Search

Background: Total colonic aganglionosis, as a rare variant of Hirschsprung Disease, still poses challenges to surgeons in terms of diagnosis and management. The optimal preparation for pullthrough is crucial for reconstructive surgery. This study aims to explore our surgical pathway for children with total colonic aganglionosis (TCA) and to describe the prehabilitation necessary to prepare for successful reconstructive pullthrough surgery. Methods: A prospective review of children with TCA receiving an abdominal surgical intervention between 1/22 and 4/24. The cohort included children receiving mapping +/− primary ileoanal pullthrough. An analysis of preoperative, perioperative, and postoperative data, and a short-term follow-up were performed. Results: A total of 18 children with TCA and no prior pullthrough received an abdominal intervention during the 29-month study period, and 5/18 (27.8%) were female. The children had a median of 4 (range 2–7) prior external surgeries; all had a stoma; 6 (33%) children received parental nutrition; 12 children underwent a mapping of the ganglia distribution and bowel length at a median age of 11 months (range 3–54), and in 10 of them, we relocated the stoma. There was a mean involvement of 15 (5–93) cm small bowel aganglionosis, with the remaining mean ganglionic small bowel having a length of 178 cm (110–254). A total of 11 children underwent straight primary ileoanal pullthrough of the stoma site at a median age of 16.7 months (10–133). Conclusions: The timely diagnosis of TCA still challenges the care team, and most children have a rough journey involving several surgeries until their diagnosis is established. The ensure bowel function with an adequate working stoma is the key to enabling enteral nutrition and growth, which are the baseline requirements to undertake a successful pullthrough procedure and restore continuity. Careful perioperative bowel management and parents’ active involvement supports children with Hirschsprung Disease achieving the best possible quality of life.

Background: Constipation and outlet obstruction may persist after successful pull-through in Hirschsprung Disease (HD). The radiographic assessment of the faecal load is widely used but exposes the child to radiation. This study aims to evaluate whether the transrectal diameter (TRD) assessed with ultrasound correlates with symptoms of faecal load and whether the TRD normalises when symptoms disappear. Method: Children with HD after pullthrough and functional constipation presenting to our colorectal clinic between 4/23 and 4/24 were assessed for symptoms of constipation, smearing and outlet obstruction, as well as healthy controls. Ultrasound measurement of the TRD was conducted. Bowel management was initiated according to our institutional pathway using Peristeen© irrigation after an orthograde disimpaction regime. Results: A total of 193 children underwent TRD assessment. Of 60 children with HD, 26 (43.3%) presented with obstructive symptoms, and 34 (56.7%) were asymptomatic. In asymptomatic patients with HD, the mean TRD of 2.26 cm (SD 0.61) was significantly (p < 0.001) lower than in HD with symptoms, with a mean TRD of 3.35 cm (SD 1.03). Individuals without colorectal pathology had a mean TRD of 2.04 cm (SD 0.37), and children with functional constipation and symptoms showed a mean TRD of 4.36 cm (SD 1.32). The mean TRD after symptom resolution was 2.37 cm. Conclusions: Children with HD without obstructive symptoms have a TRD < 3 cm, as do controls. The transrectal diameter allows the clinician to sonographically assess the faecal load in children with HD at the bedside without radiation. The TRD is useful for monitoring a bowel management program in children with HD.

Recent advancements in pediatric surgery have embraced telehealth (TH) modalities, transitioning from traditional in-person consultations to virtual care. This shift has broadened access to healthcare, potentially enhancing affordability, patient and caregiver satisfaction, and clinical outcomes. In pediatric colorectal surgery, telehealth has been effectively utilized to support Bowel Management Programs (BMPs) for children suffering from constipation and fecal incontinence. A systematic review was conducted to assess the effectiveness of virtual BMPs, analyzing studies from January 2010 to December 2023, sourced from MEDLINE (via PubMed), Embase, and the Cochrane Library, with five studies included. Remote BMPs, implemented through video or telephone consultations, reported satisfaction rates exceeding 75% among families, indicating a strong preference for virtual interactions over traditional visits. Significant findings from the studies include improvements in Vancouver and Baylor scores, reductions in the duration of multidisciplinary consultations, enhancements in pediatric quality of life and Cleveland scores, and decreased frequency of laxative treatments. The implementation of TH has facilitated patient-led care, enabling timely adjustments in treatment and efficient distribution of medical supplies. The findings suggest that virtual BMPs are a viable and effective alternative to conventional approaches, yielding high caregiver satisfaction and superior clinical outcomes while promoting patient independence.

