INTRODUCTION:Radiographic imaging is used to identify and characterize ovarian tumors. Radiographic features suspicious for malignancy should prompt further evaluation including a multidisciplinary discussion for consideration of ovarian sparing surgery versus oophorectomy. This study assessed the diagnostic capability of radiographic features alone and in combination to risk stratify between benign and malignant ovarian tumors in pediatric and adolescent patients. METHODS:We performed a planned secondary analysis of a prospective multi-institutional, interventional cohort study using a consensus-based preoperative risk stratification algorithm to decrease unnecessary oophorectomies in patients 6-21 years at 11 children's hospitals between August 2018 and January 2021. All patients underwent radiographic imaging. Radiographic features were analyzed individually and in combination. A priori clinical consensus prioritized maximizing negative predictive value to minimize risk of missed malignancy. RESULTS:Imaging was performed in 519 patients with ovarian tumors. The presence of a complex mass, solid component, or tumor size >8 cm was reported in 26 of 27 malignant tumors. All 26 malignant tumors were identified when one of these radiographic features was present, conferring a negative predictive value of 100% with 65.1% accuracy. The combination of radiographic evidence of metastatic disease and increased β-human gonadotropin, cancer antigen 125, or inhibin A identified all 13 malignancies in patients who received these tests with an accuracy of 87.6%. CONCLUSIONS:The presence or absence of radiographic features including a complex mass, solid component, or size >8 cm can be used to preoperatively risk stratify masses to direct management with ovarian sparing surgery or further evaluation including serum tumor markers and multidisciplinary discussion.

INTRODUCTION:Pediatric and adolescent patients with ovarian masses undergo radiographic and serologic workup to determine malignancy risk before surgery. Commonly examined tumor markers for detecting malignancy among these patients are α-fetoprotein, β-human chorionic gonadotropin (β-HCG), cancer antigen-125, inhibin A, and lactate dehydrogenase. This study investigated the diagnostic performance of these tumor markers, individually and in combination, to assess malignancy risk in pediatric and adolescent patients with ovarian masses. METHODS:This was a planned secondary analysis of a multi-institutional interventional study investigating a consensus-based, preoperative risk stratification algorithm in patients aged 6-21 y, undergoing surgery for an ovarian mass between August 2018 and February 2021 at 11 children's hospitals. All included patients underwent preoperative assessment with ≥ 1 tumor marker(s). Individual and all combinations of tumor marker performance were analyzed. A priori clinical consensus was that the optimal combination of tumor markers should minimize the misclassification of patients with malignancy (maximize negative predictive value). RESULTS:Tumor markers were assessed in 309 out of the 519 patients, and malignancy was found in 25 patients. β-HCG was present among all the best-performing combinations. The most accurate combination was β-HCG/inhibin A/lactate dehydrogenase (accuracy = 0.951). Three combinations did not misclassify any malignant lesions as likely benign (100% negative predictive value), and the most accurate of these three combinations was α-fetoprotein/β-HCG/cancer antigen-125/inhibin A (accuracy = 0.862). CONCLUSIONS:Tumor markers can support preoperative risk stratification for malignancy to determine which patients may benefit from ovary-sparing surgery and minimize those undergoing an unindicated oophorectomy or oophorectomy for benign disease.

STUDY OBJECTIVE:To assess the diagnostic performance of MRI to predict ovarian malignancy alone and compared with other diagnostic studies.METHODS:A retrospective analysis was conducted of patients aged 2-21 years who underwent ovarian mass resection between 2009 and 2021 at 11 pediatric hospitals. Sociodemographic information, clinical and imaging findings, tumor markers, and operative and pathology details were collected. Diagnostic performance for detecting malignancy was assessed by calculating sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) for MRI with other diagnostic modalities.RESULTS:One thousand and fifty-three patients, with a median age of 14.6 years, underwent resection of an ovarian mass; 10% (110/1053) had malignant disease on pathology, and 13% (136/1053) underwent preoperative MRI. MRI sensitivity, specificity, PPV, and NPV were 60%, 94%, 60%, and 94%. Ultrasound sensitivity, specificity, PPV, and NPV were 31%, 99%, 73%, and 95%. Tumor marker sensitivity, specificity, PPV, and NPV were 90%, 46%, 22%, and 96%. MRI and ultrasound concordance was 88%, with sensitivity, specificity, PPV, and NPV of 33%, 99%, 75%, and 94%. MRI sensitivity in ultrasound-discordant cases was 100%. MRI and tumor marker concordance was 88% with sensitivity, specificity, PPV, and NPV of 100%, 86%, 64%, and 100%. MRI specificity in tumor marker-discordant cases was 100%.CONCLUSION:Diagnostic modalities used to assess ovarian neoplasms in pediatric patients typically agree. In cases of disagreement, MRI is more sensitive for malignancy than ultrasound and more specific than tumor markers. Selective use of MRI with preoperative ultrasound and tumor markers may be beneficial when the risk of malignancy is uncertain.CONCISE ABSTRACT:This retrospective review of 1053 patients aged 2-21 years who underwent ovarian mass resection between 2009 and 2021 at 11 pediatric hospitals found that ultrasound, tumor markers, and MRI tend to agree on benign vs malignant, but in cases of disagreement, MRI is more sensitive for malignancy than ultrasound.

