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Mymensingh Medical Journal : MMJ Jan 2021Anorectal malformations (ARM) incorporate a broad spectrum of diseases, can affect both sexes, and involve the distal anus and rectum as well as the uro-genital tracts....
Anorectal malformations (ARM) incorporate a broad spectrum of diseases, can affect both sexes, and involve the distal anus and rectum as well as the uro-genital tracts. Defects range from the minor which can be treated easily with an excellent outcome, to those are complex and often associated with other anomalies are difficult to manage with poor functional prognosis. This study was done to observe the hospital incidence of Anorectal malformations, frequency of types, sex distribution and spectrum of associations with ARM. The effects of presence of associated anomalies on morbidity and mortality also observed. Detailed history, clinical examinations and relevant investigations were performed for the primary and as well as the associated anomalies. A total of 80 patients were admitted in the department of pediatric surgery in Mymensingh Medical College Hospital during the period of June 2016 to May 2017. Age of the patients was ranging from 1-180 days with the mean age of 0.49±1.002 months. Male: Female ratio was 1.6: 1. Among them 48(60%) were high and 32(40%) were low variety of ARM. In male 37(46.2%) had high and 13(13.7%) were low variety whereas in female 11(13.7%) were high and 19(23.7%) had low ARM. Associated anomalies were seen in 25(31.2%) patient -18 in males and 7 in females; 20 in high and 5 in low ARM. Associated anomalies were uro-genital 11(13.8%), cardiovascular 10(12.5%), vertebral 4(5%), limb defects 3(3.5%) and others 2(2.5%). Four patients have more than one anomaly. Anorectal malformations occur more in boys than girls. Males were more likely to have high lesions and without fistula was the common defect. Low variety ARM were found more in females with Anovestibular fistula is the commonest defect. The most common associated anomalies were recto urinary fistula (13.8%). Associations were more in high than low ARM but not significant (p>0.05). Post operative complications were more in high ARM in both sexes with associated anomalies. The effects of types and associations on morbidity and mortality were significantly different (p<0.05).
Topics: Anal Canal; Anorectal Malformations; Child; Female; Humans; Infant, Newborn; Male; Rectal Fistula; Rectum; Spine
PubMed: 33397852
DOI: No ID Found -
Indian Journal of Pediatrics Sep 2019The bowel is a challenging abdominal organ to image. The main reason is the variable location, convoluted morphology and motility. A variety of bowel disorders such as... (Review)
Review
The bowel is a challenging abdominal organ to image. The main reason is the variable location, convoluted morphology and motility. A variety of bowel disorders such as congenital, developmental, inflammatory, infectious and neoplastic lesions can affect children and most of them are either unique to this age group or have a distinct clinico-radiological appearance compared to adults. Imaging plays a very important role in characterizing these lesions and further guiding the management. This is the first part of the series on imaging of bowel disorders in children. This article will cover the imaging modalities used for the evaluation of bowel and the imaging features of congenital /developmental disorders.
Topics: Abdominal Cavity; Anorectal Malformations; Child; Diagnostic Imaging; Duodenal Diseases; Duodenal Obstruction; Esophageal Atresia; Gastrointestinal Diseases; Humans; Intestinal Atresia; Magnetic Resonance Imaging; Meconium Ileus; Pediatrics; Pyloric Stenosis, Hypertrophic; Tomography, X-Ray Computed; Tracheoesophageal Fistula; Ultrasonography
PubMed: 30767163
DOI: 10.1007/s12098-019-02877-9 -
Pediatric Surgery International Jul 2022Due to the paucity of data and controversy regarding the etiology and surgical approach for managing anorectal prolapse (ARP) after anorectoplasty, we sought to...
AIM
Due to the paucity of data and controversy regarding the etiology and surgical approach for managing anorectal prolapse (ARP) after anorectoplasty, we sought to investigate the underlying anatomic disorder and the surgical outcome in managing this challenging complication.
METHODS
We performed a retrospective study on 83 patients with ARP related to anorectal malformations (ARM). Logistic regression analyses were performed to detect the risk factors for the ARP severity. Surgical procedures were stratified according to identified anatomical abnormalities and surgical outcomes were analyzed.
