Femoral hernia in pediatric population: a diagnostic and surgical challenge.

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.

Recurrent thoracic air leak syndrome in patients affected by pulmonary graft-versus-host disease: Surgical strategies and outcome.

BACKGROUND AND AIMS: Thoracic air leak syndrome (TALS) is a complication related to chronic pulmonary graft-versus-host disease (pGvHD) that affects approximately 0.83%-3.08% patients after allogenic hematopoietic stem cell transplant. Such complication is defined as the occurrence of any form of air leak in the thorax, including spontaneous pneumomediastinum or pneumopericardium, subcutaneous emphysema, interstitial emphysema and pneumothorax and has a negative impact on post-transplant survival. The aim of the present study is to describe a single-center experience in the surgical management of recurrent TALS in adolescents and young adults and its outcome. METHODS: We retrospectively reviewed the clinical notes of patients with previous allogenic hematopoietic stem cell transplant who underwent surgical procedures for recurrent TALS from January 2016 until March 2021. We analyzed clinical data, number of episodes of thoracic air leak, surgical procedures and relative outcome. RESULTS: In the examined period, four patients, aged 16-25 years, underwent surgical procedures for TALS, including thoracostomy tube placement, thoracoscopic pleurodesis and thoracotomy. All the patients had been diagnosed with pGvHD before the onset of TALS, with a mean time lapse of 276 days (range 42-513). These patients experienced on average 4.5 air leak episodes (range 3-6). All the patients experienced at least two episodes before surgery. One patient underwent emergency tube thoracostomy only, three patients underwent thoracoscopic pleurodesis and two patients underwent thoracotomy. After surgery, patients were free from air leak symptoms for a mean time of 176 days (range 25-477). Pulmonary function progressively deteriorated, and all the patients eventually died because of respiratory failure after a mean time of 483 days (range 127-1045) after the first episode of air leak. CONCLUSIONS: Surgery provides temporary relief to symptoms related to TALS but has limited effects on the underlying pathophysiologic process. The development of TALS in a sign of progressive pulmonary function worsening and is associated with high risk of respiratory failure and mortality.

Preoperative Spinal Angiography for Thoracic Neuroblastoma: Impact of Identification of the Adamkiewicz Artery on Gross Total Resection and Neurological Sequelae.

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.

Chemotherapy-induced cavitating Wilms' tumor pulmonary metastasis: Active disease or scarring? A case report and literature review.

The second most common abdominal tumor in children is Wilms' tumor, and the lung is where it most often metastasizes. The typical metastases are multiple, peripherally located, round, and variable-sized nodules. Atypical patterns are also possible and may create diagnostic challenges, especially in patients treated with chemotherapy. Among these, cavitating metastases are an anecdotal type of atypical secondary lung lesions. Here, we report a case of a chemotherapy-induced cavitating Wilms' tumor pulmonary metastasis discovered during the follow-up for an anaplastic nephroblastoma in a 6-year-old girl. Furthermore, we conducted a review of the existing literature on this exceedingly rare radiological pattern to establish its best management.

Safety and Efficacy of Mek Inhibitors in the Treatment of Plexiform Neurofibromas: A Retrospective Study.

INTRODUCTION: Plexiform neurofibromas (PN) represent the main cause of morbidity in patients affected by Neurofibromatosis Type 1 (NF1). Until recently, surgery has been the main treatment option in these patients, but it is burdened with a low efficacy rate and a high incidence of side effects as well as recurrence. In recent years, MEK inhibitors (MEKi) such as selumetinib and trametinib have shown great promise. METHODS: We retrospectively describe a single center cohort of NF1 patients affected by PN1 and treated with MEKi since 2019 to 2021. Patients recruited in the study were affected by PN that were not eligible to complete surgical excision, symptomatic or with major cosmetic deformation or functional neurological deficits. RESULTS: Most patients experienced improvement in clinical symptoms and quality of life, with reduction or stabilization of lesions. However, no complete response was achieved. The most common adverse effects involved the skin, affecting every patient. Importantly, no life-threatening adverse effects occurred. CONCLUSIONS: In our experience, MEKi treatment has been shown to be both safe and effective in improving symptomatology and quality of life.

