Congenital Lung Malformations: Pulmonary Function Assessment Before and After Surgery.

BACKGROUND: The management of asymptomatic congenital lung malformations (CLM) is debated, and pulmonary function of patients with CLM is seldom discussed. Short-term respiratory outcome in patients operated for CLM was assessed in order to better define surgical impact. METHODS: A retrospective study on patients operated for CLM between 2012 and 2021 was performed. Respiratory function was assessed with tidal breath analysis (TBA) in spontaneous sleep within 2 years of life. Patients with comorbidities affecting pulmonary function were excluded. Three variables were studied: tPTEF/tE (time to peak tidal expiratory flow/total expiratory time), tV (tidal volume), RR (respiratory rate). Results were assessed as z-score (normal ±1.64) and expressed as median (range). Pre- and post-operative results were compared, as well as post-operative results of disease and surgery type subgroups. RESULTS: Thirty-nine patients were included. Median pre- and post-operative tPTEF/tE were normal (pre: -0.32 (-2.12; +1.56); post: -0.18 (-1.62; +2.43)). Patients with extralobar sequestration had pre-operative high median RR, which improved after sequestrectomy (pre: +1.99 (-2.49; +7.43); post -0.22 (-3.01; +1.18)). All groups had reduced pre-operative median tV, which restored after surgery (pre: -2.15 (-9.75; +0.91); post: -0.35 (-6.65; +3.14)). Patients undergoing lobectomy and patients with intralobar sequestration showed greater improvement. Median post-operative TBA results were normal for all sub-groups except for reduced tV in patients operated with thoracotomy (-2.27 (-6.12; +5.26)). CONCLUSIONS: Patients with clinically asymptomatic CLM can have pathologic pulmonary function tests, which improve after surgery. These results add to the elements in favor of an interventional approach. LEVEL OF EVIDENCE: III, Treatment study.

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.

Recurrence of Congenital Diaphragmatic Hernia: Risk Factors, Management, and Future Perspectives.

Recurrence is one of the most common surgical complications in Congenital Diaphragmatic Hernia (CDH). It could remain clinically silent for a long time or present as an acute complication week, months, or even years after the primary surgery. Several risk factors have been identified so far. An extended diaphragmatic defect represents one of the leading independent risk factors, together with indirect signs of large defect such as the liver position related to the diaphragm and the use of the prosthetic patch and with the use of a minimally invasive surgical (MIS) approach. However, the exact contribution of each factor and the overall risk of recurrence during the life span still need to be fully understood. This mini-review aims to give an overview of the current knowledge regarding CDH recurrence, focusing on predisposing factors, clinical presentation, management and follow-up of high-risk patients, and future perspectives.

Is Fetal Endoscopic Tracheal Occlusion (FETO) a Predisposing Factor for Acid Gastro-Esophageal Reflux in Infants With Congenital Diaphragmatic Hernia?

Introduction: Various anatomical defects predispose patients with congenital diaphragmatic hernia (CDH) to develop gastroesophageal reflux disease (GERD). The fetal endoscopic tracheal occlusion (FETO) has increased the survival of patients with severe CDHs. The aim of this study was to study GERD in patients who underwent FETO. Materials and Methods: We included patients with CDH treated with or without FETO ("FETO" and "no-FETO" group, respectively) from 2013 to 2016. Data on gestational age (GA), birth weight (BW), initial observed/expected lung to head ratio (O/E LHR), final O/E LHR, duration of ventilation and hospitalization, maximal tracheal diameter, and pulmonary volume were collected. All patients underwent pH-metry after 1 year of life, and the results were compared between groups and correlated to risk factors. Results: Thirty-two patients were included in the study: 10 FETO and 22 no-FETO. No significant differences were observed in the pH-metric results of the two groups. No correlation was found between GA, BW, initial O/E LHR, maximal tracheal diameter, pulmonary volume, and pH-metric results. pH-metric results were correlated with the total duration of ventilation (R = 0.5, p = 0.003) and of hospitalization (R = 0.54, p = 0.001). Gastric herniation is associated with the worse pH-metric result. Conclusions: The FETO procedure does not seem to represent an independent risk factor for GERD. However, patients with the most severe CDH have the worst GERD.

Combined Pre- and Postnatal Minimally Invasive Approach to Complicated Pulmonary Sequestrations.

Pulmonary sequestration (PS) is mostly asymptomatic but there is a proportion of fetuses that develop hydrops, leading to fetal or neonatal death. Fetal treatments are available, but postnatal management of the residual lesions is not uniformly defined. We present two cases of combined pre- and postnatal minimally invasive approach to complicated extra-lobar PS. Patient 1 presented with complicated PS at 31 weeks of gestation. Ultrasound-guided laser coagulation of the anomalous artery was successful. The patient was born asymptomatic at 38 weeks. Neonatal magnetic resonance imaging (MRI) showed a residual mass, confirmed by computed tomography (CT) at 6 months. No systemic artery was described, but perfusion was present. We decided for thoracoscopic resection. A residual artery was identified and sealed. Patient 2 presented with complicated PS at 25 weeks of gestation, underwent laser coagulation of the anomalous artery and was born asymptomatic at 38 weeks. Neonatal MRI showed persistence of the lesion, confirmed by CT scan at 4 months. We proceeded with thoracoscopic resection. A residual vessel was ligated. The patients 1 and 2 are now 24 and 21 months old, respectively, and healthy. Prenatal treatment of complicated PS is a life-saving procedure. Postnatal thoracoscopic resection of the residual lesion is feasible and safe; we believe it is the best course of treatment to grant the complete excision of the malformation.

