Sacral nerve stimulation leads to long-term improvement in fecal incontinence and quality of life for children with functional and organic defecation disorders.

BACKGROUND: Our objective was to evaluate long-term outcomes of sacral nerve stimulation (SNS) for children with functional and organic defecation disorders. METHODS: We performed a prospective study of children <21 years of age who started SNS treatment between 2012 and 2018. We recorded demographics, medical history, and diagnostic testing. We obtained measures of symptom severity and quality of life at baseline and follow up at 1, 6, 12, 24, 36, 48, and ≥60 months. Successful response was defined as bowel movements >2 times/week and fecal incontinence (FI) <1 time/week. Families were contacted to administer the Glasgow Children's Benefit Inventory and to evaluate patient satisfaction. KEY RESULTS: We included 65 patients (59% female, median age at SNS 14 years, range 9-21) with median follow-up of 32 months. Thirty patients had functional constipation (FC), 15 had non-retentive FI (NRFI), and 16 had an anorectal malformation (ARM). The percentage with FI <1 time/week improved from 30% at baseline to 64% at 1 year (p < 0.001) and 77% at most recent follow-up (p < 0.001). Patients with FC, NRFI, and ARM had sustained improvement in FI (p = 0.02, p < 0.001, p = 0.02). Patients also reported fewer hard stools (p = 0.001). Bowel movement frequency did not improve after SNS. At most recent follow-up, 77% of patients with a functional disorder and 50% with an organic disorder had responded (p = 0.03). Nearly all families reported benefit. CONCLUSIONS AND INFERENCES: SNS led to sustained improvement in FI regardless of underlying etiology, but children with functional disorders were more likely to respond than those with organic disorders.

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.

Contemporary use of prophylactic probiotics in NICUs in the United States: a survey update.

OBJECTIVE: In 2015, 14.0% of US NICUs administered probiotics to very low birth weight infants. Current probiotic use prior to and after the Fall of 2023 (when FDA warnings were issued) remains unknown. STUDY DESIGN: A survey was distributed to the American Academy of Pediatrics Section on Neonatal and Perinatal Medicine (August-November/2022) and Neonatology Solutions' Level III/IV NICUs (January-April/2023). Probiotic administration practices were investigated. RESULTS: In total, 289 unique NICUs and 406 providers responded to the survey. Of those, 29.1% of NICUs administered prophylactic probiotics to premature neonates, however, this decreased considerably after FDA warnings were issued. Additionally, 71.4% of providers stated willingness to administer probiotics to premature infants if there was an FDA-approved formulation. CONCLUSIONS: Probiotic use in US NICUs increased between 2015 and the Fall of 2023 and then dropped dramatically following warning letters from the FDA. The introduction of an FDA-approved probiotic may further expand administration.

Robotic-assisted Pediatric Thoracic and Abdominal Tumor Resection: An Initial Multi-center Review.

INTRODUCTION: Robotic-assisted minimally invasive surgery (RA-MIS) for tumor resection is an emerging technology in the pediatric population with significant promise but unproven safety and feasibility. METHODS: A multi-center retrospective review of patients ≤18 years undergoing RA-MIS tumor resection from December 2015-March 2023 was performed. Patient demographics, perioperative variables, and complication rates were analyzed. RESULTS: Thirty-nine procedures were performed on 38 patients (17 thoracic, 22 abdominal); 37% female and 68% non-Hispanic White. Median age at surgery was 8.3 years (IQR 5.7, 15.7); the youngest was 1.7 years-old. Thoracic operations included resections of neuroblastic tumors (n = 16) and a single paraganglioma. The most common abdominal operations included resections of neuroblastic tumors (n = 5), pheochromocytomas (n = 3), and angiomyolipomas (n = 3). Six patients underwent retroperitoneal lymph node dissection (RPLND) for paratesticular tumors. Median operating time for the cohort was 2:52 h (IQR 2:04, 4:31). Two thoracic cases required open conversion due to poor visualization and lack of working domain. All patients underwent complete tumor resection; one had tumor spillage from a positive margin (Wilms tumor). Median LOS was 1.5 days (IQR 1.1, 3.0). Postoperatively, one patient developed a chyle leak requiring interventional radiology drainage, but none required a return to the operating room. CONCLUSIONS: Robotic-assisted surgery is safe and feasible for tumor resection in carefully selected pediatric patients, achieving complete resection with minimal morbidity and short LOS. Resection should be performed by those with robotic expertise for optimal outcomes. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Original Clinical Research.

