Diagnosis and Management of Congenital H-Type Tracheoesophageal Fistula: Results of a National Survey.

BACKGROUND: Congenital h-type tracheoesophageal fistula (H-TEF) without esophageal atresia (EA) represents about 4% of congenital esophageal anomalies. The diagnosis is challenging, and surgery is considered curative. The aim was to report a national survey on the diagnosis, management, and outcome of patients with congenital H-TEF. METHODS: Following approval of the Italian Society of Pediatric Surgery, a survey was sent to all Pediatric Surgery Units to retrospectively collect H-TEF treated in the period 2010-2022. Descriptive analysis was performed, and results are given as prevalence, mean ± standard deviation (SD), or median and interquartile range (IQR). RESULTS: The survey was sent to 65 units. Seventeen responded with one or more cases; 78 patients were diagnosed with H-TEF during the study period. Associated malformations were present in 43%, mostly cardiac (31%). The most frequent symptoms were cough (36%), bronchopneumonia (24%), and dysphagia (19%). H-TEF was detected by tracheobronchoscopy (90%), and/or upper GI (58%), and/or esophagoscopy (32%). The median age at diagnosis was 23 days (1 day-18 years). The most common approach was cervicotomy (76%), followed by thoracoscopy (14%) and thoracotomy (9%). The fistula underwent ligation and section of the fistula in 90% of the patients and clip closure and section in 9%. In one patient, the fistula was cauterized endoscopically. H-TEF preoperative cannulation was performed in 68% of cases, and a drain was placed in 26%. One month after surgery, 13% of the patients had mild persisting symptoms, mainly hypophonia. Recurrence occurred in 5%, and a second recurrence occurred in 1%. CONCLUSIONS: H-TEF prevalence was six cases/year, consistent with the expected rate of five cases/year in our country. The diagnosis was challenging, sometimes delayed, and, in most patients, required multiple examinations. Fistula ligation and section through cervicotomy were the most frequent treatment. Long-term outcomes are good, and recurrence is a rare event.

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.

Image-guided core-needle or surgical biopsy for neuroblastoma diagnosis in children: A systematic review and meta-analysis from the International Society of Pediatric Surgical Oncology (IPSO).

BACKGROUND: Image-guided core-needle biopsy (IGCNB) is a widely used and valuable clinical tool for tissue diagnosis of pediatric neuroblastoma. However, open surgical biopsy remains common practice even if children undergo more invasive and painful procedures. This review aims to determine the diagnostic accuracy and safety of IGCNBs in pediatric patients with neuroblastoma. METHODS: We conducted a systematic review of peer-reviewed original articles published between 1980 and 2023, by searching "pediatric oncology," "biopsy," "interventional radiology," and "neuroblastoma." Exclusion criteria were patients older than 18 years, studies concerning non-neurogenic tumors, case reports, and language other than English. Both the systematic review and meta-analysis were conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. RESULTS: A total of 533 abstracts articles were analyzed. Of these, eight retrospective studies met inclusion criteria (490 infants, 270 surgical biopsies [SB], 220 image-guided biopsies). Tissue adequacy for primary diagnosis (SB: n = 265, 98%; IGCNB: n = 199, 90%; p = .1) and biological characterization (SB: n = 186, 95%; IGCNB: n = 109, 89%; p = .15) was similar with both biopsy techniques, while intraoperative transfusion rate (SB: n = 51, 22%; IGCNB: n = 12, 6%; p = .0002) and complications (%) (SB: n = 58, 21%; IGCNB: n = 14, 6%; p = .005) were higher with surgical biopsy. Length of stay was similar in both groups; however, no additional data about concurrent diagnostic or treatment procedures were available in the analyzed studies. CONCLUSIONS: IGCNB is a safe and effective strategic approach for diagnostic workup of NB and should be considered in preferance to SB wherever possible.

Stomach engineering: region-specific characterization of the decellularized porcine stomach.

