Long-Term Male Sexual Function and Fecal Incontinence Outcomes for Adult Patients with Hirschsprung Disease or Anorectal Malformation

BackgroundThis study sought to characterize sexual function and fecal incontinence related quality of life (QOL) outcomes for adult males with anorectal malformation (ARM) or Hirschsprung's Disease (HD).MethodsWe conducted a cross-sectional survey study of male patients ≥18 years with ARM or HD. Patients were identified from our institutional database, contacted and consented by telephone, and sent a REDCap survey via email. The International Index of Erectile Function (IIEF-5) and Male Sexual Health Questionnaire (MSHQ) evaluated erectile dysfunction (ED) and ejaculatory dysfunction (EjD), respectively. The Cleveland Clinic Incontinence Score (CCIS) and the Fecal Incontinence Quality of Life Scale (FIQLS) assessed fecal incontinence-related outcomes. A linear regression analysis of IIEF-5 scores compared to CCIS scores was used to evaluate for an association between ED and incontinence.ResultsOf 63 patients contacted, 48 completed the survey. The median age for respondents was 22.5 years (IQR 20–25). There were 19 patients with HD and 29 patients with ARM. On the IIEF-5 survey, 35.3% report some level of ED. On the MSHQ-EjD survey, the median score was 14 out of 15 (IQR 10.75–15), indicating few EjD concerns. The median CCIS was 5 (IQR 2.25–7.75) and the median FIQL scores ranged from 2.7 to 3.5 depending on the domain assessed, demonstrating some QOL challenges secondary to fecal incontinence. On linear regression analysis, IIEF-5 and CCIS scores were weakly associated (B = −0.55, p = 0.045).ConclusionsMale adult patients with ARM or HD may have ongoing concerns with sexual function and fecal incontinence.Level of EvidenceLevel 4.Type of StudyCross-Sectional Survey Study.     

Implementation of a Cryoablation-based Pain Management Protocol for Pectus Excavatum

IntroductionThe Nuss repair for pectus excavatum is associated with significant postoperative pain. Our institution developed protocols to standardize pain management for pectus excavatum patients in the immediate postoperative period. We present our experience with protocol implementation and patient outcomes.MethodsWe standardized regional anesthesia with a 0.25% bupivacaine incisional soaker catheter (post-implementation 1, PI1) before transitioning to intercostal nerve cryoablation (INC) (post-implementation 2, PI2). Patient outcomes were tracked using statistical process control charts in AdaptX™ OR Advisor and run charts in Tableau. Chi-squared tests assessed demographic differences between cohorts.Results244 patients were included: 78 pre-implementation, 108 PI1, and 58 PI2. Average age was 15.9–16.5 years. Patients were majority male, non-Hispanic white, and English speaking. Hospital length of stay decreased 4.1–2.4 days. INC increased surgery time (99–125 min) but decreased PACU time (112-78 min). Maximum pain scores improved in PACU (7.7–6.0) and 0–24 h postoperatively (8.3–6.8) but were not different 24–48 h postoperatively (5.4–5.8). Average opioid dosing decreased 0–48 h from 1.9 to 0.8 mg/kg morphine milliequivalents and was associated with reduction in post-operative nausea and constipation. There were no 30-day readmissions.ConclusionAn institution-wide pain management protocol using INC for pectus excavatum patients was implemented. Intercostal nerve cryoablation was found to be superior to bupivacaine incisional soaker catheters and reduced hospital length of stay, immediate postoperative pain scores, morphine milliequivalent opioid dosing, postoperative nausea, and constipation.Level of EvidenceLevel IV.     

