Relationships Between Hospital and Surgeon Operative Volumes and Surgical Outcomes in Hirschsprung's Disease.

BACKGROUND: The impact of surgical volume on outcomes in Hirschsprung's disease (HD) remains uninvestigated. We aimed to determine whether higher surgeon and hospital-level HD operative volumes are associated with improved surgical outcomes following primary surgery for neonatal HD. MATERIALS AND METHODS: Neonates who underwent either an ostomy or pull-through (PT) procedure for HD before 60 d of life and a PT procedure by age 1 y were identified in the Pediatric Health Information System (PHIS). Index admissions from January 2000 to September 2012 across 41 tertiary childrens hospitals were included. Surgeon and hospital-level HD operative volume were defined as the average annual number of PT procedures performed for HD in the 2 y preceding each included case. We examined the relationship between operative volumes and all-cause readmission, readmission for Hirschsprung's associated enterocolitis (HAEC), and rates of reoperation within 30 d and 2 y. RESULTS: A total of 1268 infants were included. There were 218 patients (17.2%) readmitted to the hospital within 30 d and 540 (42.6%) within 2 y. A total of 119 patients (9.4%) had HAEC-related readmission within 30 d, and 271 (21.4%) had HAEC-related readmission within 2 y. A total of 57 patients (4.5%) had a reoperation within 30 d and 129 (10.2%) within 2 y. In risk-adjusted analyses, there were no significant associations between either surgeon or hospital HD operative volumes and readmission/reoperation rates within 30 d or 2 y. CONCLUSIONS: Neither surgeon nor hospital PT volumes were significantly associated with readmission or reoperation rates for infants with Hirschsprung's disease. Future work is needed to evaluate whether operative volumes are associated with functional outcomes following PT for HD.

Evaluation and Management of Persistent Problems After Surgery for Hirschsprung Disease in a Child.

PURPOSE OF REVIEW: Ideally, after operative intervention, a child born with Hirschsprung disease (HD) should thrive, achieve fecal continence, and avoid recurrent episodes of abdominal distention and enterocolitis. However, a significant number of patients continue to struggle following their pull-through procedure. The purpose of this review is to present an organized and practical approach to the evaluation and management of the symptomatic patient post pull-through operation for HD. RECENT FINDINGS: Children diagnosed with HD who are not doing well after their initial operation can be categorized in three distinct groups: (1) those that have fecal incontinence, (2) those with obstructive symptoms, and (3) those with recurrent episodes of enterocolitis. It is important to have a systematic diagnostic approach for these patients based on a comprehensive protocol. All three of these patient groups can be treated with a combination of either medical management, reoperation when a specific anatomic or pathologic etiology is identified, or botulinum toxin for non-relaxing sphincters contributing to the obstructive symptoms or recurrent enterocolitis. For patients not doing well after their initial pull-through, a systematic workup should be employed to determine the etiology. Once identified, a multidisciplinary and organized approach to management of the symptomatic patients can alleviate most post pull-through symptoms.

Correlation between the lateral and anteroposterior sacral ratios in anorectal malformations.

