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Cureus Jul 2023Transanal irrigation (TAI) improves bowel function and quality of life in patients with neurogenic bowel disease compared to conservative bowel care. Its use has been...
INTRODUCTION
Transanal irrigation (TAI) improves bowel function and quality of life in patients with neurogenic bowel disease compared to conservative bowel care. Its use has been extended to a range of defecatory disorders. However, data on long-term benefits and compliance are lacking. We aim to evaluate the long-term efficacy of TAI by examining compliance and patient outcomes over a five-year period.
METHODS
This study is a five-year retrospective review of patients practising TAI in a district general hospital. Patient demographics, indications, long-term compliance, adverse events, and patient-reported Qufora bowel symptom bother scores were analysed.
RESULTS
A cohort of 18 patients had a median age of 61 (range 23-91) and were predominantly female (83.5%). The reasons for bowel dysfunction were diverse: low anterior resection syndrome, neurogenic bowel, congenital anorectal malformations, obstructed defecation, and functional disorders. Predominant symptoms were constipation (9), faecal incontinence (7), and mixed (2). Both high-volume (catheter and cone) and low-volume (mini cone) irrigation devices were used. Fourteen patients continued regular irrigation at a median follow-up of 27.7 months (range 5.1-72.3), while four had discontinued at a median follow-up of 4.8 months. The reasons for discontinuation were inadequate rectal evacuation and spontaneous improvement of symptoms. In the compliant group, there was a significant improvement in bowel symptom scores (p=0.003). No major adverse events, such as significant rectal bleeding or perforation, were noted.
CONCLUSION
In this small cohort, TAI was safe and effective for long-term use; however, a fifth of patients discontinued treatment. Further work needs to be done to identify those patients who will benefit from TAI.
PubMed: 37502470
DOI: 10.7759/cureus.42507 -
Journal of Pediatric Surgery Dec 2023Transition of care (TOC; from childhood into adulthood) of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) ensures continuation of care for...
BACKGROUND
Transition of care (TOC; from childhood into adulthood) of patients with anorectal malformations (ARM) and Hirschsprung disease (HD) ensures continuation of care for these patients. The aim of this international study was to assess the current status of TOC and adult care (AC) programs for patients with ARM and HD.
METHODS
A survey was developed by members of EUPSA, ERN eUROGEN, and ERNICA, including patient representatives (ePAGs), comprising of four domains: general information, general questions about transition to adulthood, and disease-specific questions regarding TOC and AC programs. Recruitment of centres was done by the ERNs and EUPSA, using mailing lists and social media accounts. Only descriptive statistics were reported.
RESULTS
In total, 82 centres from 21 different countries entered the survey. Approximately half of them were ERN network members. Seventy-two centres (87.8%) had a self-reported area of expertise for both ARM and HD. Specific TOC programs were installed in 44% of the centres and AC programs in 31% of these centres. When comparing centres, wide variation was observed in the content of the programs.
CONCLUSION
Despite the awareness of the importance of TOC and AC programs, these programs were installed in less than 50% of the participating centres. Various transition and AC programs were applied, with considerable heterogeneity in implementation, content and responsible caregivers involved. Sharing best practice examples and taking into account local and National Health Care Programs might lead to a better continuation of care in the future.
LEVEL OF EVIDENCE
III.
Topics: Adult; Humans; Child; Transitional Care; Hirschsprung Disease; Anorectal Malformations; Surveys and Questionnaires; Colorectal Neoplasms
PubMed: 37438237
DOI: 10.1016/j.jpedsurg.2023.06.008 -
JPMA. the Journal of the Pakistan... Jun 2023Congenital perineal grove (CPG) is a rare anorectal anomaly; only 65 cases have been reported in literature. Two cases who were referred for evaluation of a lesion in... (Review)
Review
Congenital perineal grove (CPG) is a rare anorectal anomaly; only 65 cases have been reported in literature. Two cases who were referred for evaluation of a lesion in the perineum are reported here. The patients were diagnosed clinically as CPG in neonatal period and were initially managed conservatively. Surgery was required in one case as the lesion was persistent and symptomatic. A high index of suspicion is required for diagnosis of CPG to avoid parental anxiety and unnecessary diagnostic work-up and surgery. Surgery is required only in cases where the lesion persists or there is infection, pain, and ulceration.
