Adult obstetricians and gynecologists lack knowledge of anorectal malformations - a call for action: Gynecologists lack knowledge of anorectal malformations.

OBJECTIVE: Patients with anorectal malformations (ARMs) may have concurrent gynecologic abnormalities. As patients grow, they typically transition from pediatric subspeciality care and seek adult OB/GYN related services. We aimed to assess adult OB/GYN physicians' knowledge, competency, and comfort meeting the sexual and reproductive health care needs of patients with ARM. METHODS: We performed a cross-sectional observational survey-based study of graduates from a single academic OB/GYN residency program from 2013-2022. Physicians were surveyed on experience, comfort, and challenges caring for patients with ARMs and given a knowledge assessment. Descriptive and comparative statistics between those who did and did not complete a pediatric and adolescent gynecology (PAG) rotation were generated. RESULTS: There were 59 respondents (53.6%). Fewer than half (39.0%) report caring for a patient with ARM, an appendicovesicostomy (12.3%) or an appendicostomy (5.4%). Most felt uncomfortable (80.4%) or felt they lacked competence caring for these patients (81.8%). The majority (64.3%) felt ARMs should be discussed in residency. Only one physician (1.7%) answered all questions in the knowledge assessment correctly; 33.9% did not answer any question correctly. On subgroup analysis, more physicians completing a PAG rotation recalled learning about ARMs (83.3 vs 51.9%, p=0.03); however, there were no differences in experience, comfort, competence, or willingness to learn. CONCLUSION: OB/GYN providers report lack of knowledge and comfort in caring for patients with ARMs. Development of a standardized OB/GYN residency curriculum and education for practicing OB/GYN physicians is necessary to allow access to knowledgeable sexual and reproductive health for this patient population.

Impact of Surgical Team Involvement at the Time of Trauma Activation for Pediatric Patients With Motorized Cycle or All-Terrain Vehicle Injury Mechanism.

OBJECTIVES: At our institution, level 2 trauma (L2T) activations are primarily managed by pediatric emergency medicine (PEM) physicians, whereas level 1 activations are co-managed by pediatric surgery and PEM. Starting in September 2019, the response to L2T activations due to all-terrain vehicles or motorized cycles (ATVs/MCs) changed to include surgical assessment upon patient arrival due to increased likelihood of significant injuries and need for higher level of care. The impact of PEM/surgery co-management of ATV/MC L2T patients on time to an admission decision is unknown. METHODS: We retrospectively reviewed patients <18 years of age presenting to our American College of Surgeons-verified level 1 pediatric trauma center as L2T activations with ATV/MC mechanism between 1/2016 and 10/2022. Patient demographics, injury characteristics, details of imaging, interventions, and emergency department (ED) course were recorded. The χ2 and Fisher exact tests were performed. RESULTS: One hundred fifty-five patients met the inclusion criteria prior to augmenting our response to include surgical presence at L2T-ATV/MC activations, and 216 patients were treated after our protocol change. There were no statistically significant differences in age, sex, race, transfer status, vehicle subtype, or Injury Severity Scores between groups. Trauma surgery was involved in the care of 74.8% of L2T-ATV/MC patients before protocol augmentation and 87% after (P = 0.003). Time to an admission decision significantly decreased by 22.5 minutes (117 minutes [interquartile range, 72-178] vs 94.5 minutes [interquartile range, 60-139]; P = 0.023) after protocol augmentation. There was a trend toward increased completion of mandated postsecondary survey communication huddles after protocol change (84.6% to 91.2%, P = 0.089). The median total ED length of stay did not differ between admitted and discharged patients. CONCLUSIONS: Early surgical assessment for pediatric patients with ATV/MC injuries improved time to an admission decision and trauma communication huddle compliance. Next steps include identifying process improvement opportunities to decrease ED total length of stay for patients with ATV/MC injuries.

Anorectal physiology and colonic motility in children with a history of tethered cord syndrome.

