수술후 10년 이상 장기 생존 담도폐쇄증 환장에서의 영양상태 및 임상적 특성에 관한 분석

전용순,김우기,Chun, Yong-Soon,Kim, Woo-Ki 대한소아외과학회 1998 소아외과 Vol.4 No.2

To assess the clinical and nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, MAC, TSF), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure were performed more than 10 years ago at Department of Pediatric Surgery in Seoul National University Hospital. None were below the 5th percentile in height and weight. TSF was above the 75th percentile in all patients and showed good subcutaneous fat deposition. MAC was above the 5th percentile in all patients. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Although some patients who have survived for more than 10 years after Kasai procedure developed protein malnutrition and vitamin deficiencies, growth and development and nutritional status were generally satisfactory. Five patients(42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion of long-term survivors had abnormal liver function and portal hypertension. Continuous and careful follow-up is necessary to determine when liver transplantation may be indicated.

선천성 식도 폐쇄증 - 17년간의 96예 치험 분석 -

전용순,정성은,이성철,박귀원,김우기,Chun, Yong-Soon,Jung, Sung-Eun,Lee, Seong-Cheol,Park, Kwi-Won,Kim, Woo-Ki 대한소아외과학회 1995 소아외과 Vol.1 No.2

저자들은 96예의 선천성 식도 폐쇄를 분석하여 다음의 결과를 얻었다. 1) 남녀비는 1.1:1로 비슷하였다. 2) 출생시 평균 체중은 2.8kg 였고 2.5kg 미만의 저출생 체중아가 32.3%를 차지했다. 3) 주증상은 타액 분비 과다, 수유시 호흡 곤란, 청색증 등이었다. 4) Gross 분류법에 의한 해부학적 유형은 Type C가 가장 많은 빈도로 관찰되었다(82.3%). 5) 동반 선천성 기형이 35%에서 관찰되었고 이 중 심장 기형이 가장 많은 빈도로 관찰되었다(60%). 6) 수술은 Type A, C의 경우 일차적 문합을 시행하였고 일차적 문합이 불가능한 원간격 결손 환아에서 역위관 삽입술이 시행되었다. Type E(H-type)의 경우 식도기관루 절제술이, Type F의 경우 식도협착부위 절제 및 단단 문합술이 시행되었다. 7) 수술후 주요 합병증은 문합부 누출, 문합부 협착, 기관 식도루 재발 등이었다. 8) 생존율은 86%였고 Waterston Criteria에 따른 생존율은 Class A, 93%, Class B, 86%, Class, 58%였다. 9) 추적 조사 결과 93%의 환아가 건강했고 7%의 환아가 잦은 호흡기 감염으로 입원한 병력이 있었다. To study the clinical profiles and outcome of surgery in infants with esophageal atresia, we reviewed 96 esophageal atresia cases who were treated from April, 1978 to June, 1995. There were 51 male and 45 female infants, a ratio of 1.1:1. The low birth weight(<2500g) patients were 32%. Clinical findings at the time of diagnosis included drooling in 57%, choking in 50%, cyanosis in 38%, respiratory distress in 27% and swallowing difficulty in 20%. Gross classification included 6 cases of type A esophageal atresia(6%), 79 cases of type C(82%), 3 cases of type E (3%) and 8 cases of type F(8%). Associated anomalies occurred in 34 infants(35%). Among them, cardiac anomalies were most common(60%). A primary repair of the defect was carried out in 76 patients with type A or C. A staged operation comprising a repair or gastric tube interposition after gastrostomy was performed in 8 patients. In all 3 infants with H-type, a division of fistula was performed. Esophageal resection and anastomosis was done in 8 infants with esophageal stenosis. In one infant, a gastrostomy was performed and he expired before staged operation. Anastomotic complications included leakage in 16 cases(17%), stricture in 37 cases(39%) and recurrent tracheoesopohageal fistula in 3 cases(3%). The mortality rate was 14% and the leading cause of death was pneumonia. The overall survival rate was 86%, and according to Waterston criteria, the survival rates were 93%, 85% and 58% in class A, Band C, respectively. 75 patients were followed up with median follow up 6.4 years. Among them, 93% were uneventful and 7% had frequent pneumonia.

