996
  • A very important and interesting problem occasionally seen in the neck of children and adults is a branchial cleft anomaly. It is a rare congenital neck disease and presents a painless palpable mass of neck in most cases. We have retrospectively reviewed the medical records of 28 patients operated upon for branchial cleft anomaly at Department of Surgery, Chonnam University Hospital between January, 1991 and December, 1995. Three of these lesions were considered to have originated from the first branchial cleft, and 25 from the second branchial cleft. Females are about twice more common than male patients. Pathological findings showed the cysts were lined squamous epithelium and subepithelial lymphoid follicles in most cases. Five out of 28 patients with branchial cleft anomalies had previous incision procedures. All patients after complete removal of branchial anomalies have no recurrences.

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Transposition of the Great Arteries (TGA) -Report of An Autopsy Case- (대혈관전위증 부검 1례 보고)

  • 김학제
    • Journal of Chest Surgery
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    • v.10 no.1
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    • pp.106-112
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    • 1977
  • Transposition of the great arteries is one of the commonest forms of severe congenital heart disease and produces severe cyanosis threatening survival from the day of birth. Anatomical anomalies which the aorta arises from the infundibulum of the right ventricle and the pulmonary artery arises from the outflow tract of the left ventricle make the deranged circulation. Survival is possible only if additional anomalies are present which allow mixing of the pulmonary and systemic circulations. Preoperative diagnosis as TGA was taken on the 15 day old female via the preoperative examination and the right cordioangiography. As palliative treatment for cyanosis, Blalock-Hanlon operation was performed in this patient. The results were good as 54 mmHg changed from 27 mmHg of $PO_2$ in aorta, but sudden cardiac arrest was developed in postoperative 12 hours. In order to confirm the cause of death and the cardiac anomalies, autopsy was performed on the date of death. The diagnosis of the autopsy showed; [1] Transposition of the Great Arteries. [2] Patent Ductus Arteriosus. [3] Patent Foramen Ovale. [4] Ventricular Septal Defect, 2 Muscular Type. [5] Double Ureter, Right. [6] Artificial Atrial Septal Defect. [7] Total Collapse of the left lung and Intraparenchymal hemorrhage of right lung.

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Humeral Hemimelia in a Holstein Calf (유우 송아지의 선천성 상완골 형성 부전)

  • 한동운
    • Journal of Veterinary Clinics
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    • v.20 no.3
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    • pp.419-422
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    • 2003
  • Humeral hemimelia is a rare congenital anomaly characterized by deficiency of the humerus with relatively intact scapular. The holstein calf was healthy expecting the deformity of forelimb humerus. The examination of PCR and serum neutralizing test was carried about BVD, akabane virus, Ibaraki virus and Kasba virus in cattle. The results were all negative. Affected calf showed forelimb deformation. Other abnormalitis was not observed in brain, spinal cord, liver, kidney and other visceral organs. Amelia are very rare limb anomalies. We report on a Holstein calf with forelimb deficiencies. This case appears humeral anomalies with ulnar and radius aplasia in calf. The condition in these calves was considered the result of a recurrence of a genetic mutation affecting a putative hemimelia locus.

Surgical Treatment of Congenital Aneurysms of Sinus of Valsalva A report of 10 years` experience of 18 consecutive cases (선천성 Valsalva 동 동맥류의 수술요법)