OBJECTIVES: The understanding of the impact of tethered cord syndrome (TCS) on the physiology of the colorectal area is limited. Our aim was to describe anorectal and colonic motility in children with TCS and compare the findings to those of children with functional constipation (FC). METHODS: We conducted a retrospective review of children with TCS who had an anorectal manometry (ARM) performed at our institution from January 2011 to September 2023. We recorded demographics, medical and surgical history, clinical symptoms, and treatment at time of ARM, ARM findings (resting pressure, push maneuver, rectal sensation, rectoanal inhibitory reflex [RAIR], and RAIR duration), and the final interpretation of colonic manometry (CM) if performed. We identified age and sex-matched control groups of children with FC. RESULTS: We included 24 children with TCS (50% female) who had ARM testing (median age at ARM 6.0 years, interquartile range 4.0-11.8 years). All children had constipation at time of ARM. Nineteen children had detethering surgery before ARM was performed. No significant differences in ARM parameters were found between children who had detethering surgery before ARM and children with FC. Among the 24 children, 14 also had a CM performed (13/14 after detethering surgery). No significant differences in colonic motility were found between children with a history of TCS and children with FC. CONCLUSIONS: Anorectal physiology and colonic motility are similar between children with a history of TCS and children with FC, suggesting that the underlying pathophysiology of defecatory disorders in children with and without history of TCS is similar.

Laparoscopic-Assisted Colonic Derotation in Patients With Hirschsprung Disease.

BACKGROUND: Children with Hirschsprung disease (HSCR) proximal to the splenic flexure or those needing a redo pull-through (PT) are at risk for tension and ischemia of the PT which could result in leak, stricture, or loss of ganglionated bowel. Colonic derotation is a technique used to minimize tension and avoid duodenal obstruction. The aim of this study was to describe this technique and outcomes in a series of patients requiring this intervention. METHODS: All patients underwent initial diversion and colonic mapping. The derotation procedure involves mobilization of the remaining colon, counterclockwise rotation via the stoma closure site, placement of the pull through (the right colon) lying on the right of the pelvis, and ligation of the middle colic artery with preservation of the marginal branch running from the ileocolic artery. This maneuver prevents compression of the duodenum by the mesenteric vessels and allows for an isoperistaltic, tension-free anastomosis. Intraoperative indocyanine green fluorescence angiography (ICG-FA) was utilized in many of the cases to map the blood supply of the pull-through colon. We reviewed outcomes for all children with HSCR who underwent colonic derotation from 2014 to 2023. Descriptive statistics were performed. RESULTS: There were 37 children included. Most were male (67.5%) with the original transition zone proximal to the rectosigmoid (81.1%). The median age at PT was 9.3 months [6.1-39.7]. Median operative time was 6.6 h [4.9-7.4] and 19 cases (51.4%) used ICG-FA. Most children had no 30-day postoperative complications (67.6%); in those who did develop complications, readmissions for electrolyte imbalance was most common (50.0%). There were zero cases of anastomotic leak at PT anastomosis. At long-term follow up, median 4.4 years [2.3-7.0], three children (8.1%) developed an anastomotic stricture, all were amenable to anal dilation, and five experienced episodes of enterocolitis (14.7%). Most children had between 1 and 4 stools per day (58.8%). CONCLUSION: Colonic derotation is a useful strategy to ensure well-perfused colonic length, protect the marginal artery blood supply, avoid duodenal compression, and ensure a tension-free anastomosis with minimal complications. TYPE OF STUDY: Original research, retrospective cohort. LEVEL OF EVIDENCE: III.

The effect of surgical reconstruction on bladder function in cloacal malformation: A study of urodynamics.

