BACKGROUND:Undifferentiated embryonal sarcoma of the liver (UESL) is a rare tumor for which there are few evidence-based guidelines. The aim of this study was to define current management strategies and outcomes for these patients using a multi-institutional dataset curated by the Pediatric Surgical Oncology Research Collaborative.METHODS:Data were collected retrospectively for patients with UESL treated across 17 children's hospitals in North America from 1989 to 2019. Factors analyzed included patient and tumor characteristics, PRETEXT group, operative details, and neoadjuvant/adjuvant regimens. Event-free and overall survival (EFS, OS) were the primary and secondary outcomes, respectively.RESULTS:Seventy-eight patients were identified with a median age of 9.9 years [interquartile range [IQR): 7-12]. Twenty-seven patients underwent resection at diagnosis, and 47 patients underwent delayed resection, including eight liver transplants. Neoadjuvant chemotherapy led to a median change in maximum tumor diameter of 1.6 cm [IQR: 0.0-4.4] and greater than 90% tumor necrosis in 79% of the patients undergoing delayed resection. R0 resections were accomplished in 63 patients (81%). Univariate analysis found that metastatic disease impacted OS, and completeness of resection impacted both EFS and OS, while multivariate analysis revealed that R0 resection was associated with decreased expected hazards of experiencing an event [hazard ratio (HR): 0.14, 95% confidence interval (CI): 0.04-0.6]. At a median follow-up of 4 years [IQR: 2-8], the EFS was 70.0% [95% CI: 60%-82%] and OS was 83% [95% CI: 75%-93%].CONCLUSION:Complete resection is associated with improved survival for patients with UESL. Neoadjuvant chemotherapy causes minimal radiographic response, but significant tumor necrosis.

Objective: To compare the occurrence of fetal bradycardia in open versus fetoscopic fetal spina bifida surgery. Methods: This is a single-institution retrospective cohort study of patients undergoing open (n = 25) or fetoscopic (n = 26) spina bifida repair between 2017 and 2022. From October 2017 to June 2020, spina bifida repairs were performed via an open classical hysterotomy, and from November 2020 to June 2022 fetoscopic repairs were performed following transition to this technique. Fetal heart rate (FHR) in beats per minute (bpm) was recorded via echocardiography every 15 min during the procedure. Cohort characteristics, fetal bradycardia and maternal physiologic parameters were compared between the groups. Results: Fetuses undergoing an open repair more frequently developed bradycardia defined as <110 bpm (32% vs. 3.8%, p = 0.008), and a trend was observed for FHR decreases more than 25 bpm from baseline (20% vs. 3.8%, p = 0.073). Profound bradycardia less than 80 bpm was rare, occurring in only three operations (two in open, one in fetoscopic repair) with two fetuses (one in each group) requiring emergency cesarean delivery. Conclusion: When compared to open fetal surgery, fetal bradycardia occurred less frequently in fetoscopic surgery despite a significantly greater anesthetic exposure and the use of the intraamniotic carbon dioxide insufflation.

INTRODUCTION:Fetal thoracoamniotic shunts are common lifesaving interventions but frequently require replacement. Needle fetal thoracoscopy is a technique that uses standard thoracoamniotic shunt introducer sheaths to permit direct visualization and even instrument manipulation during shunt deployment to facilitate optimal positioning and primary shunt function in the most challenging cases. CASE PRESENTATION:In this study, 5 patients who underwent needle fetal thoracoscopy-assisted thoracoamniotic shunt placement were reviewed. Three patients with large, macrocystic congenital pulmonary airway malformations (CPAMs) with evidence of worsening mediastinal shift and/or hydrops and 2 patients with large chylothorax with fetal hydrops were treated. Four cases had previous shunts that failed due to poor sonographic visualization during initial placement, cyst septations, shunt obstruction, or dislodgment. Needle fetal thoracoscopy was used to disrupt cyst walls and septations, clear hematoma, and confirm the optimal initial position of the shunt. In this series, 1 severe CPAM patient with a short cervix developed preterm labor postoperatively resulting in neonatal demise. The remaining 4 patients experienced resolution of hydrops and progressed to successful delivery with excellent neonatal outcomes. CONCLUSION:Needle fetal thoracoscopy is a procedure that may be selectively deployed in challenging thoracoamniotic shunt cases impacted by recurrent failure, poor sonographic windows, and challenging fetal positioning.

