Imperforate Anus Research Articles

Diphallia with associated congenital malformations

BACKGROUND: Diphallia is an extremely rare congenital pathology in which children are born with a double penis. Diphallia is usually accompanied by systemic abnormalities, and their degree varies from no associated abnormalities to multiple abnormalities, including genitourinary, gastrointestinal, and musculoskeletal ones. Mortality among infants born with diphallia is higher due to infectious diseases accompanying developmental anomalies. CLINICAL CASE DESCRIPTION: The presented case is an example of diphallia with associated developmental malformations and the staged surgical treatment which resulted in good outcomes. DISCUSSION: Diphallia is treated surgically, but in each case, it is necessary to take into account the concomitant pathology. The main cause of death in children with diphallia are concomitant severe developmental defects, so the treatment is carried out in stages. In this case, because of the anorectal defect, intestinal obstruction was eliminated first by forming a sigmoid colostomy. Subsequently, after the child’s condition improved and he developed further, a staged surgical treatment was performed: removal of a lipoma in the cauda equina, simultaneous surgery (removal of the penis, elimination of the rectovesical fistula, lowering the rectum and forming the anus, elimination of vesicoureteral reflux, plastic surgery of the anterior abdominal wall), osteotomy with reduction of the right femur head and fixation with an L-shaped plate. The next stage of surgical treatment is the correction of the curvature of the spine and pelvic bones. CONCLUSION: In case of diphallia associated with other pathologies of organs and systems, especially of anorectal malformations, the initial formation of a double-barreled sigmostostomy helps to stabilize the child’s condition and to postpone radical surgery to the time more convenient for the patient and the doctor.

Medical Alert Cards for patients with an anorectal malformation: a useful tool to increase awareness.

Anorectal malformations (ARMs) are rare congenital anomalies causing altered anatomy and frequent hospitalizations. Parental awareness and clear communication are crucial in family-centered care. This study assesses the impact of patient-held ARM Medical Alert Cards on healthcare providers and caregivers. Caregivers of children with ARMs, including cloacal anomalies, received Medical Alert Cards with (1) contact details; (2) disease-related information; and (3) patient-specific data. A survey evaluated these cards' effectiveness in enhancing ARM awareness among healthcare professionals and caregivers. Among 33 respondents, 29 (88%) found the cards helpful in increasing ARM awareness. Both caregivers and providers found the content coherent, with a median readability score of 9 (IQR 8-10). The card layout was well-received by 30 out of 32 respondents (94%), and all noted the clarity of the contact details, facilitating patient referrals to specialized care. Most caregivers (13 out of 14, 93%) expressed willingness to use these cards in medical settings. Suggestions for improvement included adding more patient-specific information, enhancing readability, and providing visual anatomy diagram. Medical Alert Cards for patients with ARMs can enhance understanding of ARMs among caregivers and healthcare providers, serving as a vital tool in patient-centered management strategies tailored to individual needs.

The PPP - Perineal Body Preserving PSARP (Posterior Sagittal Anorectoplasty) for anorectal malformation with rectovestibular fistula in females - report of early outcomes.

Introduction The perineal body preserving PSARP (posterior sagittal anorectoplasty) (PPP) is a novel modification of the original PSARP for female patients with rectovestibular fistulas designed to eliminate the risk of perineal body dehiscence. This study aims to examine the outcomes following PPP. Methods A retrospective, single institution study was performed examining female patients with rectovestibular fistula who underwent PPP between 1/2020-12/2023. Exposure was done through the intended anoplasty only, there was no perineal body or posterior sagittal incision utilized. No routine post-operative dilations were performed. Post-operative outcomes, day of discharge, time to first feeding, and early stooling patterns were assessed. Results Fifteen patients underwent a PPP at a median age of six months (range 2 days-19 months) and median follow up of eleven months (range 1-36). Three (20%) patients underwent repair within the first three months of life. Five (33%) had diverting ostomies prior to referral to our team. There was no incidence of dehiscence or rectal prolapse. Two (13%) patients developed an anal stricture which required revision. Fourteen (93%) patients resumed normal feeds on post operative day one. Eleven (73%) were discharged on post operative day one. All patients were stooling spontaneously at their most recent clinical encounter with eleven (73%) utilizing laxatives. Conclusions The PPP eliminates the risk of perineal body dehiscence and has a quick return to regular diet and home. There is a 13% stricture rate which could relate to a difference in the distal rectal mobilization compared to the traditional PSARP.