BACKGROUND: Girls with cloacal malformation are at risk of bladder dysfunction, with nearly 90% exhibiting some degree of dysfunction. Surgical dissection, particularly with total urogenital mobilization (TUM), has been hypothesized as a cause of worsening bladder function despite this population commonly having associated vertebral and spinal cord abnormalities that may also explain bladder dysfunction. More recently there has been great effort to select the appropriate surgical technique for cloacal repair in each patient in order to minimize dissection and potential damage to the bladder. We aimed to evaluate the effect of surgical cloacal repair on bladder function based on pre and post-surgery urodynamics (UDS) testing. METHODS: A prospectively collected database of patients with anorectal malformation at a single center was queried for girls with cloacal malformations who had undergone surgical repair from 2015 to 2022. It is our current protocol to perform UDS before and after cloacal repair. Only patients who completed both pre and post-surgery UDS were included. UDS were evaluated and classified using the UMPIRE protocol. RESULTS: A total of 48 patients were included in the cohort. The majority of patients (79.2%) had stable or improved UDS post-op leaving 10 patients (20.8%) who had worsening UDS. Long common channel (≥3 cm) was the only factor significantly associated with worsening UDS. (p = 0.03) Nearly 30% (n = 8) of those undergoing UGS had worse post-op UDS compared to 9.5% (n = 2) with TUM. All patients who worsened UDS initially had safe UDS that changed to intermediate, except for one who worsened to hostile in the setting of significant social challenges and non-compliance. Only common channel length was predictive of worsening UDS, while the type of surgical approach and spine status were not. While the overall risk of worsening UDS after TUM is only 9.5%, patients with normal spines undergoing TUM had the lowest risk, seen in only one in 15 patients (6.6%). CONCLUSIONS: Common channel length was the most significant predictor of worsening UDS, while spine status and surgical technique (TUM vs UGS) did not significantly impact this finding. By following this established surgical protocol based on common channel and urethral lengths, is rare for the surgical cloacal repair to result in worsening post-op UDS, particularly in those undergoing TUM for short common channel and normal spine.

Following a strict renal protection protocol in cloacal malformations: A descriptive analysis.

BACKGROUND: In children with cloacal malformations, renal dysfunction is a constant concern, with reported incidence as high as 50%. Multiple factors exist that may impair renal function. Our institution follows a strict renal protection protocol in this population. Incidence of renal dysfunction in these patients is unknown. OBJECTIVE: We aimed to evaluate incidence of renal dysfunction while implementing this protocol in a cohort of children with cloacal malformation. STUDY DESIGN: We reviewed a prospectively collected database of children with cloacal malformations managed at a single institution since implementation of a renal protection protocol. This involves regular laboratory evaluation, appropriate selection of total urogenital mobilization or urogenital separation, proactive imaging in patients with signs of impending renal dysfunction or urinary retention, and early catheterization teaching and implementation if necessary. Glomerular filtration rate (GFR) was calculated with the Schwartz formula and CKD grades assigned per standard definitions. Renal dysfunction was defined as CKD grade 3b or higher, need for renal replacement therapy (RRT) or transplantation. Descriptive statistics were computed. RESULTS: A total of 105 children were managed under this protocol with a median follow-up of 4.2 years [IQR: 2.0-5.9]. Six children (5.7%) had renal dysfunction at most recent follow-up; of these children, only three (2.9%) progressed from normal renal function at initial evaluation to renal dysfunction (Table). No child with normal presenting renal function thus far has progressed to require dialysis or transplantation. DISCUSSION: Previous literature estimated rates of renal dysfunction in cloaca patients as high as 50%; in contrast, we demonstrate a rate of progression to renal dysfunction of 2.9% in girls following a strict renal protection protocol. Most children who developed renal dysfunction had dysfunctional kidneys on presentation. This suggests that preservation of renal function may be possible in early childhood with a strict, multi-disciplinary renal protection protocol. CONCLUSION: In our cohort of patients with cloacal malformations following a strict renal protection protocol, incidence of progressive renal dysfunction is low at 2.9%. Most who go on to renal dysfunction present with impaired renal function.

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.

Patient-Reported Experience in Colorectal Disease is Predicted by Cleanliness.