BACKGROUND: The sacral ratio has been used as a tool for evaluating sacral development in patients with anorectal malformations. Sacral ratios can be calculated by obtaining sacral radiographs in the anteroposterior (AP) and lateral planes. OBJECTIVE: The objective of the study was to determine the correlation and agreement in sacral ratio calculations. MATERIALS AND METHODS: In this single institution retrospective cohort study, we reviewed medical charts of all pediatric anorectal malformation patients treated between March 2014 and September 2018 who had both AP and lateral images of their sacrum. All sacral ratios were measured by three radiologists. Pearson's correlation coefficients and corresponding 95% confidence intervals (CIs) were used to assess the correlation between the AP and lateral radiographs. A weighted Kappa statistic was used to measure the agreement between how the AP and lateral sacral ratios categorized observations into risk groups. RESULTS: Our initial cohort consisted of 646 observations from patients with anorectal malformations who had radiographs obtained in both AP and lateral planes. We excluded all observations (n=76) where the radiographs were deemed to be inadequate or not appropriately centered to measure sacral ratio. For a given pair of measurements, the mean lateral sacral ratio was 0.07 units greater than the AP plane (95% CI 0.06-0.09, paired t-test P-value <0.0001). AP and lateral images had a moderate positive correlation (Pearson's r=0.76, 95% CI 0.73-0.79, P<0.0001) and moderate agreement in risk categorization (unweighted kappa = 0.60, P<0.0001). AP and lateral readings conducted by all three radiologists had excellent inter-rater reliability with intraclass correlations for AP and lateral sacral ratios of 0.88 and 0.84, respectively. CONCLUSION: Even though the AP and lateral sacral ratios had moderate positive correlation, the mean sacral ratio determined by images in the lateral plane was 0.07 units greater than the AP plane. AP and lateral sacral ratios concluded different risk categories relatively often. Future studies are needed to determine whether AP or lateral sacral ratios correlate better with continence in patients with anorectal malformations.

Association between Age and Umbilical Hernia Repair Outcomes in Children: A Multistate Population-Based Cohort Study.

OBJECTIVE: To evaluate whether patient age or other sociodemographic and clinical characteristics are associated with recurrence or unplanned related hospital revisits after pediatric umbilical hernia repair. STUDY DESIGN: We performed a retrospective cohort study using the Healthcare Cost and Utilization Project State Inpatient, Emergency Department, and Ambulatory Surgery and Services Databases of 7 states. Pediatric umbilical hernia repairs performed at any hospital or surgery center in 2010-2014 were included. Hernia recurrences and occurrences of unplanned and related hospital revisits within 30 days were evaluated. RESULTS: Of 9809 included patients, 52.0% were female and 50.5% were black. The 3-year hernia recurrence rate was 0.57% (95% CI 0.42, 0.73). In multivariable analysis, the recurrence rate was higher in children <4 years of age than in children 4-10 years of age (hazard ratio [HR] 1.93, 95% CI 1.09, 3.44). Unplanned related hospital revisits within 30 days occurred in 2.5% of patients. Patient characteristics associated with the risk of an unplanned related hospital revisit included age <4 years (HR 2.17, 95% CI 1.70, 2.77) or >10 years (HR 2.11, 95% CI 1.46, 3.05), public insurance (HR 2.10, 95% CI 1.58, 2.79), asthma (HR 1.74, 95% CI 1.32, 2.29), and initial presentation to the emergency department (HR 2.46, 95% CI 1.08, 5.61). CONCLUSIONS: Rates of recurrence and unplanned related hospital revisits following pediatric umbilical hernia repair are higher in children younger than 4 years of age. These findings support delaying the repair of asymptomatic umbilical hernia in children until 4 years of age.

Does Hirschsprung-Associated Enterocolitis Differ in Children With and Without Down Syndrome?

BACKGROUND: Children with Hirschsprung disease (HD) and Down syndrome (DS) are reported to experience a higher incidence of Hirschsprung-associated enterocolitis (HAEC) than those without DS, although whether their individual episodes are more severe is unknown. METHODS: A single-institution retrospective cohort study was performed on all patients with HD admitted with HAEC between 2009 and 2017. Patients were divided into two groups based on an associated diagnosis of DS, and demographics, clinical characteristics at presentation, and clinical outcomes were compared. RESULTS: Of 86 admissions for HAEC over the study period, 12 (14%) were for patients with DS. Patients admitted with and without DS were similar in terms of age (3.0 versus 2.6 y, P = 0.72), male gender (58% versus 77%, P = 0.17), and proportion with disease proximal to the left colon (9% versus 12%, P = 0.70). Patients admitted with DS were more likely to be tachycardic (75% versus 19%, P ≤ 0.001) and hypotensive (33% versus 7%, P = 0.01) at presentation and presented for treatment after a longer duration of symptoms than those without DS (84 versus 24 h, P = 0.001). Patients admitted with DS were more likely to require intensive care unit admission (58% versus 12%, P = 0.006) and received a longer course of antibiotics (13 d versus 7 d, P = 0.02). The length of stay was similar (6 versus 4 d, P = 0.34). CONCLUSIONS: The results of our study suggest that patients with DS experience more severe episodes of HAEC. Ongoing study is needed to identify preventive strategies to decrease the morbidity of HAEC in children with HD and DS.