PURPOSE: Patients affected by microgastria, severe gastroesophageal reflux, or those who have undergone subtotal gastrectomy, have commonly described reporting dumping syndromes or other symptoms that seriously impair the quality of their life. Gastric tissue engineering may offer an alternative approach to treating these pathologies. Decellularization protocols have great potential to generate novel biomaterials for large gastric defect repair. There is an urgency to define more reliable protocols to foster clinical applications of tissue-engineered decellularized gastric grafts. METHODS: In this work, we investigated the biochemical and mechanical properties of decellularized porcine stomach tissue compared to its native counterpart. Histological and immunofluorescence analyses were performed to screen the quality of decellularized samples. Quantitative analysis was also performed to assess extracellular matrix composition. At last, we investigated the mechanical properties and cytocompatibility of the decellularized tissue compared to the native. RESULTS: The optimized decellularization protocol produced efficient cell removal, highlighted in the absence of native cellular nuclei. Decellularized scaffolds preserved collagen and elastin contents, with partial loss of sulfated glycosaminoglycans. Decellularized gastric tissue revealed increased elastic modulus and strain at break during mechanical tensile tests, while ultimate tensile strength was significantly reduced. HepG2 cells were seeded on the ECM, revealing matrix cytocompatibility and the ability to support cell proliferation. CONCLUSION: Our work reports the successful generation of acellular porcine gastric tissue able to support cell viability and proliferation of human cells.

Role of coagulation tests in the management of acute appendicitis in children.

BACKGROUND: Acute appendicitis (AA) is one of the most common acute surgical conditions in children. Coagulation tests (CoTs) are usually utilized in preoperative assessment to rule out hemorrhagic risks. Our study aimed to evaluate the role of CoTs as predictors for the severity of AA. METHODS: In a retrospective study, we compared the blood tests of two cohorts of pediatric patients with AA (group A and B) evaluated in the Emergency Department of a Pediatric Tertiary care hospital between January 2017 and January 2020. Children in Group A underwent appendectomies while those in Group B were treated with conservative management per hospital protocol. Group A was then subdivided into non-complicated (NCA) and complicated appendicitis (CA), and the CoTs were compared between the two subgroups. RESULTS: Group A consisted of 198 patients and Group B of 150 patients. Blood tests, including CoTs and inflammatory markers, were compared between the 2 groups. We found a statistically significant difference in PT ratio mean value between Group A and B, suggesting that those who underwent appendicectomies had higher PT ratio values. From a pathophysiological point of view, we speculated that the variation of PT ratio in AA might be secondary to a vitamin K absorption deficit due to enteric inflammation. CONCLUSIONS: Our study underlined that a longer PT ratio could be helpful to distinguish CA from NCA. Further investigations may lead to the role of the PT ratio in the choice between conservative and surgical management.

Comparison of outcomes following radiological reduction of intussusception with or without sedation.

BACKGROUND: Non-operative radiological reduction (NORR) is usually the first line treatment in pediatric ileo-colic intussusception. The aim of our study was to compare outcomes of NORR with or without sedation. METHODS: All patients undergoing to contrast enema NORR for intussusception between 01.01.2015 to 31.12.2020 in two hospitals were included: in one centre patients were sedated (A) while in the other patients were awake (B). Primary outcome was the rate of radiological reduction. Secondary outcomes were length-of-stay, complications and recurrence rate. RESULTS: Seventy-seven and 49 patients were included in group A and B respectively. Successful reduction rate was 72.7% in group A and 61.2% in group B (P>0.05). There were no complications related to the procedure among the 2 groups. Adverse events to sedation were observed in 3 patients. CONCLUSIONS: NORR has similar success rate when performed under sedation or awake, despite the former being graved by additional anesthesiologic risks and thus warrant careful indications.

Regenerative medicine: current research and perspective in pediatric surgery.

