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Journal of Pediatric Surgery Jun 2024The literature on transitional care in anorectal malformation (ARM) and Hirschsprung's disease (HD) is diverse and heterogeneous. There is a lack of standards and...
BACKGROUND
The literature on transitional care in anorectal malformation (ARM) and Hirschsprung's disease (HD) is diverse and heterogeneous. There is a lack of standards and guidelines specific to transitional care in these conditions. We aim to establish and systematically categorize challenges and solutions related to colorectal transition care.
METHODS
Systematic review of qualitative studies from MEDLINE, EMBASE, PubMed and Scopus databases (2008-2022) was conducted to identify the challenges and solutions of healthcare transition specific to ARM and HD. Thematic analyses are reported with reference to patient, healthcare provider and healthcare system.
RESULTS
Sixteen studies from 234 unique articles were included. Fourteen themes related to challenges and solutions, each, are identified. Most challenges identified are patient related. The key challenges pertain to: (1) patient's lack of understanding of their disorder, resulting in over-reliance on the pediatric surgical team and reluctance towards transitioning to adult services; (2) a lack of education and awareness among adult colorectal surgeons in caring for pediatric colorectal conditions and inadequate communication between pediatric and adult teams; and (3) a lack of structured transition program and joint-clinic to meet the needs of the transitioning patients. The key solutions are: (1) fostering young adult patient's autonomy and independence; (2) conducting joint pediatric-adult transition clinics; and (3) ensuring a structured and coordinated transition program is available using a standardized guideline.
CONCLUSION
A comprehensive framework related to barriers and solutions for pediatric colorectal transition is established to help benchmark care quality of transitional care services.
LEVEL OF EVIDENCE
IV.
TYPE OF STUDY
Systematic review without meta-analysis.
Topics: Humans; Hirschsprung Disease; Anorectal Malformations; Transition to Adult Care
PubMed: 37996349
DOI: 10.1016/j.jpedsurg.2023.10.066 -
Birth Defects Research. Part C, Embryo... Dec 2014Congenital anorectal malformations (ARMs) are one of the most frequently observed birth defects of the digestive system. However, their etiology remains elusive.... (Review)
Review
Congenital anorectal malformations (ARMs) are one of the most frequently observed birth defects of the digestive system. However, their etiology remains elusive. Therefore, we aim to summarize and critically appraise all existing literature on the genetic and nongenetic etiology of nonsyndromic ARM and to conclude with unifying hypotheses and directions for future research. A structured literature search on English language human studies was conducted in PubMed and Embase up to October 1, 2013, resulting in 112 included articles. Research on the identification of genes underlying nonsyndromic ARM is remarkably scarce. Most studies were focused on screening of candidate genes for mutations or single-nucleotide polymorphisms, which did not yield any substantial evidence. Nongenetic factors fairly consistently found to be associated with ARM are assisted reproductive techniques, multiple pregnancy, preterm delivery, low birth weight, maternal overweight or obesity, and preexisting diabetes. This review provides indications for the involvement of both genes and nongenetic risk factors in the etiology of ARM. In future studies, large cohorts of patients with ARM from national and international collaborations are needed to acquire new hypotheses and knowledge through hypothesis-generating approaches. Challenges for future studies may also lie in the investigation of gene-gene and gene-environment interactions.
Topics: Anal Canal; Anorectal Malformations; Anus, Imperforate; Female; Humans; Pregnancy; Rectum
PubMed: 25546370
DOI: 10.1002/bdrc.21068 -
Pediatric Surgery International Jan 2015The laparoscopically-assisted anorectal pull-through (LAARP) for recto-bladderneck and recto-prostatic anorectal malformations (RB/RP-ARMs) is believed to improve... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The laparoscopically-assisted anorectal pull-through (LAARP) for recto-bladderneck and recto-prostatic anorectal malformations (RB/RP-ARMs) is believed to improve patient outcomes. We performed a systematic review of the effect of LAARP on postoperative mucosal prolapse and defecation dysfunction.
METHODS
A comprehensive search of MEDLINE, EMBASE, CENTRAL, and grey literature was performed (2000-2014). Full-text screening, data abstraction and quality appraisal were conducted in duplicate. Included studies reported a primary diagnosis of RB/RP-ARM and compared LAARP versus open repair (OPEN).