BACKGROUND: Children with colorectal diseases such as anorectal malformations (ARM), Hirschsprung disease (HD), and functional constipation (FC) undergo bowel management programs (BMPs) to achieve cleanliness. While patient outcomes, such as cleanliness and quality of life, are well understood, patient experience, such as relationships, ability to participate in sports, and independence and self-confidence is less well understood. We aimed to assess the relationship between BMP and patient experience. METHODS: A cross-sectional survey was administered to 295 patients ≥3 years old with ARM, HD, and FC completing BMP. The survey contains 22 questions regarding patient-reported experience measures (PREMs) and 11 regarding patient-reported outcomes measures (PROMs). Each was graded on a Likert scale, with higher scores meaning better experience. Scores were compared by demographics and clinical characteristics and logistic regression was performed controlling for clinically significant variables. A p-value of ≤0.05 was significant. RESULTS: There were 205 eligible respondents (69.5%) with a median age of 8.9 years [IQR: 6.1-12.4]. ARM was most common (51.2%) and most achieved cleanliness on BMP (69.3%). There were no differences in experience scores by age, diagnosis, or bowel regimen. Patients that were clean had significantly higher PREM scores (67.7 [IQR: 64.0-83.0] vs. 64.8 [IQR: 55.0-70.1], p = 0.0002) and PROM scores (36.8 [IQR: 33.0-41.0] vs. 34.0 [31.0-38.5], p = 0.005). On regression analysis, cleanliness remained a strongly significant predictor of positive experience scores (ß 7.37, SE 1.86, p < 0.0001). CONCLUSIONS: Achieving cleanliness was associated with positive patient experience of bowel management programs. This finding suggests that achieving cleanliness, regardless of regimen, may allow patients the best functional and experiential outcomes.

Laxative Weaning Protocol for Patients With Functional Constipation: A Pilot Study.

BACKGROUND: Children with functional constipation require prolonged laxative administration for proper emptying. Whether these laxatives can be weaned after better functioning is achieved is unknown. We aim to describe a standardized protocol for stimulant laxative weaning and its early outcomes. METHODS: Patients were candidates for weaning if they had been on a stable laxative dose for six months, defined as one bowel movement per day with no soiling, impaction, or enemas. Laxative dose was decreased by 10-25% with re-evaluation every two weeks. If patients remained well without constipation, dose was weaned further by 10-25%. If there were worsening of symptoms, lower dose was maintained for 3-6 months until re-evaluation. RESULTS: There were a total of sixteen patients evaluated. Median age was 12.7 years [IQR: 11.7-15.3] with laxative duration of 8.0 years [IQR: 5.4-10.7]. All patients were on senna; some were on fiber. Median starting senna dose was 71.3 mg [IQR: 54.3-75.0] and median fiber dose was 5.5 g [IQR: 4.0-6.0]. As of most recent follow up, nine patients (56.3%) had weaned off laxatives in 3.7 months [IQR: 1.3-11.6]. For those still on laxatives, median reduction in dose was 41.4 mg [30.0-75.0], and over half weaned their dose by >50%. Almost all (90.9%) of those on high doses were able to wean. CONCLUSION: A standardized laxative weaning process can be successful in patients with functional constipation, especially on high doses. Further prospective studies will be necessary to confirm the success of this protocol. LEVEL OF EVIDENCE: III.

Bladder Management and Continence in Girls With Cloacal Malformation After 3 Years of Age.

BACKGROUND: Multiple factors impact ability to achieve urinary continence in cloacal malformation including common channel (CC) and urethral length and presence of spinal cord abnormalities. Few publications describe continence rates and bladder management in this population. We evaluated our cohort of patients with cloacal malformation to describe the bladder management and continence outcomes. METHODS: We reviewed a prospectively collected database of patients with cloacal malformation managed at our institution. We included girls ≥3 years (y) of age and evaluated their bladder management methods and continence. Dryness was defined as <1 daytime accident per week. Incontinent diversions with both vesicostomy and enterovesicostomy were considered wet. RESULTS: A total of 152 patients were included. Overall, 93 (61.2%) are dry. Nearly half (47%) voided via urethra, 65% of whom were dry. Twenty patients (13.1%) had incontinent diversions. Over 40% of the cohort performed clean intermittent catheterization (CIC), approximately half via urethra and half via abdominal channel. Over 80% of those performing CIC were dry. In total, 12.5% (n = 19) required bladder augmentation (BA). CC length was not associated with dryness (p = 0.076), need for CIC (p = 0.253), or need for abdominal channel (p = 0.497). The presence of a spinal cord abnormality was associated with need for CIC (p = 0.0117) and normal spine associated with ability to void and be dry (p = 0.004) CONCLUSIONS: In girls ≥ 3 y of age with cloacal malformation, 61.2% are dry, 65% by voiding via urethra and 82% with CIC. 12.5% require BA. Further investigation is needed to determine anatomic findings associated with urinary outcomes. LEVEL OF EVIDENCE: IV.