Hyperinsulinism due to focal or diffuse pancreatic lesions causing recurrent episodes of hypoglycemia is rare in mid-childhood. There is no consensus on the gold-standard imaging method to diagnose focal insulin-producing lesions beyond infancy. A 14-year-old boy with a complex medical history and refractory epilepsy, presented with blood glucose (BG) of 52 mg/dL (2.9 mmol/L) (normal reference range: 70-100 mg/dL [3.9-5.6 mmol/L]) and increased seizure frequency. He failed a fast within 4 hours, with BG of 48 mg/dL (2.7 mmol/L) and insulin level of 4.6 µIU/mL (24.6 pmol/L) (diagnostic at the time of hypoglycemia >1.25 μU/mL [8.7 pmol/L]). Conventional imaging studies showed no pancreatic lesion. Fluorine-18-L-dihydroxyphenylalanine positron emission tomography/magnetic resonance imaging (18F-DOPA-PET/MRI) scan premedicated with carbidopa demonstrated intense focal 18F-DOPA uptake in the distal pancreatic tail. He underwent distal pancreatectomy. Histopathology showed focal pancreatic islet cell hyperplasia, with more than 90% of the neuroendocrine islet cells being positive for chromogranin and synaptophysin, with no loss of p57 staining. Genetic studies were negative for mutations in ABCC8, KCNJ11, GCK, or GLUD1 genes, multiple endocrine neoplasia (MEN) type 1, and Beckwith-Wiedemann syndrome. BG normalized after surgery. Seizure frequency improved. This case highlights the utility of 18F-DOPA PET/MRI imaging in diagnosing focal hyperinsulinism beyond infancy.

