근,원위부 기관 식도루를 가진 식도 폐쇄증 - 1예 보고 -

남소현,김대연,김성철,김인구,Nam, So-Hyun,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 2008 소아외과 Vol.14 No.1

Esophageal atresia with double tracheoesophageal fistula is a very rare anomaly and is difficulty to diagnose preoperatively. We treated a full term baby with esophageal atresia with double tracheoesophageal fistula. At the first operation, only the distal tracheoesophageal fistula was identified and ligated. When the upper esophageal pouch was opened, intermittent air leakages in sequence with positive bagging were noticed. However, intraoperative bronchoscopy did not identify a fistula in the proximal pouch, and the operation was completed with end to end anastomosis of the esophagus. On the $7^{th}$ postoperative day, esophagography showed another tracheoesophageal fistula proximal to the esophageal anastomosis. A wire was placed in the fistula preoperatively under bronchoscopy. At the 2nd operation through the same thoracotomy incision the proximal fistula was identified and ligated. On the $12^{th}$ postoperative day, esophagography showed neither stricture nor leakage.

한 병원에서 경험한 VATER 연관기형

남소현,김성철,김인구,김대연,Nam, So-Hyun,Kim, Seong-Chul,Kim, In-Koo,Kim, Dae-Yeon 대한소아외과학회 2006 소아외과 Vol.12 No.2

VATER association is defined as a combination of 3 or more anomalies- vertebra (V), imperforate anus (A), esophageal atresia with or without tracheoesophageal fistula (TE), renal and radial anomaly(R). We reviewed our experiences in one center to determine etiology, prevalence, clinical manifestation, other associated anomaly and prognosis. Two hundred and twenty-three cases that underwent operations for imperforate anus or esophageal atresia were analyzed retrospectively through medical records at Department of Pediatric Surgery, Asan Medical Center from June, 1989 to July, 2005. The total number of neonates who had been admitted during period of study were 46,773 and VATER association was 9 (0.019 %, 1.92 persons per 10,000 neonates). Median gestational age and birth weight were $37^{+4}wk$ ($35^{+1}$ - $41^{+4}$) and 2,594 g (1,671-3,660), respectively and median age of mother was 32 years (23-38). There was no family history. Three patients were twins but their counterparts had no anomalies. Patients who have 3 anomalies were 6, 4 anomalies in two and 5 anomalies in one patient. Vertebra anomalies were detected in 7(77.7 %), imperforate anus in 8(88.9 %), esophageal atresia in 5 patients (55.6 %), renal anomaly in 6(66.7 %), and radial anomaly in 5(55.6 %), respectively. Four patients are alive, 2 patients were lost during follow up period. Three patients died due to neonatal sepsis, respiratory dysfunction and cardiac failure. VATER association did not appear to be a definite risk factor, but merely a randomized combination of 5 anomalies. The prognosis was dependent on the other associated anomalies, appropriateness of management and operation. Careful follow-up and aggressive treatmentare required for improving survival and quality of life.

3세 여아에서 발견된 십이지장 격막에 의한 십이지장 폐쇄: 증례 보고

남소현,임윤정,Nam, So Hyun,Lim, Yun-Jung 대한소아외과학회 2014 소아외과 Vol.20 No.2

Congenital duodenal obstruction is a one of the emergent surgical conditions in neonates. Almost of them were diagnosed with double-bubble sign in prenatal ultrasonography. However, partial obstruction caused from duodenal web could be overlooked. We reported a duodenal web in early childhood. A three-year-old girl visited at our pediatric clinic for constipation. She had been showed non-bilious vomiting after weaning meal since 6 months old of her age, but her weight was relevant for 50-75 percentile of growth curve. Barium enema was initially checked, but any abnormal finding was not found. We noticed the severely distended stomach and 1st portion of duodenum. Upper gastrointestinal series revealed partial obstruction in 2nd portion of duodenum. After laparotomy, we found the transitional zone of duodenum and identified a duodenal web via duodenotomy. We performed duodeno-duodenostomy without any injury of ampulla of Vater. She was recovered uneventfully. During 6 months after operation, she does well without any gastrointestinal symptoms or signs, such as vomiting or constipation.

초극소 저출생 체중아에서 복막 배액술의 경험

남소현,김대연,김성철,김애란,김기수,피수영,김인구,Nam, So-Hyun,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, Ai-Rhan,Kim, Ki-Soo,Pi, Soo-Yung,Kim, In-Koo 대한소아외과학회 2008 소아외과 Vol.14 No.1