Robotic-Assisted Surgery in Patients Less than 15 kg: A Single Center Review.

Introduction: Robotic-assisted surgery (RAS) is an increasingly utilized tool in children. However, utilization of RAS among infants and small children has not been well established. The purpose of this study was to review and characterize RAS procedures for children ≤15 kg. Methods: We performed a single institution retrospective descriptive analysis including all patients ≤15 kg undergoing RAS between January 2013 and July 2021. Data collection included procedure type, age, weight, gender, and surgical complications. Cases were further categorized according to surgical specialty: pediatric urology (PU), pediatric surgery (PS), and multiple specialties (MS). t-Tests were used for statistical analyses. Results: Since 2013, a total of 976 RAS were identified: 492 (50.4%) were performed by PU, 466 (47.8%) by PS, and 18 (1.8%) by MS. One hundred eighteen (12.1%) were performed on children ≤15 kg, consisting of 110 (93.2%) PU cases, 6 (5.1%) PS cases, and 2 (1.7%) MS cases. Procedures were significantly more common in the PU subgroup, mean of 12 cases/year, compared to PS subgroup, mean of 0.63 cases/year, (P < .01). The mean weight of PU patients (10.5 kg) was significantly less than PS patients (13.9 kg) (P < .01). Mean age was also significantly lower among PU patients (18.6 months) compared to PS (34.2 months) (P < .01). Conclusion: RAS among patients ≤15 kg is safe and feasible across pediatric surgical subspecialties. RAS was performed significantly more frequently by pediatric urologists in younger and smaller patients compared to pediatric surgeons. Further refinement of robotic technology and instrumentation should enhance the applicability of these procedures in this young group.

Giant Omphaloceles-Morbidity, Mortality, and Financial Impact of Early Versus Delayed Repair.

INTRODUCTION: We compared strategy outcomes and financial impact over the first two years of life (F2YOL) for patients with giant omphaloceles undergoing early repair (ER) (primary or staged) versus delayed repair (DR). METHODS: A retrospective review of giant omphaloceles (fascial defect > 5 cm/> 50% liver herniation) at a tertiary children's hospital between 1/1/2010 and 12/31/2019 was performed. Survival, length of stay, age at repair, ventilation days (VD), time to full enteral feeds, readmissions during the F2YOL, incidence of major associated anomalies, and total hospitalization charges during the F2YOL were compared. A subanalysis removing potential confounders and only including patients who underwent fascial closure within the F2YOL was also conducted. RESULTS: Thirty four giant omphaloceles (23DR and 11ER) were identified. The median age (days) at repair was 289 [148, 399] DR versus 10 [5, 21] ER, P < 0.001. Total cohort two-year survival was significantly higher in the DR group (95.7% versus 63.6%, P = 0.03). Including patients with a tracheostomy there was no significant difference in VD during the index hospitalization. Excluding tracheostomy patients, the DR group had significantly fewer VD during the index hospitalization, 15 [0, 15] versus 18 [10, 54], P = 0.02 and over the F2YOL 6.5 [ 0, 21] versus 18 [14, 43], P = 0.03. There were no significant differences in the incidence/type of major associated anomalies, time to full enteral feeds, index length of stay, total hospital days, total admissions, or associated hospital charges. On subanalysis, there was no significant difference in VD or survival at any time. CONCLUSIONS: Delayed and early repair strategies for giant omphaloceles have equivalent outcomes in the index hospitalization and over the course of the first two years of life. These findings are useful for family counseling and expectation setting.

A Review of Covered Abdominal Wall Defects: Cord Hernias Are Associated With Major Anomalies.