Development of a Standardized Process for Transition to Adult Care in a Pediatric Colorectal Surgery Clinic.

BACKGROUND: As pediatric patients with colorectal diseases grow, it is important to address transition to adult practice. We aim to describe our center's transition process and early outcomes. METHODS: We developed a standardized process for transition to adult practice. An annual survey is given to parents and caregivers starting at age 12 that assesses knowledge of disease, independence with healthcare tasks, and confidence and interest regarding transition. After multidisciplinary review, those eligible are recommended for transition. Those not referred are provided with tools to help with areas of weakness. Outcomes were analyzed with descriptive and regression analyses (significance at p ≤ 0.05). RESULTS: A total of 116 patients were evaluated, with 80 patients (69.0%) recommended for transition. Median age at survey was 15.5 years [IQR: 13.7-18.1], and those recommended were older (16.6 years [IQR: 14.7-19.4] vs 13.5 years [IQR: 12.5-14.9], p < 0.001)). Primary diagnosis and gender were not associated with recommendation for transition. Overall, a minority (18.1%) were able to complete healthcare tasks; this correlated strongly with transition recommendation (26.3% vs 0.0%, p < 0.0001). On regression controlling for age, diagnosis, knowledge, and confidence, age (aOR 1.98, 95% CI 1.44-2.71) and confidence (aOR 3.78, 95% CI 1.29-11.11) independently predicted transition recommendation. CONCLUSION: A standardized approach may be effective in transitioning patients from pediatric to adult colorectal surgery practice. Patients who transition are more confident and can perform healthcare tasks independently; however, these skills are not essential prior to a recommendation of transition. LEVEL OF EVIDENCE: III.

Rates of Hirschsprung-Associated Enterocolitis Decrease With Increasing Child Opportunity Index.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in patients with Hirschsprung disease (HD). There is a correlation between social determinants of health (SDOH) and outcomes in children with HD. The Child Opportunity Index (COI) is a publicly available dataset that stratifies patients by address into levels of opportunity. We aimed to understand if a relationship exists between COI and HAEC. METHODS: A single-institution, IRB-approved, retrospective cohort study was performed of children with HD. Census tract information was used to obtain COI scores, which were stratified into categories (very low, low, medium, high, very high). Subgroups with and without history of HAEC were compared. RESULTS: The cohort had 100 patients, of which 93 had a COI score. There were 27 patients (29.0%) with HAEC. There were no differences in demographics or clinical factors, including length of aganglionic colon, operative approach, and age at pull-through. As child opportunity score increased from very low to very high, there was a statistically significant decrease in the incidence of HAEC (p = 0.04). CONCLUSION: We demonstrate a significant association between increasing opportunity and decreasing incidence of HAEC. This suggests an opportunity for targeted intervention in populations with low opportunity. LEVEL OF EVIDENCE: III. IRB NUMBER: IRB14-00232.

Development of a Standardized Algorithm for Management of Newly Diagnosed Anorectal Malformations.

