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In patients with anorectal malformations (ARM), the sacral ratio (SR) serves as an established predictive marker for functional prognosis and is derived from conventional radiographs. More recently, magnetic resonance imaging (MRI) has emerged as a diagnostic alternative for preoperative/baseline assessment of patients with ARM. With this study we aimed to evaluate if the SR could be reliable be measured from MRI images and if it correlated to SR measurements obtained from radiographs.Two raters analyzed MRI data and conventional radiographs from thirty-one subjects (n = 17 with ARM; n = 14 controls). We calculated intra-class correlation coefficients (ICCs) to test inter-rater reliability and applied paired t-tests to examine if SR parameters from MRI were comparable with those from pelvic radiograph. We further computed Pearson's correlation coefficients to test the linear relationship between SR calculated from MRI and conventional radiographs.The ARM and control groups did not statistically differ in their age and weight on the day of the MRI scan. Reliability analysis revealed an excellent inter-rater agreement for SR from radiograph parameters with an intraclass correlation coefficient (ICC) of 0.94, respectively of 0.86 for MRI (frontal plane), and of 0.84 for MRI (sagittal plane). The correlation coefficient between SR calculated from the sagittal MRI and SR calculated from radiograph images was significant and high (r = 0.80, P 0.001). The SRs from MRI images did not significantly differ from SRs from radiographs, but were also not statistically equivalent.Our results demonstrate that the SR can be derived from MRI images with good inter-rater reliability. The SR value is marginal higher when calculated on MRI, presumably due to inclusion of cartilaginous yet unossified structures.

Many patients with anorectal malformations (ARM) need a bowel management program (BMP) to manage lifelong problems of fecal incontinence or severe constipation. We aimed to evaluate the sustainability of the results in such a program.A single-institution retrospective review was performed in children with ARM who attended our BMP (2015-2019). Standardized definitions and validated tools were used to assess fecal continence (Baylor Continence Scale), constipation (Cleveland Constipation Scoring System), urinary symptoms (Vancouver Symptoms Score), and the Pediatric Quality of Life (PedsQL) and health-related quality of life (HRQOL) at the start of BMP and 1-year after completion of the program.222 patients with ARM at a median age of 6.7 (IQR, 4.9-10.1) years were identified. All (100%) soiled at intake with 149 (67.1%) patients being treated with rectal or antegrade enemas and 73 (32.9%) with oral laxatives. At 1 year 150 (70.4%) were clean, 72.7% were on enemas and 27.3% were on laxatives (p = 0.08). 109 out of 148 (73.6%) patients were clean on enemas. A further 41 out of 66 (62.1%) patients were continent on laxatives with voluntary bowel movements and clean. In the group that was clean, there was improvement in Baylor Continence Scale (25 vs. 13.0, p 0.000000002), Vancouver (11 vs. 6, p = 0.0110) scores, and clinically relevant improvement in the total PedsQL HRQL (78-85) and the PedsQL HRQL physical function (86-92) and psychosocial domain (77-82). There was no improvement in Cleveland (10 vs. 9, p = 0.31) score.An intensive BMP offers significant benefits in the treatment of fecal incontinence in ARM. It appears to also improve urinary incontinence and urinary voiding as well as the patient's quality of life. These changes are sustainable over at least one year.

Aim of the Study : For the past four decades, routine daily postoperative anal dilation by parents has been the standard treatment following a primary posterior sagittal anorectoplasty (PSARP). However, the clinical benefit of this practice has never been formally investigated. It is known that dilations can have a significant negative psychological impact on patients and families, and therefore, we aimed to study if routine dilations after a PSARP are necessary. Methods : A prospective, single institution randomized controlled clinical trial was conducted on patients with anorectal malformations (ARM) at our institution between 2017 and 2019. Patients were randomized to either a dilation or non-dilation group following their PSARP. Inclusion criteria included age less than 24 months and all patients undergoing primary repair of their ARM (except for cloaca). Patient characteristics, type of ARM, presence of colostomy, postoperative stricture, need for a skin level revision (Heineke-Mikulicz anoplasty (HMA)), and need for redo PSARP were recorded. The primary outcome of the trial was stricture formation. The secondary outcome included strictures requiring interventions. A p-value of less than 0.05 was considered statistically significant. Institutional approval was obtained for this study and informed consents were obtained from all the patients. Results : 49 patients were included in our study. 5 (21%) in the dilation group and 8 (32%) in the non-dilation group developed strictures (p=0.21). Of these, 3 (13%) patients in the dilation group required HMA, and 4 (16%) patients in the non-dilation group required HMA (p=0.72). 4 patients required a redo operation for strictures: 2 in the dilation arm (these patients despite the plan to do dilations, chose not to do them consistently) and 2 in the non-dilation arm (p=0.59). Conclusion : Routine dilations after PSARP do not significantly reduce stricture formation. Based on these results, non-dilation is a viable alternative, and HM anoplasty remains a good back-up plan if a stricture develops. Level of Evidence : Level I

Introduction For the classification of the complexity of cloacal malformations and the decision on the operative approach, an exact anatomical assessment is mandatory. To benefit from using three-dimensional (3D)-printed models in preoperative planning and training, the practicability of these models should be guaranteed. The aim of this study was to evaluate the quality and feasibility of a real-size 3D-printed cloaca model for the purpose of cysto-vaginoscopic evaluation. Materials and Methods We performed a 3D reconstruction and printed a real-size, rubber-like 3D model of an infant pelvis with a cloacal malformation and asked invited pediatric surgeons and pediatric urologists to perform a cysto-vaginoscopy on the model and to complete a brief questionnaire to rate the quality and feasibility of the model and to indicate whether they would recommend the model for preoperative planning and training. Results Overall, 41 participants rated the model quality as good to very good (M = 3.28, standard deviation [SD] = 0.50, on a scale from 1 to 4). The model was rated as feasible for preoperative training (M = 4.10, SD = 0.75, on a scale from 1 to 5) and most participants (85.4%) would recommend the model for preoperative training. The cysto-vaginoscopy of the model was considered as a valid training tool for real-life cases and improved the confidence on the anatomy of a cloaca. Conclusion The results of our study indicate that patient-specific 3D-printed models might be a useful tool in the preoperative evaluation of complex anorectal malformations by simulation of cysto-vaginoscopy with an excellent view on anatomical structures to assess the whole spectrum of the individual cloacal malformation. Our model might be a valuable add-on tool for specialty training in pediatric colorectal surgery.

