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Aims and Objectives: Complications following posterior sagittal anorectoplasty (PSARP) for ARM are well known. In this article, we present our experience of managing five patients who required major redo surgeries for complications resulting from previous attempts to correct ARM. Materials and Methods: We reviewed all patients who underwent major redo surgeries in our hospital for complications from previous repairs for ARM, from June 2013 to June 2019. Data was obtained from hospital records and analysed. Results: Five patients whose ages ranged from 5 months to 14 years were included in the study. Four were boys and 1 was a girl. All patients had undergone PSARP in other hospitals. The presentations were retained distal bowel causing urinary retention and constipation (n=1), pulled through proximal urethra and bladder neck presenting as passage of urine from neo-anus (n=1), retained common channel (of cloaca) causing a 'H' type configuration (n=1), mispositioned neo anus (n=1) following a primary PSARP and lastly undivided recto-urethral fistula causing fecaluria (n=1). All of them underwent redo repairs by posterior sagittal approach with documented improvement in their symptoms. Two of them required total bowel management to remain clean. Conclusion: All the complications reported here have been described in literature nevertheless, this report will add to the body of experience. Posterior sagittal approach (PSA) has proved to be very successful technique in correcting these complications. [ABSTRACT FROM AUTHOR]

Anorectal malformation is a disease with different subtypes and anatomical and functional multisystemic involvement that requires a unique approach in each age group. Anomalies associated with vertebral defects, anal atresia, cardiac defects, tracheo-esophageal fistula, renal anomalies, and limb abnormalities (VACTERL) association require detailed investigation and management. Beginning from the neonatal period, treatment is carried out with different surgical procedures. The clinical course of these patients may be associated with medical problems, accompanying congenital anomalies, perioperative management, or late sequelae. Constipation and fecal–urinary incontinence are the most common problems encountered in long-term follow-up. Renal failure is the most important cause of long-term mortality. In addition, these patients need to be under control until adulthood due to cardiological, spinal, genital, gynecological, and endocrine problems. In this follow-up, many pediatric disciplines such as neonatal intensive care, cardiology, nephrology, gastroenterology, and endocrinology cooperate with pediatric surgeons and pediatric urologists. [ABSTRACT FROM AUTHOR]

Background: Neonatal intestinal obstruction is a challenging issue, especially in developing countries. There is an apparent difference in the etiology, complications, and mortality of intestinal obstruction in neonates in different countries. Objectives: We aimed to describe the causes, early postoperative outcomes, and predictors of morbidities in neonates with intestinal obstruction in a tertiary neonatal intensive care unit (NICU) in Iran. Design & methods: We conducted a retrospective study on neonates who were admitted with intestinal obstruction requiring surgery in the NICU of Boo-Ali Sina Hospital in northern Iran during 2018 to 2022. Demographic and clinical characteristics of the newborns, final diagnosis, postoperative complications, and mortality rate were documented. Also, the relationship between postoperative complications and risk factors, including birth weight, gestational age, and surgical intervention time, was evaluated. Results: A total of 169 neonates with intestinal obstruction requiring surgery were admitted with a male ratio of 60.9% and mean age of 3.85 ± 8.01 days. Imperforate anus with a prevalence of 42% was the most common cause of neonatal intestinal obstruction, followed by Hirschsprung's disease and duodenal atresia. Death after surgery occurred in 4.1% of the patients. Sepsis with a prevalence of 1.4% was the most common early postoperative complication. The late surgical intervention had a statistically significant relationship with the increase in postoperative sepsis (P =.048). Conclusion: The time of surgical intervention is the main predictor of complications in neonatal intestinal obstruction, so prompt diagnosis and timely treatment of these babies can significantly improve the prognosis. It is also necessary to improve access to pediatric surgery services in developing countries. [ABSTRACT FROM AUTHOR]

Anorectal malformations (ARMs) are managed classically in three stages -- colostomy at birth, anorectal pull-through after 2-3 months, and stoma closure. Single-stage pull-through has been contemplated in neonatal age aimed to reduce the number of procedures, better long-term continence, the better psycho-social status of the child, and reduced cost of treatment, especially in resource-strained countries. We conducted a systematic review comparing neonatal single-stage pull-through with stage pull-through and did a meta-analysis for the outcome and complications. Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were followed. PubMed and Scopus databases were searched and RevMan 5.4.1 was used for the meta-analysis. Fourteen comparative studies including one randomized controlled trial were included in the systematic review for meta-analysis. The meta-analysis included 1845 patients including 866 neonates undergoing single-stage pull-through. There was no statistically significant difference for the occurrence of surgical site infection (odds ratio [OR] 0.82, 95% confidence interval [CI]: 0.24-2.83), urinary tract injury (OR 1.82, 95% CI: 0.85-3.89), rectal prolapse (OR 0.98, 95% CI: 0.21-5.04), anal stenosis/stricture, voluntary bowel movements (OR 0.97, 95% CI: 0.25-3.73), constipation (OR 1.01, 95% CI: 0.61-1.67), soiling (OR 0.89, 95% CI: 0.52-1.51), mortality (OR 1.19, 95% CI: 0.04-39.74), or other complications. However, continence was seen to be better among patients undergoing neonatal pull-through (OR 1.63, 95% CI: 1.12-2.38). Thus, we can recommend single-stage pull-through for managing patients with ARMs in the neonatal age. [ABSTRACT FROM AUTHOR]

Urethral duplication is a rare anomaly with varying clinical manifestations. About 300 cases have been reported till date and are often associated with genitourinary (GU) and gastrointestinal (GI) tract anomalies. A 6-day-old neonate presented with an absent anal opening and a tiny perineal fistula having slight meconium staining. He did well after anoplasty. Low anorectal malformation (ARM) has been reported as the GI anomaly associated with urethral duplication. However, an ectopic scrotum as the principal GU tract anomaly associated with urethral duplication is being reported for the first time in literature with probable embryologic explanation. The GU tract anomalies were successfully repaired surgically later in infancy. [ABSTRACT FROM AUTHOR]

Anorectal malformations (ARMs) are common congenital anomalies in neonates. Colonic perforation is very rare in ARMs. Delay in diagnosis of neonate with ARM results in colonic perforations and life-threatening morbidity. Colonic perforation due to ARM may not be completely avoided; however, early diagnosis and management are essential in assuring better outcomes. [ABSTRACT FROM AUTHOR]

Context: Gastrointestinal (GI) duplications are rare congenital malformations with diverse presentations. They usually present in the pediatric age, especially in the first 2 years of life. Aims: To present our experience with GI duplication (cysts) at a pediatric surgery tertiary care teaching institute. Settings and Design: It is a retrospective observational study undertaken in the department of pediatric surgery at our center between 2012 and 2022 for GI duplications. Materials and Methods: All children were analyzed for their age, sex, presentation, radiological evaluation, operative management, and outcomes. Results: Thirty-two patients were diagnosed with GI duplication. Slight male predominance was present in the series (M: F ≈ 4:3). Fifteen (46.88%) patients presented in the neonatal age group; 26 (81.25%) patients were under 2 years. In the majority of cases (n = 23, 71.88%), the presentation was acute onset. Double duplication cysts on opposite sides of the diaphragm were present in one case. The most common location was ileum (n = 17), followed by gallbladder (n = 6), appendix (n = 3), gastric (n = 1), jejunum (n = 1), esophagus (n = 1), ileocecal junction (n = 1), duodenum (n = 1), sigmoid (n = 1), and anal canal (n = 1). Multiple associations (malformations/surgical pathologies) were present. Intussusception (n = 6) was the most common, followed by intestinal atresia (n = 5), anorectal malformation (n = 3), abdominal wall defect (n = 3), hemorrhagic cyst (n = 1), Meckel's diverticulum (n = 1), and sacrococcygeal teratoma (n = 1). Four cases were associated with intestinal volvulus, three cases with intestinal adhesions, and two with intestinal perforation. Favorable outcomes were present in 75% of cases. Conclusion: GI duplications have varied presentations depending on site, size, type, local mass effect, mucosal pattern, and associated complications. The importance of clinical suspicion and radiology cannot be underrated. Early diagnosis is required to prevent postoperative complications. Management is individualized as per the type of duplication anomaly and its relation with the involved GI tract. [ABSTRACT FROM AUTHOR]