Should we reduce routine surveillance imaging in pediatric germ cell tumors?

This paper retrospectively investigated the site and the detection method of relapses in children and adolescents with malignant germ cell tumors enrolled in the TCGM-AIEOP-2004 Study and subsequently developed a relapse, in order to evaluate a possible reduction in radiological exposure during follow-up. Including all malignant cases, serum tumor markers identified a relapse in more than 70% and, according to the selection criteria published by Children Oncology Group in 2018, in more than 90% of cases. These results confirm the importance of serum tumor markers as a relapse detection method, with possible reduction of radiology exams in specific subgroups.

Safety and effectiveness of subcutaneously anchored securement for tunneled central catheters in oncological pediatric patients: A retrospective study.

BACKGROUND: Proper securement of central venous catheters plays an important role in onco-hematological pediatric patients. A new subcutaneously anchored securement device has been recently introduced in the clinical practice, and it has been extensively used in children. METHOD: In our study, we have retrospectively investigated the safety and the effectiveness of such device, reviewing the experience of three Italian pediatric oncological units. We have considered only tunneled catheters (cuffed or non-cuffed) inserted in children with malignancy; all types of tunneled central catheters were included in the analysis (both centrally and peripherally inserted) as long as they were secured with a subcutaneously anchored device. We investigated the incidence of dislodgment and of other catheter-related complications, with special attention to local adverse effects potentially related to the securement device. RESULTS: We collected data from 311 tunneled catheters of different caliber: 80.4% were centrally inserted central catheters (CICC), 15.4% were peripherally inserted (PICC), and 4.2% were femorally inserted. Approximately half of the catheters (51%) were non-cuffed. Incidence of dislodgment was very low (2.6%) and the incidence of local pain or inflammation potentially related to the securement device was minimal (1.9%). Catheter related bacteremias were below 1 episode/1000 catheter days. No symptomatic catheter related thrombosis was reported. There was no significant difference in complications comparing cuffed versus non-cuffed catheters, or CICCs versus PICCs, or hematologic tumors versus solid tumors. CONCLUSION: In our retrospective analysis of a vast population of oncological pediatric patients with tunneled central catheters, the subcutaneously anchored securement device was tolerated very well, and it was highly effective in preventing dislodgment, both in cuffed and non-cuffed catheters.

The burden of central line-associated bloodstream infections in children with medical complexity.

BACKGROUND: Central line-associated bloodstream infections (CLABSI) are significant cause of complications in pediatric intensive care units (PICUs). An emerging challenge are CLABSIs in children with medical complexity (CMC) admitted to PICU. CMC are patients with chronic conditions with or without neurological impairment needing for tracheostomy and/or home mechanical or non-invasive ventilation and/or gastrostomy/jejunostomy. We evaluate CLABSI incidence in a PICU with high prevalence of CMC. METHODS: This was a retrospective study in the PICU of the Bambino Gesù Children Hospital from January 2017 to December 2020. The medical records were reviewed and demographic, clinical and microbiological data were extracted. CLABSI were defined according to the Center for Disease Control and Prevention's National Healthcare Safety Networks (NHSN) surveillance. RESULTS: A total of 101 children with 125 central lines (CLs) were included; 79/101 (78%) patients were CMC and 50/101 (50%) had a thracheostomy. CLABSI incidence was 2.75/1000 CL-days (9 cases/3269 CL-days); incidence was 0 in patients without underling conditions and 3.14/1000 in CMC (p < 0.001). CLABSI were due to gram negative bacteria in five patients, Candida spp in three and Staphylococcus hominis in one. CLs were removed in eight cases while in the later one, with CLABSI due to Pseudomonas aeruginosa, a conservative strategy was adopted cause of unavailable alternative venous access and removed at discharge with negative culture. All patients recovered. CONCLUSIONS: A target 0% CLABSI was possible in critically ill children without underling condition while a high incidence was reported in CMC and sustained by a peculiar CLABSI ecology. This ecology should be considered when a CLABSI was suspected in CMC for prompt antibiotics stewardship.