Sacral nerve stimulation in the treatment of bowel dysfunction from imperforate anus: A case report

IntroductionBowel dysfunction (fecal incontinence and constipation) presents in over 50% of patients after treatment of congenital anal malformations. Sacral nerve stimulation (SNS) for the treatment of fecal incontinence improves function in the majority of patients. We present a case report of the treatment of bowel dysfunction with sacral nerve stimulation in a patient with a history of an imperforate anus.Presentation of caseA twenty year-old female with a history of imperforate anus at birth, repaired during infancy with anorectoplasty, presented with fecal incontinence and constipation. Since childhood, she had been suffering from intermittent constipation with worsening fecal incontinence in early adulthood. Examination revealed mild anal stenosis and mucosal prolapse. Endoanal ultrasound demonstrated intact internal and external sphincter with low resting and squeeze pressures on anal manometry. Flexible sigmoidoscopy was normal. The patient underwent permanent sacral nerve stimulation with a primary goal of improvement in continence and, secondarily, for the alleviation of intermittent chronic constipation.DiscussionAt 15 month follow-up, the patient had improvement in fecal incontinence (CCIS of 14 pre-SNS to 1 post-SNS), constipation (CCCS of 28 pre-SNS to 20 post-SNS), and quality of life (FIQOL improved in lifestyle (3.7), coping/behavior (3.4), self perception (3.9), and social embarrassment (4.5).ConclusionSacral nerve stimulation for the treatment of bowel dysfunction in adults secondary to imperforate anus can be performed safely and with good results.     

Impact of rectal dissection technique on primary-school-age outcomes for a British and Irish cohort of children with Hirschsprung disease

BackgroundThis prospective cohort study compared primary-school-aged outcomes between children with Hirschsprung disease (HD) following Soave, Duhamel or Swenson procedures.MethodsChildren with histologically proven HD were identified in British/Irish paediatric surgical centers (01/10/2010-30/09/2012). Parent/clinician outcomes were collected when children were 5–8 years old and combined with management/early outcomes data. Propensity score/covariate adjusted multiple-event-Cox and multivariable logistic regression analyses were used.Results277 (91%) of 305 children underwent a pull-through (53% Soave, 37% Duhamel, 9% Swenson). Based upon 259 children (94%) with complete operative data, unplanned reoperation rates (95% CI) per-person year of follow-up were 0.11 (0.08–0.13), 0.34 (0.29–0.40) and 1.06 (0.86–1.31) in the Soave/Duhamel/Swenson groups respectively. Adjusted Hazard Ratios for unplanned reoperation compared with the Soave were 1.50 (95% CI 0.66-3.44, p = 0.335) and 7.57 (95% CI 3.39-16.93, p < 0.001) for the Duhamel/Swenson respectively. Of 217 post-pull-through children with 5–8 year follow-up, 62%, 55%, and 62% in Soave/Duhamel/Swenson groups reported faecal incontinence. In comparison to Soave, Duhamel was associated with lower risk of faecal incontinence (aOR 0.34,95%CI 0.13-0.89,p = 0.028). Of 191 children without a stoma, 42%, 59% and 30% in Soave/Duhamel/Swenson groups required assistance to maintain bowel movements; compared to Soave, the Duhamel group were more likely to require assistance (aOR 2.61,95% CI 1.03–6.60,p = 0.043).ConclusionsCompared with Soave, Swenson was associated with increased risk of unplanned reoperation, whilst Duhamel was associated with reduced risk of faecal incontinence, but increased risk of constipation at 5–8 years of age. The risk profiles described can be used to inform consent discussions between surgeons and parents.Level of evidenceLevel II     

Derivation and Validation of a Machine Learning Algorithm for Predicting Venous Thromboembolism in Injured Children

BackgroundVenous thromboembolism (VTE) causes significant morbidity in pediatric trauma patients. We applied machine learning algorithms to the Trauma Quality Improvement Program (TQIP) database to develop and validate a risk prediction model for VTE in injured children.MethodsPatients ≤18 years were identified from TQIP (2017–2019, n = 383,814). Those administered VTE prophylaxis ≤24 h and missing the outcome (VTE) were removed (n = 347,576). Feature selection identified 15 predictors: intubation, need for supplemental oxygen, spinal injury, pelvic fractures, multiple long bone fractures, major surgery (neurosurgery, thoracic, orthopedic, vascular), age, transfusion requirement, intracranial pressure monitor or external ventricular drain placement, and low Glasgow Coma Scale score. Data was split into training (n = 251,409) and testing (n = 118,175) subsets. Machine learning algorithms were trained, tested, and compared.ResultsLow-risk prediction: For the testing subset, all models outperformed the baseline rate of VTE (0.15%) with a predicted rate of 0.01–0.02% (p < 2.2e⁻¹⁶). 88.4–89.4% of patients were classified as low risk by the models.High-risk predictionAll models outperformed baseline with a predicted rate of VTE ranging from 1.13 to 1.32% (p < 2.2e⁻¹⁶). The performance of the 3 models was not significantly different.ConclusionWe developed a predictive model that differentiates injured children for development of VTE with high discrimination and can guide prophylaxis use.Level of EvidencePrognostic, Level II.Type of StudyRetrospective, Cross-sectional.     