INTRODUCTION: Covered abdominal wall defects (CAWD) can be categorized into giant omphaloceles (GOs), nongiant omphaloceles (NGOs), and umbilical cord hernias (UCHs). We sought to evaluate differences in management and outcomes of the different CAWD, treated at a large tertiary children's hospital, with regards to survival and association with other major congenital anomalies. METHODS: A retrospective review of CAWD patients between January 2010 and January 2021 was conducted. GO was defined as a fascial defect >5 cm or >50% liver herniation. UCH were defined as fascial defects ≤ 2 cm. All others were classified as NGO. Type of repair, time to fascial closure, index hospitalization length of stay (LOS), and survival rates were compared. Four major anomaly categories were identified: cardiac, midline, Beckwith-Weidemann Syndrome, and other genetic anomalies. RESULTS: We identified 105 CAWD patients (UCH n = 40; GO n = 34; and NGO n = 31). Ninety percent of UCH underwent primary repair, 10% were never repaired. NGOs were repaired by primary or staged methods in 92.9% of cases and 7.1% by delayed repair. Primary or staged repair occurred in 32.4% of GOs and delayed repair occurred in 67.6%. The median days to repair was 181 [24,427] GO, 1 [1,3] NGO, and 1 [0,1] UCHs (P < 0.01). Index hospitalization median LOS (days) was 90 [55,157] GO, 23 [10,48] NGO, 9 [5,22] UCH, (P < 0.01). There were no statistical differences in survival rates, number of patients with major anomalies (GO 35.4%, NGO 51.5%, UCH 50%), or types of anomalies. CONCLUSIONS: UCHs and omphaloceles have similar incidences of major associated anomalies. Thus, all patients with a covered abdominal wall defect should undergo workup for associated anomalies.

Rates of Air Embolism in Pediatric Patients Undergoing Surgical Procedures of the Peritoneum.

Background: Air embolism during laparoscopic surgery is a rare but feared complication in the pediatric population. The objective of this study was to identify rates of air embolus in pediatric patients during hospitalization for laparoscopic or open surgical procedures of the peritoneal cavity. Materials and Methods: Patients 0-18 years old within the Pediatric Health Information System who underwent a predefined, common inpatient laparoscopic or open surgical procedure involving the peritoneal cavity from 2015 to 2020 were studied. International Classification of Diseases, Tenth Revision, Clinical Modification diagnosis codes for air embolism were then searched among patients during the same admission. Firth logistic regression was used to compare rates of air embolism in open and laparoscopic cohorts and in patients >1 and ≤1 year. Results: Unadjusted rates of air embolism were higher in patients undergoing open compared with laparoscopic surgery (open: 9/45,080; 20.0/100,000 patients versus laparoscopic: 3/101,892; 2.9/100,000 patients). In patients ≤1 year (45,726), 2 patients undergoing open surgery (2/1,031; 9.5/100,000 patients) and all 3 patients undergoing laparoscopic surgery had an air embolism diagnosis (3/22,329; 13.4/100,000 patients). For laparoscopic surgery, a suggested lower relative risk (RR) of air embolism was demonstrated for children >1 year compared with children ≤1 year (RR: 0.05, P = .05). Conclusion: Air embolism associated with common pediatric surgical procedures of the peritoneum is rare and patients undergoing laparoscopic and open surgery have similar risks for air embolism. Although rare, the risk should be considered during surgical planning and abdominal access, especially in children ≤1 year old.

Congenital duodenal obstruction - Advances in diagnosis, surgical management, and associated controversies.

Congenital duodenal obstruction (CDO) occurs due to intrinsic and extrinsic mechanisms but is most often caused by intrinsic duodenal atresia and stenosis. This review will summarize the history, epidemiology, and etiologies associated with the most common causes of CDO. The clinical presentation, complex diagnostic considerations, and current surgical repair options for duodenal atresia and stenosis will also be discussed. Finally, both historical and recent controversies which continue to affect the surgical decision-making in the management of these patients will be highlighted.

Robotic-assisted vertical sleeve gastrectomy in adolescents: Do BMI limits apply?