The field of regenerative medicine, encompassing several disciplines including stem cell biology and tissue engineering, continues to advance with the accumulating research on cell manipulation technologies, gene therapy and new materials. Recent progress in preclinical and clinical studies may transcend the boundaries of regenerative medicine from laboratory research towards clinical reality. However, for the ultimate goal to construct bioengineered transplantable organs, a number of issues still need to be addressed. In particular, engineering of elaborate tissues and organs requires a fine combination of different relevant aspects; not only the repopulation of multiple cell phenotypes in an appropriate distribution but also the adjustment of the host environmental factors such as vascularisation, innervation and immunomodulation. The aim of this review article is to provide an overview of the recent discoveries and development in stem cells and tissue engineering, which are inseparably interconnected. The current status of research on tissue stem cells and bioengineering, and the possibilities for application in specific organs relevant to paediatric surgery have been specifically focused and outlined.

A case of vanishing gastroschisis with short bowel syndrome treated by total parenteral nutrition and intestinal lengthening procedures.

The diagnosis of vanishing gastroschisis is made when in the presence of a full-thickness intrauterine abdominal wall defect the eviscerated loops are incarcerated in the fascial interruption. Four types of vanishing gastroschisis are described (A-D). We report on the case of a newborn with a vanishing gastroschisis-D. Gastroschisis was diagnosed at the 19th week of gestation, confirmed at the 30th, when the herniated loops previously visible to the right of the funiculus were no longer visualized. At the 32nd week, delivery was induced. The neonate weighed 1600 g, and the abdomen was distended, free from skin defects. On surgical exploration, the jejunum was 13 cm in length, with a blind ending. The post-atretic intestine measured 22 cm. A jejunostomy and a colostomy were built. The child received total parenteral nutrition for 13 months due to short bowel syndrome and was then subjected to intestinal lengthening procedure when she was 18 months old. Vanishing gastroschisis is a rare entity with a worse prognosis of the "classic" gastroschisis.

Endobronchial Inflammatory Myofibroblastic Tumor in a 3-Year-Old Child.

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal tumor that can occur at any age. However, it is primarily seen in children, with the most common site being in the lung parenchyma, usually present with rare endobronchial lesions. This case reports the incidence in a 3-year-old girl diagnosed with pericardiac pneumonia treated with antibiotics with no clinical improvement. A chest computed tomography (CT) scan identified a 1.5-cm lesion in the left main bronchus. Bronchoscopy revealed complete obstruction of the left main stem bronchus. A left posterolateral thoracotomy was performed. Additionally, a left sleeve upper bronchial resection was conducted under fibroendoscopic control. Definitive histology confirmed IMT. After 2 years of endoscopic follow-up, there is no evidence of recurrence.

Congenital Diaphragmatic Hernia: Perinatal Prognostic Factors and Short-Term Outcomes in a Single-Center Series.

Background: Many prognostic factors for CDH patients are described and validated in the current literature: the size of diaphragmatic defects, need for patch repair, pulmonary hypertension and left ventricular dysfunction are recognized as the most influencing outcomes. The aim of this study is to analyze the influence of these parameters in the outcome of CDH patients in our department and identify any further prognostic factors. Methods: An observational retrospective single-center study was conducted including all patients treated at our centre with posterolateral CDH between 01.01.1997 and 12.31.2019. The main outcomes evaluated were mortality and length of hospital stay. A univariate and multivariate analysis was performed. Results: We identified 140 patients with posterolateral CDH; 34.8% died before discharge. The overall median length of stay was 24 days. A univariate analysis confirmed that both outcomes are associated with the size of diaphragmatic defects, need for patch repair and presence of spleen-up (p < 0.05). A multivariate analysis identified that the need for patch repair and maximum dopamine dose used for cardiac dysfunction are independent parameters associated with the length of stay only (p < 0.001). Conclusions: In our series, the duration of hospitalization is longer for newborns with CDH treated with higher doses of dopamine for left ventricular dysfunction or needing patch repair in large diaphragmatic defects.

Endoscopic Treatment of a Severe Vaginal Stenosis Following Battery Insertion in an 11-Year-Old Girl.