Hirschsprung-associated inflammatory bowel disease: A multicenter study from the APSA Hirschsprung disease interest group

Background/PurposeA small number of Hirschsprung disease (HD) patients develop inflammatory bowel disease (IBD)-like symptoms after pullthrough surgery. The etiology and pathophysiology of Hirschsprung-associated IBD (HD-IBD) remains unknown. This study aims to further characterize HD-IBD, to identify potential risk factors and to evaluate response to treatment in a large group of patients.MethodsRetrospective study of patients diagnosed with IBD after pullthrough surgery between 2000 and 2021 at 17 institutions. Data regarding clinical presentation and course of HD and IBD were reviewed. Effectiveness of medical therapy for IBD was recorded using a Likert scale.ResultsThere were 55 patients (78% male). 50% (n = 28) had long segment disease. Hirschsprung-associated enterocolitis (HAEC) was reported in 68% (n = 36). Ten patients (18%) had Trisomy 21. IBD was diagnosed after age 5 in 63% (n = 34). IBD presentation consisted of colonic or small bowel inflammation resembling IBD in 69% (n = 38), unexplained or persistent fistula in 18% (n = 10) and unexplained HAEC >5 years old or unresponsive to standard treatment in 13% (n = 7). Biological agents were the most effective (80%) medications. A third of patients required a surgical procedure for IBD.ConclusionMore than half of the patients were diagnosed with HD-IBD after 5 years old. Long segment disease, HAEC after pull through operation and trisomy 21 may represent risk factors for this condition. Investigation for possible IBD should be considered in children with unexplained fistulae, HAEC beyond the age of 5 or unresponsive to standard therapy, and symptoms suggestive of IBD. Biological agents were the most effective medical treatment.Level of EvidenceLevel 4     

Pediatric Pedestrian Injuries: Striking Too Close to Home

BackgroundPediatric pedestrian injuries (PPI) are a major public health concern. This study utilized geospatial analysis to characterize the risk and injury severity of PPI.MethodsA retrospective chart review of PPI patients (age < 18) from a level 1 trauma center was performed (2013–2020). A geographic information system geocoded injury location to home and other public landmarks. Incidents were aggregated to zip codes and the Local Indicators of Spatial Association statistic tested for spatial clustering of injury rates per 10,000 children. Predictors for increased injury severity were assessed by logistic regression.ResultsPPI encompassed 6% (n = 188) of pediatric traumas. Most patients were black (54%), male (58%), >13 years (56%), and with Medicaid insurance (68%). Nine zip codes comprised a statistically significant cluster of PPI. Nearly half (40%) occurred within a quarter mile of home; 7% occurred at home. Most (65%) PPI occurred within 1 mile of a school, and 45% occurred within a quarter mile of a park. Nearly all (99%) PPI occurred within a quarter mile of a major intersection and/or roadway. Using admission to ICU as a marker for injury severity, farther distance from home (OR 1.060, 95% CI 1.001–1.121, p = 0.045) and age <13 years (3.662, 95% CI 1.854–7.231, p < 0.001) were independent predictors of injury severity.ConclusionsThere are significant sociodemographic disparities in PPI. Most injuries occur near patients’ homes and other public landmarks. Multidisciplinary injury prevention collaboration can help inform policymakers, direct local safety programs, and provide a model for PPI prevention at the national level.Level of EvidenceLevel IV.     