BACKGROUND: Robotic-assisted vertical sleeve gastrectomy (VSG) in adolescent patients has been shown to have comparable outcomes to laparoscopic VSG. Recent data suggests that metabolic and bariatric surgery (performed using robotic and laparoscopic techniques) in patients with BMI ≥ 50 kg/m2 have a higher risk of adverse events compared to those with BMI < 50 kg/m2. The aim of this study was to compare the outcomes of robotic-assisted VSG in adolescents with a BMI above and below 50 kg/m2. METHODS: A retrospective analysis of all adolescents undergoing robotic-assisted VSG between January 2014 and December 2020. Subjects were categorized based on preoperative BMI; Group 1 (BMI < 50 kg/m2) or Group 2 (BMI ≥ 50 kg/m2). Data collection included patient demographics, preoperative BMI, total operative time, access time (i.e., total time for port-placement), 30 day complications, and 30 day hospital readmissions. Analysis was performed using chi-square, Fisher's Exact, and student t-test. RESULTS: Total of 115 subjects (Group 1 N = 64 and Group 2 N = 51) were included. No differences in age or ethnicity were detected; however, Group 2 had a higher percentage of male patients (27.5% vs. 4.7%, respectively, p = 0.001). Mean operative times (Group 1 = 122.2 min vs. Group 2 = 121.6 min) and access times (Group 1 = 19.1 min vs. Group 2 = 19.7 min) were similar between groups. Thirty day complication rates were similar between groups (p = 0.133); however, there was a higher rate of hospital readmission in Group 1. CONCLUSION: While recent data demonstrate an increased likelihood of adverse events occurring among patients with BMI ≥ 50 kg/m2 undergoing robotic surgery, we observed no differences in intraoperative or early postoperative outcomes based on BMI in this robotic-assisted pediatric cohort.

Management of the undescended testis in children: An American Pediatric Surgical Association Outcomes and Evidence Based Practice Committee Systematic Review.

PURPOSE: Management of undescended testes (UDT) has evolved over the last decade. While urologic societies in the United States and Europe have established some guidelines for care, management by North American pediatric surgeons remains variable. The aim of this systematic review is to evaluate the published evidence regarding the treatment of (UDT) in children. METHODS: A comprehensive search strategy and the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guidelines were utilized to identify, review, and report salient articles. Five principal questions were asked regarding imaging standards, medical treatment, surgical technique, timing of operation, and outcomes. A literature search was performed from 2005 to 2020. RESULTS: A total of 825 articles were identified in the initial search, and 260 were included in the final review. CONCLUSIONS: Pre-operative imaging and hormonal therapy are generally not recommended except in specific circumstances. Testicular growth and potential for fertility improves when orchiopexy is performed before one year of age. For a palpable testis, a single incision approach is preferred over a two-incision orchiopexy. Laparoscopic orchiopexy is associated with a slightly lower testicular atrophy rate but a higher rate of long-term testicular retraction. One and two-stage Fowler-Stephens orchiopexy have similar rates of testicular atrophy and retraction. There is a higher relative risk of testicular cancer in UDT which may be lessened by pre-pubertal orchiopexy.

The cutback revisited - The posterior rectal advancement anoplasty for certain anorectal malformations with rectoperineal fistula.

BACKGROUND: The repair of rectoperineal fistulae can pose a significant challenge to the pediatric surgeon given the proximity of the fistula to the urethra in males and vagina in females. In these children, a simple cutback procedure may leave the neoanus in a position anterior to the center of the sphincter, which theoretically could impair future continence. We devised an adaptation of the cutback anoplasty which we call the posterior rectal advancement anoplasty (PRAA) to treat patients with a rectoperineal fistula that is both narrow in lumen and located within, but at the anterior-most limit of the sphincter complex. MATERIAL AND METHODS: Patient selection, operative steps, and perioperative care of patients undergoing PRAA are detailed. RESULTS: 10 children (6 males, 4 females) underwent PRAA. There were no vaginal wall or urethral injuries. At 6 months postoperatively, all patients were passing stool spontaneously. No patients required dilation of the anoplasty in the postoperative period and there were no anal strictures identified. CONCLUSIONS: A modification of the cutback anoplasty can be performed in patients with a perineal fistula and the distal fistula tract within the sphincter complex. We have demonstrated that this can be performed safely and obviates the need for an anterior rectal wall dissection, thus eliminating the risk of injury to urethra or vagina. LEVEL OF EVIDENCE: IV.

The Use of an Inanimate Simulation Model for the Correction of an Anorectal Malformation in the Training of Colorectal Pediatric Surgery.

