Acute diverticulitis after Duhamel–Martin procedure for total colonic Hirschsprung’s disease

A 28-year-old man with a history of total colonic Hirschsprung’s disease treated with a Duhamel–Martin procedure in infancy, presented with signs and symptoms of acute colonic diverticulitis. Flexible sigmoidoscopy demonstrated a 20 cm jejunocolonic anastomosis, consistent with his previous operation in childhood, with a large diverticulum at the proximal end of the anastomosis containing fecal concretions. The patient returned one month later after a course of antibiotics for definitive resection of the diverticulum. Intra-operative colonoscopy localized three large diverticula and resection of the involved segment was performed. The case is presented as an adult complication of total colonic Hirschsprung’s disease treated with childhood resection and reconstruction.     

Systematic review and meta-analysis of enterocolitis after one-stage transanal pull-through procedure for Hirschsprung’s disease

Purpose  

The transanal one-stage pull-through procedure (TERPT) has gained worldwide popularity over open and laparoscopic-assisted one-stage techniques in children with Hirschsprung’s disease (HD). It offers the advantages of avoiding laparotomy, laparoscopy, scars, abdominal contamination, and adhesions. However, enterocolitis associated with Hirschsprung’s disease (HAEC) still remains to be a potentially life-threatening complication after pull-through operation. The reported incidence of HAEC ranges from 4.6 to 54%. This meta-analysis was designed to evaluate postoperative incidence of HAEC following TERPT procedure.     

Long-term outcome of children after single-stage transanal endorectal pull-through for Hirschsprung’s disease

Background  

Single-stage transanal endorectal pull-through (TEPT) is a new technique for surgery of Hirschsprung’s disease (HD). TEPT can be assisted by laparoscopy (laparoscopic assisted transanal pull-through, LATP) or with non-additional procedure (total transanal endorectal pull-through, TTEP). This study was undertaken to evaluate the long-term outcome of these approaches in children with HD.     

Long-term outcome of patients with enterocolitis complicating Hirschsprung’s disease

Enterocolitis remains the most serious complication of Hirschsprung’s disease (HD). The purpose of this study was to evaluate the risk factors in the development of enterocolitis and the long-term outcome in these patients. The hospital records of 259 consecutive patients with HD during 1975–2003 were examined. The data was analysed for age at presentation, associated anomalies, level of aganglionosis, clinical features, number of episodes of enterocolitis, type of pullthrough, necessity for post-pullthrough sphincterectomy. Follow up was carried out by personal interviews and interviews over the telephone with patients/parents. Enterocolitis was diagnosed on the basis of clinical features of diarrhoea, pyrexia, abdominal distention and vomiting. Of the 259 patients with HD, 74 patients (28.5%) were found to have enterocolitis. Out of 39 patients with Down’s syndrome and HD, 19 (48%) had enterocolitis. Fifteen (20%) patients had other associated anomalies. Fifty-six patients (75.6%) were male and 18 (24.3%) were female. In 30 patients enterocolitis was the presenting feature in the neonatal period, 22 of which presented in the first 2 weeks of life. Fifty-six patients (75.6%) had rectosigmoid disease and 18 (24.3%) had long segment disease or total colonic aganglionosis. Eighteen (24.3%) had only preoperative enterocolitis and 31(41.8%) had only postoperative enterocolitis. Twenty-five (33.7%) had both pre- and post-operative enterocolitis. Twenty (27%) patients had more than 2 episodes of enterocolitis. Various pullthrough procedures were performed. Twenty-six patients (35.1%) required internal sphinctermyectomy to treat the enterocolitis. At the time of follow-up, 16 patients were lost to follow-up. Of the remaining 58 patients, 3 patients died, 2 due to enterocolitis and 1 due to sepsis. Six patients remained with a stoma. Twenty-two patients were continent and were stooling normally, but 14 of these were on laxatives for several years after pullthrough procedure. The mean age at achieving full continence was 4.95 years in the 22 patients with normal bowel function. Nineteen patients are still on laxatives and 8 patients are soiling. Eight patients continue to have multiple recurrent episodes of enterocolitis at follow-up. Down’s syndrome is an important risk factor in the development of enterocolitis. The majority of patients with enterocolitis complicating HD continue to have disturbances of bowel function many years after surgery for HD.     

