• Advances in pediatric surgery
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• 제8권1호
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• pp.54-61
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• 2002

Hirschsprung's disease (HD) is usually diagnosed in the newborn period and early infancy. The common presentation of HD in newborns consists of a history of delayed passage of meconium within the first 48 hours of life. The differential diagnosis in newborns is one of the clinical challenges of this disorder. A number of medical conditions which cause functional obstruction of the intestines are easily excluded. Neonates with meconium ileus, meconium plug syndrome, distal ileal atresia and low imperforate anus often present in a manner similar to those with HD in the first few days of life. Abdominal radiographs may help to diagnose complete obstruction such as intestinal atresia. Microcolon on contrast enema can be shown in cases with total colonic aganglionosis, ileal atresia or meconium ileus. Suction rectal biopsy or frozen section biopsy at operation is essential for differential diagnosis in such cases. HD is also considered in any child who has a history of constipation regardless of age. Older children with functional constipation may have symptoms that resemble those of HD and contrast enema is usually diagnostic. However, children with other motility disorders generally referred to as chronic idiopathic intestinal pseudoobstruction present with very similar symptoms and radiographic findings. These disorders are classified according to their histologic characteristics.; visceral myopathy, visceral neuropathy, intestinal neuronal dysplasia (IND), hypoganglionosis, immature ganglia, internal sphincter achalasia. Therefore, the workup for motility disorders should include rectal biopsy not only to confirm the presence of ganglion cells but also evaluate the other pathologic conditions.

• Journal of Chest Surgery
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• 제28권6호
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• pp.565-570
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• 1995

Seventy-three patients with isolated total anomalous pulmonary venous connection the patients associated with other major cardiac anomalies such as single ventricle, DORV[Double Outlet Right Ventricle and large VSD[Ventricular Septal Defect were excluded were underwent surgical repair from January 1980 through October 1993. There were 45 boys and 28 girls. The mean age at operation was 19.9 months[range 6 days to 24 years and mean body weight was 7.1kg[range 2.6kg to 45kg . The anomalous locations of connection were supracardiac in 38, cardiac in 21, infracardiac in 5, and mixed in 9. In 38 patients[52% , the venous drainage was obstructed. The obstruction ratios according to the connection type were as follows: 53%[28/38 in supracardiac, 52%[11/21 in cardiac, 100%[5/5 in infracardiac, 22%[2/9 in mixed type. The associated cardiac anomalies were persistent left SVC[2 , tricuspid valve regurgitation[3 , cor triatriatum[1 , and mitral cleft[1 . And associated noncardiac anomalies were imperforate anus[1 and Neil Weightman syndrome[1 . The operative mortality was 23%. The causes of death were pulmonary hypertensive crisis, perioperative myocardial failure, pneumonia with sepsis, arrhythmia and etc. The statistically significant factors in postoperative mortality were the pulmonary venous obstruction and age [p<0.01 . The operative mortality was high in groups of age under 1 month and pulmonary venous obstruction. The mean follow-up was 27.1 months. There were two late deaths. The first patient was three months old boy with supracardiac type and severe obstructive symptoms. The postoperative echocardiography was showed anastomotic stenosis and reoperations were performed twice but the patients expired due to pneumonia and sepsis. The second patient was three month old boy with supracardiac type and total correction was done and was doing well postoperatively. Eight years later, he expired suddenly due to arrhythmia. But all the other patients were in NYHA Fc I and received no medications. The 5-year survival rate excluding early expired patients is 97.1 $\pm$ 0.03 %. In conclusion, although the operative mortality of total anomalous pulmonary venous connection was relatively high compared to other major cardiac anomalies, we could expect excellent long-term results by early surgical correction.

• Advances in pediatric surgery
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• 제15권1호
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• pp.44-51
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• 2009