Acquired vaginal strictures are rare entities in children. As a result, they are generally difficult to manage and tend to recur despite appropriate initial therapy. This case study reports the staged management of vaginal stenosis following the insertion of a button battery. In this case, an 11-year-old girl experienced at 4 years old a battery insertion in the vaginal canal by her neighbor's son, who was 6-year-old at the time. Two weeks from insertion, the parents noted the foreign body discharge spontaneously. The girl had not complained of any symptoms at the time and had been asymptomatic for many years. In November 2020, she came to the emergency department reporting cramping abdominal pain accompanied by mucopurulent discharge. An abdominal ultrasound showed the presence of hematometrocolpos, and a vaginal stenosis dilation under general anesthesia was performed the following day. After 3 weeks, the stenosis was still present, preventing the passage of Hegar number 4. The girl was subjected to a vaginoscopic stenosis resection utilizing a monopolar hook passed through an operative channel. A Bakri catheter filled with 120 mL of water was left in place. After 10 days, the girl was discharged home with the Bakri inserted. Two weeks after discharge, she was reevaluated in the outpatient setting, where the Bakri was removed with no signs of residual stenosis. Acquired vaginal stenosis could be demanding to treat, particularly with the sole conservative approach. A first-line option can be the Hegar dilation. The endoscopic approach can be a second-line, minimally invasive treatment, but long-term outcomes are difficult to predict.

Eosinophilic Esophagitis in Esophageal Atresia: Is It Really a New Disease?

Eosinophilic esophagitis (EoE) is a chronic, immune-mediated esophageal disease. Symptoms are related to mucosal eosinophilic-predominant inflammation that leads to esophageal dysfunction. Recent data suggest that esophageal atresia (EA) patients may have an increased incidence of EoE compared to the general population. As EoE symptoms may be confused with EA-related symptoms, they may significantly worsen morbidity in this specific group of patients. We investigated specific characteristics of patients with AE and EoE compared to those with EoE only. We conducted an observational retrospective monocentric study including all patients diagnosed with EoE from 1 January 2010 to 31 December 2021. For each patient, demographic, clinical and histopathological data were collected and then compared between the two cohorts (EA-EoE vs. EoE only). During the study period, 62 patients were included: 17 children were in the follow-up because of EA (18.1% of 94 EA patients screened in that period), while the other 45 presented EoE only. The demographic and clinical features of EA-EoE patients demonstrate a lower prevalence of allergic subjects (23.5% vs. 80%, p < 0.05), a lower age of presentation (3.1 vs. 12.2 years, p < 0.05), non-specific symptoms and a higher resolution rate with PPI therapy (64.7% vs. 17.8%, p < 0.05) compared to EoE-only patients. Our data confirm that EA patients are at high risk for developing EoE. As symptoms may overlap with the EA spectrum, early recognition of EoE may prevent patients from receiving unnecessary invasive therapeutic interventions and from developing complications from untreated EoE.

Testicular torsion during the COVID-19 pandemic: Results of a multicenter study in northern Italy.