Management of Hirschsprung’s disease: a comparison of Soave’s and Duhamel’s pull-through methods

The aim of this study was to compare the safety and benefits of Soaves and Duhamels pull-through procedures for the management of Hirschsprungs disease (HD). The patients consisted of 33 boys (85%) and six girls (15%), a ratio of 5.5:1. Their ages ranged from 1 day to 8 years. Rectal biopsy was performed to confirm the diagnosis of HD. Twenty-five patients (64%) underwent Soaves pull-through, and 13 patients (33%) underwent Duhamels pull-through. Twenty children (80%) out of the 25 undergoing Soaves pull-through recovered uneventfully, compared with 11 out of the 13 (84%) undergoing Duhamels pull-through. The complications following Soaves procedure included strictures in two patients (8%), enterocolitis in another two (8%), and anastomotic leakage in one (4%). Additional operations were required in two patients (8%). The complications following Duhamels procedure included stricture in one patient (7.6%) and enterocolitis in another (7.6%). An additional operation was required in one patient (7.6%). The rate of constipation was 16% after the Soaves pull-through compared with 15% after the Duhamels pull-through. There was no significant difference between the two procedures in postoperative surgical morbidity or in long-term risk of enterocolitis. In the light of present findings, both procedures appear comparable in terms of efficiency and associated complications.     

Long-term results of bowel function after treatment for Hirschsprung’s disease: a 29-year review

Although various surgical procedures have been described to treat Hirschsprung’s disease (HD), few studies have evaluated the long-term results of these children. The purpose of this study was to assess the long-term clinical outcome and bowel function of patients with HD. The hospital records of 259 consecutive patients with a confirmed histological diagnosis of HD during 1975–2003 were examined. Data was assessed for age at presentation, sex, clinical presentation, associated anomalies, level of aganglionosis, surgical procedures, complications and bowel function. Follow up was carried out by personal/telephone interviews with patients or their parents. Of the 259 patients with HD, 200 were males (77.2%) and 59 females (22.8%). Intestinal obstruction was the presenting feature in 147 patients (56.8%), intestinal perforation in 5 (1.9%), enterocolitis in 30 (11.6%) and constipation in 77 (29.7%). Thirty-nine patients (15.1%) had associated Down’s syndrome. Two hundred and nine patients (80.7%) had rectosigmoid disease, 31 (12%) had long segment disease and 19 (7.3%) had total colonic aganglionosis. Forty-three patients (16.6%) had preoperative enterocolitis. Primary colostomy was performed in 160 patients and a primary pull through in 90. Seven patients had a sphincteromyectomy for ultrashort HD. Two patients died prior to treatment. Various pull through procedures were performed in these patients. Postoperative complications included: pelvic abcess in 2, rectal stricture in 10, perianal excoriation in 7, anastomotic leak in 8, intestinal obstruction in 3, wound dehiscence in 1, stomal prolapse/stenosis in 5, rectovesical fistula in 2 and enterocolitis in 56. Five patients underwent a redo pull through and 46 required a post pull through sphincterectomy. At the time of follow-up, 27 were lost to follow-up, 9 died, 18 had permanent stomas and 4 were too young to assess bowel function. Of the remaining 194 patients, bowel function was normal in 132 (68%). Twenty patients (10.3%) had soiling and 42 (21.7%) had constipation requiring laxatives or enemas. There was no difference in bowel function in relation to type of pull through operation. Only 34% of patients with Down’s syndrome had normal continence. The majority of patients with HD continue to have disturbances of bowel function for many years before attaining normal continence.     

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.

