S상 결장 분절확장증

박우현,최순옥,백태원,이희정,서수지,김상표,Park, Woo-Hyun,Choi, Soon-Ok,Paik, Tae-Won,Lee, Hee-Jung,Suh, Soo-Jhi,Kim, Sang-Pyo 대한소아외과학회 1995 소아외과 Vol.1 No.1

Segmental dilatation of the colon is a very rare disease entity of unknown etiology and may mimic Hirschsprung's disease. It is characterized by dilatation of a segment of the colon of variable length with obstruction due to lack of peristalsis in a normally innervated intestine. Recently authors experienced a case of segmental dilatation of the sigmoid colon in a 6 month-old male, who presented with severe constipation, abdominal distention, and abdominal mass since 2 months of age. Down's syndrome and congenital nystagmus were associated. Barium enema demonstrated focal dilatation of the sigmoid colon, but the rectum and descending colon proximal t o the affected colon were of normal caliber. Rectal suction biopsy with acetylcholinesterase staining was normal and anorectal manometry showed normal rectosphincteric reflex. At operation, there was a massively dilated and hypertrophied sigmoid colon with increased tortuous serosal vessels, measuring 15 cm in length and 10 cm in width. Teniae coli were identifiable in the affected segment. Frozen section biopsies at the proximal, affected, and distal colon showed ganglion cells. Descending loop colostomy was constructed initially and segmental resection and end to end colocolostomy were carried out 3 months later. Final histologic examination showed 1) normal colonic mucosa with ganglion cells, 2) prominent submucosal fibrosis and marked muscular hypertrophy, 3) unremarkable acetylcholinesterase activity and immunohistochemical findings against S-100 protein. On 8 months follow-up, he has been doing well and moves bowels 1-2 times daily.

Hirschsprung씨 질환에서 직장 흡인 생검의 Acetylcholinesterase 활성도의 연령에 따른 변화

박우현,최순옥,김상표,장은숙,백태원,Park, Woo-Hyun,Choi, Soon-Ok,Kim, Sang-Pyo,Chang, Eun-Soak,Paik, Tae-Won 대한소아외과학회 1995 소아외과 Vol.1 No.1

HD 환아 26례에서 시행한 37번의 직장흡인생검을 ACHE 조직화학검사한 결과 신생아형으로 판명된 16례 전례가 생후 1달 이내 검사한 경우였다. 혼합형으로 판명된 11례 중 9례가 2개월에서 12개월 사이에 검사한 경우였으며 고전형으로 판명된 9례 중 생후 3개월 부터 12개월 사이에 검사한 경우가 6례 였으며, 나머지 3례는 모두 1년 이후에 검사한 경우였다. 위의 결과로 보아 ACHE 조직화학검사에서 콜린성 신경섬유의 형태와 분포가 시간이 지남에 따라 변화함을 알 수 있다. 다시 말해서 신생아시기에 점막근층 및 이의 직하부 점막하층에 산재한 비교적 굵은 콜린성 신경섬유가 나이가 많아 짐에 따라 점점 가늘어 지면서 고유판과 점막하층에 광범위하게 분포하게 된다. 따라서 연령에 따른 ACHE 조직화학검사양상의 변화를 알고 이를 HD 진단에 응용함으로 Meier-Ruge등의 전통적인 진단기준으로 야기되는 오류를 극복할 수 있으리라 생각된다. The acetylcholinesterase(ACHE) activity in 37 biopsies from the patients with Hirschsprung's disease was analyzed for histochemical patterns according to age, and its evolutional behavior was also assessed. The histochemical criterion used for the diagnosis of Hirschsprung's disease was that of Chow, i.e., "the presence of many coarse discrete cholinergic fibers in the muscularis mucosae and in the immediately subjacent submucosa regard less of an infiltration of cholinergic fibers in the lamina propria." The acetylcholinesterase activity in Hirschsprung's disease was further classified into 3 patterns, advocated by de Brito and Maksoud : Pattern I-many thick fibers exclusively in the muscularis mucosae and submucosa(newborn pattern). Pattern II-many thin fibers in the muscularis mucosae and submucosa with a clear infiltration of cholinergic fibers in the lamina propria(classical pattern). Pattern III-an intermediate pattern showing morphological characteristics of the two patterns with predominance of one or the other. Of 37 biopsies, Pattern 1 was seen exclusively in 16 biopsies taken from the neonates. Pattern III was 11 of 37 biopsies and it was mainly seen in children between 2 to 12 months of age. Pattern II was seen in all 3 biopsies of older than 1 year of age and in 6 ones of 3 to 12 months of age. In summary, this study reinforces the impression that there is an evolutional character of the distribution and morphology of the cholinergic ACHE positive fiber with age. In other words, ACHE activity in Hirschsprung's disease appears to evolve with age from the newborn pattern to the intermediate pattern finally to the classical pattern.