Meta-analysis of Genetic Factors Responsible for Syndromic Anorectal Malformations in Children

Aim Anorectal malformation (ARM) is a congenital malformation that affects the anus or rectum area in children with an average age of 4 years. Different types of ARM are observed in males and females, each with distinct characteristics. The malformation is generally detected in the postnatal period, with a mortality rate between 3% and 16%. The study is conducted to understand the clinical significance of the mutation in the genes that are responsible for enhanced incidences of syndromes and their direct correlation with ARM. Methods Meta-analysis was performed using MedCalc statistical software, and we explored a strong association between ARM and syndromes using an odds ratio with a 95% confidence interval (CI). Results In total, 18 syndromes were found with the clinical characteristics of ARM. A maximum number of cases were reported about Currarino syndrome, with the minimum and maximum association of 30% and 100%, respectively. The results obtained for the pooled studies were I2 = 92.85%, P value ≤ .0001, and CI = 91.36-94.08. Conclusion A strong association has been identified between ARM and various congenital syndromes, highlighting the fact that genetic factors play a significant role in its occurrence. This association can help in the early diagnosis of the anomaly so that proper management can be presented at the earliest.

LAPAROSCOPIC CECOSTOMY PLACEMENT FOR ANTEGRADE ENEMA ACCESS IN THE PEDIATRIC POPULATION

AimUse of the appendix for an antegrade continence enema (ACE) is not always possible. Various methods exist for creating cecostomy tubes, including percutaneous, endoscopic, or surgical placement. We describe our laparoscopic cecostomy technique and review short- and long-term outcomes. MethodsSingle institution retrospective review of children who underwent laparoscopic cecostomy placement from June 2016 – June 2023. The cecum is secured to the abdominal wall with trans-fascial sutures and placement of an enterostomy button under direct vision. Half-volume flushes begin after 48 hours; after two weeks, patients transition to full flushes. Demographic, intraoperative, and postoperative variables were analyzed. ResultsForty patients were included [24 (60%) female; 31 (77.5%) Caucasian]. Twenty-one (52.5%) had myelomeningocele, 15 (37.5%) had an anorectal malformation and 4 (10%) had functional constipation. Twenty-five (62.5%) underwent laparoscopic cecostomy placement alone, while 15 (37.5%) had it performed with another procedure.Median operative time was 1.12 (IQR 0:93-1.45) hours for isolated cecostomy placement, with median post-operative stay of 2.0 days (2.2 - 3.1) days. Post-operatively, one patient had severe withholding, ultimately requiring a diverting ileostomy. No other 30-day complications (surgical site infection, tube removal) were identified. One patient required revision four months post-op due to inadvertent placement in the sigmoid. At one-year follow-up, 11/36 (30.6%) children noted granulation tissue, and 11 (30.6%) noted superficial leakage. Two (6%) patients had transitioned to oral laxatives. ConclusionLaparoscopic cecostomy tube placement is a safe and alternative method of developing ACE access that can be done concurrently with other procedures.