INTRODUCTION: The literature reported an increased avoidance of the Emergency Department (ED) during COrona VIrus Disease 19 (COVID-19) pandemic, causing a subsequent increase of morbidity and mortality for acute conditions. Testicular torsion is a surgical emergency, which can lead to the loss of the affected testicle if a delayed treatment occurs. As testicular loss is time-related, outcome was hypothesized to be negatively affected by the pandemic. OBJECTIVE: The aim is to investigate whether presentation, treatment and outcomes of children with testicular torsion were delayed during COVID-19. STUDY DESIGN: Medical records of pediatric patients operated for testicular torsion of six Paediatric Surgical Units in Northern Italy between January 2019 and December 2020 were retrospectively reviewed. Patients were divided as for ones treated during (dC) or before the pandemic (pC). To reflect possible seasonality, related to lockdown restrictions, winter and summer calendar blocks were also analysed. For all cohorts, demographic data, pre-operative evaluation, operative notes and post-operative outcomes were reviewed. Primary outcomes were referral time, time from diagnosis to surgery and ischemic time, while secondary outcomes were orchiectomy and atrophy rates. Statistic was conducted as appropriate. RESULTS: A total of 188 patients with acute testicular torsion were included in the study period, 89 in the pre-COVID-19 (pC) period and 99 during COVID-19 (dC). Time from symptom onset to the access to the Emergency Department (T1) was not different among the two populations (pC: 5,5 h, dC: 6 h, p 0.374), and similarly time from diagnosis to surgery (pC: 2,5 h, dC: 2,5 h, p 0.970) and ischemic time (pC: 8,2 h, dC: 10 h, p 0.655). T1 was <6 h in 46/99 patients (46%) pC and 45/89 patients (51%) dC (p = 0.88, Fisher's exact test). Subgroup analysis accounting for different lockdown measures, confirm the absence of any difference. Orchiectomies rate was 23% (23/99) dC and 21% (19/89) pC (p = 0.861, Fisher's exact test) and rate of post-operative atrophy was 9% dC (7/76) and 14% pC (10/70), p = 0,44, Fisher's exact test. DISCUSSION: Despite worldwide pediatric ED accesses reduction, we reported that neither ischemic time nor the long-term outcomes in children with testicular torsion increased during the COVID-19 pandemic. In the available literature, few studies investigated the topic and are controversial on the results. Similarly to our findings, some studies found that timing and orchiectomy rates were not significantly different during the pandemic, while others reported a correlation to pandemic seasonality. Furthermore, in the recent pediatric literature it has been reported a delayed testicular torsion diagnosis due to shame in informing parents. Strengths of this study are the large numerosity, its multicentric design and a long study period. Its main limitation is being retrospective. CONCLUSIONS: We reported our large cohort from one of the most heavily COVID-19-affected regions, finding that referral, intra-hospital protocols and ischemic time in testicular torsion were not increased during to the pandemic, as well as orchiectomy rate and atrophy.

Intussusception and COVID-19 in Infants: Evidence for an Etiopathologic Correlation.

Nonrespiratory conditions related to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infections have been largely described. Ileocolic intussusception has been reported in association with SARS-CoV-2 infection in 10 children, raising the possibility of an etiopathologic role for the virus, but none of these cases documented tissue pathology that would have supported SARS-CoV-2 intestinal inflammation. We report 2 cases of intussusception in patients with SARS-CoV-2 infection who were treated at different pediatric tertiary centers in Europe and provide evidence of the presence of the virus in mesenteric and intestinal tissues of the patients.

SARS-CoV-2 infection and replication in human gastric organoids.

COVID-19 typically manifests as a respiratory illness, but several clinical reports have described gastrointestinal symptoms. This is particularly true in children in whom gastrointestinal symptoms are frequent and viral shedding outlasts viral clearance from the respiratory system. These observations raise the question of whether the virus can replicate within the stomach. Here we generate gastric organoids from fetal, pediatric, and adult biopsies as in vitro models of SARS-CoV-2 infection. To facilitate infection, we induce reverse polarity in the gastric organoids. We find that the pediatric and late fetal gastric organoids are susceptible to infection with SARS-CoV-2, while viral replication is significantly lower in undifferentiated organoids of early fetal and adult origin. We demonstrate that adult gastric organoids are more susceptible to infection following differentiation. We perform transcriptomic analysis to reveal a moderate innate antiviral response and a lack of differentially expressed genes belonging to the interferon family. Collectively, we show that the virus can efficiently infect the gastric epithelium, suggesting that the stomach might have an active role in fecal-oral SARS-CoV-2 transmission.

Incidental Ultrasound Diagnosis of Neonatal Intussusception Secondary to Meckel's Diverticulum in a Neurologically Impaired Child.