RESULTS
50 patients (62.7%) had high-type ARM. The original anorectoplasty had a higher rate of ARP in laparoscopic-assisted anorectoplasty (n = 49, 59.0%) versus posterior sagittal anorectoplasty (n = 11, 13.3%). ARP was associated with rectal fat hyperplasia (67.5%), dilated muscular tunnel (79.5%), longitudinal muscle (LM) discontinuity (16.9%), rectal dilation (22.9%), mislocated anus (7.2%), and excessive mobile mesorectum (3.6%). Based on the ARP severity, the patients were divided into a severe group (Group 1, n = 38) and a moderate group (Group 2, n = 45). Binary logistic regression analysis showed that hyperplasia rectal fat (OR 4.55, 95% CI 1.16-17.84), rectal dilation (OR 4.21, 95% CI 1.05-16.94), and high-type ARM (OR 2.90, 95% CI 1.14-7.39) were independent risk factors for the development of severe ARP. Complications after stratified surgical repair included wound infection in six patients (7.2%), anal stenosis in one patient (1.2%), and ARP recurrence in two patients (2.4%). Twenty-six patients without colostomy before prolapse repair were followed up for 2 to 12 years. All the patients maintained voluntary bowel movements. Following ARP repair, there was an overall higher rate of no soiling or grade 1 soiling (88.5 vs. 65.4%), but 3 of 12 patients with grade 2 constipation were upgraded to grade 3.
CONCLUSION
Our study shows that ARM-related anorectal prolapse is associated with excessive rectum, hyperplasia of rectal fat, mobile mesorectum, loose muscular tunnel, LM discontinuity, and anal mislocation. Surgical repair with techniques stratified according to the patients' underlying risk factors is effective to prevent recurrence and improve the soiling continence.
Topics: Anal Canal; Anorectal Malformations; Humans; Hyperplasia; Infant; Plastic Surgery Procedures; Rectal Prolapse; Rectum; Retrospective Studies
PubMed: 35596086
DOI: 10.1007/s00383-022-05141-y -
Neurogastroenterology and Motility Dec 2022Children with anorectal malformations may experience constipation and fecal incontinence following repair. The contribution of altered anorectal function to these... (Review)
Review
BACKGROUND
Children with anorectal malformations may experience constipation and fecal incontinence following repair. The contribution of altered anorectal function to these persistent symptoms is relatively intuitive; however, colonic motility in this cohort is less well understood. Manometry may be used to directly assess colonic motility.
PURPOSE
The purpose of this systematic review was to synthesize the available evidence regarding post-operative colonic motility in children with anorectal malformations and evaluate the reported equipment and protocols used to perform colonic manometry in this cohort. This systematic review was conducted in compliance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). We conducted a systematic review of four databases: Embase, MEDLINE, PubMed, and the Cochrane Library (1 January 1985-22 July 2021). Studies reporting colonic manometry performed in children following anorectal malformation repair were assessed for eligibility. Data were extracted independently by two authors. Four studies were eligible for inclusion. Of the combined total cohort of 151 children, post-operative colonic manometry was conducted in 35. Insufficient reporting of medical characteristics, bowel function, and manometric outcomes restricted comparison between studies, and limited clinical applicability. No results from high-resolution colonic manometry were identified. Despite the prevalence of post-operative bowel dysfunction in children with repaired anorectal malformations, this systematic review highlighted the markedly limited evidence regarding post-operative colonic motility. This cohort may benefit from assessment with high-resolution techniques; however, future work must emphasize adherence to standardized manometry protocols, and include robust reporting of surgical characteristics, bowel function, and manometric outcomes.
Topics: Child; Humans; Anorectal Malformations; Rectum; Anal Canal; Manometry; Colon; Constipation; Fecal Incontinence
PubMed: 35699343
DOI: 10.1111/nmo.14415 -
Journal of Pediatric and Adolescent... Dec 2021Cloacal malformations are among the most complex types of anorectal malformation and are characterized by the urological, genital, and intestinal tracts opening through... (Review)
Review
Cloacal malformations are among the most complex types of anorectal malformation and are characterized by the urological, genital, and intestinal tracts opening through a single common channel in the perineum. Long-term outcome is affected by multiple factors, which include anatomical variants of the malformation itself, associated anomalies, and the surgical approach. Reconsidering these variables and their influence on "patient important" function might lead to strategies that are more outcome-driven than focused on the creation of normal anatomy. Key outcomes reflect function in each of the involved tracts and the follow-up needed should therefore not only include the classical fields of colorectal surgery and urology but also focus on items such as gynecology, sexuality, family-building, and quality of life as well as other psychological aspects. Involving patients and families in determining optimal treatment strategies and outcome measures could lead to improved outcomes for the individual patient. A strategy to support delivery of personalized care for patients with cloacal malformations by aiming to define the best functional outcomes achievable for any individual, then select the treatment pathway most likely deliver that, with the minimum morbidity and cost, would be attractive. Combining the current therapies with ongoing technological advances such as tissue expansion might be a way to achieve this.