(1) Background: Bowel management contributes throughout the pathway of care for children with Hirschsprung. Preoperative bowel management prepares the child and family for the pull-through surgery. Perioperative bowel management supports early recovery and tailored bowel management in the follow-up supports the achievement of social continence. (2) Methods: We conducted a cross-sectional assessment of our institutional bowel management program to illustrate the pre-, peri- and postoperative bowel management strategies. (3) Results: A total of 31 children underwent primary pull-through, 23 without a stoma and 8 with a stoma, at a median age of 9 months. All children without a stoma were prepared for surgery by using rectal irrigations. Children with a stoma were prepared for surgery with a transfer of stoma effluent. Transanal irrigation supported early recovery. (4) Conclusions: Bowel management is a key pillar of the management of children with Hirschsprung disease. Incorporating bowel management in the pathway of care facilitates primary pull-through and supports perioperative recovery.

Background: Patients with spinal abnormalities often struggle with fecal and/or urinary incontinence (up to 87 and 92%, respectively) and require a collaborative approach to bowel management in conjunction. Methods: To define existing approaches and propose state-of-the-art bowel management, a literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published July 2013 and July 2023. Results: Patients with spinal anomalies have impaired innervation of the rectum and anal canal, decreasing the success rate from laxatives and rectal enemas. Thus, transanal irrigations and antegrade flushes are widely utilized in this group of patients. Based on spinal MRI, the potential for bowel control in these children depends on age, type, and lesion level. On referral for bowel management, a contrast study is performed to assess colonic motility and evacuation of stool, followed by a series of abdominal X-rays to define colonic emptying and adjust the regimen. The options for management include laxatives, rectal enemas, transanal irrigations, antegrade flushes, and the creation of a stoma. Approximately 22–71% of patients achieve social continence dependent on the type and level of the lesion. Conclusion: Patients with spinal anomalies require a thorough assessment for continence potential and stool burden prior to initiation of bowel management. The optimal treatment option is defined according to the patient’s age, anatomy, and mobility. The likelihood of independent bowel regimen administration should be discussed with the patients and their caregivers.

After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients with HD before age five because of the nonrelaxing sphincters that they learn to overcome with increased age. Children with a patulous anus due to iatrogenic damage of the anal sphincters are offered sphincter reconstruction. Hypermotility is managed with antidiarrheals and small-volume enemas. Family education is crucial for the early detection of HAEC and for performing at-home rectal irrigations.

Background: Constipation and outlet obstruction may persist after successful pull-through in Hirschsprung Disease (HD). The radiographic assessment of the faecal load is widely used but exposes the child to radiation. This study aims to evaluate whether the transrectal diameter (TRD) assessed with ultrasound correlates with symptoms of faecal load and whether the TRD normalises when symptoms disappear., Method: Children with HD after pullthrough and functional constipation presenting to our colorectal clinic between 4/23 and 4/24 were assessed for symptoms of constipation, smearing and outlet obstruction, as well as healthy controls. Ultrasound measurement of the TRD was conducted. Bowel management was initiated according to our institutional pathway using Peristeen© irrigation after an orthograde disimpaction regime., Results: A total of 193 children underwent TRD assessment. Of 60 children with HD, 26 (43.3%) presented with obstructive symptoms, and 34 (56.7%) were asymptomatic. In asymptomatic patients with HD, the mean TRD of 2.26 cm (SD 0.61) was significantly ( p < 0.001) lower than in HD with symptoms, with a mean TRD of 3.35 cm (SD 1.03). Individuals without colorectal pathology had a mean TRD of 2.04 cm (SD 0.37), and children with functional constipation and symptoms showed a mean TRD of 4.36 cm (SD 1.32). The mean TRD after symptom resolution was 2.37 cm., Conclusions: Children with HD without obstructive symptoms have a TRD < 3 cm, as do controls. The transrectal diameter allows the clinician to sonographically assess the faecal load in children with HD at the bedside without radiation. The TRD is useful for monitoring a bowel management program in children with HD.