The impact of neoadjuvant therapy on the functional outcome of patients with resectable rectal cancer is still controversial. The aim of the present study was to explore the effects of neoadjuvant therapy on anorectal function (ARF), urinary function and sexual function in relevant patients. PubMed, Embase, Web of Science and the Cochrane Library were searched systematically. All English-language studies, published from January 2000 to July 2021, that explored the (postoperative) effects of neoadjuvant therapy versus upfront surgery on ARF, urinary function and sexual function of patients were included (PROSPERO 2021: CRD42021281617). The data in this study were derived from 37 articles based on 33 studies; in total, 17 917 patients were enrolled. The meta-analysis revealed that the incidence of anorectal dysfunction in the neoadjuvant therapy group was significantly higher than that in the group of upfront surgery, which was manifested by a higher incidence of major low anterior resection syndrome (odds ratio = 3.09, 95% confidence interval = 2.48, 3.84; P < 0.001), reduction of mean squeeze pressure and mean resting pressure, and other manifestations, including clustering of stools, incontinence, urgency and use of pads. With the extension of follow-up time, the adverse effects of neoadjuvant therapy on major low anterior resection syndrome existed. Compared with patients undergoing upfront surgery, neoadjuvant therapy increased the risk of urinary incontinence (odds ratio = 1.31, 95% confidence interval = 1.00, 1.72; P = 0.05) and erectile dysfunction (odds ratio = 1.77, 95% confidence interval = 1.27, 2.45; P < 0.001). Compared with upfront surgery, neoadjuvant therapy is not only associated with impairment of ARF, but also with increased incidence of urinary incontinence and male erectile dysfunction. However, the influence of confounding factors (e.g. surgical method, tumour stage) needs to be considered. • Compared with upfront surgery, neoadjuvant radiotherapy is associated with anorectal dysfunction. • Neoadjuvant radiotherapy is associated with an increased incidence of patients with urinary incontinence. • Neoadjuvant radiotherapy is associated with an increased incidence of erectile dysfunction in male patients. [ABSTRACT FROM AUTHOR]

Aim: Posterior cloacal malformations (PCMs) are distinguished from classical cloacal malformations by the posterior location of the common opening in the perineum. We aim to describe our experience of management of these rare and complex malformations. Methods: This study was a retrospective chart review of all patients with PCM who underwent treatment at Kalawati Saran Children's Hospital (KSCH), New Delhi, and Sanjay Gandhi Postgraduate Institute of Medical Sciences (SGPGIMS), Lucknow, between 2013 and 2021. Individual anatomical variations and their impact on the clinical presentation, management, and final outcome (cosmesis and function) were recorded and analyzed. Results: During the study period, four girls with a median age of 2 (range: 0–5) years presented to us with PCM. Three patients were referred to KSCH as ambiguous genitalia whereas one patient was born at SGPGIMS with an antenatal diagnosis of "urorectal septal defect." On examination, three patients had the urogenital sinus (UGS) opening immediately anterior to the normally located anal opening (2 orifices), whereas one had a single orifice at the normal location of the anus with the UGS opening in the anterior rectal wall. Associated anomalies included: (a) hydrocolpos (n = 3), which was managed by a tube vaginostomy; (b) urethral duplication with dorsal atretic urethra (n = 3); (c) uterine didelphys (n = 1); (d) bilateral grade 5 vesicoureteric reflux (n = 1); and (e) vaginal calculus (n = 1). Total urogenital mobilization (anterior sagittal approach) with feminizing genitoplasty was performed for patients with UGS and normal anus (n = 3). In the patient with a single opening at the normal location of the anus, posterior sagittal ano-recto-urethro-vaginoplasty was performed. At a median follow-up of 24 months, three patients are continent for urine and stool, whereas one patient is yet to be toilet trained. Conclusions: PCMs are unusual complex malformations that necessitate meticulous clinical examination, detailed diagnostic workup, and multistaged surgical management. Management should be tailored as per each individual patient's anatomy and clinical presentation for an optimal outcome. [ABSTRACT FROM AUTHOR]

Introduction:  The correction of an anorectal malformation (ARM) is complex and relatively infrequent. Simulation training and subsequent assessment may result in better clinical outcomes. Assessment can be done using a competency assessment tool (CAT). This study aims to develop and validate a CAT for the posterior sagittal anorectoplasty (PSARP) on a simulation model.Materials and Methods:  The CAT-PSARP was developed after consultation with experts in the field. The PSARP was divided into five steps, while tissue and instrument handling were scored separately. Participants of pediatric colorectal hands-on courses in 2019 and 2020 were asked to participate. They performed one PSARP procedure on an ARM simulation model, while being assessed by two objective observers using the CAT-PSARP.Results:  A total of 82 participants were enrolled. A fair interobserver agreement was found for general skills (intraclass correlation coefficient [ICC] = 0.524, p < 0.001), a good agreement for specific skills (ICC = 0.646, p < 0.001), and overall performance (ICC = 0.669, p < 0.001). The experienced group scored higher on all steps (p < 0.001), except for "anoplasty (p = 0.540)," compared with an inexperienced group.Conclusion:  The CAT-PSARP is a suitable objective assessment tool for the overall performance of the included steps of the PSARP for repair of an ARM on a simulation model. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Background Distal colostogram and ileostogram are commonly performed radiological procedures in the pediatric population. It is sometimes difficult to identify the distal stoma while performing these procedures. Aim The aim of this study was to describe a technique for the confident identification of distal stoma. Methods Injection of a small quantity of air through the catheterized stoma and following it can help to identify the stoma, as proximal or distal, based on anatomy of the delineated bowel. Conclusion Pneumatic insufflation is a simple, quick procedure to confidently identify the distal stoma whenever in doubt. [ABSTRACT FROM AUTHOR]

