hirschsprung s disease ( hd ) is a congenital malformation that is mainly found in early infancy . the incidence is approximately 1 in 5000 live births . in developed countries , about 90% of all patients with hd the remaining cases are usually diagnosed in childhood but can even be diagnosed in adulthood . however , several studies have shown that one - stage transanal endorectal pull - through ( taerpt ) has similar long - term functional outcomes as the staged operation , , . in our hospital , we have been doing one - stage laparoscopy - assisted endorectal pull - through for late - presenting hd to improve the cosmetic outcome . all consecutive children who were older than 3 years at the time hd was diagnosed and underwent one - stage laparoscopy - assisted endorectal pull - through at the asan medical center and haeundae paik hospital in 20102012 were identified by retrospective review of the medical records . the following data were extracted : age , gender , body weight , operative findings , operation time , and post - operative bowel habits . the patients were followed up at outpatient clinics and the parents were interviewed regarding the functional outcome of surgery , namely , frequency of bowel movements , soiling , and constipation . voluntary bowel movements were defined as the urge to defecate , the capacity to verbalize this feeling , and the ability to hold the bowel movement . soiling was categorized according to a three - grade system : grade 1 was defined as occasional soiling ( 12 times per week ) ; grade 2 was defined as daily soiling but associated social problems were absent ; and grade 3 was defined as constant soiling with social problems . constipation was also categorized according to a three - grade system : grades 1 and 2 were defined as constipation that could be managed by changing the diet or laxative treatment , respectively . the study protocol was approved by our institutional review board ( amc irb 2013 - 0977 ) . informed written consent to participate in this case series report was obtained from the parents of the patients . three methods of pre - operative fecal disimpaction were used , namely , fasting and conventional enema , oral polyethylene  glycol , and rectal irrigation under general anesthesia . fasting and repeated enema was used first . if there was no improvement , the patients who lacked an intestinal obstruction ate oral polyethylene  glycol . the main operation was delayed at least 3 days to allow the intestine to recover from edema . after co2 insufflation of the peritoneal cavity , three 5-mm trocars ( one for the camera and two for the working ports ) were inserted . after identifying the transition zone , the level of pull - through was tagged after confirmation of the presence of ganglion cells by frozen biopsy . it is helpful to mobilize the splenic flexure and divide the superior rectal vessels and mesocolon by using an ultrasonic scalpel . deep pelvic dissection was extended circumferentially just outside the rectal wall , distal to peritoneal reflection . a circumferential incision was made in the mucosal layer 1  cm above the dentate line . submucosal dissection above the dentate line was performed by using a bipolar coagulator to achieve effective hemostasis . the rectum and sigmoid colon were then pulled down through the rectal sleeve until the tagged bowel emerged . the colon was transected above this point and anastomosed to the anal mucosa by using absorbable sutures . there were eight patients with late - presenting hd who underwent one - stage laparoscopy - assisted endorectal pull - through during the study period . the median age was 9.9 ( range , 3.414 ) years , the median weight was 27.6 ( range , 1450 )  kg , and the median height was 134.3 ( range , 91159.2 )  cm . the constipation usually started at birth and worsened when the patient started on a solid diet . all children had severe fecal impaction , as shown by simple abdominal x - rays . to diagnose the hd , barium enema , rectal manometry , and rectal biopsy were performed . all children underwent barium enema except one : the exception was due to severe fecal impaction . four children underwent rectal manometry ; in three cases , the rectoanal inhibitory reflex was absent . it was either suction biopsy ( n  =  3 ) or a full thickness rectal biopsy ( n  =  5 ) . only one child achieved satisfactory bowel decompression by conventional enema and fasting . the transitional zone was rectosigmoid junction in 4 patients , and was rectum in 4 patients . a median of 35 ( range , 2344 )  cm of colon was resected . the median maximal diameter of the resected bowel was 6 ( range , 313.5 )  cm . the median diameter of the proximal bowel that was anastomosed to the anus was 5 ( range , 2.56 )  cm . it is usually acceptable when the circumference of the bowel is twice that of the anus . the patients started a diet a median of 5 ( range , 48 ) days after the operation and were discharged a median of 11.5 ( range , 724 ) days post - operatively . there were no major intraoperative and early post - operative complications , including anastomotic leakage , wound infection , and enterocolitis ( table 1 ) . the median follow - up period was 37 ( range , 1756 ) months . in the first month , the frequency of defecation was up to 20 times per day . within 3 months , follow up visit , all but one patient ( 87.5% ) could feel and verbalize their bowel movements . however , one patient required regular enemas to manage the constipation ( table 2 ) . all consecutive children who were older than 3 years at the time hd was diagnosed and underwent one - stage laparoscopy - assisted endorectal pull - through at the asan medical center and haeundae paik hospital in 20102012 were identified by