Multi-Institutional Review From the Pediatric Colorectal and Pelvic Learning Consortium of Minor Spinal Cord Dysraphism in the Setting of Anorectal Malformations: Diagnosis,Treatment, and Outcomes

Background/rationaleAnorectal malformations (ARM) are associated with congenital anomalies of the spine, but the impact of a minor spinal cord dysraphism (mSCD) on fecal continence in the setting of ARM remains unclear.Materials/methodsA retrospective review was performed utilizing data from the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC) registry. The patient cohort was reviewed for ARM type, mSCD screening/incidence/neurosurgical intervention and age-based BMP utilization.Results987 patients with ARM were categorized into mild (38%), moderate (32%) or complex (19%). 694 (70%) had normal spinal (NS) status. 271 (27.5%) patients had mSCD. MRI alone (49%) was the most common screening test for mSCD. US screening had a positive predictive value of 86.3% and a negative predictive value of 67.1%. Surgical intervention rates for mSCD ranged between 13% and 77% at a median age of 0.6–5.2 years. 726 (73.6%) patients were prescribed BMP (74.4% NS, 77.5% mSCD). Laxatives were most utilized BMP in all groups <5yo. ≥5yo, enema utilization increased with ARM complexity independent of spine status (with or without neurosurgical intervention). Neurosurgical intervention did not affect BMP utilization at any age or with any ARM when mSCD was identified.ConclusionsMSCD influence on bowel function in the setting ARM remains unclear. No significant impact of mSCD was noted on ARM patient bowel management program utilization. Variability exists within PCPLC site with screening and intervention for mSCD in patients with ARM. Future studies with standardized care may be needed to elucidate the true impact of mSCD on long term patient outcomes in ARM patients.Type of StudyRetrospective Comparative Study.Level of evidenceIII.     

Laparoscopic-Assisted Total Resection and Endorectal Pull-Through Technique for Congenital Megarectum with Anorectal Malformation

Background/PurposeCongenital megarectum (CMR) is sometimes associated with anorectal malformations (ARM), although there is no established therapeutic strategy. This study aims to clarify the clinical features of ARM with CMR, and to demonstrate the effectiveness of a surgical treatment, namely laparoscopic-assisted total resection and endorectal pull-through technique.MethodsWe conducted a review of the clinical records of the patients with ARM with CMR treated at our institution between January 2003 and December 2020.ResultsSeven of 33 cases of ARM (21.2%) were diagnosed with CMR, four males and three females. The types of ARM were ‘intermediate’ in four, and ‘low’ in three patients. Five of the seven patients (71.4%) required resection of megarectum for intractable constipation and underwent laparoscopic-assisted total resection and endorectal pull-through technique. Bowel function was improved after resection in all five cases. All five specimens showed hypertrophy of the circular fibers, and three of them showed abnormal location of ganglion cells within the circular muscle fibers.ConclusionsCMR often causes intractable constipation and requires resection of the dilated rectum. Laparoscopic-assisted total resection and endorectal pull-through technique for ARM with CMR considered to be an effective, minimally invasive treatment for intractable constipation.Level of evidence for clinical research papersLevel Ⅳ.Type of studyTreatment study.     

Laparoscopic cyst enucleation is a promising technique for treatment of gastrointestinal tract duplications in children

BackgroundLaparoscopic cyst enucleation has its advantages and limitations in treatment of gastrointestinal tract (GIT) duplications. It allows to avoid bowel resection in such locations as ileocecal valve. We introduced laparoscopic cyst enucleation with further bowel plasty using cyst muscle layer and without monopolar coagulation, for cyst dissection we used straight scissors.Materials and methodsOur study included 20 children with GIT duplications, who underwent cyst enucleation from 2018 to 2021. Laparoscopy was performed in all cases with various cyst locations (stomach – 2, duodenum – 3, small bowel – 14, ileocecal area – 1). Mean age of surgery was 40 ± 35,3 days (min 6 days, max 150 days).ResultsMean operation time was 84,4 ± 27,35 min (min – 40 min, max – 160 min). We had no intraoperative complications. All patients received parenteral feeding for 3–5 days after the operation. Postoperative complications occurred in 3 cases: bowel perforation which required enterostomy (10%) and bowel volvulus required resection (5%). To our opinion, perforations occurred due to monopolar coagulation used for cyst enucleation in these children. No complications were observed in patients who underwent enucleation by straight scissors. Mean postoperative hospital stay was 15.6 ± 10.48 days (min – 4 days, max – 58 days). We observed neither stenosis nor any other complications in the long-term follow-up.ConclusionsLaparoscopic cyst enucleation is a feasible and safe approach for GIT duplications. It allows to avoid bowel resection, and the use of straight scissors instead of monopolar coagulation provides less postoperative complications such as perforation.The Level of evidenceIII.     

