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.

Approach and Technique for Cesarean Section to Immediate Resection for High-Risk Sacrococcygeal Teratomas.

INTRODUCTION: Ex-utero intrapartum treatment has been established as an option for fetal and perinatal surgeons to deliver patients with sacrococcygeal teratomas (SCTs) which are causing significant fetal distress and possible in-utero fetal demise. However, ex-utero intrapartum treatment procedures carry significant maternal risk and morbidity. Herein, we report an alternative technique of Cesarean section to immediate resection (CSIR) for managing high-risk SCTs. METHODS: A retrospective institutional review board-approved review was performed on all SCTs evaluated at our fetal center from May 2014 to September 2020. Demographics; prenatal imaging characteristics; prenatal interventions; and postnatal surgery data including operative time, estimated blood loss, pathology, and outcomes were collected. Outcomes of interest included surveillance serum alpha-fetoprotein levels, imaging surveillance, developmental milestones, and the presence or absence of constipation or fecal incontinence. RESULTS: A total of 20 patients with prenatal diagnosis of SCT were evaluated. Mothers who transferred their care to another institution after diagnosis were excluded from this study. Twelve neonates underwent standard postnatal resection. Three neonates underwent emergent CSIR for high output cardiac failure, fetal anemia, or concerns for in-utero hemorrhagic rupture. The median (interquartile range) operative time was 231.5 (113) minutes for the standard operative group versus 156 min in the CSIR group. We present three patients who underwent immediate resection after emergent Cesarean section. We report 100% survival for the three consecutive cases. CONCLUSIONS: CSIR is a safe and feasible approach for managing appropriately selected high-risk SCTs with signs of hydrops, fetal distress, or fetal anemia. Despite patient prematurity, we demonstrated 100% survival of three consecutive cases. We suggest that CSIR be considered an option in the management algorithm for high-risk SCTs.

Examining the Utility of Preoperative Telemedicine Care Across Multiple Pediatric Surgery Disciplines.

INTRODUCTION: Telemedicine use within pediatric surgery fields has been growing, but research on the utility of remote evaluation in the perioperative period remains scarce. The objective of this study was to examine the utility of perioperative telemedicine care for the pediatric patient by evaluating the outcomes following completion of an outpatient appointment with a surgical provider. MATERIALS AND METHODS: We performed a retrospective chart review of all patients who completed a telemedicine appointment with a provider across nine pediatric surgery divisions, without a limitation based on patient-specific characteristics or telemedicine platform. We examined the result of the initial telemedicine appointment and the outcome of any surgical procedure that was performed as a result. RESULTS: A total of 803 patients were evaluated by telemedicine during the study period. Of the 164 encounters (20.2%) that were followed by a surgery, nearly 70% were performed using a video. There was no discordance in the preoperative and postoperative diagnoses for more than 98% of patients. Nearly 25% of operations were followed by at least a 1-night hospital stay and 6.7% of patients developed a postoperative complication. CONCLUSIONS: Telemedicine is a safe tool for evaluating pediatric patients in the preoperative and postoperative phases of care and offers potential value for families seeking an alternative to the traditional in-person appointment. Ongoing support will require permanent legislative changes aimed at ensuring comparable compensation and the development of strategies to adapt the outpatient healthcare model to better accommodate the evolving requirements of remotely evaluating and treating pediatric patients.

Impact of Neurodevelopmental Disorders on Bowel Management Outcomes in Children with Functional Constipation.