Topics: Infant, Newborn; Humans; Female; Anorectal Malformations; Anal Canal; Perineum; Pain
PubMed: 37427639
DOI: 10.47391/JPMA.6803 -
Frontiers in Pediatrics 2023We aimed to evaluate the function of the reconstructed anal canal in postoperative anorectal malformations (ARMs) patients through three dimension (3D) high-definition...
BACKGROUND
We aimed to evaluate the function of the reconstructed anal canal in postoperative anorectal malformations (ARMs) patients through three dimension (3D) high-definition anorectal manometry.
METHODS
From January 2015 to December 2019, 3D manometry was performed as a postoperative functional assessment of patients with ARMs divided into age subgroups based on the time of manometry. Manometric parameters, such as the length of the anorectal high-pressure zone (HPZ-length), the mean resting and squeeze pressure of HPZ (HPZ-rest and HPZ-sqze), recto-anal inhibitory reflex (RAIR), and strength distribution of the anal canal, were collected and compared with age-matched controls. Their functional outcomes were analyzed with SPSS 23.0 software for statistical analysis.
RESULTS
171 manometric measurements were performed on 142 postoperative patients (3 months∼15 years). The HPZ-rest in all patients was significantly lower than in age-matched controls (< 0.05). HPZ-sqze was notably decreased in patients older than 4 years, whereas other age groups were comparable to controls (< 0.05). The proportions of asymmetric strength distribution and negative RAIR were higher in ARMs patients. The type of anorectal malformations and lower HPZ-rest were the impact factors affecting postoperative functional outcomes.
CONCLUSIONS
The majority of the ARMs patients had acceptable functional outcomes. 3D manometry can objectively assess the reconstructed anal canal function. The patients with fecal incontinence had a high proportion of extremely low HPZ-rest and HPZ-sqze, negative RAIR, and asymmetric strength distribution. The manometric details will help the clinicians explore the causes of defecation complications and guide further management.
PubMed: 37397140
DOI: 10.3389/fped.2023.1126373 -
Journal of Indian Association of... 2023Posterior sagittal is a very well accepted approach in the treatment of anorectal malformations. This approach provides good access and exposure through the perineum to...
BACKGROUND
Posterior sagittal is a very well accepted approach in the treatment of anorectal malformations. This approach provides good access and exposure through the perineum to the deep pelvic structures. It reduces risk of injury to important structures as dissection remains in midline.
AIMS AND OBJECTIVES
To access feasibility of posterior sagittal approach for non-anorectal malformation indications and to widen the spectrum.
MATERIAL AND METHODS
We present a series of 10 cases of non-anorectal malformations operated by this approach for 4 years.
RESULTS
Six patients included in the study were of Disorders of Sexual Differentiation with pseudovagina, three of Y duplication of the urethra, and one of cervical atresia. All patients had good results.
CONCLUSION
Posterior sagittal approach is feasible, safe with minimal bleeding, and no postoperative incontinence. It can safely be used for non-anorectal indications.
PubMed: 37389391
DOI: 10.4103/jiaps.jiaps_162_22 -
PloS One 2023Studies on neurodevelopmental outcomes in individuals with congenital anomalies who undergo neonatal surgery are scarce and have reported contradictory findings based on...
BACKGROUND
Studies on neurodevelopmental outcomes in individuals with congenital anomalies who undergo neonatal surgery are scarce and have reported contradictory findings based on small study groups. The congenital condition VACTERL association includes at least three malformations: vertebral anomalies, anorectal malformations, cardiac defects, tracheoesophageal fistula with or without esophageal atresia, renal anomalies and limb deformities. Most of these patients undergo surgery during their first days of life. Neurodevelopmental disorders include a broad group of disabilities involving some form of disruption to brain development. Attention deficit hyperactivity disorder (ADHD), autism spectrum disorders (ASD) and intellectual disability (ID) are diagnoses included in this group. The aim of the study was to investigate the risk of ADHD, ASD and ID in a cohort of individuals with VACTERL association.
METHOD
Data was obtained from four Swedish national health registers and analyzed using the Cox proportional hazards model. Patients born 1973-2018 in Sweden with the diagnosis of VACTERL association were included in the study. For each case five healthy controls matched for sex, gestational age at birth, birth year and birth county were obtained.