RESULTS
From 3681 retrieved articles, 7 studies enrolling 187 patients were analyzed. One was a randomized control trial, 6 were retrospective observational studies, and all were single-centre. The majority were of poor-moderate quality (MINORS scores: mean 16.42 (SD 2.225) out of 24). Mucosal prolapse was not significantly different after LAARP versus OPEN (p = 0.18). Defecation outcomes were inconsistently reported but were no different between LAARP and OPEN for either children >3 years old (p = 0.84), or all ages combined (p = 0.11).
CONCLUSION
We found no significant difference in rates of mucosal prolapse or defecation scores for LAARP compared to OPEN for children with RB/RP-ARMs. However, studies are small and of poor-moderate quality and results are heterogeneous. Comprehensive, standardized, reliable reporting is necessary to guide practice and inform postoperative guidelines.
LEVEL OF EVIDENCE
1c.
Topics: Abnormalities, Multiple; Anal Canal; Anorectal Malformations; Anus, Imperforate; Digestive System Surgical Procedures; Female; Humans; Infant, Newborn; Laparoscopy; Male; Prostatic Diseases; Rectum; Urinary Bladder Fistula
PubMed: 25316437
DOI: 10.1007/s00383-014-3626-3 -
Journal of Pediatric Surgery May 2017The ideal colostomy type for patients with anorectal malformations (ARM) is undetermined. We performed a systematic review and meta-analysis of short-term complications... (Meta-Analysis)
Meta-Analysis Review
BACKGROUND
The ideal colostomy type for patients with anorectal malformations (ARM) is undetermined. We performed a systematic review and meta-analysis of short-term complications comparing loop and divided colostomies.
METHODS
After review registration (PROSPERO: CRD42016036481), multiple databases were searched for comparative studies without language or date restrictions. Gray literature was sought. Complications investigated included stomal prolapse/hernia/retraction, wound infections, and urinary tract infections (UTIs). Two reviewers independently assessed study eligibility and the quality of included studies. Meta-analysis of selected complications was performed using Revman 5.3, with p<0.05 considered significant.
RESULTS
Twenty-six studies were included, and four were multi-institutional. Reporting standards were highly variable. Studies scored between 6 and 9 of possible nine stars on the NOS. Overall, 3866 neonates with ARM were incorporated, in which 2241 loop colostomies and 1994 divided colostomies were reported. Of 10 studies reporting short-term complications, the overall rate was 27%. Meta-analysis demonstrated no significant difference in the incidence of UTIs, (OR: 2.55 [0.76, 8.58], p=0.12), while loop colostomies had a significantly higher prolapse rate (See figure). No publication bias was noted.
CONCLUSIONS
A colostomy for patients with an ARM is a source of considerable morbidity. Divided colostomies reduce the risk of subsequent prolapse and may represent the preferred approach.
LEVEL OF EVIDENCE
3A.
Topics: Anorectal Malformations; Colostomy; Humans; Models, Statistical; Postoperative Complications; Treatment Outcome
PubMed: 28259380
DOI: 10.1016/j.jpedsurg.2017.01.044 -
World Journal of Pediatric Surgery 2022No systematic review and meta-analysis to date has examined multiple child and parent-reported social and physical quality of life (QoL) in pediatric populations... (Review)
Review
BACKGROUND
No systematic review and meta-analysis to date has examined multiple child and parent-reported social and physical quality of life (QoL) in pediatric populations affected by Hirschsprung's disease (HD) and anorectal malformations (ARM). The objective of this systematic review is to quantitatively summarize the parent-reported and child-reported psychosocial and physical functioning scores of such children.
METHODS
Records were sourced from the CENTRAL, EMBASE, and MEDLINE databases. Studies that reported child and parent reported QoL in children with HD and ARM, regardless of surgery intervention, versus children without HD and ARM, were included. The primary outcome was the psychosocial functioning scores, and the secondary outcomes were the presence of postoperative constipation, postoperative obstruction symptoms, fecal incontinence, and enterocolitis. A random effects meta-analysis was used.
RESULTS
Twenty-three studies were included in the systematic review, with 11 studies included in the meta-analysis. Totally, 1678 total pediatric patients with HD and ARM underwent surgery vs 392 healthy controls. Pooled parent-reported standardized mean (SM) scores showed better social functioning after surgery (SM 91.79, 95% CI (80.3 to 103.3), I=0). The pooled standardized mean difference (SMD) showed evidence for parent-reported incontinence but not for constipation in children with HD and ARM after surgery that had a lower mean QoL score compared with the normal population (SMD -1.24 (-1.79 to -0.69), I=76% and SMD -0.45, 95% CI (-1.12 to 0.21), I=75%). The pooled prevalence of child-reported constipation was 22% (95% CI (16% to 28%), I=0%). The pooled prevalence of parent-reported postoperative obstruction symptoms was 61% (95% CI (41% to 81%), I=41%).