In patients with Hirschsprung disease (HSCR), soiling may be related to anal sphincter damage following the initial pull-through. No optimal treatment has been developed for such patients, although enemas (rectal or antegrade) have been applied with some success. We present the one-year outcomes of a new technique for anal sphincter reconstruction.All patients with HSCR referred from other institutions for post pull-through soiling were studied. Seven patients with patulous sphincters underwent sphincter reconstruction. Six had a full preoperative evaluation and were included in the study. Their 12-month outcomes were assessed.All six patients had soiling without voluntary bowel movements (VBMs). One patient was clean on Malone flushes when referred. Three underwent pre- and post-reconstruction non-sedated three-dimensional anorectal manometry, and objectively were able to close their sphincters following the reconstruction. All patients without Down syndrome (4 of 6) showed improvement in the abbreviated Baylor Continence Scale (4.5 vs. 0.75). One patient has achieved total bowel control without antegrade flushes, three now have VBMs which they did not have before but have occasional accidents and use antegrade flushes intermittently. They reported higher productivity, the ability to participate in sports and be away from home with confidence in their regimen. Two of 6 patients have Down syndrome and required a redo pull-through for other indications and underwent empiric sphincter reconstruction. For these two patients we do not have an outcomes assessment.A new technique for sphincter reconstruction shows promising results in improvement of bowel control at one year.IV.

Introduction: Training in laparoscopic surgery seems to be an important aspect in gaining and maintaining professional competency. In experimental settings, camera navigation skills improved after simulation-based training, but the effect of camera work on the surgeon's performance is not well studied. The aim of this study was to investigate whether a fixed camera or an operated camera, as well as the experience of the camera operator has an effect on the performance of the surgeon. Materials and Methods: The study was performed on the LapSim laparoscopic training system. The task was to tie an intracorporal knot in a static surgical environment with three different camera conditions: fixed camera, camera operated by an inexperienced person (inexpert camera), and camera operated by an experienced surgeon (expert camera). The camera conditions were counterbalanced across trials. Performance variables were completion time in seconds and the extend of movements in path length and angular pathway. Gaze behavior was measured with eye-tracking glasses worn by the surgeon as well as the camera operator and was evaluated for performance-harming effects. Results: Completion time varied across conditions, with participants performing significantly longer in the fixed camera condition than in the expert or the inexpert condition. The expert and inexpert conditions did not differ. The performance-harming effect of non-focusing on the tissue was especially visible in the fixed camera condition but disappeared in the expert camera condition. Neither the camera operators' gaze behavior nor the surgeon-camera operator fixation agreement predicted task performance. Discussion: A camera operator can potentially eliminate performance-harming effects of maladaptive gaze behavior and promote optimal visual behavior of a surgeon. In our experimental task, there was no significant difference in whether the camera operator had previous training in laparoscopic surgery or not.

Background: The correction of an anorectal malformation (ARM) is highly complex and relatively infrequent. Simulation training and subsequent assessment of its effectiveness may result in better clinical outcomes. Assessment can be done using a CAT to assess surgical skills of a specific procedure. This study aims to develop and validate a competency assessment tool (CAT) for the posterior sagittal anorectoplasty (PSARP) for the repair of anorectal malformation. Methods: The CAT-PSARP was developed after consultation with experts in the field. The PSARP was divided into five steps, while general skills (tissue and instrument handling) were scored separately. During several pediatric colorectal hands-on courses in 2019 and 2020 participants were asked to voluntarily 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. Intra-class correlation coefficient analysis demonstrated a fair inter-observer agreement for ‘total score of general skills’ (ICC =0.524, pConclusion: Good inter-observer agreement was found for the total scores of the CAT-PSARP. It is a suitable objective assessment tool for the overall performance of the included steps of the PSARP for repair of an ARM.

Purpose A complication of the surgical management of anorectal malformations (ARMs) is a retained remnant of the original fistula (ROOF) formerly called a posterior urethral diverticulum (PUD). A ROOF may have multiple presentations or may be incidentally discovered during the work-up of ARM after previous surgery. We sought to define the entity and the surgical indications for excision of a ROOF when found. Methods We performed a retrospective cohort study of all male patients who presented to our center following previous repair for ARM at another institution, who came for evaluation of problems with urinary and/or fecal continence, from 2014 to 2017. Charts were reviewed for symptoms, original type of malformation, preoperative imaging, treatment, and postoperative follow-up. Results Of 180 referred male patients, 16 had a ROOF. 14 underwent surgical repair to address this and for other redo indications, and 2 did not require intervention. 13 patients had an additional reason for a redo such as anal mislocation or rectal prolapse. Indications for ROOF excision were urinary symptoms (e.g. UTI, dribbling, passage of mucous via urethra, stone formation), to make a smoother posterior urethra for intermittent catheterization, or for prophylactic reasons. Patients were repaired at an average age of 4.2 years, using a PSARP only approach with excision of the ROOF for all except one patient who needed a laparotomy due to abdominal extension of the ROOF. No patient needed a colostomy. The original ARM repairs of the patients were PSARP (9), laparoscopic assisted (4) and abdominoperineal pullthrough (3). Preoperative evaluation included pelvic MRI, VCUG, and cystoscopy. The ROOF was visualized on 14 of 16 MRIs, 10 of 14 VCUGs, and 14 of 15 cystoscopies. Urinary symptoms associated with a ROOF and ease of catheterization were improved in all repaired cases. Conclusion Patients not doing well from a urinary or bowel standpoint post ARM pull-through need a complete evaluation which should include a check for a ROOF. Both modalities MRI and cystoscopy are needed as a ROOF can be missed on either alone. A VCUG was not reliable in identifying a ROOF. Excision is needed in patients to improve urinary symptoms associated with these lesions and to minimize the small but theoretical oncologic risk present in a ROOF. Level of evidence Level III.

