Christina Granéli

Appendicostomy in preschool children with anorectal malformation: successful early bowel management with a high frequency of minor complications.

Aim. The aim of this study is to evaluate postoperatively bowel symptoms of antegrade colon enema through appendicostomies in preschool children with anorectal malformation (ARM). Method. 164 children with ARM operated on with posterior sagittal anorectal plasty were included. The malformations were classified according to Krickenbeck classification. Seventeen children in preschool age had an appendicostomy. The bowel symptoms according to the Krickenbeck follow-up were analysed pre- and postoperatively. All complications were registered. A questionnaire on the use of the appendicostomy was answered. Results. The median age (range) at the time of the appendicostomy was 4 (1–6) years. The observation time was 5 (0.5–14) years. The main indications for appendicostomy were incontinence and noncompliance to rectal enemas. Postoperatively there was a significant decrease in soiling and constipation (P < 0.001). The total complication rate was 43% with infections (29%), stenosis (12%), and retrograde leakage (0). The median time required for giving enema in the appendicostomy was 45 minutes (range: 15–120) once a day varying from 2 times/week to 3 times/day. And: complications are less frequent than in older children. Conclusion. Appendicostomy in preschool children with ARM is a way to achieve fecal cleanness before school start. The infection rate was high, but other complications are less frequent than in older children.

A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung disease

OBJECTIVE Transanal endorectal pull-through (ERPT) is the most popular technique to treat Hirschsprung disease (HD). Still, there is limited knowledge on long-term bowel function. This cross-sectional, multicenter study assessed long-term bowel function in a large HD population and examined predictors of poor outcome. METHODS Patients older than four years or their parents filled out a validated questionnaire on bowel function. Clinical details were recorded retrospectively from medical records. RESULTS 73/200 (37%) patients reported absolutely no impaired bowel function, meaning no constipation, fecal accidents, stoma, appendicostomy or need for enemas. Seven (4%) had a stoma, and 33 (17%) used antegrade or rectal colonic enemas. Most disarrangements of fecal control and constipation were significantly less common in older age group, but abnormal defecation frequency and social problems remained unchanged. Syndromic patients (n=31) experienced frequent fecal accidents (46%) more often than nonsyndromic (14%, P<0.001). Having a syndrome (adjusted OR 5.6, 95% CI 2.1-15, P=0.001) or a complete transanal ERPT (adjusted OR 2.4, 95% CI 1.1-5.7, P=0.038) was significantly associated with poor outcome defined as having a stoma, an appendicostomy, daily fecal accidents or need of regular rectal wash outs. CONCLUSION A significant number of HD patients experience bowel problems many years after definite surgery. Fecal control was significantly better in older than younger HD patients, but some continued to have considerable bowel problems also as adults. A total transanal ERPT was associated with poorer outcome. Long-term follow-up of HD patients is warranted. Prognosis Study: Level II.

Development of Frequency of Stools over Time in Children with Hirschsprung Disease Posttransanal Endorectal One-Stage Pull-through.

BACKGROUND The transanal endorectal one-stage pull-through (TERPT) procedure in children with Hirschsprung disease (HD) is frequently used worldwide. To give the childrens families realistic expectations and to plan the medical care for the period after TERPT, the outcome is of great importance. AIM The aim of this article is to collect information on the number of stools passed daily after one-stage TERPT procedure for HD. Patients and METHODS A prospective follow-up study for collecting information on the outcome of planned TERPT from 2005 through 2012 was performed. A control group consisting of age and gender matched children was used. RESULTS The results show an initial high frequency of daily stools, median 12 stools/day (range, 3-30 stools/day), reaching an acceptable situation with median 4 stools/day (range, 0-10 stools/day) after 1 year. After 4 years, the number of stools did not differ significantly from healthy controls. CONCLUSION This study shows that it takes 4 years after TERPT before the number of stools becomes normalized. To compare the long-term outcome, it would be desirable to have uniform regular reports on the daily frequency of passed stools, incontinence, and constipation during the years after TERPT.

Outcome after Computer-Assisted (Robotic) Nissen Fundoplication in Children Measured as Pre- and Postoperative Acid Reducing and Asthma Medications Use.

PURPOSE This study aims to report the clinical outcome of computer-assisted fundoplication (CAF) in children. METHODS As our center changed policy to using computer-assisted surgery only, a prospectively studied cohort of 40 children underwent CAF, during the period from January 2006 through May 2013. The collected data include patient demographics and postoperative complications as well as medication, 24-hour pH measurements and DeMeester scores before and after surgery. RESULTS In the studied group, the median percentage of the duration of the 24-hour pH < 4 decreased postoperatively from 11 (range, 5-39) to 1% (range, 0-12) (p < 0.001); the DeMeester score decreased from 40 (range, 17-137) to 5 (range, 1-42) (p < 0.001). All 40 patients required antireflux medication before the fundoplication. This number decreased significantly to 8 (20%) after the fundoplication (p < 0.001). Before the fundoplication, 22 children (55%) were using asthma medication and 12 (30%) after the fundoplication (p = 0.04). CONCLUSIONS The CAF significantly reduced the acid reflux from the stomach to the esophagus and the use of antireflux as well as asthma medication during the median observation period of 5 years. The evidence of advantages compared with conventional laparoscopic fundoplication remain to be confirmed.