The Timing of Congenital Diaphragmatic Hernia Repair on Extracorporeal Membrane Oxygenation Impacts Surgical Bleeding Risk

BackgroundThe optimal timing of surgical repair for infants with congenital diaphragmatic hernia (CDH) treated with extracorporeal membrane oxygenation (ECMO) support remains controversial. The risk of surgical bleeding is considered by many centers as a primary factor in determining the preferred timing of CDH repair for infants requiring ECMO support. This study compares surgical bleeding following CDH repair on ECMO in early versus delayed fashion.MethodsA retrospective review of 146 infants who underwent CDH repair while on ECMO support from 1995 to 2021. Early repair occurred during the first 48 h after ECMO cannulation (ER) and delayed repair after 48 h (DR). Surgical bleeding was defined by the requirement of reoperative intervention for hemostasis or decompression.Results102 infants had ER and 44 infants DR. Surgical bleeding was more frequent in the DR group (36% vs 5%, p < 0.001) with an odds ratio of 11.7 (95% CI: 3.48–39.3, p < 0.001). Blood urea nitrogen level on the day of repair was significantly elevated among those who bled (median 63 mg/dL, IQR 20–85) vs. those who did not (median 9 mg/dL, IQR 7–13) (p < 0.0001). Duration of ECMO support was shorter in the ER group (median 13 vs 18 days, p = 0.005). Survival was not statistically different between the two groups (ER 60% vs. DR 57%, p = 0.737).ConclusionWe demonstrate a significantly lower incidence of bleeding and shorter duration of ECMO with early CDH repair. Azotemia was a strong risk factor for surgical bleeding associated with delayed CDH repair on ECMO.Level of evidenceLevel III cohort study.     

Remote Ischaemic Pre-Conditioning Reduces Intestinal Ischaemia Reperfusion Injury in a Newborn Rat

ObjectiveRemote ischaemic conditioning (RIC) has been shown to reduce ischaemia-reperfusion injury(IRI) in multiple organ systems. IRI is seen in multiple bowel pathologies in the newborn, including NEC. We investigated the potential of RIC as a novel therapy for various intestinal pathologies in the newborn.MethodsWe used an established intestinal IRI model in rat pups which results in similar intestinal injury to necrotising enterocolitis (NEC). Animals were randomly allocated to IRI only(n = 14), IRI + RIC(n = 13) or sham laparotomy(n = 10). The macroscopic extent of intestinal injury is reported as a percentage of total small bowel. Injury severity was measured using Chiu-Park scoring. Neutrophil infiltration/activation was assayed by myeloperoxidase activity. Immunohistochemistry was used to assess the expression of hypoxia-inducible factor alpha (HIF-1α). Data are median (interquartile range).ResultsAnimals that underwent RIC showed a decreased extent of macroscopic injury from 100%(85–100%) in the IRI only group to 58%(15–84%, p = 0.003) in the IRI + RIC group. Microscopic injury score was significantly lower in animals that underwent RIC compared to IRI alone (3.5[1.25–5] vs 5.5[4–6], p = 0.014). Intestinal myeloperoxidase activity in animals exposed to IRI was 3.4 mU/mg of tissue (2.5–3.7) and 2.1 mU/mg(1.5–2.8) in the IRI + RIC group(p = 0.047). HIF-1α expression showed a non-significant trend towards reduced expression in the IRI + RIC group.ConclusionsRIC reduces the extent and severity of bowel injury in this animal model, supporting the hypothesis that RIC has therapeutic potential for intestinal diseases in the newborn.     

