Examining Results of Post-Thelarche Screening Pelvic Ultrasound in Females with Anorectal Malformations.

STUDY OBJECTIVE: Mullerian duct anomalies are common in females with anorectal malformations (ARMs), although there are no universally recommended screening protocols for identification. Historically, at our institution, we have recommended a screening pelvic ultrasound (PUS) 6 months after thelarche and menarche. We aimed to evaluate outcomes associated with our post-thelarche screening PUS in females with ARMs. METHODS: An institutional review board-approved retrospective chart review was performed for all female patients 8 years old or older with ARMs and documented thelarche. Data were collected on demographic characteristics and clinical course. The primary outcome was adherence to the recommended PUS. Secondary outcomes included imaging correlation with suspected Mullerian anatomy and need for intervention on the basis of imaging findings. RESULTS: A total of 112 patients met the inclusion criteria. Of them, 87 (77.7%) completed a recommended post-thelarche screening PUS. There were no differences in completion on the basis of age, race, establishment with a primary care provider, insurance status, or type of ARM. Nine patients (10.3%) had findings on their PUS that did not correlate with their suspected Mullerian anatomy; five (5.7%) required intervention, with two requiring menstrual suppression, two requiring surgical intervention, and one requiring further imaging. CONCLUSION: Most patients completed the recommended post-thelarche screening PUS. In a small subset of patients, PUS did not correlate with suspected Mullerian anatomy and generated a need for intervention. Post-thelarche PUS can be a useful adjunct in patients with ARMs to identify gynecologic abnormalities.

Both sides of the screen: Provider and patient perspective on telemedicine in pediatric surgery.

BACKGROUND: There has been increased telemedicine use secondary to the COVID-19 pandemic. The objective of this study was to assess patient/parent satisfaction with their telemedicine experience, gauge provider perspective on telemedicine for the management of pediatric colorectal disease and evaluate the quality of telemedicine care being provided. METHODS: A cross sectional study was performed at a single institution from March 2020-February 2021. Patients who completed a patient/parent telemedicine survey after a telemedicine appointment and nurse practitioners/surgeons who completed a provider telemedicine survey were included. Patient and provider characteristics and responses were analyzed using descriptive statistics. Differences between the levels of provider confidence to provide telemedicine care were analyzed using Pearson's chi-square test. RESULTS: 118 patients/parents completed the survey. The median age of patients was 7 years. Most patients were male (59%) and White (73%). The most common diagnosis was anorectal malformation (49%). 71% of parents felt the telemedicine visit was as effective or better than an in-person visit and over 70% said they prefer a telemedicine visit to an in-person visit. Ten surgeons and 8 nurse practitioners completed the provider survey. 28% had previous telemedicine experience and 94% planned to continue offering telemedicine appointments. Providers felt significantly more confident performing clinical duties via video telemedicine compared to telephone telemedicine. CONCLUSIONS: Telemedicine is a useful adjunct or alternative in pediatric surgery for complex patients who require multidisciplinary care. Providers show confidence with the use of video telemedicine and parents show high satisfaction, with the majority preferring telemedicine visits over in-person visits. LEVEL OF EVIDENCE: IV.

Outcomes of a telemedicine bowel management program during COVID-19.

PURPOSE: Due to the COVID-19 pandemic, we transitioned from an in-person bowel management program (BMP) to a telemedicine BMP. The telemedicine BMP consisted of video and/or phone call visits (remote) or a single initial in-person visit followed by remote visits (hybrid). We hypothesized that patient/family satisfaction of a telemedicine BMP would be comparable to an in-person BMP and that there would be improvement in quality of life and functional outcomes after the telemedicine BMP. METHODS: After IRB approval, demographic and outcomes data were obtained for patients who underwent the telemedicine BMP from May-October 2020. Outcomes included a parent/patient satisfaction survey, Pediatric Quality of Life Inventory (PedsQL), and parent/patient-reported outcome measures (Vancouver, Baylor, and Cleveland scores) at baseline, 1 and 3 month follow-up. Variables were compared using Chi-square or Wilcoxon-Mann-Whitney tests and a generalized mixed model was used to evaluate outcomes scores at follow-up compared to baseline. RESULTS: Sixty-seven patients were included in our analysis with an average age of 8.6 years (SD: 3.9). Patients had the following diagnoses anorectal malformation (52.2%), Hirschsprung's disease (20.9%), functional constipation (19.4%), myelomeningocele (6.0%), and spinal injury (1.5%). Forty-eight patients (72%) underwent the remote BMP and 19 (28%) underwent the hybrid BMP. Sixty-two percent of parents completed the satisfaction survey, with a median score of 5 (very satisfied) for all questions. Over 75% of parents said they would prefer a telemedicine program over an in-person program. There was significant improvement in the Baylor and Vancouver scores after the BMP (p < 0.01), but no difference in the PedsQL or Cleveland scores (p > 0.05). There was a significant improvement in stool continence after the BMP (p < 0.01). CONCLUSION: A telemedicine BMP can be an acceptable alternative to a traditional in-person program. There was high parental/patient satisfaction and significant improvement in outcomes. Further research is needed to assess long-term outcomes. LEVEL OF EVIDENCE: III.