  • Kim, Gi-Bong;Seo, Gyeong-Pil
    • Journal of Chest Surgery
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    • v.18 no.2
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    • pp.265-272
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    • 1985
  • Congenital aneurysm of sinus of Valsalva is one of the rare congenital heart disease, which is usually asymptomatic until rupture. The aneurysm usually ruptures into a cardiac chamber and produces an aorto-intracardiac fistula. Ruptured aneurysm is a grave lesion in that it causes heart failure and subsequent death. If, however, it is discovered in its early stages and operated on properly, it can be corrected with considerable success. Form January 1975 through December 1984, 18 consecutive patients with congenital aneurysm of sinus of Valsalva underwent corrective surgery using total cardiopulmonary bypass in our department of Thoracic Surgery. 1. The incidence was about 0.9% of surgical cases of congenital heart disease during that period. 2. 13 were males and 5 females, with ages ranging 12 years to 52 years. 3. Associated anomalies were VSD in 14, infundibular PS in 1, aberrant muscle band in RVOT in 1, and secondary aortic insufficiency in 9. 4. 17 were suggested to arise from right coronary sinus and 1 from noncoronary sinus; Among 17, 12 ruptured into right ventricle, and one from noncoronary sinus into right atrium. 5. Surgical correction was performed by means of direct suture closure with combined pledget or patch graft after aneurysm resection, and associated lesions were also corrected simultaneously. 6. There was only one case of operative mortality, and all the other patients were relatively uneventful in their follow-up studies.

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Cleft Palate and Congenital Alveolar Synechiae Syndrome: A Case Report and Literature Review

  • Choi, Kang-Young;Chung, Ki-Ho;Yang, Jung-Dug;Chung, Ho-Yun;Cho, Byung-Chae
    • Archives of Craniofacial Surgery
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    • v.9 no.1
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    • pp.41-44
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    • 2008
  • Cleft palate and congenital alveolar synechia is a rare syndrome. Only eight cases have been previously reported. It consists of a spectrum of facial anomalies always including cleft palate and congenital alveolar synechiae without other abnormalities. This report described an unusual case of congenital alveolar synechial band spanning posterior alveolar of the two jaws with cleft palate. Previously reported cases showed bilaterally or anteriorly located fibrous band. In our department, a new born revealed unilateral posterior synechia. Under brief intravenous sedation, synechium was divided using bipolar diathermy in the nursery at 3 days of age because of poor feeding. This division allowed full jaw opening after brief passive exercise. The patient is growing and maturing as expected with no complications. This patient is supposed to be the first reported case of isolated unilateral alveolar synechium combined with cleft palate in the worldwide.

Congenital Anonychia with Ectrodactyly of 5th Finger (원위지골 결손을 동반한 제 5수지의 선천성 무조갑증의 치험례)

  • Kim, Kook Hyun;Kim, Cheol Hann;Kang, Sang Gue;Tark, Min Sung
    • Archives of Plastic Surgery
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    • v.34 no.3
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    • pp.406-408
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    • 2007
  • Purpose: Despite a high frequency of acquired nail disease, congenital absence of the nail, also called as anonychia, is a rare anomaly. It may be seen as an isolated of phalangeal bone(ectrodactyly), nail-patella syndrome, birth trauma, impaired peripheral circulation, alopecia areata, and pemphigus, idiopathic atrophy of the nail, bullous drug eruptions, periodic shedding, lichen planus, Stenvens-Johnson syndrome and so forth. Methods: We have experienced a rare case of 40-day-old neonate, suffering from intrauterine growth retardation, but without familial history, chromosomal anomalies or any other diseases. Results: There was no nail on left 5th finger and distal phalangeal bone of same finger. So, We diagnosed as Congenital Anonychia with ectrodactyly of 5th Finger. Conclusion: We report this case as congenital anonychia of 5th finger which have developed from underlying distal phalangeal ectrodactyly. We also review other reported cased in the literatures.

Coarctation of Aorta: A Report of 4 Cases (대동맥교약증 수술치험 4예)

