Gastrointestinal Ostomies in Children: A Primer for the Pediatrician.

Despite the advancement of medical therapies in the care of the preterm neonate, in the management of short bowel syndrome and the control of pediatric inflammatory bowel disease, the need to create fecal ostomies remains a common, advantageous treatment option for many medically complex children.

Fecal continence outcomes and potential disparities for patients with anorectal malformations treated at referral institutions for pediatric colorectal surgery.

PURPOSE: Fecal incontinence is a problem for many patients born with an anorectal malformation (ARM) that can impact quality of life. It is unknown if racial, ethnic, and socioeconomic disparities relate to fecal continence in these children. We sought to examine outcomes and potential disparities in care. METHODS: We performed a multicenter retrospective study of children > 3y with ARM evaluated at sites participating in the Pediatric Colorectal and Pelvic Learning Consortium (PCPLC). The primary outcome was fecal continence. We evaluated for associations between fecal continence and race, sex, age, and insurance status. RESULTS: 509 patients with ARM from 11 institutions were included. Overall, 24% reported complete fecal continence, and fecal continence was associated with older age (p < .001). For school-aged children, 27% reported complete continence, while 53% reported none. On univariate analysis, patients with combined private and public insurance showed lower rates of continence when compared to those with private insurance (23 vs. 12%; p = 0.02). Age was associated with continence on univariate and multivariable analyses. CONCLUSION: Rates of complete fecal continence in this population are low. Differences based on payor status may exist. There were no observed disparities related to sex and race. Further investigation is warranted to improve care for this patient population. LEVEL OF EVIDENCE: III. TYPE OF STUDY: Multi-institutional retrospective comparative study.

Outpatient Botulinum Injections for Early Obstructive Symptoms in Patients with Hirschsprung Disease.

INTRODUCTION: Botulinum toxin (BT) injections may play a role in preventing Hirschsprung associated enterocolitis (HAEC) episodes related to internal anal sphincter (IAS dysfunction). Our aim was to determine the association of outpatient BT injections for early obstructive symptoms on the development of HAEC. METHODS: A retrospective review of children who underwent definitive surgery for Hirschsprung disease (HSCR) from July 2010 - July 2020 was performed. The timing from pull-through to first HAEC episode and to first BT injection was recorded. Primary analysis focused on the rate of HAEC episodes and timing between episodes in patients who did and did not receive BT injections. RESULTS: Eighty patients were included. Sixty patients (75%) were male, 15 (19%) were diagnosed with trisomy 21, and 58 (72.5%) had short-segment disease. The median time to pull-through was 150 days (IQR 16, 132). Eight patients (10%) had neither an episode of HAEC or BT injections and were not included in further analysis. Forty-six patients (64%) experienced at least one episode of HAEC, while 64 patients (89%) had at least one outpatient BT injection. Compared to patients who never received BT injections (n = 9) and those who developed HAEC prior to BT injections (n = 35), significantly fewer patients who received BT injections first (n = 28) developed enterocolitis (P < 0.001), with no patient developing more than one HAEC episode. CONCLUSION: Outpatient BT is associated with decreased episodes of HAEC and increased interval between HAEC episodes requiring inpatient treatment. Scheduling outpatient BT injections to manage obstructive symptoms may be beneficial after pull-through for HSCR.

Behind the mask: extended use of surgical masks is not associated with increased risk of surgical site infection.

PURPOSE: COVID-19 has prompted significant policy change, with critical attention to the conservation of personal protective equipment (PPE). An extended surgical mask use policy was implemented at our institution, allowing use of one disposable mask per each individual, per day, for all the cases. We investigate the clinical impact of this policy change and its effect on the rate of 30-day surgical site infection (SSI). METHODS: A single-institution retrospective review was performed for all the elective pediatric general surgery cases performed pre-COVID from August 2019 to October 2019 and under the extended mask use policy from August 2020 to October 2020. Procedure type, SSI within 30 days, and postoperative interventions were recorded. RESULTS: Four hundred and eighty-eight cases were reviewed: 240 in the pre-COVID-19 cohort and 248 in the extended surgical mask use cohort. Three SSIs were identified in the 2019 cohort, and two in the 2020 cohort. All postoperative infections were superficial and resolved within 1 month of diagnosis with oral antibiotics. There were no deep space infections, readmissions, or infections requiring re-operation. CONCLUSION: Extended surgical mask use was not associated with increased SSI in this series of pediatric general surgery cases and may be considered an effective and safe strategy for resource conservation with minimal clinical impact.

