Congenital Disseminated Malignant Rhabdoid Tumor Mimicking a Vascular Lesion.

A congenital disseminated malignant rhabdoid tumor (MRT) is an exceedingly rare and aggressive pediatric cancer marked by the presence of malignant rhabdoid cells in various organs, including the brain, kidneys, and soft tissues, at birth. It is often detected prenatally or shortly post-birth. The malignancy's aggressiveness results in a bleak prognosis, offering limited treatment options and low survival rates. Early diagnosis and comprehensive medical intervention are crucial, but managing this condition is complicated by its rarity. We herein presented a case of a 37 and 1/7 week gestation male infant with a rapidly growing arm soft tissue mass within two weeks, diagnosed as an MRT. Post-delivery examinations revealed multiple lesions in the lungs, kidney, liver, and adrenal glands. Notably, chemotherapy yielded a significant improvement in the arm lesion, contrasting with other lesions showing a limited response. This observation suggests potential tumor heterogeneity, emphasizing the necessity of diverse therapeutic regimens. Our case underscores the complexities of congenital disseminated MRT, prompting a reevaluation of treatment strategies for enhanced efficacy in managing this challenging pediatric cancer.

Utility of a Benchmarking Report for Balancing Infection Prevention and Antimicrobial Stewardship in Children with Complicated Appendicitis.

OBJECTIVE: To develop a severity-adjusted, hospital-level benchmarking comparative performance report for postoperative organ space infection and antibiotic utilization in children with complicated appendicitis. BACKGROUND: No benchmarking data exist to aid hospitals in identifying and prioritizing opportunities for infection prevention or antimicrobial stewardship in children with complicated appendicitis. METHODS: This was a multicenter cohort study using NSQIP-Pediatric data from 16 hospitals participating in a regional research consortium, augmented with antibiotic utilization data obtained through supplemental chart review. Children with complicated appendicitis who underwent appendectomy from 07/01/2015 to 06/30/2020 were included. Thirty-day postoperative OSI rates and cumulative antibiotic utilization were compared between hospitals using observed-to-expected (O/E) ratios after adjusting for disease severity using mixed effects models. Hospitals were considered outliers if the 95% confidence interval for O/E ratios did not include 1.0. RESULTS: 1790 patients were included. Overall, the OSI rate was 15.6% (hospital range: 2.6-39.4%) and median cumulative antibiotic utilization was 9.0 days (range: 3.0-13.0). Across hospitals, adjusted O/E ratios ranged 5.7-fold for OSI (0.49-2.80, P=0.03) and 2.4-fold for antibiotic utilization (0.59-1.45, P<0.01). Three (19%) hospitals were outliers for OSI (1 high and 2 low performers), and eight (50%) were outliers for antibiotic utilization (5 high and 3 low utilizers). Ten (63%) hospitals were identified as outliers in one or both measures. CONCLUSIONS: A comparative performance benchmarking report may help hospitals identify and prioritize quality improvement opportunities for infection prevention and antimicrobial stewardship, as well as identify exemplar performers for dissemination of best practices.

Postoperative Antibiotics, Outcomes, and Resource Use in Children With Gangrenous Appendicitis.