RESULTS
The study included 136 individuals with VACTERL association and 680 controls. Individuals with VACTERL had significantly higher risk of ADHD, ASD and ID than the controls; 2.25 (95% CI, 1.03-4.91), 5.15 (95% CI, 1.93-13.72) and 8.13 (95% CI, 2.66-24.87) times respectively.
CONCLUSIONS
A higher risk of ADHD, ASD and ID was found among individuals with VACTERL association compared to controls. These results are of importance to caregivers and to professionals participating in follow ups of these patients in providing early diagnosis and support, aiming to optimize the quality of life of these patients.
Topics: Infant, Newborn; Humans; Cohort Studies; Quality of Life; Limb Deformities, Congenital; Heart Defects, Congenital; Intellectual Disability
PubMed: 37384789
DOI: 10.1371/journal.pone.0288061 -
Annals of Ibadan Postgraduate Medicine Dec 2022A large proportion of patients with preoperative enterocolitis still have enterocolitis persisting even after surgery while others resolve thereafter. Some researchers...
BACKGROUND
A large proportion of patients with preoperative enterocolitis still have enterocolitis persisting even after surgery while others resolve thereafter. Some researchers have studied Calprotectin, C-reactive protein (CRP), Blood and Plasma viscosity as markers of inflammation, hence, the choice of their use. The aim of the study is to determine the sensitivity and reliability of Calprotectin, C-reactive protein (CRP), Blood and Plasma viscosity as biochemical predictors of enterocolitis in children with colorectal anomaly post-surgery at University College Hospital Ibadan.
PATIENTS AND METHODS
This is an observational analytic study of 32 patients with either Hirschsprung's disease or Anorectal malformation carried out over a year period. The demographic data of the patients, clinical condition and the preoperative and postoperative readings of the biochemical analytes were recorded in a chart. Statistical analysis were carried out using SPSS version 23 and test for statistical association done.
RESULTS
The incidence of Hirschsprung associated enterocolitis is 12.5% and for Ano rectal malformation 6.3 %. Gender difference was not statistically significant even with the observed clinical difference. Plasma viscosity and blood viscosity correlate positively with each order. C-reactive Protein and Calprotectin did not predict enterocolitis in this study and the Sensitivity of blood viscosity at T1 and T2 is as low as 66% with a Positive Predictive Value of 25 %.
CONCLUSION
The incidence of Enterocolitis associated with Hirschsprung's disease and Anorectal malformation is 19 %. Calprotectin and C-reactive protein did not predict enterocolitis in this patients. The outcome of care was satisfactory in over 90 % of the patients.
PubMed: 37384344
DOI: No ID Found -
Children (Basel, Switzerland) Jun 2023Functional constipation (FC) affects up to 32% of the pediatric population, and some of these patients are referred to pediatric surgery units to manage their... (Review)
Review
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.
PubMed: 37371309
DOI: 10.3390/children10061078 -
Children (Basel, Switzerland) Jun 2023Patients 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...
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.
PubMed: 37371268
DOI: 10.3390/children10061037 -
Children (Basel, Switzerland) May 2023Continence problems occur often in children with anorectal malformations (ARM). The aim of this study was to evaluate parental experiences with toilet facilities at...
BACKGROUND
Continence problems occur often in children with anorectal malformations (ARM). The aim of this study was to evaluate parental experiences with toilet facilities at Dutch primary schools and their experience with how schools deal with ARM children.
METHODS
This survey was developed in collaboration with the national patient advocacy group (PAG). Recruitment for participation was conducted by the PAG (email listing and social media) and one expertise center for ARM. Participants were parents of school-attending ARM children aged 3 to 12 years.
RESULTS
Sixty-one participants (31.9%) responded to the survey. The median age of the children was 7.0 years (IQR 5.0-9.0). Schools were often located in a village (63.9%) and encompassed 100-500 children (77.0%). In total, 14 parents (23.0%) experienced difficulties in finding a primary school. Experiences with the school were described as solely positive (37.7%), solely negative (9.8%), positive and negative (34.4%), and neither positive nor negative (16.4%). Regarding school toilet facilities, 65.6% of the toilets were reported clean and 78.7% were easily accessible.
CONCLUSIONS
About 25% of parents reported difficulties in enrolling their children into primary school, and 45% reported negative experiences. This highlights the need for improved guidance and the optimization of education in schools when dealing with ARM children.
PubMed: 37371156
DOI: 10.3390/children10060924