Recently, the survival rates of extremely low-birth-weight (ELBW) infants have improved with the development of neonatal intensive care. However, these infants were susceptible to intestinal perforation due to prematurity, fluid restriction, and injection of indomethacin, etc. Because of the risks of transportation, anesthesia and surgery itself, peritoneal drainage has been compared with laparotomy. Through our experience, we investigate the usefulness of peritoneal drainage retrospectively. From 1997 to 2007, six ELBW (M:F=5:1) underwent primary peritoneal drainage for intestinal perforation. Their median birth weight was 685g (405~870) and gestational age was $25^{+1}$ weeks ($24^{+3}{\sim}27^{+0}$). We noticed the intestinal perforation at median 10.5 days (8~18) after birth, and placed Penrose drain or Jackson-Pratt drain through right lower quadrant incision under local anesthesia. The cause of intestinal perforation was necrotizing enterocolitis in one patient, but that of the others was not clear. Three patients who showed normal platelet count and stable vital signs recovered uneventfully. Two patients (birth weight less than 500g) who showed unstable vital signs and low platelet count (12,000 / $mm^3$ to 30,000 / $mm^3$)expired despite aggressive resuscitation. One patient required laparotomy due to persistent intestinal obstruction after drain removal and survived. Our experience shows that peritoneal drainage was an acceptable treatment for ELBW infants and the prognosis was related to vital sign and platelet count at the time of intestinal perforation, and birth weight.

소아 위식도 역류에서 시행한 복강경 Nissen식 위바닥 주름술

남소현,김대연,김성철,김인구,Nam, So-Hyun,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 2007 소아외과 Vol.13 No.1

Fundoplication is accepted as an effective treatment of gastroesophageal reflux disease. The recent results of laparoscopic fundoplication demonstrated safety and less morbidity, shorter hospital stay and less pulmonary complication compared to the open operation. Laparoscopic fundoplication has been our first choice of operation for gastroesophageal reflux disease since 2003. Among 29 cases, there were 2 conversion cases because of severe distension of transverse colon and hepatomegaly. We studied 27 consecutive patients operated upon from January 2003 through December 2004. There were 15 boys and 12 girls, ages from 1.5 months to 12 years (median 25.3 months). Body weight ranged from 2.9 kg to 37 kg (median 9.8 kg). Neurological abnormalities were present in 23 patients. Indications for surgery included medically refractory reflux associated with vomiting, pneumopathy, otorhinolaryngologic pathology, failure to thrive, esophagitis, apnea and bradycardia. We used 4-5 trocars of 5 mm or 12 mm with $30^{\circ}$ telescope and performed the Nissen technique in all patients. In neurological impaired patients, gastrostomy tube was placed at the time of fundoplication. Median operative time was 130 minutes (70 - 300 minutes). There was no mortality nor intraoperative complication. Twenty-six patients were followed for median of 19 months (8 - 31 months). Four patients (15.4 %), who were all neurological impaired, developed recurrent symptoms of gastroesophageal reflux disease. Two of these patients had reoperation (1 laparoscopic approach, 1 open method). There were significant increases in body weight in 11 patients after fundoplication. Laparoscopic fundoplication is acceptable as a safe and effective method for gastroesophageal reflux disease.

소아 기형종의 분포 및 예후

남소현,김대연,김성철,김인구,Nam, So-Hyun,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 2007 소아외과 Vol.13 No.2

Teratomas arising from totipotential primitive germ cell are composed of 2 or 3 germ cell layers. We reviewed the records of 166 children who underwent the operation for teratoma from Jan, 1990 through April, 2007. There were 40 boys and 126 girls (average age $6.93{\pm}5.83$ years). Primary sites were ovary (n = 88), sacrococcygeum (n = 24), testis (n = 16), retroperitoneum (n = 16), mediastinum (n = 8), brain (n = 4), thymus (n = 3) and a single tumor involved the adrenal gland, neck, middle ear, oropharynx, stomach, pelvis, omentum, chest wall and scalp. Teratomas were mature in 141 patients, and immature in 25. Six patients who had ovarian immature teratomas grade 2 or 3 with peritoneal gliomatosis underwent adjuvant chemotherapy. During follow up period, 6 mature teratomas recurred at sacrococcygeal area (n = 1) and contralateral ovary (n = 5). Five patients of them underwent reoperation and diagnosed as a mature teratoma, but one who had underwent a left salpingooophrectomy with right ovary cystectomy at initial operation was observed carefully. Teratomas were dominant in female patients and developed at various organs. Complete excision was needed for good prognosis. In case of immature teratoma, complete excision and appropriate chemotherapy according to grading can contribute to favorable results.