Background Despite variability at presentation, presacral masses in patients with and without anorectal malformations (ARM) appear histologically similar. The purpose of this study was to identify differences in oncologic outcomes between these two groups. Methods A retrospective review was performed utilizing our institutional cancer and colorectal and pelvic reconstruction databases for patients with presacral masses and sacrococcygeal teratomas between 1990 and 2017. Data captured included age at surgical resection, type of ARM, tumor location within the pelvis, tumor histopathology, tumor size, adjuvant chemotherapy, recurrence, and follow-up. Results Forty-six patients comprised our cohort, of whom 12 had an ARM. The median age was older at resection for those with an ARM (1.4 years; range 1 day to 29.4 years) compared to those without an ARM (9 days; range 0 days to 6.9 years) (p = 0.01). The mean tumor size was 2.5 cm in patients with an ARM compared to 6.0 cm in patients without an ARM (p = 0.036). All patients with ARM had exclusively intrapelvic tumors, and histopathology included mature teratoma (8), yolk sac tumor (1), lipoma (1), and unknown (2). Tumor location for patients with sacral and presacral masses without ARM included exclusively extrapelvic (10), primarily extrapelvic with large intrapelvic component (7), primarily intrapelvic with extrapelvic component (1), exclusively intrapelvic (8), and unknown (8). Histopathology for patients with presacral masses without ARM included mature teratoma (20), immature teratoma (7), yolk sac tumor (3), ganglioneuroma (1), neuroblastoma (1), benign epithelial cyst (1), and unknown (1). Tumor recurrence rate was similar between patients with ARM (n = 3, 25%) and those without an ARM (n = 5, 15%) (p = 0.41). The 5-year event free survival was 65% (95% CI: 25%–87%) in the group with ARM and 81% (95% CI: 60%–92%) in the group without ARM (p = 0.44). Conclusion Sacral and presacral masses in patients with ARM are resected at a later age and are more likely to be intrapelvic. They appear histologically similar and have similar rates of recurrence and malignancy when compared to patients without ARM. Level of Evidence III Type of Study Retrospective comparative study.

Introduction Acquired skin-level strictures following posterior sagittal anorectoplasty (PSARP) and some rare cases of congenital anal stenosis can be managed using a Heineke–Mikulicz like anoplasty (HMA). We hypothesized that this procedure was an effective, safe, and durable outpatient procedure in select patients. Methods We retrospectively reviewed all patients who underwent HMA for skin level strictures following PSARP or for certain congenital anal stenoses from 2014 to 2017. Results Twenty-eight patients (19 males, 9 females) with a mean age of 5.8 years (range 0.5–24.4) underwent HMA. Twenty-six had a prior PSARP, of which 18 were redo, and 8 were primary procedures. Two patients had congenital skin level anal stenosis. The mean follow up was 1.0 years (range 0.4–2.9). The average preprocedure anal size was Hegar 8, which after HMA increased 8 Hegar sizes to 16 (95% CI 7–9, p Conclusion HMA is a safe procedure for skin-level anal strictures following PSARP (primary and redo) and can also be used in some rare cases of congenital anal stenosis. Long-term follow up to determine the restricture rate is ongoing. A plan to do an HMA if a stricture develops may offer an alternative to routine anal dilations, particularly after a redo PSARP in an older child. Type of study Case series. Level of evidence Level IV.

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.

Anorectal malformations (ARM) affect 1 in 5000 live births. The majority of ARM can be repaired through a posterior sagittal incision as the rectum is reachable from below. However there are some types of anorectal malformations which need an abdominal approach. This abdominal approach is ideally performed with minimal invasive surgery. In this chapter we highlight the indications and the surgical technique to perform laparoscopic assisted anorectal pull through.

Surgical treatment of Hirschsprung disease (HD) evolved in the last decades with the introduction of new innovative minimally invasive techniques. Nowadays minimal access can be performed with two different approaches, total transanal endorectal pull-through and laparoscopic-assisted transanal pull-through. In this chapter we emphasize the indications and surgical technique for laparoscopic-assisted approach.

Introduction Short-term international medical service trips (MSTs) provide specialized care in resource-constrained countries. There are limited data on immediate and long-term reported outcomes following specialty MST. We hypothesized that dedicated collaborative MST team and host institution produce outcomes and results comparable to those of high-income settings. Our primary aim was to analyze the long-term surgical and functional outcomes of our specialty-specific MSTs following five years of annual MST in Honduras. Methods We performed a single-institution retrospective analysis of 56 children who underwent colorectal and pelvic reconstructive operations between 2014 and 2018. Demographics, diagnosis, comorbidities, type of repair, long-term complications, and functional bowel and bladder results were recorded. Results We included a total of 56 children, 47 with ARM and 9 with HD, with a median age of 43.5 months (17–355) at the time of surgery. 25% (22) of the patients were lost to follow-up. Fecal continence was achieved by 23 (60%) patients 5 years (n = 11). Complications included constipation in 18 (42.9%) children with ARM and in 1 (12.5%) with HD. Eleven (19.6%) patients required revisional surgery for skin level anal stricture. Seventy-five percent of the patients with pediatric colorectal disorders attending the MST were compliant with continued long-term follow-up. Conclusion We were able to demonstrate that with organized, dedicated site and surgeon, results achieved can be comparable to those in the high-income countries (HICs). We conclude that this type of specialized care is feasible and beneficial for affected pediatric colorectal patients in resource-limited settings, when a strong partnership with a system of preoperative assessments and peri- and postoperative care can be established. Level of evidence Level IV (retrospective cohort study).

Introduction: Short-term international medical service trips (MST) are established means to access specialized medical aid in resource-limited areas. The field of pediatric colorectal surgery is a subspecialty in pediatric surgery that mainly treats anorectal malformations (ARM) and Hirschsprung disease (HD). This study aimed to investigate the impact of MST on the donors’ perception of competency concomitantly to the impact on patients in the donors’ home country. We also wanted to investigate whether the donors’ pre-existing experience in the field of ARM and HD affects the experience they gain during the MST, and the subjective perception in treating patients in their base country. Methods: We created a questionnaire for the international medical staff participating in MSTs on the unique topic of pediatric colorectal diseases. The questionnaire was split into three parts: essential experience (1) in the field of colorectal surgery of the participant, the experience and impact on patient care in the home country during and after the MST in ARM (2), and in HD (3). Results: We collected data from 20 participants (6 female, 14 male). The majority of them had prior experience with the MST program (75%) and came from institutions specialized in the treatment of pediatric colorectal disorders (80%). Participants felt that MST improved patient care in both the host country (p < 0.001) and their home country (p < 0.001). Experienced and less experienced participants did not differ in the overall MST evaluation (ps > 0.08). They reported that their competencies to treat ARM and HD improved significantly in response to the MST (ps < 0.001). Improvements in ARM and HD treatment were associated with the number of supervised HD surgeries during MST, while the other forms of participation were unrelated to the improvements. Conclusion: The results of our questionnaire indicate that participation in MST in the specialized field of pediatric colorectal surgery helps to improve confidence in the care and treatment of affected patients in both the host and donor countries, independent of previous surgical experience.