Background: Total colonic aganglionosis, as a rare variant of Hirschsprung Disease, still poses challenges to surgeons in terms of diagnosis and management. The optimal preparation for pullthrough is crucial for reconstructive surgery. This study aims to explore our surgical pathway for children with total colonic aganglionosis (TCA) and to describe the prehabilitation necessary to prepare for successful reconstructive pullthrough surgery. Methods: A prospective review of children with TCA receiving an abdominal surgical intervention between 1/22 and 4/24. The cohort included children receiving mapping +/- primary ileoanal pullthrough. An analysis of preoperative, perioperative, and postoperative data, and a short-term follow-up were performed. Results: A total of 18 children with TCA and no prior pullthrough received an abdominal intervention during the 29-month study period, and 5/18 (27.8%) were female. The children had a median of 4 (range 2-7) prior external surgeries; all had a stoma; 6 (33%) children received parental nutrition; 12 children underwent a mapping of the ganglia distribution and bowel length at a median age of 11 months (range 3-54), and in 10 of them, we relocated the stoma. There was a mean involvement of 15 (5-93) cm small bowel aganglionosis, with the remaining mean ganglionic small bowel having a length of 178 cm (110-254). A total of 11 children underwent straight primary ileoanal pullthrough of the stoma site at a median age of 16.7 months (10-133). Conclusions: The timely diagnosis of TCA still challenges the care team, and most children have a rough journey involving several surgeries until their diagnosis is established. The ensure bowel function with an adequate working stoma is the key to enabling enteral nutrition and growth, which are the baseline requirements to undertake a successful pullthrough procedure and restore continuity. Careful perioperative bowel management and parents' active involvement supports children with Hirschsprung Disease achieving the best possible quality of life.

Background: Patients with anorectal malformation (ARM) need long-term follow-up, in order to evaluate fecal continence; the main predictors of longer-term success are the type of ARM, associated anomalies and sacral integrity. Three-Dimensional High Resolution Anorectal Manometry (3D-HRAM) gives detailed information on pressure on the anal complex profile. Our objective was to analyze anal sphincter activity in ARM patients with 3D-HRAM establishing the correlation between manometric and clinical data. Methods: Forty ARM patients were submitted to 3D-HRAM: manometric, anatomical and clinical scores were correlated with each other and with the bowel management response (BM). Results: A positive correlation between all scores and types of ARM was found: in high ARM and in patients with spinal anomalies (regardless to ARM type) lower scores were reported and even after BM they did not achieve good continence. Conclusions: 3D-HRAM gives detailed data on the functional activity of the anal sphincter complex. Our study revealed a correlation between manometric parameters and clinical outcomes, confirming spinal malformations and ARM type as the most important prognostic risk factors for a bad outcome. Specific sphincteric defects can also be explored with manometry, allowing for tailored bowel management strategies.

Background: Functional constipation (FC) affects up to 32% of the pediatric population, and some of these patients are referred to pediatric surgery units to manage their constipation and/or fecal incontinence. The aim of the current paper is to report the recent updates on the evaluation and management of children with FC as a part of a manuscript series on bowel management in patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and FC. Methods: A literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published within the last 5–10 years. Results: The first step of management of children with FC is to exclude Hirschsprung disease with a contrast study, examination under anesthesia, anorectal manometry (AMAN). If AMAN shows absent rectoanal inhibitory reflex, a rectal biopsy is performed. Internal sphincter achalasia or high resting pressures indicate botulinum toxin injection. Medical management options include laxatives, rectal enemas, transanal irrigations, and antegrade flushes. Those who fail conservative treatment require further assessment of colonic motility and can be candidates for colonic resection. The type of resection (subtotal colonic resection vs. Deloyer’s procedure) can be guided with a balloon expulsion test. Conclusion: Most of the patients with FC referred for surgical evaluation can be managed conservatively. Further studies are required to determine an optimal strategy of surgical resection in children unresponsive to medical treatment.