retrospective review of the medical records . the following data were extracted : age , gender , body weight , operative findings , operation time , and post - operative bowel habits . the patients were followed up at outpatient clinics and the parents were interviewed regarding the functional outcome of surgery , namely , frequency of bowel movements , soiling , and constipation . voluntary bowel movements were defined as the urge to defecate , the capacity to verbalize this feeling , and the ability to hold the bowel movement . soiling was categorized according to a three - grade system : grade 1 was defined as occasional soiling ( 12 times per week ) ; grade 2 was defined as daily soiling but associated social problems were absent ; and grade 3 was defined as constant soiling with social problems . constipation was also categorized according to a three - grade system : grades 1 and 2 were defined as constipation that could be managed by changing the diet or laxative treatment , respectively . the study protocol was approved by our institutional review board ( amc irb 2013 - 0977 ) . informed written consent to participate in this case series report was obtained from the parents of the patients . three methods of pre - operative fecal disimpaction were used , namely , fasting and conventional enema , oral polyethylene  glycol , and rectal irrigation under general anesthesia . fasting and repeated enema was used first . if there was no improvement , the patients who lacked an intestinal obstruction ate oral polyethylene  glycol . the main operation was delayed at least 3 days to allow the intestine to recover from edema . after co2 insufflation of the peritoneal cavity , three 5-mm trocars ( one for the camera and two for the working ports ) were inserted . after identifying the transition zone , the level of pull - through was tagged after confirmation of the presence of ganglion cells by frozen biopsy . it is helpful to mobilize the splenic flexure and divide the superior rectal vessels and mesocolon by using an ultrasonic scalpel . deep pelvic dissection was extended circumferentially just outside the rectal wall , distal to peritoneal reflection . a circumferential incision was made in the mucosal layer 1  cm above the dentate line . submucosal dissection above the dentate line was performed by using a bipolar coagulator to achieve effective hemostasis . the rectum and sigmoid colon were then pulled down through the rectal sleeve until the tagged bowel emerged . the colon was transected above this point and anastomosed to the anal mucosa by using absorbable sutures . after co2 insufflation of the peritoneal cavity , three 5-mm trocars ( one for the camera and two for the working ports ) were inserted . after identifying the transition zone , the level of pull - through was tagged after confirmation of the presence of ganglion cells by frozen biopsy . it is helpful to mobilize the splenic flexure and divide the superior rectal vessels and mesocolon by using an ultrasonic scalpel . deep pelvic dissection was extended circumferentially just outside the rectal wall , distal to peritoneal reflection . a circumferential incision was made in the mucosal layer 1  cm above the dentate line . submucosal dissection above the dentate line was performed by using a bipolar coagulator to achieve effective hemostasis . the rectum and sigmoid colon were then pulled down through the rectal sleeve until the tagged bowel emerged . the colon was transected above this point and anastomosed to the anal mucosa by using absorbable sutures . there were eight patients with late - presenting hd who underwent one - stage laparoscopy - assisted endorectal pull - through during the study period . the median age was 9.9 ( range , 3.414 ) years , the median weight was 27.6 ( range , 1450 )  kg , and the median height was 134.3 ( range , 91159.2 )  cm . the constipation usually started at birth and worsened when the patient started on a solid diet . all children had severe fecal impaction , as shown by simple abdominal x - rays . to diagnose the hd , all children underwent barium enema except one : the exception was due to severe fecal impaction . four children underwent rectal manometry ; in three cases , the rectoanal inhibitory reflex was absent . it was either suction biopsy ( n  =  3 ) or a full thickness rectal biopsy ( n  =  5 ) . only one child achieved satisfactory bowel decompression by conventional enema and fasting . the transitional zone was rectosigmoid junction in 4 patients , and was rectum in 4 patients . a median of 35 ( range , 2344 )  cm of colon was resected . the median maximal diameter of the resected bowel was 6 ( range , 313.5 )  cm . the median diameter of the proximal bowel that was anastomosed to the anus was 5 ( range , 2.56 )  cm . it is usually acceptable when the circumference of the bowel is twice that of the anus . the patients started a diet a median of 5 ( range , 48 ) days after the operation and were discharged a median of 11.5 ( range , 724 ) days post - operatively . there were no major intraoperative and early post - operative complications , including anastomotic leakage , wound infection , and enterocolitis ( table 1 ) . in the first month , the frequency of defecation was up to 20 times per day . within 3 months , follow up visit , all but one patient ( 87.5% ) could feel and verbalize their bowel movements . another two patients treated with laxatives . however , one patient required regular enemas to manage the constipation ( table 2 ) . while most cases of hd manifest during the neonatal period , some of hd is diagnosed during childhood or adulthood , . such late - presenting hd cases are usually present with severe constipation , abdominal distension , vomiting , and recurrent