Postoperative continuation of antibiotic prophylaxis beyond 24 h is unnecessary for abdominal surgeries in children and neonates

PurposeDespite recent WHO recommendations, antibiotic prophylaxis is routinely continued for several days after surgery. We conducted a retrospective study to evaluate the safety and efficacy of antibiotic prophylaxis cessation within 24 h after abdominal surgeries MethodsWe retrospectively reviewed the charts of patients who underwent 3 representative gastrointestinal surgeries (1; transumbilical small bowel procedure for intestinal atresia or stenosis, 2; extrahepatic bile duct resection, and hepaticojejunostomy for congenital biliary dilatation, 3; stoma closure for intestinal perforation, anorectal malformation, or inflammatory bowel disease) at our institution between 2011 and 2023. The demographic data collected included age, preoperative hemoglobin level, albumin level, body mass index, and ASA class. Intraoperative and postoperative data were also collected. The patients were divided into 2 groups according to the duration of perioperative antibiotics (‘within 24 h’ and ‘beyond 24 h’), and the incidence of superficial incisional SSI (siSSI) was compared between the 2 groups in each procedure ResultsDuring the study period, 25 cases underwent transumbilical small bowel procedure, 23 cases underwent hepaticojejunostomy, and 45 cases underwent stoma closure. The preoperative care bundle including bowel preparation, cleansing, and operative procedures has not changed during the study period. First- and third-generation cephalosporins were administered to all the patients. All patients received their first single dose of antibiotics within 60 min before the skin incision. In particular, 14 patients who underwent small-bowel procedures received only a single dose. The SSI rates for small bowel procedures, hepaticojejunostomy, and stoma closure were 0%, 4.4%, and 4.4%, respectively. No SSIs were observed in any of the procedures in the 'within 24 h' group ConclusionBased on our study, we conclude that ≤24 h of postoperative continuation of antibiotic prophylaxis is sufficient for siSSI prevention in hepaticojejunostomy and stoma closure. Additionally, single-dose antibiotic prophylaxis is adequate for neonatal small bowel procedures.

060. Chronic Megacolon with Recto Vaginal Fistulo Associated with Atresia Ani: A 26-Year History and Case Report

Background: Large bowel obstruction and megacolon formation with rectovaginal fistule ascociated with atresia ani is rare. abnormal colon dilatation that falls into one of three categories: toxic, chronic, or acute. Acute megacolon, sometimes called Ogilvie syndrome, is a condition that frequently affects sick or postoperative individuals and is linked to autonomic nervous system dysfunction. Its source is unknown. On the other hand, intestinal dysfunction brought on by neurological or muscle conditions is the cause of chronic megacolon. The symptoms of all three kinds include bloating, stomach pain, and constipation. Case: We present a case of an 26-year-old woman surviving large bowel obstruction and mega-megacolon formation secondary atresia ani and fistulo rectovagina for 26years alive, with an presentation of abdominal distention. Results The patient’s symptoms, laboratory test results, and imaging were consistent with large bowel obstruction. The patient underwent urgent exploratory laparotomy. Upon entry in the abdomen, it was unexpected that the extreme colonic wall thickening, indicating the longtime course of illness. In this instance, we performed a number of procedures, including ligation of the obstructed colon, anus repair, and vaginal fistula repair. Conclusion: This report aims to point out the atypical and in-extremes presentation of an otherwise common disease.

042. Colonic Duplication with Recto-Vestibular Fistula: A Case Report

Background: Anorectal malformations associated with colonic duplications are a rare condition. Colonic duplication may have different presentations according to its location and size, with an incidence of less than 15% of all gastrointestinal duplications. Colonic duplication is less common than ileal duplication, whether cystic or tubular. Duplications are considered congenital malformations because they are thought to arise from disturbances in embryonic development. This report aims to present and analyze the clinical presentation, diagnostic approach, and management of anorectal malformations associated with colonic duplications in an 11-month-old child at our hospital, highlighting the challenges and treatment strategies for such a rare case. Case: We present a case of an 11-month-old girl, referred from a network hospital with a post-colostomy at 4 months of age for recto-vestibular fistula with findings of colonic duplications at colostomy. Exploratory laparotomy was performed until the colonic duplications were identified and completely resected. Conclusion: Colonic duplication is a rare congenital disorder, and its association with anorectal abnormalities is even rarer. The surgical treatment includes resection of the duplicated colon. Surgical intervention is the treatment of choice.

