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.

Congenital lung malformations: a nationwide survey on management aspects by the Italian Society of Pediatric Surgery.

INTRODUCTION: Over the years, congenital lung malformations (CLM) management remains a controversial topic in pediatric thoracic surgery. The Italian Society of Pediatric Surgery performed a national survey to study the current management variability among centers, trying to define national guidelines and a standardized approach of children with congenital lung malformations. METHODS: Following a National Society approval, an electronic survey including 35 items on post-natal management was designed, focusing on surgical, anesthesiology, radiology and pneumology aspects. The survey was conducted contacting all pediatric surgical units performing thoracic surgery. RESULTS: 39 pediatric surgery units (97.5%) participated in the study. 13 centers (33.3%) were classified as high-volume (Group A), while 26 centers (66.7%) were low volume (Group B). Variances in diagnostic imaging protocols were observed, with Group A performing fewer CT scans compared to Group B (p = 0.012). Surgical indications favored operative approaches for asymptomatic CLM and pulmonary sequestrations in both groups, while a wait-and-see approach was common for congenital lobar emphysema. Surgical timing for asymptomatic CLM differed significantly, with most high-volume centers operating on patients younger than 12 months (p = 0.02). Thoracoscopy was the preferred approach for asymptomatic CLM in most of centers, while postoperative long-term follow-up was not performed in most of the centers. CONCLUSION: Thoracoscopic approach seems uniform in asymptomatic CLM patients and variable in symptomatic children. Lack of uniformity in surgical timing and preoperative imaging assessment has been identified as key areas to establish a common national pattern of care for CLM.

Classic ketogenic diet in parenteral nutrition in a GLUT1DS patient: Doing more with less in an acute surgical setting.

Ketogenic Dietary Treatments (KDTs) are to date the gold-standard treatment for glucose transporter type 1 (GLUT1) deficiency syndrome. Administration of KDTs is generally per os; however, in some conditions including the acute gastro-enteric post-surgical setting, short-term parenteral (PN) administration might be needed. We report the case of a 14-year-old GLUT1DS patient, following classic KDT for many years, who underwent urgent laparoscopic appendectomy. PN-KDT was required, after 1 day of fasting. No ad hoc PN-KDTs products were available and the patient received infusions of OLIMEL N4 (Baxter). On the sixth day postoperatively enteral nutrition was progressively reintroduced. The outcome was optimal with rapid recovery and no exacerbation of neurological manifestations. Our patient is the first pediatric patient with GLUT1DS in chronic treatment with KDT efficiently treated with exclusive PN for five days. This case reports on real-word management and the ideal recommendations for PN-KDT in an acute surgical setting.

Withdrawn: Parenteral Nutrition in a GLUT1DS Patient Following Classic Ketogenic Diet: Ideal versus Real-World Management in an Acute Surgical Setting.

Withdrawal: C Varesio et al., Parenteral Nutrition in a GLUT1DS Patient Following Classic Ketogenic Diet: Ideal versus Real-World Management in an Acute Surgical Setting, Journal of Parenteral and Enteral Nutrition 2022 (https://doi.org/10.1002/jpen.2361). The above article, published online on March 1st, 2022 in Wiley Online Library (wileyonlinelibrary.com), has been withdrawn by agreement between the journal Editor-in-Chief, Kelly A. Tappenden, PhD, RD, FASPEN, and Wiley Periodicals LLC. The article was published as the result of an administrative error.

Globalization in Pediatric Surgical Training: The Benefit of an International Fellowship in a Low-to-Middle-Income Country Academic Hospital.

OBJECTIVE: A relative oversupply of pediatric surgeons led to increasing difficulties in surgical training in high-income countries (HIC), popularizing international fellowships in low-to-middle-income countries (LMIC). The aim of this study was to evaluate the benefit of an international fellowship in an LMIC for the training of pediatric surgery trainees from HICs. METHODS: We retrospectively reviewed and compared the prospectively maintained surgical logbooks of international pediatric surgical trainees who completed a fellowship at Chris Hani Baragwanath Academic Hospital in the last 10 years. We analyzed the number of surgeries, type of involvement, and level of supervision in the operations. Data are provided in mean differences between South Africa and the respective home country. RESULTS: Seven fellows were included. Operative experience was higher in South Africa in general (Δx̅ = 381; 95% confidence interval [CI]: 236-656; p < 0.0001) and index cases (Δx̅ = 178; 95% CI: 109-279; p < 0.0001). In South Africa, fellows performed more index cases unsupervised (Δx̅ = 71; 95% CI: 42-111; p < 0.0001), but a similar number under supervision (Δx̅ = -1; 95% CI: -25-24; p = 0.901). Fellows were exposed to more surgical procedures in each pediatric surgical subspecialty. CONCLUSION: An international fellowship in a high-volume subspecialized unit in an LMIC can be highly beneficial for HIC trainees, allowing exposure to higher caseload, opportunity to operate independently, and to receive a wider exposure to the different fields of pediatric surgery. The associated benefit for the local trainees is some reduction in their clinical responsibilities due to the additional workforce, providing them with the opportunity for protected academic and research time.