     

The impact of Down’s syndrome on the immediate and long-term outcomes of children with Hirschsprung’s disease

Hirschsprung’s disease (HD) in Down’s syndrome (DS) patients is stated to have a worse outcome than HD alone. In our study we reviewed the immediate and long-term outcomes of these children and questioned whether DS should influence the operative management. Data were collected on all children with HD (including total colonic aganglionosis), between January 1990 and December 2000. They were divided into two groups based on the presence or absence of Trisomy 21 and compared retrospectively. In this time period we treated 173 children with HD; 17 of these had DS. Both the groups were comparable in their mean gestational age, birth weight and presentation except that the DS group had a significantly higher overall incidence of pre and/or postoperative enterocolitis. A tota1 of 164 children underwent a Swenson pull-through and 9 had a Soave’s procedure. Follow-up ranged from 1 to 10 years. Continence assessed using the Wingspread scoring system in children over the age of 4 years showed no significant difference. Although children with both HD and DS are predisposed to complications and required a more cautious management, long-term outcome in terms of continence was not significantly worse than in HD alone. Thus the co-existence of DS should not influence the decision to offer these children and their parents the choice of definitive repair.     

Clinical experience with a new modified transanal endorectal pull-through for Hirschsprung’s disease

Single-stage transanal endorectal pull-through (TEPT) procedure is now widely used for rectosigmoid Hirschsprung’s disease (HD). However, this procedure is associated with some common complications like stricture, enterocolitis, constipation, anastomotic dehiscence and perianal excoriation. The aim of this study was to evaluate the clinical outcomes of a modified TEPT operation in the management of rectosigmoid HD. 45 patients with rectosigmoid HD admitted between August, 2004 and July, 2008 were included in this study. Pre-operative barium enema was done in all of them. Patients in whom the transition zone was well delineated were included in the study. Frozen section biopsy was done to confirm the presence of ganglion cells in the pull-through bowel. All odd number patients were included in group A, where conventional TEPT procedure was performed. All even number patients and the last five patients of the series were in group B. In this group, a modified transanal endorectal procedure was done. The initial part of submucosal dissection and mobilization of aganglionic rectum was the same as in the conventional procedure. The anorectal mucosa was anastomosed end-to-side to the seromuscular coat of the ganglionic bowel. Mobilized bowel was divided at about 2 cm distal to the anastomosis. Six hitching stitches were applied from the free edge of the bowel to the perianal skin so that the anastomosis remains outside the anal verge. The distal redundant bowel was excised on eighth post-operative day. Pre-operative bowel preparation was done in all patients in group A. The bowel preparation was not mandatory in group B patients. Post-operative results of both these groups were compared and analyzed using the Mann–Whitney U test. Mean operative time in group A was 86 min (range 75–95 min) versus 90 min (range 70–100 min) in group B. Average length of the bowel resected was 20 cm (range 10–26 cm) in group A and 22 cm (12–40 cm) in group B. Post-operative feeding was started after 48 h in group A and after 6 h in group B. Average hospital stay was about 8 days in both the groups. Median follow-up was 36 months in group A (range 14–54 months) and 32 months (range 12–52 months) in group B. Anastomotic stricture or stenosis was noted in two patients in group A and none in group B (p < 0.01). Partial anastomotic dehiscence was noted in two patients in group A and none in group B (p < 0.01). Average bowel movement in group A was 6–8 per day at 1 month and 2–6 per day at 6 months. In group B, it was 8–10 at 1 month and 2–8 at 6 months. Cuff narrowing causing constipation was noted in two patients in group A and none in group B (p < 0.01). Enterocolitis following pull-through was noted in two patients in group A and three patients in group B. Four patients in group A and one patient in group B had post-operative constipation. All of them responded to bowel management program: only one of them (group A) required enema; soiling was noted in one patient each in both the groups. Modified TEPT procedure is associated with excellent clinical outcome with fewer complications, and permits early post-operative feeding. Operating time, hospital stay and post-operative bowel movement were comparable with the conventional technique.     