Inter-rater Reliability of Sacral Ratio Measurements in Patients with Anorectal Malformations.

BACKGROUND: Anorectal malformations (ARMs) are a spectrum of congenital anomalies with varying prognosis for fecal continence. The sacral ratio (SR) is a measure of sacral development that has been proposed as a method to predict future fecal continence in children with ARM. The aim of this study was to quantify the inter-rater reliability (IRR) of SR calculations by radiologists at different institutions. MATERIALS AND METHODS: x-Rays in the anteroposterior (AP) and lateral planes were reviewed by a pediatric radiologist at each of six different institutions. Subsequently, images were reviewed by a single, central radiologist. The IRR was assessed by calculating Pearson correlation coefficients and intraclass correlation coefficients from linear mixed models with patient and rater-level random intercepts. RESULTS: Imaging from 263 patients was included in the study. The mean inter-rater absolute difference in the AP SR was 0.05 (interquartile range, 0.02-0.10), and in the lateral SR was 0.16 (interquartile range, 0.06-0.25). Overall, the IRR was excellent for AP SRs (intraclass correlation coefficient [ICC], 81.5%; 95% confidence interval, 75.1%-86.0%) and poor for lateral SRs (ICC, 44.0%; 95% CI, 29.5%-59.2%). For both AP and lateral SRs, ICCs were similar when examined by the type of radiograph used for calculation, severity of the ARM, presence of sacral or spinal anomalies, and age at imaging. CONCLUSIONS: Across radiologists, the reliability of SR calculations was excellent for the AP plane but poor for the lateral plane. These results suggest that better standardization of lateral SR measurements is needed if they are going to be used to counsel families of children with ARM.

Development of a Patient-reported Experience and Outcome Measures in Pediatric Patients Undergoing Bowel Management for Constipation and Fecal Incontinence.

AIMS AND OBJECTIVES: The aim of this review is develop a reliable and valid questionnaire that assesses patient-reported experience measures (PREMs) and patient-reported outcome measures (PROMs) of caregivers, families, and patients with severe constipation and fecal incontinence who failed conservative treatments and require a formal bowel management program (BMP). METHODS: A 5-step iterative process was utilized to ensure the reliability and validity of the final instrument. Parents or guardians of patients undergoing our week-long BMP were first asked 20 open-ended questions aimed at understanding the challenges in managing their child's condition. Responses were transformed into an initial 41-item survey constituted of 2 constructs relating to PREMs (24 items) and PROMs (17 items). Survey items were then administered to 359 parents and guardians undergoing BMP. Cognitive interviews were performed with 20 families to assess face validity and to further refine the survey. Instrument readability and reliability was assessed by Flesch-Kincaid and Crohnbach α analyses. Items that weakly correlated were deleted to yield a final instrument that was both valid and reliable. RESULTS: A 33-statement survey was developed that encompasses important physical and emotional health concerns, quality of life, treatment success, financial considerations, schooling, parental employment, and social concerns. The final instrument, the Patient-Reported Experience and Outcome Measure in a Bowel Management Program (PREOM-BMP), is divided into 2 separate constructs that assess important patient-reported experience (22 items) and outcome (11 items) measures. Reliability analyses on the final instrument yielded a Crohnbach α of 0.925. CONCLUSIONS: The PREOM-BMP offers physicians and nursing professionals a brief and valid tool to measure the impact of a bowel management program on both the child and the family unit.

Recurrence of Pilonidal Disease: Our Best is Not Good Enough.