Neonates with a new diagnosis of anorectal malformation (ARM) present a unique challenge to the clinical team. ARM is strongly associated with additional midline malformations, such as those observed in the VACTERL sequence, including vertebral, cardiac, and renal malformations. Timely assessment is necessary to identify anomalies requiring intervention and to prevent undue stress and delayed treatment. We utilized a multidisciplinary team to develop an algorithm guiding the midline workup of patients newly diagnosed with ARM. Patients were included if born in or transferred to our neonatal intensive care unit (NICU), or if seen in clinic within one month of life. Complete imaging was defined as an echocardiogram, renal ultrasound, and spinal magnetic resonance imaging or ultrasound within the first month of life. We compared three periods: prior to implementation (2010-2014), adoption period (2015), and delayed implementation (2022); p ≤ 0.05 was considered significant. Rates of complete imaging significantly improved from pre-implementation to delayed implementation (65.2% vs. 50.0% vs. 97.0%, p = 0.0003); the most growth was observed in spinal imaging (71.0% vs. 90.0% vs. 100.0%, p = 0.001). While there were no differences in the rates of identified anomalies, there were fewer missed diagnoses with the algorithm (10.0% vs. 47.6%, p = 0.05). We demonstrate that the implementation of a standardized algorithm can significantly increase appropriate screening for anomalies associated with a new diagnosis of ARM and can decrease delayed diagnosis. Further qualitative studies will help to refine and optimize the algorithm moving forward.

Unusual Management of Fecal Impaction in an Adult With Functional Constipation: A Urological Adventure.

A 27-year-old male with a history of functional constipation presented for ileostomy closure. He had a 12-cm impacted rectal stool ball precluding safe ileostomy takedown. He underwent multiple unsuccessful attempts at removal, including colotomy, antegrade and retrograde enemas, and manual disimpaction. The urological team suggested a novel approach using lithotripsy. A 26-French rigid nephroscope and the Olympus ShockPulse SE ultrasonic lithotripter were utilized transanally to break up the impacted stool ball. The patient was discharged the same day without complication. Ultrasonic lithotripsy is an unusual yet effective modality for prolonged fecal impaction. By employing a unique, multidisciplinary technique, operative morbidity was avoided.

Using chest X-ray to predict tube thoracostomy in traumatic pneumothorax: A single-institution retrospective review.

BACKGROUND: Traumatic pneumothorax (PTX) is a common occurrence in thoracic trauma patients, with a majority requiring tube thoracostomy (TT) for management. Recently, the "35-mm" rule has advocated for observation of patients with PTX less than 35 mm on chest computed tomography (CT) scan. This rule has not been examined in chest x-ray (CXR). We hypothesize that a similar size cutoff can be determined in CXR predictive of need for tube thoracostomy. METHODS: We performed a single-institution retrospective review of patients with traumatic PTX from 2018 to 2022, excluding those who underwent TT prior to CXR. Primary outcomes were size of pneumothorax on CXR and need for TT; secondary outcome was failed observation, defined as TT more than 4 hours after presentation. To determine the size cutoff on CXR to predict TT need, area under the receiver operating curve (AUROC) analyses were performed and Youden's index calculated (significance at p < 0.05). Predictors of failure were calculated using logistic regression. RESULTS: There were 341 pneumothoraces in 304 patients (94.4% blunt trauma, median injury severity score 14). Of these, 82 (24.0%) had a TT placed within the first 4 hours. Fifty-five of observed patients (21.2%) failed, and these patients had a larger PTX on CXR (8.6 mm [5.0-18.0 mm] vs. 0.0 mm [0.0-2.3 mm] ( p < 0.001)). Chest x-ray PTX size correlated moderately with CT size (r = 0.31, p < 0.001) and was highly predictive of need for TT insertion (AUC 0.75, p < 0.0001), with an optimal size cutoff predicting TT need of 38 mm. CONCLUSION: Chest x-ray imaging size was predictive of need for TT, with an optimal size cutoff on CXR of 38 mm, approaching the "35-mm rule." In addition to size, failed observation was predicted by presenting lactic acidosis and need for supplemental oxygen. This demonstrates this cutoff should be considered for prospective study in CXR. LEVEL OF EVIDENCE: Therapeutic/Care Management; Level IV.

Outcomes of children with constipation and autism spectrum disorder treated with antegrade continence enemas.