Mentoring in Pediatric Thoracoscopy: From Theory to Practice.

Introduction: Thoracoscopy represents the most challenging area of pediatric minimally invasive surgery due to its technical difficulty. A standardized training program would be advisable. The aim of this study is to evaluate the results of our surgical training. Materials and Methods: A retrospective, single-center, cohort study was performed. The following four-step program was tested: (1) theoretical part; (2) experimental training; (3) training in centers of reference; (4) personal operative experience. Particular attention was focused on the choice of mentor. Times and modality of adherence to the program were evaluated. The effectiveness and safety of the training were evaluated according to the surgical results of esophageal atresia (EA/TEF) repair and resection of congenital lung malformations (CLM). The study was conducted from January 2014 to May 2020. Attending surgeons with previous experience in neonatal and pediatric laparoscopy were selected for the training program after being evaluated by the head of Department. Results: The training program was fully completed in 2 years. Twenty-four lobectomies, 9 sequestrectomies, 2 bronchogenic cyst resections and 20 EA/TEF repair were performed. Thoracoscopy was always feasible and effective, with no conversion. The operative times progressively decreased. Only three minor complications were recorded, all treated conservatively. Conclusions: A standardized training program is highly desirable to learn how to safely perform advanced pediatric thoracoscopy. The 4-steps design seems a valid educational option. The choice of the mentor is crucial. An experience-based profile for pediatric surgeons who may teach thoracoscopy is advisable.

Bedside surgery in the newborn infants: survey of the Italian society of pediatric surgery.

INTRODUCTION: This is the report of the first official survey from the Italian Society of Pediatric Surgery (ISPS) to appraise the distribution and organization of bedside surgery in the neonatal intensive care units (NICU) in Italy. METHODS: A questionnaire requesting general data, staff data and workload data of the centers was developed and sent by means of an online cloud-based software instrument to all Italian pediatric surgery Units. RESULTS: The survey was answered by 34 (65%) out of 52 centers. NICU bedside surgery is reported in 81.8% of the pediatric surgery centers. A lower prevalence of bedside surgical practice in the NICU was reported for Southern Italy and the islands than for Northern Italy and Central Italy (Southern

Left extralobar pulmonary sequestration and a right aorto-to-pulmonary vein fistula in a newborn: a 3-mm thoracoscopic monolateral approach.

An extralobar pulmonary sequestration (EPS) associated with a contralateral aorto-to-pulmonary vein fistula is rare. We report the case of a female newborn with left EPS fed by an artery originating from the distal thoracic aorta and, symmetrically on the controlateral side, an artery shunting in the inferior right pulmonary vein. Echocardiography showed dilatation of the left atrium. On the 34th day since birth (weight 4500 g), the patient was operated on thoracoscopically. The EPS was closed with a 3-mm sealing system, divided and removed. A window in the mediastinal pleura was created, and the origin of the fistula was identified and sealed. The postoperative course was uneventful. The patient was discharged on Day 4 with no echocardiographic signs of persistence of the fistula and of the congestive heart failure. This is the first case report of a thoracic large systemic circulation-to-pulmonary vein fistula causing heart failure associated with EPS. The thoracoscopic monolateral approach and the availability of 3-mm instruments guaranteed a maximum level of minimal invasiveness.

Start a Neonatal Extracorporeal Membrane Oxygenation Program: A Multistep Team Training.