Importance: Gangrenous, suppurative, and exudative (GSE) findings have been associated with increased surgical site infection (SSI) risk and resource use in children with nonperforated appendicitis. Establishing the role for postoperative antibiotics may have important implications for infection prevention and antimicrobial stewardship. Objective: To compare SSI rates in children with nonperforated appendicitis with GSE findings who did and did not receive postoperative antibiotics. Design, Setting, and Participants: This was a retrospective cohort study using American College of Surgeons' National Surgical Quality Improvement Program (NSQIP)-Pediatric Appendectomy Targeted data from 16 hospitals participating in a regional research consortium. NSQIP data were augmented with operative report and antibiotic use data obtained through supplemental medical record review. Children with nonperforated appendicitis with GSE findings who underwent appendectomy between July 1, 2015, and June 30, 2020, were identified using previously validated intraoperative criteria. Data were analyzed from October 2022 to July 2023. Exposure: Continuation of antibiotics after appendectomy. Main Outcomes and Measures: Rate of 30-day postoperative SSI including both incisional and organ space infections. Complementary hospital and patient-level analyses were conducted to explore the association between postoperative antibiotic use and severity-adjusted outcomes. The hospital-level analysis explored the correlation between postoperative antibiotic use and observed to expected (O/E) SSI rate ratios after adjusting for differences in disease severity (presence of gangrene and postoperative length of stay) among hospital populations. In the patient-level analysis, propensity score matching was used to balance groups on disease severity, and outcomes were compared using mixed-effects logistic regression to adjust for hospital-level clustering. Results: A total of 958 children (mean [SD] age, 10.7 [3.7] years; 567 male [59.2%]) were included in the hospital-level analysis, of which 573 (59.8%) received postoperative antibiotics. No correlation was found between hospital-level SSI O/E ratios and postoperative antibiotic use when analyzed by either overall rate of use (hospital median, 53.6%; range, 31.6%-100%; Spearman ρ = -0.10; P = .71) or by postoperative antibiotic duration (hospital median, 1 day; range, 0-7 days; Spearman ρ = -0.07; P = .79). In the propensity-matched patient-level analysis including 404 patients, children who received postoperative antibiotics had similar rates of SSI compared with children who did not receive postoperative antibiotics (3 of 202 [1.5%] vs 4 of 202 [2.0%]; odds ratio, 0.75; 95% CI, 0.16-3.39; P = .70). Conclusions and Relevance: Use of postoperative antibiotics did not improve outcomes in children with nonperforated appendicitis with gangrenous, suppurative, or exudative findings.

Transcystic Laparoscopic Common Bile Duct Exploration for Pediatric Patients with Choledocholithiasis: A Multi-Center Study.

BACKGROUND: Patients with choledocholithiasis are often treated with endoscopic retrograde cholangiopancreatography (ERCP) followed by laparoscopic cholecystectomy (LC). Upfront LC, intraoperative cholangiogram (IOC), and possible transcystic laparoscopic common bile duct exploration (LCBDE) could potentially avoid the need for ERCP. We hypothesized that upfront LC + IOC ± LCBDE will decrease length of stay (LOS) and the total number of interventions for children with suspected choledocholithiasis. METHODS: A multicenter, retrospective cohort study was performed on pediatric patients (<18 years) between 2018 and 2022 with suspected choledocholithiasis. Demographic and clinical data were compared for upfront LC + IOC ± LCBDE and possible postoperative ERCP (OR1st) versus preoperative ERCP prior to LC (OR2nd). Complications were defined as postoperative pancreatitis, recurrent choledocholithiasis, bleeding, or abscess. RESULTS: Across four centers, 252 children with suspected choledocholithiasis were treated with OR1st (n = 156) or OR2nd (n = 96). There were no differences in age, gender, or body mass index. Of the LCBDE patients (72/156), 86% had definitive intraoperative management with the remaining 14% requiring postoperative ERCP. Complications were fewer and LOS was shorter with OR1st (3/156 vs. 15/96; 2.39 vs 3.84 days, p < 0.05). CONCLUSION: Upfront LC + IOC ± LCBDE for children with choledocholithiasis is associated with fewer ERCPs, lower LOS, and decreased complications. Postoperative ERCP remains an essential adjunct for patients who fail LCBDE. Further educational efforts are needed to increase the skill level for IOC and LCBDE in pediatric patients with suspected choledocholithiasis. LEVEL OF EVIDENCE: Level III.

Use of Antipseudomonal Antibiotics is not Associated with Lower Rates of Postoperative Drainage Procedures or More Favorable Culture Profiles in Children with Complicated Appendicitis: Results from a Multicenter Regional Research Consortium.

