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Introduction: Patients affected by metastatic germ cell tumors may occasionally experience enlargement of masses with concurrent normalization of tumor markers during or after chemotherapy. This phenomenon is described as growing teratoma syndrome (GTS). The aim of the pre sent study is to assess the prevalence of GTS in the pediatric population and its implications in terms of surgical outcome., Patients and Methods: The clinical notes of patients diagnosed with stage III and IV malignant germ cell tumors from January 2010 until December 2020 at our Institution were retrospectively reviewed. The prevalence of GTS, treatment strategies, survival, and outcome were analyzed., Results: Thirty-three patients with high-stage malignant germ cell tumors were diagnosed in our institution in the analyzed period. Nine patients (28%) had radiologic evidence of enlargement of persistent masses with normal markers after chemotherapy; these patients were classified as GTS patients. All nine patients underwent resection of metastatic lymph nodes, and six had surgery on visceral metastases. In six patients, radical excision of all metastatic sites was achieved; five patients are alive and in complete remission, while one died because of peri-operative complications. Out of the three patients who could not achieve radical excision of the metastases, two died of progressive disease, and one is alive with progressive disease., Conclusions: Patients affected by GTS have a risk of progression of chemotherapy-resistant disease and death. Radical surgical excision is essential to achieve disease control and long-term survival., (© 2024 The Author(s). Pediatric Blood & Cancer published by Wiley Periodicals LLC.)

Purpose: Femoral hernia (FH) is a rare and often misdiagnosed pathology in pediatric population. The aim of our study was to describe the experience of a Tertiary Center in children with FH, underlying diagnostic and surgical details that could improve its management., Methods: A retrospective study of pediatric patients who underwent FH repair from January 2010 to June 2023 at our Institution was performed., Results: In the analyzed period, 31 patients underwent surgical procedure for FH at our institution, of whom 16 (51.6%) were female. The mean age at time of surgery was 5.8 years (range 0.5-17.1 years). The rate of pre-operative misdiagnosis was 35.5% and open approach was adopted in all cases (16.1% with mesh application). Only two patients (6.4%) experienced surgical complications: one recurrence (repaired six months later) and one post-operative hematoma (treated successfully with conservative method)., Conclusion: Due to the high rate of misdiagnosis, the variety of surgical approaches proposed, and the potential for intraoperative complications, FH poses a challenge for pediatric surgeons and urologists, as confirmed by the literature. It is essential to underline the importance of a proper clinical examination in order to correctly diagnose FH, make the best surgical plan for the patient and prevent post-operative complications., (© 2024. The Author(s), under exclusive licence to Springer-Verlag France SAS, part of Springer Nature.)

Background: No age-specific pediatric guidelines exist for the management of spontaneous pneumothorax (SP) in children and adolescents. Treatment remains heterogeneous and center dependent. The role of computed tomography (CT) has yet to be defined., Aims: Review the management of SP in children and adolescents, with emphasis on conservative management and role of CT., Methods: Retrospective analysis of 61 consecutive patients with SP at single tertiary center. Clinical, radiological, surgical data, follow-up, and outcomes were revised., Results: First-line management was conservative for 32 (53%) patients and operative for 29 (47%). Asymptomatic/paucisymptomatic patients managed conservatively experienced less first-line treatment failure. Furthermore, the patients needing at least a chest drain or surgery during the follow-up were significantly lower in the conservative group. Conservative and operative patients showed no significant differences regarding ipsilateral recurrences or contralateral occurrences. Of the 61 overall CTs performed, 14 (23%) had an impact on management. Forty-three (70%) patients had at least a CT, in 22 (51%) the CT was positive for blebs. For 10 of these patients (45%) the presence of blebs had an impact on management. Patients with and without blebs showed no differences regarding ipsilateral recurrence, contralateral occurrences, or the need for at least a chest drain or surgery during the follow-up., Conclusions: First-line conservative management had a significantly shorter hospitalization and better outcome, with a similar incidence of recurrences. The presence of blebs at CT does not predict the risk of recurrence. The CT scan should be reserved for a small number of selected patients who have post-VATS refractory or recurrent pneumothorax., (© 2023 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Background: Laparoscopic appendectomy followed by postoperative intravenous (IV) antibiotics is the standard of care for acute appendicitis and postoperative prevention of intra-abdominal abscesses. The aim of or study was to determine if intraperitoneal irrigation with antibiotics could help prevent intra-abdominal abscess formation after laparoscopic appendectomy for complicated appendicitis in pediatric patients., Methods: A retrospective study was conducted on consecutive pediatric patients with acute appendicitis who had appendectomy in our Pediatric Surgery Department between August 2020 and February 2022. We compared two groups with similar age and symptoms. The first group (A) was treated with the normal standard of care, i.e., laparoscopic appendectomy and postoperative IV antibiotic therapy. For the second group (B) intraperitoneal cefazoline irrigation was added at the end of the laparoscopic procedure. Postoperative intra-abdominal abscess was diagnosed with ultrasound examination, performed after clinical suspicion/abnormal blood test results., Results: One hundred sixty patients (males:females 109:51; median age 10.5 years [range 3-17 years]) who had laparosopic appendectomy for complicated appendicitis were included, 82 in group A and 78 in group B. In the first 7 days after surgery, 18 patients in group and 5 in group B developed an intra-abdominal abscess (p < 0.005). Drains were positioned in 38 patients in group A vs. 9 in group B. One patient in group A had a different complication which was infection of the surgical incision., Conclusions: Intraperitoneal cefazoline irrigation at the end of the laparoscopic appendectomy in pediatric patients significantly reduces the formation of intra-abdominal abscesses.