Background: Cat eye syndrome (CES) is a rare chromosomal disease, with estimated incidence of about 1 in 100,000 live newborns. The classic triad of iris coloboma, anorectal malformations, and auricular abnormalities is present in 40% of patients, and other congenital defects may also be observed. The typical associated cytogenetic anomaly relies on an extra chromosome, derived from an inverted duplication of short arm and proximal long arm of chromosome 22, resulting in partial trisomy or tetrasomy of such regions (inv dup 22pter-22q11.2). Case presentation: We report on a full-term newborn, referred to us soon after birth. Physical examination showed facial dysmorphisms, including hypertelorism, down slanted palpebral fissures, and dysplastic ears with tragus hypoplasia and pre-auricular pit. Ophthalmologic evaluation and heart ultrasound identified left chorioretinal and iris coloboma and ostium secundum type atrial septal defect, respectively. Based on the suspicion of cat eye syndrome, a standard karyotype analysis was performed, and detected an extra small marker chromosome confirming the CES diagnosis. The chromosomal abnormality was then defined by array comparative genome hybridization (a-CGH, performed also in the parents), which identified the size of the rearrangement (3 Mb), and its de novo occurrence. Postnatally, our newborn presented with persistent hypoglycemia and cholestatic jaundice. Endocrine tests revealed congenital hypothyroidism, cortisol and growth hormone (GH) deficiencies, which were treated with replacement therapies (levotiroxine and hydrocortisone). Brain magnetic resonance imaging, later performed, showed aplasia of the anterior pituitary gland, agenesis of the stalk and ectopic neurohypophysis, confirming the congenital hypopituitarism diagnosis. She was discharged at 2 months of age, and included in a multidisciplinary follow-up. She currently is 7 months old and shows a severe global growth failure, and developmental delay. She started GH replacement treatment, and continues oral hydrocortisone, along with ursodeoxycholic acid and levothyroxine, allowing an adequate control of glycemic and thyroid profiles as well as of cholestasis. Conclusions: CES phenotypic spectrum is wide and highly variable. Our report highlights how among the possible associated endocrine disorders, congenital hypopituitarism may occur, leading to persistent hypoglycemia and cholestasis. These patients should be promptly assessed for complete hormonal evaluations, in addition to major malformations and midline anomalies. Early recognition of such defects is necessary to decrease fatal events, as well as short and long-term related adverse outcomes. [ABSTRACT FROM AUTHOR]

Background: With the global assimilation of "publish and perish" culture into institutional academics, there has been an exponential rise in the publication numbers. There are ~2500 PUBMED entries related to "anorectal malformation (ARM)." The young clinician in his pursuit to translate experimental research to bedside often finds himself lost "in the midst of plenty." This bibliometric analysis has been conducted to codify the seminal work on ARM for future reference and pay tribute to the most impactful articles. Materials and Methods: Thomson Reuters Web of Science citation indexing database and research platform was used to retrieve the most cited articles in ARM using appropriate search strings. The characteristics (name of authors, the total number of authors, the title of publication, journal of publication, year of publication, etc.,) of the 50 top-cited articles were analyzed. Results: The analysis revealed that the Journal of Paediatric Surgery was leading the choice of journal for publication. While most of the publications originated from the United States of America, Alberto Pena was the most influential author. The most studied topic was on associated malformations, and the most common study design was cohort studies. Conclusion: The approach of citation analysis provided us an opportunity to retrieve the most influential articles on ARM. The trends in research in ARM have also been analyzed, spreading over five decades. [ABSTRACT FROM AUTHOR]

Anorectal malformations (ARMs) encompass a complex spectrum of congenital anomalies and H/N type anorectal malformations are extremely rare. In the presence of colostomy, an augmented pressure colostogram with or without retrograde or micturating cystourethrogram is the investigation of choice. Transperineal ultrasound is an imaging technique that allows a fairly accurate morphological assessment of ARMs along with dynamic evaluation of the anorectal structures and pelvic floor anatomy. Here we describe the role of transperineal ultrasound as an adjunctive modality in diagnosis of one such complex anomaly. [ABSTRACT FROM AUTHOR]

Introduction:  An anorectal malformation (ARM) is a congenital malformation that requires surgical correction. To acquire the skills needed to perform this complex procedure, an affordable simulation model has previously been developed and validated. The aim of this study is to evaluate the suitability of this ARM model (with perineal fistula) for training in hands-on workshops.Materials and Methods:  The ARM model consists of a wooden casing with disposable perineal body. Participants in several international pediatric colorectal hands-on workshops in 2019 and 2020 were asked to participate. They were divided in a target group and an experienced group based on experience. All practiced the posterior sagittal anorectoplasty procedure on the model with multimodality guidance. Subsequently, statements on the suitability of the model for use during hands-on workshops were scored on a 5-point Likert scale.Results:  A total of 80 participants were included (43 surgical specialists, 13 pediatric surgery fellows, and 25 residents). Nearly, all statements scored at least a mean of >4.0, all scored significantly better than a neutral opinion. The target group (n = 58) scored higher compared with the experienced group (n = 22) on "transferability of the skills to the clinical setting" (means 4.4 vs. 4.0, p = 0.038); however, the "suitability as a replacement for an animal model" scored significantly lower (means 3.6 vs. 3.9, p = 0.049). No other differences were found.Conclusion:  This affordable ARM model was regarded a suitable model for training during preclinical hands-on workshops and could be used for the specified steps of the procedure. [ABSTRACT FROM AUTHOR]

Background: Gastrointestinal (GI) malformations have varied short-term and long-term outcomes reported across various neonatal units in India. Methods: This descriptive study was done to study the clinical profile, outcomes and predictors of mortality in neonates operated for congenital GI malformations in a tertiary neonatal care unit in South India between years 2011 and 2020. Details were collected by retrospective review of the case sheets. Results: Total of 68 neonates were included with esophageal atresia (EA) in 10, infantile hypertrophic pyloric stenosis (IHPS) in 9, duodenal atresia (DA) in 10, ileal atresia in 8, jejunal atresia in 5, anorectal malformations (ARM) in 11, meconium ileus/peritonitis in 9, malrotation in 2, and Hirschsprung's disease (HD) in 4. Antenatal diagnosis was highest in DA (80%). Associated anomalies were maximum in EA (50%), the most common being vertebral, anal atresia, cardiac defects, tracheoesophageal fistula, renal and radial abnormalities, and limb abnormalities association (VACTERL). Overall mortality was 15%. IHPS, DA, Malrotation, HD and ARM had 100 % survival while ileal atresia had the least survival (38%). Gestational age <32 weeks (odds ratio [OR] 12.77 [1.96, 82.89]) and outborn babies (OR 5.55 [1.01, 30.33]) were significant predictors of mortality in babies operated for small intestinal anomalies. None of the surviving infants were moderately or severely underweight at follow-up. Conclusion: Overall survival of surgically correctable GI anomalies is good. Among the predictors for mortality, modifiable factors such as in-utero referral of antenatally diagnosed congenital anomalies need attention. One-fifth had associated anomalies highlighting the need to actively look for the same. Although these neonates are vulnerable for growth failure, they had optimal growth on follow-up possibly due to standardized total parenteral nutritional policy during neonatal intensive care unit stay. [ABSTRACT FROM AUTHOR]

