Percutaneous endoscopic gastrostomy in children receiving peritoneal dialysis: A tertiary centre long-term experience and literature review.

BACKGROUND: Children with severe chronic kidney disease receiving maintenance peritoneal dialysis (PD) are often malnourished and may require nutritional supplementation. Recent PD guidelines address laparoscopic and open surgical gastrostomy as safe approaches in children established on PD, while existing evidence on percutaneous endoscopic gastrostomy (PEG) is still lacking; as well as the role of perioperative antibiotic and antifungal prophylaxis. Hence, this study aimed to report our experience with PEG placement in patients on PD and compare it with the available literature. METHODS: We retrospectively reviewed the medical records from patients on PD, who underwent PEG placement at a tertiary referral centre between 2000 and 2020. Data on perioperative management, complications and outcomes were retrieved. An extensive literature search was performed; studies describing PEG placement and perioperative prophylaxis in patients on PD were used as a comparison. Descriptive statistical analysis was conducted. RESULTS: Seven patients (five males) were included. Perioperative antibiotic and antifungal prophylaxis were standard practice. At a median follow-up of 27 months (10-75), the peritonitis rate was 0.2 patient/years. No statistical significance was found between the peritonitis rate before and after PEG placement (p = 0.2). Patients' demographics and postoperative complications were comparable to the reported studies. CONCLUSIONS: Based on our experience, our technique of PEG insertion with antimicrobial prophylaxis is feasible and associated with an acceptable complication risk in patients on PD. Further multicentric studies about surgical technique in patients on PD will be necessary to verify the feasibility of PEG and standardise the perioperative protocol.

Laparoscopic Heller-Dor Is a Persistently Effective Treatment for Achalasia Even in Pediatric Patients: A 25-Year Experience at a Single Tertiary Center.

BACKGROUND: Esophageal achalasia (EA) is a rare primary motility disorder in any age group, and particularly rare in the pediatric population, with a reported incidence of 0.18 per 100,000 children a year. EA in pediatric age is currently treated in the same way as in adults, but this approach is based on only a few studies on small case series. The aim of this retrospective study was to assess the long-term outcome of the laparoscopic Heller-Dor (LHD) procedure when performed in pediatric patients with EA at our university hospital. MATERIALS AND METHODS: We considered children and adolescents younger than 16 years old diagnosed with EA and treated with LHD between 1996 and 2022. Clinical data were prospectively collected in an ongoing database. Symptoms were recorded and their severity was calculated using the Eckardt score. Barium swallow, esophageal manometry (conventional or high-resolution), and endoscopy were performed before and after the surgical procedure. RESULTS: During the study period, 40 children with a median age of 14 years (interquartile range [IQR]: 11-15) underwent LHD. At a median follow-up of 10.5 years (IQR: 4.5-13.9), a good outcome was achieved in 36/40 patients (90%). Two of the four patients whose surgical procedure failed underwent complementary pneumatic dilations successfully, thus increasing the overall success rate to 95%. A previous endoscopic treatment (in five patients) did not affect the final outcome (p = 0.49). An intraoperative mucosal lesion was detected in only one patient (2.5%) and was repaired at the time without further consequences. During the follow-up, 22 patients underwent endoscopy, and 17 had pH monitoring as well: only 2 of these patients showed reflux esophagitis at endoscopy (one of them with abnormal findings on pH monitoring), amounting to a 9.1% rate of instrumentally confirmed postoperative reflux. CONCLUSION: LHD is a safe and persistently effective treatment for EA in pediatric age, with a success rate comparable with what is usually obtained in adults, and better than what has been reported to date in the pediatric literature. Adding a fundoplication certainly helps ensure an optimal long-term control of any gastroesophageal reflux induced by the myotomy.

Hao-Fountain syndrome and genital disorders: report of a new possible association.

BACKGROUND: Hao-Fountain syndrome is a neurodevelopmental disorder characterized by global developmental delay, variably impaired intellectual development with significant speech delay and, in some males patients, it has been reported an association with hypogonadism. At present less than 50 cases are reported in literature. CASE PRESENTATION: We report a case of this rare syndrome in a young female with isolated tubal torsion; our patients had different hospitalizations without treatment but during the last episode we decide to perform an abdominal surgical explortion. This is the first case in Literature with a new USP7 mutation. CONCLUSIONS: This case opens new perspective in this rare syndrome and a review approach to isolated tubal torsion. These symptoms should be always well checked.