Fetal airway obstruction in one twin of a diamniotic pregnancy presents unique challenges. Very few cases of ex-utero-intrapartum-treatment (EXIT) procedures for twin pregnancy have been reported and only in dichorionic pregnancies. We report a singular methodology for EXIT-to-airway procedures in two pregnancies involving monochorionic and dichorionic twins. Two cases of EXIT-to-airway in twin pregnancies were performed in 2018 and 2019 at a regional fetal treatment center. Case 1 involved a giant cervical teratoma in a monochorionic-diamniotic twin pregnancy with preterm labor at 29 weeks. Case 2 involved a dichorionic-diamniotic pregnancy with a large cervical lymphatic malformation with preterm labor at 36 weeks. In each case, the polyhydramnios caused the affected twin's amniotic sac to be the presenting sac for the surgical approach. Bronchoscopy and successful intubation was completed after 22 and 10 minutes of uteroplacental bypass, respectively. The bystander twins were delivered second without intubation and resuscitated without perinatal distress. EXIT-to-airway appears to be a reasonable option for twins including monochorionic pregnancies, via delivery of the affected twin first followed by delivery of the bystander twin. Thoughtful preparation and counseling by an experienced multidisciplinary team permits an EXIT-to-airway approach for twin pregnancies even in an emergent setting.

Management of splenic cysts in children remains undefined. Sclerotherapy is an innovative, less invasive treatment. This study examined the safety and preliminary effectiveness of sclerotherapy for splenic cysts in children compared with those of surgical treatment. A retrospective review of pediatric patients treated for nonparasitic splenic cysts from 2007 to 2021 was performed at a single institution. Posttreatment outcomes for patients who underwent either expectant management, sclerotherapy, or surgery were reviewed. Thirty patients aged between 0 and 18 years met the inclusion criteria. Cysts in 3 of 8 patients who underwent sclerotherapy were either unresolved or recurred. Patients who underwent sclerotherapy and required surgery for residual symptomatic cyst had an initial cyst diameter of >8 cm. Symptoms resolved in 5 of 8 patients who underwent sclerotherapy, with a significantly reduced cyst size compared with that in patients with continued symp-toms who underwent sclerotherapy (61.4% vs 7.0%, P = .01). Sclerotherapy is an effective treatment for splenic cysts, particularly those measuring <8 cm. However, surgical excision may be preferable for large cysts.

Introduction: Appendectomy for acute appendicitis is the most common pediatric intra-abdominal operation. Current literature supports the notion that modest in-hospital, preoperative delays are not associated with greater patient morbidity. However, there is less certainty regarding the role that hour-of-presentation plays in determining the timing of surgery. Thus, we aimed to evaluate how after-hours presentation may relate to the timing of surgery and to assess the outcomes and resource utilization associated with expedited appendectomy compared to nonexpedited.Methods: Patient records for children who underwent an appendectomy at a freestanding pediatric hospital from 2015 to 2019 were reviewed. Business hour presentations were defined as arrival at the emergency department from 7 AM to 6 PM. Primary outcomes were hospital length of stay (LOS), cost derived from the Pediatric Health Information System database, perforation, surgical complications, and 30-day readmissions.Results: Nine hundred forty-two patients underwent appendectomy over the study period. The median time to OR was 2.0 h in the expedited cohort and 9.8 h in the nonexpedited group. Presentation during business hours was associated with 4.4 higher odds (P < 0.001) of expedited workflow. Expedited appendectomies were associated with shorter hospital LOS (11.5 h, P < 0.001), less costly admissions ($1,155, P < 0.001); LOS measured in mid-nights, perforation and readmission rates were similar between groups. Conclusions: We found reduced resource utilization associated with expedited appendec-tomy. Additionally, the demonstrated association between the time of presentation to the emergency department (ED) and the timing of surgery may be utilized to inform staffing and resource deployment decisions. Further research regarding the generalizability and sustainability of an expedited presurgical workflow in pediatric appendectomy is certainly indicated.(c) 2022 Elsevier Inc. All rights reserved.