Impact of Botulinum Toxin on Hirschsprung-Associated Enterocolitis After Primary Pull-Through.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is a serious potential complication after primary pull-through surgery for Hirschsprung's disease (HSCR). Administration of anal botulinum toxin (BT) injection may improve obstructive symptoms at the internal anal sphincter, leading to improved fecal passage. The timing of administration and effects on delay or prevention of HAEC are unknown. We hypothesized that BT administration increased the postoperative time to HAEC and aimed to investigate whether anal BT administration after primary pull-through surgery for HSCR is associated with increased time to inpatient HAEC admission development. METHODS: We performed a retrospective cohort study examining children with HSCR at US children's hospitals from 2008 to 2018 using the Pediatric Health Information System database with an associated primary pull-through operation performed before 60 d of age. The intervention assessed was the administration of BT concerning the timing of primary pull-through, and two groups were identified: PRO (received BT at or after primary pull-through, before HAEC) and NOT (never received BT, or received BT after HAEC). The primary outcome was time from pull-through to the first HAEC admission. The Cox proportional hazards model was developed to examine the BT administration effect on the primary outcome after controlling for patient-level covariates. RESULTS: We examined a total of 1439 children (67 in the PRO and 1372 in the NOT groups). A total of 308 (21.4%) developed at least one episode of HAEC, including 76 (5.3%) who had two or more episodes. Between 2008 and 2018, the frequency of BT administration has increased from three to 20 hospitals with a frequency of administration between 2.2% and 16.2%. Prophylactic BT (PRO) was not associated with increased time to HAEC event on adjusted analysis. CONCLUSIONS: Among children with HSCR undergoing primary pull-through surgery, prophylactic BT administration did not demonstrate increased time to first HAEC event. A better-powered study with prophylactic BT is required to determine the effect on HAEC occurrence and timing. LEVEL OF EVIDENCE: Level II (retrospective cohort study).

The impact of botulinum injection for hospitalized children with Hirschsprung-associated enterocolitis.

INTRODUCTION: Stasis from obstruction at the level of the internal anal sphincter (IAS) can lead to Hirschsprung-associated enterocolitis (HAEC) and may be improved by botulinum toxin (BT) injections. Our aim was to determine if BT injection during HAEC episodes decreased the number of recurrent HAEC episodes and/or increased the interval between readmissions. METHODS: A retrospective review was performed of patients admitted for HAEC from January 2010 to December 2019. Demographics and outcomes of patients who received BT were compared to patients who did not receive BT during their hospital stay. RESULTS: A total of 120 episodes of HAEC occurred in 40 patients; 30 patients (75%) were male, 7 (18%) had Trisomy 21 and 10 (25%) had long-segment disease. On multivariate analysis, patients who received BT during their inpatient HAEC episode had a longer median time between readmissions (p = 0.04) and trending toward an association with fewer readmissions prior to a follow-up clinic visit (p = 0.08). CONCLUSION: The use of BT in HD patients hospitalized for HAEC is associated with an increased time between recurrent HAEC episodes and trended toward fewer recurrent episodes. The use of BT should be considered in the management of patients admitted with HAEC.

Optimization of Pediatric Bowel Management Using an Antegrade Enema Troubleshooting Algorithm.

BACKGROUND: A successful flush is the ability to flush through the appendicostomy or cecostomy channel, empty the flush through the colon, and achieve fecal cleanliness. We evaluated our experience with patients who were having flush difficulties based on a designed algorithm. METHODS: Eight patients with flush difficulties were initially evaluated. Based on the need for additional surgery versus changes in bowel management therapy (BMT), we developed an algorithm to guide future management. The algorithm divided flush issues into before, during, and after flushing. Children aged <20 y who presented with flush issues from September 2018 to August 2019 were evaluated to determine our algorithm's efficacy. Specific outcomes analyzed included changes in BMT versus need for additional surgery. RESULTS: After algorithm creation, 29 patients were evaluated for flush issues. The median age was 8.4 y (interquartile range: 6, 14); 66% (n = 19) were men. Underlying diagnoses included anorectal malformations (n = 17), functional constipation (n = 7), Hirschsprung's disease (n = 2), spina bifida (n = 2), and prune belly (n = 1). A total of 35 flush issues/complaints were noted: 29% before the flush, 9% during the flush, and 63% after the flush. Eighty percent of issues before the flush required surgical intervention, wherease 92% of issues during or after the flush were managed with changes in BMT. CONCLUSIONS: Most flush issues respond to changes in BMT. This algorithm can help delineate which types of flush issues would benefit from surgical intervention and what problems might be present if patients are not responding to changes in their flush regimen.