A systematic review and meta-analysis of Hirschsprung’s disease presenting after childhood

Background  

Hirschsprung’s disease (HD) is characterised by an absence of ganglion cells in the distal bowel, beginning at the internal sphincter and extending proximally to varying distances. It is usually diagnosed in the newborn period, with usual presentation of delayed passage of meconium and abdominal distension, with or without bilious vomiting. HD in adults is rare and is thus often undiagnosed or misdiagnosed. The purpose of this meta-analysis was to review the presentation, treatment and clinical outcome of HD presenting after childhood.     

Hirschsprung’s disease associated with Down syndrome: a meta-analysis of incidence, functional outcomes and mortality

Purpose

Down syndrome (DS) is the most frequent chromosomal abnormality associated with Hirschsprung’s disease (HD). It has often been suggested that this association results in poorer outcomes with regard to postoperative complications, continence and mortality. On the other hand, the results after surgical treatment of HD in patients with DS are reportedly similar to those in cases with HD alone. The objective of this study was to determine the incidence of DS in cohorts with HD, and to compare pre-/postoperative complications, functional outcome and mortality between cohorts with and without coexisting DS.

Methods

A systematic literature-based search for relevant cohorts was conducted using multiple online databases. The number of DS cases in HD cohorts was recorded and data on pre-/postoperative complications, functional outcome and mortality were extracted. Pooled odds ratios with 95 % confidence intervals were calculated using meta-analysis methodology.

Results

Sixty-one articles met defined inclusion criteria, comprising data from 16,497 patients with HD. The overall incidence of DS among them was 7.32 %. Vice versa, the incidence of HD in 29,418 patients with DS was 2.62 %. There were no significant differences regarding the male-to-female ratio between cohorts with and without coexisting DS (4:1 vs. 3:1 respectively; P = 0.5376). The rate of additional comorbidities was significantly higher in HD associated with DS (P < 0.0001). Recto-sigmoid HD was in both cohorts the most common type of HD (P = 0.8231). Long-segment HD was significantly more frequent in HD with coexisting DS (P = 0.0267), while total colonic aganglionosis occurred significantly more often in HD without DS (P = 0.0003). There were no significant differences in preoperative constipation/obstruction (P = 0.5967), but the rate of preoperative enterocolitis was significantly higher in HD associated with DS (P = 0.0486). Postoperative complications such as recurrent enterocolitis (P = 0.0112) and soiling (P = 0.0002) were significantly more frequent in HD with coexisting DS. Although not statistically significant, fecal incontinence (P = 0.1014) and persistent constipation (P = 0.1670) occurred more often after surgical treatment of HD with DS. The mortality rate was significantly higher in HD associated with DS (P < 0.0001).

Conclusions

The association of HD with DS is well-recognized with an incidence of 7.32 %. A large number of patients with DS continue to have persistent bowel dysfunction after surgical treatment of HD. Our data provide strong evidence that the coexistence of HD and DS is associated with higher rates of pre-/postoperative enterocolitis, poorer functional outcomes and increased mortality.     

Experience with primary laparoscopy-assisted endorectal pull-through for Hirschsprung’s disease

Abstract From October 1996 to July 2001, 61 patients received primary laparoscopy-assisted endorectal pull-through (LAERPT) for biopsy-proven Hirschsprungs disease (HD) at Mackay Memorial Hospital. The patients ages at operation ranged from 7 days to 8 years. The patients were followed up for an average of 3.8 years (range 1–5.5 years). Major complications occurred in five (8.1%) patients, of whom four had surgical complications (two instances of anastomotic leakage, one colon perforation, and one delayed formation of colovesical fistula) and one had postoperative Salmonella infection-induced colonic stricture. The five patients required a diverting colostomy and a redo-pull-through procedure. Postoperative enterocolitis developed in 13 (21%) infants. All of them had enterocolitis before the diagnosis of HD was established. The majority of the episodes of postoperative enterocolitis were mild and resolved spontaneously, but four neonates were hospitalized with significant systemic manifestations, and two of them needed a second operation to solve the problem. After primary LAERPT, stooling frequency in young infants declined rapidly from 10.5±3.2 to 4.4±1.6 bowel movements per day in the first 3 months and more slowly thereafter. Most of these infants had regular bowel movements one to two times per day 1 year after operation. Continence evaluation of the 43 patients over 3 years of age was graded as normal in 24 (56%) patients and good in 19 (44%) patients. Of the 13 patients between 1 and 3 years of age, 11 (85%) had regular normal stools, and two (15%) had occasional soiling. From the current study, the authors concluded that primary LAERPT is a safe and effective method of managing HD with excellent continence results. Considering the complications of surgery and postoperative enterocolitis, patients with long segment aganglionosis, severe enterocolitis, or prominently dilated colon are not good candidates for primary LAERPT.     