Topics: Anal Canal; Animals; Anorectal Malformations; Child; Cloaca; Humans; Postoperative Care; Quality of Life
PubMed: 34419606
DOI: 10.1016/j.jpag.2021.08.009 -
European Journal of Radiology Apr 2020To document the imaging findings suggestive of anorectal malformation (ARMs) on prenatal US and MRI.
PURPOSE
To document the imaging findings suggestive of anorectal malformation (ARMs) on prenatal US and MRI.
METHODS
Retrospective evaluation of the screening US and prenatal MRI exams of the rectum and ano-perineal region in normal fetuses and in patients with ARMs.
RESULTS
Examples showing the normal rectal and anoperineal anatomy on prenatal US and MRI exams and the imaging findings observed in different types of confirmed ARMS.
CONCLUSIONS
Prenatal diagnosis of ARMs requires both a systematic evaluation of the fetal pelvis and perineum and an appropriate knowledge of its suggestive imaging findings.
Topics: Adult; Anal Canal; Anorectal Malformations; Female; Humans; Magnetic Resonance Imaging; Male; Perineum; Pregnancy; Prenatal Diagnosis; Rectum; Retrospective Studies
PubMed: 32078893
DOI: 10.1016/j.ejrad.2020.108859 -
Pediatric Neurosurgery 2022The reported prevalence of tethered spinal cord in patients with anorectal malformations (ARMs) ranges from 9% to 64%. Practice patterns surrounding the diagnosis and...
INTRODUCTION
The reported prevalence of tethered spinal cord in patients with anorectal malformations (ARMs) ranges from 9% to 64%. Practice patterns surrounding the diagnosis and management of tethered cord (TC) are suspected to vary, with consideration to the type of spine imaging, adjunct imaging modalities, what patients are offered surgical intervention, and how patients are followed after detethering. We sought to determine what consensus, if any, exists among pediatric neurosurgeons in the USA in terms of diagnosis and management of TC and, specifically, patients with TC and ARM.
METHODS
A survey was sent to members of the American Society of Pediatric Neurosurgeons (ASPN). Members of the ASPN received an email with a link to an anonymous REDCap survey that asked about their experience with detethering procedures, indications for surgery, diagnostic tools used, and follow-up protocols.
RESULTS
The survey was completed by 93 of the 192 ASPN members (48%). When respondents were asked about the total number of all simple filum detetherings they performed annually, 61% (N = 57) indicated they perform less than 10 for all TC patients. Ninety-three percent (N = 87) of neurosurgeons performed these procedures in patients with simple filum TC and ARM patients (TC + ARM) specifically. When asked about prophylactic detethering in those with a confirmed diagnosis of low-lying conus and with a filum fatty terminale, 59.1% (N = 55) indicated they would offer this to TC + ARM patients regardless of their age. Regarding preoperative workup for simple filum detethering, all respondents indicated they would order an MRI in both TC and TC + ARM patients, with a minority also requiring additional testing such as urodynamics, neurodevelopmental assessments, and anorectal manometry for both groups. When following patients postoperatively, almost all respondents indicated they would require clinical neurosurgical follow-up with a clinic visit (100% in all simple filum TC patients, 98.9% in fatty filum/low-lying conus TC + ARM patients), but there was wide variation in the use of other tools such as urological testing, neurodevelopmental assessment, and anorectal manometry.
DISCUSSION/CONCLUSIONS
A wide variety of diagnostic criteria and indication for procedural intervention exists for management of TC patients with and without ARM. Further studies are needed to determine outcomes. Prospective protocols need to be developed and evaluated to standardize care for this patient population and determine best practices.
Topics: Child; Humans; United States; Anorectal Malformations; Neurosurgeons; Prospective Studies; Neural Tube Defects; Spine; Cauda Equina; Magnetic Resonance Imaging; Retrospective Studies
PubMed: 36257293
DOI: 10.1159/000527389 -
Journal of Pediatric Surgery Aug 2023Little is known about fertility and pregnancy outcomes in patients with anorectal malformations (ARM), particularly those with long common channel cloaca and cloacal... (Review)
Review
BACKGROUND
Little is known about fertility and pregnancy outcomes in patients with anorectal malformations (ARM), particularly those with long common channel cloaca and cloacal exstrophy who may have impaired fertility. The purpose of this study is to describe pregnancy and offspring data from a cohort of patients with ARM.
METHODS
A retrospective review of female patients with ARM from our database, which includes patients operated on since 1980, was performed as well as a review of the literature. Demographic, operative, and self-reported fertility, obstetric, and offspring data were collected.
