Transanal Pull-through Operation Research Articles

Congenital hyperinsulinism associated with Hirschsprung\u2019s disease\u2014a report of an extremely rare case

BackgroundCongenital hyperinsulinism (CH) is a rare disease, characterized by severe hypoglycemia induced by inappropriate insulin secretion from pancreatic beta-cells in neonate and infant. Hirschsprung’s disease (HD) is also a rare disease in which infants show severe bowel movement disorder. We herein report an extremely rare case of combined CH and HD.Case presentationThe patient was a full-term male infant who showed poor feeding, vomiting, and hypotonia with lethargy on the day of birth. He was transferred to tertiary hospital after a laboratory analysis revealed hyperinsulinemic hypoglycemia. The patient showed remarkable abdominal distension without meconium defecation. An abdominal X-ray showed marked dilatation of the large bowel. He was diagnosed with CH (nesidioblastosis) associated with suspected HD. He was initially treated with an intravenous infusion of high-dose glucose with the intermittent injection of glucagon. This was successfully followed by treatment with diazoxide and octreotide (a somatostatin analog). At 8 months of age, HD was confirmed by the acetylcholinesterase staining of a rectal mucosal biopsy specimen, and a transanal pull-through operation was performed to treat HD. At 14 months of age, subtotal pancreatectomy was performed for the treatment of focal CH located in the pancreatic body. His postoperative course over the past 12 years has been uneventful without any neurologic or bowel movement disorders.ConclusionsAlthough it is extremely rare for CH to be associated with HD, associated HD should be considered when a patient with CH presents severe constipation.

Are all patients with short segment Hirschsprung's disease equal? A retrospective multicenter study.

Short segment Hirschsprung's disease (HSCR) carries a better prognosis than long segment disease, but the definition of short is controversial. The objective of this study is to determine anatomically the extent of disease involvement that would be associated with a better functional outcome. This is a retrospective multicenter (n = 3) study with patients (≥ 3years) who had transanal pullthrough operation done for aganglionosis limited to the recto-sigmoid colon were reviewed. The extent of disease involvement and bowel resection was retrieved by reviewing the operative records as well as histopathological reports of the resected specimens. Clinical assessment was performed according to the criteria of a seven-itemed bowel function score (BFS) (maximum score = 20). Manometric assessment was performed with anorectal manometry. The study period started from 2003 to 45 patients were studied with median age at assessment = 52.0 months and operation = 3.0months. The disease involvement was categorized into upper sigmoid-descending colon (DC) (n = 8), sigmoid colon (SC) (n = 12), upper rectum (UR) (n = 14) and lower rectum (LR) (n = 11) according to the level of normal biopsy result. There was no significant difference in the age of assessment between the four groups. The median BFSs in the DC, SC, UR and LR were 13, 15, 17 and 17, respectively (p = 0.01). Nine patients from the DC and SC groups reported soiling for more than twice per week. Sub-group analysis comparing patients with and without the entire sigmoid colon resected revealed worse functional outcomes in terms of the incidence of soiling (40.7 vs 22.2%, p = 0.05) and the BFS (14 vs 18, p = 0.04) in the former group. Anorectal manometry did not reveal any significant difference between the four groups, but a higher proportion of patients in the UR and LR groups appeared to have a normal sphincter resting pressure (DC vs SC vs UR vs LR = 62.5 vs 75.0 vs 85.7 vs 80.0%, p = 0.10). Patients with short segment HSCR are not equal at all. HSCR patients with aganglionosis limited to the rectum without the need of removing the entire sigmoid colon have a better bowel control and overall functional score. Less bowel loss and colonic dissection maybe the underlying reasons. Although future studies with a larger sample size and a longer follow-up period are required to validate the results of this study, it has provided a new insight to the current understanding of short segment disease in HSCR.

Laparoscopic-assisted pull-through operation for Hirschsprung's disease: a systematic review and meta-analysis.

