Left extralobar pulmonary sequestration and a right aorto-topulmonary 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

duodenal hematoma and pancreatitis complicating endoscopic intestinal biopsy in a boy with noonan syndrome

Duodenal intramural hematoma is a rare condition, mostly described in children and young adults that can be a complication of duodenal biopsy, especially in patients with predisposing hemorrhagic diathesis. It can determine secondary pancreatitis because of ampullary hematoma. Noonan Syndrome (NS) is an autosomal dominant disorder characterized by short stature, typical facial dysmorphisms, congenital heart defects and other anomalies such as bleeding problems which have been reported in up to 55% of patients. We herein report a case of duodenal hematoma with pancreatitis developed after endoscopic biopsy in a boy who was initially suspected of having celiac disease on the base of his short stature and growth retardation. Afterwards a more careful past medical history collection and objective examination revealed characteristic features of NS which could have been picked-up in advance, thus avoiding an investigation, such as the duodenal endoscopic biopsy, which in NS patient is potentially more risky

Possible Approach to Esophageal Lung with Long Tracheobronchial Gap

Esophageal lung is a rare bronchopulmonary foregut malformation characterized by an anomalous origin of one of the main bronchi which arises from the esophagus. Less than 30 cases are reported in the literature. Therefore, there are no standardized guidelines for the treatment of this condition. We report a case of right esophageal lung diagnosed in a neonate. The patient was treated with thoracoscopic closure of the ectopic main bronchus in the neonatal period, followed by delayed pneumonectomy at 5 months of age. No prosthetic substitute was implanted in the ipsilateral hemithorax after pneumonectomy. The patient is now 4 years old and doing well, postpneumonectomy syndrome was never observed. Our strategy and the possible alternatives are discussed here

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

Endoscopic Surveillance of Esophageal Atresia Population according to ESPGHAN-NASPGHAN 2016 Guidelines: Incidence of Eosinophilic Esophagitis and New Histological Findings

Follow-up of children born with esophageal atresia (EA) is mandatory due to high incidence of comorbidities. We evaluated endoscopic findings at follow-up of EA patients performed at our Centre according to ESPGHAN-NASPGHAN 2016 guidelines. A retrospective observational study was performed using data from January 2016 to January 2021. We included EA patients (age range: 1–18 years) who were offered a program of endoscopic and histological high gastrointestinal (GI) tract examinations as per ESPGHAN-NASPGHAN 2016 guidelines. Clinical, surgical, auxological, endoscopic, and histological data were reviewed; variables as polyhydramnios, EA type, surgical type, enteral feeding introduction age, growth data, and symptoms were correlated to endoscopic and histological findings. The population included 75 patients (47 males), with mean age of 5 ± 4 years. In 40/75 (53.3%) patients, we recorded oral feeding problems, and upper gastrointestinal or respiratory symptoms suspicious of gastroesophageal reflux. Eosinophilic esophagitis (EoE) incidence was 9/75 (12%), significantly higher than in general population (p < 0.0001), and 10/75 (13.3%) presented non-specific duodenal mucosal lesions. EoE represents a frequent comorbidity of EA, as previously known. EA is also burdened by high, never-described incidence of non-specific duodenal mucosal lesions. Embedding high GI tract biopsies in EA endoscopic follow-up should be mandatory from pediatric age

Aortopexy for Tracheomalacia in Children: A Systematic Review and Meta-Analysis

Introduction: Aortopexy is the most common surgical option for the treatment of severe tracheomalacia (TM) in children. Despite being described over 75 years ago, several aspects of the procedure remain controversial. Materials and Methods: A systematic review of aortopexy was conducted following the PRISMA guidelines. All original articles describing at least one case of aortopexy performed in children for the treatment of TM were included. Patients’ characteristics, surgical indications, approaches and details were collected. Outcomes were reviewed, and potential factors associated with procedural success were investigated. Results: Of 243 papers, 17 were included in this review, encompassing a total of 473 patients who underwent aortopexy. Of these, 65.3% were male, with a mean age at surgery of 12.2 months (5 days–18 years). Primary TM accounted for 7.9% of cases, while 92.1% were secondary to other anomalies, including esophageal atresia (54.1%), aberrant innominate artery (16.1%) and vascular rings (8.3%). There is a general consensus on the surgical indication for TM with severe symptoms, although the definition of severity is not well established. Overall success was 84%, but 40.8% of patients experienced some persistent symptoms. Sternotomy and thoracotomy were the most successful approaches (92.6% and 84.2%, respectively). Thoracoscopy had a similar success rate to sternotomy when pericardiotomy, thymectomy and pledgeted suture were performed (86.4% vs. 92.6% p = 0.41). Conclusions: Aortopexy is associated with good outcomes, but no evidence-based guidelines are currently available. Success appears to be associated with specific surgical steps rather than the surgical approach. Prospective studies are desirable for establishing precise guidelines

Respiratory morbidity in children with repaired congenital esophageal atresia with or without tracheoesophageal fistula