Case Presentation A preterm neonate (34 weeks of gestation) weighing 2.5 kg with a history of fetal supraventricular tachycardia (SVT, successfully treated with maternal flecainide, though fetal development of ascites was noted) was transferred to our institution at 10 days of life. Pretransfer electrocardiogram revealed preexcitation, with intermittent orthodromic narrow complex tachycardia at a rate of 220 bpm (Figure 1). Before transfer, antiarrhythmic treatment strategies included propranolol, flecainide, amiodarone, and electrical cardioversion. On the day of transfer, the patient developed incessant SVT, with onset of emesis, hematochezia, abdominal distension, and pneumatosis on abdominal x-ray concerning for necrotizing enterocolitis (NEC).Figure 1.: A: Baseline echocardiogram showing sinus bradycardia at a rate of 90 bpm, left axis deviation, low voltage, and preexcitation. B: Orthodromic reentrant tachycardia at a rate of 220 bpm with delayed atrioventricular conduction secondary to recent Flecainide loading. aVF, augmented vector foot; aVL, augmented vector left; aVR, augmented vector right.Initial assessment by clinical exam, laboratory markers of oxygen delivery, and echocardiogram showed evidence of preserved cardiac output. An infusion of procainamide was attempted, resulting in modestly improved rate control without conversion to sinus rhythm. Mild lactatemia soon developed. Given concerns that NEC was a manifestation of compromised oxygen delivery and that the SVT duration was approaching 30 hours, we “electively” cannulated on to venoarterial (VA) extracorporeal membrane oxygenation (ECMO) to maintain end-organ perfusion while facilitating further antiarrhythmic management. Cannulation was uneventful using 8 and 10 French arterial and venous cannulas and an Affinity CP centrifugal pump (Medtronic, Minneapolis, MN). ECMO flow was established at 0.4 L/min. Systemic anticoagulation with bivalirudin was started per institutional guideline1 at a dose of 0.1 mg/kg/h, followed by amiodarone loading and successful electrical cardioversion over the following 12 hours. Despite ECMO support, the patient deteriorated with distributive shock requiring new vasopressor support and frequent episodes of nonsustained SVT, lasting for minutes. Leukocytosis and thrombocytopenia developed, and his abdominal exam became increasingly concerning for intestinal perforation. Without the need for systemic anticoagulation, surgical intervention would typically be pursued, but it was considered high risk due to potentially life-threatening hemorrhage. The next several hours showed rising lactatemia and worsening frequency of breakthrough SVT. The heightened concern for perforation and bowel necrosis shifted the risk-benefit of surgical intervention toward performing a bedside laparotomy while on VA-ECMO on the second day of admission. The ECMO anticoagulation requirements along with the need for abdominal surgery were challenging. Before the procedure, platelets were administered; bivalirudin infusion was stopped; resuscitative blood products, factor VIIa, and aminocaproic acid (ACA) were made available at bedside. One hour after bivalirudin discontinuation, the laparotomy started. Severe hemorrhaging was quickly noted, requiring 125 ml/kg of packed red blood cells, platelets, and fresh frozen plasma in addition to two units of cryoprecipitate for resuscitation. Medication management included two doses of 0.08 mg/kg of factor VIIa and an ACA infusion of 20 mg/kg/h for 1 hour before hemostasis was achieved. This was further complicated by frequent adenosine-responsive breakthrough episodes of SVT, which worsened in burden during the procedure. At the time of operation, enteric contents were evacuated, and a right colon perforation secondary to full thickness necrosis was identified. In addition, there was necrosis of the entire colon extending to just proximal to the peritoneal reflection. A subtotal colectomy was performed with the bowel left in discontinuity and abdomen left open with plans for a second-look surgery. We elected to hold bivalirudin and continue ACA infusion due to high risk of hemorrhage with open abdomen, a strategy that proved effective. The postsurgical course was complicated by postsurgical inflammatory response. Combined with the massive product resuscitation, this led to progressive third spacing, interfering with ECMO flows. Marked vasodilation with frequent recurrent nonsustained SVT persisted. As such, catecholaminergic medications that may worsen the arrhythmia were avoided. Instead, vasopressin was used at the cost of potentially limiting splanchnic circulation and further intestinal injury. The ACA infusion continued for 24 hours postoperatively without additional anticoagulation. There was gradual improvement in his hemodynamics without further hemorrhage or substantial worsening of ECMO clot burden for the next 72 hours. A second-look laparotomy was performed on day 5 of admission to assess intestinal viability, and an additional 36 cm of ilium was resected. An ACA infusion was restarted at procedure onset, and no significant hemorrhage occurred intra- or postoperatively. Four hours after the procedure, bivalirudin was restarted at 0.05 mg/kg/h with more conservative anticoagulation/hematologic goals (partial thromboplastin time of 40–50 sec, fibrinogen of >150 mg/dL, platelet of >30 K/cumm, and hemoglobin >10 g/dL). The ACA infusion was continued, overlapping with bivalirudin for additional 24 hours. Three days later, a third abdominal exploration was required to assess intestinal viability without the need for further intestinal resection. The same hemostatic strategy was repeated and again proved to be effective. Removal of necrotic bowel, relief of abdominal pressure, and antibiotic therapy allowed for resolution of the distributive shock and improved hemodynamics. SVT burden decreased as well but did not resolve, with nonsustained episodes occurring one to five times daily. The hemodynamic stability allowed the patient to undergo ablation 12 days after initial presentation, where successful cryoablation of a right posteroseptal accessory pathway was achieved while supported on VA-ECMO. Two day postprocedure, patient was successfully decannulated from ECMO. Gastrointestinal rehabilitation continued slowly. There were no further episodes of SVT. Three more abdominal operations were performed for washout, to establish ileocolic anastomosis and to close the abdominal wall 120 days after presentation. At the time of this report, patient was discharged home on total parenteral nutrition with no other reliance on technology. Discussion This is an example of a critically ill child in whom “elective” ECMO use can prevent deterioration of oxygen delivery and irreversible organ injury. There is a time window during cardiopulmonary deterioration, when the risks associated with impaired oxygen delivery outweigh those related to ECMO. Despite advances in ECMO technology, objective criteria for timing elective ECMO utilization are lacking, and determining the optimal time before irreversible organ deterioration or cardiopulmonary decompensation remains challenging. Here, “elective” ECMO was decided without the full knowledge of the degree of NEC and systemic involvement, but with concern for uncontrolled arrhythmia leading to compromised end-organ function and potential cardiovascular collapse. Nonetheless, the patient developed distributive shock compounded by later intraoperative hemorrhagic shock. To our knowledge, the management of surgical NEC on ECMO has not been previously reported. Reports of abdominal sepsis requiring surgery2 and gastric rupture3 in pediatric ECMO have been described and, as seen in this case, resulted in life-threatening hemorrhage. Abdominal surgery here was necessary to remove necrotic bowel that would lead to overwhelming sepsis with bacterial translocation, multiorgan dysfunction, and death even with VA-ECMO support. Without relief of abdominal pressure, abdominal compartment syndrome may have progressed and impeded venous return to the ECMO circuit, compromising ECMO support.4 Hemostatic management under these circumstances was challenging. Institutionally, we have recently transitioned to bivalirudin as our primary anticoagulant for patients outside of the neonatal intensive care unit with similar outcomes to our previous heparin experience.5 During the intestinal resection, massive bleeding occurred requiring 1-to-1-to-1 blood product resuscitation of four times the approximated blood volume of this neonate in addition to ACA and factor VIIa. ACA binds competitively to plasminogen blocking its binding to fibrin and subsequent conversion to plasmin, resulting in inhibition of fibrinolysis.6 ACA has previously been reported to alleviate surgical bleeding on ECMO with heparin anticoagulation.6–9 These reports describe hemostatic strategies for congenital diaphragmatic hernia repair on ECMO involving ACA loading preoperatively with continuous infusion for at least 24 hour postoperatively and at times up to ECMO decannulation. We utilized a similar approach with bivalirudin anticoagulation because of the inherent risk of thrombosis in a neonate limited by low-flow ECMO of 0.4 L/min. To our knowledge, this strategy has not previously been described. Bivalirudin directly inhibits both circulating and clot-bound thrombin, preventing thrombin-mediated cleavage of fibrinogen to fibrin.5 We chose to overlap ACA and bivalirudin to manipulate the balance of unwanted ECMO-related fibrin deposition and necessary fibrin formation in sites of surgical bleeding. This required close monitoring for bleeding and conservative anticoagulation goals (Figure 2), and after the initial operative hemorrhage, ACA was used without bivalirudin. It is important to note that this patient did not suffer any significant thromboembolic events. The combination of continuous bivalirudin infusion with ACA may be a safe approach to prevent bleeding and clotting during and immediately after procedural interventions while on ECMO.Figure 2.: Recommended approach for a high-risk-for-bleeding/bleeding patient on extracorporeal membrane oxygenation requiring invasive procedure. Prior to procedure initiation, obtain bedside resuscitative blood products and medications including factor VIIa and ACA (aminocaporic acid). Discontinue bivalirudin 1 hour prior to procedure start time. At procedure start time, initiate both platelet and ACA infusions (20–30 mg/kg/hr) with consideration for an ACA loading dose (100 mg/kg). If hemorrhaging is encountered during or after the procedure, consider an ACA loading dose (if not previously given) and/or factor VIIa replacement. Moving forward, continue blood product resuscitation as needed with plan for continued ACA infusion post-procedurally for approximately 24 hours (or at least 6 hours if hemostasis achieved). If clinical evidence of thrombosis appears during this time period, consider overlapping the ACA infusion with a bivalirudin infusion. We recommend a conservative bivlairudin starting dose of 0.05 mg/kg/hr with conservative laboratory monitoring (partial thromboplastin time, 40–50 sec; Fibrinogen, >200 mg/dL; platelets, >50 K/cumm; hemoglobin, >10 g/dL; thromboelastography (TEG) reaction time, 8–10 min) with reliance on TEG to guide product resuscitation. ACA, aminocaporic acid.Management of the refractory SVT was challenging. Catheter ablation on ECMO was a reasonable approach even in such a complex patient. Safety profiles for ablation have been described in refractory cases for infants of this weight range and on ECMO support as early as 20 years ago.10 Here, ablation was performed on the patient having an open abdomen and recovering from distributive shock and intestinal resection. A recent multi-institutional review of the management of pediatric tachyarrhythmias on ECMO described the risk of ablation as no more than ablation without ECMO.11 In summary, early recognition, communication, and multidisciplinary shared understanding of the disease process were crucial in achieving a successful outcome. Critical care and cardiology subspecialty management of the underlying arrhythmia and cardiopulmonary dynamics, combined with early use of ECMO, created a safer environment to treat the arrhythmia (medically and transcatheter ablation) and to allow pediatric surgery to adequately approach surgical NEC. A thoughtful hemostatic strategy with aid from clinical pharmacists allowed for an outcome that may not have been possible under other circumstances.