Introduction: Laparoscopic anorectoplasty (LAARP) is useful for the management of rectoprostatic urethral fistula (RPUF), due to easier rectal mobilization, avoidance of posterior sagittal muscular incision, and shorter hospital stay. However, its role in rectobulbar urethral fistula (RBUF) is still debated as there is a chance of urethral diverticulum (UD), due to incomplete dissection. Laparoscopy-assisted modified posterior sagittal anorectoplasty (LAMPSARP) utilizes advantages of LAARP combined with fistula dissection using small sagittal incision preserving puborectalis. The present study compares the results of LAMPSARP with LAARP for correction of RBUF associated with anorectal malformations (ARMs). Materials and Methods: All male ARM with RBUF presenting in a tertiary center in Central India (January 2014–January 2016) were included. Low male ARM, RPUF, rectovesical fistula, and congenital pouch colon were excluded. They were randomized into LAARP and LAMPSARP groups. Complications were assessed in terms of anal stenosis, mucosal prolapse, and UD. Kelly's scoring and Krickenbeck scoring were used to assess continence, and visible anal cosmesis scale (VACS) was used to assess wanal cosmesis. Results were statistically analyzed using a comparison of means and 2 × 2 contingency tables. Results: Fifty-six colostomized patients with RBUF (26 LAARP, 30 LAMPSARP) were included. Mean operative duration in the LAARP group and LAMPSARP group was 42 ± 10 min and 56 ± 12 min, respectively (P < 0.0001). On mean follow-up of 4.5 years, mucosal prolapse (53.9%) and UD (15.38%) were significantly higher in LAARP group, while anal stenosis was similar. All three, Kelly's score, Krickenbeck score, and VACS, were better (P < 0.05) in the LAMPSARP group. Conclusion: Laparoscopy-assisted modified posterior sagittal approach is better for RBUF and offers better surgical outcome. [ABSTRACT FROM AUTHOR]

Background: In children with anorectal malformations (ARM), the vertical fibres of the striated muscle complex (SMC) are believed to be located within the limits of the anal dimple (AD). Methods: Forty five cases of ARM underwent posterior sagittal anorectoplasty (PSARP), median age 8.5 months. During PSARP the anterior, posterior limits and midpoint of the AD and SMC were marked. The location of AD was correlated with SMC. Results: A 'well developed' AD and SMC was seen in 80 % and 86.7 % patients respectively. The mean width of the AD and SMC was more in females than in males (20.96 vs. 18.98 mm and 14.24mm vs. 13.45mm respectively). In 36 cases (80 %), across the spectrum of ARM, the SMC was posterior in relation to the AD. In 7 cases (15.5 %) it correlated in position with the AD and in 2 cases (4.44 %), it was anterior to the AD. Conclusions: In the majority of cases AD and SMC were 'well developed' and the location of the SMC does not correlate with that of the AD. This has significant practical value in the important step of optimizing the placement of the rectum through the center of the SMC during repair. [ABSTRACT FROM AUTHOR]

Anorectal malformations (ARMs) are one of the common congenital gastrointestinal malformations with a prevalence of 0.2–0.6/1000 live births. There are only a few reported cases of familial ARMs in literature suggesting different patterns of inheritance. Among them, isolated ARMs in monozygotic twins are quite rare with significant male preponderance. Most of these ARMs are either of intermediate or low anomaly type. We reported two pairs of male monozygotic isolated ARMs from a tertiary care hospital in India and reviewed the literature in details adding to the total of nine pairs of twins till date. [ABSTRACT FROM AUTHOR]

Introduction: Associated congenital anomalies, operative structural injury, and postoperative neurovesical dysfunction combine to cause urinary retention in children with a history of surgery for anorectal malformation (ARM). Aim: To study the presentation and management of urinary retention in patients with a history of ARM surgery. Methodology: Retrospective study. Results and Discussion: Twenty-five children presented with urinary retention with a history of ARM surgery performed elsewhere. There were 17 males (high – 14, intermediate – 2, and low – 1) and 8 females (cloaca). Sixteen children had an abnormal spine. Eight children had urethral injury (total transection – 3) and three had a large residual rectal stump. 41/48 renal units were dilated and 27 refluxing. Seventeen children had abnormal estimated glomerular filtration rate and five had undergone urinary diversion. Management: Definitive surgical management was individualized, the most pertinent consideration being whether normal voiding would be feasible or whether a continent low pressure urinary reservoir with clean intermittent catheterization (CIC) would be a safer option. Operative management included excision of the rectal stump (3), urethral reconstruction (2), bladder augmentation (17), Mitrofanoff port (22), bladder neck closure (2), and antireflux surgery (13). Follow-up estimated glomerular filtration rate had improved/normalized in all but two patients. HUN resolved/improved in all and 25/27 refluxing units ceased refluxing. All are socially continent with ten voiding normally and the rest on CIC. Conclusion: Urinary retention after ARM surgery is multifactorial and requires prompt recognition and possibly urinary diversion. Final reconstruction aims at achieving continence with safe upper tracts. Urethral voiding is possible in the selected cases. [ABSTRACT FROM AUTHOR]

Aim: The aim of the study was o define the preoperative diagnostic clinical and radiological features in girls with congenital pouch colon (CPC). Materials and Methods: Records of 47 girls with CPC, managed from 1996 to 2018, were reviewed. There were two age groups: Group A (newborn to 12 months; n = 26) and Group B (>12 months to 20 years; n = 21). The important clinical and radiologic features to help in a preoperative diagnosis were noted. Results: The most common subtype was Type II (57.4%), followed by Type I (23.4%) and Type III (12.8%). The features common to both the groups were abdominal distension (A = 53.8%; B = 9.52%), severe perineal excoriation (A = 19.2%; B = 23/8%), and urinary incontinence (A = 30.7%; B = 85.7%). In addition, in Group B, fecaloma on abdominal palpation was noted in 28.6% of patients. The characteristic appearance of the perineum including external genitalia and findings on plain abdominal X-ray (AXR) were 100% accurate and hence diagnostic. These unique features helped us formulate an algorithm for preoperative diagnosis of this uncommon form of anorectal malformation in girls seen in North India. Conclusions: The characteristic features on clinical examination should alert one to the presence of CPC in the outpatient clinic. The AXR was diagnostic in 100% of cases and is mandatory. If any doubt persists, examination of the genitalia under anesthesia with more retraction of the labial folds and endoscopy can be performed for confirmation. These measures should enable a clinician to make an accurate preoperative diagnosis in every girl with CPC. [ABSTRACT FROM AUTHOR]

We report a case of colonic agenesis with anorectal malformation in a newborn girl. The patient also presented with congenital heart disease. We are presenting the clinical features, intraoperative findings, and treatment plan. [ABSTRACT FROM AUTHOR]

Anorectal malformation (ARM) is a relatively frequently occurring congenital anomaly of hindgut development with a prevalence of 1 in 3,000 live births. ARM may present as an isolated anomaly, but it can also be associated with other anomalies, sometimes as part of a recognizable syndrome. After birth, much medical attention is given to the treatment and restoring of bowel function in children with ARM. Effort should also be given to studying the etiology of the ARM in these patients. This information is important to both the medical community and the family, because it can help guide treatment and provides information on the long-term prognosis of the patient and recurrence risk in the family.In this article, we will review the current knowledge on the (genetic) etiology of (syndromic) ARM and provide guidelines for (family) history taking and clinical and genetic studies of ARM patients and their families, which is needed to study the causal factors in an ARM patient and for genetic counseling of the families. [ABSTRACT FROM AUTHOR]