  • 김용진
    • Journal of Chest Surgery
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    • v.11 no.4
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    • pp.529-534
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    • 1978
  • Recent advances in the surgical treatment of congenital disorders of the heart have necessitated an accurate preoperative diagnosis. Right heart catheterization has become widely accepted as a research tool and diagnostic test to detect the heart diseases, especially in the congenital heart anomalies. Right heart catheterizations were carried out in 50 cases of congenital heart diseases at Department of Thoracic and Cardiovascular Surgery, Kyungpook National University Hospital, during the period of June, 1975 through September 1978. In age distribution, 27cases were below 10 years of age, 18 cases between 11 and 20, and 5 cases above 20 male to female ratio was 2.8:1. The distribution of congenital heart diseases was VSD [42%], TOF [36%], PDA [10%], ASD [8%], and PS[4%]. Of these, 44 cases [88%], were compatible with the clinical impressions that were made preliminarily before cardiac catheterization, and all the cases except 1 case of VSD was correlated well with the postoperative diagnosis. The right heart catheterization is considered to be reliable and accurate toll in the preoperative diagnosis of congenital heart diseases. These procedures caused complications such as left side hemiplegia [lcase], occlusion of the femoral artery [lcase], and transient ventricular tachycardia [1case], and so the complication rate of right heart catheterization was 6% [3 cases]. None of patients who have undergone right heart catheterization was died.

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Clinical Experience of Open Heart Surgery A Report of Annual 108 Cases (1984 년도 연간 개심술 108례 보고)

  • 박병순
    • Journal of Chest Surgery
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    • v.18 no.3
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    • pp.383-390
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    • 1985
  • 108 cases of open heart surgery were done at our department in 1984. There were 58 male and 50 female patients ranging in age from 20 months to 52 years. 75 cases were congenital heart disease, and 33 cases were acquired heart disease. There were 75 congenital heart anomalies with 5 operative deaths [6.7%], consisting of 62 acyanotic cases with 2 deaths [3.2%] and 13 cases of cyanotic cases with 3 deaths [23.1]. In 33 patients of acquired valvular disease, 29 valves were implanted; 20 mitral valve replacement with 2 death [10%], 2 aortic valve replacement with 1 death [50%], 2 double valve replacement [MVR+AVR] and 2 open mitral commissurotomy plus aortic valve replacement with no death. Postoperative, Warfarin sodium was medicated with checking prothrombin time. Finally, the operative mortality was 9.2% in congenital anomaly, and 9.1% in acquired heart disease, overall mortality rate was 9.3%.

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Single Coronary Artery with Coronary Arterioveous Fistula (단관상동맥에 발생한 관상동정맥루 -치험1례-)

  • Ahn, Byoung-Hee;Lee, Dong-Jun
    • Journal of Chest Surgery
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    • v.15 no.3
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    • pp.366-369
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    • 1982
  • Single coronary artery is rare congenital anomaly with incidence about 0.04%. Above half of single coronary artery is symptomless, and about 41% of cases are associated with another congenital cardiac anomalies . Single coronary artery is sometimes important for cardiac operation to avoid fatal result from cutting of coronary artery during the right ventriculotomy. Among the associated anomaly of the single coronary artery, coronary arteriovenous fistula is very rare. We experienced congenital single coronary artery with coronary arteriovenous fistula drained into the right ventricle, and so we report this case with literatures.

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Clinical analysis of 60 cases of open heart surgery (개심술 치험 60례 보)

  • Kim, Su-Seong;Kim, Yeong-Ho;Kim, Gong-Su
    • Journal of Chest Surgery
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    • v.17 no.3
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    • pp.389-397
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    • 1984
  • Sixty cases of open heart surgery were performed in the Department of Thoracic and Cardiovascular Surgery of Chonbuk National University Hospital from July, 1983 to June, 1984. The patients were consisted of 40 [66%] congenital anomalies containing 26 [43%] patients of acyanotic group and 4 [23%] of cyanotic group, and 20 [34%] acquired heart diseases which involved one or more cardiac valves. The male patients were 42 and the female 18. In 20 valvular heart diseases, open mitral commissurotomy was done in 5 patients, mitral valvular replacement with tissue valve in 6, mitral valvular replacement with mechanical valve in 5, mitral valvular replacement with tricuspid annuloplasty in 2, mitral annuloplasty in 1, and mitral and aortic valvular replacements with mechanical valves in 1. The most frequency complication was low cardiac output syndrome occurred in 9, and the next was urethral stenosis, ARDS, and postoperative bleeding, etc. The perioperative mortality was 21% in congenital cyanotic heart disease, 12% in congenital acyanotic heart disease, and 5% in acquired heart disease.

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