The Volume of Intestinal Decompression can Predict the Necessity of Surgical Intervention for Adhesive Small Bowel Obstruction

BackgroundThere is no standard timing for switching to surgical management for children with adhesive small bowel obstruction (ASBO) who initially receive conservative treatment. We hypothesized that an increased gastrointestinal drainage volume may indicate the need for surgical intervention.MethodsThe study population included 150 episodes in the patients less than 20 years of age who received treatment for ASBO in our department from January 2008 to August 2019. Patients were divided into two groups: the successful conservative treatment group (CT) and the eventual surgical treatment group (ST). Following the analysis of all episodes (Study 1), we limited our analysis to only first ASBO episodes (Study 2). We retrospectively reviewed their medical records.ResultsThere were statistically significant differences in the volume on the 2nd day in both Study 1 (9.1 ml/kg vs. 18.7 ml/kg; p < 0.01) and study 2 (8.1 ml/kg vs. 19.7 ml/kg; p < 0.01). The cut-off value was the same for both Study 1 and Study 2 (11.7 ml/kg).ConclusionsThe gastrointestinal drainage volume on the 2nd day in ST was significantly larger than that in CT. Accordingly, we considered that the drainage volume may predict eventual surgical intervention for children with ASBO who initially receive conservative treatment.Level of evidenceLevel IV.     

Disruption of Enterohepatic Circulation of Bile Acids Ameliorates Small Bowel Resection Associated Hepatic Injury

BackgroundMassive small bowel resection (SBR) is associated with liver injury and fibrosis. Efforts to elucidate the driving force behind hepatic injury have identified multiple factors, including the generation of toxic bile acid metabolites.MethodsSham, 50% proximal, and 50% distal SBR were carried out in C57BL/6 mice to determine the effect of jejunal (proximal SBR) versus ileocecal resection (distal SBR) on bile acid metabolism and liver injury. Tissues were harvested at 2 and 10-week postoperative timepoints.ResultsWhen compared with 50% proximal SBR, mice that underwent distal SBR exhibited less hepatic oxidative stress as verified by decreased mRNA expression of tumor necrosis factor-α (TNFα, p ≤ 0.0001), nicotinamide adenine dinucleotide phosphate oxidase (NOX, p ≤ 0.0001), and glutathione synthetase (GSS, p ≤ 0.05). Distal SBR mice also exhibited a more hydrophilic bile acid profile with reduced abundance of insoluble bile acids (cholic acid (CA), taurodeoxycholic acid (TCA), and taurolithocholic acid (TLCA)), and increased abundance of soluble bile acids (tauroursodeoxycholic acid (TUDCA)). In contrast with proximal SBR, ileocecal resection alters enterohepatic circulation leading to reduced oxidative stress and promotes physiological bile acid metabolism.ConclusionThese findings challenge the notion that preservation of the ileocecal region is beneficial in patients with short bowel syndrome. Administration of selected bile acids may present potential therapy to mitigate resection-associated liver injury.Level of evidenceIII—Case-Control Study.     

The Use of the Perioperative Nutrition Score in Postoperative Pediatric Inflammatory Bowel Disease Patients

Background and objectivesPreoperative malnutrition is associated with increased postoperative morbidity. The perioperative nutrition score (PONS) was developed to identify patients at risk of malnutrition. We sought to assess the correlation between preoperative PONS and postoperative outcomes in pediatric inflammatory bowel disease (IBD) patients.MethodsWe performed a retrospective cohort study of IBD patients, less than 21 years of age, who underwent elective bowel resection between June 2018 and November 2021. Patients were divided based upon whether they met PONS criteria. The primary outcome was postoperative surgical site infections.Results96 patients were included. Sixty-one patients (64%) met at least one PONS criteria, while 35 patients (36%) met none. PONS positive patients more frequently received preoperative TPN supplementation (p < .001). There was no difference in preoperative oral nutritional supplementation between groups. Patients that screened positive for PONS had a longer hospital stay (p = .002), more readmissions (p = .029), and more surgical site infections (p = .002).ConclusionsOur data highlight the prevalence of malnutrition in the pediatric IBD population. Patients who screened positive had worse postoperative outcomes. Further, very few of these patients received preoperative optimization with oral nutritional supplementation. There is a need for standardization of nutritional evaluation to improve preoperative nutritional status and postoperative outcomes.Level of EvidenceIII.Type of StudyRetrospective Cohort.     