OBJECTIVE: To compare rates of postoperative drainage and culture profiles in children with complicated appendicitis treated with the two most common antibiotic regimens with and without antipseudomonal activity (piperacillin-tazobactam [PT] and ceftriaxone with metronidazole [CM]). SUMMARY OF BACKGROUND DATA: Variation in use of antipseudomonal antibiotics has been driven by a paucity of multicenter data reporting clinically relevant, culture-based outcomes. METHODS: Retrospective cohort study of patients with complicated appendicitis (7/2015-6/2020) using NSQIP-Pediatric data from 15 hospitals participating in a regional research consortium. Operative report details, antibiotic utilization, and culture data were obtained through supplemental chart review. Rates of 30-day postoperative drainage and organism-specific culture positivity were compared between groups using mixed effects regression to adjust for clustering after propensity matching on measures of disease severity. RESULTS: 1002 children met criteria for matching (58.9% received CM and 41.1% received PT). In the matched sample of 778 patients, children treated with PT had similar rates of drainage overall (PT: 11.8%, CM: 12.1%; OR 1.44 [OR:0.71-2.94]) and higher rates of drainage associated with growth of any organism (PT: 7.7%, CM: 4.6%; OR 2.41 [95%CI:1.08-5.39]) and Escherichia coli (PT: 4.6%, CM: 1.8%; OR 3.42 [95%CI:1.07-10.92]) compared to treatment with CM. Rates were similar between groups for drainage associated with multiple organisms (PT: 2.6%, CM: 1.5%; OR 3.81 [95%CI:0.96-15.08]) and Pseudomonas (PT: 1.0%, CM: 1.3%; OR 3.42 [95%CI:0.55-21.28]). CONCLUSIONS AND RELEVANCE: Use of antipseudomonal antibiotics is not associated with lower rates of postoperative drainage procedures or more favorable culture profiles in children with complicated appendicitis.

Utilizing a critical airway response team expedites esophageal button battery removal.

BACKGROUND: Esophageal button battery ingestion is a significant problem that can lead to significant complications such as tracheoesophageal fistula, esophageal perforation, and aortoesophageal fistula. Due to this, prompt recognition and treatment is integral in the care of these patients. METHODS: Patients who presented to a single institution from August 2015 to April 2022 with esophageal button battery ingestion were included in this study. All esophageal button battery ingestion patients were included in a clinical algorithm for Critical Airway Response Team (CART) activation in October 2019. Time from diagnosis to treatment was compared for pre-CART clinical algorithm implementation to post-CART. RESULTS: Data on pre-CART patients (n = 6) and post-CART patients (n = 7) was collected. Including esophageal button battery ingestions to CART activations shortened the time from chest x-ray to button battery removal from 73 ± 32 min to 35 ± 11 min (p < 0.05). CONCLUSION: These data highlight the importance of implementation of a clinical care algorithm to shorten the time from diagnosis to treatment in patients with esophageal button battery ingestion. LEVEL OF EVIDENCE: III.

Gertrude Herzfeld: The woman who dared to be a surgeon.

Gertrude Marianne Amalia Herzfeld (1890-1981), the first practicing female surgeon in Scotland, overcame bias against women in medicine and pediatric specialists in surgery. After her graduation from the University of Edinburgh Medical School (1914), she became the first female house surgeon at the Royal Hospital for Sick Children, Edinburgh. In 1920, she became the first practicing woman surgeon to become a Fellow of the Royal College of Surgeons of Edinburgh. She left a handful of publications in pediatric surgery: a "radical cure" for inguinal hernia, i.e., early surgery (1925); a review of abdominal surgery in infancy and childhood (1937); surgery for the acute abdomen (1939); and intestinal obstruction (1945). They offer a twenty-year window into how children's surgery was once practiced, when operations were done in patients' homes, and decisions for operation depended solely on the history and physical exam without laboratory testing and radiological imaging. As a series of snapshots over two decades, they reveal how her practice evolved in such areas as fluid resuscitation and radiological reduction of intussusception. She remained steadfast to a careful physical examination and early operation. While she did not document her practice in the care of children with ambiguous genitalia and intersex conditions, she approached the formidable anatomic, psychological, and social challenges of her patients and their families with patience and understanding. Herzfeld was devoted to the care of yet another marginalized population that today is subsumed by the acronym LGBTQIA, yet another area where she was far ahead of her time. Level of evidence: Level VII.

Childhood injury prevention: Where we've been and where we need to be.

Injury remains the leading cause of death for children and adolescents in the US, and firearm injury has overtaken motor-vehicle crashes as the leading mechanism in the US since the topic of injury prevention was last reviewed in this journal. The success of injury prevention efforts relies on multidisciplinary collaboration, and pediatric surgeons play a central role as clinicians who provide and coordinate the care for injured children, as trauma program leaders, and as advocates for children's health and safety. This review will provide a concise history of injury prevention in the US and highlights three areas where pediatric surgeons have an opportunity to impart impactful change in their communities that could lead to lower injury rates.

