Isolated intestinal neuronal dysplasia Type B (IND-B) in Japan: results from a nationwide survey

Purpose

Intestinal neuronal dysplasia Type B (IND-B) has been proposed to be an allied disorder of Hirschsprung’s disease (ADHD). The original histological criteria included hyperganglionosis, giant ganglia, ectopic ganglion cells and an increased AChE activity in the lamina propria. The criteria for IND-B have been gradually revised. The present diagnostic criteria are [1] more than 20 % of the submucosal ganglia contain nine or more ganglion cells and [2] the patient is older than 1 year. To clarify the current status of IND-B in Japan, a nationwide retrospective cohort study was performed.

Methods

Questionnaires were sent to 161 major institutes of pediatric surgery and gastroenterology in Japan.

Results

A total of 355 cases of ADHD were collected, including 18 cases of IND-B (5 %). Based on original criteria, 13 out of 18 cases were diagnosed as IND-B. However, only four cases met the current criteria. Three of the four patients (75 %) required pull-through operation. All of the patients exhibited giant ganglia and ganglioneuromatosis-like hyperplasia of the myenteric plexus.

Conclusions

IND-B cases matching the current criteria are thought to be quite rare and they are associated with marked hyperplasia of the myenteric plexus. “True” IND-B is a rare and intractable disease.     

Visualisation of the rectoanal inhibitory reflex with a modified contrast enema in children with suspected Hirschsprung disease

Background

Patients with Hirschsprung disease lack the normal rectoanal inhibitory reflex, which can be studied with anorectal manometry or US.

Objective

To see whether the rectoanal inhibitory reflex could be visualised with a modified contrast enema, thereby increasing the diagnostic accuracy of the contrast enema and reducing the number of rectal biopsies.

Materials and methods

Fifty-nine boys and 42 girls (median age, 12 months) with suspected Hirschsprung disease were examined with a modified contrast enema, supplemented with two injections of cold, water-soluble contrast medium, to induce the reflex. Two paediatric radiologists evaluated the anonymised examinations in consensus. The contrast enema findings were correlated with the results of rectal biopsy or clinical follow-up.

Results

Five boys and one girl (median age, 7.5 days) were diagnosed with Hirschsprung disease. The negative predictive value of the rectoanal inhibitory reflex was 100%. A contrast enema with signs of Hirschsprung disease in combination with an absent rectoanal inhibitory reflex had the specificity of 98% and sensitivity of 100% for Hirschsprung disease.

Conclusion

The modified contrast enema improves the radiological diagnosis of Hirschsprung disease. By demonstrating the rectoanal inhibitory reflex in children without Hirschsprung disease, we can reduce the proportion of unnecessary rectal biopsies.     

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

Purpose

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.

Methods

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.

Results

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).

Conclusions

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.

     

Single-incision laparoscopic surgery (SILS) for children with Crohn’s disease

Purpose

Single-incision laparoscopic surgery (SILS) has been described in adults with Crohn’s disease, but its use in pediatric Crohn’s patients has been limited. The purpose of this study was to review our experience with SILS in pediatric patients with Crohn’s disease.

Methods

A retrospective review was performed for patients diagnosed with Crohn’s disease who underwent small bowel resection or ileocecectomy at a freestanding children’s hospital from 2006 to 2014. Data collected included demographic data, interval from diagnosis to surgery, operative time, length of stay, and postoperative outcomes.

Results

Analysis identified 19 patients who underwent open surgery (OS) and 41 patients who underwent SILS. One patient (2.4 %) within the SILS group required conversion to OS. Demographic characteristics were similar between the 2 cohorts. The most common indication for surgery was stricture/obstruction (SILS 70.7 % vs. OS 68.4 %, p = 0.86), and ileocecectomy was the most common primary procedure performed (SILS 90.2 % vs. OS 100 % OS). Operative times were longer for SILS (135 ± 50 vs. 105 ± 37 min, p = 0.02). However, when the last 20 SILS cases were compared to all OS cases, the difference was no longer statistically significant (SILS 123.3 ± 34.2 vs. OS 105 ± 36.5, p = 0.12). No difference was noted in postoperative length of stay (SILS 6.5 ± 2.2 days vs. OS 7.4 ± 2.2 days, p = 0.16) or overall complication rate (SILS 24.4 % vs. OS 26.3 %, p = 0.16).