Background: Extracorporeal membrane oxygenation (ECMO) is a complex life-saving support for acute cardio-respiratory failure, unresponsive to medical treatment. Emergency events on ECMO are rare but require immediate and proficient management. Multidisciplinary ECMO team members need to acquire and maintain over time cognitive, technical and behavioral skills, to safely face life-threatening clinical scenarios. Methods: A multistep educational program was delivered in a 4-year period to 32 ECMO team members, based on guidelines from the Extracorporeal Life Support Organization. A first traditional module was provided through didactic lectures, hands-on water drills, and laboratory animal training. The second phase consisted of a multi-edition high-fidelity simulation-based training on a modified neonatal mannequin (SimNewB®). In each session, participants were called to face, in small groups, ten critical scenarios, followed by debriefing time. Trainees underwent a pre-test for baseline competency assessment. Once completed the full training program, a post-test was administered. Pre- and post-test scores were compared. Trainees rated the educational program through survey questionnaires. Results: 28 trainees (87.5%) completed the full educational program. ECMO staff skills improved from a median pre-test score of 7.5/18 (IQR = 6-11) to 14/18 (IQR = 14-16) at post-test (P < 0.001, Wilcoxon rank test). All trainees highly rated the educational program and its impact on their practice. They reported high-fidelity simulations to be beneficial to novice learners as it increased self-confidence in ECMO-emergencies (according to 100% of surveyed), theoretical knowledge (61.5%) and team-work/communicative skills (58%). Conclusions: The multistep ECMO team training increased staff' knowledge, technical skills, teamwork, and self-confidence, allowing the successful development of a neonatal respiratory ECMO program. Conventional training was perceived as relevant in the early phase of the program development, while the active learning emerged to be more beneficial to master ECMO knowledge, specific skills, and team performance.

Infant Percutaneous Endoscopic Gastrostomy: Risks or Benefits?

BACKGROUND/AIMS: To present a single center's experience with percutaneous endoscopic gastrostomy (PEG) tube placement in infants. METHODS: Clinical records of infants who underwent PEG tube placement between January 2010 and December 2015 were reviewed. All patients underwent an upper gastrointestinal contrast study and an abdominal ultrasonography before the procedure. PEGs were performed with a 6-mm endoscope using the standard pull-through technique. Data regarding gestational age, birth weight, age and weight, days to feeding start, days to full diet, and complications were reviewed. RESULTS: Twenty-three patients were included. The most common indication was dysphagia related to hypoxic-ischemic encephalopathy. Median gestational age was 37 weeks (range, 24-41) and median birth weight was 2,605 grams (560-4,460). Patients underwent PEG procedures at a median age of 114 days (48-350); mean weight was 5.1 kg (3.2-8.8). In all patients but one, a 12-Fr tube was positioned. Median feeding start was 3 days (1-5) and on average full diet was achieved 5 days after the procedure (2-11). Six minor complications were recorded and effectively treated in the outpatient clinic; no major complications were recorded. CONCLUSIONS: PEG is safe and feasible in infants when performed by highly experienced physicians.

Classification of Esophageal Strictures following Esophageal Atresia Repair.

INTRODUCTION: The aim of this study was to stratify anastomotic strictures (AS) following esophageal atresia (EA) repair and to establish predictors for the need of dilations. MATERIALS AND METHODS: A retrospective study on children operated on for EA between 2004 and 2014 was conducted. The stricture index (SI) was measured both radiologically (SIXR) and endoscopically (SIEND). A correlation analysis between the SI and the number of dilations was performed using Spearman's test and linear regression analysis. RESULTS: In this study, 40 patients were included: 35 (87.5%) presented with Gross's type C EA, 3 (7.5%) type A, 1 (2.5%) type B, and 1 (2.5%) type D. The mean follow-up time was 101 ± 71.1 months (range: 7.8-232.5, median: 97.6). The mean SIXR was 0.56 ± 0.16 (range: 0.15-0.86). The mean SIEND was 0.45 ± 0.22 (range: 0.15-0.85). Twenty-four patients (60%) underwent a mean of 2 endoscopic dilations (range: 1-9). The number of dilations was poorly correlated with SIXR, while significantly correlated with SIEND. Patients who did not need dilations had a SIEND < 0.33, patients who needed only one dilation had 0.33 ≤ SIEND < 0.44, and those with SIEND ≥ 0.44 needed two or more dilations. No significant association with other clinical variables was found. All patients were asymptomatic at the time of the first endoscopy. CONCLUSION: SIEND is a useful tool to classify AS and can represent a predictor of the need for endoscopic dilation. The role of the SIEND becomes even more important as clinical characteristics have a low predictive value for the development of an AS and the need for subsequent endoscopic esophageal dilatations.

Follow-up of Congenital Diaphragmatic Hernia: Need for Routinary Assessment of Acid Gastroesophageal Reflux with pH-metry.