Limited nutrient availability in the tumor microenvironment renders pancreatic tumors sensitive to allosteric IDH1 inhibitors.

Nutrient-deprived conditions in the tumor microenvironment (TME) restrain cancer cell viability due to increased free radicals and reduced energy production. In pancreatic cancer cells a cytosolic metabolic enzyme, wild-type isocitrate dehydrogenase 1 (wtIDH1), enables adaptation to these conditions. Under nutrient starvation, wtIDH1 oxidizes isocitrate to generate α-ketoglutarate (αKG) for anaplerosis and NADPH to support antioxidant defense. In this study, we show that allosteric inhibitors of mutant IDH1 (mIDH1) are potent wtIDH1 inhibitors under conditions present in the TME. We demonstrate that low magnesium levels facilitate allosteric inhibition of wtIDH1, which is lethal to cancer cells when nutrients are limited. Furthermore, the Food & Drug Administration (FDA)-approved mIDH1 inhibitor ivosidenib (AG-120) dramatically inhibited tumor growth in preclinical models of pancreatic cancer, highlighting this approach as a potential therapeutic strategy against wild-type IDH1 cancers.

Patching of the Inferior Vena Cava Following Lateral Venorrhaphy in Penetrating Traumatic Injury.

Penetrating injury to the inferior vena cava (IVC) is associated with high morbidity and mortality. Luminal narrowing can occur following lateral venorrhaphy and can lead to future morbidity. This case report discusses the success of patch repair following lateral venorrhaphy in two trauma patients. We describe the use of patch repair to eliminate stenosis of the IVC resulting from primary repair in the setting of traumatic injury. Furthermore, trauma patients are known to be at high risk for venous thromboembolism, and we describe the use of low molecular weight heparin as chemical prophylaxis for prevention of this complication following patch repair.

Primary Sternal Cleft Repair in First Week of Life.

We present a full-term neonate with a postnatal diagnosis of a sternal cleft. Cardiac evaluation was otherwise remarkable for a patent ductus arteriosus and patent foramen ovale. Computed tomography scan confirmed the absence of a sternum except for a small inferior portion, including the xyphoid process. The patient underwent primary repair at five days of age through a midline incision where an absence of pericardium anteriorly was noted. She was extubated successfully the next day and discharged home on postoperative day 3. On follow-up examination, her sternum has been healing well without any clinical concerns. We advocate early repair of such defects in order to allow primary closure in a tension-free manner.

Preoperative Blood Transfusions and Morbidity in Neonates Undergoing Surgery.

BACKGROUND: Blood transfusions in the neonatal patient population are common, but there are no established guidelines regarding transfusion thresholds. Little is known about postoperative outcomes in neonates who receive preoperative blood transfusions (PBTs). METHODS: Using the American College of Surgeons National Surgical Quality Improvement Program-Pediatric Participant Use Data Files from 2012 to 2015, we identified all neonates who underwent surgery. Mortality and composite morbidity (defined as any postoperative complication) in neonates who received a PBT within 48 hours of surgery were compared with that in neonates who did not receive a transfusion. RESULTS: A total of 12 184 neonates were identified, of whom 1209 (9.9%) received a PBT. Neonates who received a PBT had higher rates of preoperative comorbidities and worse postoperative outcomes when compared with those who did not receive a transfusion (composite morbidity: 46.2% vs 16.2%; P < .01). On multivariable regression analysis, PBTs were independently associated with increased 30-day morbidity (odds ratio [OR] = 1.90; 95% confidence interval [CI]: 1.63-2.22; P < .01) and mortality (OR = 1.98; 95% CI: 1.55-2.55; P < .01). In a propensity score-matched analysis, PBTs continued to be associated with increased 30-day morbidity (OR = 1.53; 95% CI: 1.29-1.81; P < .01) and mortality (OR = 1.58; 95% CI: 1.24-2.01; P = .01). CONCLUSIONS: In a propensity score-matched model, PBTs are independently associated with increased morbidity and mortality in neonates who undergo surgery. Prospective data are needed to better understand the potential effects of a red blood cell transfusion in this patient population.

Necrotizing enterocolitis totalis: High mortality in the absence of an aggressive surgical approach.