Study Objective: Describe the current practice patterns and diagnostic accuracy of frozen section (FS) pathology for children and adolescents with ovarian massesDesign: Prospective cohort study from 2018 to 2021Setting: Eleven children's hospitalsParticipants: Females age 6-21 years undergoing surgical management of an ovarian massInterventions: Obtaining intraoperative FS pathologyMain Outcome Measure: Diagnostic accuracy of FS pathologyResults: Of 691 patients who underwent surgical management of an ovarian mass, FS was performed in 27 (3.9%), of which 9 (33.3%) had a final malignant pathology. Among FS patients, 12 of 27 (4 4.4%) underwent ovary-sparing surgery, and 15 of 27 (55.5%) underwent oophorectomy with or without other procedures. FS results were disparate from final pathology in 7 of 27 (25.9%) cases. FS had a sensi-tivity of 44.4% and specificity of 94.4% for identifying malignancy, with a c-statistic of 0.69. Malignant diagnoses missed on FS included serous borderline tumor ( n = 1), mucinous borderline tumor ( n = 2), mucinous carcinoma ( n = 1), and immature teratoma ( n = 1). FS did not guide intervention in 10 of 27 (37.0%) patients: 9 with benign FS underwent oophorectomy, and 1 with malignant FS did not undergo oophorectomy. Of the 9 patients who underwent oophorectomy with benign FS, 5 (55.6%) had benign and 4 (4 4.4%) had malignant final pathology.Conclusions: FSs are infrequently utilized for pediatric and adolescent ovarian masses and could be inaccurate for predicting malignancy and guiding operative decision-making. We recommend continued assessment and refinement of guidance before any standardization of use of FS to assist with intraoperative decision-making for surgical resection and staging in children and adolescents with ovarian masses.