Anesthesia for fetal operative procedures: A systematic review.

Objective: The anesthetic management of fetal operative procedures (FOP) is a highly debated topic. Literature on fetal pain perception and response to external stimuli is rapidly expanding. Nonetheless, there is no consensus on the fetal consciousness nor on the instruments to measure pain levels. As a result, no guidelines or clinical recommendations on anesthesia modality during FOP are available. This systematic literature review aimed to collect the available knowledge on the most common fetal interventions, and summarize the reported outcomes for each anesthetic approach. Additional aim was to provide an overall evaluation of the most commonly used anesthetic agents. Methods: Two systematic literature searches were performed in Embase, Medline, Web of Science Core Collection and Cochrane Central Register of Controlled Trials up to December 2021. To best cover the available evidence, one literature search was mostly focused on fetal surgical procedures; while anesthesia during FOP was the main target for the second search. The following fetal procedures were included: fetal transfusion, laser ablation of placental anastomosis, twin-reversed arterial perfusion treatment, fetoscopic endoluminal tracheal occlusion, thoraco-amniotic shunt, vesico-amniotic shunt, myelomeningocele repair, resection of sacrococcygeal teratoma, ligation of amniotic bands, balloon valvuloplasty/septoplasty, ex-utero intrapartum treatment, and ovarian cyst resection/aspiration. Yielded articles were screened against the same inclusion criteria. Studies reporting anesthesia details and procedures' outcomes were considered. Descriptive statistical analysis was performed and findings were reported in a narrative manner. Results: The literature searches yielded 1,679 articles, with 429 being selected for full-text evaluation. A total of 168 articles were included. Overall, no significant differences were found among procedures performed under maternal anesthesia or maternal-fetal anesthesia. Procedures requiring invasive fetal manipulation resulted to be more effective when performed under maternal anesthesia only. Based on the available data, a wide range of anesthetic agents are currently deployed and no consistency has been found neither between centers nor procedures. Conclusions: This systematic review shows great variance in the anesthetic management during FOP. Further studies, systematically reporting intraoperative fetal monitoring and fetal hormonal responses to external stimuli, are necessary to identify the best anesthetic approach. Additional investigations on pain pathways and fetal pain perception are advisable.

Pediatric Surgery and Anesthesia in Low-Middle Income Countries: Current Situation and Ethical Challenges.

Low-middle income countries (LMICs) are currently experiencing an important population growth, leading to a substantial raise in the number of children living in those areas. As a consequence, the existing gap between the need for surgical and anesthetic care and the available therapeutic options will increase. To overcome this, an improvement in the available expertise, infrastructures, and supplies will be mandatory. The implementation of educational and training programs for local healthcare providers should be a top priority. Alongside, the population's awareness on the necessity to seek for medical care should be deployed, together with an eased access to health facilities. Based on the existing literature and our 20-years' experience in humanitarian missions, our article aims to investigate the status of pediatric surgery in LMICs, and the role of western aids in the implementation of this ever-increasing field of expertise.

Lung Ultrasound to Assess One Lung Ventilation: A Pediatric Case Series.

Objectives: One lung ventilation (OLV) is the preferred ventilation technique for thoracoscopy as it provides a better exposure of the operative field and grants the protection of the healthy lung. Preoperative evaluation of lung exclusion is necessary and different methods are available. In recent years lung ultrasound (US) gained popularity and its use for monitoring the endotracheal tube position is widely reported. The existing evidence on adults addresses lung US as effective, yet only few data are available in children. Therefore, we present our experience with lung US as verification method for pediatric OLV. Methods: All patients undergoing OLV for video-assisted thoracoscopic surgery from January 2019 to May 2021 and for whom lung exclusion was confirmed through lung US were involved. Lung exclusion was considered effective when absence of lung motion and presence of lung pulse were encountered. When lung US did not match these criteria, repositioning of the endobronchial device followed by US verification was performed. When lung US met the exclusion criteria surgery was started and direct thoracoscopic observation was used to verify lung exclusion. Results: A total of 20 patients, accounting for 22 procedures, were involved. Absence of lung motion and presence of lung pulse were assessed in the operative-side lung for all patients. Lung exclusion was confirmed through thoracoscopy. Postoperative lung US proved the reappearance of lung motion in the previously excluded lung. Conclusions: In our center experience lung US resulted to be a safe, effective, and time-saving verification method for OLV. Further studies are needed to define its sensitivity and specificity.