Surgeons have a steep learning capacity to understand 2-D images provided by conventional cloacagrams. Imaging advances now allow for 3-D reconstruction and 3-D models; but no evaluation of the value of these techniques exists in the literature. Therefore, we sought to determine if advances in 3-D imaging would benefit surgeons, lead to accelerated learning, and improve understanding for operative planning of a cloaca reconstruction. Questionnaires were used to assess the understanding of 2-D and 3-D images by pediatric surgical faculty and trainees. For the same case of a cloacal malformation, a 2D contrast study cloacagram, a 3D model rotatable CT scan reconstruction, a software enhanced 3D video animation (which allowed the observer to manipulate the structure in any orientation), and a printed physical 3D cloaca model that could be held in the observer’s hand were employed. Logistic mixed effect models assessed whether the proportion of questions about the case that were answered correctly differed by imaging modality, and whether the proportion answered correctly differed between trainee and attending surgeons for any particular modality. Twenty-nine pediatric surgery trainees (27 pediatric general surgery and 2 pediatric urology surgery trainees) and 30 pediatric surgery and urology faculty participated. For trainees, the percentage of questions answered correctly was: 2-D 10.5%, 3-D PACS 46.7%, 3-D Enhanced 67.1%, and 3-D Printed 73.8%. For faculty, the total percentage of questions answered correctly was: 2-D 22.2%, 3-D PACS 54.8%, 3D Enhanced 66.2%, and 3-D printed 74.0%. The differences in rates of correctness across all four modalities were significant in both fellows and attendings (p

Published health-care costs related to constipation in children in the USA are estimated at $3.9 billion/year. We sought to assess the effect of a bowel management program (BMP) on health-care utilization and costs.At two collaborating centers, BMP involves an outpatient week during which a treatment plan is implemented and objective assessment of stool burden is performed with daily radiography. We reviewed all patients with severe functional constipation who participated in the program from March 2011 to June 2015 in center 1 and from April 2014 to April 2016 in center 2. ED visits, hospital admissions, and constipation-related morbidities (abdominal pain, fecal impaction, urinary retention, urinary tract infections) 12 months before and 12 months after completion of the BMP were recorded.One hundred eighty-four patients were included (center 1 = 96, center 2 = 88). Sixty-three (34.2%) patients had at least one unplanned visit to the ED before treatment. ED visits decreased to 23 (12.5%) or by 64% (p 0.0005). Unplanned hospital admissions decreased from 65 to 28, i.e., a 56.9% reduction (p 0.0005).In children with severe functional constipation, a structured BMP decreases unplanned visits to the ED, hospital admissions, and costs for constipation-related health care.3.

Introduction Previous research in children with Hirschsprung's disease (HD) and Down's syndrome (DS) has focused on colorectal outcomes. We set out to review urinary outcomes in this patient group. Materials and Methods The medical records of all patients aged five years and older with HD were reviewed, and patients and caregivers filled out the Vancouver Symptom Score at intake, which is designed and validated to diagnose dysfunctional elimination syndrome. Results A total of 104 patients with HD were included in this study. Of these, 16 (15%) patients had DS. There were no significant differences in the prevalence of enterocolitis or colorectal symptoms between patients with or without DS. Five of 88 (6%) patients without DS and 7 of 16 (44%) (p = 0.00001) with DS reported having urinary accidents. Patients with HD and DS scored higher on the Vancouver score (9 vs. 17.5; p = 0.007), indicating more severe urinary symptoms. Patients who also reported fecal accidents scored significantly higher on the Vancouver (12 vs. 9; n = 61; p = 0.016), indicating more problems. Conclusion Patients with DS appear to be a unique subset of HD patients who have a higher prevalence of urinary symptoms after surgery. In the postoperative care of patients with HD and DS, a strong focus should be placed on postoperative urinary care in addition to their bowel care. This could significantly ease care and contribute to the quality of life of the parents and the patient.

Cloacal malformations represent a uniquely complex challenge for surgeons. The surgical approach to date has been based on the common channel (CC) length with two patient groups considered: less than or greater than 3cm, which we believe is an oversimplification. We reviewed 19 patients, referred after surgery done elsewhere. Eight had postoperative urinary complications, 3 had constant urinary leakage and had been left after surgery with a urethra1cm, .5 with an original 3 to 5cm common channel, who had undergone total urogenital mobilization (TUM), experienced peri-operative urethral loss needing a vesicostomy, and later, a Mitrofanoff. These patients together with a review of the cloacal and urological literature led us to design a new algorithm where urethral length is a key determinant for care.We prospectively collected data on 31 consecutive cloaca patients referred to our team (2014 to 2016) and managed according to this new protocol. The CC length, urethral length, surgical technique employed, and initial outcomes were recorded.Of 31 primary cases, CC length was 1 to 3cm in 20, 3 to 5cm in 9, and greater than 5cm in 2. In the 1 to 3cm and the 3 to 5cm groups, a urethra less than 1.5cm led us to perform an urogenital separation. We only performed a TUM if the urethra was greater than 1.5cm. Using this protocol, we performed a urogenital separation in 1 of 20 in the 1 to 3cm CC group, 6 of 9 in the 3 to 5cm CC group, and 2 of 2 in the greater than 5cm CC group. Seven patients underwent separation, who with the previous approach, would have had a TUM. Thus far, no urinary leakage or urethral loss has occurred in any patient, but follow-up is less than 3years.Urethral length appears to be a vitally important component in cloacal reconstruction. A short urethra left after repair can lead to urinary leakage. A TUM done under the wrong circumstances can lead to urethral loss. We describe a new technical approach to cloacal repair which considers urethral length but recognize that long term urological outcomes will need to be carefully documented.Clinical cohort study with no comparative group.Level 4.

Anorectal malformations (ARMs) occur in approximately 1 of every 5000 newborns and management still differs widely among practitioners. In this review, we adress some of the current accepted concepts in management. Missmanagement can have devastating consequences such as faecal incontinence, urinary incontinence and sexual disfunction. We briefly review common pecularities of the most common cases the and initial management oft he newborn. In this article I intend to present a brief overview without going into details but hopefully will motivate interest and further reading. Further the recommendation is made that case referal to a centre with a high volume load provides the highest benefits fort he patient in the form of better prognosis and long term management.

A subset of patients with cloacal malformations requires vaginal replacement during their primary reconstruction, increasing the surgical complexity. Identifying factors which predict the need for vaginal replacement would facilitate operative planning.We retrospectively reviewed patients who underwent primary cloacal reconstruction at our Center (2014-2018) and assessed the length of the common channel, urethra, and vagina. The presence of hydrocolpos at birth, Müllerian anomalies, sacral ratio, and tethered cord were also assessed between patients who did and did not require vaginal replacement.50 patients were identified. 17/50 patients (34%) underwent a total urogenital mobilization (TUM), and none required vaginal replacement. 33/50 (66%) patients underwent a urogenital separation. 19/33 (58%) required vaginal replacement. This group had a shorter vagina (4.2 cm vs 6.6 cm, p 0.01). There was no difference in urethral or common channel length, number of cervices, sacral ratio, presence of a vaginal septum, hydrocolpos, or tethered cord between those who did and those who did not require vaginal replacement.Urethral and common channel lengths were used to successfully determine the operative plan (TUM or urogenital separation) to reconstruct cloacal malformations. The need for urogenital separation and a shorter vaginal length were predictive of the need for vaginal replacement.Level IV.Case series with no comparison groups.