Conclusion

SILS ileocecectomy is feasible in pediatric patients with Crohn’s disease, achieving outcomes similar to OS. As experience increased, operative times also became comparable.

     

Long-term results of transanal pull-through for Hirschsprung’s disease: a meta-analysis

Introduction

Several operative techniques have been developed for the treatment of Hirschsprung’s disease (HD) in the past decades. Since one-stage transanal pull-through (TAPT) was first performed in 1998, multiple studies have shown favourable short-and midterm results compared to other techniques with shorter operation length, shorter hospital stay and lower complication rates. The aim of this meta-analysis was to determine the longterm results following TAPT for HD.

Methods

A systematic literature search for relevant articles was performed in four databases using the following terms “Hirschsprung/Hirschsprung’s disease”, “aganglionosis”, “transanal”, “pullthrough/pull-through”, “longterm/long-term” “results”, “follow-up” and “outcome”. A meta-analysis was conducted for relevant articles for one-stage transanal pull-through for HD with a minimal follow-up of median 36 months regarding constipation, incontinence/soiling, enterocolitis and secondary operations. Odds ratio (OR) with 95 % confidence intervals (CI) were calculated.

Results

Six studies with 316 patients matched the set criteria and were included in this analysis. Overall 45 (14.2 %) patients had disturbances of bowel function (OR 0.05, 95 % CI 0.03–0.07, p < 0.00001). Of these, 24 (53.3 %) patients experienced constipation, 8 (17.8 %) incontinence/soiling and 13 (28.9 %) enterocolitis. 10 (3.2 %) patients developed complications requiring secondary surgery. Most patients had a daily defecation frequency of 1–3 bowel movements 3 years postoperatively, resembling the stooling patterns of healthy controls.

Conclusion

Nearly 15 % of all patients operated with TAPT for HD continue to experience persistent bowel symptoms with constipation as the main problem. Further studies on the long-term outcome of children operated with this technique for HD are necessary to evaluate stooling patterns, urinary and sexual function as well as general quality of life during adolescence and adulthood.

     

Contrast enema findings in patients presenting with poor functional outcome after primary repair for Hirschsprung disease

Background

The radiologic evaluation of Hirschsprung disease is well described in the literature. However, there is a paucity of literature describing the appearance of the neo-rectum and colon after repair, specifically describing findings in patients with poor functional outcome, which would suggest the need for reoperation.

Objective

We describe findings on contrast enema and correlate them with surgical findings at reoperation in children with poor functional outcome after primary repair for Hirschsprung disease who suffer from bowel dysfunction that can manifest with either soiling or obstructive symptoms such as enterocolitis.

Materials and methods

Children were identified from our colorectal surgery database. At the time of abstract submission, 35 children had contrast enemas prior to reoperation. Additional children continue to present for evaluation. The majority of children included in the study had their primary repair performed elsewhere. The initial procedures included: Duhamel (n?=?11), Soave (n?=?20) or Swenson (n?=?3). One child had undergone a primary Soave repair and subsequently had a Swenson-type reoperation but continued to have a poor outcome. One child’s initial surgical repair could not be determined. Images were reviewed by a staff pediatric radiologist and a pediatric radiology fellow.

Results

Findings encountered on contrast enema in these children include a distal narrowed segment due to stricture or aganglionic/transitional zone segment (8), dilated/hypomotile distal segment (7), thickened presacral space due to compressing Soave cuff (11), dilated Duhamel pouch (8), active enterocolitis (3) and partially obstructing twist of the pull-through segment (1).

Conclusion

Multiple anatomical and pathological complications exist that can lead to bowel dysfunction in children after repair of Hirschsprung disease. Little recent literature exists regarding the radiographic findings in children. We had the opportunity to review a substantial series of these children, describe the contrast enema findings in these difficult cases and correlate them with operative findings. Radiologic evaluation is key to assessing such patients; it defines the potential anatomical problem with the pull-through and facilitates surgical planning.     

Work-related upper limb musculoskeletal disorders in pediatric minimally invasive surgery: a multicentric survey comparing laparoscopic and sils ergonomy

Purpose

Surgeons are at risk for developing work-related musculoskeletal symptoms (WMS). The present study aims to compare laparoscopy and SILS ergonomy among pediatric surgeons.

Methods

A questionnaire formed by 17 questions was mailed to 14 pediatric surgeons, seven with a large experience in laparoscopy and seven in SILS. All surgeons completed the survey. The questionnaires were focused on the type of laparoscopic or SILS activity, location and type of pain, need for drugs and its physical consequences. Results were analyzed using χ ² test.

