• Title/Summary/Keyword: Congenital anomalies

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The Clinical Applicability of Transoral Transpharyngeal Approach to the Craniovertebral Junction Lesions (두개 경추 이행부의 병소에 대한 경구적 접근법의 유용성)

  • Cho, Tae Goo;Park, Kwan;Cho, Yang-Sun;Baek, Chung-Hwan;Nam, Do Hyun;Kim, Jong Soo;Hong, Seung-Chyul;Shin, Hyung Jin;Eoh, Whan;Kim, Jong Hyun
    • Journal of Korean Neurosurgical Society
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    • v.29 no.3
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    • pp.379-388
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    • 2000
  • Objective : Although transoral transpharyngeal approach is a very useful method for the lesions of craniovertebral junction, it is not frequently used because of anatomical unfamilarity, risk of cerebrospinal fluid(CSF) leakage, and resultant postoperative meningitis. To evaluate the usefulness of transoral transpharyngeal approach for various lesions of craniovertebral junction, clinical characteristics and the results of this approach are investigated. Methods : Transoral transpharyngeal approaches were performed in eight cases between 1996 and 1999. Among them, there were three basilar invaginations due to congenital anomalies, two odontoid type I fractures, two atlantoaxial dislocations, and one pseudotumor. Surgical methods included five cases of anterior decompression and posterior fusion, two anterior approaches for decompression and one transoral approach for biopsy. Results : This procedure allowed immediate clinical improvement in all cases. In seven patients with preoperative motor deficit showed a progressive neurological improvement. The follow-up plain x-rays demonstrated successful bony fusion in all patients. Only one patient suffered from postoperative wound dehiscence, but she completely recovered after wound revision. There was no complication of postoperative CSF leakages. Conclusions : Transoral transpharyngeal approach for the ventral lesions of craniovertebral junction, can be used as a relatively simple and effective method.

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20 Years Surgical Experiences for Ebstein's Anomaly (엡스타인 기형의 20년 수술 치험)

  • Lee, Sak;Park, Han-Ki;Lee, Chang-Young;Chang, Byung-Chul;Park, Young-Hwan
    • Journal of Chest Surgery
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    • v.40 no.4 s.273
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    • pp.280-287
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    • 2007
  • Background: We retrospectively evaluated the clinical results of surgically managing patients with Ebstein's anomaly. Material and Method: Between Feb. f 984 and June 2006, 50 patients who underwent surgical treatment for Ebstein's anomaly at Yonsei Cardiovascular Center were retrospectively reviewed. The mean age of the patients was 26.9 years and 19 patients were male, Associated anomalies included atrial septal defect (33), patent ductus arteriosus (2), ventricular septal defect (1), and pulmonary stenosis (4), and 90%, (45/50) of the patients had more than a moderate degree of tricuspid regurgitation. Carpentier type A was present in 6 patients, type B in 26, type C in 14 and type D in 4. Ten patients were associated with WPW syndrome. Conservative surgery was possible in 31 patients (tricuspid annuloplasty, plication of the atrialized RV), Fontan's operation was peformed in 4 patients, tricuspid valve replacement was done in 12 and palliative surgery was done in 2 patients. Thirteen patients were associated with hi-directional cavopulmonary shunt (BCPS: one and a half ventricular repairs): 10 patients with WPW syndrome and 4 patients with atrial fibrillation underwent concomitant ablation. Result: The postoperative median NYHA functional class $(3{\rightarrow}1)$ and the mean cardio-thoracic ratio $(0.65{\rightarrow}0.59)$ were decreased significantly (p<0.001, p=0.014). The mean oxygen saturation $(86.6{\rightarrow}94.1%)$, and median TR grade $(4{\rightarrow}1)$ were also significantly improved (p=0.004, p<0.001). For comparison of BCPS and conservative surgery, the preoperative right ventricular pressure (33.0 vs. 41.3 mmHg), the ICU stay (2.80 vs. 1.89 days), the hospital say (10.6 vs. 16.8 days), and the left ventricular ejection fraction (64.3 vs. 72.8%) were statistically different. Postoperative mortality occurred in 3 patients (6%) due to biventricular failure in 2 patients and sepsis in the other patient. The mean follow up duration was 101.5 months, and one patient died of Fontan failure and 6 patients required reoperation (bioprosthetic degenerative change (2) and Fontan conversion (4)). The overall survival rate at 10 years was 90.2%, the freedom from reoperation rate and rate of cardiac related events were 78.9% and 49.2%, respectively. Conclusion: Surgical management of Ebstein's anomaly can be performed safely, and the associated BCPS may be helpful for high-risk patients. Adequate application of surgical management may increase the long-term survival with a reduced rate of reoperation.