INTRODUCTION: We aim to assess gastroesophageal reflux (GER) in patients treated for congenital diaphragmatic hernia (CDH) and to determine whether a pH-metry investigation should be routinely performed in follow-up. MATERIALS AND METHODS: Twenty-four-hour pH-metry at 1 year was performed in all patients treated for CDH between January 2014 and April 2015 (Group 1). We compared pH-metry results to those of two other groups: children treated for esophageal atresia (EA) (Group 2) and normal babies presenting with typical symptoms (Group 3). All the pH-metric findings were analyzed and compared. Intra-group analysis was performed in Group 1. RESULTS: Group 1 consisted of 21, Group 2 of 24, and Group 3 of 21 patients. Mean pH-metry values for Groups 1, 2, and 3 were, respectively: reflux index (RI) 4.3, 5.1, and 3.9; total number of refluxes (NR) 79.5, 88.8, and 88.7; refluxes longer than 5' (R > 5) 1.7, 2.3, and 1.47; and longest reflux episode (LR) 11.4, 13.3, and 8.6. No significant differences were found between Group 1 and the others. Only two CDH patients presented with GER-related symptoms. Patch was associated with significantly higher RI (8.5 vs. 2.98, p = 0.03). The worse was the defect, the worse were the pH-metric results (RI: A3.09, B3.15, and C9.1). CONCLUSION: We believe that a routine GER assessment should be performed in all CDH patients regardless the presence of symptoms. LEVEL OF EVIDENCE: This is a Level II study.

Severe Pneumonia Caused by Influenza A (H1N1) Virus Successfully Managed with Extracorporeal Life Support in a Comorbid Former Preterm Infant.

Influenza A (H1N1) virus infection is a global health burden, leading to significant pediatric morbidity and mortality. Prematurity, young age and comorbidities are important risk factors for unfavorable outcomes. Preventive strategies, such as healthcare workers and household contacts vaccination as well as the implementation of infection control practices during the epidemic season, are crucial to protect the most vulnerable populations. Early diagnosis, timely administration of antiviral drugs and supportive therapy are crucial to lead to a complete recovery. When conventional treatment fails, extracorporeal life support (ECLS) may be employed. In neonates and young infants, this high-tech support is burdened by specific technical complexity. Despite the potential risks related to this aggressive approach, ECLS is a life-saving procedure in 65% of pediatric viral pneumonia and in 73% of sepsis cases. Here, we report the successful outcome of a 51-day formerly preterm infant, suffering from a surgical necrotizing enterocolitis (NEC), complicated with hospital-acquired pneumonia due to influenza A (H1N1) virus. She developed a severe respiratory failure, unresponsive to conventional therapy, and successfully treated with ECLS. To our knowledge, this is the first report on the use of ECLS in a formerly preterm infant, suffering from NEC complicated by influenza A (H1N1) virus infection.

Acid Gastroesophageal Reflux Disease and Apparent Life-Threatening Events: Simultaneous pH-metry and Cardiorespiratory Monitoring.

AIM: To investigate the prevalence and the characteristics of gastroesophageal reflux disease (GERD) in infants with apparent life threatening events (ALTE). MATERIALS AND METHODS: Infants with at least one episode of ALTE in absence of predisposing factors were included. All infants underwent a cardiorespiratory recording with simultaneous 24-hour pH-monitoring. Patients were divided into 3 groups according to the severity of GERD: A. Reflux Index (RI) <3%, B. RI = 3-7%, C. RI >7%. Monthly evaluations were performed and the anti-reflux therapy was maintained till normalization of monitoring and clinic. RESULTS: 41 infants were enrolled. GERD was found in 80% of patients (moderate in 54%, severe in 27%). A normalization of the cardiorespiratory tracks was recorded on average after 1 month for group A, 7 months for the group B and 9.5 months for group C. A significant difference was registered between group A and both group B and C (P < 0.0001), as well as between the group B and C (P < 0.05). CONCLUSION: GERD influences significantly the time of normalization of the cardiorespiratory monitoring in infants with ALTE. GERD diagnosis and treatment are mandatory in these patients.

Fetoscopic Endoluminal Tracheal Occlusion in Fetuses with Severe Diaphragmatic Hernia: A Three-Year Single-Center Experience.