Results

Results indicated a similar incidence of WMS with shoulder symptoms (>75 %) in both groups. In laparoscopic group this pain is evident only after a long lasting procedure, while in SILS group the pain is present after each procedure performed. SILS surgeons used painkillers and other therapies statistically more frequently than laparoscopic group (χ ² = 0.001).

Conclusions

This study confirms there is a strong association between WMS and MIS surgery. The incidence of pain is similar in both groups. Pain was present only after long lasting procedures in laparoscopic group, while SILS surgeons have pain after each procedure performed. In addition SILS surgeons use more frequently painkillers and other therapies compared to laparoscopic surgeons. In conclusion, it seems that SILS has a worse ergonomy compared to laparoscopy.     

Long-term outcomes and quality of life after subtotal colectomy combined with modified Duhamel procedure for adult Hirschsprung’s disease

Background

Subtotal colectomy combined with modified Duhamel procedure (Jinling procedure) was used in patients with adult Hirschsprung’s disease (AHD) at Jinling Hospital in the last decade. The aim of this study is to evaluate the safety, effectiveness and quality of life of Jinling procedure for AHD.

Materials and methods

All the data are from the database of the Jinling Hospital Constipation Registry System. Primary outcomes, including safety (morbidity and adverse events), effectiveness (satisfaction rate, Wexner constipation scale (WCS) and bowel function score (BFS)) and gastrointestinal quality of life index (GIQLI), were all evaluated.

Results

Fifty-nine patients were available for this study and the mean postoperative period was 44 months. Seventeen major complications were found in 11 patients and most of the complications could be managed conservatively. A significant improvement in WCS, BFS and GIQLI at 6 months postoperatively was obtained.

Conclusion

Jinling procedure is safe and effective for AHD.     

Total colonic aganglionosis: a systematic review and meta-analysis of long-term clinical outcome

Purpose

Total colonic aganglionosis (TCA) is a severe form of Hirschsprung’s disease (HD), occurring in less than 10 % of the cases. It is a challenging surgical condition and outcomes of pull-through (PT) surgery are reported to be inferior to those in patients with recto-sigmoid HD. As even large centres only see a few patients with TCA, there is little information on the long-term outcome of patients after PT operation for TCA. The aim of this meta-analysis was to investigate the long-term clinical outcome in patients with TCA.

Methods

MEDLINE^® and EMBASE^® databases were searched for relevant articles that reported the outcomes of patients with TCA published between 1980 and 2011. The search terms were “Hirschsprung’s disease”, “Total colonic aganglionosis” AND “Outcome”. All published studies containing adequate clinical data for a mean follow-up period of not less than 4 years were included. Reference lists of retrieved articles were reviewed for additional cases. Detailed records of morbidity and mortality were extracted and analysed.

Results

This search yielded 225 articles reporting on outcomes in TCA. Of these, 189 were excluded for having too short a follow-up period, small or single case series, inadequate clinical data and duplicated patient groups. Ultimately, 36 articles from 37 centres containing useful clinical information on the outcomes of TCA in 969 patients were identified. There were 152 early deaths prior to PT (15.7 %). Of 817 survivors, 739 underwent PT. The mortality rate for TCA post-PT was 5.7 %. The most frequently reported post-operative complication was enterocolitis in 42 % of the cases. 17.5 % of patients underwent subsequent major surgery including redo PT, stoma reformation or other laparotomy. Long-term follow-up data were available in 396 patients. Satisfactory or normal bowel control was reported in 60 % of the patients. Soiling, faecal incontinence or other poor outcome was reported in 33.5 % of the cases and 6.5 % of the patients had undergone conversion to a permanent ileostomy for post-operative complications.

Conclusion

This meta-analysis reveals that a large number of patients with TCA have long-term problems with bowel control.     

Effects of potato-derived protease inhibitors on perianal dermatitis after colon resection for long-segment Hirschsprung’s disease

Background

After resection of long-segment Hirschsprung??s disease, severe perianal dermatitis (SPAD) may occur because of high stool frequency and elevated concentrations of fecal pancreatic proteases. We investigated prospectively the effect of potato-derived protease inhibitors (PPI) on skin conditions in children with postoperative SPAD.

