• Journal of Yeungnam Medical Science
• /
• v.24 no.1
• /
• 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
• /
• v.10 no.1
• /
• pp.60-62
• /
• 2004

Leveling colostomy with a frozen-section biopsy in a Hirschsprung's disease is an important factor for a successful procedure. Two neonatal cases of Hirschsprung's disease in the descending colon are reported. In both cases, loop ileostomy was established because of the unavailability of frozen-section biopsy on an emergency basis. At the time of definitive procedure of the first case, transition zone at the splenic flexure was noted and was compatible with the frozen section biopsy. In the second case, an unexpected longer resection at a higher level than transition zone was required because of the poor vascularity after dissection. In conclusion, a leveling colostomy should be selected as a choice in long-segment Hirschsprung's disease. Confirming preservation of the marginal artery of Drummond is particularly important in case of Hirschsprung's disease in the descending colon.

• Advances in pediatric surgery
• /
• v.8 no.1
• /
• pp.62-63
• /
• 2002

Various surgical techniques for Hirschsprung's disease including total colonic aganglionosis have been performed with similar results. The type of redo pull-through procedure is determined by the cause of failure and the type of primary pull-through.

• Advances in pediatric surgery
• /
• v.8 no.1
• /
• pp.68-70
• /
• 2002

Enterocolitis associated with Hirschsprung's disease has been a major cause of morbidity and even mortality, and before and after definitieve surgical treatment. It shows typical clinical characteristics, however, its pathogenesis has been poorly understood. Treatment is diverse, and consists of conservative tertment with intravenous hydration, antibiotics and rectal wash out, and surgical tertment with temporatory enterostomy, and other surgical procedures.

• Advances in pediatric surgery
• /
• v.1 no.2
• /
• pp.186-189
• /
• 1995

Neonatal intestinal perforation is mainly caused by necrotizing enterocolitis, intestinal atresia, meconium ileus or unknown etiology. Occasionally, Hirschsprung's disease presents with neonatal intestinal perforation, of which, it is known that total colonic aganglionosis is common. Therefore, Hirschsprung's disease should be considered as a cause of neonatal intestinal perforation. The authors have experienced 3 cases of neonatal Hirschsprung's disease associated with colonic perforations. Cecal perforations were noted in 2 cases with aganglionosis from descending colon and sigmoid perforation in a case with aganglionosis in rectum. These cases will be discussed with literature review.

• Advances in pediatric surgery
• /
• v.7 no.2
• /
• pp.130-136
• /
• 2001

The aim of this study was to evaluate the short-term outcomes of the trans anal one-stage pull-through procedure(TOP) in Hirschsprung's disease. Eight patients aged 3 weeks to 8 months with Hirschsprung's disease underwent this procedure. A rectal mucosectomy was performed from just proximal to dentate line to the level of peritoneal reflexion, where muscle layer was incised circumferentially. Rectosigmoid was mobilized out through the anus, and full-thickness frozen biopsy was taken for confirmation of ganglionic cells. After the rectal muscular cuff was divided longitudinally in the posterior aspect, aganglionic bowel was removed and ganglionic colon was anastomozed to the anus. The mean operating time was 161 minutes, and the mean hospital stay after operation was 3.8 days. Five patients had three to four bowel movement per day without other therapy at mean postoperative 39.2 days. Although long-term follow-up will be required, the TOP might be the new alternative surgical procedure for Hirschsprung's disease.

• Advances in pediatric surgery
• /
• v.19 no.2
• /
• pp.130-139
• /
• 2013

The application of laparoscopic techniques for the surgical management of Hirschsprung's disease is the recent trend. We described the surgical technique and postoperative long-term outcomes of the one-stage, laparoscopic-assiseted endorectal pull-through operation for Hirschsprung's disease. The technique uses three to four small abdominal ports. Laparoscopic mobilization of the sigmoid colon and rectum is performed and marginal artery-preserving colon pedicle is prepared. The rectal mobilization is performed using a transanal endorectal sleeve technique. The anastomosis is performed 0.5~1 cm above the dentate line. The age at surgery ranged from 6 days to 4 years. The average operative time was 144 minutes. Almost all of the patients passed stool and flatus within 36 hours of surgery. The average hospital stay after surgery was 6.5 days. Among 42 patients, 32 patients older than 3 years old were evaluated for function on defecation. All 32 patients have been continent, of those who needed laxatives were 11 (34.3%) due to constipation and overflow incontinence. Four children (12.5%) have remained dependent on laxatives. Laparoscopic-assisted endorectal pull-through operation for Hirschsprung's disease appears to be safe, provides the less pain, shorter time to full feeding, shorter hospital stay, and excellent cosmetic outcomes. Helping patients and parents ensure the quality of life, they should be provided with counseling, education, and longer-term follow-up care.

• Advances in pediatric surgery
• /
• v.4 no.2
• /
• pp.172-175
• /
• 1998

With the recent advances in instruments and techniques, laparoscopic procedure have extended to neonates with congenital anomalies. The author reports a 6-day-old boy with Hirschsprung's disease, treated successfully by the laparoscopic endorectall pull-through procedure. The technique and its potential role in the treatment of Hirschsprung's disease are described. One camera port and three working ports were used for access to the peritoneal cavity. The descending and sigmoid colon were mobilized laparoscopically. The submucosal dissection was done transanally. The colon was then pulled down in continuity, divided above the transition zone, and secured to the anal mucosa about 10 mm above the pectinate line. Author concluded that endorectal pull-through can be performed safety with the laparoscope.

• Advances in pediatric surgery
• /
• v.13 no.1
• /
• 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.

• Advances in pediatric surgery
• /
• v.10 no.1
• /
• 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.