CONCLUSION
The results demonstrate better social functioning after surgery, lower QoL scores for incontinence versus controls, and remaining constipation and postoperative obstruction symptoms after surgery in children with HD and ARM.
PubMed: 36474732
DOI: 10.1136/wjps-2022-000447 -
Diseases of the Colon and Rectum Mar 2018Anorectal malformations are one of the most common congenital intestinal anomalies affecting newborns. Despite advances in neonatal care and surgical techniques, many... (Review)
Review
BACKGROUND
Anorectal malformations are one of the most common congenital intestinal anomalies affecting newborns. Despite advances in neonatal care and surgical techniques, many patients with a history of anorectal malformations are affected by long-term challenges involving bowel and bladder dysfunction, sexual dysfunction, and psychosocial issues. These outcomes or challenges are additionally exacerbated by the lack of a structured transition of care from the pediatric to the adult setting.
OBJECTIVE
The purpose of this review is to describe the long-term outcomes affecting patients with a history of anorectal malformations, review the current literature on transition of care, and make recommendations for developing a standardized program for transitioning care for a select group of colorectal surgical patients.
DATA SOURCES
An extensive PubMed review of articles in English was performed to evaluate current best practices for chronic illnesses of childhood with residual symptoms or need for medical care into adulthood.
STUDY SELECTION
Meta-Analysis of Observational Studies in Epidemiology group guidelines were followed.
MAIN OUTCOME MEASURES
The primary outcome for this review was the existence of transitional services for patients with a history of anorectal malformations and evaluations of long-term outcomes affecting patients with a history of anorectal malformations.
RESULTS
Systematic review revealed improved results in transition programs as determined by patient follow-up, medication adherence, and patient and family satisfaction through the use of multidisciplinary teams. Standardized tools for assessing all aspects of patient outcomes and quality of life are essential for describing the burden of disease affecting a transitioning population.
LIMITATIONS
This is a retrospective review of the current status of a complex and rapidly evolving field of delivery of care. More work is needed to apply uniform approaches and assess the impact, patient outcomes, and quality of life.
CONCLUSIONS
Patients who undergo childhood procedures for anorectal malformations often experience chronic symptoms related to the bowel, bladder, and reproductive organs, as well as psychosocial disturbances. This population will benefit from appropriate engagement in transitional care plans. See Video Abstract at http://links.lww.com/DCR/A543.
Topics: Anal Canal; Anorectal Malformations; Female; Humans; Male; Postoperative Complications; Rectum; Transition to Adult Care; Treatment Outcome
PubMed: 29420431
DOI: 10.1097/DCR.0000000000001033 -
Pediatric Surgery International Mar 2016Disparity still exists in the outcome of neonatal surgery between high-income countries and low-income and middle-income countries. This study reviews publications on... (Review)
Review
BACKGROUND
Disparity still exists in the outcome of neonatal surgery between high-income countries and low-income and middle-income countries. This study reviews publications on neonatal surgery in Africa over 20 years with a focus on challenges of management, trends in outcome, and potential interventions to improve outcome.
METHODS
We did a literature review by searching PubMed and African Index Medicus for original articles published in any language between January 1995 and September 2014. A data extraction sheet was used to collect information, including type of study, demographics, number of cases, outcome, challenges, and suggestions to improve outcome.
RESULTS
A total of 51 studies from 11 countries met the inclusion criteria. The 16 studies in the first 10 years (1995-2004; group A) were compared with the 35 in the last 10 years (2005-2014; group B). Nigeria (n = 32; 62.7 %), South Africa (n = 7; 13.7 %), Tanzania (n = 2; 3.9 %), and Tunisia (n = 2; 3.9 %) were the predominant sources of the publications, which were retrospective in 38 (74.5 %) studies and prospective in 13 (25.5 %) studies. The mean sample size of the studies was 95.1 (range 5-640). Overall, 4849 neonates were studied, with median age of 6 days (range 1-30 days). Common neonatal conditions reported were intestinal atresia in 28 (54.9 %) studies, abdominal wall defects in 27 (52.9 %), anorectal malformations 25 in (49.0 %), and Hirschsprung's disease, necrotising enterocolitis, and volvulus neonatorum in 23 (45.1 %) each. Mortality was lowest (<3 %) in spina bifida and facial cleft procedures, and highest (>50 %) in emergency neonatal surgeries involving bowel perforation, bowel resection, congenital diaphragmatic hernia, oesophageal atresia, and ruptured omphalocele or gastroschisis. Overall average mortality rate was higher in group A than group B (36.9 vs 29.1 %; p < 0.001), and varied between the groups for some conditions. The major documented challenges were delayed presentation and inadequate facilities in 39 (76.5 %) studies, dearth of trained support personnel in 32 (62.7 %), and absence of neonatal intensive care in 29 (56.9 %). The challenges varied from country to country but did not differ in the two groups.