Percutaneous treatment of chest wall chondroid hamartomas: the experience of a single center.

BACKGROUND: Thoracic mesenchymal hamartomas are rare benign lesions. Rarely symptomatic, they may compress pulmonary parenchyma, leading to respiratory distress. Although spontaneous regression has been documented, the more common outcome is progressive growth. The treatment of choice is en bloc excision of the involved portion of the chest wall, frequently leading to significant deformity. OBJECTIVE: The aim of our study was to describe percutaneous techniques to treat these lesions. MATERIALS AND METHODS: We collected data of children with thoracic mesenchymal hamartomas who were treated at our institution from 2005 to 2020 using various percutaneous techniques. Techniques included radiofrequency thermoablation, microwave thermoablation (microwave thermoablation) and cryoablation. RESULTS: Five children were treated for chest wall hamartomas; one child showed bilateral localization of the mass. Two children underwent microwave thermoablation, one radiofrequency thermoablation and two cryoablation; one child treated with cryoablation also had radiofrequency thermoablation because mass volume increased after the cryoablation procedure. The median reduction of tumor volume was 69.6% (24.0-96.5%). One child treated with microwave thermoablation showed volumetric increase of the mass and underwent surgical removal of the tumor. No major complication was reported. CONCLUSION: Percutaneous ablation is technically feasible for expert radiologists and might represent a valid and less invasive treatment for chest wall chondroid hamartoma, avoiding skeletal deformities.

Intraoperative Neuromonitoring for Thyroid Surgery in Children and Adolescents: A Single Center Experience.

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.

Uterine leiomyoma in pediatric population: A case report and review of the literature.

Uterine leiomyomas are rare in the pediatric population with less than 20 cases in adolescences reported in the literature. Furthermore, these masses represent a common presentation of gynecologic tumors with increasing age. We report a case of a 14-year-old female who presented with abdominal pain and increasing abdominal girth. Workup with ultrasound, CT and MRI demonstrated a large pelvic mass. Complete resection by median laparotomy was performed. The mass weighed 5,596 g and was 29.5 cm × 27 cm × 19 cm; the pathological examination confirmed the hypothesis of leiomyoma. The patient remained asymptomatic at 3 months follow up.

Intraoperative Neuromonitoring for Pediatric Pelvic Tumors.

Background: Tumors of the pre-sacral and sacral spaces are a rare occurrence in children. Total tumor excision is required due to the significant risk of relapse in the event of partial surgery, but the surgical procedure may lead to postoperative problems such as urinary, sexual, and anorectal dysfunctions. Intraoperative neuromonitoring (IONM) has gained popularity in recent years as a strategy for preventing the onset of neurologic impairments by combining several neurophysiological techniques. The aim of our study is to describe the experience of Bambino Gesù Children's Hospital in the use of IONM in pediatric pelvic surgery. Materials and Methods: The data of patients treated for pelvic malignancies at Bambino Gesù Children's Hospital from 2015 to 2019 were retrospectively collected. All patients were assessed from a neurologic and neuro-urologic point of view at different time-points (before and immediately after surgery, after 6 months, and 1-year follow-up). They were all monitored during a surgical procedure using multimodal IONM including transcranial motor evoked potentials (TcMEP), triggered-EMG (t-EMG), pudendal somatosensory evoked potentials (PSSEP), and bulbocavernosus reflex (BCR). Results: During the study period, ten children underwent pelvic tumor removal at our Institution. In all cases, intraoperative neurophysiological recordings were stable and feasible. The preservation of neurophysiological response at the same intensity during surgical procedures correlated with no new deficits for all neurophysiological techniques. Discussion: Although the impact of the IONM on surgical strategies and clinical follow-up is unknown, this preliminary experience suggests that the appropriate use of several neurophysiological techniques can influence both the radicality of pelvic tumor removal and the neurological and urological outcome at clinical follow-up. Finally, because of the highly complex anatomy and inter-individual variances, this is especially useful in this type of surgery.