enterocolitis . barium enema is not helpful for very short segment hd and total colonic aganglionosis , because the transitional zone tends to be very short in late - presenting hd cases , , . the success with which rectal manometry diagnoses hd also varies , ranging from 75% to 100% of cases , , . in our study , rectal manometry showed 25% of false negative . we usually employ the suction biopsy technique but it can be difficult to get an adequate specimen that includes submucosal tissue in children who have a thickened bowel wall . in such cases , a full thickness rectal biopsy under general anesthesia is helpful . in our opinion , combining rectal irrigation with a rectal full thickness biopsy is an good option as it allows the simultaneous diagnosis of hd and preparation of the bowel . for many years , one - stage operations were avoided in older patients with hd because of difficulties in decompressing the bowel , , . chronic constipation with a huge load of feces often induces colonic obstruction with fecaloma and gradual dilatation with severe wall thickening . the presence of impacted feces is also an enormous obstacle when performing one - stage endorectal pull - through , which is why many surgeons recommend colostomy as the first stage , , , . in a recent study of 41 children who presented with hd after 1 year of age , 35 ( 85.4% ) underwent colostomy to achieve decompression . of these , 68.6% patients experienced colostomy - related complications that included skin excoriation ( n  =  11 ) , stoma prolapse ( n  =  11 ) , and stoma stenosis ( n  =  2 ) . 26% of patients suffered from complications after colostomy closure . in our series , half of the patients were adolescents . enterostomy can devastate the daily life of an adolescent ; it can also distort the patient s self - image and induce psychological damage . indeed , these considerations caused us to favor a one - stage operation for older children with hd . to overcome fecal impaction , we tried various methods to clean the colon before the operation , including massive colon irrigation under general anesthesia . late - presenting hd also associates with thickened mesentery , inflamed mucosa , longstanding dilated colon , and rigid pelvis , all of which impose further technical difficulties . in newborns and younger infants , the fixation of the colon to the retroperitoneum is looser , which makes it easier to resect long segments of colon through the anus . by contrast , in children older than 3 years , the transanal procedure of submucosal dissection is very difficult because of the mucosal inflammation and fibrotic changes resulting from recurrent enterocolitis . moreover , ligation of the thick mesenteric vessels through the anal canal often results in tearing of the stretched vessels and bleeding . to prevent bleeding complications . moreover , laparoscopic ligation of the mesenteric vessels with an ultrasonic scalpel prior to submucosal dissection can reduce the possibility of massive bleeding . laparoscopic assistance provides crucial clues for determining the correct level of bowel resection needed to ensure tension - free anastomosis . also , laparoscopy is essential for protecting the sphincter . dilated bowel and thickened mesentery can cause over - dilation of the sphincter , which results in a weakened fecal continence mechanism . in general , patients with late - presenting hd take longer to recover normal bowel habits than neonates because older children have a stretched pelvic floor and a decreased sense of defecation . in our series , , this dropped to 12 defecations per day . to manage constipation after surgery , we used laxatives and regular enemas and diet changes . at the end of the median follow - up period of 37 ( range , 2356 ) months , one patient still had to undergo regular enemas because of constipation and one patient had intermittent soiling . although , surgical treatment of late - presenting hd will be more difficult than in neonates , we found that one - stage laparoscopy - assisted endorectal pull - through was feasible in patients with late - presenting hd , even in cases with a large fecaloma with obstruction . the technical difficulties of this approach are ameliorated by preparing the bowel by rectal irrigation under general anesthesia and by using laparoscopy and a bipolar coagulator to perform the surgery . however , the functional outcome of our patients was still not as satisfactory as that of neonates . nevertheless , it also takes a long time to restore bowel function after staged operations for late - presenting hd . to promote gradual improvement over time one - stage laparoscopy - assisted endorectal pull - through in children with late - presenting hd is feasible and safe avoiding enterostomy complications . there are no relationships / conditions / circumstances that present a potential conflict of interest . the study protocol was approved by our institutional review board ( amc irb 2013 - 0977 ) . all patients provided written informed consent for publication of this case series report and the accompanying images . a copy of the written consent is available for review by the editor - in - chief of this journal on request . so hyun nam , m.d . , ph.d . and dae yeon kim , m.d . , ph.d .
highlightsthe operation of hirschsprung s disease in children older than 3 years is traditionally staged operation with enterostomy because of severe dilated bowel.we did one stage laparoscopy - assisted endorectal pull - through with short segment hirschsprung s disease for late presented children.one stage laparoscopy - assisted endorectal pull - through is safe with excellent cosmetic benefit.constipation is still concerning problem for late presented hd , but it is comparable with staged operation .