기도결찰을 시행한 토끼 태자의 실험적 횡격막탈장에서 Lamellar body counts

전용순,정수진,이정녀,Chun, Yong-Soon,Jung, Soo-Jin,Lee, Jeong-Nyeo 대한소아외과학회 2011 소아외과 Vol.17 No.2

Experimental tracheal ligation (TL) has been shown to reverse the pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) and to normalize gas exchange. The purpose of this study was to determine whether the TL would correct the surfactant deficiency present in the fetal rabbit model of CDH by using lamellar body count. Lamellar bodies are synthesized and secreted by the type II pneumocytes of fetal lung. The phospholipids present in these bodies constitute the major component of pulmonary surfactant. Twenty-one pregnant New Zealand rabbits underwent hysterotomy and fetal surgery on gestational day 24. Two fetuses of each pregnant rabbit were operated. In the fetus of one end of bicornuate uterus, left DH was created by excision of fetal diaphragm through open thoracotomy (DH Group). In the fetus of the other end of bicornuate uterus, left DH and TL were created (TL Group). The fetuses were delivered by Cesarean section on gestational day 31. Fourteen in control group, 12 in the DH group and 13 in TL group were born alive. En bloc excision of lungs, bronchi and trachea was done in all newborn rabbits. A five Fr catheter was inserted through trachea and repeated irrigations with 10 cc normal saline were done. The irrigated fluid was centrifuged at $280{\times}g$ for 5 minutes and the lamellar bodies were counted with the upper level fluid in platelet channel of electronic cell counter. The average lamellar body counts were $37.1{\pm}14.2{\times}10^3/{\mu}L$ in control group, $11.5{\pm}4.4 {\times}10^3/{\mu}L$ in DH group, and $6.5{\pm}0.9{\times}10^3/{\mu}L$ in TL group. Lamellar body count in DH group was lower than in control group and did not increase after TL. This study shows TL has no therapeutic effect on decreased surfactant level of CDH and the pregnant rabbit is appropriate for the animal model of CDH.

Pena 술식으로 치료한 쇄항 환자들의 장기 추적 결과

전용순(Yong-Soon Chun),김우기(Woo-Ki Kim),박귀원(Kwi-Won Park),이성철(Seong-Cheol Lee),정성은(Sung-Eun Jung) 대한외과학회 2000 Annals of Surgical Treatment and Research Vol.59 No.4

소아 담관낭종 80예 분석

전용순(Yong-Soon Chun),정성은(Sung-Eun Jung),이성철(Seong-Cheol Lee),박귀원(Kwi-Won Park),김우기(Woo-Ki Kim) 대한외과학회 1998 Annals of Surgical Treatment and Research Vol.55 No.6

성인의 담관 낭종

전용순(Yong Soon Chun),김선희(Sun Whe Kim),윤용범(Young Bum Yoon),박용현(Young Hyun park) 대한소화기학회 1994 대한소화기학회지 Vol.26 No.3

N/A Choledochal cyst is not so rare in the far east Asian countries as in the western countries. Reflux of pancreatic juice to the bile duct through anomalous pancreatobiliary ductal union (APBD) is considered as one of the important etiologic factors. To study the clinical charac- teristics of choledochal cyst in adults and the significance of APBD and reflux of pancreatic juice into bile duct in this disease, we reviewed 57 choledochal cyst cases. APBD is defined as long common channel(>1.5cm) and we interpreted as presence of pancreatic juice reflux if bile amylase was greater than 600 IU/dl. Ages ranged from 16 to 72 years and the fourth decade was the most prevalent. One third of the patients had all triad symptoms-abdominal pain, mass and jaundice. Diagnosis was made by ultrasonography and cholangiography in most of the cases, and preoperative diagnosis was rnade in 82%. Gallstones were associated in 45.6% of the cases (26 cases) and biliary tract malignancy in 8.8% (5 cases). Three of the latter patients expired within 1 year aft.er operation. Todani type I(68.4%), and IV(26.3%) are the two most common types. Long common channel was found in 65% of the cases (11/ 17) and bile amylase was above 600 IU in 78.6% of the cases (22/28). Forty six patients were followed up for a median period of 6.5 years after initia] operation. Among 17 patients who underwent cystojejunostomy, pancreatitis or cholangitis developed in 3 patients and 1 case expired due to periampullary cancer 1 year after operation and 1 case developed CBD cancer and expired 10 months after a second operation. Among 21 patients who underwent cyst excision, 5 patients suffered frorn cholangitis or pancreatitis. In conclusion, choledochal cyst in adults is frequently associated with other biliary tract disease, the most significantly biliary tract malignancy. APBD seems to play an important role in the pathogenesis of choledochal cyst, especially type Ia and 1V. Excision of the cyst is the procedure of choice for the treatment because it eliminate APBD and potentials to develop malignancy or preexisting malignancy. (Korean J Gastroenterol 1994; 26: 549 560)