007. Distal Ileal Atresia and Total Colon Agenesis with Anorectal Malformation: A Case Report

Background: Atresia of the distal ileum and colon agenesis are rare conditions and can be the cause of obstructive symptoms. Case: A 1 day old baby girl complained of no anus since birth. The patient has no bowel movements or vomiting. The patient's stomach appears increasingly bloated. On physical examination of the abdomen revealed distension accompanied by difficult to hear bowel sounds. There was no anus or fistula in the patient but there is a dimple in the anus. Another abnormality found in the patient was the presence of CTEV on both of the patient's legs. The patient was planned for exploratory laparotomy and colostomy surgery. the length of the jejunum and ileum proximal to the ligament of Treitz was found to be 50 cm. The distal ileum, caecum, colon, rectum and anus were not found. An ileostomy was performed on this patient. The patient died 13 hours after surgery. Conclusion: Total colonic agenesis is a rare case and usually occurs with other congenital abnormalities. Early diagnosis and appropriate initial treatment are very important in this case. colonic agenesis causes congenital short bowel (CSB). CSB causes malabsorption due to inadequate intestinal length, which interferes with absorption. Staged surgery is a good option in the management of total colonic agenesis.

The Analysis of the Fetal Abdominal Wall and Gastrointestinal Tract Abnormalities in a Single Tertiary Center

OBJECTIVE: We aimed to evaluate the fetuses with gastrointestinal anomalies and anterior abdominal wall defects detected in the prenatal period in our clinic and to analyze their perinatal outcomes. STUDY DESIGN: This retrospective study analyzed the data of 67 cases evaluated by the perinatology department of the Basaksehir Cam and Sakura City Hospital between January 2021 and May 2023 with a diagnosis of fetal anterior abdominal wall and gastrointestinal tract abnormalities. RESULTS: A total of 67 cases were included in our study with a mean maternal age of 26.4 ± 3.1 years and a median gestational week of first assessment at our center of 22 weeks (12-39 weeks). The most common anterior abdominal wall malformations were omphalocele (n=29, 43.3%) and gastroschisis (6 cases, 9.0%), and the most common fetal gastrointestinal tract anomalies were duodenal atresia (n=10, 14.9%), dilated bowel (n=5, 7.4%), and intestinal atresia (n=4, 5.9%). While genetic testing was carried out in 18 cases (27%), chromosomal abnormality was found in 6 (9.0%) cases. Termination of pregnancy was performed in 6 cases (9.0%). Postnatally, surgery was performed in 49 cases (73.1%) with a diagnosis of postpartum omphalocele, duodenal atresia, gastroschisis, intestinal and anal atresia. An uneventful surgical course was recorded for 47 cases that underwent surgery. CONCLUSION: The most common abdominal wall defects are omphalocele and gastroschisis, and the most frequent gastrointestinal anomalies are small bowel abnormalities (duodenal atresia, dilated bowel, intestinal atresia) in our study cohort. These abnormalities might be an isolated anomaly or part of a syndrome or associated with chromosomal abnormalities. The prognosis for infants with gastrointestinal malformation depends on the presence of associated anomalies or karyotype anomalies.

Burden and mortality of congenital gastrointestinal anomalies: insights from a nationwide cohort study.

Improved perioperative care has enhanced survival in children with congenital gastrointestinal conditions and abdominal wall defects (AWD). However, epidemiological and surgical outcomes in developing nations are still scarce. Our aim was to assess the burden and mortality of common congenital gastrointestinal anomalies and AWD in Malaysia, and their influencing factors. Using the Global PaedSurg study protocol with permission, we performed a prospective cohort study on children presenting for the first time between October 2021 and April 2022 with these conditions: Esophageal atresia (EA), congenital diaphragmatic hernia (CDH), intestinal atresia, gastroschisis, exomphalos, anorectal malformation (ARM) and Hirschsprung's disease. We compared mortality and 30-day outcome data across different geographical regions in Malaysia. There were 228 patients with 242 study conditions (EA n = 28, CDH n = 36, intestinal atresia n = 49, gastroschisis n = 12, exomphalos n = 8, ARM n = 77, Hirschsprung's disease n = 32). Our mortality rate was 8.8%; 60% of these were CDH patients. Factors significantly associated with mortality were CDH diagnosis, central venous access requirement, higher American Society of Anesthesiologists (ASA) score, blood transfusion and ventilation requirement. Diagnosis of CDH is the most important predictor for sepsis on arrival and mortality, therefore measures should be taken for early recognition and aggressive management.