BACKGROUND: Necrotizing enterocolitis is the leading case of gastrointestinal-related morbidity in premature infants. Necrotizing enterocolitis totalis is an aggressive form of necrotizing enterocolitis, which has traditionally been managed with comfort care. Recent advances in management of short bowel syndrome have resulted in some reported long-term survival. METHODS: Using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, studies that reported outcomes in children with necrotizing enterocolitis totalis were identified. The definition of necrotizing enterocolitis totalis was captured along with length of follow-up, patient demographics, and outcomes. RESULTS: A total of 766 articles were screened, of which 166 were selected for full article review. Of these, 32 articles included data on 414 patients with necrotizing enterocolitis totalis. In the majority of studies (52%), necrotizing enterocolitis totalis was not defined. Aggressive surgical therapy (defined as bowel resection or fecal diversion) was undertaken in 32 patients (7.7%), with a mortality rate of 68.8%. In contrast, nonaggressive surgical therapy was undertaken in 382 patients (92.3%), and the mortality in these patients was 95%. Long-term outcomes for necrotizing enterocolitis totalis survivors, such as length of time on parenteral nutrition, progression to liver and/or small bowel transplant, and quality of life, were not reported. CONCLUSION: We found that there is no accepted definition of necrotizing enterocolitis totalis. Aggressive surgical therapy is rarely pursued, which likely drives the overall high mortality rate. This study underscores the importance of standardizing the definition of necrotizing enterocolitis totalis and capturing short and long-term outcomes prospectively. With more aggressive surgical therapy, more infants are likely to survive this abdominal catastrophe, which was once thought to be uniformly fatal.

Adherence to Inpatient Venous Thromboembolism Prophylaxis: A Single Institution's Concurrent Review.

Hospital-acquired venous thromboembolism (VTE) affects morbidity and mortality and increases health care costs. Poor adherence to recommended prophylaxis may be a potential cause of ongoing events. This study aims to identify institutional adherence rates and barriers to optimal VTE prophylaxis. The authors performed patient and nurse interviews and a concurrent review of clinical documentation, utilizing a cloud-based, HIPAA-compliant tool, on a convenience sample of hospitalized patients. Adherence and agreement between different assessment modalities were calculated. Seventy-six patients consented for participation. Nurse documented adherence was 66% (29/44), 44% (27/61), and 89% (50/56) for mechanical, ambulatory, and chemoprophylactic prophylaxis, respectively. Patient report and nurse documentation showed moderate agreement for mechanical and no agreement for ambulatory adherence (κ = 0.51 and 0.07, respectively). Concurrent review using a cloud-based tool can provide robust, timely, and relevant information on adherence to recommended VTE prophylaxis. Iterative concurrent reviews can guide efforts to improve adherence and reduce rates of hospital-acquired VTE.

Dr. Orvar Swenson and the Pull-Through.

Dr. Orvar Swenson is best remembered for developing the Swenson pull-through, a technique he developed to treat Hirschsprung's disease. After graduating from Harvard Medical School and beginning his residency at Peter Bent Brigham Hospital, Dr. Swenson observed that patients with Hirschsprung's disease and toxic megacolon resumed normal bowel function after placement of transverse colostomies. His observation led to studying the patency of his patients' colons using barium enema contrast studies. At the collapsed portion of the colon, he performed rectal biopsies leading to the discovery that the cause of Hirschsprung's disease is that the collapsed portion of the colon lacks the Auerbach plexus. The Swenson pull-through removes this aganglionic portion of the colon and cures the patient. His career grew from there as he traveled to academic institutions teaching his technique. He is remembered fondly for his contributions to pediatric surgery through the restructuring of pediatric surgery departments, pediatric surgery research, and writing and editing multiple volumes of Pediatric Surgery, the standard textbook for pediatric surgeons. He died peacefully in 2012 at the age of 103 years.

Short-term outcomes in children undergoing restorative proctocolectomy with ileal-pouch anal anastomosis.