OBJECTIVES: Patients experiencing functional constipation (FC) can participate in structured bowel management programs (BMPs) to manage constipation or fecal incontinence when standard management fails. We sought to evaluate the efficacy of BMPs for children with FC with and without neurodevelopmental disorders. METHODS: We performed a retrospective review of children with FC who participated in our BMP from 2014 to 2021. Stool/urinary continence, bowel regimen, surgical history, parent-reported outcomes measures (PROMs: Cleveland Clinic Constipation Score, Baylor Continence Scale, Vancouver Symptom Score for Dysfunctional Elimination), and Pediatric Quality of Life Inventory (PedsQL) were assessed pre- and at least 9 months post-BMP. RESULTS: The cohort included 156 patients with a median age of 9 years and follow-up of 627 days (IQR: 389-808 days). Two sub-cohorts included patients with FC only (69%) and FC plus a neurodevelopmental disorder (31%): 59% attention-deficit/hyperactivity disorder, 33% autism spectrum disorder, and 8% obsessive-compulsive disorder. Both groups had significantly improved follow-up bowel movement frequency and continence (39%-90% neurodevelopmental, 44%-82% FC only, P < 0.001) and urinary continence (65%-90% neurodevelopmental, 69%-91% FC only, P < 0.02). There was a significant improvement in most of the PROMs at follow-up. Both groups experienced a clinically meaningful improvement in overall PedsQL scores (pre- and postBMP difference of >4.5). CONCLUSIONS: Patients with FC with and without a neurodevelopmental disorder had significant improvement in stool and urinary continence after undergoing a BMP. Further studies are needed to see if this improvement is durable over a longer period of time in this challenging cohort.

Recognizing the Benefit of Telemedicine Before and After COVID-19: A Survey of Pediatric Surgery Providers.

BACKGROUND: Prior to the COVID-19 pandemic, the use of telemedicine to evaluate pediatric surgery patients was uncommon. Due in part to restrictions imposed to mitigate the spread of the virus, the use of telemedicine within pediatric surgery has significantly expanded. METHODS: Prior to the use of telemedicine within surgery divisions at our institution, pediatric surgeons were surveyed to determine their perspectives on the use of telemedicine. Following the expanded use of telemedicine in response to the COVID-19 pandemic, a follow up survey was distributed to determine the impact of telemedicine and the perceived benefits and barriers of continuing its use going forward. RESULTS: The pre-COVID survey was completed by 37 surgeons and the post-COVID survey by 36 surgeons and advanced practice providers across 10 pediatric surgical divisions. General surgeons were the most represented division for both the pre- (25%) and post-COVID (33.3%) survey. Less than 25% of providers reported use of telemedicine at any point in their career prior to COVID-19; but following the expanded use of telemedicine 95% of respondents reported interest in continuing its use. After expansion, 25% of respondents were concerned with the possibility of inaccurate diagnoses when using telemedicine compared to nearly 50% prior to expanded use. CONCLUSION: Following the expanded use of telemedicine within pediatric surgery, there was a decrease in the concern for inaccurate diagnoses and a near uniform desire to continue its use. Going forward, it will be imperative for pediatric surgeons to take an active role in creating a process for implementing telemedicine that best fits their needs and the needs of their patients and patients' families.

Review at a multidisciplinary tumor board impacts critical management decisions of pediatric patients with cancer.

BACKGROUND: Optimal cancer care requires a multidisciplinary approach. The purpose of the current study was to evaluate the impact of a multidisciplinary tumor board on the treatment plans of children with solid tumors. PROCEDURES: The records of 158 consecutive patients discussed at a formal multidisciplinary pediatric tumor board between July 2012 and April 2014 were reviewed. Treatment plans were based on clinical practice guidelines and on current Children's Oncology Group protocols. Alterations in radiologic, pathologic, surgical, and medical interpretations were analyzed to determine the impact on changes in recommendations for clinical management. RESULTS: Overall, 55 of 158 children (35%) had alterations in radiologic, pathologic, medical, or surgical interpretation of clinical data following multidisciplinary discussion. Of these, 64% had changes to the initial recommendation for clinical management. Review of imaging studies resulted in interpretation changes in 30 of 158 patients studied (19%), with 12 clinical management changes. Six of 158 patients (3.9%) had changes in pathologic interpretation, with four patients (2.5%) requiring treatment changes. In eight patients (5%), a change in medical management was recommended, while in 11 patients (7%) there were changes in surgical management that were based solely on discussion and not on interpretation of imaging or pathology. CONCLUSIONS: Formal multidisciplinary review led to alterations in interpretation of clinical data in 35% of patients, and the majority led to changes in recommendations for treatment. Comprehensive multidisciplinary tumor board incorporated into the care of children with cancer provides additional perspectives for families and care providers when delineating optimal treatment plans.

Giant aortic aneurysm in a child with Takayasu arteritis.