Background: Controversy exists regarding how operative timing affects patient safety and resource uti-lization for acute appendicitis. Over 3 years, our institution trialed efforts to optimize appendectomy workflow. Our aim is to describe the ramifications of expediting appendectomy and implementing stan-dardized protocols relative to historic controls.Methods: Patient records at a freestanding children's hospital were reviewed from synchronized 6-month periods from 2019 to 2021. During Year 1 (historic), no standardized workflows existed. In Year 2 (ex-pedited), appendicitis management was protocoled using a clinical quality improvement bundle, which included performing appendectomies within two hours of diagnosis. In Year 3 (QI), operative timing was relaxed to the same calendar day while all prior QI initiatives continued. Descriptive statistics were per-formed, using hospital length of stay (LOS) as the primary outcome. Results: 298 patients underwent appendectomy for acute appendicitis. The median expedited workflow LOS was 15.3 hours shorter ( p = 0.003) than historic controls; however, this was sustained despite relax-ation of surgical urgency in the QI workflow. No differences in perforation rates were observed. During the expedited workflow, OR overtime staffing expense increased by $90,0 0 0 with no significant change in hospital costs. In multivariate regression, perforation was the only variable associated with LOS.Conclusion: Hospital LOS can be shortened by expediting appendectomy. However, in our institution this did not decrease hospital costs and was furthermore balanced by higher personnel expenses. A sustained decrease in LOS after relaxing operative urgency standards implies that concurrent QI initiatives represent a more effective and cost-efficient strategy to decrease hospital resource utilization. Level of evidence: Level III (c) 2022 Elsevier Inc. All rights reserved.

A detailed understanding of protective immunity against SARS-CoV-2 is incredibly important in fighting the pandemic. Central to protective immunity is the ability of the immune system to recall previous exposures. Although antibody and T cell immunity have gained considerable attention, the contribution of the NK cell compartment to immune recall and protection from SARS-CoV-2 has not been explored. In this study, we investigate the NK cell responses to stimulation with SARS-CoV-2 in previously exposed and non-exposed individuals. We show that NK cells demonstrate an enhanced CD4+ T cell dependent response when re-exposed to SARS-CoV-2 antigen. The enhanced response is dependent on T cells and correlates with the number of SARS-CoV-2 specific CD4 T cells. We find that IL-2 is a critical mediator of NK cell function. These findings suggest that NK cells contribute to the protective responses against SARS-CoV-2 through a cooperation with antigen-specific CD4 T cells and have significant implications on our understanding of protective immunity in SARS-CoV-2.