10세 여아에서 발견된 선천성 십이지장 격막 1예

박우현,최순옥,황진복,Park, Woo-Hyun,Choi, Soon-Ok,Hwang, Jin-Bok 대한소아외과학회 2005 소아외과 Vol.11 No.2

Chronic duodenal obstruction related to a congenital web is a rare anomaly, and is sometimes difficult to diagnose preoperatively. A case of partial duodenal obstruction by a foreign body in a 10-year-old girl with a congenital duodenal web is presented. She had a year history of intermittent epigastric discomfort without nausea, vomiting or growth retardation. Upper gastrointestinal series and gastroduodenoscopy disclosed a perforated web in the 2nd portion of the duodenum and a dark go stone just proximal to the web. The web was partly excised through a longitudinal duodenotomy crossing over the web. The Ampulla of Vater was located at 7 o'clock on the posterior surface of the duodenal web and was preserved. The duodenum was closed in transverse fashion. In cases of a longstanding duodenal foreign body, a congenital web should be considered.

부갑상선 선종에 의한 원발성 부갑상선 기능 항진증

박우현,배병진,최순옥,Park, Woo-Hyun,Bae, Byung-Jin,Choi, Soon-Ok 대한소아외과학회 2000 소아외과 Vol.6 No.1

A case of primary hyperparathyroidism due to parathyroid adenoma is presented. A 14 year-old male was admitted to the hospital comptaining of voiding difficulty. The intravenous pyelogram demonstrated a stone in the proximal one third of the left ureter and marked hydronephrosis of the left kidney. The Tc-99m sestamibi nuclear scan demonstrated a hot spot below the lower pole of the left lobe of the thyroid. Laboratory study demonstrated hypercalcemia (12.4 mg/dL) and elevated parathyroid hormone (143.67 pg/mL). A parathyroid gland located below the lower pole of the left lobe of the thyroid was excised. A parathyroid adenoma, consisting of mainly chief cells was found on pathologic examination. Postoperatively the patient had transient hypocalcemic symptoms, which resolved with administration of calcium preparation and vitamin D.

카사이 수술 후 발생한 간내 담관 낭종

박우현,최순옥,Park, Woo-Hyun,Choi, Soon-Ok 대한소아외과학회 1999 소아외과 Vol.5 No.2

A 6 and a half year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst ($4.5{\times}4.0$ cm in size) in the left hepatic lobe and another tubular dilatation ($2.0{\times}0.8$ cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangiodrainage(PTCD) and cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring $6.6{\times}5.0$ cm in size. Following drainage and administration of parenteral antibiotics she became afebrile and anicteric. However she continued to drain 45-150 cc of bile per day via the tube over the next 2 weeks. The patient successfully underwent intrahepatic cystojejunostomy with intraoperative ultrasonographic guidance. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, successfully managed with PTCD followed by an internal drainage procedure.

급성 림프구성 백혈병에 합병된 천공성 typhlitis

박우현,안근수,최순옥,Park, Woo-Hyun,Ahn, Keun-Soo,Choi, Soon-Ok 대한소아외과학회 2001 소아외과 Vol.7 No.1

소아 ALL 환자 2례에서 천공성 맹장염을 경험하였다. 주요 증상으로 고열, 복통, 복부팽만 설사가 있었으며, 혈액 검사에서 백혈구 감소증 및 혈소판 감소증이 있었다. 진단은 초음파검사와 콤푸터 단층촬영으로 비정상적인 장벽 비후를 봄으로 가능하였다. 1예는 수술적 치료를 함으로 1예는 내과적 치료로 좋은 결과를 얻었다. 백혈병환자에서 항암 치료 중 고열, 하복부 동통이 있고 백혈구 감소증이 있을 경우는 맹장염의 기능성을 생각하고 조기에 초음파 검사 또는 컴퓨터 단층촬영을 하여 적절히 대처하여야 할 것으로 생각된다. 그리고 비록 천공성 맹장염이라도 증상이 국소적 일 때는 내과적 치료를 시도해 볼 필요가 있다고 생각된다. The authors, over the last 6 months, have treated 2 patients with perforated typhlitis complicating acute lymphocytic leukemia (ALL) with good outcome. The first patient was a 13-year-old male who developed intermittent high fever, abdominal pain, abdominal distention and diarrhea during the course of maintenance chemotherapy. The peripheral leukocyte ranged from 230-470/$mm^3$. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal and ascending colonic walls and subsequent ragged perforation of the posterior wall of the cecum. He survived after treatment by right hemicolectomy and aggressive supportive measures. The patient case was a 3 year-old female who developed intermittent high fever, right lower abdominal pain, a mass, and watery diarrhea during the course of maintenance chemotherapy. Serial ultra sonograms and CT scans demonstrated irregular thickening of the cecal wall (6-15mm in thickness) and subsequent small perforation of the posterior wall of the cecum with thick-walled localized abscess. She has recovered completely after aggressive medical management. We learned two lessons from our experience treating these patients:1) early diagnosis provided by a high index of suspicion and the use of ultra sonogram or CT scan is essential. And 2) although perforation is one of the surgical indications for the treatment of typhlitis, it is possible to manage the perforation nonoperatively in selected cases with localized abscess.