INTRODUCTION: Patients with familial adenomatous polyposis (FAP) and ulcerative colitis (UC) commonly undergo restorative proctocolectomy with ileal-pouch anal anastomosis (RP-IPAA). We sought to describe patient characteristics and postoperative outcomes in this patient population. METHODS: Using the National Surgical Quality Improvement Program-Pediatric Participant Use Files from 2012 to 2015, children who were 6-18years old who underwent RP-IPAA for FAP or UC were identified. Postoperative morbidity, including reoperation and readmission were quantified. Associations between preoperative characteristics and postoperative outcomes were analyzed. RESULTS: A total of 260 children met the inclusion criteria, of which 56.2% had UC. Most cases were performed laparoscopically (58.1%), and the operative time was longer with a laparoscopic versus open approach (326 [257-408] versus 281 [216-391] minutes, p=0.02). The overall morbidity was 11.5%, and there were high reoperation and readmission rates (12.7% and 21.5%, respectively). On bivariate analysis, preoperative steroid use was associated with reoperation (22.5% versus 10.9%, p=0.04). On multivariable regression analysis, obesity was independently associated with reoperation (odds ratio: 3.34 [95% confidence intervals: 1.08-10.38], p=0.04). CONCLUSIONS: Children who undergo RP-IPAA have high rates of overall morbidity, reoperation, and readmission. Obesity was independently associated with reoperation. This data can be used by practitioners in the preoperative setting to better counsel families and establish expectations for the postoperative setting. TYPE OF STUDY: Retrospective Comparative Study. LEVEL OF EVIDENCE: Level III.

Euglycemic Diabetic Ketoacidosis Due to Sodium-Glucose Cotransporter 2 Inhibitor Use in Two Patients Undergoing Pancreatectomy.

Background: Euglycemic diabetic ketoacidosis (euDKA) is a potential side effect associated with inhibitors of the sodium-glucose cotransporter 2 (SGLT-2). This effect is most often recognized during physiologic stress (i.e., sepsis) or in patients who undergo surgery. Case presentations: Case 1: A 66-year-old woman underwent distal pancreatectomy with en bloc splenectomy after presenting with a biopsy-proven pancreatic adenocarcinoma in the body of the pancreas noted incidentally on a screening magnetic resonance imaging for an ovarian mass. The patient had a history of type 2 diabetes mellitus (T2DM) and used canagliflozin, which she was instructed to hold 24 h before surgery. Case 2: A 75-year-old man underwent a pylorus-preserving pancreaticoduodenectomy after presenting with obstructive jaundice. This patient also had a history of T2DM and was on dapagliflozin, which he was also instructed to hold 24 h before surgery. Postoperatively, both patients were diagnosed with euDKA, which was suspected primarily because of intraoperative and postoperative polyuria. Conclusions: SGLT-2 inhibitors are associated with euDKA that can be potentiated in patients who undergo surgery. This medication side effect can be easily unrecognized and potentially lead to significant morbidity.

Outcomes in Children Undergoing Surgery for Congenital Pulmonary Airway Malformations in the First Year of Life.

BACKGROUND: Treatment of congenital pulmonary airway malformations (CPAMs) is generally surgical resection; however, there is controversy regarding the optimal timing of surgical intervention, especially in asymptomatic patients. STUDY DESIGN: Using the American College of Surgeons National Surgical Quality Improvement Program-Pediatric Participant Use Files from 2012 to 2015, children who underwent lung resection for CPAMs were identified. Outcomes in children who underwent lung resection during the neonatal period were compared with those who underwent resection beyond the neonatal period, but during the first year of life (non-neonates). RESULTS: A total of 541 patients (20.7% neonates and 79.3% non-neonates) were identified. Neonates had higher rates of preoperative comorbidities and worse postoperative outcomes when compared with non-neonates (morbidity 19.6% vs 5.4%, p < 0.0001). On multivariable regression analysis, the presence of preoperative symptoms (defined as oxygen dependence or ventilatory support) was independently associated with increased morbidity (odds ratio 3.91 [range 1.6 to 9.57], p = 0.003). In a subgroup analysis of asymptomatic neonates compared with asymptomatic non-neonates, there was no difference in overall morbidity (7.4% vs 4.4%, p = 0.33). CONCLUSIONS: These data suggest that lung resection for CPAMs in the neonatal period in asymptomatic children are not associated with increased 30-day morbidity. The presence of preoperative symptoms was independently associated with increased morbidity in a multivariable regression model. More data are needed to better understand the long-term outcomes and better define the optimal timing of surgery in this patient population.