Up to 79% of patients with anorectal malformations (ARMs) experience constipation and/or soiling after a primary posterior sagittal anoplasty (PSARP) and are referred to a bowel management program. We aim to report the recent updates in evaluating and managing these patients as part of the manuscript series on the current bowel management protocols for patients with colorectal diseases (ARMs, Hirschsprung disease, functional constipation, and spinal anomalies). The unique anatomic features of ARM patients, such as maldeveloped sphincter complex, impaired anal sensation, and associated spine and sacrum anomalies, indicate their bowel management plan. The evaluation includes an examination under anesthesia and a contrast study to exclude anatomic causes of poor bowel function. The potential for bowel control is discussed with the families based on the ARM index calculated from the quality of the spine and sacrum. The bowel management options include laxatives, rectal enemas, transanal irrigations, and antegrade continence enemas. In ARM patients, stool softeners should be avoided as they can worsen soiling.

A bowel management program (BMP) to treat fecal incontinence and severe constipation is utilized for patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and functional constipation, decreasing the rate of emergency department visits, and hospital admissions. This review is part of a manuscript series and focuses on updates in the use of antegrade flushes for bowel management, as well as organizational aspects, collaborative approach, telemedicine, the importance of family education, and one-year outcomes of the bowel management program. Implementation of a multidisciplinary program involving physicians, nurses, advanced practice providers, coordinators, psychologists, and social workers leads to rapid center growth and enhances surgical referrals. Education of the families is crucial for postoperative outcomes, prevention, and early detection of complications, especially Hirschsprung-associated enterocolitis. Telemedicine can be proposed to patients with a defined anatomy and is associated with high parent satisfaction and decreased patient stress in comparison to in-person visits. The BMP has proved to be effective in all groups of colorectal patients at a 1- and 2-year follow-up with social continence achieved in 70–72% and 78% of patients, respectively, and an improvement in the patients’ quality of life. A transitional care to adult program is essential to maintain the same quality of care, and continuity of care and to achieve desired long-term outcomes as the patient reaches adult age.

Recent advancements in pediatric surgery have embraced telehealth (TH) modalities, transitioning from traditional in-person consultations to virtual care. This shift has broadened access to healthcare, potentially enhancing affordability, patient and caregiver satisfaction, and clinical outcomes. In pediatric colorectal surgery, telehealth has been effectively utilized to support Bowel Management Programs (BMPs) for children suffering from constipation and fecal incontinence. A systematic review was conducted to assess the effectiveness of virtual BMPs, analyzing studies from January 2010 to December 2023, sourced from MEDLINE (via PubMed), Embase, and the Cochrane Library, with five studies included. Remote BMPs, implemented through video or telephone consultations, reported satisfaction rates exceeding 75% among families, indicating a strong preference for virtual interactions over traditional visits. Significant findings from the studies include improvements in Vancouver and Baylor scores, reductions in the duration of multidisciplinary consultations, enhancements in pediatric quality of life and Cleveland scores, and decreased frequency of laxative treatments. The implementation of TH has facilitated patient-led care, enabling timely adjustments in treatment and efficient distribution of medical supplies. The findings suggest that virtual BMPs are a viable and effective alternative to conventional approaches, yielding high caregiver satisfaction and superior clinical outcomes while promoting patient independence., Competing Interests: The authors declare no conflicts of interest.

(1) Background: Bowel management contributes throughout the pathway of care for children with Hirschsprung. Preoperative bowel management prepares the child and family for the pull-through surgery. Perioperative bowel management supports early recovery and tailored bowel management in the follow-up supports the achievement of social continence. (2) Methods: We conducted a cross-sectional assessment of our institutional bowel management program to illustrate the pre-, peri- and postoperative bowel management strategies. (3) Results: A total of 31 children underwent primary pull-through, 23 without a stoma and 8 with a stoma, at a median age of 9 months. All children without a stoma were prepared for surgery by using rectal irrigations. Children with a stoma were prepared for surgery with a transfer of stoma effluent. Transanal irrigation supported early recovery. (4) Conclusions: Bowel management is a key pillar of the management of children with Hirschsprung disease. Incorporating bowel management in the pathway of care facilitates primary pull-through and supports perioperative recovery.