In the last two decades, laparoscopic-assisted pull-through (LAPT) has gained much popularity in the treatment of Hirschsprung's disease. The aim of this meta-analysis was to determine the long-term outcome of patients treated laparoscopically. A systematic literature-based search for relevant cohorts was performed using the terms "Hirschsprung's disease and Laparoscopy", "Laparoscopic-assisted pull-through outcome", "Laparoscopic-assisted Soave pull-through" "Laparoscopic-assisted Swenson pull-through" and Laparoscopic-assisted Duhamel pull-through. The relevant cohorts of laparoscopic operated HD were systematically searched for outcome regarding continence, constipation, secondary surgery related to the laparoscopic approach and enterocolitis. Pooled incidence rates and odds ratios (ORs) with 95% confidence intervals (CI) were calculated using standardized statistical methodology. Sixteen studies met defined inclusion criteria, reporting a total of 820 patients. All studies were retrospective case series, with variability in outcome assessment quality and length of follow-up. The median cohort size consisted of 28 patients (range 15-218). In the long-term follow-up, 97 patients (11.14%) experienced constipation (OR 0.06, 95% CI 0.05-0.08, p<0.00001), 53 (6.46%) incontinence/soiling (OR 0.01 95% CI 0.01-0.01, p<0.00001), 75 (9.14%) recurrent enterocolitis (OR 0.02 95% CI 0.01-0.02, p<0.00001) and 69 (8.4%) developed complications requiring secondary surgery (OR 0.01 95% CI 0.01-0.02, p<0.00001). Overall events in long-term follow-up occurred in 225 (27.5%) patients (OR 0.24 95% CI 0.20-0.30, p<0.00001). This meta-analysis shows that nearly one-third of the patients continue to have long-term bowel problems, such as constipation, soiling and recurrent enterocolitis following LAPT. Many patients treated by LAPT require secondary surgery. Large randomized studies with long-term follow-up are necessary to determine the difference in outcome between LAPT and completely transanal pull-through operation.

Long-term health-related quality of life for patients with Hirschsprung's disease at 5 years after transanal endorectal pull-through operation.

This study aims to evaluate the health-related quality of life (HRQoL) for children as well as parents' satisfaction 5-7 years after transanal pull-through operation of Hirschsprung's disease. The PedsQL™ 4.0-Core Measurement Model-and the PedsQL™ 3.0 Healthcare Satisfaction Generic Module were used to evaluate the quality of life and parents' satisfaction, respectively. Parents of 53 children who were operated by transanal pull-through operation 5-7 years earlier were included in the study. The HRQoL of children was generally good. The mean scores were 87.3, 95.5, 92.3 and 93.2 % for physical, emotional, social and school functioning, respectively. Overflow incontinence was significantly affecting physical (ß = -.261), emotional (ß = -.299), social (ß = -.42) and school functioning (ß = -.534). Constipation significantly affected emotional (ß = -.742), social (ß = -.108) and school (ß = -.282) functioning. Failure to thrive was significantly affecting social (ß = -.215) and school functioning (ß = -.176). Age at time of surgery was affecting physical, emotional, social and school functioning (ß = -.686, -.627, -.865 and -.907, respectively). Parents were generally satisfied from the healthcare service with an overall satisfaction of 90.7 with the least satisfaction (79.8) in family inclusion category. Transanal pull-through operation disease showed a good postoperative long-term HRQoL. Overflow incontinence and age at time of surgery had a significant negative effect on all the aspects of children's HRQoL. Parental satisfaction was good and could be improved by more family inclusion.

Laparoseopy-assisted transanal endorectal pull-through surgery for Hirschsprung' s disease in 63 children

Objective To compare the efficacy of laparoseopy-assisted transanal endorectal pullthrough surgery with that of transanal Soave technique without laparoseopy for Hirschsprung' s disease.Methods We reviewed the data of 121 children with Hirsehsprung' s disease who had been treated during January 2004 to November 2012.The patients were divided into 2 groups:laparoseopy-assisted transanal endorectal pull-through surgery (n=63) and transanal Soave technique without laparoseopy (n=58).The surgical duration,time of passing feces or flatus,and complications were analyzed.Results There were no differences between the two groups in severity of enterocolitis,anal stenosis,anastomotic complications,and time of passing flatus.The surgical duration (2 ± 0.8)h was significantly shorter (t=2.586,P＜0.05); and the amount of intraoperative bleeding (20 ± 3.5)ml was smaller in laparoseopy group than in non-laparoseopy group (t=1.834,P＜0.05).Conclusions Laparoseopy-assisted transanal pull-through surgery is a more extended,effective,safer procedure. Key words: Hirschsprung' s disease; Modified Soave; Laparoscopy

Comparison of two techniques for single-stage treatment of Hirschsprung disease in neonates

Introduction: The aim of this study is to determine the incidence, morbidity and functional outcome of two techniques for Hirschsprung disease (single-stage Duhamel pull-through and tans-anal pull-through) in the neonatal age group. Method: All the neonates with Hirschsprung disease with short aganglionic segment (transition zone at recto-sigmoid junction) admitted during the last 6 years were included. A total of 44 cases were included in the study of which 24 were treated by Duhamel pullthrough (group A) and 20 were treated with trans-anal pull-through (TEPT, group B). The time of operation, acceptance of first oral feed, duration of hospital stay, complications and anal continence were evaluated for both procedures. Result: There was no difference in age and sex and length of the aganglionic segment of bowel. The average operative time was 140-180 min. in group A and 120-170 min. in group B. Per-oral feed was started on the 5-7th day in group A and on the 5-6th day in group B. Hospital stay was longer in group A whereas complications were more common in group B and were found to be statistically significant. Anal continence was almost similar in both groups. Conclusion: Single-stage Duhamel pull-through operation is better than trans-anal pull-through operation in long-term follow-up although duration of hospital stay was shorter in TEPT.