Goal-directed fluid therapy guided by Plethysmographic Variability Index (PVI) versus conventional liberal fluid administration in children during elective abdominal surgery: A randomized controlled trial

BackgroundPVI has been shown to be an accurate predictor of fluid responsiveness in paediatric patients. Evidence regarding the role of PVI to guide intraoperative fluid therapy in paediatric abdominal surgery is lacking. We aimed to assess the effect of PVI-guided fluid therapy on the volume of intraoperative fluids administered and post-operative biochemical and recovery profile in children undergoing elective abdominal surgery.Methods42 children, 6 months-3 years scheduled for elective open bowel surgery were randomised to receive either ‘conventional liberal intraoperative fluids’ (liberal group) or ‘goal-directed intraoperative fluids’ (GDT group). PVI <13 was targeted in the GDT group. The primary outcome was the volume of intraoperative fluids administered. Postoperative serum lactate, base excess, hematocrit, recovery of bowel function and duration of postoperative hospital stay were the secondary outcomes.ResultsThe mean fluid administered intra-operatively was significantly lower in the GDT group as compared to the liberal group (24.1 ± 9.6 mL/kg vs 37.0 ± 8.9 mL/kg, p < 0.001). The postoperative hemoglobin concentration (g%) was significantly lower in the liberal group as compared to the GDT group (8.1 ± 1.3 vs 9.2 ± 1.4, p = 0.008). Recovery of bowel function (hours) was significantly delayed in the liberal group as compared to the GDT group (58.2 ± 17.9 vs 36.5 ± 14.1, p < 0.001).ConclusionIntraoperative PVI-guided fluid therapy significantly reduces the volume of intravenous crystalloids administered to children undergoing open bowel surgery. These children also had faster recovery of bowel function and less hemodilution in the immediate postoperative period, compared to those who received liberal intraoperative fluid therapy.Type of study: Randomized Clinical Trial.Level of evidence: Treatment Study (LEVEL 1).     

Aggressive Pursuit of No Evidence of Disease Status in Hepatoblastoma Improves Survival: An Observational Study

BackgroundThe utility of repeated surgical interventions in hepatoblastoma to achieve no evidence of disease (NED) is not well-defined. We examined the effect of aggressive pursuit of NED status on event-free (EFS) and overall survival (OS) in hepatoblastoma with subgroup analysis of high-risk patients.MethodsHospital records were queried for patients with hepatoblastoma from 2005 to 2021. Primary outcomes were OS and EFS stratified by risk and NED status. Group comparisons were performed using univariate analysis and simple logistic regression. Survival differences were compared with log-rank tests.ResultsFifty consecutive patients with hepatoblastoma were treated. Forty-one (82%) were rendered NED. NED was inversely correlated with 5-year mortality (OR 0.006; CI 0.001–0.056; P < .01). Ten-year OS (P < .01) and EFS (P < .01) were improved by achieving NED. Ten-year OS was similar between 24 high-risk and 26 not high-risk patients when NED was attained (P = .83). Fourteen high-risk patients underwent a median of 2.5 pulmonary metastasectomies, 7 for unilateral disease, and 7 for bilateral, with a median of 4.5 nodules resected. Five high-risk patients relapsed, and three were salvaged.ConclusionsNED status is necessary for survival in hepatoblastoma. Repeated pulmonary metastasectomy and/or complex local control strategies to obtain NED can achieve long-term survival in high-risk patients.Level of evidenceLevel III – Treatment Study - Retrospective Comparative Study.     