Introduction: The use of personal watercrafts (PWCs) has increased in popularity, size and engine power over time. Hydrostatic injuries when the passenger falls into water and the pelvic floor is directly hurt by the water jet remain rarely reported in literature but potentially lethal. Perineal, gynaecological and rectosigmoid regions present significant points of anatomical weakness to the force vector of the water. Biomechanical aspects on water-associated injuries on PWC may include disruption of the perineal soft tissues, inefficient anal sphincter and hydrodynamic insufflation. Methods: This is a study involving a literature review from 1972 to 2020 using suitable search terms to identify all hydrostatic PWC injuries following PRISMA guidelines. Data were extracted from suitable articles on mechanism of injury, injuries sustained, treatment and outcomes. Results: Thirty-two patients with major perineal, gynaecological and/or digestive injuries due to hydrostatic PWC traumas were identified. Major risk factors were female gender (84.4%), young age (25.5 years), being a rear passenger and wearing a standard swimsuit (100%). The injuries were digestive tract only (81.3%), gynaecological only (46.9%) or combined (28.1%). The interdisciplinary surgical management must include a vaginal and anal exploration under general anaesthesia and an exploratory surgery. Vaginal repair (41.9%) may be associated with a transanal anorectal suturing for lacerations of the intraperitoneal rectum (40.6%). A defunctioning stoma was performed in 62.5% and consisted of a loop sigmoidostomy (43.5%) or Hartmann's procedure (34.8%) depending on laceration complexity. Conclusions: PWC-related hydrostatic injuries are still rare but associated with a mortality rate of 6.3% which rises to 25% if initial haemodynamic instability was present. Expert guidelines such as adding an automated engine shut-off switch for the rear passenger and wearing a protective and safety clothing should be more widely respected. [ABSTRACT FROM AUTHOR]

Introduction. Perineal groove is an infrequent midline malformation. It is a humid, mucosal, non-keratinized groove located at the perineal midline, extending from the vulvar fourchette to the anterior anal border. It is rare and usually asymptomatic, and it heals spontaneously in most cases. It is frequently mistaken for other malformations, which means correctly identifying it is essential to avoid iatrogenesis. Clinical case. We present the case of a female newborn with an asymptomatic lesion at the perineal midline consistent with anorectal malformation. Following assessment by the Pediatric Surgery Department, she was diagnosed with perineal groove. Discussion. Perineal groove is a little known malformation among healthcare professionals as it is infrequent and there are not many publications in the literature about it. This case demonstrates how important it is to keep this abnormality in mind to avoid erroneous diagnoses, unnecessary treatments, and family stress. [ABSTRACT FROM AUTHOR]

Rectal temperature dynamic was studied in newborn goat kids of different genders during the first hour of postnatal life. Goats were kept in a barn and rectal temperature of the newborns goat kids was recorded at birth, at 15 min of birth, at 30 min, 45 min and 60 min after delivery. The dynamic of the rectal temperature during the first hour after birth did not differ significantly in male and female kids. The rectal temperature established at the indicated intervals was: in female kids (39.11 °C, 38.47 °C, 38.21 °C, 38.10 °C, 38.22 °C) and in male kids (39.14 °C, 38.55 °C, 38.49 °C, 38.55 °C, 38.51 °C), respectively. After lowering the rectal temperature of both sexes after 15 minutes, the female kids maintained a stable, almost unchanged temperature until the end of the first hour after birth. The lowest temperature measured in males was on the 45th minute and in females – on the 30th minute. At the time of birth, the kids had a well-developed thermoregulatory potential and were able to maintain homeothermia with existing goat nurturing technology during the barn period. [ABSTRACT FROM AUTHOR]

Background: Congenital pouch colon (CPC) is a rare variant of anorectal malformations (ARM) with its highest reported incidence in India. We aimed to describe five patients affected by CPC, in which the tissue from the terminal dilated colon has been successfully used and to discuss our results on the light of an extended revision of the literature. Materials and Methods: The clinical details of five cases treated for CPC in two Italian Centers were retrospectively reviewed assessing the fate of the terminal dilated colon. Results: In all cases, the tissue from dilated colon has been used. The double vascular system of the dilated pouch allowed increasing bladder capacity (case 4), reconstruction of the vagina (case 3, 5), and lengthening of the colon (case 1, 2, 5). In our series, 3/5 have a good bowel control with daily bowel management after ARM correction. In literature, there are not differences in terms of dependence from bowel management in patients with pouch resected and in patients with pouch saved (P = 0.16). Conclusions: We acknowledge that the analysis of the available literature is limited by the absence of studies with high level of evidence and the removal or the preservation of the abnormal colon tissue seems to follow the surgeon preferences. [ABSTRACT FROM AUTHOR]

Patients with anorectal illness (AI) must deal with shame from social stigma and difficulties in the medical context. Recovering from shame is a challenge. Applying shame resilience theory (SRT) to the Chinese health care setting, this study explores how patients with AI develop resilience to shame using humor to facilitate the narrative's five functions. The method is a thematic narrative analysis of 60 stories from a Chinese online community. Four main themes were identified: understanding shame events, normalizing them, shifting priorities, and transforming shame into pride. Storytellers can use humor to externalize shame, reflect on their concealment and avoidance, eliminate the shame associated with making health decisions, re-establish shameless identities, achieve positive agency, and build illness communities through "aligning moments" with their audiences and subverting hierarchies of normality and abnormality. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Normal bowel function requires intact sensory pathways. Diminished rectal sensation (rectal hyposensitivity [RH]) is associated with constipation, although its clinical importance remains unclear. METHODS: Consecutive patients (aged 18-80) attending a tertiary center (2004-2016) for investigation of refractory functional constipation (Rome IV core criteria defined, applied post hoc) were included. Patients completed a clinical symptom questionnaire and underwent anorectal physiologic investigations, including rectal sensory testing (balloon distension) to determine 3 well-established sensory thresholds. Multivariate regression analyses were performed to evaluate associations between RH, symptomology, and allied physiologic investigations. RESULTS: Of 2,876 patients meeting inclusion criteria, 722 (25%) had RH based on ≥1 elevated sensory thresholds (0: n = 2,154 [74.9%]; 1: n = 327 [11.4%]; 2: n = 209 [7.3%]; and 3: n = 186 [6.5%]). A linear relationship existed between increasing number of elevated sensory thresholds and constipation severity (Cleveland Clinic constipation score: mean difference per threshold [95% confidence interval] 0.69 [0.48-0.90]; P < 0.001). Several symptoms were significantly (P < 0.05) associated with RH including: infrequent defecation (odds ratio 1.29 [1.17-1.42]), painful evacuation (1.15 [1.05-1.27]), prolonged toileting (1.14 [1.05-1.24]), and digitation or enema use (1.18 [1.08-1.30]). On defecography, a "functional" evacuation disorder was also associated with RH (1.37 [1.25-1.50], P < 0.001), as was megarectum (2.52 [2.08-3.05], P < 0.001). DISCUSSION: RH occurs in 25% of patients with refractory functional constipation. Increased number of elevated sensory thresholds is associated with more severe constipation phenotype. These data, in the largest study to date, provide for the first time evidence to show that RH is a major pathophysiologic mechanism in constipation, with recognized clinical impact (http://links.lww.com/AJG/B765). [ABSTRACT FROM AUTHOR]