Role of MRI in Preoperative and Postoperative Evaluation of Anorectal Malformation

Role of MRI in Preoperative and Postoperative Evaluation of Anorectal Malformation

ERN eUROGEN Guidelines on the Management of Anorectal Malformations Part I: Diagnostics.

Anorectal malformations (ARMs) are rare congenital anomalies that involve the anus, rectum, and oftentimes the genitourinary tract. The management of ARM patients is complex, and many controversies exist. To address this issue, the European Reference Network eUROGEN for rare and complex urogenital conditions aimed to develop comprehensive guidelines for the management of ARM. The Dutch Quality Standard for ARM served as the basis for the development of guidelines applicable on a European level. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from 7 European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected, and recommendations were formed considering current evidence and/or expert consensus. Prenatal and neonatal diagnostic workup as well as postsurgical follow-up of anorectal, genitourinary tract, and neurologic system were reviewed. Seven new studies were identified. The panel adapted 13 recommendations, adopted 7, and developed 8 de novo. The availability of high-quality evidence was limited, and most recommendations were based on retrospective studies, case series, or expert opinion. Patients with ARM and their families require highly specialized and comprehensive care from the prenatal period to adulthood. This guideline provides recommendations for a comprehensive diagnostic workup of children with ARM throughout their life that is applicable on a European level.

ERN eUROGEN Guidelines on the Management of Anorectal Malformations, Part IV: Organization of Care and Communication between Providers.

Being born with an anorectal malformation (ARM) can have profound and lifelong implications for patients and parents. Organization of care and communication between health care providers is an overlooked area of patient care. The European Reference Network eUROGEN for rare and complex urogenital conditions assembled a panel of experts to address these challenges and develop comprehensive guidelines for the management of ARM. The Dutch Quality Standard for ARM served as the basis for the development of guidelines. Literature was searched in Medline, Embase, and Cochrane. The ADAPTE method was utilized to incorporate the newest available evidence. A panel of 15 experts from seven European countries assessed currency, acceptability, and applicability of recommendations. Recommendations from the Dutch Quality Standard were adapted, adopted, or rejected and recommendations were formed considering all available evidence, expert consensus, and the European context. Aspects pertaining to organization of care, patient/parent/health care provider communication, and referral and collaboration between providers caring for ARM patients were assessed. Two new studies were identified. In total, the panel adapted 12 recommendations, adopted 7, and developed 2 de novo. The overall level of newly found evidence was considered low and most recommendations were based on expert opinion. Collaborative care and organization of care are gaining importance in the field of ARM. This guideline gives practical guidance on how to achieve better communication and collaboration between all involved parties, applicable at the European level.

Sexual function and fertility in young female adults surgically treated for anorectal malformations

PurposeThe aim was to investigate sexual function and fertility in female adults operated on for anorectal malformations (ARM).MethodsThis was a cross-sectional questionnaire-based study including female adult patients treated for ARM at our institution between 1994 and 2003. Sexual function in females was assessed using the Profile of Sexual Function (PFSF). Additional questions regarding fertility were answered by the participants. Patient characteristics were retrospectively retrieved from the medical records and descriptive statistics were used for analysis. Sexual function outcomes were compared to a control group from a previously published group of females. Composite outcome analysis was performed using previously published data to determine the potential impact of bowel function and health-related quality of life on sexual function. The ethics review authorities approved the study.ResultsA total of 14 of 30 (46.7%) females responded to the questionnaires and had a mean age of 21.1 years (range 18–26). No association was found between PFSF and age or bowel function (Bowel Function Score), however, a strong correlation was found between PFSF and health-related quality of life (HRQoL) with a Spearman correlation of ρ 0.82 (p = 0.0011). The general satisfaction question was strongly associated with their total PFSF score (ρ = 0.71, p = 0.0092). Except for the “desire” item, the females in this cohort did not have significantly worse sexual function than the control population(p = 0.015). Ten of fourteen (71.4%) females had had their sexual debut at a mean age of 16.3 years and two of these women (20%) have been pregnant. All females had had menarche at a mean age of 12.7 years.ConclusionSexual function in adult females was comparable to healthy controls except for the “desire” item where the cohort reported poorer outcomes. The cohort’s sexual function had a direct association with their reported HRQoL where individuals with worse HRQoL also reported poorer sexual function.Level of evidenceIII