Takayasu arteritis is a chronic, idiopathic, granulomatous vasculitis involving the aorta, its major branches, and occasionally the pulmonary arteries. Although rare in children, it is the third most common vasculitis in the paediatric population. Although aneurysmal disease has been reported in adults with Takayasu arteritis, it is a rare entity in children. We present the case of a 10-year-old boy with a giant ascending and arch aneurysm that necessitated follow-up surgery for a new aneurysm and occlusive disease. This is also the first published case involving endovascular aortic graft placement for the management of vascular sequela of Takayasu arteritis in a child.

Surgical Management of Severe Colitis in the Intensive Care Unit.

Severe colitis, an umbrella encompassing several entities, is one of the most common acute gastrointestinal disorders resulting in critical illness. Clostridium difficile infection is responsible for the majority of nosocomial diarrhea with fulminant C difficile colitis (CDC) carrying a high mortality. Optimal outcomes can be achieved by early identification and treatment of fulminant CDC, with appropriate surgical intervention when indicated. Ischemic colitis, on the other hand, is uncommon with a range of etiological factors including abdominal aortic surgery, inotropic drugs, rheumatoid diseases, or often no obvious triggering factor. Most cases resolve with nonsurgical management; however, prompt recognition of full-thickness necrosis and gangrene is crucial for good patient outcomes. Fulminant colitis is a severe disease secondary to progressive ulcerative colitis with systemic deterioration. Surgical intervention is indicated for hemorrhage, perforation, or peritonitis and failure of medical therapy to control the disease. Although, failure of medical management is the most common indication, it can be difficult to define objectively and requires a collaborative multidisciplinary approach. This article proposes some simple management algorithms for these clinical entities, with a focus on critically ill patients.

Obstructive jaundice secondary to Morgagni hernia in an infant with Fontan circulation.

The vast majority of congenital diaphragmatic cases present with severe respiratory distress in the immediate neonatal period. Obstructive jaundice secondary to CDH is a rare presenting symptom, previously unreported with Morgagni type hernias which comprise 2% of all CDHs. Although the safety of minimally invasive repair for CDH has been shown previously, there has been little data on its safety for patients with complex congenital cardiac disease. In this case report, we present an 18-month old infant with Fontan circulation presenting with jaundice secondary to Morgagni hernia who ultimately underwent laparoscopic repair. A brief discussion of the safety of laparoscopic surgery in patients post-Fontan is included.

Valproic acid for the treatment of hemorrhagic shock: a dose-optimization study.

BACKGROUND: Valproic acid (VPA) has been shown to improve survival in animal models of hemorrhagic shock at a dose of 300 mg/kg. Our aim was to identify the ideal dose through dose-escalation, split-dosing, and dose de-escalation regimens. MATERIALS AND METHODS: Rats were subjected to sublethal 40% hemorrhage and treated with vehicle or VPA (dose of 300, 400, or 450 mg/kg) after 30 min of shock. Acetylated histones and activated proteins from the PI3K-Akt-GSK-3ß survival pathway at different time points were quantified by Western blot analysis. In a similar model, a VPA dose of 200 mg/kg followed 2 h later by another dose of 100 mg/kg was administered. Finally, animals were subjected to a lethal 50% hemorrhage and VPA was administered in a dose de-escalation manner (starting at dose of 300 mg/kg) until a significant drop in percent survival was observed. RESULTS: Larger doses of VPA resulted in greater acetylation of histone 3 and increased activation of PI3K pathway proteins. Dose-dependent differences were significant in histone acetylation but not in the activation of the survival pathway proteins. Split-dose administration of VPA resulted in similar results to a single full dose. Survival was as follows: 87.5% with 300 and 250 mg/kg of VPA, 50% with 200 mg/kg of VPA, and 14% with vehicle-treated animals. CONCLUSIONS: Although higher doses of VPA result in greater histone acetylation and activation of prosurvival protein signaling, doses as low as 250 mg/kg of VPA confer the same survival advantage in lethal hemorrhagic shock. Also, VPA can be given in a split-dose fashion without a reduction in its cytoprotective effectiveness.

Selective inhibition of histone deacetylase 6 alters the composition of circulating blood cells in a lethal septic model.