BACKGROUND: Treatment of functional constipation (FC) in children with autism spectrum disorder (ASD) is challenging due to sensory and behavioral issues. We aimed to understand whether antegrade continence enemas (ACEs) are successful in the treatment of FC in children with ASD. METHODS: A single-institution retrospective review was performed in children diagnosed with ASD and FC who underwent appendicostomy or cecostomy placement from 2007 to 2019. Descriptive statistics regarding soiling and complications were calculated. RESULTS: There were 33 patients included, with a median age of 9.7 years at the time of ACE initiation. The average intelligence quotient was 63.6 (SD = 18.0, n = 12), the average behavioral adaptive score was 59.9 (SD = 11.1, n = 13), and the average total Child Behavioral Checklist score was 72.5 (SD = 7.1, n = 10). Soiling rates were significantly lower following ACE initiation (42.3% vs. 14.8%, p = 0.04). Behavioral issues only prevented 1 patient (3.0%) from proper ACE use. Eleven patients (36.6%) were able to transition to laxatives. There were significant improvements in patient-reported outcomes measures and quality of life. CONCLUSION: Placement of an appendicostomy or cecostomy for management of FC in children with severe ASD was successful in treating constipation and improving quality of life.

Social Determinants of Health Are Associated with Failed Bowel Management for Children with Anorectal Malformations.

INTRODUCTION: Children with anorectal malformations (ARMs) benefit from bowel management programs (BMPs) to manage constipation or fecal incontinence. We aimed to understand the role of social determinants of health (SDOH) in outcomes following BMPs in this population. MATERIALS AND METHODS: A single-institution, institutional review board (IRB) approved, retrospective review was performed in children with ARM who underwent BMP from 2014 to 2021. Clinical, surgical, and SDOH data were collected. Children were stratified as clean or not clean per the Rome IV criteria at the completion of BMP. Descriptive statistics were computed. Categorical variables were analyzed via Fisher's exact tests and continuous variables with Mood's median tests. RESULTS: In total, 239 patients who underwent BMP were identified; their median age was 6.62 years (interquartile range [IQR]: 4.78-9.83). Of these, 81 (34%) were not clean after completing BMP. Children with prior history of antegrade enema procedures had a higher rate of failure. Children who held public insurance, lived within driving distance, had unmarried parents, lived with extended family, and lacked formal support systems had a significant association with BMP failure (p < 0.05 for all). Type of ARM, age at repair, type of repair, age at BMP, and type of BMP regimen were not significantly associated with failure. CONCLUSIONS: There is a significant correlation of failure of BMPs with several SDOH elements in patients with ARM. Attention to SDOH may help identify high-risk patients in whom additional care may lead improved outcomes following BMP.

Surgical Apgar scores predict complications after emergency general surgery laparotomy.

BACKGROUND: The Surgical Apgar Score (SAS) is a 10-point validated score comprised of three intraoperative variables (blood loss, lowest heart rate, and lowest mean arterial pressure). Lower scores are worse and predict major postoperative complications. The SAS has not been applied in emergency general surgery (EGS) but may help guide postoperative disposition. We hypothesize that SAS can predict complications in EGS patients undergoing a laparotomy. METHODS: We performed a retrospective review of adult patients at a single, quaternary care center who underwent an exploratory laparotomy for EGS conditions within 6 hours of surgical consultation from 2015 to 2019. Patients were grouped by whether they experienced a postoperative complication (systemic, surgical, and/or death). Multivariable regression was performed to predict complications, accounting for SAS and other statistically significant variables between groups. Using this model, predicted probabilities of a complication were generated for each SAS. RESULTS: The cohort comprised 482 patients: 32.8% (n = 158) experienced a complication, while 67.2% (n = 324) did not. Patients with complications were older, frailer, more often male, had worse SAS (6 vs. 7, p < 0.0001) and American Society of Anesthesiologists scores, and higher rates of perforated hollow viscus ( p = 0.0003) and open abdomens ( p < 0.0001). On multivariable regression, an increasing SAS independently predicted less complications (adjusted odds ratio, 0.85; 95% confidence interval, 0.75-0.96; p = 0.009). An SAS ≤4 was associated with a 49.2% predicted chance of complications, greater rates of septic shock (9.7% vs. 3%, p = 0.01), respiratory failure (20.5% vs. 10.8%, p = 0.02), and death (24.1% vs. 7.5%, p < 0.0001). An SAS ≤ 4 did not correlate with surgical complications ( p = 0.1). CONCLUSION: The SAS accurately predicts postoperative complications in EGS patients undergoing urgent laparotomy, with an SAS ≤ 4 identifying patients at risk for septic shock, respiratory failure, and mortality. This tool can aid in rapidly determining postoperative disposition and resource allocation. LEVEL OF EVIDENCE: Therapeutic/Care Management; Level IV.