Importance Although most ovarian masses in children and adolescents are benign, many are managed with oophorectomy, which may be unnecessary and can have lifelong negative effects on health. Objective To evaluate the ability of a consensus-based preoperative risk stratification algorithm to discriminate between benign and malignant ovarian pathology and decrease unnecessary oophorectomies. Design, Setting, and Participants Pre/post interventional study of a risk stratification algorithm in patients aged 6 to 21 years undergoing surgery for an ovarian mass in an inpatient setting in 11 children’s hospitals in the United States between August 2018 and January 2021, with 1-year follow-up. Intervention Implementation of a consensus-based, preoperative risk stratification algorithm with 6 months of preintervention assessment, 6 months of intervention adoption, and 18 months of intervention. The intervention adoption cohort was excluded from statistical comparisons. Main Outcomes and Measures Unnecessary oophorectomies, defined as oophorectomy for a benign ovarian neoplasm based on final pathology or mass resolution. Results A total of 519 patients with a median age of 15.1 (IQR, 13.0-16.8) years were included in 3 phases: 96 in the preintervention phase (median age, 15.4 [IQR, 13.4-17.2] years; 11.5% non-Hispanic Black; 68.8% non-Hispanic White); 105 in the adoption phase; and 318 in the intervention phase (median age, 15.0 [IQR, 12.9-16.6)] years; 13.8% non-Hispanic Black; 53.5% non-Hispanic White). Benign disease was present in 93 (96.9%) in the preintervention cohort and 298 (93.7%) in the intervention cohort. The percentage of unnecessary oophorectomies decreased from 16.1% (15/93) preintervention to 8.4% (25/298) during the intervention (absolute reduction, 7.7% [95% CI, 0.4%-15.9%]; P = .03). Algorithm test performance for identifying benign lesions in the intervention cohort resulted in a sensitivity of 91.6% (95% CI, 88.5%-94.8%), a specificity of 90.0% (95% CI, 76.9%-100%), a positive predictive value of 99.3% (95% CI, 98.3%-100%), and a negative predictive value of 41.9% (95% CI, 27.1%-56.6%). The proportion of misclassification in the intervention phase (malignant disease treated with ovary-sparing surgery) was 0.7%. Algorithm adherence during the intervention phase was 95.0%, with fidelity of 81.8%. Conclusions and Relevance Unnecessary oophorectomies decreased with use of a preoperative risk stratification algorithm to identify lesions with a high likelihood of benign pathology that are appropriate for ovary-sparing surgery. Adoption of this algorithm might prevent unnecessary oophorectomy during adolescence and its lifelong consequences. Further studies are needed to determine barriers to algorithm adherence.

STUDY OBJECTIVE:To assess the preoperative imaging impression and surgeon diagnostic accuracy for pediatric ovarian mature cystic teratomas (MCTs) DESIGN: Retrospective review SETTING: Eleven pediatric hospitals PARTICIPANTS: Patients ages 2 to 21 who underwent surgical management of an ovarian neoplasm or adnexal torsion with an associated ovarian lesion INTERVENTION: None MAIN OUTCOME MEASURES: Preoperative imaging impression, surgeon diagnosis, tumor markers, and pathology RESULTS: Our cohort included 946 females. Final pathology identified 422 (45%) MCTs, 405 (43%) other benign pathologies, and 119 (12%) malignancies. Preoperative imaging impression for MCTs had a 70% sensitivity, 92% specificity, 88% positive predictive value (PPV), and 79% negative predictive value (NPV). For the preoperative surgeon diagnosis, sensitivity was 59%, specificity 96%, PPV 92%, and NPV 74%. Some measures of diagnostic accuracy were affected by the presence of torsion, size of the lesion on imaging, imaging modality, and surgeon specialty. Of the 352 masses preoperatively thought to be MCTs, 14 were malignancies (4%). Eleven patients with inaccurately diagnosed malignancies had tumor markers evaluated and 82% had at least 1 elevated tumor marker, compared with 49% of those with MCTs.CONCLUSIONS:Diagnostic accuracy for the preoperative imaging impression and surgeon diagnosis is lower than expected for pediatric ovarian MCTs. For all ovarian neoplasms, preoperative risk assessment including a panel of tumor markers and a multidisciplinary review is recommended. This process could minimize the risk of misdiagnosis and improve operative planning to maximize the use of ovarian-sparing surgery for benign lesions and allow for appropriate resection and staging for lesions suspected to be malignant.