To the Editor: Near-infrared technology is increasingly available in the modern pediatric operating room. In addition to venous injection of indocyanine green for pulmonary and biliary applications, recent advances allow visualized instrumentation, opening a host of new potential applications in pediatric surgery. As archetypal example, the Infrared Illumination System (IRIS) by Stryker Endoscopy (San Jose, CA) includes sterile 6-French close-ended ureteral catheters with NIR-emitting 75-mm filaments that can be placed by cystoscopy. The lighted stent is hard to see and requires turning down the light on surrounding structures which is suboptimal. Stents emitting near infrared may therefore be useful for solving this problem by allowing for visualization of difficult structures without decreasing light to surrounding tissue. These stents have become popular with adult colorectal surgeons, but no dedicated pediatric infrared hardware is currently available. This letter presents an innovative application of these stents by removing the fibers from the flexible sheath and deploying via alternate orifices to illuminate the rectum in two neonatal colorectal operations. The first patient was a 4-month-old female undergoing reresection of a sacrococcygeal teratoma after incomplete resection at birth for an Altman Type IV lesion. In prone jackknife position, the re-operative tissue planes inferior to the coccyx were difficult to dissect. To better delineate the rectum, insertion of an infrared ureteral stent was attempted; however, the stent was too stiff to easily traverse the valves of Houston in the distal rectum. The inner fibers were removed from two stents and easily advanced per anus into the rectum. Using the open-field camera (Figure 1A), additional tissue outside of rectal serosa was identified and resected, facilitating a more complete dissection. The second patient was an infant with an anorectal malformation comprising imperforate anus with a prostatic rectourethral fistula. As a full-term newborn, he underwent diverting colostomy; subsequently, at 6 weeks, he underwent definitive repair. Cystoscopy confirmed the preoperative distal colostogram suggestion of a fistula from the prostatic urethra to the rectum. The working channel of the scope— and the fistula itself—were too small to accommodate the ureteral stent. Therefore, the infraredfiberswere removed and passed through the fistula easily under visualization. The fistula was clearly identified at laparoscopy (Figure 1B), informing appropriate dissection margins. The infant was turned prone for posterior sagittal anorectoplasty and the open-field camera additionally located the fistula, expediting safe ligation without injury to the prostate or urethra. Written informed consent was obtained from both patients families. We describe an off-label modification borne by clinical need. The stents themselves were unable to be used due to their size and rigidity which was incompatible with pediatric tissues. After removal of the NIR fibers from the stent, direct insertion into intestinal orifices can be successfully accomplished. Fracture of the optic fiber is possible in or out of the sheath; however the fibers themselves are very pliable. We were aware of the potential risk for fracture, nevertheless, and prepared to retrieve if necessary. We also chose to perform this procedural innovation only when working distally in the rectum where retrieval would be easily accomplished. These novel uses of the ureteral stent components may trigger additional commercial development, and will need to be assessed on a larger scale to determine ideal applications and limitations. Collaborations between pediatric surgeons and industry will help to develop fluorescent instrumentation dedicated specifically for

Ott, Katherine C. MD; Oria, Marc PhD; Redden, Catherine C. MS; Kang, Hee K. PhD; Skital, Veronica M. MD; Turner, Lucas E. BS; Alhajjat, Amir M. MD; Duru, Soner MD; Peiro, Jose L. MD; Shaaban, Aimen F. MD, FACS Author Information

Care for pediatric patients with serious or potentially life-limiting illness involves the interplay of multiple medical and surgical teams within the hospital. Pediatric surgeons are capable of performing procedures that can improve the quality of life for children facing serious illness, but which also carry the potential for significant risk and burden. Patients and families are often faced with decisions about invasive surgical procedures and interventions, stressing the need for seamless collaboration between palliative care and surgical providers. Equally important is the need for clear and open-ended communication with patients and families by all medical teams to determine if potential surgical procedures and interventions align with their goals and to ensure that the perceived benefits of interventions outweigh any risks. Over the last two decades, pediatric palliative care has grown into a thriving medical subspecialty Despite the importance of collaborative care, there is lack of literature on the interaction of pediatric surgery and palliative care and the role of pediatric surgeons in providing primary palliative care. This review defines surgical pediatric palliative care, and provides an in-depth discussion of the unique complexities involved in caring for children with serious and potentially life-limiting illness, while highlighting specific challenges through detailed case presentations.

Hepatocellular carcinoma (HCC) is a rare cancer in children, with various histologic subtypes and a paucity of data to guide clinical management and predict prognosis.