Rare association of Hirschsprung’s disease and Joubert syndrome

We present the case of an 18-month-old boy with Hirschsprung’s disease who had psychomotor retardation. His clinical and radiological findings were consistent with Joubert syndrome. The patient was the second case to show the association between Hirschsprung’s disease and Joubert syndrome in the literature. As in our case, association of these entities by chance seems to be unlikely. Genetic analysis of new Joubert syndrome and Hirschsprung’s disease patients may identify the candidate genes.     

Classification and diagnostic criteria of variants of Hirschsprung’s disease

“Variants of Hirschsprung’s disease” are conditions that clinically resemble Hirschsprung’s disease (HD), despite the presence of ganglion cells in rectal suction biopsies. The diagnosis and management of these patients can be challenging. Specific histological, immunohistochemical and electron microscopic investigations are required to characterize this heterogeneous group of functional bowel disorders. Variants of HD include intestinal neuronal dysplasia, intestinal ganglioneuromatosis, isolated hypoganglionosis, immature ganglia, absence of the argyrophil plexus, internal anal sphincter achalasia and congenital smooth muscle cell disorders such as megacystis microcolon intestinal hypoperistalsis syndrome. This review article systematically classifies variants of HD based on current diagnostic criteria with an additional focus on pathogenesis, epidemiology, clinical presentation, management and outcome.     

Is the Rehbein procedure obsolete in the treatment of Hirschsprung’s disease?

Purpose  

After 25 years of practice and positive results of the Rehbein-procedure (RB) for children with Hirschsprung Disease (HD), we changed to the less invasive transanal endorectal pull through (TERPT). The aim of this study was to compare short- and mid-term complications of these two procedures in our patients with HD.     

Prospective analysis of primary modified Georgeson’s laparoscopy-assisted endorectal pull-through for Hirschsprung’s disease: short- to mid-term results

The aim of this study was to analyze the short- to mid-term outcome of primary modified Georgeson’s laparoscopy-assisted endorectal pull-through (PMGLEPT) for Hirschsprung’s disease (HD). HD patients treated by PMGLEPT were evaluated prospectively by a single surgeon using a standard structured questionnaire to assess complications, incidence of enterocolitis, and evaluate continence (CE). CE involved scoring five parameters (frequency of motions, severity of staining/soiling, severity of perianal erosions, anal shape, and requirement for medications) on a 3-point scale (0, 1, and 2 for each parameter). Thus, scores for CE were: 10 = normal, 8–9 = good, 6–7 = fair, and 0–5 = poor. Our modifications include transanal rectal dissection starting below or on the dentate line, near total excision of the posterior rectal cuff, and intraoperative acetylcholinesterase staining to accurately identify normal colon. Patients with total colon aganglionosis or trisomy-21 were excluded, leaving 33 cases of PMGLEPT performed between 1997 and 2004. Mean operative age was 11.0 months. Follow-up ranged from 8 months to 7 years (mean 4.0 years). There were no intraoperative complications. Post-PMGLEP, bowel obstruction occurred in 1 subject who required middle colic division for pull-through (PT), and enterocolitis occurred in 3 (9.1%) of 33 patients. In 20 subjects aged over 3 years with a follow-up period of more than 12 months, final CE was normal in 5, good in 10, fair in 4, and poor in 1, despite staining/soiling being present in 12 (60%) of 20 subjects. None of the 33 had constipation. Our results suggest that PMGLEPT is safe with acceptable outcome in the short- to mid-term. However, careful long-term follow-up is mandatory as there appears to be a relatively high incidence of staining/soiling on short- to mid-term follow-up.     