BACKGROUND: Phagocytes, especially monocytes, macrophages, and dendritic cells, play a pivotal role in the innate and adaptive immune responses during sepsis. We have shown that inhibition of histone deacetylase 6 improves survival and increases bacterial clearance in a mouse model of cecal ligation and puncture (CLP). The aim of this study was to determine whether this effect was associated with changes in the number and composition of different blood cell types in the circulation. METHODS: C57BL/6J mice were subjected to CLP, and 1 h later given an intraperitoneal injection of either Tubastatin A dissolved in dimethyl sulfoxide, or dimethyl sulfoxide only. Sham-operated animals were treated in an identical fashion but not subjected to CLP. Forty-eight hours later, peripheral blood was obtained via cardiac puncture and analyzed using a HemaTrue veterinary hematology analyzer. RESULTS: Tubastatin A administration increased the number of circulating monocytes in the sham-operated and the CLP animals. In comparison with the sham, CLP animals displayed an increase in the granulocyte percentage in white blood cells and decrease in the lymphocyte number and percentage, with a resultant increase in the granulocyte-to-lymphocyte ratio. Treatment of CLP animals with Tubastatin A decreased the granulocyte percentage and restored the lymphocyte number and percentage, which decreased the granulocyte-to-lymphocyte ratio. In the sham animals, Tubastatin A increased red blood cell number, hematocrit, and hemoglobin. This effect was not seen in CLP animals. CONCLUSIONS: Tubastatin A treatment has significant impact on the composition of circulating blood cells. It increases the number of circulating monocytes and the red blood cell mass in sham-operated animals. In the CLP animals, it increases the monocyte count, decreases the percentage of granulocytes, restores the lymphocyte population, and decreases the granulocyte-to-lymphocyte ratio. These results may explain why Tubastatin A treatment improves survival in the septic models.

Treatment with a histone deacetylase inhibitor, valproic acid, is associated with increased platelet activation in a large animal model of traumatic brain injury and hemorrhagic shock.

BACKGROUND: We have previously shown that resuscitation with fresh frozen plasma (FFP) in a large animal model of traumatic brain injury (TBI) and hemorrhagic shock (HS) decreases the size of the brain lesion, and that addition of a histone deacetylase inhibitor, valproic acid (VPA), provides synergistic benefits. In this study, we hypothesized that VPA administration would be associated with a conservation of platelet function as measured by increased platelet activation after resuscitation. MATERIALS AND METHODS: Ten swine (42-50 kg) were subjected to TBI and HS (40% blood loss). Animals were left in shock for 2 h before resuscitation with either FFP or FFP+VPA (300 mg/kg). Serum levels of platelet activation markers transforming growth factor beta, CD40 L, P-selectin, and platelet endothelial cell adhesion molecule (PECAM) 1 were measured at baseline, postresuscitation, and after a 6-h observation period. Platelet activation markers were also measured in the brain whole cell lysates and immunohistochemistry. RESULTS: Circulating P-selectin levels were significantly higher in the FFP+VPA group compared with the FFP alone group (70.85±4.70 versus 48.44±7.28 ng/mL; P<0.01). Likewise, immunohistochemistry data showed elevated P-selectin in the VPA treatment group (22.30±10.39% versus 8.125±3.94%, P<0.01). Serum sCD40L levels were also higher in the FFP+VPA group (3.21±0.124 versus 2.38±0.124 ng/mL; P<0.01), as was brain sCD40L levels (1.41±0.15 versus 1.22±0.12 ng/mL; P=0.05). Circulating transforming growth factor beta levels were elevated in the FFP+VPA group, but this did not reach statistical significance (11.20±1.46 versus 8.09±1.41 ng/mL; P=0.17). Brain platelet endothelial cell adhesion molecule 1 levels were significantly lower in the FFP+VPA group compared with the FFP group (5.22±2.00 pg/mL versus 7.99±1.13 pg/mL; P=0.03). CONCLUSIONS: In this clinically relevant large animal model of combined TBI+HS, the addition of VPA to FFP resuscitation results in an early upregulation of platelet activation in the circulation and the brain. The previously observed neuroprotective effects of VPA may be due to a conservation of platelet function as measured by a higher platelet activation response after resuscitation.

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.

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.

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.

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.

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.

Hirschsprung-associated enterocolitis: pathogenesis, treatment and prevention.