BACKGROUND: Recurrence rates of pilonidal disease have been reported to be high as 30%. Patients with recurrent pilonidal disease often develop chronic wounds and draining sinuses that incur long-term morbidity, disability, and decreased quality of life. The aim of this study was to characterize rates of recurrence in patients with pilonidal disease treated by pediatric surgeons. METHODS: A single center retrospective review of patients with pilonidal disease evaluated by pediatric surgeons from 2010 to 2015 was performed. Recurrence of pilonidal disease was defined as an episode of active pilonidal disease that required medical or surgical intervention >30 days from the preceding treatment. Repeated events proportional hazards regression modeling was performed to identify factors associated with time to recurrence. RESULTS: Among 307 patients treated for pilonidal disease, nearly 50% were male, and the median age at initial evaluation was 16 years (IQR 15-17). Approximately 45% were obese (BMI ≥ 95th percentile). The initial treatment during the study period was surgical excision in two-thirds and incision and drainage and/or antibiotics in one-third. The overall recurrence rate was 33%, with the majority of recurrences (80%) occurring within the first year. On multivariable analysis, obese BMI was the only factor independently associated with time to disease recurrence. CONCLUSIONS: Pilonidal disease has a substantial recurrence rate even after surgical excision. Future studies investigating treatments that can prevent disease recurrence are needed.

Open repair of a ruptured abdominal aorta with an aortoiliac vein fistula in a 7-month-old infant and review of the literature.

Ruptured abdominal aortic aneurysms are extremely rare in the pediatric population. In this video case report, we describe the successful repair of a ruptured abdominal aortic aneurysm in a 7-month-old female infant.

Anal Botulinum Toxin in Children with Hirschsprung Disease and Functional Constipation: A Prospective Cohort study.

INTRODUCTION: Anal sphincter botulinum toxin injections (BTIs) are used in the treatment of children with severe defecation disorders, including Hirschsprung disease (HD) and functional constipation (FC). Our objective was to evaluate the outcomes of BTI in these children. MATERIALS AND METHODS: We performed a prospective cohort study of children undergoing BTI from July 2018 to December 2018. We recorded perceived effect of the BTI, including effectiveness ranging from 0 (not at all effective) to 4 (extremely effective). In addition, we recorded symptoms and the Cleveland Clinic Constipation Score (CCCS). Data were collected at baseline and at 2 weeks, 2 months, and 4 months post-injection. RESULTS: Forty-two children (HD = 25, FC = 17) were included in the study (median age 4.3 years, IQR 2.4-7.2, 52% male). Twenty-two (88%) children with HD and eight (47%) children with FC had previously undergone a BTI. BTIs were perceived effective in 16 (76%) and 12 (71%) children with HD and eight (47%) and seven (47%) children with FC at 2-week and 2-month follow-up, respectively. Effectiveness was not rated differently between groups except at the 2-month follow-up, when patients with HD rated the BTI more effective compared to those with FC (median 2 [HD] vs. median 1 [FC], p = 0.022). Over the course of the study, 17/39 (44%) children reported self-limiting adverse effects such as fecal incontinence and pain at the injection site. CONCLUSION: Anal sphincter BTIs can be effective in the treatment of constipation in both HD and FC patients.

ALK+ anaplastic large cell lymphoma exhibits phosphatidylinositol-3 kinase/Akt activity with retained but inactivated PTEN--a report from the Children's Oncology Group.

