Anorectal physiology and colonic motility in children with a history of tethered cord syndrome.

OBJECTIVES: The understanding of the impact of tethered cord syndrome (TCS) on the physiology of the colorectal area is limited. Our aim was to describe anorectal and colonic motility in children with TCS and compare the findings to those of children with functional constipation (FC). METHODS: We conducted a retrospective review of children with TCS who had an anorectal manometry (ARM) performed at our institution from January 2011 to September 2023. We recorded demographics, medical and surgical history, clinical symptoms, and treatment at time of ARM, ARM findings (resting pressure, push maneuver, rectal sensation, rectoanal inhibitory reflex [RAIR], and RAIR duration), and the final interpretation of colonic manometry (CM) if performed. We identified age and sex-matched control groups of children with FC. RESULTS: We included 24 children with TCS (50% female) who had ARM testing (median age at ARM 6.0 years, interquartile range 4.0-11.8 years). All children had constipation at time of ARM. Nineteen children had detethering surgery before ARM was performed. No significant differences in ARM parameters were found between children who had detethering surgery before ARM and children with FC. Among the 24 children, 14 also had a CM performed (13/14 after detethering surgery). No significant differences in colonic motility were found between children with a history of TCS and children with FC. CONCLUSIONS: Anorectal physiology and colonic motility are similar between children with a history of TCS and children with FC, suggesting that the underlying pathophysiology of defecatory disorders in children with and without history of TCS is similar.

Current practice in the care of children with functional constipation: What is the hold up?

OBJECTIVES: For children with intractable functional constipation (FC), there are no evidence-based guidelines for subsequent evaluation and treatment. Our objective was to assess the practice patterns of a large, international cohort of pediatric gastroenterologists. METHODS: We administered a survey to physicians who attended the 2nd World Congress of Pediatric Neurogastroenterology and Motility held in Columbus, Ohio (USA) in September 2023. The survey included 29 questions on diagnostic testing, nonpharmacological and pharmacological treatment, and surgical options for children with intractable FC. RESULTS: Ninety physicians from 18 countries completed the survey. For children with intractable FC, anorectal manometry was the most commonly used diagnostic test. North American responders were more likely than Europeans to use stimulant laxatives (97% vs. 77%, p = 0.032), prosecretory medications (69% vs. 8%, p < 0.001), and antegrade continence enemas (ACE; 83% vs. 46%, p = 0.009) for management. Europeans were more likely than North Americans to require colonic transit testing before surgery (85% vs. 30%, p < 0.001). We found major differences in management practices between Americans and the rest of the world, including use of prosecretory drugs (73% vs. 7%, p < 0.001), anal botulinum toxin injections (81% vs. 58%, p = 0.018), ACE (81% vs. 58% p = 0.018), diverting ileostomies (56% vs. 26%, p = 0.006), and colonic resections (42% vs. 16%, p = 0.012). No differences were found when respondents were compared by years of experience. CONCLUSIONS: Practice patterns in the evaluation and treatment of children with intractable FC differ widely among pediatric gastroenterologists from around the world. A clinical guideline regarding diagnostic testing and surgical decision-making is needed.

Anal Botulinum Toxin in Children with Hirschsprung Disease and Functional Constipation: A Prospective Cohort study.

INTRODUCTION: Anal sphincter botulinum toxin injections (BTIs) are used in the treatment of children with severe defecation disorders, including Hirschsprung disease (HD) and functional constipation (FC). Our objective was to evaluate the outcomes of BTI in these children. MATERIALS AND METHODS: We performed a prospective cohort study of children undergoing BTI from July 2018 to December 2018. We recorded perceived effect of the BTI, including effectiveness ranging from 0 (not at all effective) to 4 (extremely effective). In addition, we recorded symptoms and the Cleveland Clinic Constipation Score (CCCS). Data were collected at baseline and at 2 weeks, 2 months, and 4 months post-injection. RESULTS: Forty-two children (HD = 25, FC = 17) were included in the study (median age 4.3 years, IQR 2.4-7.2, 52% male). Twenty-two (88%) children with HD and eight (47%) children with FC had previously undergone a BTI. BTIs were perceived effective in 16 (76%) and 12 (71%) children with HD and eight (47%) and seven (47%) children with FC at 2-week and 2-month follow-up, respectively. Effectiveness was not rated differently between groups except at the 2-month follow-up, when patients with HD rated the BTI more effective compared to those with FC (median 2 [HD] vs. median 1 [FC], p = 0.022). Over the course of the study, 17/39 (44%) children reported self-limiting adverse effects such as fecal incontinence and pain at the injection site. CONCLUSION: Anal sphincter BTIs can be effective in the treatment of constipation in both HD and FC patients.