Neurodevelopmental outcomes among children with congenital gastrointestinal anomalies using Korean National Health Insurance claims data.

This study investigated neurodevelopment and risk factors in children surgically treated for congenital gastrointestinal anomalies (CGIA), excluding those with known high-risk factors such as low birth weight or chromosomal anomalies. Data of children born between 2008 and 2015 who underwent surgical treatment for CGIA were retrieved from the Korean National Health Insurance Database. CGIA included esophageal atresia, duodenal atresia, jejunoileal atresia, anorectal malformations, and congenital megacolon. Neurodevelopmental impairment (NDI) was defined as Korean Ages and Stages Questionnaire scores below the determined cut-off or Korean Developmental Screening Test scores < 2 standard deviations at 3 years of age. Children with CGIA had a significantly higher risk of NDI than controls (6.2% vs. 2.7%, p < 0.001). Growth failure was correlated with NDI. Longer durations of oxygen support (adjusted odds ratio [aOR], 1.037; 95% confidence interval [CI], 1.013-1.063), mechanical ventilation (aOR, 1.053; 95% CI, 1.018-1.089), and number of surgeries (aOR, 1.137; 95% CI, 1.016-1.273) were significantly associated with NDI. These findings emphasize that cautious yet proactive neurodevelopmental monitoring is crucial in affected children, ensuring timely intervention and that excessive concern among families is unnecessary.

X-Linked Intellectual Disability with NEXMIF Gene Mutation and Developmental Delay with GNAO1 Gene Mutation: Case Report

X-linked intellectual disability (XLID) is a genetically heterogeneous disorder. Currently, 162 genes linked to XLID have been found, but the cause of XLID is still unclear. While the GNAO1 gene is crucial for hypotonia, epilepsy, developmental delay, and movement disorders, the NEXMIF gene, also known as KIAA2022, has associations with XLID, autism, and epilepsy. The subject of the study, a 5-year-old girl has lots of congenital defects, including a cleft palate, anal atresia, hypotonia in her lower limbs, and thumb missing. A variety of eye abnormalities, such as scoliosis, finger malformations, and craniofacial dysmorphism. Radiological tests revealed substantial heart problems, bilateral renal hypoplasia, and brain abnormalities. She met milestones more later than her contemporaries, indicating clear developmental deficits. The NEXMIF and GNAO1 genes both include heterozygous frameshift variants that were discovered through genetic research using next-generation sequencing. The complex and varied clinical signs of XLID are shown in this case. The clinical picture is further complicated by the co-occurrence of mutations in the NEXMIF and GNAO1 genes, which emphasizes the need for an approach to offer suitable therapy solutions. Future studies are necessary to understand the complex interactions between these genes and how they affect XLID and related symptoms.

Mullerian and vaginal anomalies.

To provide a comprehensive resource for pediatric surgeons and pediatricians caring for patients with Mullerian and vaginal anomalies, with a focus on recent changes in practice. Herein, we provide a foundation for understanding the anatomy, presentation, and work up of Mullerian and vaginal anomalies. We review how to approach management of these anomalies when in isolation or when associated with anorectal malformations. We discuss the evolution of the management of neovaginal discussion with a current emphasis on patient-centered and directed care. Review of current understanding of and approach to Mullerian and vaginal anomalies in children.