Background: The primary aim was to describe patient-reported morbidity from neurogenic bladder and bowel dysfunction in a cohort of children with spina bifida. The secondary aim was to describe the overall surgical burden in these children. Methods: Children with meningocele or myelomeningocele, born between 2000–2016, and followed by a tertiary spina bifida center were evaluated in a cross-sectional cohort study using data from charts and a prospective national follow-up program. Results: In the group of 62 patients, clean intermittent catheterization (CIC) was used by 47 (76%) of the patients, and anticholinergic treatment was used by 36 (58%). More than one third of the patients reported inadequate results with daily urinary leakage. Laxatives and enema were used regularly by 45 (73%) and 39 (63%) patients, respectively. Inadequate results were reported by seven (11%) patients. One or more urogenital or gastrointestinal operations had been performed in 26 (42%) patients, with a total of 109 procedures overall. Conclusions: Despite substantial bowel and bladder management, a significant portion of children suffered from inadequate results concerning bladder and bowel control. Many surgeries were performed in a defined group of the children. Prospective, long-term studies can evaluate if more aggressive medical and/or surgical management could increase bowel and bladder control.

Aim of the study: To assess the efficacy of a novel technique (echo-assisted intersphincteric autologous microfragmented adipose tissue injection, also called “anal-lipofilling”) in the management of non-responsive fecal incontinence in children born with anorectal malformations (ARMs). Methods: Following ethical committee approval (CHPED-MAR-18-02), anal-lipofilling was proposed to patients with fecal incontinence not responsive to medications or bowel management (bowel enema and/or transanal irrigation automatic systems), then a prospective study was conducted. Anal-lipofilling consisted of three phases: lipoaspiration from the abdominal wall, processing of the lipoaspirate with a Lipogems system and intersphincteric injection of the processed fat tissue via endosonographic assistance. A questionnaire based on Krickenbeck’s scale (KS) was administered to the patients to evaluate the clinical outcome. Main Results: Four male patients (three recto-urethral fistula, and one recto-perineal fistula) underwent the anal-lipofilling procedure at a mean age of 13.0 ± 4.2 yrs. There were no complications during or after the procedure. From an initial assessment of the patients there was an improvement in the bowel function at a median follow up of 6 months, with better scores at KS (100% Soiling grade three pre-treatment vs. 75% grade one post-treatment). Conclusions: Even if our Study is preliminary, echo-assisted anal-lipofilling could be considered as a feasible and safe alternative technique in the management of the fecal incontinence in non-responding ARMs patients. More studies are still necessary to support the validity of the implant of autologous adipose tissue in the anal sphincter as a therapy for fecal incontinence in children born with ARMs.

Background: Patients with spinal abnormalities often struggle with fecal and/or urinary incontinence (up to 87 and 92%, respectively) and require a collaborative approach to bowel management in conjunction., Methods: To define existing approaches and propose state-of-the-art bowel management, a literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published July 2013 and July 2023., Results: Patients with spinal anomalies have impaired innervation of the rectum and anal canal, decreasing the success rate from laxatives and rectal enemas. Thus, transanal irrigations and antegrade flushes are widely utilized in this group of patients. Based on spinal MRI, the potential for bowel control in these children depends on age, type, and lesion level. On referral for bowel management, a contrast study is performed to assess colonic motility and evacuation of stool, followed by a series of abdominal X-rays to define colonic emptying and adjust the regimen. The options for management include laxatives, rectal enemas, transanal irrigations, antegrade flushes, and the creation of a stoma. Approximately 22-71% of patients achieve social continence dependent on the type and level of the lesion., Conclusion: Patients with spinal anomalies require a thorough assessment for continence potential and stool burden prior to initiation of bowel management. The optimal treatment option is defined according to the patient's age, anatomy, and mobility. The likelihood of independent bowel regimen administration should be discussed with the patients and their caregivers.