The Use of Linaclotide in Children with Functional Constipation or Irritable Bowel Syndrome: A Retrospective Chart Review.

BACKGROUND: Linaclotide is a well-tolerated and effective agent for adults with functional constipation (FC) or irritable bowel syndrome with constipation (IBS-C). However, data in children are lacking. The aim of this study is to examine the efficacy and safety of linaclotide in children. METHODS: We performed a retrospective review of children < 18 years old who started linaclotide at our institution (Nationwide Children's Hospital, Columbus, Ohio). We excluded children already using linaclotide or whom had an organic cause of constipation or abdominal pain. We recorded information on patient characteristics, medical and surgical history, symptoms, clinical response, course of treatment, and adverse events at baseline, first follow-up, and after 1 year of linaclotide use. A positive clinical response was based on the physician's global assessment of symptoms at the time of the visit as documented. RESULTS: We included 93 children treated with linaclotide for FC (n = 60) or IBS-C (n = 33); 60% were female; median age was 14.7 years (IQR 13.2-16.6). Forty-five percent of patients with FC and 42% with IBS-C had a positive clinical response at first follow-up a median of 2.5 and 2.4 months after starting linaclotide, respectively. Approximately a third of patients experienced adverse events and eventually 27% stopped using linaclotide due to adverse events. The most common adverse events were diarrhea, abdominal pain, nausea, and bloating. CONCLUSION: Nearly half of children with FC or IBS-C benefited from linaclotide, but adverse events were relatively common. Further prospective, controlled studies are needed to confirm these findings and to identify which patients are most likely to benefit from linaclotide.

Comparison of antegrade continence enema treatment and sacral nerve stimulation for children with severe functional constipation and fecal incontinence.

BACKGROUND: To compare antegrade continence enema (ACE) treatment and sacral nerve stimulation (SNS) in children with intractable functional constipation (FC) and fecal incontinence (FI). METHODS: We performed a retrospective review of children 6-18 years old with FC and FI treated with either ACE or SNS at our institution. We recorded symptoms at baseline, 6 months, 12 months, 24 months, and their most recent visit after starting treatment. We compared improvement in FI, bowel movement (BM) frequency, abdominal pain, laxative use, and complications. Patients were contacted to evaluate perceived benefit using the Glasgow Children's Benefit Inventory. KEY RESULTS: We included 23 patients treated with ACE (52% female, median age 10 years) and 19 patients treated with SNS (74% female, median age 10 years). Improvement in FI was greater with SNS than ACE at 12 months (92.9% vs 57.1%, P = .03) and 24 months (100% vs 57.1%, P = .02). Improvement in BM frequency was greater with ACE, and children were more likely to discontinue laxatives at all follow-up time points (all P < .05). Improvement in abdominal pain was greater with ACE at the most recent visit (P < .05). Rate of complications requiring surgery was similar between groups (26.3% vs 21.7%). Benefit was reported in 83.3% and 100% of ACE and SNS groups, respectively (NS). CONCLUSIONS AND INFERENCES: Although both ACE and SNS can lead to durable improvement in children with FC and FI, SNS appears more effective for FI and ACE more effective in improving BM frequency and abdominal pain and in discontinuation of laxatives.

Evaluation of a Novel Educational Tool in Adolescents With Inflammatory Bowel Disease: The NEAT Study.