BACKGROUND: ALK+ anaplastic large cell lymphoma (ALCL) is usually a disease of young patients. We investigated phosphatidylinositol-3 kinase (PI3K)/Akt pathway-associated factors in pediatric cases and cell lines. PROCEDURE: Patient materials consisted of tissue slides of ALK+/CD30+ ALCL from 33 patients treated on Pediatric Oncology Group protocols (9219, n = 8 and 9315, n = 25). Slides were examined by immunohistochemistry for phospho(p)-Akt and PTEN, the primary feedback regulator of the pathway, as well as for p27kip1 and stathmin-1. ALCL cell lines SUDHL-1 and Karpas-299 were examined for ALK, pALK, pAkt, p27/Kip1, PTEN, pPTEN, CD30, pSTAT3, and pSTAT5; ALK inhibition was performed using compound PF-2341066 and PTEN genes were sequenced. RESULTS: A majority of patients expressed pAkt, PTEN, and stathmin, with p27kip1 levels less than controls. Cell lines showed expression of ALK, pALK, pSTAT3, pSTAT5, CD30, pAkt, PTEN, and pPTEN, with p27 slightly less than positive controls, and germline PTEN DNA. There was evidence of phosphorylated PTEN (pPTEN) associated with inhibited function. Pharmacologic inhibition of activated ALK diminished pSTAT3, pSTAT5, and CD30 expression but not pAkt or pPTEN in cultured cell lines. CONCLUSION: We conclude that the PI3K/Akt pathway is activated in many, though not all, pediatric ALK+ ALCL. Our data suggest that activation of this pathway involves post-translational regulation of PTEN. Pharmacologic inhibition of activated ALK does not reduce modest levels of activated Akt as it does with the more abundant levels of activated STAT3 or STAT5. Future therapy of ALCL might, in selected patients, best combine agents inhibiting PI3K/Akt with those targeting ALK.

Evaluation and treatment of the post pull-through Hirschsprung patient who is not doing well; Update for 2022.

After operative intervention for Hirschsprung disease (HD) a child should thrive, be fecally continent, and avoid recurrent episodes of abdominal distention and enterocolitis. This is unfortunately not the case for a significant number of patients who struggle following their pull-through procedure. Many clinicians are puzzled by these outcomes as they can occur in patients who they believe have had a technically satisfactory described operation. This review presents an organized approach to the evaluation and treatment of the post HD pull-through patient who is not doing well. Patients with HD who have problems after their initial operation can have: (1) fecal incontinence, (2) obstructive symptoms, and (3) recurrent episodes of enterocolitis (a more severe subset of obstructive symptoms). After employing a systematic diagnostic approach, successful treatments can be implemented in almost every case. Patients may need medical management (behavioral interventions, dietary changes, laxatives, or mechanical emptying of the colon), a reoperation when a specific anatomic or pathologic cause is identified, or botulinum toxin when non-relaxing sphincters are the cause of the obstructive symptoms or recurrent enterocolitis.

The cutback revisited - The posterior rectal advancement anoplasty for certain anorectal malformations with rectoperineal fistula.

BACKGROUND: The repair of rectoperineal fistulae can pose a significant challenge to the pediatric surgeon given the proximity of the fistula to the urethra in males and vagina in females. In these children, a simple cutback procedure may leave the neoanus in a position anterior to the center of the sphincter, which theoretically could impair future continence. We devised an adaptation of the cutback anoplasty which we call the posterior rectal advancement anoplasty (PRAA) to treat patients with a rectoperineal fistula that is both narrow in lumen and located within, but at the anterior-most limit of the sphincter complex. MATERIAL AND METHODS: Patient selection, operative steps, and perioperative care of patients undergoing PRAA are detailed. RESULTS: 10 children (6 males, 4 females) underwent PRAA. There were no vaginal wall or urethral injuries. At 6 months postoperatively, all patients were passing stool spontaneously. No patients required dilation of the anoplasty in the postoperative period and there were no anal strictures identified. CONCLUSIONS: A modification of the cutback anoplasty can be performed in patients with a perineal fistula and the distal fistula tract within the sphincter complex. We have demonstrated that this can be performed safely and obviates the need for an anterior rectal wall dissection, thus eliminating the risk of injury to urethra or vagina. LEVEL OF EVIDENCE: IV.

A pediatric colorectal and pelvic reconstruction course improves content exposure for pediatric surgery fellows: A three-year consecutive study.