Background and Objectives: Marfan syndrome (MS) is a systemic disease of connective tissues consisting of a variable combination of anomalies. These patients have an increased risk of spontaneous pneumothorax (SP). However, there is a scarcity of pediatric literature on management, and no specific guidelines exist. Our aim was to analyze the management of spontaneous pneumothorax in children and adolescents with Marfan syndrome, comparing syndromic and non-syndromic patients., Methods: Retrospective analysis of pediatric patients (18 years) with SP diagnosed at our tertiary pediatric hospital (January 10-June 22), with special emphasis on diagnosis, treatment, and follow-up (FU)., Results: Sixty-six patients with SP were identified, with nine (13%) having MS. In terms of baseline, there were no significant differences between the groups (age, sex, asthma, symptoms, and side, first-line treatment and hospitalization length). Overall, Marfan patients had significantly more first-line treatment failures requiring additional surgery, as well as more contralateral occurrences and the need for surgery/chest drain during the follow-up. Instead, conservative management resulted in significantly more ipsilateral recurrences and the need for surgery/chest drain in Marfan patients than controls during the follow-up., Conclusions: Treatment failure, contralateral occurrence, ipsilateral recurrence, and the need for surgery/chest drain during follow-up make management of patients with Marfan syndrome and spontaneous pneumothorax more difficult. In patients with a diagnosed MS a more aggressive first-line management should be considered, bearing in mind the higher risks of this population., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision. The reviewer GC declared a past co-authorship with the author AI to the handling editor., (© 2023 Zarfati, Frediani, Pardi, Aloi, Madafferi, Accinni, Bertocchini and Inserra.)

Background: Patients with thoracic neuroblastoma (TNB) are at high risk of postoperative neurologic complications due to iatrogenic lesions of the artery of Adamkiewicz (AKA). The role of performing a preoperative spinal angiography (POSA) in these patients must be clarified. The present study sought to further understand the relationship between POSA and TNB, as well as the effects of identifying the AKA on surgical excision and neurological consequences., Methods: Data from patients with TNB who underwent POSA between November 2015 and February 2022 at our tertiary pediatric center were retrospectively analyzed., Results: Six patients were identified, five of whom (83%) were considered eligible for surgical excision. Gross total resection (GTR) was achieved in three patients (60%), which included two patients with an AKA contralateral to the tumor, and one with an homolateral AKAl. After a median follow-up of 4.1 years from diagnosis, no patients developed neurological complications; five (83%) were alive and well, and one died from refractory recurrence., Conclusions: Among patients with TNB, POSA was useful for identifying the AKA and defining the optimal surgical strategy. POSA should be considered in the preoperative evaluation of TNB to increase the likelihood of GTR and reduce the threats of iatrogenic neurologic sequelae.

Intraoperative neuromonitoring (IONM) of the recurrent laryngeal nerve (RLN) has been shown in adults to minimize nerve palsy after thyroid surgery, but only few studies on its efficacy in a pediatric population have been reported. We conducted a retrospective study on patients operated for thyroid lesions from 2016 to 2022. The analyzed population was divided in two groups: patients treated from 2016 to 2020, when the identification of the RLN was performed without IONM (Group A); and patients treated since 2021, when IONM was implemented in every surgical procedure on the thyroid (Group B). Intraoperative Neurophysiological Monitoring was performed by using corticobulbar motor-evoked potentials and continuous electromyography. Twentyfive children underwent thyroid resection, 19 (76%) of which due to thyroid carcinoma. Each patient's recurrent nerve was identified; IONM was used in 13 patients. In Group A, one temporary nerve palsy was identified postoperatively (8.3%), while in group B one nerve dysfunction occurred (7.7%). No statistically significant difference was found between the two groups in terms of post-operative RLN palsy. No surgical complication due to the use of IONM was reported. In children and teenagers, intraoperative neuromonitoring of the recurrent laryngeal nerve is a safe and accurate method, minimizing the risk of nerve damage.

