한 병원에서 경험한 VATER 연관 기형

남소현,김대연,김성철,김인구 대한외과학회 2005 대한외과학회 학술대회 초록집 Vol.2005 No.10

Intestinal obstruction caused by acute perforated appendicitis misdiagnosed as Hirschsprung’s disease in a 31-month-old boy

남소현 대한소아응급의학회 2022 대한소아응급의학회지 Vol.9 No.2

Symptomatic abdominal distension in young children requires a differential diagnosis, such as overeating, constipation or surgical emergency. If the distension is associated with intestinal obstruction, clinicians should determine the cause, such as adhesion, hernia, tumor or inflammatory lesions. Herein, the author reports a case of intestinal obstruction caused by acute perforated appendicitis. A 31-month-old boy was referred with a 10-day history of abdominal distension and a presumptive diagnosis of Hirschsprung’s disease. He had undergone an unsuccessful enema. Initial plain radiography and computed tomography showed severely dilated bowel loops with the colon collapsed. During laparotomy, the perforated appendix was found attached to the retroperitoneum and mesentery, which resulted in ileal stenosis with ischemia. The author resected 10 cm of the ileum, and performed ileocecectomy. Postoperatively, the boy recovered uneventfully, except wound seroma. Due to the low incidence and vague symptoms, it is difficult to diagnose intestinal obstruction caused by appendicitis, particularly in young children. This case may serve as a reminder that acute perforated appendicitis can cause sudden onset of intestinal obstruction in young children without a history of surgery.

대한외과학회 E-learning

남소현 대한외과학회 2022 대한외과학회 학술대회 초록집 Vol.2022 No.5

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.

발효 조건이 막걸리 유래 다당의 면역자극 활성에 미치는 효과

남소현,이영경,김혜련,조장원,이영철,홍희도 한국식품영양과학회 2012 한국식품영양과학회 학술대회발표집 Vol.2012 No.10

직장-질 누공을 동반한 관형 뒤창자 중복

남소현,김대연,김성철,김인구,Nam, So-Hyeon,Kim, Dae-Yeon,Kim, Seong-Cheol,Kim, In-Gu Korean Association of Pediatric Surgeons 2009 소아외과 Vol.15 No.1

환아는 4개월부터 직장 탈출증과 심한 변비를 보였으며, 9개월에 질로부터 대변이 배출되는 증상을 나타냈으나 검사상 우측 이상 형성 콩팥 및 이소성 요관 이외 특이 소견이 없어 경과 관찰을 시행하였다. 16개월에 전신 마취하에 이학적 검사를 시행하여 누공을 확인하였고 이를 통한 대장 조영술에서 직장 중복증을 진단하여 수술을 시행하였다. 수술 시야에서 직장부터 횡행 결장에 이르는 관형의 뒤창자 중복을 확인하고, 직장-질 누공을 분리하고 결장 및 직장에는 중격절개술을 시행하였으며, 동반된 기형인 이소성 요관과 무기능 우측 신장은 절제하였다. 환아는 수술 후 3년간 잘 지내고 있다. 직장-질 누공을 동반한 관형의 뒤창자 중복은 매우 드문 선천성 기형으로 저자들의 경험을 문헌 고찰과 함께 보고하는 바이다.

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.

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.

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.