Ethics of randomized trials in pediatric surgery.

To provide the best evidence-based treatment for children, and timely evaluation of innovations, the role of the pediatric surgeon's participation in randomized controlled trials (RCTs) and prospective comparative studies is required. The ethical considerations of pediatric surgical RCTs pose unique practical difficulties in the design and performance of clinical trials in children. There are several ethical issues unique to pediatric surgical RCTs: diseases with low volume, an inability to conduct Phase 1 and 2 trials, parental emotional involvement, difficulty with recruitment in surgical trials, volume, and modified study design, issues with permission vs. assent and investigator bias. This article reviews the ethical aspects unique to pediatric surgical RCTs and prospective comparative studies.Level of evidence: Level 3.

Inpatient management of Hirschsprung's associated enterocolitis treatment: the benefits of standardized care.

INTRODUCTION: Patients with Hirschsprung's disease (HSCR) remain at risk of developing Hirschsprung-associated enterocolitis (HAEC) after surgical intervention. As inpatient management remains variable, our institution implemented an algorithm directed at standardizing treatment practices. This study aimed to compare the outcomes of patients pre- and post-algorithm. METHODS: A retrospective review of patients admitted for HAEC was performed; January 2017-June 2018 encompassed the pre-implementation period, and October 2018-October 2019 was the post-implementation period. Demographics and outcomes were compared between the two groups. RESULTS: Sixty-two episodes of HAEC occurred in 27 patients during the entire study period. Sixteen patients (59%) had more than one episode. The most common levels of the transition zone were the rectosigmoid (50%) and descending colon (27%). Following algorithm implementation, the median length of stay (2 vs. 7 days, p < 0.001), TPN duration (0 vs. 5.5 days, p < 0.001), and days to full enteral diet (6 days vs. 2 days, p < 0.001) decreased significantly. Readmission rates for recurrent enterocolitis were similar pre- and post-algorithm implementation. CONCLUSION: The use of a standardized algorithm significantly decreases the length of stay and duration of intravenous antibiotic administration without increasing readmission rates, while still providing appropriate treatment for HAEC. LEVEL OF EVIDENCE: III level. TYPE OF STUDY: Retrospective comparative study.

Therapeutic Direction Versus Adverse Outcomes in Children Undergoing Lung Biopsy.

BACKGROUND: Lung biopsy is part of the diagnostic workup for multiple diseases. Although the morbidity of the procedure has decreased with the use of thoracoscopy, lung biopsy still holds substantial risk for patients. Therefore, we evaluated the likelihood of lung biopsies impacting treatment compared to complications. MATERIAL AND METHODS: This was a single-institution, retrospective chart review of patients less than aged 18 y undergoing lung biopsy from 2010 to 2016. Details of demographics, hospital course, adverse events, complications, pathology, and follow-up were recorded. All values are reported as medians with interquartile range. RESULTS: Thirty-seven patients met inclusion criteria. Median age was 7 y old (interquartile range 1.4, 15). Eighty-seven percent (33) of biopsies were performed thoracoscopically, with a 3% conversion rate. Adverse events occurred in 25% (9) of cases with the majority involving prolonged respiratory failure (n = 7). Complications occurred in 16% (6) of cases including pneumothorax (13%, n = 5) and cardiac arrest (3%, n = 1). A third of these complications (n = 2) required reoperation, and both were decompressions of tension pneumothoraces. Pathology established a diagnosis in 62% (n = 23) of cases, yet treatment was changed in only 43% of cases. No preoperative variables were associated with the pathology establishing a diagnosis or changing treatment. CONCLUSIONS: Lung biopsy for questionable pulmonary disease changed treatment in less than half of cases, with significant perioperative morbidity. Careful consideration should therefore be given to who would benefit most from lung biopsy.

Role of intestinal Hsp70 in barrier maintenance: contribution of milk to the induction of Hsp70.2.