Diagnosis, Symptoms, and Outcomes of Hirschsprung’s Disease from the Perspective of Gender

Background/Aim. Hirschsprungs disease (HD) has a skewed gender distribution, with a female to male ratio of 1 : 4. This study aims to examine differences between boys and girls with HD regarding preoperative features and postoperative treatment and outcome. Method. The first part of the study was conducted as a retrospective review of all HD patients who underwent transanal endorectal pull-through (TERPT). Pre-, peri-, immediate post-, and first-year postoperative data were collected. The second part was conducted as an observational cross-sectional study by comparing bowel function scores (BFS) determined by structured interviews of patients 4 years old and older. Results. Included were 39 boys and 12 girls. Of these, 25 boys and 9 girls were older than 4 years and participated in the BFS interview. Boys had a higher frequency of hospitalizations during the first postoperative year compared to girls (n = 20 and n = 2, p < 0.05). At long-term follow-up, more boys reported abnormal frequency of defecation, 16 compared to 2 (p < 0.05). There was no difference between genders in terms of preoperative symptoms and overall bowel function later. Conclusion. Boys with HD had more hospitalizations and a higher rate of abnormal frequency of defecation than girls with HD.

Patient-reported Swedish nationwide outcomes of children and adolescents with total colonic aganglionosis.

BACKGROUND The aim of this study was to evaluate the nationwide outcome of children with total colonic aganglionosis (TCA) during the last 20years. METHODS This was an observational, cross-sectional study where all patients with TCA, including aganglionosis of 0-50cm of ileum, born in Sweden 1995-2014 were included. Data were collected from the medical records. Patients >4years old without stoma answered a questionnaire regarding bowel function (bowel function score, BFS, score 1-20), medical treatment and nutrition. RESULTS Twenty-seven children were included. Twenty-five children were reconstructed at median age of 56 (4-236) weeks. Reconstruction procedures included Swenson (6), Soave (5), mucosectomy with short muscular cuff with or without J-pouch (9), Duhamel (3) and Rehbein (2). There was no mortality. The median follow-up time was 9.5years (8months-20years). At follow-up 7 (26%) patients had an ileostomy, 4 with a syndrome. Eight patients required parenteral support, until a median age of 11 (2-24) months. Oral energy support was used by 5/27 (15%), still 5/22 (23%) were underweighted. Obstructive symptoms were reported by 7/20 (31%). All 17 patients >4years old completed the BFS questionnaire at median age of 10 (4-20) years. Median stool frequency/24h was 5 (1-30). Fecal accidents at least once per week was reported by 4 (24%), and social problems by 8 (47%). The median BFS was 15 (11-19) without any gender differences. CONCLUSION One-third of patients with TCA report obstructive symptoms, one-third need additional nutrition and one-fifth require a permanent stoma. TCA have a negative impact on social life. Subsequently, children with TCA need a careful lifelong follow-up of specialized teams.

Treatment and Patient Reported Outcome in Children with Hirschsprung Disease and Concomitant Congenital Heart Disease

Purpose. Congenital heart disease (CHD) is reported to be associated with Hirschsprung disease (HD). The aim was to evaluate any differences between children with HD with and without CHD, respectively, with regard to patient characteristics, medical care, and patient reported bowel function. Method. This is a retrospective chart study and a cross-sectional long-term follow-up of patients older than 4 years old, including all children with HD operated on with transanal endorectal pull-through (TERPT) at a tertiary center of pediatric surgery. Information about patient characteristics, diagnostics, surgery, and medical care was compiled. At long-term follow-up, bowel function was assessed by Bowel Function Score. Results. Included were 53 HD-patients, 13 with CHD and 40 without CHD. Children with CHD more commonly presented with failure to thrive; 4 (23%) compared to those without CHD (0%) (p < 0.01). In the long-term follow-up, including 32 patients (6 with CHD), constipation was more commonly reported by children with CHD 5 (83%) than by children without CHD 4 (27%) (p = 0.01). No differences were shown in the other parameters such as fecal control and incontinence. Conclusion. HD-patients with CHD more commonly presented with failure to thrive and more frequently reported constipation than HD-patients without CHD. The findings indicate that HD-patients with CHD might need special consideration in their initial care and long-term follow-up.

Clinical Examinations of the Rectoanal Inhibitory Reflex Correlated with Anography Findings, Histopathological Findings, and Clinical Outcomes

The diagnostic method for Hirschsprung’s disease (HD) involves rectal biopsy to determine the presence of histopathological findings for aganglionosis. Contrast enema (CE) and anorectal manometry help to support the indication for biopsies. Patients with HD lack a rectoanal inhibitory reflex (RAIR) that can be studied using manometry, ultrasound, or a modified contrast enema (CE), which provokes the RAIR with an injection of cold fluid. A question that arises is whether the RAIR also could be visualized with only a specific clinical examination. Objective: The purpose of the study was to test the hypothesis that the RAIR could be seen with a cold fluid injection into the rectum to identify children without HD and thus to avoid additional investigations involving a CE or rectal biopsy. Materials and method: In a prospective study all children who were suspected to have HD and who had clinical symptoms were examined using modified CEs. In children in whom the RAIR was found with a modified CE but who still had constipation, further examinations including biopsy were necessary. These children were examined under general anesthesia. Cold water was injected in the rectum to induce and register the RAIR, and a rectal biopsy was conducted. The results were correlated with the modified CE results, histopathological findings from the rectal biopsy, and clinical follow-up outcomes. Results: The clinical cold fluid examination was not comparable with the modified CE for demonstrating a RAIR in children without HD (p < 0.001) and did not correlate with the rectal biopsy (p < 0.001). Thus, the proportion of unnecessary x-ray examinations, as well as the number of rectal biopsies, could not be reduced with a clinical examination using cold water only Conclusion: The findings demonstrated that an examination using cold water was not a reliable method for evoking the RAIR..