The anorectal malformation with long perineal fistula is a rare anomaly in the spectrum of anorectal malformations. Aim of the study is to describe the series of patients with anorectal malformation with long perineal fistula and compare the outcome with patient with standard perineal fistula. From March 2012 to January 2019, 7 patients who suffered from anorectal malformation with long perineal fistula were retrospectively reviewed. Three were operated on primarily by our department, and 4 cases were re-operated after a perineal anoplasty repair performed elsewhere. Four were operated by laparoscopy assisted anorectoplasty, and 3 cases were repaired by posterior sagittal anorectoplasty. The follow-up outcomes were compared with 71 cases of normal perineal fistula (NPF) in the same period. 7 cases have been followed up for 0.5–4 years (M = 2.57 ± 1.26) after definitive surgery. Their bowel function score was lower than normal perineal fistula (SPF = 12, range: 5–18; NPF = 18.5, range: 18–20). Four cases underwent anorectomanometry. The incidence of rectoanal inhibitory reflex was lower in the special type group. (p = 0.14). Three cases of contrast enema using barium: 2 cases of colorectal dilatation and thickening changes, 1 case showed no obvious abnormalities. Anorectal perineal fistula should be examined by distal colostogram at preoperation. This should be altered in: When suspecting a case of anorectal malformation type long perineal fistula a preoperative contrast enema could give insight of the anatomy befor performing a anoplasty. [ABSTRACT FROM AUTHOR]

Background: Hirschsprung's disease (HD) rarely co-occurs with anorectal malformation (ARM). If it occurs, the classical variety of HD is mostly associated. Total Colonic Aganglionosis (TCA) in a case with ARM is exceedingly rare. Case Presentation: A 3-day-old female neonate presented with neonatal intestinal obstruction. of such a rare association. Examination revealed a perineal fistula. A colostomy was formed for persistent abdominal distension, but it did not work properly. Re-exploration revealed TCA with a transition zone at the level of the terminal ileum. Conclusion: Though ARM is associated with several anomalies of various body systems, its association with TCA is exceedingly rare. [ABSTRACT FROM AUTHOR]

Colon duplication (CD) is a rare congenital abnormality with varied presentations. We present a neonatal case with complete tubular CD terminating uniquely as a common channel, a single rectovestibular fistula. We summarize surgical management options in CD as well as factors that will help to decide the best surgical treatment in individual cases. [ABSTRACT FROM AUTHOR]

Aim: To clarify the characteristics of patients with rectal prolapse after laparoscopically assisted anorectoplasty (LAARP), estimate the causes, and evaluate its impact on postoperative bowel function. Methods: The medical records of patients who underwent LAARP for high- or intermediate-type anorectal malformation between 2000 and 2019 were retrospectively reviewed. Clinical data were compared between patients with (Group P) and without prolapse (normal, Group N). Fecal continence was evaluated using the clinical assessment score for fecal continence developed by the Japanese Study Group of Anorectal Anomalies. For patients who underwent pelvic magnetic resonance imaging (MRI) before LAARP, atrophy, or asymmetry of the anal sphincter and levator ani was evaluated by a radiologist. Results: Of the 49 patients, 29 (59%) had rectal prolapse after LAARP (Group P) and 20 did not (Group N). We found no significant difference in gender, type of malformations, incidence of associated spinal or lumbosacral anomalies, procedure time, and postoperative bowel function at ages 4, 8, 12, and 16 years. However, LAARP was performed significantly earlier in Group N (median [range], 180 [123-498] days) than in Group P (210 [141-570] days). In Group P, 18 patients (62%) developed prolapse before colostomy takedown. Eight of 26 patients who underwent surgical prolapse repair required redo procedures. Twenty-five patients who underwent preoperative pelvic MRI showed no significant relationship between the muscular abnormalities and the incidence of postoperative rectal prolapse. Conclusions: Although recurrence is common, performing LAARP at a younger age might prevent postoperative prolapse development. [ABSTRACT FROM AUTHOR]

The aim of this study is to develop a tool that is aligned with patients' and health professionals' needs to address sexual health in the context of anorectal malformations and Hirschsprung's disease. A multiphased participatory action–research was conducted. First, an inventory of needs was made through interviews (11 patients, 11 professionals), three online focus groups (4 patients, 20 professionals), and a questionnaire (38 patients). Subsequently, four cocreation sessions with in total four patients and nine professionals were organized to translate the needs into a tool (in the form of a website). The websites' functionality was assessed via a questionnaire (n = 34). The website, directed to patients, their parents, and professionals, stimulates awareness, fills knowledge gaps, and shows possibilities for support. The website is expected to change restrictive attitudes toward sexual health and improve the legitimization of the topic needed for the allocation of resources and sexologists' involvement in current care pathways. [ABSTRACT FROM AUTHOR]

Aim: This study reviewed the literature on the postoperative complications following laparoscopic-assisted anorectoplasty (LAARP).Methods: A Medline and Embase search was performed for the terms "anorectal malformation" (ARM) "laparoscopic" and "complication". Articles without English full text, review articles, systematic reviews, case reports, case series < 5 cases and duplicate articles were excluded. Two reviewers independently performed the eligibility assessment and data extraction. Data were collected for type of malformation, surgical technique, postoperative complications and functional outcomes RESULTS: The search retrieved 108 articles, of which 38 met the inclusion criteria and offered 1058 patients for analysis. Rectoprostatic and rectobladder neck fistula were the most common types of ARM in males, whereas it was the common cloaca in females. Analysis of complications demonstrated rectal prolapse (n = 149; 14.08%) being the most prominent, followed by urethral diverticulum (n = 32; 3.02%), anal stenosis (n = 37; 3.49%), recurrent fistula (n = 7; 0.66%) and rectal stricture (n = 4; 0.37%). Krickenbeck classification was used for functional assessment in 638 patients, with fecal soiling grade 2 or > 2 in 79. Data on functional outcome specific to the type of malformation was available for 246 patients: fecal soiling grade 2 or > 2 in 15/94 (15.95%) with rectoprostatic fistula, 26/73 (35.61%) with rectobladder neck fistula, 6/47 (12.76%) with common cloaca, and 1/22 (4.54%) with no fistula.Conclusion: Rectal prolapse, posterior urethral diverticulum and anal stenosis are the most common complications after LAARP. Inconsistent and non-uniform functional assessment and non-availability of information about the sacrum and spine make it difficult to analyze the functional outcome following LAARP. [ABSTRACT FROM AUTHOR]

Purpose: Laparoscopic-assisted anorectoplasty (LAARP) is considered to benefit the male patients with anorectal malformation (ARM). This study evaluates LAARP management for intermediate type rectovestibular fistula (IRVF) in the female patient with ARM.Methods: Twelve patients with IRVF (aged 3-5 months) underwent LAARP from 2017 to 2019 in our institute. LAARP was performed for mobilization of the rectum, visualization and enlargement of the center of the sphincter muscle complex (SMC) from pelvic and perineal aspects, intra-fistula mucosectomy and rectal pull-through in the SMC with the fourchette and the perineal body unattached.Results: LARRP was performed in all patients without conversion to open procedure. No patient suffered from wound infection, vaginal injury, recurrent fistula and anal stenosis. The parents were satisfied with the appearance of the wound. Rectal prolapse developed in one patient and needed surgical correction. The patients were followed up for a mean of 19.7 months (ranged from 12 to 35 months).Conclusion: Our preliminary experience shows that LAARP offers an alternative method of correction for the IRVF with good visualization of the SMC and may diminish the risks of wound dehiscence and vaginal injury. [ABSTRACT FROM AUTHOR]

Congenital pouch colon (CPC) is classified on the basis of anatomic morphology into five types. Congenital segmental intestinal dilatation associated with anorectal malformation (ARM) is very rare. We are adding two neonates of CPC type 2 associated with segmental dilatation of the ileum to single similar case described in the literature till date. Temporary stomas with excision of either of the pouches should be considered. "Defective mesenchyme formation during organogenesis" due to abnormal mesenchymal precursor cells is hypothesized as the cause for both ARM and different types of CPC. [ABSTRACT FROM AUTHOR]