Methods

Four children (aged 12 to 24 months) with therapy-resistant SPAD after transanal endorectal pull-through for long-segment Hirschsprung??s disease received topical PPI (1% in 20% zinc ointment) with each diaper-change. Parents noted down a subjective dermatitis score daily. Photo documentation and outpatient visits were made to assess the treatment results.

Results

No adverse effects were observed after treatment with PPI. A remarkable improvement of the dermatitis in 3 of the 4 patients as well as improvements in pain and sleep disorders were observed during the PPI-treatment course.

Conclusion

The results of the study suggest that PPI may reduce otherwise intractable protease-induced skin irritation in infants.     

Nuclear transit study in children with chronic faecal soiling after Hirschsprung disease (HSCR) surgery has revealed a group with rapid proximal colonic treatment and possible adverse reactions to food

Background/purpose

Long-term problems with faecal incontinence occur in up to 50 % of patients after pull-through for Hirschsprung disease (HSCR). The cause often remains unknown, leading to empirical treatments. Using nuclear transit study, we found some patients surprisingly had rapid proximal colonic transit, suspicious of occult diarrhoea. We aimed to assess whether these patients had unrecognized adverse reactions to food.

Methods

Patients (n = 10, all males, 9.6 year; 4.25–15.5 years) with persistent faecal incontinence following pull-through for HSCR referred to the senior author and after exclusion of anatomical defects, underwent nuclear transit studies. Most (8) subsequently underwent breath hydrogen tests for sugar malabsorption and were tested for adverse reactions to food. Exclusion diets for protein allergens, lactose or fructose were then trialed.

Results

Of the 10 patients with rapid intestinal transit proven on nuclear transit study, breath hydrogen tests for fructose and/or lactose malabsorption were done in 8, and were positive in 7/8 patients. Exclusion diets contributed to either resolution or improvement in faecal incontinence in 9/10 patients.

Conclusions

Rapid transit in the proximal, ganglionated colon may be present in children with faecal incontinence following pull-through for HSCR, possibly secondary to adverse reactions to food. This study suggests that children with post-operative soiling may benefit from a transit study and hydrogen breath tests to diagnose adverse reactions to food caused by sugar malabsorption.

     

Diagnosis and surgical treatment of isolated hypoganglionosis

Background

Some patients suspected with Hirschsprung’s disease (HD), however, were diagnosed as having isolated hypoganglionosis according to the updated pathohistologic methods. This study was undertaken to investigate the diagnostic methods and the therapeutic results of isolated hypoganglionosis in children.

Methods

A retrospective analysis was made on 17 patients with isolated hypoganglionosis (hypoganglionosis group) identified pathologically after operation. The data included clinical presentations, barium enema, anorectal manometry, histochemical staining for acetylcholinesterase (AChE) before operation, histological results after operation and follow-up outcomes. The data of hypoganglionosis with HD (HD group) were compared retrospectively.

Results

Common complaint of the patients with hypoganglionosis and HD was intractable constipation. Barium enema showed typical narrowing and distended segment of the colon in 9 patients in the hypoganglionosis group (9/16) and in 15 patients in the HD group (15/18). In the hypoganglionosis group, in 15 patients who underwent anorectal manometry only 5 showed absent rectal anal inhibitory reflex, significantly lower than the rate in the HD group (17/18) (P<0.05). From 16 patients in hypoganglionosis group, positive staining for AChE was noted in 3 patients (3/16, 18.8%), significantly lower than that in the HD group (16/18, 88.9%) (P<0.05). Thirteen patients in the hypoganglionosis group received subtotal colectomy, while only 5 patients needed subtotal colectomy in the HD group. In the hypoganglionosis group, except 2 patients who suffered from mild enterocolitis after operation and recovered after conservative therapy, all patients recovered uneventfully without wound dehiscence, intestinal fistula, fecal incontinence or constipation recurrence. In the HD group, one patient suffered from anastomotic leak and got secondary operation, one patient had anastomotic stricture at 1 year after operation and recovered by dilatation, and other three patients suffered from mild enterocolitis after operation and recovered after conservative therapy.

Conclusions

Hypoganglionosis is a common disease, and could be finally confirmed by full-thickness biopsies in different bowel segments. The resection range can be estimated according to barium enema and 24-hour delayed X-ray findings, by which the satisfactory result in short-term follow-up can be obtained.     

Total colectomy for ulcerative colitis in children: when are we operating?

Purpose

Ulcerative colitis (UC) in children is frequently severe and treatment-refractory. While medical therapy is well standardized, little is known regarding factors that contribute to surgical indications. Our aim was to identify factors associated with progression to colectomy in a large cohort of pediatric UC patients.