소아 환자의 당일 수술 경험

남소현,김대연,김성철,곽미정,이동명,김인구,Nam, So-Hyun,Kim, Dae-Yeon,Kim, Seong-Chul,Gwak, Mi-Jeung,Lee, Dong-Myung,Kim, In-Koo 대한소아외과학회 2006 소아외과 Vol.12 No.2

One day surgery in children has been practiced for last 10 years in this institution. This study is to examine its safety and effectiveness for patients younger than 15 years old treated at the Department of Pediatric Surgery, Asan Medical Center, from September. 1996 to December, 2005. A total of 3,709 patients, 37 % of the total pediatric operations, are included in this retrospective study. The most prevalent ages were between 1 and 3 years olds (1,199 patients). Twenty patients were younger than 6 months, and they all had one day surgery safely. Operations were herniorrhaphy in 3,126 patients,followed by excisional biopsy, chemoport removal, and OK 432 injection. There were 12 cases (0.32 %) of unplanned admissions, 7 occurred within 6 months of one day surgery. Perioperative fever was the most common cause of admission in 4 cases. The related conditions of unplanned admission were bleeding in 2 cases and radical surgery in 2. One day surgery in this institution was easily accessible and safe. This is to the result of appropriate selection of patients, cooperation with anesthesiologists, adequate control of postoperative pain, and home care programs.

재발성 서혜부 탈장으로 오인된 지방모세포종

남소현,임윤정,김연미,Nam, So Hyun,Lim, Yun-Jung,Kim, Yeon Mee 대한소아외과학회 2014 소아외과 Vol.20 No.2

Palpable inguinal mass in children should be differentiated from inguinal hernia, hydrocele, lymph node, and tumor. Though using ultrasonography, fatty tumor would be misdiagnosed as incarcerated inguinal hernia containing fatty component. We experienced the huge inguinal lipoblastoma in 5-year-old girl mimicking recurrent incarcerated hernia. Laparoscopic exploration revealed it was not incarcerated hernia but well demarcated bulging mass from abdominal wall. Mass was about $10{\times}4{\times}3cm$ and extended from internal inguinal ring to saphenous opening. It was near total excised because of right external iliac vein injury. Pathologically, it was proven as lipoblastoma containing mature adipocyte with lipoblast and fibrous septa. Postoperatively, we noticed a segmental thrombotic occlusion of external iliac vein. After 1 year, she has no symptom related to occluded vessel. The remained lipoblastoma showed no interval change. Even lipoblastoma has a good prognosis with low recurrence rate, we need careful follow-up.

소아 Lipoblastoma의 임상 양상

남소현,김대연,김성철,김인구,Nam, So-Hyun,Kim, Dae-Yeon,Kim, Seong-Chul,Kim, In-Koo 대한소아외과학회 2007 소아외과 Vol.13 No.2

Lipoblastoma is a rare benign soft tissue tumor occurring in infancy and early childhood. It is characterized by fat lobules with varying degrees of maturity, multivaculoated lipoblasts, fibrocapillary networks and myxoid stroma. Lipoblastoma has a good prognosis with no metastases despite its potential for local invasion. From Jan, 1990 through April, 2007, 12 children underwent the operation for lipoblastoma, 7 boys and 5 girls, diagnosed at median 22 months (5 ~ 43 months). Median follow up was 6 year 7 months. Primary sites included back (n = 5), intraabdominal (n = 2) and one in each of buttock, chest wall, neck, nose and scalp. Tumors presented with a growing mass in 9 patients, abdominal distension in 2, and an incidental finding on chest radiography in one. Complete excisions were done in all patients. There was one recurrence in a patient with a scalp mass. After reoperation, he has been doing well without evidence of recurrence. Lipoblastoma has a favorable prognosis, but recurrence can occur even with complete excision. Regular follow up is necessary to detect recurrences.

소아의 천공성 충수염에 시행한 개복 충수절제술과 복강경 충수절제술의 비교

남소현(So-Hyun Nam),김대연(Dae-Yeon Kim),김성철(Seong-Chul Kim),김인구(In-Koo Kim) 대한외과학회 2009 Annals of Surgical Treatment and Research(ASRT) Vol.76 No.1

Purpose: It is controversial to do laparoscopic appendectomy in children with perforated appendicitis. Recently, we have adopted laparoscopic appendectomy as a first-choice method in children with perforated appendicitis. We investigate the results of laparoscopic appendectomy in perforated appendicitis and compare it with open technique. Methods: We studied retrospectively the patients who underwent the appendectomy by either a laparoscopic or open technique for perforated appendicitis between January 2001 and December 2005. There were 117 patients in the open appendectomy (OA) group and 45 patients in the laparoscopic (LA) group. Results: The operation times were significantly longer for the LA group than for the OA group (OA group 76.3±23.7 vs. LA group 95.4±32.4 min) (P<0.05). The hospital days were also longer in the LA group than the OA group (OA group 8.7±4.4 vs. LA group 11.0±6.3 (days)). However, there was no difference in time to diet (OA group: 5.0±3.3 vs. LA group: 5.4±2.9 (days)) (P>0.05) and in the rate of complications (OA group: 36/117 vs. LA group: 13/45) (P>0.05) between the two groups. The incidence of postoperative ileus, intraabdominal abscess and wound infection was lower in LA group, but not significantly. Conclusion: Operation times and hospital days were longer in the LA group. But the incidence of complication was similar between the two groups. Considering little postoperative scar and less pain, laparoscopic appendectomy could be regarded as the optimal surgical therapy in children with perforated appendicitis.