Duhamel Versus Swenson Pull-Through for Total Colonic Aganglionosis: A Multi-Institutional Study.

BACKGROUND: Total colonic aganglionosis (TCA) is a rare variant of Hirschsprung disease (HD) where the colon and portion of distal ileum lack ganglion cells. Most pediatric use either a straight ileoanal (Swenson or Yancey-Soave) or a short Duhamel pull-through for TCA. There are no large studies comparing these techniques. We aimed to compare short-and medium-term outcomes between these approaches. METHOD: A retrospective review was performed among children with TCA from 2001 to 2019 undergoing a primary Duhamel or Swenson pull-through across three large children's hospitals. Patients undergoing redo and patients with greater than 30 % small bowel aganglionosis were excluded. We gathered data on demographics, operative approach, and outcomes at one, two, and three years. Continuous variables were analyzed with t-tests and categorical variables with Chi square or Fisher's tests. RESULTS: There were 54 patients, with 26 (48 %) undergoing Duhamel and 28 (52 %) undergoing Swenson pull-through. There were no differences in sex, age, medical comorbidities, or operative details, including age at pull-through, laparoscopic vs open, length of involved small bowel, and operative time. Length of stay and post-operative complications were not different. Three years after pull-through, patients undergoing Duhamel had fewer stools per day (1-3 stools 69.6 % vs 14.3 %, p = 0.003) and were less likely to be prescribed fiber supplementation (4.2 % vs 43.8 %, p = 0.003). There were no differences in irrigations, botulinum toxin administration, loperamide, or HD admissions. CONCLUSION: Both Duhamel and straight pull-throughs are safe for treatment of TCA, with acceptable short- and medium-term outcomes. Further studies on patient-reported outcomes are necessary to examine long-term differences. LEVEL OF EVIDENCE: III.

The effect of amantadine on acute cognitive disability after severe traumatic brain injury: An institutional pilot study.

BACKGROUND: Amantadine is used in the post-acute care setting to improve cognitive function after a traumatic brain injury. Its utility in the acute postinjury period is unknown. In this pilot study, we sought to examine the effect of amantadine on short-term cognitive disability among patients with a severe traumatic brain injury and hypothesized that patients receiving amantadine would have a greater improvement in disability throughout their acute hospitalization. METHODS: We performed a prospective, observational study of patients ≥18 years with severe traumatic brain injury (Glasgow Coma Scale ≤8) at a level I trauma center between 2020 and 2022. Patients with penetrating trauma, death within 48 hours of admission, and no radiographic evidence of intracranial pathology were excluded. Patients were grouped according to whether they received amantadine. Our primary outcome was the change in cognitive disability, measured by the Disability Rating Scale (DRS), over the index hospitalization. RESULTS: There were 55 patients in the cohort: 41.8% (n = 23) received amantadine and 58.2% (n = 32) did not. There were higher rates of motor vehicle collisions (65.2% vs 46.9%, P = .02), diffuse axonal injury (47.8% vs 18.8%, P = .02), intracranial pressure monitor use (73.9% vs 21.9%, P = .0001), and propranolol use (73.9% vs 21.9%, P = .0001) in the amantadine. There was a larger improvement in DRS scores among patients receiving amantadine (7.8 vs 3.6, P = .001), and amantadine independently predicted improvement in DRS scores (ß, 1.61; 95% confidence interval, 0.20-3.02, P = .03). Rates of discharge to traumatic brain injury rehabilitation were significantly higher in the amantadine group (73.9% vs 21.9%, P = .0002). CONCLUSION: Among patients with severe traumatic brain injury, amantadine use in the acute postinjury period may be associated with an improvement in cognitive disability and discharge to traumatic brain injury rehabilitation.