Discontinuation of the OriGen® Dual-Lumen Right Atrial Cannula Decreased Venovenous ECMO Usage in Neonates and Older Children: A Survey of the American Pediatric Surgical Association

IntroductionDual-lumen cannulas for venovenous (VV)-ECMO are widely used in pediatric patients. The popular OriGen® dual-lumen right atrial cannula was discontinued in 2019 without a comparable replacement.MethodsA survey covering VV-ECMO practice and opinions was distributed to attending members of the American Pediatric Surgical Association.Results137 pediatric surgeons responded (14%). Prior to discontinuation of the OriGen®, 82.5% offered VV-ECMO to neonates, and 79.6% cannulated with the OriGen®. Following its discontinuation, those that offered only venoarterial (VA)-ECMO to neonates increased to 37.6% from 17.5% (p = 0.0002). An additional 33.8% changed their practice to sometimes use VA-ECMO when VV-ECMO was indicated. Reasons for not incorporating dual-lumen bi-caval cannulation into practice included risk of cardiac injury (51.7%), inexperience with bi-caval cannulation in neonates (36.8%), difficulty with placement (31.0%), and recirculation and/or positioning problems (27.6%). For the pediatric/adolescent population, 95.5% of surgeons offered VV-ECMO prior to OriGen® discontinuation. Few switched to exclusive VA-ECMO (1.9%) when the OriGen® was discontinued, but 17.8% of surgeons began to incorporate selective use of VA-ECMO.ConclusionDiscontinuation of the OriGen® cannula drove pediatric surgeons to alter their cannulation practices, dramatically increasing VA-ECMO use for neonatal and pediatric respiratory failure. These data may suggest a need for targeted education accompanying major technological shifts.Level of evidenceLevel IV.     

Decreased Incidence of Hirschsprung-Associated Enterocolitis During COVID-19 Across United States Children's Hospitals

BackgroundThe Coronavirus Disease 2019 pandemic provided a natural experiment to study the effect of social distancing on the risk of developing Hirschsprung's Associated Enterocolitis (HAEC).MethodsUsing the Pediatric Health Information System (PHIS), a retrospective cohort study of children (<18 years) with Hirschsprung's Disease (HSCR) across 47 United States children's hospitals was performed. The primary outcome was HAEC admissions per 10,000 patient-days. The exposure (COVID-19) was defined as April 2020–December 2021. The unexposed (historical control) period was April 2018–December 2019. Secondary outcomes included sepsis, bowel perforation, intensive care unit (ICU) admission, mortality, and length of stay.ResultsOverall, we included 5707 patients with HSCR during the study period. There were 984 and 834 HAEC admissions during the pre-pandemic and pandemic periods, respectively (2.6 vs. 1.9 HAEC admissions per 10,000 patient-days, incident rate ratio [95% confidence interval]: 0.74 [0.67, 0.81], p < 0.001). Compared to pre-pandemic, those with HAEC during the pandemic were younger (median [IQR]: 566 [162, 1430] days pandemic vs. 746 [259, 1609] days pre-pandemic, p < 0.001) and more likely to live in the lowest quartile of median household income zip codes (24% pandemic vs. 19% pre-pandemic, p = 0.02). There were no significant differences in rates of sepsis (6.1% pandemic vs. 6.1% pre-pandemic, p > 0.9), bowel perforation (1.3% pandemic vs. 1.2% pre-pandemic, p = 0.8), ICU admissions (9.6% pandemic vs. 12% pre-pandemic, p = 0.2), mortality (0.5% pandemic vs. 0.6% pre-pandemic, p = 0.8), or length of stay (median [interquartile range]: 4 [(Pastor et al., 2009; Gosain and Brinkman, 2015) 2,112,11 days pandemic vs. 5 [(Pastor et al., 2009; Tang et al., 2020) 2,102,10 days pre-pandemic, p = 0.4).ConclusionsThe COVID-19 pandemic was associated with significantly decreased incidence of HAEC admissions across US children's hospitals. Possible etiologies such as social distancing should be explored.Level of evidenceII.