Objective Liver herniation is a known risk factor for increased severity in CDH and is associated with clinically significant pulmonary hypoplasia and pulmonary hypertension. Better studies are needed to understand the growth of the herniated liver compared to the liver that remains in the abdomen and how this liver growth then affects lung development. Serial hi-resolution fetal MRI enables characterization of liver growth throughout gestation and examination of macroscopic features that may regulate liver growth. Here, we hypothesized that the nature of liver herniation affects liver growth and, in turn, affects lung growth. Methods Clinical data were retrospectively collected from consecutive cases of prenatally diagnosed isolated left-sided or right-sided CDH from June 2006 to August 2021. Only those cases with MRI lung volumetry for both mid-gestation and late-gestation time points were recruited for analysis. Cases with fetal chromosomal abnormalities and other major structural abnormalities were excluded. Fractional liver volume and liver growth was indexed to estimated fetal weight and compared to lung growth. Results Data was collected from 28 fetuses with a left liver-down CDH (LLD), 37 left liver-up CDH (LLU) and 9 right liver-up CDH (RLU). Overall, RLU fetuses had greater overall and fractional (intra-thoracic vs. intra-abdominal) liver growth when compared to LLD and LLU fetuses. Additionally, intra-thoracic liver growth was consistently slower than intra-abdominal liver growth for either right- or left-sided CDH. When the liver was not herniated, a positive correlation was seen between liver growth and lung growth. However, when the liver was herniated above the diaphragm, this positive correlation was lost. Conclusion Right-sided CDH fetuses exhibit greater liver growth compared to left-sided CDH. Liver herniation disrupts the normal positive correlation between liver and lung growth that is seen when the liver is entirely within the abdomen.

BACKGROUND Performance of a trauma tertiary survey (TTS) reduces rates of missed injuries, but performance has been inconsistent at trauma centers. The objectives of this study were to assess whether quality improvement (QI) efforts would increase the frequency of TTS documentation and determine if TTS documentation would increase identification of traumatic injuries. Our hypothesis was that QI efforts would improve documentation of the TTS. METHODS Before-and-after analysis of QI interventions at a level 1 trauma center was performed. The interventions included an electronic template for TTS documentation, customized educational sessions, and emphasis from trauma leadership on TTS performance. The primary outcome was documentation of the TTS. Detection of additional injuries based on tertiary evaluation was a secondary outcome. Associations between outcomes and categorical patient and encounter characteristics were assessed using χ2 tests. RESULTS Overall, 592 trauma encounters were reviewed (296 preimplementation and 296 postimplementation). Trauma tertiary survey documentation was significantly higher after implementation of the interventions (30.1% preimplementation vs. 85.1% postimplementation, p < 0.001). Preimplementation documentation of the TTS was less likely earlier in the academic year (14.3% first academic quarter vs. 46.5% last academic quarter, p < 0.001), but this temporal pattern was no longer evident postimplementation (88.5% first academic quarter vs. 77.9% last academic quarter, p = 0.126). Patients were more likely to have a missed traumatic injury diagnosed on TTS postimplementation (1.7% in preimplementation vs. 5.7% postimplementation, p = 0.009). CONCLUSION Documentation of the TTS and missed injury detection rates were significantly increased following implementation of a bundle of QI interventions. The association between time of year and documentation of the TTS was also attenuated, likely through reduction of the resident learning curve. Targeted efforts to improve TTS performance may improve outcomes for trauma patients at teaching hospitals. LEVEL OF EVIDENCE Care management, Level IV.