Background: Anorectal malformations (ARMs) in the sub-Saharan Africa are a common cause of neonatal referral for intestinal obstruction, and the posterior sagittal anorectoplasty (PSARP) approach is rapidly spreading. The small number of paediatric surgeons and the low-resource context limit children's access to care and constrain the quality of results. A retrospective, observational study has been done on a consecutive series of ARM cases admitted to a Sudanese tertiary paediatric surgical centre within the framework of a partnership between Italian and Sudanese academic institutions addressed to review and upgrade the standard of care of major congenital anomalies.Materials and Methods: The authors collected 94 ARM cases in a 3 years' period. Conditions on referral, operative procedures, post-operative course and follow-up were recorded and examined. Their correlations with complications and outcome were analysed.Results: The male/female ratio was 47/47. Eighty patients presented with an untreated ARM; 66 had a divided stoma and 14 had already a PSARP procedure, followed by a poor outcome or sequelae. In 25% of the cases, colostomy required re-doing. In 57 cases, a staged PSARP (primary or re-do) was done. Surgical-site infections occurred in nine patients. Some patients were lost to follow-up after preliminary colostomy. Post-operative dilatation programme suffered from the lack of systematic follow-up, and colostomy closure was possible in 46% of the cases due to problems in travelling and accessing hospital care. Anal stenosis was frequently observed among unfollowed patients.Conclusion: Despite PSARP's widespread adoption in Africa, the risk of complications and failures is high. Primary management is often inappropriate, and a high rate of colostomy-related complications is observed. Poverty and lack of transportation reduce attendance to follow-up, hampering the final results. Investments in healthcare facilities and retention of trained health providers are needed to improve the standard of care. [ABSTRACT FROM AUTHOR]

Objective: 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. Materials and Methods: 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. Results: Fourteen patients underwent LAARP for primary (N = 4) and redo (N = 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. Conclusion: LAARP for CS is safe and effective. [ABSTRACT FROM AUTHOR]

Context: Anterior sagittal anorectoplasty (ASARP) is accepted as one of the techniques for the repair of vestibular fistula (VF) and low-type anomalies, but some may have reservations. Aims: The aim of the study is to describe the technique, important features, and functional and cosmetic outcomes of ASARP for the treatment of anorectal malformation (ARM) in females. Settings and Design: A prospective study was performed from 1992 to 2017. Materials and Methods: The study included 157 pediatric patients (aged 1-15 years) with diagnosis of ARMs with VF, perineal fistula (perineal ectopic anus), and rectovaginal fistula managed by ASARP. Results: Most cases (36.94%) were 1-6 months' age group; 92.99% of patients (146) were having VF, 5.09% (8) perineal fistula, and 1.91% (3) rectovaginal fistula. Associated anomalies (37) were present in 19.75% (31) of patients. Primary ASARP was performed in 85.35% (134) of cases. The mean operative time was 105 (±15) min. Intraoperative complications were seen in 3.82% (6) of patients. Early postoperative complications were seen in 5.09% (8) of patients - wound infection (4), wound dehiscence (3), and retraction of the rectum (1). Late complications were seen in 12.73% (20) of cases. Overall, five patients developed anal stenosis, two responded to dilatation therapy, and three required anoplasty. The external appearance of the perineum after the 3rd month (postoperatively) was satisfactory in 95.54% (150); overall, 4.46% (7) of patients required the second procedure. Stooling pattern could be assessed in 80.25% (126) of patients at 3 years' age group. Only one had poor outcome with severe soiling (incontinence) and perineal excoriation that also had myelomeningocele. Conclusions: ASARP is an excellent procedure for VF as it results in optimal correction with minimal sphincteric damage, without additional complexity or difficulties. Primary ASARP is a quick and effective technique and does not require colostomy if performed after due preoperative gut preparation and by an experienced pediatric surgeon. [ABSTRACT FROM AUTHOR]

Background: This study aimed to evaluate outcome of children on chronic peritoneal dialysis (PD) with a concurrent colostomy. Methods: Patients were identified through the International Pediatric Peritoneal Dialysis Network (IPPN) registry. Matched controls were randomly selected from the registry. Data were collected through the IPPN database and a survey disseminated to all participating sites. Results: Fifteen centers reported 20 children who received chronic PD with a co-existing colostomy. The most common cause of end stage kidney disease was congenital anomalies of the kidney and urinary tract (n = 16, 80%). The main reason for colostomy placement was anorectal malformation (n = 13, 65%). The median age at colostomy creation and PD catheter (PDC) insertion were 0.1 (IQR, 0–2.2) and 2.8 (IQR 0.2–18.8) months, respectively. The colostomies and PDCs were present together for a median 18 (IQR, 4.9–35.8) months. The median age at PDC placement in 46 controls was 3.4 (IQR, 0.2–7.4) months of age. Fourteen patients (70%) developed 39 episodes of peritonitis. The annualized peritonitis rate was significantly higher in the colostomy group (1.13 vs. 0.70 episodes per patient year; p = 0.02). Predominant causative microorganisms were Staphylococcus aureus (15%) and Pseudomonas aeruginosa (13%). There were 12 exit site infection (ESI) episodes reported exclusively in colostomy patients. Seven colostomy children (35%) died during their course of PD, in two cases due to peritonitis. Conclusion: Although feasible in children with a colostomy, chronic PD is associated with an increased risk of peritonitis and mortality. Continued efforts to reduce infection risk for this complex patient population are essential. [ABSTRACT FROM AUTHOR]

Background: Congenital pouch colon (CPC) is a rare variant of anorectal malformation. In male patients, CPC communicates distally with the urogenital tract by a large fistula. The CPC cases which do not fulfill the criteria as mentioned in the classical description are reported along with the pertinent literature review. Materials and Methods: This was a retrospective study from January 2004 to December 2017 of male children with Type IV CPC. We evaluated clinical presentation, primary management, anatomical relationship, previous surgical intervention, definitive management, result, and outcome in terms of continence status on Templeton score. Results: Fifty-one children were included in the study among whom 36 children (Group 1) had a colovesical fistula and 15 children (Group 2) had no communication of the pouch with the genitourinary tract. In Group 2 children, the clinical presentations and management were varied: 4 underwent primary pull-through procedure, whereas 11 underwent staged procedure. Group 2 included three children in whom a narrow and thin-walled anal canal or anal canal with lower rectum was present, which was incorporated during the pull-through procedure. On continence assessment, only one child in Group 1 had "good" continence score compared to four children (three having anal canal) in Group 2. Conclusion: CPC Type IV can present without genitourinary tract communication (fistula), contrary to its emblematic description. Awareness about anatomical variations and adaptation of surgical technique accordingly is vital. The identification of the anal canal with or without the lower part of the rectum (even though apparently narrow and thin walled) and incorporation of this in bowel continuity lead to better outcomes in terms of continence. [ABSTRACT FROM AUTHOR]