CONCLUSION
Improvement has been achieved in outcomes of neonatal surgery in Africa in the past two decades, although several of the studies reviewed are retrospective and poorly designed. Cost effective adaptations for neonatal intensive care, improved health-care funding, coordinated neonatal surgical care via regional centres, and collaboration with international partners are potential interventions that could help to address the challenges and further improve outcome.
Topics: Africa; Congenital Abnormalities; Developing Countries; Humans; Infant, Newborn; Intensive Care Units, Neonatal; Outcome Assessment, Health Care; Postoperative Complications
PubMed: 26783085
DOI: 10.1007/s00383-016-3861-x -
Orphanet Journal of Rare Diseases May 2018Origin of anorectal malformations (ARM) are considered multifactorial. Several genetic and non-genetic risk factors are discussed in literature. Maternal... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Origin of anorectal malformations (ARM) are considered multifactorial. Several genetic and non-genetic risk factors are discussed in literature. Maternal periconceptional medical drug use as possible risk factor, however, has not been reviewed systematically.
METHODS
Studies published between 1977 and April 2017 were reviewed through systematic search in PubMed, ISI Web of Knowledge and Scopus databases. Furthermore, related and cross-referencing publications were reviewed. Pooled odds ratios (95% confidence intervals) were determined to quantify associations of maternal periconceptional use of folic acid, multivitamins, anti-asthma medication (separated in any anti-asthma medication, inhaled corticosteroids and salbutamol), thyroid hormone supplements, psychiatric drugs (separated in antidepressants, any selective serotonin reuptake inhibitors [SSRI], sertraline, citalopram, fluoxetine, paroxetine, hypnotics and benzodiazepine) and aspirin with ARM using meta-analyses.
RESULTS
Thirty-seven studies that reported on the association between maternal periconceptional drug intake and infants born with ARM were included in this review. These were conducted in the United States of America (n = 14), Sweden (n = 6), Hungary (n = 5), Germany (n = 3), the Netherlands (n = 3), Denmark (n = 2), France (n = 2), Norway (n = 1) and the UK (n = 1). However, only few of these studies reported on the same risk factors. Studies were heterogeneous with respect to case numbers, period ingestion of medical drug use, control selection and adjustment for covariates. Consistently increased risks were observed for any anti-asthma medication, and hypnotics and benzodiazepine, but not for folic acid, multivitamins, inhaled corticosteroids, salbutamol, thyroid hormone supplements, antidepressants, any SSRI, sertraline, citalopram, fluoxetine, paroxetine and aspirin. In meta-analyses, pooled odds ratios (95% confidence intervals) for any anti-asthma medication, and hypnotics and benzodiazepine were 1.64 (1.22-2.21), and 2.43 (1.03-5.73), respectively.
CONCLUSION
Evidence on maternal drug use before conception and during pregnancy as risk factor for ARM from epidemiological studies is still very limited. Nevertheless, the few available studies indicate any anti-asthma medication, and hypnotics and benzodiazepine to be associated with increased risks. Further, ideally large-scale multicenter and register-based studies are needed to clarify the role of maternal drug intake for the development of ARM.
Topics: Anorectal Malformations; Anti-Asthmatic Agents; Anus, Imperforate; Benzodiazepines; Congenital Abnormalities; Female; Humans; Hypnotics and Sedatives; Pregnancy; Risk Factors
PubMed: 29747656
DOI: 10.1186/s13023-018-0789-3 -
PloS One 2017Anorectal malformations (ARMs) are one of the commonest anomalies in neonates. Both laparoscopically assisted anorectal pull-through (LAARP) and posterior sagittal... (Comparative Study)
Comparative Study Meta-Analysis Review
Laparoscopically Assisted Anorectal Pull-Through versus Posterior Sagittal Anorectoplasty for High and Intermediate Anorectal Malformations: A Systematic Review and Meta-Analysis.