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.     

Comparative review of functional outcomes post surgery for Hirschsprung’s disease utilizing the paediatric incontinence and constipation scoring system

Purpose

We aim to analyze differences in functional outcomes in children operated on for Hirschsprung’s disease (HD) using the Paediatric incontinence/constipation scoring system (PICSS) validated in a normative group.

Methods

A retrospective review of the records of all children operated on for HD between 1997 and 2010 was performed. Patients had either a Soave or transanal endorectal pull-through. Children with total colonic aganglionosis and Down’s syndrome were excluded. Utilizing the PICSS children who scored below their age-specific lower limit 95?% confidence interval PICSS scores were considered to have incomplete continence or constipation. The rates of incomplete continence and constipation were compared between groups. Significance was set at p?<?0.05.

Results

PICSS analysis could be completed in 51 (Soave 35, transanal 16). The median age at interview was 71?months (range 6–191?months). The rate of incomplete continence was 75?% (n?=?21) and 71?% (n?=?10) for the Soave and transanal groups, respectively (p?=?1.00). The constipation rate was 34?% (n?=?12) and 25?% (n?=?4) for the Soave and transanal groups, respectively (p?=?0.74). The overall rates of incomplete continence and constipation rates were 74 and 31?%, respectively, compared with 14 and 10?%, respectively, when rates were calculated by review of records.

Conclusion

The PICSS is a sensitive tool for assessing functional outcome post HD surgery. The Soave and transanal procedures have similar functional outcomes.     

Detection of intestinal bifidobacteria and lactobacilli in patients with Hirschsprung’s disease associated enterocolitis

Background  The etiology of Hirschsprung’s disease associated enterocolitis (HAEC) is unknown. Previous investigations have suggested that several factors such as dilation of proximal bowel, changes in colonic mucosal defence, and overgrowth of toxigenic bacteria may be related with it. This study was to quantify bifidobacteria and lactobacilli in the feces of Hirschsprung’s disease (HD) patients with or without enterocolitis and those of normal children. Methods  Fresh stool specimens were collected at the first three days of the admission from 30 HD patients (aged 2 weeks to 2 years) and 15 healthy age-matched non-HD patients in the morning once a day for at least three days. All of them have not been given probiotics or antibiotics at least 7 days before stool collection. Hematoxylin-eosin and acetylcholinesterase histochemical staining on rectal biopsies of patients with HD confirmed the diagnosis of HD in all 30 patients. The 30 HD patients were divided into two groups based on the clinical history of enterocolitis: the HAEC group (n=10) and HD group (n=20). Fecal bifidobacteria and lactobacilli were consecutively quantified by SYBR Green I-based real-time PCR assay. Data were analyzed using SAS v. 12.6 for Windows. All tests were twotailed, and P values <0.05 were considered statistically significant. Results  The mean levels of bifidobacteria were 7.35±0.59, 8.16±1.17, and 8.35±0.74 in the HAEC, HD and control groups, respectively. The bifidobacteria colonization levels were lower in the HAEC group than in the HD and control groups (P<0.05, P<0.001 respectively). The mean level of lactobacilli in the HAEC (5.51±0.65) and HD groups (5.87±0.78) was significantly lower than that in the control group (6.39±0.56) (P<0.05). But there was no difference in log numbers of lactobacilli between HAEC and HD groups (P>0.05). Conclusions  The scarcity of bifidobacteria and lactobacilli in HAEC patients may result in a decrease in epithelial barrier function and be a predisposing factor in the development of HAEC. This decline suggests that treatment with probiotics or prebiotics may be beneficial in these individuals. Further research will focus on whether probiotics can decrease the incidence of HAEC.     

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.