Introduction Total colonic Hirschsprung's disease (TCHD) presents a postoperative challenge due to multiple stools and perineal rash. We propose a protocol developed by pediatric surgeons and ostomy nurses to help prevent and treat hypermotility and severe perineal rash, especially in younger children who are not toilet trained. Materials and Methods We retrospectively reviewed our TCHD patients' charts from 2014 to 2017. All patients received a prescribed protocol for the treatment of hypermotility and perineal rash. We describe patients who underwent their pull through before and after the age of urine toilet training, and assessed the number of bowel movements, the perineal skin status, and growth. Results We treated 25 patients. Out of 25, 9 patients received a straight ileoanal pull through before the age of 18 months. Nine of 25 patients presented for a second opinion and had redo pull through. The remaining seven presented for bowel management after having a pull through at another institution. All these were treated following the hypermotility protocol. In total, 19 of 25 patients were not toilet trained. The mean number of bowel movements in all groups was 4 (3–5). All had a resolution of perineal rash and liquid stools after 3 months. Eleven of the 25 patients presented with failure to thrive. Two older patients experienced severe proctalgia requiring replacement of the ileostomy. Conclusion TCHD patients who underwent definitive pull through had nine high incidence of multiple stool, perineal rash, and low growth. With the implementation of bowel management care to slow the stools and a perineal skin protocol to treat the skin, we believe that these symptoms can be minimized even in patients who are not toilet trained. Since the implementation of this protocol, we have changed our practice to perform the pull through in such patients between the age of 6 and 18 months.

Cloacal malformations are rare anomalies which occur in one in 50,000 live births. Anatomically these anomalies are defined by the presence of a single perineal orifice. There is however a substantial range in their complexity. Defining these differences is key to surgical planning and timely referral of selected cases to centers with the capabilities to manage the most challenging cases. Traditionally the common channel length as measured during cysto-vaginoscopy has been used to differentiate between patients that can be repaired with a reproducible operation and those requiring a more complex repair. The quality and range of imaging available has advanced and thus a more detailed anatomic picture is now possible to help with pre-operative planning. Cross sectional imaging with 3D reconstruction has enhanced the understanding of the anatomic variations in these patients. In addition, the sacral ratio, previously thought to only have an influence on long term continence predictions, has been shown to not only forecast the presence of urological anomalies, but also the complexity of the malformation. In principle, cloacal malformations have two major components to the reconstruction. First, the rectum needs to be separated from the urogenital tract and second, the urogenital sinus needs to be managed to create a urethral orifice and vaginal introitus. The length of the urethra has been shown to be vital in deciding between the two main surgical maneuvers; a total urogenital mobilization (TUM) and a urogenital separation. The technical demands of a urogenital separation are significant and discussed here in detail. The need for vaginal replacement adds further complexity to the care of these patients and has also been shown to be related to the length of the urethra. Predicting complexity in an accurate and non-invasive way will facilitate the care of the most complex cloacal malformations and improve outcomes.

Introduction Repair of anorectal malformations (ARMs), primarily or with a reoperation, may be performed in certain circumstances without a diverting stoma. Postoperatively, the passage of bulky stool can cause wound dehiscence and anastomotic disruption. To avoid this, some surgeons keep patients NPO (nothing by mouth) for a prolonged period. Here, we report the results of a change to our routine from NPO for 7 days to clear fluids or breast milk. Materials and Methods After primary or redo ARM surgery, patients given clear liquids were compared to those who were kept strictly NPO. Age, indication for surgery, incision type, use of a peripherally inserted central catheter (PICC) line, and wound complications were recorded. Results There were 52 patients, including 15 primary and 37 redo cases. Group 1 comprised 11 female and 15 male patients. The mean age at surgery was 4.9 years (standard deviation [SD]: 2.3). There were 8 primary cases and 18 redo cases. Twelve (46.6%) received a PICC line. The average start of clear liquids was on day 5.3 (SD: 2.2) after examination of the wound, and the diet advanced as tolerated. The first stool passage was recorded on average on day 2.3 (SD: 1.3). Four minor wound complications and no major wound complications occurred.Group 2 comprised 14 females and 12 male patients. The mean age at surgery was 3.5 (SD: 2.4) years. There were 7 primary and 19 redo cases. One (3.8%) patient required a PICC line. A clear liquid diet was started within 24 hours after surgery. A regular diet was started on average on day 5.8 (SD: 1.3). The first stool passage was recorded on an average of day 1.6 (SD: 0.9). Three minor wound complications occurred; however, there was no significant difference between the two groups (SD: 0.71). One major wound complication occurred. However, there was no significant difference in major wound complications between the groups (SD: 0.33). Conclusion No increase in wound problems was noted in children receiving clear liquids or breast milk compared with the strict NPO group, and PICC line use was reduced. We believe this change in practice simplifies postoperative care without increasing the risk of wound complications.