OBJECTIVE: To report on our experience in the prenatal treatment of severe congenital diaphragmatic hernia (CDH) by fetoscopic endoluminal tracheal occlusion (FETO). METHODS: Between 2012 and 2014, FETO was performed at our center in 21 cases of CDH considered to be severe based on sonographic measurement of observed/expected lung-to-head ratio (O/E LHR) and side of the defect. We reported pre- and postoperative ultrasound findings, procedure-related complications, pregnancy outcome and survival at 1-3 years of age. RESULTS: The median gestational age (GA) at balloon insertion was 28.1 weeks (range 26.0-31.1) and the median GA at delivery 34.7 weeks (range 31.6-39.0); delivery before 32 and 34 weeks occurred in 2 (9.5%) and 7 (33.3%) cases, respectively. Postnatal survival at 1-3 years of age in the 17 cases with isolated unilateral CDH was 47.1%. The percentage difference between pre-balloon removal O/E LHR and pre-FETO O/E LHR was significantly higher in survivors compared to neonates who died (40.8 vs. 21.2%, respectively; p < 0.05). CONCLUSIONS: In this study, FETO was associated with an infant survival of 47% in cases with isolated unilateral severe CDH. The post-FETO increase in O/E LHR was higher in fetuses that survived compared to those who died.

Endoscopic Surveillance for Congenital Diaphragmatic Hernia: Unexpected Prevalence of Silent Esophagitis.

Introduction Gastroesophageal reflux disease (GERD) is a frequent comorbidity after congenital diaphragmatic hernia (CDH) repair. Our aim was to evaluate the clinical and endoscopic prevalence of esophagitis in a long-term follow-up of CDH patients. Materials and Methods Patients operated on for posterolateral CDH and undergoing general anesthesia for concomitant pathologies between January and October 2013 were included in the study. GERD was investigated both clinically (Manterola questionnaire) and endoscopically. The severity of esophagitis was evaluated according to the Hetzel-Dent classification and multiple biopsies were performed. The correlation between clinical score and severity of esophagitis was evaluated. Results Twelve patients were included in the study (mean age: 14.5 years; range, 9-18 years). Only three children (25%) had a pathological questionnaire. At endoscopy, three children (25%) were affected by grade 1 esophagitis, six (50%) by grade 2, two (17%) by grade 3, and one (8%) by grade 4. One of the children presented Barrett esophagus. A moderate negative correlation was found between clinical data and endoscopic findings (r: -0.54 and p: 0.067). Conclusion Even in the absence of symptoms, esophagitis revealed to have a high prevalence in CDH patients. A long-term clinical and instrumental follow-up is mandatory to early diagnose and treat GERD.

Thoracoscopic removal of retained thoracoamniotic shunt catheters in newborns.

Fetal hydrothorax is associated with significant mortality. However, the development of fetal thoracoamniotic shunting has reduced the mortality rate. Fetal thoracoamniotic shunting can be characterized by significant complications, such as intrathoracic dislodgement of the catheter. The ideal management of dislodged catheters postnatally is not known. We report two newborns with a prenatal diagnosis of fetal hydrothorax who underwent thoracoamniotic shunting complicated by intrathoracic dislodgement of the catheters requiring thoracoscopic removal of the shunts in the neonatal period.

Oral viscous budesonide as a first-line approach to esophageal stenosis in epidermolysis bullosa: an open-label trial in six children.

BACKGROUND: Esophageal and pharyngeal problems are common in the majority of patients with epidermolysis bullosa (EB). Repeated blister formation and ulceration, coupled with chronic inflammation, result in scarring and development of esophageal strictures. OBJECTIVE: This study aimed to evaluate whether oral viscous budesonide (OVB) was useful for treating esophageal structures in six pediatric patients (aged 8-17 years) with EB who were affected by dysphagia and esophageal strictures. METHODS: Patients were treated for 4 months with twice-daily oral budesonide nebulizer solution 0.5 mg/2 mL mixed with maltodextrin 5 g and artificial sweeteners. RESULTS: One patient developed a severe oral mycotic infection and discontinued treatment. The other five patients completed the treatment regimen and displayed significantly lower stricture indices (SIs) post-treatment (mean SI ± standard deviation 0.736 ± 0.101 pre-treatment versus 0.558 ± 0.162 post-treatment; p = 0.008). Patients experienced a mean SI decrease of 0.178 (range 0.026-0.296), as well as improved dietary habits in the absence of side effects. CONCLUSION: These findings indicated that topical corticosteroids may significantly alleviate strictures in pediatric patients with EB, thereby limiting the need for endoscopic dilation and considerably improving patients' quality of life.