Methods

We conducted a retrospective cohort study using the Pediatric Health Information System database. We identified all patients under age 18 discharged between January 1, 2004 and September 30, 2011 with a primary diagnosis of UC. Primary outcome was odds of total colectomy.

Results

Of 8,688 patients, 240 (2.8 %) underwent colectomy. Compared with non-operative patients, a greater proportion of colectomy patients received advanced therapies during admission, including corticosteroids (84.2 vs. 71.3 %) and biological therapy (25.4 vs. 13.6 %). Odds of colectomy were increased with malnutrition (OR 1.86), anemia (OR 2.17), electrolyte imbalance (OR 2.31), and Clostridium difficile infection (OR 1.69). TPN requirement also independently predicted colectomy (OR 3.86). Each successive UC admission significantly increased the odds of colectomy (OR 1.08).

Conclusion

These data identify factors associated with progression to colectomy in children hospitalized with UC. Our findings help to identify factors that should be incorporated into future studies aiming to reduce the variability in surgical treatment of childhood UC.     

Isolated hypoganglionosis: results of a nationwide survey in Japan

Purpose

This study analyzes how isolated hypoganglionosis (IH) is diagnosed and treated in Japanese pediatric surgical departments.

Methods

A second questionnaire was sent to 90 pediatric surgical departments that took part in an initial survey on variant Hirschsprung’s disease.

Results

Of 109 cases registered as having a certain diagnosis of IH, we targeted 90 patients and excluded the remaining 19. Symptom onset was neonatal ileus with a male:female ratio of 34:56. In most cases, results from radio-contrast enemas and suction rectal biopsies were normal. Anorectal manometry demonstrated that there was no relaxation in 73 % of the 37 patients examined. Furthermore, out of 55 patients who were examined intraoperatively, IH was diagnosed or suspected in 29 cases. Another 12 cases were eventually diagnosed with IH by other institutions using paraffin-embedded specimens. Stomas were initially created in 89 out of 90 cases and the mortality rate of the group that initially received jejunostomies was significantly lower than that of the ileostomy group (p < 0.05).

Conclusion

Preoperative diagnosis of IH is often difficult and intraoperative biopsies were only able to provide a correct diagnosis in 52 % of cases. More accurate diagnosis and the initial creation of jejunostomies rather than ileostomies may assist in improving patient management and outcomes.     

Association of Hirschsprung’s disease and anorectal malformation: a systematic review

Background

The association of Hirschsprung’s disease (HD) and anorectal malformation (ARM) is rare. The exact incidence of this association is not known but HD coexisting with ARM has been reported in 2.3 to 3.4 % of ARM cases. Most of the reported cases in the literature have been single case reports. The aim of this systematic review was to determine the incidence of HD associated with ARM and its relationship to other syndromes.

Methods

A systematic review of the literature was performed for the keywords “association of Hirschsprung’s disease and anorectal malformation”, “aganglionosis and anorectal malformation” as well as “congenital megacolon and anorectal malformation”. Resulting publications were reviewed for epidemiology, operative treatment and morbidity. Reference lists were screened for additional cases.

Results

A total of 38 articles reported 90 cases of HD coexisting with ARM from 1952 to 2013. Twenty eight articles reported 40 single case reports of this association. Ten articles reported 50 cases of HD in case series of 2,465 ARM patients, resulting in an incidence of 2 % of this association. Gender was reported in 63 cases, with 30 males (48 %) and 33 females (52 %). Associated syndromes were reported in 23 patients: Currarino syndrome in 11, Down syndrome in 8, Cat eye syndrome in 3 and Pallister-Hall syndrome in one case. Extent of aganglionosis was reported in 49 cases and included classical rectosigmoid disease in 36, long segment aganglionosis in 5, total colonic aganglionosis in 7 and total intestinal aganglionosis in one patient. In 35 % of the patients stoma was created in the aganglionotic region and failed to work. There was a median delay of 8 months for the diagnosis of HD from initial diagnosis of ARM. Various surgical techniques were employed for the pull-through operation for HD.

Conclusion

The review confirms that the recognition of HD is often delayed because of the initial diagnosis of ARM and the fact that the dysfunctional colostomy is usually proximal to the affected aganglionotic bowel. There is a high incidence of associated syndromes when HD coexists with ARM.     