INTRODUCTION: An anorectal malformation (ARM) is a congenital malformation that requires surgical correction. To acquire the skills needed to perform this complex procedure, an affordable simulation model has previously been developed and validated. The aim of this study is to evaluate the suitability of this ARM model (with perineal fistula) for training in hands-on workshops. MATERIALS AND METHODS: The ARM model consists of a wooden casing with disposable perineal body. Participants in several international pediatric colorectal hands-on workshops in 2019 and 2020 were asked to participate. They were divided in a target group and an experienced group based on experience. All practiced the posterior sagittal anorectoplasty procedure on the model with multimodality guidance. Subsequently, statements on the suitability of the model for use during hands-on workshops were scored on a 5-point Likert scale. RESULTS: A total of 80 participants were included (43 surgical specialists, 13 pediatric surgery fellows, and 25 residents). Nearly, all statements scored at least a mean of >4.0, all scored significantly better than a neutral opinion. The target group (n = 58) scored higher compared with the experienced group (n = 22) on "transferability of the skills to the clinical setting" (means 4.4 vs. 4.0, p = 0.038); however, the "suitability as a replacement for an animal model" scored significantly lower (means 3.6 vs. 3.9, p = 0.049). No other differences were found. CONCLUSION: This affordable ARM model was regarded a suitable model for training during preclinical hands-on workshops and could be used for the specified steps of the procedure.

Gastric electrical stimulation improves symptoms and need for supplemental nutrition in children with severe nausea and vomiting: A ten-year experience.

BACKGROUND: Gastric electrical stimulation (GES) is a promising therapy for children with severe nausea and vomiting, but information on long-term outcomes is limited. We sought to evaluate the long-term efficacy and safety of GES and to describe patient benefit and satisfaction. METHODS: Using a prospective registry, we identified patients aged <21 years treated with GES at our institution between 2009 and 2019. Encounters were selected at baseline prior to GES and at follow-up at 1 month, 12 months, and the most recent visit. We compared symptoms, route of nutrition, and medication usage at baseline to follow-up timepoints. Factors associated with improvement were evaluated. We recorded complications and need for battery replacement. We contacted families to administer the Glasgow Children's Benefit Inventory (GCBI) and a parent satisfaction questionnaire. KEY RESULTS: Eighty-five patients (68.2% female, median age 15.8 years) completed a trial of temporary GES due to severe nausea and vomiting. Seventy-seven (90.6%) had a positive response and underwent permanent stimulator placement. Use of tube feeding or parenteral nutrition (PN) decreased from 72.7% at baseline to 29.9% at the most recent follow-up (p < 0.001). Higher baseline vomiting severity was associated with the ability to stop supplemental nutrition by 1 month (p < 0.05). Fourteen patients (18.2%) had complications, primarily due to stimulator-associated discomfort, and 29 (37.7%) required battery replacement. Median GCBI was +52.1, indicating health-related benefit. CONCLUSIONS AND INFERENCES: Children with severe nausea and vomiting treated with GES experienced significant and durable improvement in symptom severity and their ability to tolerate oral nutrition.

Are routine postoperative dilations necessary after primary posterior sagittal anorectoplasty? A randomized controlled trial.

AIM OF THE STUDY: For the past four decades, routine daily postoperative anal dilation by parents has been the standard treatment following a primary posterior sagittal anorectoplasty (PSARP). However, the clinical benefit of this practice has never been formally investigated. It is known that dilations can have a significant negative psychological impact on patients and families, and therefore, we aimed to study if routine dilations after a PSARP are necessary. METHODS: A prospective, single institution randomized controlled clinical trial was conducted on patients with anorectal malformations (ARM) at our institution between 2017 and 2019. Patients were randomized to either a dilation or non-dilation group following their PSARP. Inclusion criteria included age less than 24 months and all patients undergoing primary repair of their ARM (except for cloaca). Patient characteristics, type of ARM, presence of colostomy, postoperative stricture, need for a skin level revision (Heineke-Mikulicz anoplasty (HMA)), and need for redo PSARP were recorded. The primary outcome of the trial was stricture formation. The secondary outcome included strictures requiring interventions. A p-value of less than 0.05 was considered statistically significant. Institutional approval was obtained for this study and informed consents were obtained from all the patients. RESULTS: 49 patients were included in our study. 5 (21%) in the dilation group and 8 (32%) in the non-dilation group developed strictures (p=0.21). Of these, 3 (13%) patients in the dilation group required HMA, and 4 (16%) patients in the non-dilation group required HMA (p=0.72). 4 patients required a redo operation for strictures: 2 in the dilation arm (these patients despite the plan to do dilations, chose not to do them consistently) and 2 in the non-dilation arm (p=0.59). CONCLUSION: Routine dilations after PSARP do not significantly reduce stricture formation. Based on these results, non-dilation is a viable alternative, and HM anoplasty remains a good back-up plan if a stricture develops. LEVEL OF EVIDENCE: Level I.