High-Resolution Transperineal Ultrasound in Anorectal Malformations-Can We Replace the Distal Colostogram?

Introduction Anorectal malformations (ARM) affect 1 in 5,000 newborns with a wide range of defects. In the absence of a visible fistula, the diagnosis and classification of ARM require an augmented pressure distal colostogram. This procedure can be done after a diverting colostomy has been performed and implies exposing the child to radiation. We hypothesized that high-resolution transperineal ultrasound could correctly diagnose the type of ARM, thus sparing radiation exposure. Case Description Four full-term male newborns with ARM and no visible anal opening were referred to our center for further management. A diverting descendostomy was performed in the first 48 hours of life in all cases. Prior to the reconstructive surgery, we performed a high-resolution transperineal ultrasound with 3D tomographic reconstruction of the perineal region to assess the urethra, the rectum, and a possible fistula. Findings were compared with a conventional augmented pressure distal colostogram. The image acquisition was fast and did not cause any additional distress to the children. Conclusion In all cases the results of the distal colostogram nicely correlated with the high-resolution transperineal ultrasound with 3D tomographic reconstruction. In the future, we envision a time when it can potentially replace the distal colostogram in preoperative assessment of ARM with no distress and exposure to radiation.

One-year impact of a bowel management program in treating fecal incontinence in patients with anorectal malformations.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Are routine postoperative dilations necessary after primary posterior sagittal anorectoplasty? A randomized controlled trial.

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.

Assessment of sacral ratio in patients with anorectal malformations: Can magnetic resonance imaging replace conventional radiograph?

INTRODUCTION: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Quality outcomes for pediatric colorectal surgery treated during short-term international medical service trips at a dedicated site in Honduras.

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 who reported toilet training (n = 39) and by 5 (45%) 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).

The Effect of Pediatric Colorectal Short-Term Medical Service Trips on Self-Reported Confidence in Patient Care in Volunteers in the Home Country.

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.

Joint Attention in a Laparoscopic Simulation-Based Training: A Pilot Study on Camera Work, Gaze Behavior, and Surgical Performance in Laparoscopic Surgery.

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.

Cloacal Malformations: Technical Aspects of the Reconstruction and Factors Which Predict Surgical Complexity.

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.

The use of rotational fluoroscopy and 3-D reconstruction in the diagnosis and surgical planning for complex cloacal malformations.

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.

Primary or Redo Posterior Sagittal Anorectoplasty without a Stoma: To Feed or Not to Feed?

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.

A descriptive model for a multidisciplinary unit for colorectal and pelvic malformations.

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.

Obstetrical Outcomes in Adult Patients Born with Complex Anorectal Malformations and Cloacal Anomalies: A Literature Review.

Patients born with complex anorectal malformations often have associated Müllerian 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. Müllerian 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 Müllerian 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.

The Impact of Manuscript Learning vs. Video Learning on a Surgeon's Confidence in Performing a Difficult Procedure.

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.

A structured bowel management program for patients with severe functional constipation can help decrease emergency department visits, hospital admissions, and healthcare costs.

BACKGROUND: 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. METHODS: 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. RESULTS: 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). CONCLUSION: In children with severe functional constipation, a structured BMP decreases unplanned visits to the ED, hospital admissions, and costs for constipation-related health care. LEVEL OF EVIDENCE: 3.

Health literacy and health-related quality of life in patients with anorectal malformations: A comparison between a charity hospital in Honduras and a tertiary care center in the United States.

BACKGROUND/PURPOSE: We conduct an annual medical mission to Hospital Ruth Paz para Niños Quemados y Cirugía Pediátrica 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.

Cloaca reconstruction: a new algorithm which considers the role of urethral length in determining surgical planning.

BACKGROUND: 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 urethra <1cm, .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. METHODS: 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. RESULTS: 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. CONCLUSION: 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. TYPE OF STUDY: Clinical cohort study with no comparative group. LEVEL OF EVIDENCE: Level 4.