• Advances in pediatric surgery
• /
• 제13권1호
• /
• pp.61-65
• /
• 2007

Conventional treatment of Hirschsprung's disease consists of initial colostomy followed by pull-through operation. But, the treatment of Hirschsprung's disease has been changed along with the development of new surgical technique. Since 1995, endo-GIA has been available at our hospital and one stage Duhamel operation has been performed for neonatal Hirschsprung's disease. Between May 1995 and April 2006, 26 neonates have been treated with one stage pull-through operation by one pediatric surgeon at HanYang University Hospital. The sex ratio was 4.2:1 with male predominance. Clinical findings included abdominal distension (96.2 %), vomiting (50.0 %), delayed passage of meconium (46.2 %), constipation (23.1 %), and enterocolitis (15.4 %). Twenty two cases (84.6 %) were short-segment and 4 cases (15.4 %) were long-segment disease, of which 2 cases were total colon aganglionosis. One of the two patients with total colonic aganglionosis had double transition zones - distal ileum and hepatic flexure of the colon. The average age at operation was $14.56{\pm}8.77$ days and the average weight at operation was $3.26{\pm}0.66kg$. Primary Duhamel operations were performed in 25 patients and Soave-Boley operations was performed in one patient. The endo-GIA 35 (Ethicon, USA) was used from 1995 until 1997, and after that endo-GIA 60 (USSC, USA) was used. The average Duhamel operation time was $88.57{\pm}22.80$ minutes. Wound abscess (n = 2) and septum formation (n =1) occurred after Duhamel operation. Bowel function was normalized in 59 % within 3 months and in 95% within 1 year after operation. There was no mortality after one stage pull-through operation in neonate.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제16권3호
• /
• pp.200-205
• /
• 2013

We present a case of a 47-month-old female suffering from acute urinary bladder neck obstruction and bilateral hydronephrosis secondary to a fecaloma. Fecaloma is defined as an accumulation of inspissated feces in the colon or rectum giving the appearance of an abdominal mass. A fecaloma can be developed by diverse causes and the causes of the fecaloma in this case were septum reformation after the Duhamel procedure and long-term constipation. Chronic constipation is very common at outpatient clinic. However, acute urinary retention and voiding difficulty caused by fecaloma in the giant Duhamel pouch has never been reported in Korea. We would like to present our case with acute urinary retention due to a fecaloma and suggest that fecaloma might be considered as one of the causes for acute urinary retention, especially in cases with previous Duhamel operation for repair of Hischsprung disease.

• 대한기관식도과학회:학술대회논문집
• /
• 대한기관식도과학회 1983년도 제17차 학술대회연제순서 및 초록
• /
• pp.18.1-18
• /
• 1983

1923년 Pierre-Robin이 소하악증, 설하수증 및 고궁구개(high-arched palate)혹은 구개 파열의 3주징을 처음으로 기술하였다. 기도장애가 자주 발생하고, 신속하며 적절한 치료를 필요로 한다. 이 증후군은 출생아 5만명 중 1명 정도로 드물게 발생한다. 이 증후군의 원인은 불명확하지만 하악골발달의 일차적 부전 때문으로 알려져 있다. 소하악증으로 인해 혀의 기저부가 후방으로 이동하여 인두후벽에 접근하여 기도폐쇄의 증상이 발생하며 이는 영아가 앙와위를 취할 때 악화된다. 환자를 복와위로 유지시킴으로서 경한 기도폐쇄의 문제들은 해결되지만, 섭식장애가 있을 경우에는 기도폐쇄가 악화되므로 빨리 설고정술을 실시하는 것이 좋으나 기관절개술은 최대한 피하는 것이 좋다. 설고정술에는 Douglas 수술법, Kirschner wire 삽입법 및 Duhamel 봉합법 등이 있다고 알려져 있다. 저자들은 최근 호흡곤란 및 섭식장애를 주소로 내원한 출생 2개월 된 남아에서 소하악증, 설하수증 및 고궁구개 (high-arched palate)를 확인하였으며 Duhamel 봉합법으로 기도폐쇄의 증상이 호전되지 않아 기관절개술을 시행한 후 관찰 중인 이 환자를 Pierre-Robin Syndrome으로 생각하기에 보고하는 바이다.

• Advances in pediatric surgery
• /
• 제10권1호
• /
• pp.1-8
• /
• 2004

A re-pull-through operation for Hirschsprung's disease is performed when the primary operation has failed because of the remnant or acquired aganglionosis, anastomotic stricture and/or fistula. The purpose of this study is to review our experience of the re-pull-through procedure for Hirschsprung's disease. From May 1978 to July 2003, 26 patients who underwent re-pull-through operations at the Department of Pediatric Surgery, Seoul National University Children's Hospital, were studied retrospectively by means of chart review as well as telephone interview. The mean age at primary operation and re-operation were llmonths (2 months - 10 years) and 43 months (1 year - 23 years 3 months), respectively. Initial operation for Hirschsprung's disease was Duhamel's procedure in 17, Swenson's in 6 and Soave's 3. Causes of failure of primary operation were remains of secondary aganglionic segment (n=23), vascular arcade injury (n= 1), rectoperineal fistula (n=2, due to mesenteric torsion and poor blood supply), Mean interval between the primary operation and the re-operation was 34 months (6 months-22 years). Reoperation methods were Soave's in 12, Duhamel's in 8, APSP (abdomino-posterosagittal pull-through procedure) in 5, and Swenson's in 1 case. In 2 cases of repeated rectoperineal fistula or rectourethral fistula, re-APSP were performed 3 times, respectively. A total of 29 re-pull-through operations were performed. Postoperative complications were wound infection (n=1), adhesive ileus (n=1), rectoperineal fistula (n=3), rectourethral fistula (n=2), and death due to pulmonary embolism (n=1). Mean follow up period of reoperation was 78 months (1 month-23years). In current state, 2 patients have an ileostomy because of repeated rectoperineal fistula and rectourethral fistula. Of the remaining 23 patients, 21(91 %) are totally continent with or without minimal soiling. Reoperation for Hirschsprung's disease was effective and safe for the patients complicated to the initial pull-through operation.