Stooling patterns and colonic motility after transanal one-stage pull-through operation for Hirschsprung's disease in children

Background The transanal one-stage endorectal pull-through operation for Hirschsprung's disease is relatively new and makes assessment of the functional outcome and colonic motility difficult. The aim of this study was to evaluate the stooling patterns and colonic motility after a one-stage transanal pull-through operation for Hirschsprung's disease in children. Methods Twenty-two children who underwent a one-stage transanal pull-through operation for Hirschsprung's disease were followed up for at least 6 months. The children (17 boys and 5 girls) were from 12 months to 13 years of age (mean age, 4 years). All patients had an aganglionic segment confined to the rectosigmoid area (confirmed by preoperative barium enema and postoperative histology). Clinical outcome was assessed by interviews and questionnaires, and children were divided into symptomatic and nonsymptomatic groups. Contrast barium enema and defecography and determination of total and segmental colonic transit time (using radio-opaque markers) were performed on all 22 children. Results The stooling patterns were considered satisfactory in 17 children. Of all the children, the mean stool times were 1 to 2 per day and only 2 were 8 to 10 per day; postoperative soiling was found in 4, constipation was observed in 2, and Hirschsprung-associated enterocolitis in 1. There was no incontinence, cuff infection, anastomotic leak, or mortality noted. Barium enema showed that the dilated and spastic colonic segment disappeared in all 22 children. The dilated sigmoid loops decreased in 17 (2 symptomatic, 15 nonsymptomatic) and disappeared in 5 (4 symptomatic, 1 nonsymptomatic). There was a significant difference between the decreasing and disappearing loop group in regard to stooling disorders ( P < .05). Postoperative defecography showed that the anorectal angle of all children was open, fixed, and significantly larger than that of the preoperative and control groups (123.3° ± 15.1° vs 84.7° ± 8.3° vs 79.0° ± 11.6°, P < .01) and larger in the symptomatic group when compared with the nonsymptomatic group (135.6° ± 15.9° vs 111.0° ± 14.3°, P < .05). Postoperatively, the total gastrointestinal transit time, left colonic transit time, and rectosigmoid colonic transit time of all the children were shorter than preoperatively (26.8 ± 8.2 vs >188 hours, P < .01; 6.3 ± 4.1 vs >60 hours, P < .01; 11.8 ± 4.4 vs >120 hours, P < .01) and similar to controls. The total gastrointestinal transit time and rectosigmoid colonic transit time of the symptomatic group were significantly shorter than the nonsymptomatic group (25.2 ± 5.6 vs 28.1 ± 10.1 hours, P < .05; 12.2 ± 6.7 vs 9.8 ± 4.0 hours, P < .05). Conclusions The stooling pattern and colonic motility are satisfactory in most children after the one-stage transanal pull-through operation for Hirschsprung's disease. Normalization of colon appearance and total and segmental colonic transit time are signs of recovery of colonic motility. Stooling disorders were noted in a few cases and may be related to decrease or disappearance of the sigmoid loop, dysfunction of the “neorectosigmoid”, an open and fixed anorectal angle, and ischemia of the pull-through segment.

Swenson revisited: a one-stage, transanal pull-through procedure for Hirschsprung’s disease

Background: Hirschsprung’s disease is a common cause of neonatal intestinal obstruction. A variety of operations have been used to treat this neurogenic form of bowel obstruction. This report describes an experience with a 1-stage abdominal and transanal pull-through operation in 15 patients with rectosigmoid disease. Methods: Fifteen infants with Hirschsprung’s disease included 11 boys and 4 girls. Median age at diagnosis was 3 days, and median age of operation was 30 days. Diagnosis was confirmed by rectal biopsy. Biopsies to confirm the transition zone were performed intraabdominally through a left lower quadrent incision. A Swenson pull-through was performed via transanal dissection. Results: There were no instances of anastomotic leaks, wound infections or postoperative bowel obstructions. All patients survived. Two had postoperative enterocolitis. Twelve of 15 patients did well and had 2–3 bowel motions per day. One with Down’s syndrome had enterocolitis and required a colostomy. Two were lost to follow up. Conclusions: A one-stage transanal pull-through procedure in infancy is a safe alternative to staged procedures for Hirschsprung’s disease.