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.

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.

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.

Decreasing surgical site infections in pediatric stoma closures.

INTRODUCTION: Gastrointestinal (GI) operations represent a significant proportion of the surgical site infection (SSI) burden in pediatric patients, resulting in significant morbidity. We have previously demonstrated that a GI bundle decreases SSI rates, length of stay (LOS), and hospital charges. Following this success, we hypothesized that by targeting the preoperative antibiotics for stoma closures based on organisms found in infected wounds, we could further decrease SSI rates. METHODS: As part of a broad quality improvement effort to reduce SSI rates, we reviewed the responsible pathogens and their sensitivities as well as the preoperative antibiotic used, and found that 15% of wound infections were caused by enterococcus. Based on this information, starting in April 2017, we changed the prior preoperative antibiotic cefoxitin to ampicillin-sulbactam, which more accurately targeted the prevalent pathogens from April 2017 to October 2018. RESULTS: The baseline SSI rate for all stoma takedown patients was 21.4% (25 of 119). After bundle implementation, this decreased to 7.9% (17 of 221; p = 0.03) over a period of 2.5 years. Then, after changing the preoperative antibiotics, our rate of SSI decreased further to 2.2% (1 of 44; p = 0.039) over a period of 1.5 years. CONCLUSION: Significant reduction of SSI in GI surgery can be accomplished with several prevention strategies (our GI bundle). Then a change of the preoperative antibiotic choice, chosen based on causative wound infection organisms, may further decrease SSI rates. We recommend an institution specific analysis of wound infections and modification of preoperative antibiotics if the responsible organisms are resistant to the original antibiotic choice. TYPE OF STUDY: Retrospective cohort study. LEVEL OF EVIDENCE: Level III.

Factors predicting the need for vaginal replacement at the time of primary reconstruction of a cloacal malformation.

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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 OF EVIDENCE: Level IV. TYPE OF STUDY: Case series with no comparison groups.

Anatomic factors predict urinary continence in patient with anorectal malformation.

It has been described that patients with more complex anorectal malformations (ARM), lower sacral ratios and spinal anomalies have poorer rates of fecal and urinary continence. While the ARM subtype has been shown to be an independent predictor of fecal continence, it is not well understood how each of these anatomic factors impact urinary continence. The purpose of this study was to identify anatomic factors associated with urinary continence in children born with ARM. We performed a retrospective review of a large prospectively collected database of children with ARM. Inclusion criteria included diagnosis of ARM, age >4 years, available lateral sacral ratio measurement and presence of spinal MRI. Any child with incomplete or absent continence data was excluded. Continence was defined as voiding per urethra volitionally, dry between voids and ≤1 urinary accident per week. Bivariable tests of association and log-binomial regression models were used to examine association between anatomic factors and urinary continence. A total of 434 patients were included in the study. 57.8% (n = 251) were male. Median age was 8.4 years (IQR 6.0-12.3). With regards to severity of ARM, 20.3% (n = 88) were complex, 23.3% (n = 101) were moderate and 56.5% (n = 245) were simple. Lateral sacral ratio included 11.1% (n = 48) that were <0.4, 36.2% (n = 157) 0.4-0.7 and 52.8% (n = 229) > 0.7. Spine status was found to be myelomeningocele in 4.4% (n = 19), low conus or tethered cord in 34.8% (n = 151) and normal or fatty filum in 60.8% (n = 264). Overall 62.2% were continent. ARM severity, lateral sacral ratio and spine status were each independent predictors of urinary continence on univariate and multivariable analysis. We conclude that in children born with ARM, the severity of ARM, lateral sacral ratio and spine status each independently predict urinary continence. These results allow us to better understand these complex patients and their ability to develop urinary continence. This is crucial in enabling proper patient and family counseling and thus, setting appropriate expectations.