BACKGROUND Intussusception is the most common cause of intestinal obstruction in children, with a peak incidence usually before the second year of age, while in neonates it is a rare entity. We describe a delayed and incidental diagnosis of neonatal intussusception secondary to Meckel's diverticulum in a neonate with shaken baby syndrome (SBS). This is, to the best of our knowledge, the first reported case of a neonatal intussusception with a Meckel's diverticulum as a lead point in a neurologically impaired child. CASE REPORT A term baby presented at 22 days of age at our Emergency Department in severe conditions due to a suspected SBS. Eight days following hospitalization in the Intensive Care Unit, an isolated episode of rectal bleeding occurred, without any worsening of general conditions or abdominal distension. The ultrasonography showed a "doughnut sign" with high suspicion of ileocecal intussusception. A rectal barium contrast enema was performed but was not resolutive. At exploratory laparotomy an ileocecal intussusception with Meckel's diverticulum acting as a lead point was identified and an intestinal resection was needed due to the ischemic condition of the ileum. The post-operative course was uneventful and the baby recovered well; the residual neurological impairment needed long-term follow-up. CONCLUSIONS Intussusception is a rare entity in neonates and, when severe neurological impairment is present, the diagnosis can be missed because of the compromised condition of the baby and the paucity of gastrointestinal manifestations. In addition, due to the high incidence of lead point in neonatal cases, we recommend reserving non-operative treatment only for selected cases.

Laparo-endoscopic combination for the safe extraction of an open safety pin in a 9-month-old child. Case report.

INTRODUCTION: and importance: Accidental ingestion of foreign bodies (FBs) is common among infants. In case of sharp FBs, the risk of accidental organ damage with potential life-threatening complications constitutes an absolute indication for removal. We present the case of a child, who, following the ingestion of an open safety pin, was successfully treated exclusively with minimally invasive techniques. CASE PRESENTATION: A 9-month-old male patient was admitted for hematemesis. An anteroposterior and lateral X-ray of the thorax and abdomen revealed the presence of an open safety pin in the epi-mesogastric region, without a precise localization. Upper and lower gastrointestinal endoscopy, fluoroscopy, and laparoscopy were combined in the same intervention to localize and safely remove the foreign body. The patient was dismissed on a postoperative day 1. CLINICAL DISCUSSION AND CONCLUSION: The two main pitfalls of this scenario were the initially uncertain location of the foreign body and the young age of the patient. A combination of different techniques was used to safely locate and remove the foreign body, reducing hospitalization and avoiding repeated radiological exposure. An experienced team in a tertiary paediatric surgical and endoscopic centre increases the chances of success and minimizes invasiveness and the risk of complications.

Long-Term Analysis of Respiratory-Related Complications Following Gastrostomy Placement with or without Fundoplication in Neurologically Impaired Children: A Retrospective Cohort Study.

Gastrostomy placement is crucial in neurologically impaired (NI) children to ensure an adequate food intake and a safe route for drugs administration and to reduce the risk of primary aspiration. NI patents are more prone to gastroesophageal reflux. The association with fundoplication is deemed to reduce reflux-related respiratory complications. However, long-term benefits of this approach are not clear. We therefore aimed to compare long-term reflux-related respiratory complications of gastrostomy only (GO) to gastrostomy with fundoplication (GF). We retrospectively reviewed 145 consecutive NI children managed from 2008 to 2018. As long-term outcomes, we analyzed number and length of hospital admissions (Reflux-Related-Hospitalization, RRH) and emergency department accesses (Reflux-Related-Accesses, RRA) due to respiratory problems. Results were analyzed with appropriate statistical method. Median age at referral and at gastrostomy placement were 2.2 and 3.4 years (SD 5.6), respectively. Median follow-up was four years (range 1-12). Anti-reflux procedures were performed in 26/145 patients (18%); tracheotomy in 23/145 (16%). RRH following surgery showed lower number of admissions/year (0.32 vs. 1 for GO vs. GF, p < 0.005) and days hospitalization/year (3 vs. 13, p = 0.08) in GO compared to GF; RRA was similar (0.60 vs. 0.65, p = 0.43). Gastrostomy placement alone appeared not to be inferior to gastrostomy plus fundoplication with respect to long-term respiratory-related outcomes for NI children in our center.