• Journal of Yeungnam Medical Science
• /
• 제24권1호
• /
• pp.11-23
• /
• 2007

Hirschsprung's disease is one of the most common causes of intestinal obstruction in neonates and infants. The underlying pathology of this disease is the absence of the ganglion cells in both the myenteric (Auerbach's) plexus and the submucosal (Meissner's) plexus. Since Hirschsprung's report in 1886, there have been thousands of papers on Hirschsprung's disease but the cause of the absence of the ganglion cells has not been identified. Hirschsprung's disease can be successfully treated with the Swenson, the Duhamel, and the Soave operations even though the pathogenesis is unknown. With the recent progress of molecular biology and genetics, a more detailed approach to the pathogenesis of Hirschsprung's disease can be undertaken. In addition, there have been recent developments in the surgical approach. In this review, recent advances in surgery for Hirschsprung's disease are presented.

• Advances in pediatric surgery
• /
• 제2권1호
• /
• pp.33-41
• /
• 1996

This report present the result of the national survey of pediatric surgeons' preferences on diagnosis and treatment of Hirschsprung's disease(HD) carried out in 1993. The questionnaires were sent to twenty-seven members of the Korean Association of Pediatric Surgeons (KAPS) working in twenty-four institutions. The questionnaires were designed to determine the individual surgeon's preference for the methods of diagnosis and treatment of the disease. Twenty-three pediatric surgeons from twenty institutions returned completed forms. The total number of patients diagnosed with HD in 1992 was 190 in this group. The estimated incidence of HD was 1/3,900. The most important symptom was delayed meconium passing and the most preferred diagnostic procedure was barium study. Anorectal manometric examination was carried out by 13 pediatric surgeons and 19 confirmed the diagnosis before operation by rectal biopsy, 12 with full-thickness biopsy and 7 with suction. Frozen section biopsy during operation was done by 22 surgeons. Eight surgeons did one stage operation if the age of the patient is suitable. Definitive operation was usually done at the age of 6 to 11 months. The most preferred operation was Duhamel procedure done by 19. Enterocolitis was the most serious complication of HD. Most of patients had normal continence within 6 to 12 months after operation. The follow-up period was less than 6 years in 16 surgeons. The results were presented at the 9th annual meeting of KAPS in June of 1993. This is the first national survey of HD and it can provide guidelines of diagnosis and treatment of Hirschprung's disease even though it is not a detailed study of patient data.

• Advances in pediatric surgery
• /
• 제7권1호
• /
• pp.15-20
• /
• 2001

The pathophysiology of Hirschsprung's disease (HD) is not fully understood, but recent studies have disclosed that neural cell adhesion molecule (NCAM) and glial cell line-derived neurotrophic factor (GDNF) play important roles in the formation of aganglionic bowel of Hirschsprung's disease. To evaluate the roles of NCAM and GDNF in HD, immunohistochemical analysis was performed using formalin-fixed and paraffin-embedded tissue sections. On the basis of the results, we tried to evaluate them as diagnostic markers. The specimens were obtained from 7 patients with HD who underwent modified Duhamel operation. The diagnosis was based on the clinical findings and the absence of ganglion cells in the nerve plexuses by routine microscopy. NCAM immunoreactivity was found in the nerve plexuses and scattered nerve fibers in the smooth muscle layers of ganglionic segments. In aganglionic segments, the number of NCAM positive nerve fibers in the smooth muscle layers was significantly reduced compared with ganglionic segments. In two cases the nerve plexuses in aganglionic segments, NCAM was negligible. The smooth muscle cells showed diffuse immunoreactivity for GDNF and the staining intensity was not different in the aganglionic and ganglionic segments. However, higher expression of GDNF in the nerve plexus of the ganglionic segments was noted comparing to aganglionic segments. These data suggest that both NCAM and GDNF may play important roles in pathogenesis of Hirschsprung's disease and immunohistochemical staining for NCAM can be used as an ancillary diagnostic tool for HD.

• Advances in pediatric surgery
• /
• 제16권2호
• /
• pp.162-169
• /
• 2010

남녀의 비는 1.8로 남아가 많았으며, 진단시 나이는 76.9 % 에서 4주 이내 신생아시기에 진단되었다. 임상증상은 복부팽만 84.6 %, 태변 배출지연이 46.1 %, 설사 와 변비가 23.1 % 였다. 무신경절의 범위가 89.7 %에서 직장 및 에스자형 결장에 한정되었으며, 전결장에 침범한 경우가 8 %였다. 69.2 %에서 결장조루술 후 근치적 수술을 시행하였으며, 30.8 %에서 바로 근치적 수술을 시행하였다. 수술 후 6개월 이상 추적은 54 % 였으며, 추적 관찰 환자 중 14.3 %에서 대변 지림을 보였고, 19.0 %에서 변비를 보였다.