Button battery ingestion can cause serious injury or death in young children who cannot communicate symptoms. An 18-month-old male presented after his mother noted drooling, nonbilious emesis and a metallic smell to his breath. He underwent rigid esophagoscopy and a 3-V 20-mm button battery was removed. Subsequent bronchoscopy after a 1-week interval revealed progression to a large broncho-esophageal fistula on the posterior wall of the right mainstem bronchus past the carina. A fenestrated nasogastric tube for local control of secretion and a feeding jejunostomy was placed. Six weeks later, the patient underwent a right thoracotomy for division and repair of the fistula and intercostal muscle flap interposition. Utilizing a well-placed fenestrated nasogastric tube to manage secretions can help reduce fistula size and improve conservative management results. When surgical repair is required, an intercostal muscle flap can reinforce fistula closure while simultaneously buttressing the bronchus and esophagus.

Fetal airway obstruction in one twin of a diamniotic pregnancy presents unique challenges especially when a single placenta is shared between the fetuses. Very few cases of Ex-Utero-Intrapartum-Treatment (EXIT) in twin pregnancies have been reported, and only in dichorionic pregnancies. We report a single methodology for EXIT-to-airway procedures in monochorionic and dichorionic twins. We review two EXIT-to-airway in twin pregnancies with a giant cervical mass in one fetus. Two cases of EXIT-to-airway in twin pregnancies were performed in 2018-2019 at a regional fetal treatment center. A multi-disciplinary evaluation was completed, and treatment options developed to accommodate parental goals balancing the risks for both fetuses. Case 1 involved a dichorionic-diamniotic pregnancy with a large cervical lymphatic malformation with labor at 36 weeks. Case 2 involved a giant cervical teratoma in a monochorionic-diamniotic twin pregnancy with preterm labor at 29 weeks. In each case, the polyhydramnios caused the affected twin's amniotic sac to be the presenting sac for the surgical approach. Continuous monitoring of both fetuses was obtained via pulse-oximetry and intermittent transthoracic echocardiography and continuous transuterine echocardiography. Bronchoscopy and intubation were completed after 10 and 22 minutes of uteroplacental bypass respectively. In each case, the affected fetus was transferred to adjacent operating room for complete mass resection. The unaffected twins were delivered as second without intubation and resuscitated without any perinatal distress. Twins and mothers recovered without complications. EXIT-to-airway appears to be a reasonable option for the safe transition of complicated twins even in the setting of significant prematurity in a monochorionic pregnancy. Continuous monitoring permits safe algorithm-based airway management of the affected twin first and subsequent delivery of the unaffected twin. Thoughtful preparation by an experienced multidisciplinary team permits an EXIT-to-airway approach for twin pregnancies in an emergency setting.

Abstract Introduction: Neural tube defects (NTDs), are severe malformations of the developing central nervous system. After having a pregnancy affected by NTD, the recurrence risk increases 20x. The predisposition to having children affected by NTDs has not been explained and may relate to surveillance by the maternal immune system as maternal immune cells such as NK and T cells have been shown to traffic in the fetal circulation. We hypothesized that maternal NK and T cells are necessary for selective fetal absorption after the development of an NTD. Methods: We challenged this hypothesis in an outbred murine model of valproic acid (VA)-induced fetal neural tube teratogenesis (NTT) performed of pregnant CD1 dams at E8. To evaluate protection from NTTs, maternal NK cells were selectively eliminated (mAb PK136) on E7. Maternal CD4+ and CD8+ T cells were selectively eliminated (GK1.5 and 2.43) on E6. All litters were harvested on E14 and prevalence of NTT compared between each treatment group and untreated controls. Results: The frequency of litters with VA-induced NTT was significantly higher when maternal NK cells or maternal T cells were selectively depleted compared to controls (100% and 88.2% vs 80.0%, p<0.05). Only 10% of NK depleted dams had greater than 3 late absorptions per litter and T cell depleted dams had 0% compared to controls that had 30%, p<0.05. This data shows that both cell types play a critical role in the late absorption process. Litter sizes were similar between the groups. Conclusion: These findings demonstrate that selective fetal absorption for NTT is dependent on intact maternal immune cell function. Further studies are needed to define the roles that maternal NK and T cells have in the absorption process during abnormal development.