Impact of cow’s milk allergy on enterocolitis associated with Hirschsprung’s disease

Purpose

To investigate the impact of cow’s milk allergy (CMA) on infants with Hirschsprung’s disease (HD).

Methods

Twenty-four patients, who developed gastrointestinal symptoms before the age of 60 days and underwent surgery for HD in the period between January 2003 and December 2012, were enrolled in this study. They were divided into two groups based on CMA-related findings: stimulation index of lymphocyte stimulation test >300 % and the presence of eosinophilic infiltration in the resected colon. Ten patients were determined specimen as not having CMA (Group A), because they did not satisfy any of the criteria. The remaining 14 were determined as having possible CMA (Group B), because they satisfied either or both findings. Patient background characteristics, pre- and postoperative clinical history, and laboratory data were compared between Groups A and B.

Results

Pre- and postoperative enterocolitis did not occur in Group A patients. Postoperative enterocolitis was more frequent in Group B than in Group A (p = 0.04). Other clinical and laboratory data did not show significant difference between the two groups.

Conclusion

CMA is a possible risk factor for postoperative enterocolitis in patients with HD.     

Inflammatory bowel disease in patients with Hirschsprung’s disease: a systematic review and meta-analysis

Aim and objectives

Hirschsprung-associated enterocolitis (HAEC) continues to be an important cause of morbidity in patients with Hirschsprung’s disease (HSCR). HAEC can occur at any time during the course of the disease. The reported incidence of HAEC before surgery ranges from 6 to 50%, and after surgery, it ranges from 2 to 35%. HAEC and inflammatory bowel disease (IBD) have similar clinical presentation including diarrhea, hematochezia, and abdominal pain. In recent years, isolated cases of IBD have been reported in patients who had surgical treatment for HSCR. The exact pathogenesis of HAEC or IBD is not known. However, both conditions are characterized by an abnormal intestinal mucosal barrier function, which may be a common pathway. The purpose of this meta-analysis was to determine the clinical presentation and outcome in patients with HSCR who developed IBD after pull-through operation.

Materials and methods

A systematic literature search for relevant articles was performed in four databases using the combinations of the following terms “inflammatory bowel disease”, “Crohn/Crohn’s disease”, “ulcerative colitis”, and “Hirschsprung disease/Hirschsprung’s disease” for studies published between 1990 and 2017. The relevant cohorts of HSCR associated with IBD were systematically searched for clinical presentation and outcomes.

Results

14 studies met defined inclusion criteria, reporting a total of 66 patients who had HSCR associated with IBD. Mean age at first operation for HSCR was 5.8 months, mean age at diagnosis of IBD was 7.7 years, and the majority of patients were male (73%). The extent of aganglionosis was total colonic aganglionosis in 41% of patients, long segment in 45%, and rectosigmoid in 14%. The majority of patients underwent a Duhamel procedure (84%) for HSCR. The distribution of IBD was Crohn’s disease in 72.3% of patients, ulcerative colitis in 16.9%, and others in 10.8%. Eight articles (47 patients) reported about HAEC, and 22 patients (47%) had experienced HAEC after surgery for HSCR.

Conclusion

Male patients with extensive colonic aganglionosis who continue to suffer from postoperative HAEC after a Duhamel procedure are more susceptible to develop IBD. Recognition of IBD may be important in the long-term follow-up of HSCR patients who have had postoperative HAEC.

     

Laparoscopic approach for children with inflammatory bowel diseases

Background

Minimally invasive surgery is being increasingly applied to inflammatory bowel diseases (IBDs). Few pediatric series from selected research have been described to date. This study describes a unicentric experience of laparoscopic treatment of children with IBDs.

Materials and methods

All consecutive patients with IBDs between February 2006 and February 2010 who underwent laparoscopic treatment were included. We reviewed notes and recorded demographic data, indications, perioperative management, surgical details, length of surgery, complications, postoperative management, length of hospitalization and functional outcome.

Results

We performed 25 procedures on 16 patients (12 ulcerative colitis, 3 Crohn??s disease, and 1 indeterminate colitis). Median age was 12?years. A total of 50% patients underwent elective surgery; 11 underwent staged laparoscopic subtotal colectomy (LSTC) followed by J-pouch ileorectal anastomosis (JPIRA). Three patients underwent straight LSTC?+?JPIRA. All procedures included protective ileostomy. Length of surgery ranged between 120 and 380?min depending on the procedure (LSTC?±?JPIRA). No conversion was required. Length of hospitalization ranged between 3 and 18?days. We observed six complications (24%) mainly represented by adhesions that were effectively treated laparoscopically. Ten patients were restored (ileostomy closure) and were assessed for continence that turned out to be good in 80%.