BACKGROUND: Necrotizing enterocolitis (NEC) is a gastrointestinal disease of complex etiology resulting in devastating systemic inflammation and often death in premature newborns. We previously demonstrated that formula feeding inhibits ileal expression of heat shock protein-70 (Hsp70), a critical stress protein within the intestine. Barrier function for the premature intestine is critical. We sought to determine whether reduced Hsp70 protein expression increases neonatal intestinal permeability. METHODS: Young adult mouse colon cells (YAMC) were utilized to evaluate barrier function as well as intestine from Hsp70-/- pups (KO). Sections of intestine were analyzed by Western blot, immunohistochemistry, and real time PCR. YAMC cells were sub-lethally heated or treated with expressed milk (EM) to induce Hsp70. RESULTS: Immunostaining demonstrates co-localized Hsp70 and tight junction protein zona occludens-1 (ZO-1), suggesting physical interaction to protect tight junction function. The permeability of YAMC monolayers increases following oxidant injury and is partially blocked by Hsp70 induction either by prior heat stress or EM. RT-PCR analysis demonstrated that the Hsp70 isoforms, 70.1 and 70.3, predominate in WT pup; however, Hsp70.2 predominates in the KO pups. While Hsp70 is present in WT milk, it is not present in KO EM. Hsp70 associates with ZO-1 to maintain epithelial barrier function. CONCLUSION: Both induction of Hsp70 and exposure to EM prevent stress-induced increased permeability. Hsp70.2 is present in both WT and KO neonatal intestine, suggesting a crucial role in epithelial integrity. Induction of the Hsp70.2 isoform appears to be mediated by mother's milk. These results suggest that mother's milk feeding modulates Hsp70.2 expression and could attenuate injury leading to NEC. LEVEL OF EVIDENCE: Level III.

Right trisegmentectomy after portal vein embolization in a high-risk toddler with hepatoblastoma.

Portal vein embolization (PVE) is a pre-operative treatment modality in adults undergoing hepatectomy with concerns of post-operative liver failure from insufficient future liver remnant (FLR). PVE induces growth in the FLR. The success of this technique is well described in adults, but not in young children with hepatoblastoma.

Utility of pediatric female fertility preservation discussions following pelvic radiation.

INTRODUCTION: While many childhood cancers are curable with therapy, adverse consequences in fertility exist. We sought to assess the number of female patients with pelvic tumors receiving radiation therapy, and the proportion that undergo measures for fertility preservation (FP). METHODS: A total of 53 female patients treated with pelvic tumors from 2000 to 2016 were retrospectively identified. RESULTS: 19 (34%) of these patients underwent pelvic radiation therapy (pXRT). Three of the patients received pXRT for palliative treatment. Of the 19 female patients receiving pXRT, six (31%) were prepubertal and 13 (68%) were postpubertal. Three patients (16%) had documentation of a discussion of FP measures prior to pXRT. One was prepubertal and the others were post-pubertal. Six patients (32%) were evaluated by endocrinology after radiation therapy, diagnosed with ovarian failure, and placed on hormone therapy. Current guidelines recommend discussion of FP in pre-and postpubertal patients with cancer. This 16-year retrospective review of female patients that underwent pXRT for pelvic tumors demonstrated < 17% of patients have documentation of a discussion of FP measures. CONCLUSION: Female pediatric patients who underwent chemotherapy and pXRT suffer a high rate of premature ovarian failure, high morbidity and mortality as well as low rates of documented FP discussions. Based on these findings we have established a multi-disciplinary fertility preservation team available for consultation and a protocol for discussing and documenting the impact of pXRT, along with other treatments, on fertility. LEVEL OF EVIDENCE: III.

Pediatric Rectal Prolapse.

Rectal prolapse is a common and self-limiting condition in infancy and early childhood. Most cases respond to conservative management. Patients younger than 4 years with an associated condition have a better prognosis. Patients older than 4 years require surgery more often than younger children. Multiple operative and procedural approaches to rectal prolapse exist with variable recurrence rates and without a clearly superior operation. These include sclerotherapy, Thiersch's anal cerclage, Ekehorn's rectopexy, laparoscopic suture rectopexy, and posterior sagittal rectopexy.

Pediatric appendicitis: state of the art review.

Appendicitis is a common cause of abdominal pain in children. The diagnosis and treatment of the disease have undergone major changes in the past two decades, primarily as a result of the application of an evidence-based approach. Data from several randomized controlled trials, large database studies, and meta-analyses have fundamentally affected patient care. The best diagnostic approach is a standardized clinical pathway with a scoring system and selective imaging. Non-operative management of simple appendicitis is a reasonable option in selected cases, with the caveat that data in children remain limited. A minimally invasive (laparoscopic) appendectomy is the current standard in US and European children's hospitals. This article reviews the current 'state of the art' in the evaluation and management of pediatric appendicitis.