Introduction Gastroschisis feeding practices vary. Standardized neonatal feeding protocols have been demonstrated to improve nutritional outcomes. We report outcomes of infants with gastroschisis that were fed with and without a protocol. Materials and Methods A retrospective study of neonates with uncomplicated gastroschisis at 11 children's hospitals from 2013 to 2016 was performed. Outcomes of infants fed via institutional-specific protocols were compared with those fed without a protocol. Subgroup analyses of protocol use with immediate versus delayed closure and with sutured versus sutureless closure were conducted. Results Among 315 neonates, protocol-based feeding was utilized in 204 (65%) while no feeding protocol was used in 111 (35%). There were less surgical site infections (SSI) in those fed with a protocol (7 vs. 16%, p =0.019). There were no differences in TPN duration, time to initial oral intake, time to goal feeds, ventilator use, peripherally inserted central catheter line deep venous thromboses, or length of stay. Of those fed via protocol, less SSIs occurred in those who underwent sutured closure (9 vs. 19%, p =0.026). Further analyses based on closure timing or closure method did not demonstrate any significant differences. Conclusion Across this multi-institutional cohort of infants with uncomplicated gastroschisis, there were more SSIs in those fed without an institutional-based feeding protocol but no differences in other outcomes.

OBJECTIVE:To provide generalizable estimates for expected outcomes of simple gastroschisis (SG) and complex gastroschisis (CG) patients from a large multi-institutional cohort for use during counseling.STUDY DESIGN:A retrospective study of 394 neonates with gastroschisis at 11 children's hospitals from January 2013 to March 2017 was performed. Analysis by Fisher's exact tests and Wilcoxon rank sum tests were performed. Outcomes of complex and simple gastroschisis are reported.RESULT:There were 315 (80%) SG and 79 (20%) CG. CG had increased time from birth to closure (6 vs 4.4 days), closure to goal feeds (69 vs 23 days), ventilator use (90% vs 73%), SSIs (31% vs 11%), NEC (14% vs 6%), PN use (71 vs 24 days), LOS (104.5 vs 33 days), and mortality (11% vs 0%).CONCLUSION:This study provides generalizable estimates for expected outcomes of patients with both SG and CG that can be utilized during counseling. CG has significantly worse in-hospital outcomes.

Pediatric tumors in the apex of the thoracic cavity are often diagnosed late due to the absence of symptoms. These tumors can be quite large at presentation with involvement of the chest wall, sympathetic chain, spine, and aortic arch. The tumors can also extend into the thoracic inlet and encircle the brachial plexus. Depending on the diagnosis, treatment may involve chemotherapy with subsequent surgery or require primary resection. Optimal exposure to resect large apical tumors with thoracic inlet extension is a surgical challenge. To date, several surgical techniques have been described to resect these tumors – including both anterior and posterior thoracic approaches. Each of these techniques can be limited by inadequate exposure of the mass. We describe an alternative approach to surgical resection of these masses that employs an extended sternotomy with a lateral neck incision. This report details two successful resections of large left apical masses with thoracic inlet involvement in children using this technique (Level of evidence 4).

Background Management of ovarian torsion has evolved toward ovarian preservation regardless of ovarian appearance during surgery. However, patients with torsion and an ovarian neoplasm undergo a disproportionately high rate of oophorectomy. Our objectives were to identify factors associated with ovarian torsion among females with an ovarian mass and to determine if torsion is associated with malignancy. Methods A retrospective review of females aged 2-21 y who underwent an operation for an ovarian cyst or neoplasm between 2010 and 2016 at 10 children's hospitals was performed. Multivariate logistic regression was used to assess factors associated with torsion. Imaging data were assessed for sensitivity, specificity, and predictive value in identifying ovarian torsion. Results Of 814 girls with an ovarian neoplasm, 180 (22%) had torsion. In risk-adjusted analyses, patients with a younger age, mass size >5 cm, abdominal pain, and vomiting had an increased likelihood of torsion (P < 0.01 for all). Patients with a mass >5 cm had two times the odds of torsion (odds ratio: 2.1; confidence interval: 1.2, 3.6). Imaging was not reliable at identifying torsion (sensitivity 34%, positive predictive value 49%) or excluding torsion (specificity 72%, negative predictive value 87%). The rates of malignancy were lower in those with an ovarian mass and torsion than those without torsion (10% versus 17%, P = 0.01). Among the 180 girls with torsion and a mass, 48% underwent oophorectomy of which 14% (n = 12) had a malignancy. Conclusions In females with an ovarian neoplasm, torsion is not associated with an increased risk of malignancy and ovarian preservation should be considered.