Conclusions

Laparoscopy proved to be feasible, safe and effective for the treatment of IBD in children. Although we observed a relatively low incidence of complications, stoma site adhesions still remain the major issue, which can be effectively dealt with laparoscopically. Functional outcome as well as cosmesis is satisfactory. As results are encouraging, at present we prefer laparoscopy for the surgical treatment of IBD in pediatric patients.     

Use of anoctamin 1 (ANO1) to evaluate interstitial cells of Cajal in Hirschsprung’s disease

Purpose

Interstitial cells of Cajal (ICCs) are pacemaker cells involved in facilitating neurotransmission and the generation of slow electrical waves necessary for colonic peristalsis. Their distribution has been found to be abnormal in the aganglionic and ganglionic colon in Hirschsprung’s disease (HSCR) using c-kit-labelling. Anoctamin-1 (ANO1) is a Ca²⁺-activated Cl^? channel thought to be specifically expressed on ICCs. Unlike c-kit, it plays a key role in ICC pacemaker activity. We aimed to investigate the utility of ANO1 in evaluating the colonic ICC network in HSCR.

Materials and methods

We collected full-length pull-through specimens from children with HSCR (n = 10). Control colon specimens were collected at colostomy closure in children with anorectal malformation (n = 6). The distribution of ANO1 and c-kit expression was evaluated using immunofluorescence and confocal microscopy. ANO1 expression was quantified using Western blot analysis.

Results

ANO1 was not expressed on 23 % of c-kit immuno-positive cells in the circular muscle; however, 100 % of ANO1-positive ICCs were c-kit positive. The distribution of ANO1-positive ICCs was sparse in aganglionic colon, with a modest reduction in ICCs seen in the ganglionic colon in HSCR compared to controls (p = 0.044). ANO1 protein expression was reduced in aganglionic colon but similar in ganglionic colon relative to controls.

Conclusions

ANO1 is preferential to c-kit in evaluating the ICC network in HSCR due to its specificity and functional importance. Abnormal distribution of ANO1-positive ICCs in the ganglionic colon in HSCR may contribute to persistent bowel symptoms in some patients after pull-through surgery.

     

A variant pattern of Calretinin immunohistochemistry on rectal suction-biopsies is fully specific of short-segment Hirschsprung’s disease

Background/purpose

Calretinin immunohistochemistry is now widely used to diagnose Hirschsprung’s disease (HD), since loss of calretinin expression within the mucosa and muscularis mucosae of rectal suction-biopsy is pathognomonic of HD. However, a stippled staining may be observed within hypertrophic nerves in the submucosae in some HD patients. The aim of the study was to test the hypothesis that such findings may announce the beginning of the transitional zone.

Methods

We retrieved 44 consecutive patients (10 girls and 34 boys; median age 6.5 days), diagnosed with aganglionosis on rectal suction biopsies, followed by surgery. According to calretinin immunohistochemistry performed on all paraffin-embedded rectal biopsies, we defined two HD groups: P? showing an absence of any staining within mucosa, muscularis mucosae and submucosa et P+ showing an absence of staining within the mucosa and muscularis mucosae, but a positivity of some submucosal hypertrophic nerves. These data were correlated to the length of total pathological segment (aganglionic and transitional zones) obtained from the original surgery reports.

Results

18/44 patients (40.9 %) belonged to the P+ group and 26/44 (59 %) patients were within the P? group. In the P+ group, the maximal length of the aganglionic zone was 9 cm [mean 4 (1–9)] and the total pathological zone never exceeded 14 cm [mean 8 (3.8–14)]. In the P? group, the maximal length of aganglionic zone was 55.5 cm [mean 11.3 (2.5; 55.5)] and the total pathological zone extended to 59.5 cm [mean 17.75 (4.5; 59.5)]. Aganglionic segment was significantly shorter in the P+ group (p < 0.0001).

Conclusion

Staining of some hypertrophic nerves in the submucosa in suction rectalbiopsy of HD patients using calretinin immunohistochemistry is only encountered in short-segment aganglionosis with a pathological zone always restricted to rectal and sigmoid colon. This information could be crucial for the surgeons in the decision to choose a transanal procedure.