Innovative Techniques Associated with Traditional Abdominal Surgery in Complex Pediatric Cases: A Tertiary Center Experience.

Pediatric abdominal surgery is constantly evolving, alongside the advent of new surgical technologies. A combined use of new tools and traditional surgical approaches can be useful in the management of complex cases, allowing less invasive procedures and sometimes even avoiding multiple interventions. This combination of techniques has implications even from the anesthetic point of view, especially in post-operative pain control. Thereby, tertiary level centres, including highly-specialized professionals and advanced equipment, can maximize the effectiveness of treatments to improve the final outcomes. Our paper aims to present some possible combinations of techniques recently used at our institution to provide a one-session, minimally invasive treatment within different areas of abdominal surgery.

Results of an International Survey on Feeding Management in Infants With Short Bowel Syndrome-Associated Intestinal Failure.

OBJECTIVES: Short bowel syndrome (SBS) is a complex and rare condition (incidence 1200/100,000 live births) that requires a multidisciplinary team approach to management. In January 2019, the first European Reference Network on Rare and Inherited Congenital Anomalies (ERNICA) Intestinal Failure (IF) workshop was held. Several questions about the strategies used in managing IF associated with SBS were devised. The aim of our study was to collect data on the enteral feeding strategies adopted by the ERNICA centres. METHODS: A questionnaire (36 questions) about strategies used to introduce enteral nutrition post-operatively and start complementary food/solids in infants with SBS associated IF was developed and sent to 24 centres in 15 countries that participated in the ERNICA-IF workshop. The answers were collated and compared with the literature. RESULTS: There was a 100% response rate. In infants, enteral nutrition was introduced as soon as possible, ideally within 24-48 hours post-small intestinal surgical resection. In 10 of 24 centres, bolus feeding was used, in nine continuous, and in five a combination of both. Twenty-three centres used mothers' own milk as the first choice of feed with extensively hydrolysed feed, amino acid-based feed, donor human milk or standard preterm/term formula as the second choice. Although 22 centres introduced complementary/solid food by 6 months of age, food choice varied greatly between centres and appeared to be culturally based. CONCLUSIONS: There is diversity in post-surgical enteral feeding strategies among centres in Europe. Further multi-centre studies could help to increase evidence-based medicine and management on this topic.

Anesthesia in Children with Neuroblastoma, Perioperative and Operative Management.

Neuroblastoma (NB) is the most common extracranial, solid, pediatric malignancy and, despite the constant progress of treatment and development of innovative therapies, remains a complex, challenging disease causing major morbidity and mortality in children. There is significant variability in the management of neuroblastoma, partially due to the heterogeneity of the clinical and biological behavior, and partially secondary to the different approaches between treating institutions. Anesthesia takes an integral part in the multidisciplinary care of patients with NB, from diagnosis to surgery and pain control. This paper aims to review and discuss the critical steps of the perioperative and operative management of children undergoing surgery for neuroblastoma. Anesthesia and analgesia largely depend on tumor location, surgical approach, and extension of the surgical dissection. Attention should be paid to the physio-pathological changes on cardiovascular, gastrointestinal, and immune systems induced by the tumor or by chemotherapy. At the time of surgery meticulous patient preparation needs to be carried out to optimize intraoperative monitoring and minimize the risk of complications. The cross-sectional role of anesthesia in cancer care requires effective communication between all members of the multidisciplinary team.

Surgical Approaches to Neuroblastoma: Review of the Operative Techniques.