Introduction Functional constipation is a common problem in children. It usually can be managed with laxatives but a small subset of patients develop intolerable cramps and need to be temporarily treated with enemas. The senior author has previously reported: 1) open sigmoid resection as a surgical option, but this did not sufficiently reduce the laxative need, then 2) a transanal approach (with resection of rectosigmoid), but this led to a high rate of soiling due to extensive stretching of the anal canal and loss of the rectal reservoir. The understanding of these procedures' results has led us to use a laparoscopic sigmoid ± left colonic resection with a Malone appendicostomy for these patients, to decrease the laxative requirements, temporarily treat with antegrade flushes, and to reduce postoperative soiling. Methods A single-institution retrospective review (3/2014–9/2015) included patients who failed our laxative protocol, and therefore were considered surgical candidates. Patients with anorectal malformation (ARM), Hirschsprung disease, spina bifida, tethered cord, trisomy 21, cerebral palsy, mitochondrial disease, prior colon resection at other facilities, or those that did not participate in our laxative program were excluded. Demographics, duration of symptoms, prior treatments, postoperative complications, and postoperative bowel regimens were evaluated. Results A total of 6 patients (3 males; median age of 12.5 years) presented with soiling related to constipation and intolerance to laxatives. Four patients failed preoperative cecostomy (done prior to referral to us). An average of 4.7 medication treatments were previously tried. In all, 4 patients had required in-patient disimpactions. Duration of symptoms was 7.5 years (median). The median senna dose was 30 mg (range, 15–150 mg), and all patients had intolerable symptoms or failed to empty their colon, which we considered a failed laxative trial. All had contrast enemas that demonstrated a dilated and/or redundant sigmoid colon, and colonic manometry was abnormal in 4. All patients underwent laparoscopic sigmoid and left colon resection, or only sigmoid resection (a low anterior resection). Two patients had postoperative colitis treated with oral antibiotics. The median follow-up was 52 days (range, 8–304 days). Five patients are on antegrade enemas with plans to convert to laxatives at 6 months, 1 is taking laxatives alone at a 33% lower dosage. Five of six are completely clean, 1 soils occasionally and their daily flush is being adjusted. Conclusion Only a minority of patients with functional constipation are medically unmanageable. This preliminary report shows that laparoscopic colon resection combined with antegrade flushes is an effective surgical technique to treat that group. A laparoscopic approach, guided by contrast enema and colonic manometry, allows for a defined resection of the abnormal segment of colon with the advantages of minimally invasive surgery including allowing for an extensive rectal resection (an improvement over open sigmoid resection) and avoidance of overstretching of the anal canal and removal of the rectal reservoir (an improvement over the transanal approach). Having antegrade access is useful to manage soiling and avoiding cramping from laxatives in the early postoperative period. Although our series is small, we believe that long-term most patients can avoid antegrade flushes and be on no, or a dramatically reduced, laxative dose.

Rectal atresia and anal stenosis are rare forms of anorectal malformations. The aim of the definitive surgical repair in such cases is to preserve the anal canal, the dentate line, and the sphincter complex. We present a case of rectal atresia and anal stenosis to demonstrate the differences in the operative repair. The techniques described leave the anterior wall of the very distal anal canal untouched in both rectal stenosis and anal atresia; however, the dissection of the rectum differs. The atretic rectum in rectal atresia is mobilized and sutured to the anal canal circumferentially. In anal stenosis, the posterior rectum is mobilized in the form of rectal advancement, and the posterior 180° is anastomosed directly to the skin (as in a standard PSARP) with preservation of the anal canal as the anterior 180° of the final anoplasty. These patients have an excellent prognosis for bowel control and fecal continence, and therefore, complete mobilization and resection of the anal canal must be avoided.

Introduction: Operative surgical videos are a popular educational resource, not commonly a part of a peer-reviewed article. We wanted to evaluate the impact of either reading a peer-reviewed manuscript or watching an operative video on a surgeon's confidence in performing a complex case.Methods: Pediatric surgeons and fellows were asked to complete an initial questionnaire to assess their confidence (formulated as a score) in the diagnosis and operative repair of anal stenosis and rectal atresia.Results: Of 101 pediatric surgeons and fellows, 52 (51%) were randomized into a “manuscript” group and 49 (49%) into a “video” group. The mean confidence before the intervention was the same in the two groups (6.4 vs. 6.6). Attending surgeons started with more confidence than trainees (7.1 vs. 5.3, p < 0.001). In the manuscript group, the average confidence increased to 7.7 (p = 0.005), and in the video group the average confidence increased to 7.9 (p = 0.001) globally. Trainees in the video group significantly improved their confidence to a score of 6.6 (p = 0.035), as did attending surgeons to 8.5 (p = 0.01). In the manuscript group, only attendings significantly improved their confidence by 1.5–8.3 (p < 0.001), whereas trainees did not with a difference of 1.3 (p = 0.194). When considering experience level, physicians who reported never having performed this surgery improved only by reading the manuscript (3.9–6.2) (p = 0.004), not by watching the video (5.4–6.6) (p = 0.106). Surgeons with experience doing this operation (>5 times) did not improve their confidence by reading the manuscript (p = 0.10), nor by watching the video (p = 0.112).Conclusion: Reviewing either a detailed manuscript or operative video on the surgical management of rectal atresia and anal stenosis demonstrated a significant increase in self-reported confidence. Trainees benefitted the most from operative videos, whereas experienced surgeons did not improve their confidence by reading the manuscript nor watching the video.

Patients born with complex anorectal malformations often have associated Mullerian anomalies, which might affect fertility and obstetrical outcomes. Other vertebral-anorectal-tracheoesophageal-renal-limb associations, such as renal or cardiac anomalies, could also affect pregnancy intention, fertility rates, and recommendations about mode of delivery or obstetrical outcomes. Associated conditions present at birth, like hydrocolpos, could also potentially affect fertility. Depending on the complexity of the anomaly, primary reconstruction might include vaginoplasty, vaginal interposition, perineal body reconstruction, and extensive pelvic dissection. After the initial reconstruction, patients might have multiple additional surgeries for stoma reversal, bladder augmentation, and creation of conduits, all with potential for pelvic adhesions. Pregnancy intention, fertility rates, mode of delivery, and obstetrical outcomes data are limited in this patient population, making it challenging to counsel patients and their families. We sought to evaluate all available literature in an attempt to better counsel families. A PubMed literature search was undertaken to review this topic. Search terms of “cloaca,” “anorectal malformation,” “pregnancy,” “cloacal exstrophy,” “vaginal delivery,” and “cesarean section” were used and citation lists from all identified articles were checked to ensure that all possible articles were included in the review. We also outline comorbidities from the fetal period to adulthood that might affect reproductive health. Of the articles on anorectal and cloacal anomalies, 13 reports were identified that covered obstetrical outcomes. They were in patients with previous anorectal malformation, cloaca, and cloacal exstrophy repair. Twenty-four pregnancies were reported in 16 patients. Two ectopic pregnancies, 5 spontaneous miscarriages, 1 triplet pregnancy, and 16 singleton pregnancies were reported with a total of 19 live births. Regarding the method of conception, 15/18 pregnancies occurred spontaneously and 3/18 were via assisted reproductive technology with in vitro fertilization. There were 19 live births, of which at least 8 were preterm. Mullerian anatomy was reported in 8 of 13 articles. Only 2 patients underwent vaginal delivery (1 patient with repaired cloaca malformation had an operative vaginal delivery and 1 patient with repaired imperforate anus with rectovaginal fistula had a normal spontaneous vaginal delivery). The remaining patients all underwent a cesarean section. There were no reported cases of maternal mortality, and maternal morbidity was limited to recurrent urinary tract infections and worsening chronic kidney disease. There is a paucity of information regarding obstetrical outcomes in adult anorectal and cloaca patients. However, patients with previous cloacal repairs have achieved pregnancy spontaneously, as well as with in vitro fertilization. Patients with repaired cloacal malformations are at increased risk of preterm birth and cesarean delivery. Most patients with cloacal anomalies have an associated Mullerian anomaly and therefore have an increased risk of preterm labor. From our review we conclude that contraception should be offered to patients not desiring pregnancy, and cesarean section is likely the preferred mode of delivery. On the basis of this review, we recommend proactive data collection of all such patients to document outcomes and collaboration among providers and between centers devoted to this complex patient population.