Hirschsprung-associated enterocolitis (HAEC) is a common and sometimes life-threatening complication of Hirschsprung disease (HD). Presenting either before or after definitive surgery for HD, HAEC may manifest clinically as abdominal distension and explosive diarrhea, along with emesis, fever, lethargy, and even shock. The pathogenesis of HAEC, the subject of ongoing research, likely involves a complex interplay between a dysfunctional enteric nervous system, abnormal mucin production, insufficient immunoglobulin secretion, and unbalanced intestinal microflora. Early recognition of HAEC and preventative practices, such as rectal washouts following a pull-through, can lead to improved outcomes. Treatment strategies for acute HAEC include timely resuscitation, colonic decompression, and antibiotics. Recurrent or persistent HAEC requires evaluation for mechanical obstruction or residual aganglionosis, and may require surgical treatment with posterior myotomy/myectomy or redo pull-through. This chapter describes the incidence, pathogenesis, treatment, and preventative strategies in management of HAEC.

Social Determinants of Health and Hirschsprung-associated Enterocolitis.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality in patients with Hirschsprung disease (HD). The objective of this study was to examine the association of social determinants of health (SDOH) with HAEC. METHODS: A review of patients who underwent primary pull through for HD at our institution from 2014 to 2021 was performed. Clinical, surgical, and SDOH data were collected. HAEC was defined by an international scoring system. Categorical variables were analyzed via Fisher's exact tests and continuous variables with Mood's median tests. RESULTS: One hundred patients were identified with 29 patients (29%) having at least one episode of HAEC during a median follow-up of 31 months (IQR: 11.7-55.7). Children who utilized public transportation for clinic visits, had one or more missed appointments, had any reported safety concerns, were involved with Child Protective Services, had parents/guardians who were not married, lived with people other than their immediate family, or had mothers who reported drug use or lack of prenatal care were found to have a higher likelihood of developing HAEC (p<0.04 for all). Age at HD diagnosis, age at pull through, operative approach, length of aganglionic colon, and Trisomy 21 were not significant predictors of HAEC. CONCLUSIONS: In our series of 100 patients undergoing primary pull through, there was a significant correlation of HAEC with several social determinants of health elements while anatomical and clinical factors were not associated with HAEC. Attention to social determinants of health and identifying high-risk patients may serve to prevent morbidity and mortality from HAEC. LEVEL OF EVIDENCE: III.

A Modification of the Anoplasty Technique during a Posterior Sagittal Anorectoplasty and Anorectal Vaginal Urethroplasty Closure: The Para-U-Stitch to Prevent Wound Dehiscence.

OBJECTIVE: Wound dehiscence after posterior sagittal anorectoplasty (PSARP) or anorectal vaginal urethroplasty (PSARVUP) for anorectal malformation (ARM) is a morbid complication. We present a novel anoplasty technique employing para-U-stitches along the anterior and posterior portions of the anoplasty, which helps buttress the midline U-stitch and evert the rectal mucosa. We hypothesized that, in addition to standardized pre- and postoperative protocols, this technique would lower rates of wound dehiscence. MATERIALS AND METHODS: A retrospective review of patievnts who underwent primary PSARP or PSARVUP with the para-U-stitch technique from 2015 to 2021 was performed. Wound dehiscence was defined as wound disruption requiring operative intervention within 30 days of the index operation. Superficial wound separations were excluded. Descriptive statistics were calculated. The final cohort included 232 patients. RESULTS: Rectoperineal fistula (28.9%) was the most common ARM subtype. PSARP was performed in 75% and PSARVUP in 25%. The majority were reconstructed with a stoma in place (63.4%). Wound dehiscence requiring operative intervention occurred in four patients, for an overall dehiscence rate of 1.7%. The dehiscence rate was lower in PSARPs compared with PSARVUPs (0.6 vs. 5.2%) and lower for reconstruction without a stoma compared with a stoma (1.2 vs. 2.0%). There were additional six patients (2.6%) with superficial wound infections managed conservatively. CONCLUSION: We present the para-U-stitch anoplasty technique, which is an adjunct to the standard anoplasty during PSARP and PSARVUP. In conjunction with standardized pre- and postoperative protocols, this technique can help decrease rates of wound dehiscence in this patient population.