태아 소장 부분염전에 의한 회장 무공증

박우현,박상만,최순옥,Park, Woo-Hyun,Park, Sang-Man,Choi, Soon-Ok 대한소아외과학회 1995 소아외과 Vol.1 No.2

A 2-day-old male (Premie, Large for gestational age(LGA), Intrauterine period(IUP) 33 weeks, birth weight 2,955 gram) was transferred with marked abdominal distention, bilious return via the orogastric tube, respiratory difficulty, and generalized edema (hydrops fetalis). He was born by cesarean section to a 36 year-old mother. Antenatal ultrasonogram at IUP 31 weeks demonstrated multiple dilated bowel loops suggestive of intestinal obstruction. There was no family history of cystic fibrosis. Simple abdominal films disclosed diffuse haziness and suspicious fine calcifications in the right lower quadrant. Barium enema demonstrated a microcolon. Sweat chloride test was not available in our institution. At laparotomy, there noted 1) a segmental volvulus of the small bowel with gangrenous change, associated with meconium peritonitis, 2) an atresia of the ileum at the base of the volvulus, and 3) the terminal ileum distal to the volvulus was narrow and impacted with rabbit pellets-like thick meconium. These findings appeared to be very similar to those of a complicated meconium ileus. In summary, the ileal atresia and meconium peritonitis seemed to be caused by antenatal segmental volvulus of the small intestine in a patient with probable meconeum ileus.

Hirschsprung's Disease의 임상 양상 및 진단

박우현,Park, Woo-Hyun 대한소아외과학회 2002 소아외과 Vol.8 No.1

Diagnosing Hirschprungs disease (HD) is a clinical challenge to pediatric surgeons. The cardinal symptoms are failure of passage of meconium within first 24 hours of life, abdominal distension, and vomiting. The severity of these symptoms and the degree of consitpation vary considerably between patients. HD is suspected on the basis of history and clinical findings and the diagnosis is established by radiological examination, anorectal manometry, and histochemical analysis of biopsy specimens. In this review, the advantages and pitfalls of each diagnostic modality are discussed. And a diagnostic approach utilizing these diagnostic modalities in children with suspicious HD is presented.

최근 9년간의 담도폐쇄증 경험 - 새로운 초음파 진단법 소개 -

박우현,최순옥,Park, Woo-Hyun,Choi, Soon-Ok 대한소아외과학회 2000 소아외과 Vol.6 No.1

This paper reports our 9-year experience treating 34 infants with biliary atresia utilizing a new non-invasive diagnostic method, ultrasonographic "triangular cord"(TC) sign. The TC sign is present when there is visualization of a triangular or a band-like echogenicity just cranial to the portal vein. The ultrasonographic TC sign appears to be a simple, non-invasive, time-saving and useful tool in the diagnosis of biliary atresia. Sensitivity is 84 %. Active bile excretion was restored in 90 % of the patients who were treated between 31-60days, 78 % of those between 61-90 days, and 33 % of those being 91days or older. The incidence of postoperative cholangitis was 36 %. Construction of an antireflux valve in the Roux-en-Y loop did not affect the incidence of postoperative cholangitis(p=0.18). Among 34 infants with biliary atresia, 23(68 %) are alive for 2-102 months after operation, and 12 are alive for more than 5 years. Five-year estimate survival by Kaplan-Meier method was 66 %.

다중 시계열 모델을 이용한 단기 부하 무효전력 예측

박우현(Woo-Hyun Park),이윤호(Yunho Lee),정창호(Chang-Ho Jung),김진오(Jin-O Kim) 대한전기학회 2002 대한전기학회 학술대회 논문집 Vol.2002 No.11