A Computer Program for Assessing Histoanatomical Morphometrics in Ultra-High-Frequency Ultrasound Images of the Bowel Wall in Children: Development and Inter-Observer Variability.

Ultra-high-frequency ultrasound (UHFUS) has a reported potential to differentiate between aganglionic and ganglionic bowel wall, referred to as histoanatomical differences. A good correlation between histoanatomy and UHFUS of the bowel wall has been proven. In order to perform more precise and objective histoanatomical morphometrics, the main research objective of this study was to develop a computer program for the assessment and automatic calculation of the histoanatomical morphometrics of the bowel wall in UHFUS images. A computer program for UHFUS diagnostics was developed and presented. A user interface was developed in close collaboration between pediatric surgeons and biomedical engineers, to enable interaction with UHFUS images. Images from ex vivo bowel wall samples of 23 children with recto-sigmoid Hirschsprung's disease were inserted. The program calculated both thickness and amplitudes (image whiteness) within different histoanatomical bowel wall layers. Two observers assessed the images using the program and the inter-observer variability was evaluated. There was an excellent agreement between observers, with an intraclass correlation coefficient range of 0.970-0.998. Bland-Altman plots showed flat and narrow distributions. The mean differences ranged from 0.005 to 0.016 mm in thickness and 0 to 0.7 in amplitude units, corresponding to 1.1-3.6% and 0.0-0.8% from the overall mean. The computer program enables and ensures objective, accurate and time-efficient measurements of histoanatomical thicknesses and amplitudes in UHFUS images of the bowel wall. The program can potentially be used for several bowel wall conditions, accelerating research within UHFUS diagnostics.

Children with Hirschsprung's Disease Report Dietary Effects on Gastrointestinal Complaints More Frequently than Controls.

Hirschspung's disease (HD) is a congenital gastrointestinal (GI) disorder frequently accompanied by GI complaints. Despite the lack of evidence regarding whether diet affects GI symptoms, advice on dietary changes is common. The aim was to investigate self-reported dietary effects on GI symptoms, comparing children with HD with healthy children. This was an observational, cross-sectional, self-reported case-control study using the validated Diet and Bowel Function questionnaire. All children with HD aged 1-18 years were surgically treated during 2003-2021 at a national HD center, and their parents were invited to participate. Healthy children served as controls. The data were presented as median (range) and n (%). 71/85 children with HD (6 years (1-17); 76% boys) and 265/300 controls (9 years (1-18); 52% boys) participated. Dietary effects on GI symptoms were reported more frequently by children with HD than controls (55/71 [77%] vs. 137/265 [52%], p ≤ 0.001), as were dietary adjustments to improve GI symptoms (49/71 [69%] vs. 84/265 [32%], p ≤ 0.001), and social limitations due to dietary adjustments (20/48 [42%] vs. 22/121 [18%], p = 0.002). Of 90 food items, children with HD reported that more of the items induced GI symptoms compared to controls (7 (0-66) vs. 2 (0-34), p = 0.001). Diet-induced GI symptoms and dietary adjustments' impact on daily life are reported more frequently by children with HD than controls. Moreover, the number and types of food items causing GI symptoms differ. The results indicate the need for disease-specific dietary advice to improve support for families of children with HD.

Self-Reported Effects of Diet on Gastrointestinal Symptoms in Healthy Children.

OBJECTIVES: Evidence on dietary effects on gastrointestinal (GI) symptoms in healthy children is lacking. Despite this, dietary advice is still common practice in the treatment of children's GI symptoms. The aim was to investigate self-reported dietary effects on GI symptoms in healthy children. METHODS: In this observational cross-sectional study on children, a validated self-reporting questionnaire including 90 specified food items was used. Healthy children aged 1-18 years old and their parents were invited to participate. Descriptive data were presented as median (range) and n (%). RESULTS: In total, 265 of 300 children (9 years [1-18]; 52% boys) answered the questionnaire. Overall, 21 of 265 (8%) reported that diet induced GI symptoms regularly. In total, 2 (0-34) food items were reported per child as inducing GI symptoms. The most frequently reported items were beans (24%), plums (21%), and cream (14%). More children with GI symptoms (constipation, abdominal pain, troublesome gases) than with No/Seldom GI symptoms reported that diet could potentially induce GI symptoms (17/77 [22%] vs 4/188 [2%], P ≤ 0.001). Furthermore, they adjusted their diet to regulate GI symptoms (16/77 [21%] vs 8/188 [4%], P ≤ 0.001). CONCLUSIONS: Few healthy children reported that diet induced GI symptoms, and only a minority of food items were reported to induce GI symptoms. Children who had already experienced GI symptoms reported that diet impacted on GI symptoms to a greater, but still very limited, extent. Results can be used to determine accurate expectations and goals of dietary treatment of GI symptoms in children.