Neuroblastoma (NB) is the most commonly occurring soft-tissue malignancy of childhood. Surgery plays an important role in multidisciplinary treatment and its principal aim is a local control of the disease, respecting the integrity of the surrounding structures. There is no unanimous consensus on the best surgical technique, and the operative approach largely depends on the anatomical location and the extension of the mass. To have a complete overview of the different type of treatment, we made a review of the literature from the last twenty years of all the surgical approaches applied for NBs resection, accordingly to the anatomical site.

The Safety of Intralesional Steroid Injections in Young Children and Their Effectiveness in Anastomotic Esophageal Strictures-A Meta-Analysis and Systematic Review.

OBJECTIVE: Intralesional steroid injections (ISI) are a widely used technique for various pediatric indications and represent a possible adjuvant treatment for anastomotic esophageal strictures. Yet, no consensus has been reached neither on their safety in the pediatric population or their effectiveness in esophageal atresia patients. This systematic review aimed to assess the safety of ISI in young children through a meta-analysis and to summarize the current knowledge on the effectiveness of ISI in anastomotic esophageal strictures. METHODS: A systematic literature search was performed in Embase, Medline, Web of Science Core Collection, Cochrane Central Register of Controlled Trials and Google Scholar up to August 16 2021. Studies focusing on ISI and involving children up to 2 years were included in the meta-analysis for the safety assessment. All studies evaluating the use of ISI as adjuvant treatment in anastomotic esophageal strictures in children were included in the systematic review to assess the effectiveness of the intervention. RESULTS: The literature search yielded 8,253 articles. A total of 57 studies were included, of which 55 for the safety and five for the effectiveness assessment. The overall complication rate was 7%, with a greater incidence of local complications compared to systemic complications. Six studies (with a total of 367 patients) evaluated adrenocorticotropic hormone and cortisol levels, of which four reported hypothalamic-pituitary axis suppression. Two children (0.6%) received replacement therapy and all patients recovered uneventfully. A mean number of 1.67 ISI were performed per esophageal atresia (EA) patient. A reduction of needed dilatations was seen after ISI, compared to the number of dilatations performed before the intervention (5.2 vs. 1.3). CONCLUSION: The insufficient data emphasized the need for further prospective and comparative studies. Results from this meta-analysis and systematic review address ISI as a safe and effective technique. Close clinical follow-up and growth curve evaluation are advisable in patients receiving ISI. SYSTEMATIC REVIEW REGISTRATION: PROSPERO, identifier: CRD42021281584.

Could a careful clinical examination distinguish physiologic phimosis from balanitis xerotica obliterans in children?

The diagnosis of balanitis xerotica obliterans (BXO) in children may be challenging, since clinical examination only could lead to an underestimation of its incidence. The aim of this retrospective and single-centre study is to assess the diagnostic performance of clinical examination, together with clinical history, in identifying BXO. Ninety-seven children underwent circumcision for phimosis from 2015 to 2019. Histology was routinely performed. Cohen's kappa coefficient, sensitivity, specificity, predictive values, likelihood ratios and accuracy of macroscopic appearance of the foreskin, steroid administration and past medical history were estimated. Forty-eight patients (50%) were affected by BXO; 31 of them (69%) presented with suggestive clinical signs. A strictured or whitish urethral meatus was detected during surgery in nine cases (19%); this was associated to allergic or immune diseases (p = 0.046). Foreskin appearance alone mildly correlated with histology (k = 0.494; p < 0.001) and it showed a diagnostic accuracy of 75%. The specificity and positive predictive value of abnormal macroscopic findings at examination, together with a positive clinical history for other allergic or immune diseases, and/or for balanitis, were 100% and the positive likelihood ratio was greater than 10. Conversely, sensitivity decreased to 4.5% (95% CI 0-11%).Conclusion: Foreskin appearance together with clinical history could predict BXO with certainty. However, since the absence of a positive medical history could not exclude the diagnosis, foreskin histology is still highly recommended. What is Known: • Occurrence of balanitis xerotica obliterans may be underestimated in children and it could lead to long-term complications. • The diagnostic accuracy of clinical examination is controversial. What is New: • Clinical signs together with patients' medical history present high specificity and positive predictive values but low sensitivity. • When suggestive clinical aspects are present, patient should be referred to surgery avoiding prolonged conservative treatment; and preputial histology is highly recommended.