The study on the cleft lip and/or palate patients who visited Dept. of Orthodontics, Seoul National University Dental Hospital during last 11 years (1988.3-1999.2) (최근 11년간 서울대학교병원 교정과에 내원한 순구개열 환자의 내원 현황에 관한 연구(1988.3 - 1999.2))

  • Yang, Won-Sik;Baek, Seung-Hak
    • The korean journal of orthodontics
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    • v.29 no.4 s.75
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    • pp.467-481
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    • 1999
  • Cleft lip and/or palate is one of the most common congenital craniofacial anomalies. According to previous epidemiologic studies, incidence of cleft lip and/or palate has been increasing nowadays. However, there is no report about epidemiologic study of cleft lip and/or palate patients who visited dept. of orthodontics in Korea. So the purpose of this study was to provide the epidemiological characteristics and important basic clinical data for the diagnosis and the treatment of the cleft lip and/or palate patients. With the orthodontic and cleft charts, diagnostic models and X-ray films from 250 patients with cleft lip and/or palate who visited Dept. of Orthodontics, Seoul National University Dental Hospital during the last 11 years, the authors investigated patient's visiting yew, types of cleft, patient's gender, and Angle's classification of malocclusion, and surgery timing. The results were as follows ; 1. The number of cleft patients who visited Dept. of Orthodontics, SNUDH increased during 1988-1990 and then it declined until 1992. From 1993 to 1996, it showed a stationary trend. After 1997 it showed an overwhelmingly increasing trend. 2. In the cleft type, the ratio of cleft lip cleft lip and alveolus cleft palate : cleft lip and palate was 7.6:19.2:9.6:63.6. In cleft position, unilateral clefts were more than bilateral ones (cleft lip 79:21, cleft lip and alveolus 77:23, cleft lip and palate 75.5:24.5). In cleft side, left clefts were mote than right clefts (cleft lip 53.3:46.7 cleft lip and alveolus 59.5:40.5, cleft lip and palate 59.2:40.8). 3. In gender ratio, males were more than females in cleft lip (57.9:42.1), cleft lip and alveolus (68.8:31.2) and cleft lip and palate (76.1:23.9). But in cleft Palate females were more than males as 41.7: 58.3. 4. In the age groups, 7-12 year group was the most abundant as $52\%$, and then 0-6 year group ($20.4\%$), 13-18 year group ($17.2\%$), more than 18 yew group ($10.4\%$) were followed as descending order. 5. Most of the cleft lip repair surgeries were operated in 0-3 month ($60.3\%$) and 4-6 month ($17.9\%$). 6. The cleft palate repair surgeries were done in 1-2 year ($31.7\%$), 0-1 year ($25.6\%$), 2-3 year ($12.1\%$), more than 5 year ($11.6\%$) as descending order. 7. The lip scar revision surgeries were done before admission at elementary school in $60\%$. (4-6 you ($27.5\%$), 6-8 year ($19.6\%$), more than 10 year ($19.6\%$), 2-4 year ($13.7\%$) as descending order) 8. The rhinoplasties were done before admission at elementary school in $51.7\%$. (0-2 year ($7.1\%$), 2-4 year ($14.3\%$), 4-6 year ($21.4\%$), 6-8 year ($14.3\%$)). 9. The pharyngeal flap were done at 6 Y (72.5 months) after birth on average and there was even distribution of surgery timing. 10. In relationship between Angle's classification of malocclusion and cleft types, Class I was most abundant and Class III, Class II were followed as descending order in cleft lip group. But Class III was most abundant and Class I, Class II were followed as descending order in cleft lip and alveolus group, cleft palate group, and cleft lip and Palate group. The percentage of frequency in Class III malocclusion was overwhelmingly higher in cleft lip and palate group than any other groups. 11. Because the frequency of class III malocclusion was most prevalent in all age groups, anterior crossbite was the most common chief complaint of cleft patients.

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