Diet and bowel function in children with Hirschsprung's disease: development and content validation of a patient-reported questionnaire.

BACKGROUND: Although dietary adjustments are recommended frequently for bowel symptoms, evidence of diet's impact on bowel function is lacking. The aim was to develop a patient-reported outcome instrument, for children with and without Hirschsprung's disease (HD), to explore experiences of dietary effects on bowel function. METHODS: Children with and without HD and their parents participated. Questionnaire items regarding the impact of diet on bowel function originated from focus group discussions. Specific food items, reported in the literature or in focus groups to cause bowel functional effects, were listed requesting each item's effect size and effect type. Content validity was tested within two separate semistructured interviews. A pilot test was performed. Assessing comprehension, relevance and wording clarity structurally, revisions were made accordingly. Children's bowel function was assessed through the validated Rintala Bowel Function Score. RESULTS: A total of 13 children with and without HD, median age 7 (range 2-15) years, and 18 parents participated in the validation. Each question's relevance had been ranked highly early in the validation process but most questions needed refining for improving clarity and comprehension. Wordings regarding bowel symptoms and emotions connected to food in particular were perceived to be sensitive and complex. Specifically wording regarding some bowel symptoms (gases, pain) and parental stress emotions (guilt, ambivalence) were, consistent with participants' opinions, subjected to multiple step revisions. Following the validation process, which included two semistructure interviews with different participants and then a pilot test with a third cohort, a full track overview of changes and rewording made in all steps of the validation process was presented. The final questionnaire then comprised 13 questions assessing foods' significance for bowel function, emotions, social impact and 90 specific food items' possible effects and effect sizes on bowel function. CONCLUSIONS: The Diet and Bowel Function questionnaire, enabling answering by children, was developed and the content validated qualitatively. This report presents insights into the whole validation process, declaring reasons for the selected question- and answering options, and their wordings. The Diet and Bowel Function questionnaire can be used as a survey questionnaire to enhance understanding of dietary effects on bowel function in children, and its results can be supportive in improving dietary-treatment programs.

Ultra-High Frequency Ultrasound Imaging of Bowel Wall in Hirschsprung's Disease-Correlation and Agreement Analyses of Histoanatomy.

Hirschsprung's disease (HD) is characterized by aganglionosis in the bowel wall, requiring resection. Ultra-high frequency ultrasound (UHFUS) imaging of the bowel wall has been suggested to be an instantaneous method of deciding resection length. The aim of this study was to validate UHFUS imaging of the bowel wall in children with HD by exploring the correlation and systematic differences between UHFUS and histopathology. Resected fresh bowel specimens of children 0-1 years old, operated on for rectosigmoid aganglionosis at a national HD center 2018-2021, were examined ex vivo with UHFUS center frequency 50 MHz. Aganglionosis and ganglionosis were confirmed by histopathological staining and immunohistochemistry. Histoanatomical layers of bowel wall in histopathological and UHFUS images, respectively, were outlined using MATLAB programs. Both histopathological and UHFUS images were available for 19 aganglionic and 18 ganglionic specimens. The thickness of muscularis interna correlated positively between histopathology and UHFUS in both aganglionosis (R = 0.651, p = 0.003) and ganglionosis (R = 0.534, p = 0.023). The muscularis interna was systematically thicker in histopathology than in UHFUS images in both aganglionosis (0.499 vs. 0.309 mm; p < 0.001) and ganglionosis (0.644 versus 0.556 mm; p = 0.003). Significant correlations and systematic differences between histopathological and UHFUS images support the hypothesis that UHFUS reproduces the histoanatomy of the bowel wall in HD accurately.

Systematic orientation of fresh rectal suction biopsies improves histopathological diagnostics in hirschsprung's disease - a method description and preliminary report.