Congenital esophageal atresia with or without tracheoesophageal fistula (CEA ± TEF) is a relatively common malformation that occurs in 1 of 2500-4500 live births. Despite the refinement of surgical techniques, a considerable proportion of children experience short- and long-term respiratory complications, which can significantly affect their health through adulthood. This review focuses on the underlying mechanisms and clinical presentation of respiratory morbidity in children with repaired CEA ± TEF. The reasons for the short-term pulmonary impairments are multifactorial and related to the surgical complications, such as anastomotic leaks, stenosis, and recurrence of fistula. Long-term respiratory morbidity is grouped into four categories according to the body section or function mainly involved: upper respiratory tract, lower respiratory tract, gastrointestinal tract, and aspiration and dysphagia. The reasons for the persistence of respiratory morbidity to adulthood are not univocal. The malformation itself, the acquired damage after the surgical repair, various co-morbidities, and the recurrence of lower respiratory tract infections at an early age can contribute to pulmonary impairment. Nevertheless, other conditions, including smoking habits and, in particular, atopy can play a role in the recurrence of infections. In conclusion, our manuscript shows that most children born with CEA ± TEF survive into adulthood, but many comorbidities, mainly esophageal and respiratory issues, may persist. The pulmonary impairment involves many underlying mechanisms, which begin in the first years of life. Therefore, early detection and management of pulmonary morbidity may be important to prevent impairment in pulmonary function and serious long-term complications. To obtain a successful outcome, it is fundamental to ensure a standardized follow-up that must continue until adulthood

Respiratory morbidity in children with repaired congenital esophageal atresia with or without tracheoesophageal fistula

Congenital esophageal atresia with or without tracheoesophageal fistula (CEA \ub1 TEF) is a relatively common malformation that occurs in 1 of 2500-4500 live births. Despite the refinement of surgical techniques, a considerable proportion of children experience short- and long-term respiratory complications, which can significantly affect their health through adulthood. This review focuses on the underlying mechanisms and clinical presentation of respiratory morbidity in children with repaired CEA \ub1 TEF. The reasons for the short-term pulmonary impairments are multifactorial and related to the surgical complications, such as anastomotic leaks, stenosis, and recurrence of fistula. Long-term respiratory morbidity is grouped into four categories according to the body section or function mainly involved: upper respiratory tract, lower respiratory tract, gastrointestinal tract, and aspiration and dysphagia. The reasons for the persistence of respiratory morbidity to adulthood are not univocal. The malformation itself, the acquired damage after the surgical repair, various co-morbidities, and the recurrence of lower respiratory tract infections at an early age can contribute to pulmonary impairment. Nevertheless, other conditions, including smoking habits and, in particular, atopy can play a role in the recurrence of infections. In conclusion, our manuscript shows that most children born with CEA \ub1 TEF survive into adulthood, but many comorbidities, mainly esophageal and respiratory issues, may persist. The pulmonary impairment involves many underlying mechanisms, which begin in the first years of life. Therefore, early detection and management of pulmonary morbidity may be important to prevent impairment in pulmonary function and serious long-term complications. To obtain a successful outcome, it is fundamental to ensure a standardized follow-up that must continue until adulthood

Surgical expertise in neonatal extracorporeal membrane oxygenation (ECMO) : A single center experience

Introduction: The surgical technique for peripheral cannulation aimed at providing extracorporeal membrane oxygenation (ECMO) is well described. Training methods for surgeons still need proper standardization, especially in newborn patients. This study aims to evaluate the surgical training outcomes of a neonatal ECMO team. Materials and Methods: A 4 year training program (2014–2018) was developed to achieve the skills in the surgical technique for neonatal veno-arterial ECMO. Surgeons with experience in neonatal and vascular surgery were selected for the training. The training consisted of educational sessions, high-fidelity simulations, in vivo swine model procedures, international fellowship, and periodical simulations. The preliminary clinical experience in surgical neonatal ECMO management (2016-present) was analyzed by recording the following data: indications for ECMO and patients’ data; effectiveness of cannulations (number; perioperative complications of cannulation; major surgical events during ECMO); efficacy of decannulation (number and perioperative complications). Results: 12 neonates (5 females) fitted the ELSO criteria for ECMO. Nine newborns were affected by CDH; 1 by H1N1 flu-related pneumonia; 1 by meconium aspiration syndrome and one by Respiratory Syncytial Virus related bronchiolitis. Mean weight at cannulation was 3,281 g (range 2,330–3,840 g); mean gestational age was 36 weeks. No procedure was aborted, and no intra-operatory mortality was recorded. Mean operative time was 86 ± 30 min. The caliber of the carotideal cannulas ranged from 8F (8 patients) to 10F (2 patients); the caliber of the jugular cannulas were: 8F cannula (2 patients), 10F (6 patients), and 12F (2 patients). Four complications occurred: a case of air in the circuit, two cases of azygous vein cannulation and a partial dislocation of the venous cannula during the daily care maneuvers. All of them were promptly recognized and successfully treated. The mean ECMO duration was 7.1 ± 4.2 days (range 2–16 days). Seven patients (78%) were decannulated effectively. Mean decannulation time was 53 min (range 45–80 min). No complications occurred during the decannulation process. No ECMO–related deaths were recorded. Conclusions: Neonatal respiratory ECMO still represents a challenge. Experienced neonatal surgeons can manage the neck vascular cannulation. The codified procedure must be adhered to after appropriate training and following a proper learning curve