Objective: Purpose of our study was to aggregate and analyse rare cases of Ectopic Ureter (EU), their association with other anomalies, clinical features, diagnosis and management. Material and methods: A total of nine patients with rare presentation of EU were evaluated. Combination of endoscopic and imaging modalities was used as required to define the anatomy and devise the best surgical approach in these cases. Results: Among six females and three males with EU, four cases had bilateral EU, four unilateral EU and one case had EU of a solitary kidney. Urinary incontinence was encountered in five cases including one male patient whilst other cases presented with varied clinical features and associated anomalies. Two patients had anorectal malformations, and two had uterine anomalies in the form of bicornuate uterus. Other patients had multiple rare associations such as triplication of ureter, bilateral absence of seminal vesicles with infertility, multicystic dysplastic kidney, ureter draining in uterus, renal failure, absence of bladder trigone, and hypospadias etc. Ureteric reimplantation was performed in four cases, two required ureteroureterostomy, another two had undergone upper pole nephrectomy and in one case renal transplant had been carried out owing to chronic renal failure. Conclusion: EU is among group of those congenital entities which remain shrouded until adulthood, when symptoms become distressing. Fortunately, prognosis is favourable after surgical correction, in spite of its rarity. Complexity arises when other associated anomalies are identified. [ABSTRACT FROM AUTHOR]

Purpose: Many kinds of operative procedures have been proposed for anorectal malformation (ARM) patients. At our institution, sacroperineal or sacroabdominoperineal anorectoplasty (SP-SAP) have been performed from 1984 to 2007. The aim of this study is clarify the change over the time in the postoperative bowel function in male ARM patients.Methods: Patient data were collected from 1984 to 2007. Fifty-two male patients with high- and intermediate-type ARM were enrolled. The patients' characteristics and bowel function were reviewed and analyzed retrospectively. The bowel function was evaluated according to the evacuation score (ES) of the Japan Society of ARM Study Group.Results: The operative procedures were SP-SAP in 52 male patients. The total ES improved chronologically and significantly until 11 years of age. Regarding the clinical stratification of the ES, the ratio of "excellent" and "good" results was over 91.9% at 11 years of age. A satisfactory bowel movement score was achieved by 9 years of age. The constipation, incontinence and soiling scores improved slowly but continuously until 11 years of age.Conclusion: The ES showed continuous improvement after a definitive operation. An understanding of the characteristics of improvement is very important in managing the postoperative bowel function in ARM patients. [ABSTRACT FROM AUTHOR]

Purpose: The aim of this study was to investigate risk factors for recurrence in the perineal canal (PC).Methods: Patients with PC who underwent operations were enrolled in this study and were divided into recurrence and non-recurrence groups. Preoperative infection, the age at the operation, the presence of colostomy and the treatment procedure for fistula were retrospectively investigated. Regarding the treatment procedure for fistula, either closure of the rectal wall with stitches or ligation of fistula in the rectum was performed. These factors were compared between the two groups.Results: Six of 17 patients with PC who underwent surgical treatment had recurrence. There were no significant differences in the incidence of preoperative infection, age at operation or presence of colostomy (p = 0.60, 0.38, 1.00, respectively). In the recurrence group, all patients were treated by closure of the rectal wall. In the non-recurrence group, five were treated by the closure of the rectal wall with stitches and six by ligation of the fistula. There was a significant association between recurrence and the treatment procedure for fistula (p = 0.04).Conclusion: Closure of the rectal wall with stitches is a risk factor for the recurrence of PC. [ABSTRACT FROM AUTHOR]

Purpose: Despite medical advances, individuals with anorectal malformations (ARM) experience significant medical and psychosocial challenges due to their complex conditions. This study aimed to obtain the perspectives of adults with ARM throughout their lifetime regarding their medical, school/vocational, and psychosocial functioning.Methods: A 40-item survey was administered electronically to members of an international ARM Facebook group (56% response rate; n = 125). Survey items included demographics, medical diagnosis/treatment, school/workplace accommodations, mental health diagnosis/treatment, and life perspectives.Results: Majority of respondents were female (73%), aged 25-34 years (31%), Caucasian (92%), US residents (60%), and attended public school (86%). 53% of respondents are currently employed. 32% of respondents received school-based accommodations and 24% at work. 58% of respondents had a mental health diagnosis, with depression (82%) and anxiety (81%) being the most common.Conclusions: Results suggest that adults with ARM experience ongoing difficulties related to schooling, employment, and mental health, in addition to medical complications. It is becoming increasingly clear that improving patients' physical well-being is not enough; psychosocial concerns must also be addressed directly. Thus, it is important for clinicians to be aware of and partner with psychosocial providers to support these challenges associated with ARM, to maximize patients' overall health and well-being. [ABSTRACT FROM AUTHOR]

In a female neonate with anorectal malformation (ARM), the diagnosis is usually simple. But when there are two openings in the introitus and absent anal opening at the normal site, the scenario poses a diagnostic challenge. Careful and detailed evaluation of anomaly is therefore necessary before planning definitive correction. Imperforate hymen, although is not commonly associated with ARM should always be kept in mind as a differential diagnosis and other vaginal anomalies like Mayer--Rokitansky--Küster--Hauser syndrome should be ruled out before definitive surgical correction. [ABSTRACT FROM AUTHOR]

We report a case of urethral duplication with high anorectal malformation (ARM). A 12-year-old boy who had undergone transverse colostomy on day 2 of life and posterior sagittal anorectoplasty (PSARP) for ARM (rectobulbar fistula) at 8 years and also had urinary dribbling from a midscrotal opening in addition to normal voiding, presented with recurrent urinary tract infections and dysuria. Midscrotal dribbling had continued postoperatively. Colostomy had been closed 3 months after PSARP. Investigations revealed a large urethral diverticulum at the site of rectourethral fistula due to a segment of the gut being left behind during fistula closure. In addition, he had a sagittal urethral duplication originating at bulbar urethra and opening externally at midscrotum. The highlight of this report is successful endoscopic management of both the urethral duplication and the diverticulum. [ABSTRACT FROM AUTHOR]

Although pancreatic and gastric heterotopias are common findings in the gastrointestinal tract, heterotopic respiratory mucosa (HRM) in the rectum is extremely rare and has only been reported twice previously. We are presenting, to our knowledge, the third case of HRM in the rectum. A 56-year-old man with a history of chronic diarrhea presented for diagnostic colonoscopy, where he was found to have a rectal subepithelial nodule. He was subsequently referred to a tertiary medical center for further evaluation with rectal endoscopic ultrasound. Endoscopically, the nodule was hypoechoic, 2 to 3 mm in size, located in the submucosa, and did not appear to invade the muscularis propria. An uncomplicated endoscopic submucosal resection was subsequently performed. Microscopically, the nodule showed a multicystic complex lesion located in the submucosa, lined by ciliated pseudostratified columnar epithelium and surrounded by thin to moderately thick smooth muscle bundles and multiple lobules of seromucinous glands. There was associated acute and chronic inflammation. The rectum overlying the subepithelial lesion was lined by congested and edematous colonic mucosa and demonstrated no connection with the underlying cystic lesion. Immunohistochemical stains showed positive p63 basal cell staining in the respiratory epithelium of the lesion, while CDX2, TTF-1, and estrogen receptors were all negative. HRM is a benign nonneoplastic lesion with unclear etiology. Pathologists and gastroenterologists should be aware of this entity and consider it in their differential diagnosis for a subepithelial nodule in the rectum, keeping in mind that neoplastic processes can also develop in this location. [ABSTRACT FROM AUTHOR]