Routine use of chest radiographs in the post-operative management of pectus bar removal: necessity or futility.

BACKGROUND: While there is literature on techniques for pectus bar removal, there are limited reports on post-operative management. This can include obtaining a postoperative chest radiograph (CXR) despite the minimal risk of associated intra-thoracic complications. This is a review of our experience with bar removal and lack of routine post-operative CXR. METHODS: A single institution retrospective chart review was performed from 2000 to 2015. Patients who underwent a pectus bar removal procedure were included. We assessed operative timing of bar placement and removal, procedure length, intra-operative and post-operative complications and post-operative CXR findings, specifically the rate of pneumothoraces. RESULTS: 450 patients were identified in this study. Median duration of bar placement prior to removal was 35 months (interquartile range 30 and 36 months). Sixtey-four patients obtained a post-operative CXR. Of these, only one (58%) film revealed a pneumothorax; this was not drained. A CXR was not obtained in 386 (86%) patients with no immediate or delayed complications from this practice. Median follow-up time for all patients was 11 months (interquartile range 7.5-17 months). DISCUSSION: The risk for a clinically relevant pneumothorax is minimal following bar removal. This suggests that not obtaining routine imaging following bar removal may be a safe practice.

Review of surgical and anesthetic management for esophageal atresia with tracheoesophageal fistula, unilateral pulmonary agenesis and dextrocardia.

Association of unilateral severe pulmonary atresia or agenesis and esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) and dextrocardia is a rare and highly lethal combination. We report a case of a full-term female infant who had EA with TEF, right lung agenesis, and dextrocardia. Repair of the fistula took place on day of life 3. We describe anesthetic and surgical concerns of this patient's case which are keys to a good operative outcome as well as follow-up for the patient.

Intestinal alkaline phosphatase to treat necrotizing enterocolitis.

BACKGROUND: Intestinal alkaline phosphatase (IAP) activity is decreased in necrotizing enterocolitis (NEC), and IAP supplementation prevents NEC development. It is not known if IAP given after NEC onset can reverse the course of the disease. We hypothesized that enteral IAP given after NEC induction would not reverse intestinal injury. MATERIALS AND METHODS: NEC was induced in Sprague-Dawley pups by delivery preterm followed by formula feedings with lipopolysaccharide (LPS) and hypoxia exposure and continued up to 4 d. IAP was added to feeds on day 2 until being sacrificed on day 4. NEC severity was scored based on hematoxylin and eosin-stained terminal ileum sections, and AP activity was measured using a colorimetric assay. IAP and interleukin-6 expression were measured using real time polymerase chain reaction. RESULTS: NEC pups' alkaline phosphatase (AP) activity was decreased to 0.18 U/mg compared with controls of 0.57 U/mg (P < 0.01). Discontinuation of LPS and hypoxia after 2 d increased AP activity to 0.36 U/mg (P < 0.01). IAP supplementation in matched groups did not impact total AP activity or expression. Discontinuing LPS and hypoxia after NEC onset improved intestinal injury scores to 1.14 compared with continued stressors, score 2.25 (P < 0.01). IAP supplementation decreased interleukin-6 expression two-fold (P < 0.05), though did not reverse NEC intestinal damage (P = 0.5). CONCLUSIONS: This is the first work to demonstrate that removing the source of NEC improves intestinal damage and increases AP activity. When used as a rescue treatment, IAP decreased intestinal inflammation though did not impact injury making it likely that IAP is best used preventatively to those neonates at risk.

Twelve Tips for Improving the General Surgery Resident Night Float Experience.

Restriction of resident duty hours has resulted in the implementation of night float systems in surgical and medical programs. Many papers have examined the benefits and structure of night float, but few have addressed patient safety issues, quality patient care, and the impact on the residency education system. The objective of this review is to provide practical tips to optimize the night float experience for resident training while continuing to emphasize patient care. The tips provided are based on the experiences and reflections of residents, supervising staff, group discussions, and the available literature in a hospital-based general surgery residency program. Utilizing these resources, we concluded that the night float system addresses resident work hour restrictions; however, it ultimately creates new issues. Adaptations will help achieve a balance between resident education and patient safety.