New ultrasound features in diagnosing fetal anal atresia: a multicenter prospective cohort study

This research aimed to assess the validity of ultrasound scans with new features in detecting fetal anal atresia and verify the effectiveness of these new features. Additionally, we aimed at investigating the perinatal incidence of anal atresia. This multicenter prospective study recruited 94,617 normal gravidas and 84 gravidas with anal atresia fetuses. The gold standard for diagnosing perinatal anal atresia is routine neonatal anus examinations. The incidence calculation was based on the results of the gold standard. The validity of our new approach was evaluated via a diagnostic test involving all 94,701 subjects. The effectiveness of our new features was assessed through an ablation study in a randomly established new dataset, with the ratio of anal atresia to non-anal atresia cases of 1:4. The annual perinatal incidence of anal atresia between 2019 and 2023 ranges from 0.57‰ to 1.29‰. Our new method performed great regarding the Youden index, diagnostic odds ratio (DOR), area under the curve (AUC) of the receiver operating characteristic curve (ROCC), AUC of the precision-recall curve (PRC), F1-score, and Cramer’s V. In the ablation study, our new approach surpassed its competitors concerning Youden index, DOR, AUC of the ROCC, and AUC of the PRC. Ultrasound scans show high validity and clinical value in detecting fetal anal atresia. Our new ultrasound features significantly promote the detection of fetal anal atresia.

Suprapubic vesicostomy buttons: Indications, complications and bladder outcomes

IntroductionVesicostomy button drainage is a recognised alternative to clean intermittent catheterization (CIC) in children with urethral obstruction, sensate urethra or neurological/ behavioural issues. AimTo report the indications, complications and long-term bladder functional outcomes in a 15-year cohort of patients with button vesicostomy. Materials and MethodsAMT Mini one gastrostomy buttonR was inserted via a surgical vesicostomy, or percutaneously under cystoscopic guidance. Retrospective data included demographics, indications, complications, and long-term bladder capacity/ emptying pre-post-button removal. Results29 children (23 males) underwent vesicostomy button insertion at 3.5 (0.5-14.5) years. Diagnosis was neurogenic (11), bladder outlet obstruction (9), cloaca/urogenital sinus (3), anorectal malformation (ARM) (2), other bladder dysfunction (3) and diversion (1). There were no short-term complications. UTI occurred in 31%, leakage in 28% and blockage in 7%. At a median of 10 (2-18) years, 14 (48%) are still on button drainage; 6 (21%) progressed to Mitrofanoff catheterisation. In 9 (31%) who no longer require the button, all children were able to void urethrally, with good emptying, at 4.5 (1- 7) years follow-up. DiscussionContinent vesicostomy allows toilet-training and improved quality of life. We estimated that the cost of a button vesicostomy and tubing approximates £1502 per year. 5-6 Speedicaths per day (costing £38 per pack) cost £2772 per year. Asymptomatic bacterial colonisation does not require antibiotic treatment; it is best avoided by changing the button every 12 weeks. Symptomatic febrile UTI’s are commonly secondary to the underlying pathology; we recommend changing the button half-way through the antibiotic treatment course. Leakage was managed by increasing the water in the balloon. Button blockage, commonly due to balloon encrustation, is preventable by regular button changes.Button drainage may be temporary (until bladder dysfunction resolves, or changed to a Mitrofanoff), or a long-term (in life-long neuro-developmental/ behavioural issues). The button was no longer required in 9: bladder function improved post spinal cord un-tethering in 3; 2 PUV; 2 ARM; 1 myopathy and 1 diversion. Of note, the button did not appear to affect bladder dynamics with sustained resolution of bladder dysfunction in 31%.Our main limitation was diversity of pathologies, making comparison of urodynamics more challenging: a larger study with more numbers in each patient group would be the next step. ConclusionSuprapubic buttons are a safe second-line bladder drainage option in patients who are unable to CIC. The technique may be a temporary solution where bladder dysfunction may resolve, or until the child is ready for catheterisation via a Mitrofanoff.