This study is a retrospective analysis of 1244 cases of the inguinal hernia in children under the age of fifteen years who were operated at the department of pediatric surgery, Inje University Busan Paik Hospital from March, 1997 to February, 2007. The ratio of male to female was 3.6:1. The type of hernia was indirect in all of the cases. The hernia was on the right side in 656 cases (53.9 %), left side in 467 cases (37.5 %), and bilateral in 121 cases (9.7 %). The hernia presented most frequently in infants under age 12 months; 364 cases (29.2 %). Fifty-nine cases (21.7 %) were in female and 305 cases (31.3 %) in male. There were 428 cases (33.6 %) in 1-3 years age group, 295 cases (23.7 %) in 4-6 years, 112 cases (9.0 %) in 7-9 years, 39 cases (3.1 %) in 10-12 years and 16 (1.2 %) in 13-15 years. The content of hernia sac was small bowel (59 %), omentum (31 %) in males and the ovary and tube (54 %) and small bowel (26 %) in female. The incidence of combined operation at the time was 3.2 %, and consisting of orchiopexy (67.5 %), frenulotomy (12.5 %), appendectomy (10 %), circumcision (5 %), and fistulotomy (5%). The incidence of combined disease was 2.8 % and consisting of undescended testis, Hirschsprung's disease, idiopathic hypertrophic pyloric stenosis, imperforate anus, and congenital heart disease. After unilateral inguinal hernia repairs, contralateral hernias developed in 34 patients. The laterality of the primary site of hernias were left in 19 cases (55.8 %), and right 15 cases (44.1 %). The 936 cases (75.2 %) were operated under general anesthesia; Mask bagging 663 cases (53.2 %), endotrachea intubation 257 cases (20.6 %), and laryngeal mask 16 cases (1.2 %). The remainder 308 cases (24.7 %) were operated under regional caudal anesthesia.

• 한국임상수의학회지
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• 제30권5호
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• pp.376-379
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• 2013

4주령의 0.6 kg 암컷 스콧티쉬 폴드 고양이가 예방 접종을 위해 내원하였다. 신체 검사에서 수양성 분변이 질에서 나오는 것이 확인 되었고 항문은 폐쇄되어 있었다. 방사선 양성 조영 검사에서 직장질루, 확장된 결장이 확인 되었다. 영상 검사에 기초하여 Type III 의 항문 무형성과 직장 질루가 병발한 것으로 최종 진단 하였다. 수술적인 직장 질루의 폐쇄와 항문의 재건이 실시되었다. 수술 후 소화능력을 향상시키기 위해서 락툴로즈와 식이요법을 실시 하였고, 고양이는 수술 후 2주에 배변 조절이 가능해졌으며, 8개월 후 추적조사에서 합병증 없이 배변조절 상태가 유지 되었다.

• Advances in pediatric surgery
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• 제2권1호
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• pp.26-32
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• 1996

To understand the current status of neonatal surgery in Korea, a survey was made among the 27 members of Korean Association of Pediatric Surgeons. Response rate among surgeons was 78 percent, eighteen hospitals participated in this study. Five hundred and three cases of neonatal surgical patients were analyzed. In Korea, about 50% of cases were treated at the hospital in the capital city area. Regional number of patients were closely related to the regional population. Imperforate anus(19%), atresia/stenosis of gut(12%), and Hirschsprung's diseases(12%) were sitting at the top in the list. Majority of operation was done within the first week of life, especially during the first 24 hour period. Eighty per cent was major or so called index cases. Mortality in so-called index cases was 17%. High mortality was observed in patients with diaphragmatic hernia(47%), gastrointestinal perforation(65%) and esophageal atresia(28%). Low birth weight babies showed higher mortality in gastrointestinal perforation, esophageal atresia and abdominal wall defect. These were compared to 1993 survey of Japanese Society of Pediatric Surgeons.

• Advances in pediatric surgery
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• 제17권1호
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• pp.65-71
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• 2011

Currarino syndrome is a hereditary syndrome characterized by the triad of a sacral bony defect, presacral mass and anorectal malformation. We retrospectively reviewed 13 Currarino syndrome patients who were treated in our center between 1997 and 2010. Demographic data, initial symptoms, initial diagnosis. pathologic diagnosis of presacral mass, associated anomalies and managements were analyzed. There were 8 boys and 5 girls. Four patients were diagnosed as Currarino syndrome immediately after birth with failure of passage of meconium and abdominal distension. Four patients underwent surgery for imperforate anus immediately after birth and W8re diagnosed as Currarino syndrome later and underwent reoperation. Three patients were diagnosed during work-up and management with of the tentative diagnosis of Hirschsprung's disease. Diagnosis of the remaining two patients was at the age of 26 months and 9 years and anorectal malformation was not associated. Twelve patients showed hemi-sacrum and one patient showed bilateral sacral subtotal agenesis. Two patients without anorectal malformation underwent presacral mass excision, untethering of spinal cord and repair of myelomeningocele. Six out of 8 patients, excluding 3 that expired or were lost to follow up, with anorectal malformation underwent colostomy, presacral mass excision, untethering of spinal cord, repair of myelomeningocele, posterior sagittal anorectoplasty and colostomy repair. One patient underwent only posterior sagittal anorectoplasty after colostomy. One waits the scheduled operation only with Hegar dilatation. Pathologic examation of presacral masses showed myelomeningoceles in 4 patients, lipomyelomeningoceles in 3 patients and dermoid Cyst in one patient. Teratoma was combined in 2 patients. Eight patients needed neurosurgical operation for spinal cord problems. Seven patients had urologic anomalies and two of them underwent operation. Currarino syndrome should be considered as a differential diagnosis in pediatric patients with abdominal distension, constipation and anorectal malforlnation. For proper evaluation and treatment, a multi-disciplinary approach is recommended.