RESULTS
There were 37 females identified in our database who reported any pregnancy or having children. There were 59 pregnancies, 48 (81.3%) of which resulted in live birth. The most common mode of delivery was cesarean delivery. There were five patients with long channel cloaca (>3 cm) and one with cloacal exstrophy that reported 11 total pregnancies, eight of which resulted in live birth. Four cloaca patients in which the native vagina was pulled through were able to conceive spontaneously. Three patients with cloacal anomalies required in vitro fertilization to conceive; one was unsuccessful. No patients who underwent bowel partial vaginal replacement became pregnant. Women with ARM face many unique challenges in assisted reproduction, pregnancy, and delivery owing to their anatomy and associated anomalies.
CONCLUSIONS
Women with recto-perineal, recto-vestibular, and cloacas in which the native vagina was pulled through are capable of spontaneous pregnancy. Assisted reproduction, however, may be needed those with more complex anomalies and surgical repairs.
LEVEL OF EVIDENCE
IV.
Topics: Urogenital System; Anorectal Malformations; Urogenital Abnormalities; Humans; Pregnancy; Female; Pregnancy Outcome
PubMed: 36229236
DOI: 10.1016/j.jpedsurg.2022.09.004 -
Journal of Laparoendoscopic & Advanced... Jul 2020Currarino syndrome (CS) is characterized by the triad of sacral anomalies, presacral tumor, and anorectal malformation (ARM). This study evaluates the feasibility and...
Currarino syndrome (CS) is characterized by the triad of sacral anomalies, presacral tumor, and anorectal malformation (ARM). This study evaluates the feasibility and outcomes of laparoscopic-assisted anorectal pull-through (LAARP) for CS. Children admitted for primary or redo repair of CS through LAARP between 2016 and 2019 were reviewed. The indication of redo included constipation with megarectosigmoid, residual presacral mass, anastomosis leak, and secondary fistula. ARM was corrected by excision of rectal stenosis, fistula, and its associated megarectosigmoid. This was followed by a complete surgical resection of the presacral tumor, with subsequent pull-through and anocolic anastomosis. This was done with a combined laparoscopic and transanal approach. The detailed surgical techniques, early postoperative complications, and mid-term functional outcomes were summarized. Fourteen patients underwent LAARP for primary ( = 4) and redo ( = 10) repair of CS. Four of them had colostomy previously. Mean age at operation was 20.7 ± 13.9 months. Mean operative time was 120 ± 25 minutes. Median hospital stay was 8 days (range 7-9 days) postoperatively. None of the patients developed early postoperative complications such as anastomotic leaks, presacral abscesses, recurrent fistulas, and residual mass. Bowel function was assessed 1 year after LAARP in 10 patients. Mean follow-up time was 15.9 months. The frequency of bowel movements was 3.3 ± 1.5/day. Constipation occurred in 2 patients. Occasional soiling (<3 times/week) was reported in 4 patients and frequent soiling (>3 times/week) was in 1. LAARP for CS is safe and effective.
Topics: Anal Canal; Anastomosis, Surgical; Anorectal Malformations; Child, Preschool; Colostomy; Defecation; Digestive System Abnormalities; Female; Humans; Infant; Laparoscopy; Length of Stay; Male; Operative Time; Postoperative Complications; Rectum; Retrospective Studies; Sacrum; Syringomyelia
PubMed: 32302513
DOI: 10.1089/lap.2019.0779 -
European Journal of Pediatric Surgery :... Jun 2020Anorectal malformation (ARM) and Hirschsprung's disease (HD) are the most common congenital colorectal anomalies. Despite advances in surgical techniques and... (Review)
Review
Anorectal malformation (ARM) and Hirschsprung's disease (HD) are the most common congenital colorectal anomalies. Despite advances in surgical techniques and improvements in postoperative clinical care, short- and long-term physical and psychosocial morbidity of these patients remains high. This review outlines the current literature on the physical and psychosocial aspects of health-related quality of life (HrQoL) and its confounders in patients with ARM/HD. Moreover, important coping strategies are summarized to further improve HrQoL of ARM/HD patients and their families.
Topics: Adaptation, Psychological; Anorectal Malformations; Child; Female; Hirschsprung Disease; Humans; Infertility; Male; Quality of Life; Severity of Illness Index; Sex Distribution; Sexual Dysfunction, Physiological; Surveys and Questionnaires; Urinary Incontinence
PubMed: 32590868
DOI: 10.1055/s-0040-1713597