Introduction We previously reported our surgical technique for functional constipation for patients who have failed medical management using a novel collaborative approach with gastroenterology input, pre-operative contrast enema, colonic manometry, and laxative protocol combined with a laparoscopic colonic resection with Malone appendicostomy. Now we report our intermediate outcomes. Methods Patients who failed bowel management program for functional constipation were reviewed from 3/2014–2/2017. Patients with Hirschsprung disease, anorectal malformation, tethered cord, spina bifida, Trisomy 21, cerebral palsy, mitochondrial disease, or prior colon resection were excluded. Results Of 31 patients (14 females; median age 12years, follow-up 10.3months) with functional constipation and failed medical management, 26 (84%) had preoperative colonic manometry which, in addition to the contrast enema, guided laparoscopic colon resection. Ten patients (32.3%) are clean with no flushes (1 takes no laxatives, 8 are on low dose laxatives only, and 1 patient was clean on laxatives but chose to switch back to flushes). Of the 21 patients that remain on antegrade flushes, 20 (95.2%) are clean, and one patient (4.8%) continues to soil. We define clean as no soiling and no abnormal stool burden on x-ray. Laxative trials are planned for all patients on an antegrade flush regimen. Conclusion Our intermediate results show that laparoscopic colon resection with Malone appendicostomy allows the majority of patients to be clean on antegrade flushes, and some to be on no or minimal laxatives. Type of study Retrospective review. Level of Evidence 3

Introduction Patients with anorectal malformations (ARM), Hirschsprung disease (HD), and colonic motility disorders often require care from specialists across a variety of fields, including colorectal surgery, urology, gynecology, and GI motility. We sought to describe the process of creating a collaborative process for the care of these complex patients. Methods We developed a model of a devoted center for these conditions that includes physicians, psychologists, social workers, nurses, and advanced practice nurses. Our weekly planning strategy includes a meeting with representatives of all specialties to review all patients prior to evaluation in our multidisciplinary clinic, followed by combined exams under anesthesia or surgical intervention as needed. Results There are 31 people working directly in the Center at present. From the Center's start in 2014 until 2017, 1258 patients were cared for from all 50 United States and 62 countries. 360 patients had an ARM (110 had a cloacal malformation, 11 had cloacal exstrophy), 223 presented with HD, 71 had a spinal malformation or injury causing neurogenic bowel, 321 had severe functional constipation or colonic dysmotility, and 162 had other diagnoses including familial polyposis, Crohn's disease, or ulcerative colitis. We have had 170 multidisciplinary meetings, 170 multispecialty outpatient, and 52 nurse practitioner clinics. In our bowel management program we have seen a total of 514 patients in 36 sessions. Conclusion This is the first report describing the design of a multidisciplinary team approach for patients with colorectal and complex pelvic malformations. We found that approaching these patients in a collaborative way allows for combined medical and surgical decisions with many providers simultaneously, facilitates therapy, and can potentially improve patient outcomes. We hope that this model will help establish new-devoted centers in other locations to encourage centralized care for these rare malformations. Level of evidence IV.

Aim of the study Many patients with an anorectal malformation (ARM) or pelvic anomaly have associated urologic or gynecologic problems. We hypothesized that our multidisciplinary center, which integrates pediatric colorectal, urologic, gynecologic and GI motility services, could impact a patient’s anesthetic exposures and hospital visits. Methods We tabulated during 2015 anesthetic/surgical events, endotracheal intubations, and clinic/hospital visits for all patients having a combined procedure. Main results 82 patients underwent 132 combined procedures (table 1). The median age at intervention was 3 years [0.2-17], and length of follow up was 25 months[7-31]. The number of procedures in patients who underwent combined surgery was lower as compared to if they had been done independently [1(1-5) vs 3(2-7)(p

Introduction Cloacal malformations, a confluence of the urinary tract, vagina and rectum into a single common channel, has a broad and complex anatomic spectrum requiring an imaging tool for visualization, measurement, and surgical planning for the reconstruction of these structures. We evaluated the role of 3-D fluoroscopy for this purpose, as it offers a combination of spatial correlation with precise anatomic measurements. Methods We examined our imaging protocol for patients with a cloacal malformation and report our experience with rotational fluoroscopy and 3-D reconstruction in 16 consecutive patients referred for cloacal reconstruction. The length of the common channel (CC), the length of the urethra from the bladder neck to the common channel, and the height (and existence or absence) of a vagina or vaginas were determinants of the surgical procedures used for the repair. Results We performed 16 consecutive 3-D cloacagrams (age range 4 months to 9 years) using a new protocol (Figure 1) that provided the following data which helped with surgical planning: Gynecologic: 3 cases with a single vagina, 5 cases with a duplicated Mullerian system (3 of which were asymmetric) and 2 cases with high vaginas requiring vaginal replacement. Colorectal: Four had a high rectum requiring an abdominal approach, and 6 had a rectum reachable via a posterior sagittal approach. Urologic: Two ectopic ureters requiring reimplantation, 3 patients had vesicoureteral reflux (1 bilateral, 2 unilateral), 1 patient had no bladder, and 7 had a normal sized bladder. Common channel length and urethral length were demonstrated in all cases and used to decide between a total urogenital mobilization or a separation of vagina(s) from the common channel, urogenital separation. Conclusion The 3-D cloacagram can help predict the surgical plan for urologic, gynecologic, and colorectal components of the cloacal repair. It can predict the CC length as well as the length of the urethra. It helps with predicting the need for vaginal replacement and whether an abdominal approach is needed for the rectum. Its effectiveness is based on the ability to adequately distend structures and see their distal most extent, an advantage over other modalities such as MRI. Added benefits (particularly from the 3D view) include a better spatial understanding of the defect and the diagnosis of concomitant urological abnormalities such as vesicoureteral reflux and ectopic ureters. Disadvantages to this procedure include the need for general anesthesia and a higher exposure to radiation. Level of evidence 3.