BACKGROUND: Optimizing rectal suction biopsy (RSB) diagnostics in Hirschsprung's disease (HD) may shorten diagnostic time and prevent need for repeated biopsies. AIM: To explore whether systematic orientation of fresh RSB specimens increased biopsy quality, diagnostic times, diagnostic efficacy, and histopathologic workload, and to explore these outcome measures for aganglionic specimens. MATERIALS/METHODS: This was an observational case-control study conducted at a national referral center for HD on data collected from the local HD-diagnostic register. From 2019 each fresh RSB was oriented by the collector in a notch in a foam cushion, placed in a separate cassette, and sent in formalin for pathological analysis. Outcome measures of oriented RSB samples collected 2019-2021 were compared to those of non-oriented RSB samples collected 2015-2018. Staining/immunohistochemistry consisted of hematoxylin eosin, S-100 and calretinin. RESULTS: 78 children with 81 RSBs and 242 biopsy analyzes were included. The frequency of high-quality RSB specimens was higher in oriented: 40% (42/106) versus non-oriented 25% (34/136) (p = 0.018), the diagnostic turnaround time was shorter: 2 days (1-5) versus 3 days (2-8) (p = 0.015), and the number of additional sectioning/leveling/re-orientation per biopsy was lower: 7 (3-26) versus 16 (7-72) (p = 0.011). Specifically for aganglionic specimens, the frequency of high-quality biopsies was generally higher in oriented than in non-oriented RSB specimens: 47% (28/59) versus 14% (7/50) (p < 0.001); the diagnostic efficacy was higher 95% (19/20) versus 60% (9/15) (p = 0.027) and the diagnostic turnaround time shorter: 2 days (2-3) versus 3 days (2-8) (p = 0.036). CONCLUSIONS: Systematic orientation of fresh RSB specimens improves HD diagnostics. Improvement was consistent in aganglionic specimens.

Design of a Pediatric Rectal Ultrasound Probe Intended for Ultra-High Frequency Ultrasound Diagnostics.

It has been shown that ultra-high frequency (UHF) ultrasound applied to the external bowel wall can delineate the histo-anatomic layers in detail and distinguish normal bowel from aganglionosis. This would potentially reduce or lessen the need for biopsies that are currently mandatory for the diagnosis of Hirschsprung's disease. However, to our knowledge, no suitable rectal probes for such a use are on the market. The aim was to define the specifications of an UHF transrectal ultrasound probe (50 MHz center frequency) suitable for use in infants. Probe requirements according to patient anatomy, clinicians' requests, and biomedical engineering UHF prerequisites were collected within an expert group. Suitable probes on the market and in clinical use were reviewed. The requirements were transferred into the sketching of potential UHF ultrasound transrectal probes followed by their 3D prototype printing. Two prototypes were created and tested by five pediatric surgeons. The larger and straight 8 mm head and shaft probe was preferred as it facilitated stability, ease of anal insertion, and possible UHF technique including 128 piezoelectric elements in a linear array. We hereby present the procedure and considerations behind the development of a proposed new UHF transrectal pediatric probe. Such a device can open new possibilities for the diagnostics of pediatric anorectal conditions.

Histopathological dimensions differ between aganglionic and ganglionic bowel wall in children with Hirschsprung's disease.

BACKGROUND: In the validation of new imaging technology for children with Hirschsprung's disease (HSCR), basic anatomical parameters of the bowel wall must be established specifically for this patient group. AIM: To explore differences in histoanatomical layers of bowel wall, comparing ganglionic and aganglionic bowel walls, and to examine if the bowel wall thickness is linked to patient weight. METHODS: This was an observational study of bowel specimens from children weighing 0-10 kg, operated on consecutively during 2018-2020. Ganglionic and aganglionic bowel walls were measured in digitalized microscopy images from 10 sites per trans-sectional specimen and compared regarding the thickness of their histoanatomical layers. RESULTS: Bowel walls were measured in 21 children. Full bowel wall thickness did not differ between aganglionic and ganglionic bowel (2.20 vs 2.04; p = 0.802) while weight at surgery correlated positively with both ganglionic and aganglionic bowel wall thickness (r = 0.688 and 0.849, respectively), and age at surgery with ganglionic bowel wall thickness (r = 0.517). In aganglionic segments, the muscularis externa layer was thicker compared to that in ganglionosis (0.45 vs 0.31 mm, p = 0.012) whereas the muscularis interna was thinner (0.45 vs 0.62 mm, p < 0.001). A diagnostic index was identified whereby a lower ratio of muscularis interna/externa thickness followed by a thinner muscularis interna differed between aganglionic and ganglionic bowel in all specimens. CONCLUSION: Thicknesses of the bowel wall's muscle layers differ between aganglionic and ganglionic bowel walls in children with HSCR. These findings support a diagnostic index that could be validated for transfer to instant diagnostic imaging techniques. LEVEL OF EVIDENCE: Diagnostic: 3.