Preliminary Use of Indocyanine Green Fluorescence Angiography and Value in Predicting the Vascular Supply of Tissues Needed to Perform Cloacal, Anorectal Malformation, and Hirschsprung Reconstructions.

INTRODUCTION: Reconstructive techniques for cloaca, anorectal malformations (ARM), and Hirschsprung disease (HD) may require intestinal flaps on vascular pedicles for vaginal reconstruction and/or colonic pull-throughs. Visual assessment of tissue perfusion is typically the only modality used. We investigated the utility of intraoperative indocyanine green fluorescence angiography (ICG-FA) and hypothesized that it would be more accurate than the surgeon's eye. MATERIALS AND METHODS: Thirteen consecutive patients undergoing cloacal reconstruction (9), HD (3), and ARM repair (1) underwent ICG-FA laser SPY imaging to assess colonic, rectal, vaginal, and neovaginal tissue perfusion following intraoperative visual clinical assessment. Operative findings were correlated with healing at 6 weeks, 3 months, and 1 year postoperatively. RESULTS: ICG-FA resulted in a change in the operative plan in 4 of the 13 (31%) cases. In three cases, ICG-FA resulted in the distal bowel being transected at a level (>10 cm) higher than originally planned, and in one case the distal bowel was discarded, and the colostomy used for pull-through. CONCLUSION: ICG-FA correctly identified patients who might have developed a complication from poor tissue perfusion. Employing this technology to assess rectal or neovaginal pull-throughs in cloacal reconstructions, complex HD, and ARM cases may be a valuable technology.

Total Colonic Hirschsprung's Disease: The Hypermotility and Skin Rash Protocol.

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.

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.

Presacral masses and sacrococcygeal teratomas in patients with and without anorectal malformations: A single institution comparative study.

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.

Assessment of the Heineke-Mikulicz anoplasty for skin level postoperative anal strictures and congenital anal stenosis.

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 < 0.001). There were no operative complications. One patient restenosed and required a secondary procedure. 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.

Urinary Outcomes in Patients with Down's Syndrome and Hirschsprung's Disease.

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.

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.

A comparison of Malone appendicostomy and cecostomy for antegrade access as adjuncts to a bowel management program for patients with functional constipation or fecal incontinence.

BACKGROUND: Appendicostomy and cecostomy are two approaches for antegrade enema access for children with severe constipation or fecal incontinence as adjuncts to a mechanical bowel management program. Each technique is associated with a unique set of complications. The purpose of our study was to report the rates of various complications associated with antegrade enema access techniques to help guide which option a clinician offers to their patients. METHODS: We reviewed all patients in our Center who received an appendicostomy or cecostomy from 2014 to 2017 who were participants in our bowel management program. RESULTS: 204 patients underwent an antegrade access procedure (150 appendicostomies and 54 cecostomies). Skin-level leakage (3% vs. 22%) and wound infections (7% vs. 28%) occurred less frequently in patients with appendicostomy compared to cecostomy. Nineteen (13%) appendicostomies required revision for stenosis, 4 (3%) for mucosal prolapse, and 1 (1%) for leakage. The rates of stenosis (33 vs. 12%) and wound infection (13 vs. 6%) were higher in patients who received a neoappendicostomy compared to an in situ appendicostomy. Intervention was needed in 19 (35%) cecostomy patients, 15 (28%) for an inability to flush or a dislodged tube, and 5 for major complications including intraperitoneal spillage in 4 (7%) and 1 (2%) for a tube misplaced in the ileum, all occurring in patients with a percutaneously placed cecostomy. One appendicostomy (1%) patient required laparoscopic revision after the appendicostomy detached from the skin. CONCLUSION: Patients had a lower rate of minor and major complications after appendicostomy compared to cecostomy. The unique complication profile of each technique should be considered for patients needing these procedures as an adjunct to their care for constipation or fecal incontinence. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.

Diagnosis and management of a remnant of the original fistula (ROOF) in males following surgery for anorectal malformations.

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.

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.

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.