Management and successful use of vascular access are critical issues in pediatric patients affected by malignancies. Prolonged course of disease, complex and various treatment protocols require long-lasting vascular access providing adequate tools to administrate those therapies and to collect routine blood sampling without painful and repeated venipuncture. For these reasons, central venous catheters are currently an important component in pediatric onco-hematological care, with a direct influence on outcome. Indeed, there are peculiar issues (techniques of insertion, management, complications etc.) which must be well-known in order to improve the outcome and the quality of life of children with cancer.

Aim: Paraplegia is an infrequent although fearsome complication of anesthesia and surgical procedures, such as epidural anesthesia and thoracotomy. It may occur in both adults and children and a medullary lesion may be confirmed by magnetic resonance imaging, rather than computed tomography. The aim of this study is to describe the experience of two pediatric tertiary centers, contextualizing it with the other cases reported in literature., Material and Methods: We reported three pediatric cases of post-operative paraplegia in oncological patients, focusing on the potential causes and underling the possible strategies to prevent this complication., Results: From our study, two principal features emerged: 1) Epidural anesthesia may expose children to a greater risk of spinal cord permanent damage due to the execution of the procedure under general anesthesia, which deprives the anesthesiologist of an important feedback about the position of the device; 2) In thoracotomy, the risk of paraplegia tends to increase along with the proximity to the costo-vertebral angle, especially if electrocautery or hemostatic materials are used., Conclusions: A prompt post-surgical neurological status routine assessment in pediatric patients undergoing epidural anesthesia or thoracotomy should be mandatory and, associated with the correct imaging study, may lead to the most appropriate therapeutic pathway and to a better prognosis., Key Words: Anesthesia, Children, Epidural, Thoracotomy, Paraplegia.

Introduction: The management of congenital splenic cysts continues to evolve. In the past the standard treatment was splenectomy, but increased knowledge about the spleen's immunologic function has led most pediatric surgeons to preserve splenic tissue. A great number of studies using sclerosing substances have been published, but to date reports in children have been limited. Our study concerns a group of 15 children with congenital splenic cysts treated with percutaneous drainage and sclerosis with alcohol. We performed the procedure under general anesthesia and checked radiologically for possible leakage., Methods: In 2000 our group started managing pediatric patients with splenic cysts. During the first eight years surgery was the treatment of choice. From April 2008 to December 2014, a prospective study was conducted on 15 consecutive patients treated with percutaneous sclerotherapy. The outcomes regarding cystic dimensional variations before and after treatment were analyzed., Results: In 20% of patients complete disappearance of the cystic lesion was achieved. In 67% of the patients the maximum diameter of the cyst was reduced to below 50mm., Conclusion: Our results should encourage the use of this treatment because it is a valid and safe option in childhood. The high success rate achieved with percutaneous drainage and sclerotherapy of cystic lesions supports our results., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: Local control is always considered in metastatic neuroblastoma (NBL). The aim of this study is to evaluate the impact of radical surgery on survival in children over 1 year of age., Methods: Fifty-eight patients older than 1 year of age with metastatic NBL were treated with conventional plus high-dose chemotherapy with or without addition of local radiotherapy (RT, 21Gy). Surgery was classified as radical surgery (complete resection and gross total resection) or non-radical surgery. The Kaplan-Meier method and the Cox proportional hazard model were used to calculate the probability of progression free and overall survival (PFS and OS) and for multivariate analysis., Results: The 5-year PFS and OS for patients with radical surgery were 26% (95% CI 14-40%) and 38% (95% CI 23-53%) respectively, while the PFS and OS for patients without radical surgery were 33% (95% CI 10-59%) and 31% (95% CI 10-55%) (respectively, P 0.85 and P 0.42). The 5-year PFS and OS for patients who received RT were 36% (95% CI 19-53%) and 46% (95% CI 26-64%) respectively, while the 5-year PFS and OS for patients who did not receive RT were 22% (95% CI 9-38%) and 27% (95% CI 13-42%) respectively (P 0.02 for PFS). Multivariate analysis confirmed the role of well-known prognostic factors, such as the presence of MYCN amplification, age and response before high-dose chemotherapy., Conclusions: Our data suggest that the degree of resection does not influence survival in metastatic NBL patients treated with high-dose chemotherapy; local RT contributes to local disease control.