PurposeTo report outcomes of sutured and sutureless closure for gastroschisis across a large multi-institutional cohort.MethodsA retrospective study of infants with uncomplicated gastroschisis at 11 children's from 2014 to 2016 was performed. Outcomes of sutured and sutureless abdominal wall closure were compared.ResultsAmong 315 neonates with uncomplicated gastroschisis, sutured closure was performed in 248 (79%); 212 undergoing sutured closure after silo and 36 undergoing primary sutured closure. Sutureless closure was performed in 67 (21%); 37 primary sutureless closure, 30 sutureless closure after silo placement. There was no significant difference in gestational age, gender, birth weight, total days on TPN, and time from closure to initial oral intake or goal feeds. Sutureless closure patients had less general anesthetics, ventilator use/time, time from birth to final closure, antibiotic use after closure, and surgical site/deep space infections. Subgroup analysis demonstrated primary sutureless closure had less ventilator use and anesthetics than primary sutured closure. Sutureless closure after silo led to less ventilator use/time, anesthetics, and antibiotics compared to those with sutured closure after silo.ConclusionSutureless abdominal wall closure of neonates with gastroschisis was associated with less general anesthetics, antibiotic use, surgical site/deep space infections, and decreased ventilator time. These findings support further prospective study by our group.Level of EvidenceLevel III.

The differential diagnosis for pediatric patients presenting with abdominal pain can be vast. Our purpose was to review the presentation and imaging associated with wandering spleen, a rare condition that usually presents with abdominal pain and other nonspecific symptoms that can make the clinical diagnosis challenging.

Introduction: Endobronchial granular cell tumors are uncommon in the pediatric population. Case report: A 9-year-old female presented with respiratory failure due to an endobronchial tumor. After debulking and diagnosis, she underwent thoracotomy with right upper lobe resection and bronchoplasty. Pathology demonstrated an endobronchial S-100 negative granular cell tumor, which to our knowledge, is the first such report in the literature. Conclusion: Endobronchial granular cell tumors may cause obstructive respiratory failure, are amenable to surgery, and may be S-100 negative.

Purpose: The purpose of this study was to determine the diagnostic accuracy of tumor markers for malignancy in girls with ovarian neoplasms. Methods: A retrospective review of girls 2-21 years who presented for surgical management of an ovarian neoplasm across 10 children's hospitals between 2010 and 2016 was performed. Patients who had at least one concerning feature on imaging and had tumor marker testing were included in the study. Sensitivity, specificity, and negative and positive predictive values (PPV) of tumor markers were calculated. Results: Our cohort included 401 patients; 22.4% had a malignancy. Testing for tumor markers was inconsistent. APP had high specificity (98%) and low sensitivity (42%) with a PPV of 86%. The sensitivity, specificity, and PPV of beta-hCG was 44%, 76%, and 32%, respectively. LDH had high sensitivity (95%) and Inhibin A and Inhibin B had high specificity (97% and 92%, respectively). Conclusions: Tumor marker testing is helpful in preoperative risk stratification of ovarian neoplasms for malignancy. Given the variety of potential tumor types, no single marker provides enough reliability, and therefore a panel of tumor marker testing is recommended if there is concern for malignancy. Prospective studies may help further elucidate the predictive value of tumor markers in a pediatric ovarian neoplasm population. Type of Study: Retrospective Cohort Review. (C) 2019 Elsevier Inc. All rights reserved.