OBJECTIVES: Among adolescents with inflammatory bowel disease (IBD), nonadherence rates are 50 to 88% across medications. Improving education in adults with IBD has been shown to improve coping and adherence to treatment in adults with IBD. Therapeutic patient education (TPE) has been used in patients with chronic diseases to train patients in skills to support treatment adaptation and condition management. This study tested the feasibility and preliminary efficacy of a novel TPE intervention in adolescents with IBD. METHODS: In this pilot, mixed-methods study, we evaluated the feasibility and preliminary efficacy of TPE with the IBD Pocket Guide on medication adherence, IBD knowledge, and transition readiness in adolescents ages 11 to 18 years. Medication adherence was monitored using a MedMinder Pill Dispensing system. Participants who were <90% adherent during a 4-week pre-intervention monitoring period were randomized to either a usual care group or an educational intervention (EI) group. Participants were followed for an additional 4 weeks after intervention. RESULTS: Trends were found in the EI group indicating improved medication adherence and IBD knowledge compared with the usual care group, though differences between groups did not reach statistical significance. Qualitative data showed that participants perceived that they had improved knowledge after the educational intervention. CONCLUSIONS: Therapeutic patient education may be beneficial for improving patient medication adherence and IBD knowledge. Future directions include testing the effects of the intervention with a larger sample.

Anal sphincter botulinum toxin injection in children with functional anorectal and colonic disorders: A large institutional study and review of the literature focusing on complications.

BACKGROUND AND AIM: Botulinum toxin (botox) is a commonly used treatment for functional anorectal and colonic disorders. Although generally regarded as safe, complications associated with botox injection into the anal sphincters in children with severe defecation disorders are not well described. We aimed to review our institutional experience and the existing literature to better understand the safety of this practice. METHODS: We performed a retrospective review of pediatric patients undergoing botox administration into the anal sphincter for treatment of a variety of defecation disorders between 2014 and 2018. Additionally, we performed a review of all published literature reporting complications from botox injection in this patient population. RESULTS: 881 patients ranging from 5 weeks to 19.7 years underwent a total of 1332 botox injections including our institution (332 patients/526 injections) and the reviewed series (549 patients/806 injections). Overall, complications were seen after 9 (0.7%) injections and included urinary incontinence (n = 5), pelvic muscle paresis (n = 2), perianal abscess (n = 1), pruritis ani (n = 1), and rectal prolapse (n = 1). Patient age, weight, and diagnosis were not associated with an increased rate of complication in our institutional experience. All complications were self-limited and did not require intervention. There were no episodes of systemic botulinum toxicity. CONCLUSION: Botox injection into the anal sphincters is accepted practice in children with Hirschsprung disease, severe functional constipation, and internal anal sphincter achalasia and appears to be safe from this review. The precise dosing and age at which complications are more likely to arise could not be ascertained and require further study. LEVEL OF EVIDENCE: IV TYPE OF STUDY: Retrospective cohort study.

A comparison of Malone appendicostomy and cecostomy for antegrade access as adjuncts to a bowel management program for patients with functional constipation or fecal incontinence.

BACKGROUND: Appendicostomy and cecostomy are two approaches for antegrade enema access for children with severe constipation or fecal incontinence as adjuncts to a mechanical bowel management program. Each technique is associated with a unique set of complications. The purpose of our study was to report the rates of various complications associated with antegrade enema access techniques to help guide which option a clinician offers to their patients. METHODS: We reviewed all patients in our Center who received an appendicostomy or cecostomy from 2014 to 2017 who were participants in our bowel management program. RESULTS: 204 patients underwent an antegrade access procedure (150 appendicostomies and 54 cecostomies). Skin-level leakage (3% vs. 22%) and wound infections (7% vs. 28%) occurred less frequently in patients with appendicostomy compared to cecostomy. Nineteen (13%) appendicostomies required revision for stenosis, 4 (3%) for mucosal prolapse, and 1 (1%) for leakage. The rates of stenosis (33 vs. 12%) and wound infection (13 vs. 6%) were higher in patients who received a neoappendicostomy compared to an in situ appendicostomy. Intervention was needed in 19 (35%) cecostomy patients, 15 (28%) for an inability to flush or a dislodged tube, and 5 for major complications including intraperitoneal spillage in 4 (7%) and 1 (2%) for a tube misplaced in the ileum, all occurring in patients with a percutaneously placed cecostomy. One appendicostomy (1%) patient required laparoscopic revision after the appendicostomy detached from the skin. CONCLUSION: Patients had a lower rate of minor and major complications after appendicostomy compared to cecostomy. The unique complication profile of each technique should be considered for patients needing these procedures as an adjunct to their care for constipation or fecal incontinence. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.