Thyroid cancer in children: A multicenter international study highlighting clinical features and surgical outcomes of primary and secondary tumors.

Background: Thyroid gland malignancies are rare in pediatric patients (0.7% of tumors); only 1.8% are observed in patients aged <20 years, with a higher prevalence recorded in women and adolescents. Risk factors include genetic syndromes, MEN disorders, autoimmune diseases, and exposure to ionizing radiation. Radiotherapy is also associated with an increased risk of secondary thyroid cancer. This study describes the clinical features and surgical outcomes of primary and secondary thyroid tumors in pediatric patients. Methods: Institutional data were collected from eight international surgical oncology centers for pediatric patients with thyroid cancer between 2000 and 2020. Statistical analyses were performed using the GraphPad Prism software. Results: Among 255 total cases of thyroid cancer, only 13 (5.1%) were secondary tumors. Primary thyroid malignancies were more likely to be multifocal in origin (odds ratio [OR] 1.993, 95% confidence interval [CI].7466-5.132, p = 0.2323), have bilateral glandular location (OR 2.847, 95% CI.6835-12.68, p = 0.2648), and be metastatic at first diagnosis (OR 1.259, 95% CI.3267-5.696, p > 0.999). Secondary tumors showed a higher incidence of disease relapse (OR 1.556, 95% CI.4579-5.57, p = 0.4525) and surgical complications (OR 2.042, 95% CI 0.7917-5.221, p = 0.1614), including hypoparathyroidism and recurrent laryngeal nerve injury. The overall survival (OS) was 99% at 1 year and 97% after 10 years. No EFS differences were evident between the primary and secondary tumors (chi-square 0.7307, p = 0.39026). Conclusions: This multicenter study demonstrated excellent survival in pediatric thyroid malignancies. Secondary tumors exhibited greater disease relapse (15.8 vs. 10.5%) and a higher incidence of surgical complications (36.8 vs. 22.2%).

Case report: Bilateral pleural effusion secondary to late migration of a tunneled central venous catheter in a patient affected by high risk neuroblastoma.

The insertion of long-term central venous catheters is a standard of care for children affected by malignancies, although it can be associated with life-threatening complications. The present paper reports an unusual mechanical complication related to the use of a long term tunneled central venous catheter in a pediatric oncologic patient. An 18 months old child, diagnosed with stage M high-risk retroperitoneal neuroblastoma, underwent ultrasound-guided placement of a 6 Fr bilumen long-term tunneled central venous catheter in the right internal jugular vein prior to the beginning of induction chemotherapy. The correct position of the distal tip of the catheter was confirmed by fluoroscopy. After 4 months of regular use of the device, the patient experienced neck swelling during high-dose chemotherapy infusion. A chest x-ray showed a dislocated catheter and bilateral pleural effusion. CT scan demonstrated the tip of the catheter rupturing the medial wall of the right jugular vein and entering the mediastinum; furthermore, pneumomediastinum, subcutaneous neck emphysema and bilateral pleural effusion were noticed and a thrombus was evident in the right jugular vein at the insertion in the brachiocephalic vein. The patient was then transferred to the Intensive Care Unit and bilateral thoracostomy tubes were placed urgently (500 mL of clear fluid were evacuated from pleural spaces). The dislocated catheter was removed electively on the following day under fluoroscopy. Despite ultrasound-guided placement and long-term uneventful use of the catheter, life-threatening central venous catheter-related mechanical complications can occur; the current case report emphasizes the importance of careful monitoring of patients with central venous catheters in order to quickly diagnose and treat potentially lethal complications.