After an initial pull-though, patients with Hirschsprung disease (HD) can present with obstructive symptoms, Hirschsprung-associated enterocolitis (HAEC), failure to thrive, or fecal soiling. This current review focuses on algorithms for evaluation and treatment in children with HD as a part of a manuscript series on updates in bowel management. In constipated patients, anatomic causes of obstruction should be excluded. Once anatomy is confirmed to be normal, laxatives, fiber, osmotic laxatives, or mechanical management can be utilized. Botulinum toxin injections are performed in all patients with HD before age five because of the nonrelaxing sphincters that they learn to overcome with increased age. Children with a patulous anus due to iatrogenic damage of the anal sphincters are offered sphincter reconstruction. Hypermotility is managed with antidiarrheals and small-volume enemas. Family education is crucial for the early detection of HAEC and for performing at-home rectal irrigations.

Background: Functional constipation (FC) affects up to 32% of the pediatric population, and some of these patients are referred to pediatric surgery units to manage their constipation and/or fecal incontinence. The aim of the current paper is to report the recent updates on the evaluation and management of children with FC as a part of a manuscript series on bowel management in patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and FC., Methods: A literature search was performed using Medline/PubMed, Google Scholar, Cochrane, and EMBASE databases and focusing on the manuscripts published within the last 5-10 years., Results: The first step of management of children with FC is to exclude Hirschsprung disease with a contrast study, examination under anesthesia, anorectal manometry (AMAN). If AMAN shows absent rectoanal inhibitory reflex, a rectal biopsy is performed. Internal sphincter achalasia or high resting pressures indicate botulinum toxin injection. Medical management options include laxatives, rectal enemas, transanal irrigations, and antegrade flushes. Those who fail conservative treatment require further assessment of colonic motility and can be candidates for colonic resection. The type of resection (subtotal colonic resection vs. Deloyer's procedure) can be guided with a balloon expulsion test., Conclusion: Most of the patients with FC referred for surgical evaluation can be managed conservatively. Further studies are required to determine an optimal strategy of surgical resection in children unresponsive to medical treatment.

Background: Patients with anorectal malformation (ARM) need long-term follow-up, in order to evaluate fecal continence; the main predictors of longer-term success are the type of ARM, associated anomalies and sacral integrity. Three-Dimensional High Resolution Anorectal Manometry (3D-HRAM) gives detailed information on pressure on the anal complex profile. Our objective was to analyze anal sphincter activity in ARM patients with 3D-HRAM establishing the correlation between manometric and clinical data., Methods: Forty ARM patients were submitted to 3D-HRAM: manometric, anatomical and clinical scores were correlated with each other and with the bowel management response (BM)., Results: A positive correlation between all scores and types of ARM was found: in high ARM and in patients with spinal anomalies (regardless to ARM type) lower scores were reported and even after BM they did not achieve good continence., Conclusions: 3D-HRAM gives detailed data on the functional activity of the anal sphincter complex. Our study revealed a correlation between manometric parameters and clinical outcomes, confirming spinal malformations and ARM type as the most important prognostic risk factors for a bad outcome. Specific sphincteric defects can also be explored with manometry, allowing for tailored bowel management strategies.

Up to 79% of patients with anorectal malformations (ARMs) experience constipation and/or soiling after a primary posterior sagittal anoplasty (PSARP) and are referred to a bowel management program. We aim to report the recent updates in evaluating and managing these patients as part of the manuscript series on the current bowel management protocols for patients with colorectal diseases (ARMs, Hirschsprung disease, functional constipation, and spinal anomalies). The unique anatomic features of ARM patients, such as maldeveloped sphincter complex, impaired anal sensation, and associated spine and sacrum anomalies, indicate their bowel management plan. The evaluation includes an examination under anesthesia and a contrast study to exclude anatomic causes of poor bowel function. The potential for bowel control is discussed with the families based on the ARM index calculated from the quality of the spine and sacrum. The bowel management options include laxatives, rectal enemas, transanal irrigations, and antegrade continence enemas. In ARM patients, stool softeners should be avoided as they can worsen soiling.