BACKGROUND:Available evidence supports ovary-sparing surgery for benign ovarian neoplasms; however, preoperative risk stratification of pediatric ovarian masses can be difficult. Our objective of this study was to characterize the surgical management of pediatric ovarian neoplasms across 10 children's hospitals and to identify factors that could potentially aid in the preoperative risk stratification of these lesions.METHODS:A retrospective review of girls and women aged 2 to 21 years who underwent surgery for an ovarian neoplasm between 2010 and 2016 at 10 children's hospitals was performed. Multivariable logistic regression was used to examine the relationships between the preoperative cohort characteristics, procedure performed, and risk of malignancy.RESULTS:Among 819 girls and women undergoing surgery for an ovarian neoplasm, malignant lesions were identified in 11%. The overall oophorectomy rate for benign disease was 33% (range: 15%-49%) across institutions. Oophorectomy for benign lesions was independently associated with provider specialty (P = .002: adult gynecologist, 45%; pediatric surgeon, 32%; pediatric gynecologist, 18%), premenarchal status (P = .02), preoperative suspicion for malignancy (P < .0001), larger lesion size (P < .0001), and presence of solid components (P < .0001). Preoperative findings independently associated with malignancy included increasing size (P < .0001), solid components (P = .003), and age (P < .0001).CONCLUSIONS:The rate of oophorectomy for benign ovarian disease remains high within the pediatric population. Identification of factors associated with the choice of procedure and the risk of malignancy may allow for improved preoperative risk stratification and fewer unnecessary oophorectomies. These results have been used to develop and validate a multidisciplinary preoperative risk stratification algorithm that is currently being studied prospectively across 10 institutions.

To evaluate the efficacy of nontransplant surgery for pediatric cholestasis, 58 clinically diagnosed children, including 20 with Alagille syndrome (ALGS), 16 with familial intrahepatic cholestasis‐1 (FIC1), 18 with bile salt export pump (BSEP) disease, and 4 others with low γ‐glutamyl transpeptidase disease (levels <100 U/L), were identified across 14 Childhood Liver Disease Research Network (ChiLDReN) centers. Data were collected retrospectively from individuals who collectively had 39 partial external biliary diversions (PEBDs), 11 ileal exclusions (IEs), and seven gallbladder‐to‐colon (GBC) diversions. Serum total bilirubin decreased after PEBD in FIC1 (8.1 ± 4.0 vs. 2.9 ± 4.1 mg/dL, preoperatively vs. 12‐24 months postoperatively, respectively; P = 0.02), but not in ALGS or BSEP. Total serum cholesterol decreased after PEBD in ALGS patients (695 ± 465 vs. 457 ± 319 mg/dL, preoperatively vs. 12‐24 months postoperatively, respectively; P = 0.0001). Alanine aminotransferase levels increased in ALGS after PEBD (182 ± 70 vs. 260 ± 73 IU/L, preoperatively vs. 24 months; P = 0.03), but not in FIC1 or BSEP. ALGS, FIC1, and BSEP patients experienced less severely scored pruritus after PEBD (ALGS, 100% vs. 9% severe; FIC1, 64% vs. 10%; BSEP, 50% vs. 20%, preoperatively vs. >24 months postoperatively, respectively; P < 0.001). ALGS patients experienced a trend toward greater freedom from xanthomata after PEBD. There was a trend toward decreased pruritus in FIC1 after IE and GBC. Vitamin K supplementation increased in ALGS after PEBD (33% vs. 77%; P = 0.03). Overall, there were 15 major complications after surgery. Twelve patients (3 ALGS, 3 FIC1, and 6 BSEP) subsequently underwent liver transplantation. Conclusion: This was a multicenter analysis of nontransplant surgical approaches to intrahepatic cholestasis. Approaches vary, are well tolerated, and generally, although not uniformly, result in improvement of pruritus and cholestasis. (Hepatology 2017;65:1645‐1654).

Background/purpose: Hirschsprung disease (HD) is diagnosed by rectal biopsy, with suction rectal biopsy (SRB), the preferred technique in neonates. Reported SRB adequacy has varied overallwith concern for decreased diagnostic yield in older children. The study aim was to assess SRB adequacy by age in children with the current device used at our institution.Methods: Following IRB approval, a retrospective cohort of children (1 to 18 years) evaluated by SRB for HD was identified through billing records. Data regarding demographics, procedure, results, and complications were collected and analyzed using SPSS.Results: 56 children (median age 3.9 years) underwent SRBwith an 80.4% overall success rate. Patients older than 5 years had 90.5% adequacy rate compared to 74.3% in those younger. Univariate analysis revealed weak association of inadequate specimens with younger age and males, and no association with insurance, race/ethnicity, weight-height or BMI percentile, sedation type, or procedure location. SRB under general anesthesia (GA) had 100% adequacy (n = 6). Patients with inadequate initial biopsy achieved diagnosis by SRB with increased sedation (n = 5) or full thickness biopsy under GA (n = 5).Conclusion: With adequacy of 80.4% overall and 90.5% for patients greater than 5 years, SRB is effective in evaluating the older child for HD. (C) 2016 Elsevier Inc. All rights reserved.

A 4-month-old girl presented with poor feeding and nonbilious emesis. At 1 month of age, she had been hospitalized briefly for emesis but was able to feed until 1 week before presentation. Radiographs showed gastric pneumatosis.