Thoracoscopic Surgery for Congenital Lung Malformation Using Miniaturized 3-mm Vessel Sealing and 5-mm Stapling Devices: Single-Center Experience.

Aim: To evaluate the outcomes of thoracoscopic resection of congenital lung malformations (CLM) by using JustRight® (Bolder Surgical) 3-mm vessel sealing system and 5-mm stapler. Methods: Patients who underwent thoracoscopic resection of CLM in our Center from January 2016 to July 2019 were selected and divided into two groups: G1 (2018-2019), treated with the new JustRight instruments, and G2 (2016-2017), treated with Ligasure® and Hem-o-lok. Surgical outcomes in term of length of surgery and complications were compared between groups, stratifying the groups for type of resection (lobectomy, extralobar sequestration [ES] resection, and bronchogenic cyst [BC] resection). Results: Thirteen patients were included in G1, and 16 patients were included in G2. Seven lobectomies, 5 ES resections, and 1 BC resection were performed in G1; whereas 12 lobectomies, 3 ES resections, and 1 BC resection were performed in G2. Mean age at the time of surgery was 7.8 ± 6 months (G1) and 6.8 ± 3.3 months (G2) (P = .57). The average length of surgery was shorter for G1 (lobectomies: 120.7 ± 28.2 versus 171.7 ± 37.5, P = .006; ES resection: 63 ± 21.4 versus 91.7 ± 29.3, P = .15; BC 40 minutes versus 100 minutes). No significant difference was found for length of stay (4 ± 1 days versus 5 ± 2 days, P = .18). Neither the need for conversion nor major complications were observed in either group. Conclusion: In our experience, thoracoscopic resections of CLM with the new JustRight instruments were revealed to be safe and effective. On account of having adequate dimensions for small cavities, these instruments can facilitate the procedure and help to reduce the length of surgery.

Thoracoscopic Resection of Congenital Lung Malformation: Looking for the Right Preoperative Assessment.

INTRODUCTION: Consensus on the best postnatal radiological evaluation of congenital lung malformations (CLMs) is still lacking. In recent years, the interest on magnetic resonance imaging (MRI) has grown, but its role is still unknown. AIM: The aim of the study was to identify the best preoperative diagnostic assessment for CLM. MATERIALS AND METHODS: All patients with a prenatal suspicion of CLM between January 2014 and February 2018 were studied. Asymptomatic newborns underwent MRI, during spontaneous sleep without contrast. Patients with a positive MRI were scheduled for computed tomography (CT) within the fourth month of life. Thoracoscopic resection was performed in cases with a pathological CT. MRI, CT, and surgical findings were compared based on dimension, localization, and features of the CLM using the Cohen's kappa test (K). RESULTS: A total of 20 patients were included (10 males). No difference was found in the diameter and site of the lesions always localized in the same side (K = 1) and in the same pulmonary lobe (K = 1). Infants who underwent thoracoscopic resection included: three congenital pulmonary airway malformations (CPAMs), five extralobar and eight intralobar sequestrations (bronchopulmonary sequestrations [BPSs]), three bronchogenic cysts, and one congenital emphysema. The concordance between MRI and CT and between radiological investigations and pathology was satisfactory for the greatest part of the studied variables. MRI showed sensitivity of 100%, specificity of 82%, positive predictive value of 50% and negative predictive value of 100% for CPAM and 77, 100, 100, and 80% for BPS, respectively. CONCLUSION: MRI proved to be a reliable diagnostic investigation for CLM with high sensitivity and specificity. Early MRI in spontaneous sleep without contrast and preoperative contrast CT scan is a valuable preoperatory assessment.

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.

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.

Spleen histology in children with sickle cell disease and hereditary spherocytosis: hints on the disease pathophysiology.

Hereditary spherocytosis (HS) and sickle cell disease (SCD) are associated with splenomegaly and spleen dysfunction in pediatric patients. Scant data exist on possible correlations between spleen morphology and function in HS and SCD. This study aimed to assess the histologic and morphometric features of HS and SCD spleens, to get possible correlations with disease pathophysiology. In a large series of spleens from SCD, HS, and control patients, the following parameters were considered: (i) macroscopic features, (ii) lymphoid follicle (LF) density, (iii) presence of perifollicular marginal zones, (iv) presence of Gamna-Gandy bodies, (v) density of CD8-positive sinusoids, (vi) density of CD34-positive microvessels, (vii) presence/distribution of fibrosis and smooth muscle actin (SMA)-positive myoid cells, and (viii) density of CD68-positive macrophages. SCD and HS spleens had similar macroscopic features. SCD spleens had lower LF density and fewer marginal zones than did HS spleens and controls. SCD also showed lower CD8-positive sinusoid density, increased CD34-positive microvessel density and SMA-positive myoid cells, and higher prevalence of fibrosis and Gamna-Gandy bodies. HS had lower LF and CD8-positive sinusoid density than did controls. No significant differences were noted in red pulp macrophages. By multivariate analysis, most HS spleens clustered with controls, whereas SCD grouped separately. A multiparametric score could predict the degree of spleen changes irrespective of the underlying disease. In conclusion, SCD spleens display greater histologic effacement than HS, and SCD-related changes suggest impaired function due to vascular damage. These observations may contribute to guide the clinical management of patients.