A bowel management program (BMP) to treat fecal incontinence and severe constipation is utilized for patients with anorectal malformations, Hirschsprung disease, spinal anomalies, and functional constipation, decreasing the rate of emergency department visits, and hospital admissions. This review is part of a manuscript series and focuses on updates in the use of antegrade flushes for bowel management, as well as organizational aspects, collaborative approach, telemedicine, the importance of family education, and one-year outcomes of the bowel management program. Implementation of a multidisciplinary program involving physicians, nurses, advanced practice providers, coordinators, psychologists, and social workers leads to rapid center growth and enhances surgical referrals. Education of the families is crucial for postoperative outcomes, prevention, and early detection of complications, especially Hirschsprung-associated enterocolitis. Telemedicine can be proposed to patients with a defined anatomy and is associated with high parent satisfaction and decreased patient stress in comparison to in-person visits. The BMP has proved to be effective in all groups of colorectal patients at a 1- and 2-year follow-up with social continence achieved in 70-72% and 78% of patients, respectively, and an improvement in the patients' quality of life. A transitional care to adult program is essential to maintain the same quality of care, and continuity of care and to achieve desired long-term outcomes as the patient reaches adult age.

Background: The primary aim was to describe patient-reported morbidity from neurogenic bladder and bowel dysfunction in a cohort of children with spina bifida. The secondary aim was to describe the overall surgical burden in these children., Methods: Children with meningocele or myelomeningocele, born between 2000-2016, and followed by a tertiary spina bifida center were evaluated in a cross-sectional cohort study using data from charts and a prospective national follow-up program., Results: In the group of 62 patients, clean intermittent catheterization (CIC) was used by 47 (76%) of the patients, and anticholinergic treatment was used by 36 (58%). More than one third of the patients reported inadequate results with daily urinary leakage. Laxatives and enema were used regularly by 45 (73%) and 39 (63%) patients, respectively. Inadequate results were reported by seven (11%) patients. One or more urogenital or gastrointestinal operations had been performed in 26 (42%) patients, with a total of 109 procedures overall., Conclusions: Despite substantial bowel and bladder management, a significant portion of children suffered from inadequate results concerning bladder and bowel control. Many surgeries were performed in a defined group of the children. Prospective, long-term studies can evaluate if more aggressive medical and/or surgical management could increase bowel and bladder control.

Aim of the Study: To assess the efficacy of a novel technique (echo-assisted intersphincteric autologous microfragmented adipose tissue injection, also called "anal-lipofilling") in the management of non-responsive fecal incontinence in children born with anorectal malformations (ARMs)., Methods: Following ethical committee approval (CHPED-MAR-18-02), anal-lipofilling was proposed to patients with fecal incontinence not responsive to medications or bowel management (bowel enema and/or transanal irrigation automatic systems), then a prospective study was conducted. Anal-lipofilling consisted of three phases: lipoaspiration from the abdominal wall, processing of the lipoaspirate with a Lipogems system and intersphincteric injection of the processed fat tissue via endosonographic assistance. A questionnaire based on Krickenbeck's scale (KS) was administered to the patients to evaluate the clinical outcome., Main Results: Four male patients (three recto-urethral fistula, and one recto-perineal fistula) underwent the anal-lipofilling procedure at a mean age of 13.0 ± 4.2 yrs. There were no complications during or after the procedure. From an initial assessment of the patients there was an improvement in the bowel function at a median follow up of 6 months, with better scores at KS (100% Soiling grade three pre-treatment vs. 75% grade one post-treatment)., Conclusions: Even if our Study is preliminary, echo-assisted anal-lipofilling could be considered as a feasible and safe alternative technique in the management of the fecal incontinence in non-responding ARMs patients. More studies are still necessary to support the validity of the implant of autologous adipose tissue in the anal sphincter as a therapy for fecal incontinence in children born with ARMs.