PURPOSE: Interactive courses play an important role in meeting the educational needs of pediatric surgical trainees. We investigated the impact of a multimodal pediatric colorectal and pelvic reconstruction course on pediatric surgery trainees. METHODS: A retrospective evaluation was performed of pre- and post-course surveys for an annual colorectal and pelvic reconstruction course over 3 consecutive years (2017-2019). The course included didactic and case-based content, interactive questions, video, and live case demonstration, and a hands-on lab. Pre- and post-course surveys were distributed to participants. Comfort with operative/case procedures was scored on a 5-point Likert scale (1 uncomfortable, 5 very comfortable). The primary outcome was improved confidence and content knowledge for pediatric colorectal surgical conditions. RESULTS: 165 pediatric surgical fellow participants with a 70 responses (42.4% response rate) comprised the cohort. Participants had limited advanced pediatric colorectal experience. At the time of the course, participants reported a median of 5 [3,10] Hirschsprung pull-throughs, 6 [3,10] anorectal malformation, and 1 [0,1] cloaca cases. Participants transitioned from discomfort to feeling comfortable with pediatric colorectal operative set-up and case management (pre-course 2 [2,3] and post-course 4 [4,5] p<0.001). CONCLUSION: Pediatric surgery trainees report limited exposure to advanced pediatric colorectal and pelvic reconstruction cases and management during their pediatric surgical fellowship training but report improved content knowledge- and technical understanding of complex pediatric disorders upon completion of a dedicated course. The course is an important adjunct to the experience gained in pediatric surgery fellowship for achieving competency in managing patients with Hirschsprung disease, anorectal malformation, and cloacal reconstructions.

Functional fecal and urinary outcomes after sacrococcygeal mass resection in pediatric patients.

BACKGROUND: Sacrococcygeal masses (SCM) are uncommon in children. The purpose of this study is to review the functional fecal and urinary outcomes following resection of SCM and to determine the impact of a multidisciplinary clinic (MDC) on these outcomes. METHODS: A retrospective review was performed of patients who underwent SCM resection between 1979 and 2019. Baylor Social Continence Scale (BCS), Vancouver Symptom Score (VSS) and Cleveland constipation score (CSS) surveys were used to assess fecal and urinary continence at time of most recent follow up. Age, tumor characteristics, histopathology, and type of anorectal malformations (ARM), if present, were also recorded. RESULTS: 75 patients were included. 51 (69%) patients were females and 23 (31%) had an associated ARM. The median age at resection was 8.5 months (IQR 0-26.8). 41 (56%) patients were followed in the MDC. 27 (82%) of patients seen in the MDC were clean for stool and 26 (87%) were dry for urine, while only 17 (59%) of patients not seen in the MDC were clean for stool and dry for urine (p<0.05). There was improvement in Baylor, Vancouver and Cleveland scores. CONCLUSIONS: A multidisciplinary approach to the care of patients following SCM resection may improve bowel and bladder outcomes.

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.

Correlation of anorectal malformation complexity and associated urologic abnormalities.

BACKGROUND: Patients with anorectal malformations (ARM) commonly have associated urologic anomalies. Few large studies exist to accurately characterize the incidence or associations between severity of malformation and urologic diagnosis. The purpose of our study was to determine the incidence of urologic diagnoses in a large cohort of children with ARM and evaluate for any correlation between severity of ARM and the incidence and number of associated urologic diagnoses. METHODS: A retrospective review was performed of patients with ARM treated at our pediatric colorectal center. All patients underwent protocolized urologic screening. ARM subtypes were ordered with increasing severity as follows in males: perineal, bulbar, prostatic and bladder neck fistulae. Females were similarly categorized as perineal, vestibular and vaginal fistulae followed by cloaca with <3 cm common channel and cloaca with >3 cm common channel. The following urologic diagnoses were assessed to determine whether a correlation existed with the severity of the ARM subtype: hydronephrosis, vesicoureteral reflux (VUR), solitary kidney, renal ascent anomalies (ectopic or pelvic), renal fusion anomalies (horseshoe or cross fused kidney), duplex kidney, hypospadias and undescended testicle. ARM subtypes were defined by distal rectal anatomy. RESULTS: A total of 712 patients were included in our study with a mean age of 4 years and of whom 45% were male. The overall rate of urologic anomalies was greater in males than females (65% vs 56% p < 0.026). In both sexes, the rate of urologic anomalies increased with increasing severity of ARM subtype (p<0.00010) finding that males with bladder neck fistula and females with cloacal malformations, particularly with long common channels, being the highest incidence. In males and females, the rate of hydronephrosis increased as the complexity of ARM increased and this correlated significantly (p < 0.0001 vs p < 0.0003 respectively). Similarly, the incidence of VUR also increased as complexity of ARM increased in both males and females (p = 0.01 and p<0.0001 respectively). The remaining urologic diagnoses were not significantly correlated with severity of ARM. CONCLUSIONS: Urologic anomalies occur at a high rate in children with ARM and appear to increase in frequency with increasing complexity of ARM subtype. These findings stress the importance of proper ARM screening and proactive collaboration with a clinician with expertise in pediatric urology early in the management of such children to improve early recognition of urologic diagnoses. LEVEL OF EVIDENCE: Level III.