선천성 식도 폐쇄에서의 역위관 거치술

허승,전용순,박귀원,김우기,Huh, Seung,Chun, Yong-Soon,Park, Kwi-Won,Kim, Woo-Ki 대한소아외과학회 1995 소아외과 Vol.1 No.2

Since 1988, we have performed esophageal replacement with the reversed gastric tube on four esophageal atresia patients. Three patients had long-gap esophageal atresias and one patient had recurrent tracheoesophageal fistula that was previously operated on three times. One combined imperforate anus. The youngest patient was 6-month-old and the oldest, 34-month-old at the time of procedure. The technique of gastric tube construction is described. There have been both major and minor complications. Although two patients had shown distal tube strictures as late complications. those were solved with tuboplasties on 29 months and 48 months, postoperatively. Growth and development have been acceptable in all four patients, although most remain in the lower percentiles for growth and height, a condition that usually predates the esophageal substitution. Conclusively, reversed gastric tube interposition has proved very satisfactory for long-gap esophageal atresia that cannot be anastomosed primarily even by spiral myotomy and esophageal atresia with recurrent tracheoesophageal fistula having destroyed esophagus due to previous operation.

경부 이소성 흉선종 1예 보고

이기윤,전용순,남정수,이난주,윤혜경,Lee, Ki-Yun,Chun, Yong-Soon,Nam, Jeong-Su,Lee, Nan-Joo,Yoon, Hye-Kyoung 대한소아외과학회 2008 소아외과 Vol.14 No.1

Accessory cervical thymic tissue has been rarely reported. However, it should be included in the differential diagnosis of neck masses in children, along with branchial anomalies, lymph nodes and other tumors. This lesion occurs along the descending line of the thymus between the angle of mandible and superior mediastinum. A 2-month-old infant presented with an asymptomatic left sided neck mass. MRI revealed a well defined homogeneous mass in the deep lobe of left parotid gland. At operation, an easily identified soft tissue mass was found in the left submandibular area, measuring $3{\times}1.5cm$. It was completely excised. Microscopic examination revealed normal thymic tissue.

유미성 장간막 낭종 1예 보고

주인호,전용순,이난주,윤원화,Joo, In-Ho,Chun, Yong-Soon,Lee, Nan-Joo,Yoon, Won-Hwa 대한소아외과학회 2008 소아외과 Vol.14 No.1

Chylous mesenteric cyst is a rare variant of mesenteric cystic lesions. Pathologically there is lack of communication of the main lymphatic vessels, resulting in cystic mass formation. Clinical presentation is diverse and can range from an incidentally apparent abdominal mass to symptoms of an acute abdomen. A 5-year-old girl presented with abdominal distension without pain. CT scan showed a huge and thin-walled cystic mass without solid portion. Laparotomy showed a $20{\times}18cm$ sized huge mesenteric cyst containing chylous fluid. Pathological diagnosis was cystic lymphangioma.

소아 서혜부 탈장에 대한 임상적 고찰; 단일병원 10년간 1244예 후향적 고찰

윤원화,허찬영,전용순,Yoon, Won-Hwa,Heo, Chan-Young,Chun, Yong-Soon 대한소아외과학회 2009 소아외과 Vol.15 No.1

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.