Surgical management of functional constipation: An intermediate report of a new approach using a laparoscopic sigmoid resection combined with malone appendicostomy.

INTRODUCTION: We previously reported our surgical technique for functional constipation for patients who have failed medical management using a novel collaborative approach with gastroenterology input, pre-operative contrast enema, colonic manometry, and laxative protocol combined with a laparoscopic colonic resection with Malone appendicostomy. Now we report our intermediate outcomes. METHODS: Patients who failed bowel management program for functional constipation were reviewed from 3/2014-2/2017. Patients with Hirschsprung disease, anorectal malformation, tethered cord, spina bifida, Trisomy 21, cerebral palsy, mitochondrial disease, or prior colon resection were excluded. RESULTS: Of 31 patients (14 females; median age 12years, follow-up 10.3months) with functional constipation and failed medical management, 26 (84%) had preoperative colonic manometry which, in addition to the contrast enema, guided laparoscopic colon resection. Ten patients (32.3%) are clean with no flushes (1 takes no laxatives, 8 are on low dose laxatives only, and 1 patient was clean on laxatives but chose to switch back to flushes). Of the 21 patients that remain on antegrade flushes, 20 (95.2%) are clean, and one patient (4.8%) continues to soil. We define clean as no soiling and no abnormal stool burden on x-ray. Laxative trials are planned for all patients on an antegrade flush regimen. CONCLUSION: Our intermediate results show that laparoscopic colon resection with Malone appendicostomy allows the majority of patients to be clean on antegrade flushes, and some to be on no or minimal laxatives. TYPE OF STUDY: Retrospective review. LEVEL OF EVIDENCE: 3.

Avaliaçäo da triangulaçäo da anastomose término-terminal de fragmento de mucosa bucal na reconstruçäo uretral: estudo experimental no cäo / Evaluation of triangulation of end-to-end anastomosis using oral mucosa fragment for urethral reconstruction: experimental study in dogs

O uso da mucosa bucal para tratar a estenose uretral tem sido considerado um excelente método alternativo para substituir o tecido uretral. OBJETIVO: Investigar os resultados da realizaçäo da triangulaçäo da anastomose término-terminal de fragmento de mucosa bucal na reconstruçäo uretral. MÉTODOS: Foram utilizados 12 cäes entre 13 e 17 Kg, sem raça definida, aparentemente sadios. Após anestesia, introduziu-se sonda na uretra dos animais que foi mantida durante todo ato operatório. Inicialmente procedeu-se a ressecçäo de um fragmento de mucosa bucal do lábio superior e imerso em soluçäo salina com cloranfenicol aquoso. A seguir procedeu-se a exposiçäo da uretra peniana e ressecou-se um segmento de dois centrímetros. Foi realizada a triangulaçäo da anastomose término-terminal do fragmento de mucosa bucal na reconstruçäo uretral. RESULTADOS: Após 60 dias observou-se a ocorrência de estenose em 25 por cento dos animais. Foram observadas fístulas uretro-cutâneas e extravasamento de contraste no local das anastomoses. CONCLUSÃO: A triangulaçäo da anastomose término-terminal de fragmento de mucosa bucal na reconstruçäo uretral näo evita a ocorrência de estenose.