Ten years of paediatric robotic surgery: Lessons learned.

BACKGROUND: Costs and a low total number of cases may be obstacles to the successful implementation of a paediatric robotic surgery programme. The aim of this study was to evaluate a decade of paediatric robotic surgery and to reflect upon factors for success and to consider obstacles. MATERIALS AND METHODS: All children operated on with robotic-assisted laparoscopic surgery between 2006 and 2016 were included in a retrospective, single-institutional study in Lund, Sweden. RESULTS: A total of 152 children underwent robotic surgery during the study time with the most frequent procedures being fundoplication (n = 55) and pyeloplasty (n = 53). Procedure times decreased significantly during the study period. Overall, 18 (12%) of the operations were converted to open surgery, and seven (5%) patients required a reoperation. CONCLUSIONS: Despite a low volume of surgery, we have successfully introduced robotic paediatric surgery in our department. Our operative times and conversion rates are continuously decreasing.

Diagnostic Efficacy of Rectal Suction Biopsy with Regard to Weight in Children Investigated for Hirschsprung's Disease.

BACKGROUND/AIM: Diagnostic efficacy, defined as the percentage of rectal suction biopsy (RSB) specimens sufficient enough to determine the absence of ganglia cells in Hirschsprung's disease (HD) diagnosis, has been reported to be low, requiring repeated biopsies. The aim was to explore whether RSB diagnostic efficacy was influenced by the child's weight and to ascertain whether RSB efficacy differed between aganglionic and ganglionic tissue. MATERIALS AND METHODS: Efficacy analyses were conducted in a national HD-center's register on children 0-15 kg, examined between 2011-2019. First-time RSB diagnostic efficacy was correlated to the children's weight and final HD diagnosis. RESULTS: Among the 84 children who had first-time RSB, the overall diagnostic efficacy was 85% (71/84). The efficacy was higher among children weighing less than the identified cut-off of 9.0 kg (89% in 0-9.0 kg versus 62% in 9.01-15.0 kg, p = 0.026). Among children diagnosed with HD, 96% (26/27) weighed 0-9.0 kg. In this weight group, the diagnostic efficacy was lower in aganglionosis compared to ganglionosis (77%; 20/26 versus 96%; 43/45), p = 0.045). CONCLUSIONS: The RSB diagnostic efficacy was significantly higher in children weighing less than 9.0 kg and was less in aganglionic compared to ganglionic tissue. Therefore, weight can be useful to predict RSB diagnostic efficacy.

Sex and age differences in lower urinary tract dysfunction in healthy children.

AIM: Information about healthy children's urinary tract symptoms is scarce but would be helpful in children with congenital urinary tract conditions. The aim of this study was to develop and evaluate a Lower Urinary Tract Dysfunction (LUTD) questionnaire. METHODS: A 15-item questionnaire based on definitions by the International Children's Continence Society (ICCS) about urinary tract function, was given to children 4-15 years old with no gastrointestinal or urinary tract conditions. The study was approved ethically. RESULTS: The response rate was 82% (311/377), 50% (n = 155) were girls. Children were of the age groups 3.5-7 years (n = 136), 8-12 years (n = 127), and 13-15 years (n = 48). More girls than boys reported urinary tract infections (20% vs 3%, p < 0.001), while prevalences of incontinence and enuresis were equivalent in both sexes. In the youngest age group, enuresis was the most frequently reported symptom (11%), then daytime incontinence (10%). The older children more frequently reported previous urinary tract infections (12% and 17% in respective groups) and daytime incontinence (9% and 6%, respectively). CONCLUSION: A LUTD questionnaire is developed and evaluated within this study. Daytime urinary incontinence is the overall most common lower urinary tract symptom and girls report infections more frequently than boys.

Ultra high frequency ultrasonography to distinguish ganglionic from aganglionic bowel wall in Hirschsprung disease: A first report.