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.

Significant rate of lower urinary tract dysfunction in patients with sacrococcygeal teratomas.

BACKGROUND: Sacrococcygeal teratoma (SCT) is the most commonly occurring presacral tumor and can affect the lower urinary tract by direct mass effect, or as a sequela from surgical resection. Despite the potential impact on urologic function, there is no current standard for urological involvement or follow-up for these patients. The purpose of this study was to evaluate the need for urologic involvement for lower urinary tract dysfunction (LUTD) in patients with SCTs at our institution. METHODS: We performed a retrospective chart review of patients diagnosed with SCT and managed at our institution between 1990 and 2019. Data collected included: patient demographics, surgical and pathology reports, presence of tethered cord or anorectal malformation, and need for urologic involvement for LUTD. LUTD included acute urinary retention, need for chronic intermittent catheterization, and/or urinary incontinence. Acute urinary retention was defined as requiring catheterization to empty the bladder for a limited time (outside the standard post-operative indwelling catheter time period) and the eventual return to spontaneous voiding. Chronic intermittent catheterization was defined as those with urinary retention that has persisted and required continued catheterization at the time of chart review. Urinary incontinence was defined as urine leakage in children older than 3 years of age. Patients with unavailable records were excluded. Comparison between groups was performed with Mann Whitney and chi-squared tests. RESULTS: Forty-five patients with SCTs were identified. LUTD was identified in 23 patients (51%). The most common reason for LUTD was urinary retention (n = 16, 70%): 9 patients had acute retention and 7 had chronic retention (Fig. 1). Nine patients (39%) had urinary incontinence: 2 of these patients (4% of all SCT patients) had urinary fistulas (vesicovaginal (n = 1) and urethrovaginal (n = 1)). Only 5 patients (22%) had LUTD recognized preoperatively. Nine patients had concomitant tethered cord, and 7 of these (78%) had LUTD. Of patients with LUTD, Altman type IV was the most common location (n = 10, 43%). There was no significant difference in tumor type between those with and without LUTD. Patients without LUTD had significantly shorter follow-up. CONCLUSIONS: Greater than 50% of patients with SCTs have known LUTD. Two of these patients were found to have urinary fistulas requiring urinary diversion. A multidisciplinary team including urology should be involved upfront in the management of patients with SCTs, and LUTD should be routinely assessed at follow-up visits. Evaluation for a urinary fistula should occur in the presence of urinary incontinence.

Adjuncts to bowel management for fecal incontinence and constipation, the role of surgery; appendicostomy, cecostomy, neoappendicostomy, and colonic resection.

Constipation and fecal incontinence are common problems in children after repair of an anorectal malformation (ARM). While many children can be effectively managed with an oral laxative regimen, others require a mechanical colonic washout to achieve social continence. Appendicostomy and cecostomy are two techniques which permit antegrade access to the colon for the purpose of enema delivery, which improves compliance and quality of life for patients and families. The purpose of this article is to review, using a case-based approach, the indications for placement of a channel for antegrade enema access, clinical scenarios in which one technique would be preferred over another, common complications following each procedure.