OBJECTIVE
Anorectal malformations (ARMs) are one of the commonest anomalies in neonates. Both laparoscopically assisted anorectal pull-through (LAARP) and posterior sagittal anorectoplasty (PSARP) can be used for the treatment of ARMs. The aim of this systematic review and meta-analysis is to compare these two approaches in terms of intraoperative and postoperative outcomes.
METHODS
MEDLINE, Embase, Web of Science and the Cochrane Library were searched from 2000 to August 2016. Both randomized and non-randomized studies, assessing LAARP and PSARP in pediatric patients with high/intermediate ARMs, were included. The primary outcome measures were operative time, length of hospital stay and total postoperative complications. The second outcome measures were rectal prolapse, anal stenosis, wound infection/dehiscence, anorectal manometry, Kelly's clinical score, and Krickenbeck classification. The quality of the randomized and non-randomized studies was assessed using the Cochrane Collaboration's Risk of Bias tool and Newcastle-Ottawa scale (NOS) respectively. The quality of evidence was assessed by GRADEpro.
RESULTS
From 332 retrieved articles, 1, 1, and 8 of randomized control, prospective and retrospective studies, respectively, met the inclusion criteria. The randomized clinical trial was judged to be of low risk of bias, and the nine cohort studies were of moderate to high quality. 191 and 169 pediatric participants had undergone LAARP and PSARP, respectively. Shorter hospital stays, less wound infection/dehiscence, higher anal canal resting pressure, and a lower incidence of grade 2 or 3 constipation were obtained after LAARP compared with PSARP group values. Besides, the LAARP group had marginally less total postoperative complications. However, the result of operative time was inconclusive; meanwhile, there was no significant difference in rectal prolapse, anal stenosis, anorectal manometry, Kelly's clinical score and Krickenbeck classification.
CONCLUSION
For pediatric patients with high/intermediate anorectal malformations, LAARP is a better option compared with PSARP. However, the quality of evidence was very low to moderate.
Topics: Anal Canal; Anorectal Malformations; Humans; Infant, Newborn; Laparoscopy; Length of Stay; Postoperative Complications; Rectum; Treatment Outcome
PubMed: 28099464
DOI: 10.1371/journal.pone.0170421 -
Gastroenterology Report Aug 2016Heterotopic gastric mucosa (HGM) is the most reported epithelial heterotopia, but it is very rare in the rectum and anus. (Review)
Review
BACKGROUND
Heterotopic gastric mucosa (HGM) is the most reported epithelial heterotopia, but it is very rare in the rectum and anus.
METHODS
The first case of an asymptomatic adult male with a large nonpolypoid HGM in the low rectum underwent complete resection by endoscopic submucosal dissection (ESD) is reported. The systematic review was based on a comprehensive search of MEDLINE, EMBASE and Google Scholar. Studies on humans were identified with the term 'heterotopic gastric mucosa in the rectum and /or anus.'
RESULTS
The search identified 79 citations, and 72 cases were evaluated comprising the present report. Congenital malformations were observed in 17 (24%) patients; rectal duplication accounted for most of the cases. The HGM was located in the anus and perineal rectum in 25 cases (41%) and low, middle and proximal pelvic rectum in 20 (33%), five (8%) and 11 cases (18%), respectively. Morphology was nonpolypoid in 37 cases (51%), polypoid in 26 cases (36%) and ulcerated in nine cases (13%). Specific anorectal symptoms were reported by 50 (69%) patients of the whole study population, and by 33 (97%) of 34 patients ≤ 18 years. Complications were observed in 23 cases (32%). The HGM was excised in 50 cases (83%). Endoscopic resection was performed in 17 cases (34%); resection was piecemeal in five of 12 lesions ≥15 mm, required argon plasma coagulation in two cases and was associated with residual tissue in two (17%). Intestinal metaplasia and an adenoma with low-grade dysplasia were described in three adults (4%).
DISCUSSION
This systematic review shows that the HGM in the rectum and anus may be associated with specific rectal symptoms and serious complications, mainly in the pediatric population, and a risk of malignancy in adults. Its complete excision should be recommended, and the ESD can overcome the technical limits of conventional endoscopic snare resection and avoid unnecessary surgery.
PubMed: 27103738
DOI: 10.1093/gastro/gow006