The Fight Just Born-Neonatal Cancer: Rare Occurrence with a Favorable Outcome but Challenging Management.

The occurrence of cancer in newborns within the first 28 days of life is uncommon, with different clinical presentation from other age groups. Prenatal diagnosis is reported in about half of patients, while a genetic predisposition condition is supposed. The management of a newborn with cancer can be challenging and needs to be tailored according to the histology and the primary tumor site; surgery represents the main strategy, while chemotherapy should be considered with caution because of the higher toxicity and mortality due to different pharmacokinetics in neonates compared to older children. We describe the first Italian series over a 15-year period of patients affected by both benign and malignant neoplastic diseases diagnosed within the first 28 days of life; 74 newborns were diagnosed with neonatal tumors, representing 1.5% of the cancer population in the same period, and a prevalence of germ cell tumors (55%) and neuroblastoma (16%) was observed. Surgery was performed on 80% of patients, while chemotherapy was necessary for about 20% of patients. The 5-year overall survival (OS) exceeded 90%; treatment-related deaths are a major concern, representing 80% of overall deaths. A genetic/syndromic condition was detected in 16% of the population; additionally, a cancer predisposition syndrome (CPS) was identified in about 10% of patients. According to our experience, all newborns affected by cancer should warrant genetic counselling and a screening test for CPS.

Does letting adolescent and young adult inpatients share decisions in choosing the central-line insertion site reduce central-line-associated bloodstream infections? An empty systematic review.

To study whether allowing adolescents and young adults (AYA) with chronic or oncologic diseases admitted to tertiary or intensive care units to share decisions in choosing the insertion site for central-venous catheters (CVC) implanted for intravenous therapies or parenteral nutrition reduces central-line-associated and catheter-related bloodstream infections (CLABSI and CRBSI). Following the PRISMA guidelines, we systematically reviewed the literature by searching MEDLINE, Embase, CINAHL, CENTRAL, SCOPUS, Cochrane Library, and Web of Science up to December 2019. According to our aims, the review identified no study that could be included. This empty systematic review on healthcare teams allowing AYA with chronic or oncologic diseases admitted in tertiary or intensive care units to share decisions in choosing the site for implanting CVC prompts further research on clinical pathways on this hot-topic. By considering purportedly risk-taking behaviors in youngsters thus reducing CLABSI and CRBSI, healthcare teams should test specific strategies by engaging AYA empathetically in sharing decisions on the site for implanting CVC to improve quality in health care bundles.

Vascular Access in Pediatric Oncology and Hematology: State of the Art.

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.

Guidelines of the Italian Association of Pediatric Hematology and Oncology for the management of the central venous access devices in pediatric patients with onco-hematological disease.

INTRODUCTION: Central venous accesses devices (CVADs) have a fundamental importance for diagnostic and therapeutic purposes in pediatric onco-hematological patients. The treatment of pediatric onco-hematological diseases is complex and requires the use of integrated multimodal therapies. Long-lasting and safe central venous access is therefore a cornerstone for any successful treatment. METHODS: The aim of this work is to define pediatric guidelines about the management of CVADs in onco-hematology. A panel of experts belonging to the working groups on Infections and Supportive Therapy, Surgery and Nursing of the Italian Pediatric Hematology Oncology Association (AIEOP) revised the scientific literature systematically, scored the level of evidence and prepared these guidelines. The content of the following guidelines was approved by the Scientific Board of AIEOP. RESULTS AND CONCLUSIONS: Important innovations have been developed recently in the field of CVADs, leading to new insertion methods, new materials and new strategy in the overall management of the device, especially in the adult population. These guidelines recommend how to apply these innovations in the pediatric population, and are directed to all physicians, nurses and health personnel active in the daily management of CVADs. Their aim is to update the knowledge on CVAD and improve the standard of care in pediatric patients with malignancies.