BACKGROUND/PURPOSE: In Hirschsprung disease (HD) surgery, confirming ganglionic bowel is essential. A faster diagnostic method than the current frozen biopsy is desirable. This study investigated whether aganglionic and ganglionic intestinal wall can be distinguished from each other by ultra high frequency ultrasound (UHF ultrasound). METHODS: In an HD center during 2019, intestinal walls of recto-sigmoid specimens from HD patients were examined ex vivo with a 70 MHz UHF ultrasound transducer. Data from four sites were described. Histopathologic analysis was compared to the ultrasonography outcome at each site. Each patient's specimen served as its own control. RESULTS: 11 resected recto-sigmoid specimens (median 20 cm long [range 6.5-33]) with transition zones of 5 cm (2-11 cm) were taken from children aged 22 days (13-48) weighing 3668 g (3500-5508); 44 key sites were analyzed. There was full concordance for 42/44 (95%) key sites and 10 of 11 (91%) specimens. The specimen with discordance of two key sites contained a segment of aganglionosis (3 cm) and a transition zone (1 cm): the site discordance was limited to the transition zone ends. CONCLUSIONS: This first report on UHF ultrasound in recto-sigmoid HD shows promising results in identifying aganglionosis, transition zones and ganglionic bowel. Further in vivo studies are required.

Children with Hirschsprung's Disease and Syndromes with Cognitive Dysfunction: Manifestations, Treatment, and Outcomes.

Introduction To assess differences in initial symptoms, treatments, and bowel function between children with Hirschsprung's disease (HD) with or without a cognitive dysfunction (CD). Materials and Methods The study included children with HD who underwent transanal endorectal pull-through. A retrospective chart review was performed to collect data on patient characteristics, diagnosis, and treatment. Data on bowel symptoms in children older than 4 years without a colostomy were compiled during a cross-sectional, patient-reported follow-up. Results Fifty-three children with HD were included; of these, 12 (23%) had CD. The median birth weight was lower, frequency of vomiting as the presenting symptom was lower, and time until the first contact with a pediatric surgeon was higher in children with CD than in those without (3,295 vs. 3,623 g, p = 0.013; 28 vs. 66%, p = 0.02; and 4 days vs. 1 day, p = 0.048, respectively). At follow-up, 5 (15%) of 33 children aged over 4 years had CD. More children without CD had some ability to hold back defecation and sense the urge to defecate than those with CD ( p = 0.002 and p = 0.001, respectively). Conclusion HD children who have CD present with different initial symptoms, have a delay in the first consultation with a pediatric surgeon, and experience poorer bowel function outcomes than HD children without CD. Therefore, HD children with CD should receive special attention in both clinical practice and research.

Urinary tract anomalies and urinary tract dysfunction in children with Hirschsprung disease-Is follow-up indicated?

BACKGROUND/PURPOSE: Urinary tract function in children with Hirschsprung disease (HD) is rarely considered. AIM: to evaluate the prevalence of urinary tract anomalies and dysfunction in children with HD compared to controls. METHODS: This was an observational cross sectional case-control study. Children with HD who underwent transanal endorectal pull-through technique (TERPT) from 2005 to 2017 were invited to participate. Ultrasound of the urinary tract was performed postoperatively. Children >4 years were asked to answer a urinary tract function questionnaire. Controls were age-matched healthy children. Ethical approval was obtained. RESULTS: Seventy two children with HD and TERPT were included. Ultrasound was performed in 58 children (83%) post-TERPT. Ten anomalies were diagnosed in six children (10%). Structural anomalies included abnormal kidney size (7%), renal agenesis (2%), prominent calyces (2%) and renal pelvis anomaly (25). Probable acquired anomalies included hydronephrosis (2%), hydroureter (2%) and parenchymal damage (2%). One child had a prior nephrectomy owing to a Wilms' tumor. All 37 children >4 years (27 boys and 10 girls), median aged 8 years (range 4-12), answered the questionnaire as did 284 healthy controls (144 boys and 140 girls). Boys with HD reported a higher frequency of enuresis: 65% versus 9% (p = 0.001) and urinary tract infections: 18% versus 3% (p = 0.012). Girls with HD reported enuresis more frequently (60%) than healthy girls (7%) (p = 0.001). Children with HD with constipation reported enuresis more frequently (p = 0.038). CONCLUSIONS: Urinary tract anomalies and dysfunction deserve attention in the follow-up of children with HD. We suggest screening for urinary tract anomalies and urinary tract symptoms in follow-up of children with HD. TYPE OF STUDY: Treatment study. LEVEL: III.

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

Background/Aim. Hirschsprung's 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.

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.

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.

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.

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.

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 children's 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.