• Advances in pediatric surgery
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• 제20권1호
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• pp.1-6
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• 2014

Minimal invasive surgery (MIS) has rapidly gained acceptance for the management of a wide variety of pediatric diseases. A questionnaire was sent to all members of the Korean Association of Pediatric Surgeons. Thirty one members (25.4%) took part in the survey that included data for the year 2012: demographic details, opinion regarding minimal invasive surgery and robotic surgery, spectrum of minimally invasive operations, and quantity of procedures. 48.4% of the respondents had more than 10 years experience, 35.5% less than 10 years experience, and 16.1 % had no experience. The respondents of the recommend MIS and perform MIS for surgical procedures are as follow; inguinal hernia (61.3%), simple appendicitis (87.1%), complicated appendicitis (80.6%), reduction of intussusceptions (83.9%), pyloromyotomy (90.3%), fundoplication (96.8%), biopsy and corrective surgery of Hirschsprung's disease (93.5%/90.3%), imperforate anus (77.4%), congenital diaphragmatic hernia (80.6%), and esophageal atresia (74.2%). The MIS procedures with more than 70% were lung resection (100%), cholecystectomy (100%), appendectomy (96.2%), ovarian torsion (86.7%), fundoplication (86.8%), hiatal hernia repair (82.6%), and splenectomy (71.4%). The MIS procedures with less than 30% were congenial diaphragmatic hernia reapir (29.6%), esophageal atresia (26.2%), correction of malroatation (24.4%), inguinal hernia repair (11.4%), anorectal malformation (6.8%), Kasai operation (3.6%).

• Advances in pediatric surgery
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• 제6권2호
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• pp.106-123
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• 2000

The purpose of the survey was to clarify the clinical features and management of the anorectal malformations (ARM) in Korea. Twenty-seven members of the Korean Association of Pediatric Surgeons were surveyed. The members completed a case registration form for each patient during the two years period of 1996-1997, and a questionnaire that contained their management preferences for primary and complicated patients. Twenty-four members of the 22 institutions registered 295 cases of the ARM, and 27 out of 34 members (77.1 %) responded to the questionnaire. The patients were classified by the Wingspread classification of ARM(1984). The case registration form was a modification of Wingspread workshop for the postoperative assessment and case registration. The male to female ratio was 1.7 : 1. The average number of patients per surgeon was 6.1 cases per year. Prematurity(< 36 weeks) was present in 1.9 % of cases and low birth weight (< 2.6 Kg) in 12.1 %. Among 187 male patients, 62 were high(H) type, 29 intermediate(l) type, 88 low(L), and 8 unclassified. In female, there were 2 cases of cloacal anomalies(C), 4 H type, 30 I type, 66 L type and 6 unclass ified. For male, 87.9 % of H and I type were operated by the Pena procedure, but only 7.9 % in L types. In female, all of C, H and I types, and 40.9 % of L type were done by the Pena procedure. One case expired (MR; 0.003%) as a result of surgical complication. Over-all complication rate was 12.5 %. Among 6 cases of reoperation, 4 were failed Pena procedures. Among 140 colostomies sigmoid colon was utilized in 75.7%, and loop colostomy was commonly used. Ten surgeons prefered primary maturation of the stoma. In conclusion, posterior sagittal anorectoplasty is popular for high types of ARM in this country, but considerable number of patients are still suffering from failed operations. For better understanding and analysis, necessity of prospective study by new classification was discussed.