The sacral ratio (SR) is a well-established tool to quantify sacral development in patients with anorectal malformations (ARM) and can be used as a predictor of fecal continence. We hypothesized that a lower SR correlated with the presence of urologic and renal malformations.We retrospectively reviewed the medical records of patients with ARM treated at our center from 2014 to 2016. We measured the lateral SR as a marker for sacral development and assessed the spine for the presence of tethered cord (TC). Urological and renal anomalies, including single kidney, hydronephrosis, hypospadias, vesicoureteral reflux (VUR), ectopic ureter, and penoscrotal transposition were assessed. Analysis of variance (ANOVA), t-tests, and multivariable linear regression were used to test for differences in SR with consideration of associated urologic malformations and tethered cord.283 patients with ARM were included for analysis (156 females). The median age was 39months (10-90). Among these, 178 (55.6%) had 1 or more urologic malformations, and 81 (25.3%) had a TC. Hydronephrosis, high-grade VUR (3-5), solitary kidney, and tethered cord were significantly associated with lower SR (p0.01). In multivariable regression models, the presence of urologic abnormalities remained significantly associated with lower a SR despite the presence or absence of TC (p0.001).SR is a potentially useful indicator of certain urologic anomalies including hydronephrosis, high grade VUR, and solitary kidney in patients with ARM. This association is independent of the presence of TC. A sacral ratio as a part of the VACTERL screening can help the surgeon identify which patients need closer urologic follow up.IV.

Introduction Most patients with a cloacal malformation have a Mullerian anomaly. We sought to examine our patients with a cloacal malformation to determine the proportion of them we felt we knew their Mullerian anatomy and which proportion we felt would most benefit from longitudinal assessment to define their anatomy, reproductive potential, and risk of outflow tract obstruction after puberty. We also compared the preoperative assessment of reproductive anatomy (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) and intra-operative abdominal findings (at the time of primary cloacal reconstruction or subsequent abdominal procedures) to see how these correlated with and which preoperative assessment tool was most predictive of intraoperative anatomy. We also sought to confirm what we expected to be a lack of ovarian pathology. Methods A single site retrospective chart review was performed on all patients with a cloacal anomaly seen between May 2014 and September 2017. Preoperative assessment (pelvic ultrasound, MRI, cloaca gram, and vaginoscopy) and operative reports (both primary reconstruction and later abdominal procedures) were reviewed to ascertain Mullerian and ovarian anatomy. Results 30 of 36 (83%) of patients had defined Mullerian anatomy after preoperative assessment (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) with or without the addition intraoperative assessment of Mullerian structures obtained during laparoscopy or laparotomy. 19/30 (63%) had duplication of their Mullerian structures. 25/36 (69%) had intraoperative assessment of Mullerian anatomy during laparoscopy or laparotomy. In this group, preoperative assessment with pelvic ultrasound correlated in 4/8 patients (50%), MRI correlated in 3/4 patients (75%), cloacagram in 10/15 patients (67%), and vaginoscopy in 23/25 patients (92%). 14/36 (39%) patients were found to require longitudinal assessment to define anatomy, reproductive potential or risk of outflow tract obstruction after puberty. Patients with ovarian findings described at the time of laparoscopy or laparotomy had no evidence of ovarian pathology. Conclusions The majority of patients with cloaca in our series (83%) had their Mullerian anatomy defined by either preoperative assessment and/or findings at the time of laparoscopy or laparotomy. Duplication of the vagina and uterus was the most commonly described Mullerian anatomy (63%) in our series. Vaginoscopy appears to be superior to pelvic ultrasound, MRI, and cloacagram in predicting Mullerian anatomy. Fourteen of our 36 (39%) patients will require longitudinal assessment follow for reproductive potential and/or risk of outflow tract obstruction after puberty as their Mullerian anatomy is not known. There was no evidence of ovarian pathology in any cloaca patient. While we felt as though we could define Mullerian anatomy in most of our patients, any opportunity for intraoperative assessment of Mullerian anatomy should be utilized and therefore teams who are involved in the management of these patients must have a systematic and collaborative method established to ensure that Mullerian structures are thoroughly evaluated intra-operatively and documented in a standardized fashion. Type of study Retrospective Chart review. Level of Evidence III.

Background/purpose We conduct an annual medical mission to Hospital Ruth Paz para Ninos Quemados y Cirugia Pediatrica in Honduras to operate on cases of anorectal malformations (ARM). To improve our knowledge of these patients, we compared their health-related quality of life (HRQoL), and the health literacy of their caregivers from this hospital and ours in the United States. Methods The BRIEF Health Literacy Screen (BHLS) and Pediatric Quality of Life Inventory 4.0 (PedsQL) were used, respectively, to assess the health literacy and HRQoL of their guardians. All patients scheduled for an ARM-related operation in October 2016 were eligible for inclusion, and a matched population was selected in Nationwide Children's Hospital, Columbus, Ohio. Results The sample comprised 127 patients, with 22 from Honduras and 105, the US. About 13.6% and 80% of Honduran and American caregivers, respectively, had adequate literacy. Honduran and American caregivers of patients aged 12 months and below rated the HRQoL of their children at 87 and 82, respectively; aged between two and four years, at 84 and 77; aged between two and four years, at 85 and 79; and of teens, at 59 and 66. For adults, the rate was 71 and 77 in Honduras and the US, respectively. Conclusion Although health literacy is extremely low in the Honduran group, its HRQoL was comparable to that of its American counterpart. Improving health literacy by educating caregivers could be an additional goal for medical missions in the future. Level of Evidence Level III

Introduction Health literacy is low in an estimated one-third of the US population. Little is known about the health literacy of caregivers of children with colorectal conditions. The objective of this study was to investigate whether a timed health literacy intervention could improve health literacy in this population. Methods We used the BRIEF Health Literacy screening (BHLS) tool on caregivers of children who came to our colorectal clinic. Health literacy was categorized as inadequate, marginal, or adequate. The number of caregivers with adequate health literacy was compared to the number of clinic visits and socioeconomic status. Results We included 233 caregivers. The average number of clinic visits was 3.5 over 1.2 years. At the first clinic visit, 70% (n = 98) of caregivers had “adequate” health literacy. Scores improved to 88% (p = 0.024) after the fourth visit. Socioeconomic factors were not associated with health literacy. Patients of caregivers with “adequate” health literacy visited our clinic 3.8 times, compared to 2.7 times for those with lower literacy (p = 0.006). Conclusion Emphasis on providing an education-based approach at each visit increased health literacy significantly. As expected, health literacy was lowest during the first visit, which we believe is the optimal time to implement educational interventions. Type of study Case Control/Retrospective Comparative Study. Level of evidence Level III.